Yesterday I drove our happy-go-lucky, feeling-well Gracie girl into Oakland to have her pacemaker changed. No matter how many surgeries (this was #41) or how many times under general anesthesia (this was #29... not counting "procedural sedations"), we never, ever hand our daughter over without a pit in our stomachs. It just doesn't get easier. It also doesn't get easier trying to help her understand that she is going to sleep feeling well and will wake up in pain... or managed pain. That stinks beyond explanation. It always feels like such a betrayal to her. All I can do to remedy that is be by her side and do everything within my power to care for her, advocate for her and love on her.
I drove her in and John met us there. Before he arrived, the cardiac team - - Gracie's cardiologist, the CVICU nurse, the surgeon, the Medtronic rep and the anesthesiologist - - all gathered at her bedside and informed me that today she would likely be having open heart surgery to replace her ventricular lead. (The surgery, as scheduled, was to replace her pacemaker generator only. Since it is implanted in her left abdomen, it is a fairly "easy" surgery that doesn't actually involve the heart - - other than monitoring, of course. The leads, on the other hand, are implanted directly into the heart and run to the generator.) I nearly fainted. Really... I felt the beads of sweat instantly appear on my forehead, the blood rush from my face and my heart instantly raced. "We DID NOT sign up for open heart surgery today," was all I could think. I am SO NOT prepared... neither is John and, most certainly, not Gracie. They went on to explain that they noticed some "noise" in the testing on that lead and that they would run further tests prior to surgery, but that I needed to be prepared for them to also replace the lead. The leads are the originals placed in August of 2004. They are nearly ten years old and it makes perfect sense that there might be an issue... it's actually a question I ask all the time. "How much time do you think we have left on these leads?" They've never mentioned an issue... and certainly didn't mention one in our pre-op appointment. The answer has always been a version of, "In some ways she is so lucky that she doesn't grow much and that her activity level is not typical of a child her age, otherwise she would have outgrown her leads and her twice-repaired/re-built valve." I always take that comment as a good cardiac outcome, but really, would much rather her be more active, bigger and have needed one extra surgery to replace something she'd "outgrown." Anyway, it took another 30 minutes or so, but they finally determined that the need to replace the lead was "unlikely." Good grief almighty. I was exhausted at that point and they hadn't even finished prepping Gracie for the OR.
Then, the nurse laid another doozie on me... "Did you bring urine?" Me... still reeling from the massive surge in my blood pressure, "Um... urine? No...?" The nurse (super, duper sweet... lucky for her, because I was already done and we hadn't even started!) said, "We are supposed to get urine for any patient Gracie's age and older to check for pregnancy prior to anesthesia. If you didn't bring any, we will have to catheterize her." Oh. HECK. NO. This "policy" was news to me. It was also preposterous. And, while I am perfectly willing to bring in urine if I am told to, I am not willing to put Gracie through a catheterization while awake due to the negligence of the pre-op team who failed to provide me with this information. So, I told the sweet (again, lucky for her) nurse that they are welcome to catheterize her once she is out (which, of course, defeats their entire purpose) or they can forgo the urine and trust the fact that Gracie is not pregnant. She then earned double-sweet-stripes by fully supporting my decision - - not that I needed her approval, I was standing firm. But knowing she was in Gracie's corner made me feel so much better. Thankfully, after some bantering back and forth amongst the team and the hospital "powers," we (docs and I) signed additional disclosures and agreed to move forward without poking and prodding at Gracie for some potty (which I had also argued they'd be hard pressed to get considering she had been off fluids for hours!).
And then, after all of that chaos was settled, in saunters my husband. Seriously... the man missed it all! I had been through a virtual emotional roller coaster and he walks in as the dust all settled!! How does that even work?! Ha! All that matters, though, is that all of the hoopla prior to surgery was the only hoopla for the rest of the day. Surgery went well, they reconfirmed in the OR that they didn't need to replace her ventricular lead, and she spent one (long) night in the ICU. Children's Oakland has an open-floor ICU... something we are definitely not accustomed to. There are only curtains separating the beds, the lights cannot be dimmed enough for Gracie's liking and it is SO LOUD. The sound, of course, did not bother Gracie, but not being able to control the lighting around us was horrible. She just kept signing "light" over and over. I didn't really want to leave her side for a second, but I wasn't even allowed to have a water bottle at her bedside, so I was forced to hydrate outside the unit. I am just so thankful everything went well and she only had to be there one full day/night... because any longer and I might have gone nuts!
We are home now and Gracie is asleep. I am hopeful that her pain will be well managed with the meds we have in our makeshift hospital. She was very feisty on the drive home as well as when we arrived... all of her energy spent fixated on trying to remove her surgical dressing. The fear of infection is huge for us given her history, so keeping her little germy hands away is crucial. She understands that she is not supposed to touch the dressing, but she doesn't understand why - - not that it would make a big difference! She is determined to do what she wants to do whenever she wants to do it. This girl... the spunk and sass are what I love most and what are, many times, most challenging! For now, though, she sleeps and ignores that site.
Showing posts with label CHARGE. Show all posts
Showing posts with label CHARGE. Show all posts
Thursday, May 15, 2014
Friday, February 8, 2013
When NOT needing heart surgery is bittersweet
The title surely sounds ridiculous to anyone reading this. Who would ever actually hope for the news that another open heart surgery is needed for their child? When Gracie saw her cardiologist on Wednesday, I did… sort of. Granted, I don’t really want her to have surgery, but had the answer been that she needed it imminently due to further deteriorated heart function, it would have explained so much about certain things going on with her. Instead, it only further solidified the fact that her deteriorating gross-motor stamina (especially her lack of desire to walk any great distance) has very little cardiac component and is, sadly, more to do with a laundry list of other issues. As for her heart, she would have, if “otherwise typically active,” had a mitral valve replacement several years ago. She was born with an incomplete AV canal and has undergone a total of three open heart surgeries to date. She will need a fourth to replace her mitral valve - - the question is when. Her cardiologist will be presenting her records to the esteemed and well-loved, Dr. Hanley, who will weigh in on the matter.
Back to why I (sort of) hoped that her heart held more answers to the current issues… Gracie’s desire and ability to walk longer distances (longer for her… not actually very long) has decreased tremendously over the last several months. I posted about this a year ago:
Since we moved and I have been establishing her with an entire new team (a total of nine specialists, plus a pediatrician, a dentist, a physical therapist and an occupational therapist), I have repeated the laundry list of reasons that Gracie does not yet walk independently. I know the reasons… and I hear myself when listing them. But, on October 22, 2012, when we had to meet with a doctor at the CCS-MTU (California Children’s Services Medical Therapy Unit) to obtain a prescription to have her wheelchair adjusted, I heard those reasons in a whole different way.
The doctor, a Physiatrist (not included in that list of nine specialists), said to me, “Is she wanting to walk or are you wanting her to walk?” He went on to (very bluntly) say that, by his assessment, it is no surprise that she has had a decline in her ability and desire to walk. His report (wherein he couldn’t be bothered to spell her name correctly) lists those reasons with equal brusqueness… “significant kyphosocoliosis, restricted hip abduction, ankles only extend above neutral by 2 or 3 degrees, feet are windswept to the right, when walking, she does not bring her knee to a neutral position, knee flexion contractures, hamstring contractures…” the list goes on as does his alluding to the fact that the numerous specialists we had seen in the eight plus years prior to him should have followed a different course of treatment for her regarding bracing, etc. (renowned doctors, mind you, with second and third opinions from other renowned doctors).
Nothing like making me feel like 1) we did not seek the right treatment for her (I know that’s not true, but his bite definitely left teeth marks that day)
2) we are forcing something (possibly cruelly forcing) that she is never going to do because of that list in his report and the one I already compiled
3) crying… hard.
I don’t cry in appointments. But that day, I could barely stop the tears from flowing. I sucked it up pretty well considering his lack of bedside manner and the burning hot tears that stung my eyes and tingled my nose. I left with a giant lump in my throat, a broken heart and the sinking, helpless feeling that I had failed my daughter both through treatment that differed from his opinion and by pushing her so hard to accomplish independent walking - - or even walking with a walker independently.
Since that day, we have continued the daily stretching exercises and even tried a different kind of stander, The Magician, for Gracie. The stander, they hoped, would help with stretching. She hated it. We would set the timer for 30 minutes (they wanted 60 twice a day) and she would sob for 30 minutes. It didn’t help that the one we borrowed was too big for her, so she could easily shift herself out of the desired position. Her therapists have made school visits to establish some plans for keeping her active with her walker and transitioning. And, Kaitlyn, her aide, does a phenomenal job of getting Gracie moving (and respecting her limits). With all of this, there still hasn’t been much change.
So, when we headed out to the cardiologist on Wednesday, I was kind of hoping that there would be an obvious cardiac component to her lack of energy. That would be an explanation… one with a surgical solution… and, once past recovery, it would cure the lack of energy and thus, give her back the ability to rebuild strength and stamina. But, Dr. Saba felt strongly that the decrease in Gracie’s activity level is not from any changes in her heart function - - heart function which is limited, of course, but is relatively unchanged in the last couple of years. Thus… bittersweet.
While Gracie’s activity level has decreased, there are many pieces of good (really good… and great) news. She continues to enjoy school - - not so much the work part, but definitely all the fun stuff! Her aide, Kaitlyn, is amazing with her. Gracie… DRUMROLL, please… goes potty on the toilet every single day… often more than once! This is a HUGE milestone for her. She rides (like a speed demon on some days!) an adaptive bicycle on the playground at school and loves it! (This is not a surprise to us, but considering what her team at her old school claimed she would/would not do at school, this is a great step in the right direction!) She is getting her own iPad for school (finally!) and everything will be able to transition to home use on our iPad thru the cloud. And more!! Her feedings - - always the biggest health challenge in her nearly ten years - - are going better than ever (with the exception of her abdominal migraines) and her weight is the highest and most stable it has been in her entire life.
Instead of dwelling on all those laundry lists of reasons why Gracie isn't yet walking, I am taking that list of good things and placing my focus there. I will continue to believe and hope that Gracie will regain her strength and stamina. I will continue to push her on the days I know she can handle it and respect her limits on the days she makes it clear that she can’t. And, while I certainly understand the physical and medical conditions that challenge Gracie (and could, quite possibly, permanently limit her), I will not EVER allow the opinion of any doctor destroy our hopes and dreams regarding her potential.
Back to why I (sort of) hoped that her heart held more answers to the current issues… Gracie’s desire and ability to walk longer distances (longer for her… not actually very long) has decreased tremendously over the last several months. I posted about this a year ago:
"Why doesn't she walk, yet?" is commonly asked, even amongst those "in the know" in the world of CHARGE. I have fielded a bevy of questions on this subject, especially of late. Doctors asking, parents of other kids with various special needs, parents of kids with CHARGE... "Isn't she in therapy?" I can go into the details of her therapy regimen - - it is intensive, both in session and at home - - but the point of all this is that these inquiries (which are sometimes judgments) only remind me that all of her hard work (and ours) has yet to yield the desired result. There are numerous explanations for her delay in this area... she has no semi-circular canals, her vestibular nerve has not been visualized in a CT and is believed to be malformed or missing entirely. She has scoliosis that has affected her hip alignment and her leg lengths. She has mild diplegic cerebral palsy. Her hospitalizations and chronic illnesses have created roadblocks to maintaining strength and stamina. And, still, after all these years, I will not give up... but I also won't pretend that at moments I feel sad, frustrated, deflated and defeated.
Since we moved and I have been establishing her with an entire new team (a total of nine specialists, plus a pediatrician, a dentist, a physical therapist and an occupational therapist), I have repeated the laundry list of reasons that Gracie does not yet walk independently. I know the reasons… and I hear myself when listing them. But, on October 22, 2012, when we had to meet with a doctor at the CCS-MTU (California Children’s Services Medical Therapy Unit) to obtain a prescription to have her wheelchair adjusted, I heard those reasons in a whole different way.
The doctor, a Physiatrist (not included in that list of nine specialists), said to me, “Is she wanting to walk or are you wanting her to walk?” He went on to (very bluntly) say that, by his assessment, it is no surprise that she has had a decline in her ability and desire to walk. His report (wherein he couldn’t be bothered to spell her name correctly) lists those reasons with equal brusqueness… “significant kyphosocoliosis, restricted hip abduction, ankles only extend above neutral by 2 or 3 degrees, feet are windswept to the right, when walking, she does not bring her knee to a neutral position, knee flexion contractures, hamstring contractures…” the list goes on as does his alluding to the fact that the numerous specialists we had seen in the eight plus years prior to him should have followed a different course of treatment for her regarding bracing, etc. (renowned doctors, mind you, with second and third opinions from other renowned doctors).
Nothing like making me feel like 1) we did not seek the right treatment for her (I know that’s not true, but his bite definitely left teeth marks that day)
2) we are forcing something (possibly cruelly forcing) that she is never going to do because of that list in his report and the one I already compiled
3) crying… hard.
I don’t cry in appointments. But that day, I could barely stop the tears from flowing. I sucked it up pretty well considering his lack of bedside manner and the burning hot tears that stung my eyes and tingled my nose. I left with a giant lump in my throat, a broken heart and the sinking, helpless feeling that I had failed my daughter both through treatment that differed from his opinion and by pushing her so hard to accomplish independent walking - - or even walking with a walker independently.
Since that day, we have continued the daily stretching exercises and even tried a different kind of stander, The Magician, for Gracie. The stander, they hoped, would help with stretching. She hated it. We would set the timer for 30 minutes (they wanted 60 twice a day) and she would sob for 30 minutes. It didn’t help that the one we borrowed was too big for her, so she could easily shift herself out of the desired position. Her therapists have made school visits to establish some plans for keeping her active with her walker and transitioning. And, Kaitlyn, her aide, does a phenomenal job of getting Gracie moving (and respecting her limits). With all of this, there still hasn’t been much change.
So, when we headed out to the cardiologist on Wednesday, I was kind of hoping that there would be an obvious cardiac component to her lack of energy. That would be an explanation… one with a surgical solution… and, once past recovery, it would cure the lack of energy and thus, give her back the ability to rebuild strength and stamina. But, Dr. Saba felt strongly that the decrease in Gracie’s activity level is not from any changes in her heart function - - heart function which is limited, of course, but is relatively unchanged in the last couple of years. Thus… bittersweet.
While Gracie’s activity level has decreased, there are many pieces of good (really good… and great) news. She continues to enjoy school - - not so much the work part, but definitely all the fun stuff! Her aide, Kaitlyn, is amazing with her. Gracie… DRUMROLL, please… goes potty on the toilet every single day… often more than once! This is a HUGE milestone for her. She rides (like a speed demon on some days!) an adaptive bicycle on the playground at school and loves it! (This is not a surprise to us, but considering what her team at her old school claimed she would/would not do at school, this is a great step in the right direction!) She is getting her own iPad for school (finally!) and everything will be able to transition to home use on our iPad thru the cloud. And more!! Her feedings - - always the biggest health challenge in her nearly ten years - - are going better than ever (with the exception of her abdominal migraines) and her weight is the highest and most stable it has been in her entire life.
Instead of dwelling on all those laundry lists of reasons why Gracie isn't yet walking, I am taking that list of good things and placing my focus there. I will continue to believe and hope that Gracie will regain her strength and stamina. I will continue to push her on the days I know she can handle it and respect her limits on the days she makes it clear that she can’t. And, while I certainly understand the physical and medical conditions that challenge Gracie (and could, quite possibly, permanently limit her), I will not EVER allow the opinion of any doctor destroy our hopes and dreams regarding her potential.
Sunday, February 6, 2011
Appointments Galore
Gracie's last abdominal migraine cycle ended, in it's entirety, January 23rd. My absence here since that horrible week, I'm happy to report, is due to a lot of busy days... school, medical appointments, fun, therapy appointments, visitors, more appointments, and so on. Being that the cycles last between 4-6 days and occur approximately every 16-21 days, we capitalize on the good days and accomplish as much as we can, not just for Gracie, but for all of us. The space between is exhausting for wonderful, "normal" reasons, and though I am downright spent at the end of a day, you won't hear me complaining about being that kind of tired.
It's no secret that Gracie has a level of fight and resilience beyond words, but after that last cycle, her return to hard work at school and therapy (OT and PT twice a week each, plus mommy therapy) reached a new level in my opinion. She went from being in terrible pain, having dusky pallor, being listless, retching, sweating and unable tolerate pedialyte (let alone formula) to having two great weeks in a row at school. Last Monday was a marathon day with school (where she had a great day), a cardiology appointment (more on that in a moment) and then an hour of therapy where she rocked it by working on stairs with her PT and sit-to-stand while painting (thus, reaching and using one hand to balance on the wall) with her OT. She was tired, but she rallied through. She had been up since 4:15am, left the house at 8am and didn't get home until almost 6pm. ROCKSTAR. Her PT even commented on how amazingly well she continues to bounce back without skipping a beat. That's my girl!
The Medical Appointments:
GI
During her last migraine cycle, we saw her GI doc. My mission with him was to show him what she's going through, how she looks, why it's impossible to get nutrition in her during this time, etc., and to show him my records of her calorie intake since her last multiple-day hospitalization in October. Because of the frequency of the cycles, Gracie's missing a lot of nutrition. The good news is that I can bump up her intake in the space between and give her more (because when she's well, she now tolerates her feedings beautifully!). But even with that, I'm limited on volume for cardiac reasons and still can't catch her up enough - - averaging out a month, she's short roughly 300 calories per day. Thankfully, this doc (unlike another in the same practice) completely agrees with the way we handle her feedings during a migraine. He, too, is concerned with how hard she can retch and the risk of her slipping the repairs that were surgically made last summer. He urged me to try a calorie booster that was unsuccessful in the past (she vomited and retched for hours) to see if she'd do better now. I was skeptical, but said I'd try it over a weekend so that she'd not miss school if it made her miserable. Guess what?! It worked! We've been giving it to her for a week without issue... Woo Hoo!
We will see him in another month to check Gracie's weight and height. The goal is to get some weight on her... she's had a bit of linear growth since the fall, but still only weighs 29.5 pounds.
Neurology
I brought my records of Gracie's cycles (for the last year... I have records since she was just a few months old), a list of the symptoms, video and pictures. For the umpteenth time I expressed my belief that this appears to have a cranial nerve and sinus related component. (She does a lot of pulling on her face - especially her mouth, her color changes drastically, she covers her eyes and/or presses them, she has a increased secretions - - saliva, not mucous - - through the whole thing, etc.) He didn't really want to look at any of it... not in an unsympathetic way... just that there isn't much he can do other than mess with her meds. He'd like to get an MRI of her brain and take a close look at the nerves, etc. but Gracie has a pacemaker and it's simply out of the question. She had one as a baby, but her nerves were so small then. A CT of her brain won't show us anything in enough detail (with regard to nerves - - she's had three brain CTs in her life and we already know her brain structure is not normal), so, once again, he made some medication adjustments. (We are looking into pursuing another go at alternative medicine, though.) We will call him in two months to let him know if the pattern has changed at all. This is so very frustrating, not to mention heartbreaking... as this central nervous system/neurological piece of Gracie's medical drama has the most impact on her... it affects her nutrition (which, thankfully, is the best it's been since 2007), but more than anything, it affects her quality of life... every 16-21 days.
Opthamology
Gracie has a cataract in her right eye... which is, thankfully, her non-sighted eye. We will follow-up with the doctor in April to determine when to do a lensectomy, though she did say she'd like to wait as long as possible. The biggest concern with Gracie's right eye is that it is so small - - the orbital is smaller than normal, but the eye is even smaller than that - - and therefore, more prone to irritation and infection. The opthamologist stressed how fragile that eye is and mentioned that there is a possibility that, at some point, her right eye may have to be removed if it doesn't remain healthy. Our first opthamologist had mentioned this to us years ago, so I was not shocked by the statement. And, even though it is a "possibility down the road," I'd be lying if I said I didn't feel my stomach drop just a bit at the thought. In the wise words of my mama, I will "not borrow trouble." There are plenty of "for sure things" on my plate (and Gracie's) right now.
Orthopaedist
Gracie's scoliosis is slightly worsened (not too surprising given how sick and inactive she was for many months last year). The rotation in her pelvis has also increased and has caused her to sit even more crooked. The bracing "contraption" that the doctor ordered for her hips last year is not a safe option as it forces Gracie to lay flat on her back - - not good for a kiddo with a trach who needs to often roll to her side to clear her airway (not to mention how incredibly uncomfortable it is). She has had other braces for her scoliosis in the past, but they restrict her mobility so much that everyone (including the ordering docs) feel that it's better to give her the option to move and gain strength as those activities will also help with slowing the deterioration of the curve. We go back in April.
ENT
Fluid in her ears... as always. That's why she can't wear BTEs (Behind the Ear hearing aids). My bigger concern during that visit was the irritation under the right side of her trach. Ever since the pulomonologist increased her trach size (and thus the shape of her trach) we've dealt with a very irritated spot... so bad that it became a Grade 3 pressure ulcer and her ENT was just as displeased as we were. Much to the chagrin of the doc that wanted her trach size increased, I have since put back in the smaller trach to relieve the pressure on her skin and, thankfully, the nasty ulcer quickly healed. Now we need to see pulmonology to find out what they want to do. The only thing I know is we won't be putting Gracie through that kind of discomfort again... they'll have to figure out another way to protect her floppy airway.
And, speaking of trachs... My sweet girl has become a trach yanker in the last week. (John and I have always felt so lucky that Gracie has been a "perfect trach child" - - never really tugging at it or pulling too much on her ties. Those days, sadly, seem to be over.) What does "trach yanker" mean, you ask? Well, last Friday, she was in the family room laughing hysterically and I realized she sounded "different." I came out from the kitchen to find her trach out and her hand over her stoma as she's blowing air against it, cracking up and quite pleased with her little self. Then, this past Friday night she did it again. And, yesterday, she did it four times. For those of you who don't know, this is NOT good. Trachs must be sterile (or clean) and once it's out, it shouldn't go back in without proper cleaning. I have four more trachs to clean today. She just cracks up at me when I sternly sign "no" and put in a new trach. What's terrifying about this, more than the germ factor (which is huge, of course), is the fact that while the trach is out, something could easily enter that stoma and get lodged in there and/or inhaled into her lungs. She also has a floppy airway and the trach helps support that and keep it open. And, she can't swallow her secretions, so if the stoma closes up enough (which can happen quickly) that I can't even get our smaller, emergency trach in, she could choke or drown on her own saliva. If only she understood this... perhaps she wouldn't think it is so hilarious. Grrr.
Cardiology
Pressures remain stable, volume is still within acceptable (non-surgical) ranges and though her mitral regurgitation is moderate, she is no longer showing any signs of borderline heart failure. Great news!
We will continue to see him every three months to monitor the pacemaker in between the phone monitoring we do at home. He said, "I don't think we need to echo her again for a year unless she is symptomatic or starts having more "normal" activity for a child her age." I took this with a grain of salt, chuckled at him a bit and asked him to knock on wood because in the spring of 2006, we heard something very similar from her cardiologist. By July she was in early stages of heart failure and by December 2006 she was having her third open heart surgery. (By "normal activity" he means walking, running, etc. It might sound weird to say I hope he needs to echo her sooner, but with respect to "normal activities," I can only dream he'd need to!)
Dentist
Both kids went last week. We feel so lucky to have two kids who truly enjoy going to the dentist! :-) Unfortunately, we learned that Gracie's enamel didn't form properly on her permanent molars (probably due to how sick she was during the time the enamel formed on those teeth), so we will be having to keep a close eye on those... plus she is such a grinder, which doesn't help matters. She also has terrible recession of her gums where her bottom two front teeth are, so we will also be keeping a close eye on that as, sadly, some grafting will probably be in her future. He said that it's not anything we are or aren't doing... just the way it is for her. Bummer.
That's it... for now! Isn't that enough?!
Coming soon:
Follow-ups on most of these appointments, plus endocrinology, infectious disease, pulmonology and audiology (which has been cancelled four times since last June due to hopsitalizations or illness). Good grief!
It's no secret that Gracie has a level of fight and resilience beyond words, but after that last cycle, her return to hard work at school and therapy (OT and PT twice a week each, plus mommy therapy) reached a new level in my opinion. She went from being in terrible pain, having dusky pallor, being listless, retching, sweating and unable tolerate pedialyte (let alone formula) to having two great weeks in a row at school. Last Monday was a marathon day with school (where she had a great day), a cardiology appointment (more on that in a moment) and then an hour of therapy where she rocked it by working on stairs with her PT and sit-to-stand while painting (thus, reaching and using one hand to balance on the wall) with her OT. She was tired, but she rallied through. She had been up since 4:15am, left the house at 8am and didn't get home until almost 6pm. ROCKSTAR. Her PT even commented on how amazingly well she continues to bounce back without skipping a beat. That's my girl!
The Medical Appointments:
GI
During her last migraine cycle, we saw her GI doc. My mission with him was to show him what she's going through, how she looks, why it's impossible to get nutrition in her during this time, etc., and to show him my records of her calorie intake since her last multiple-day hospitalization in October. Because of the frequency of the cycles, Gracie's missing a lot of nutrition. The good news is that I can bump up her intake in the space between and give her more (because when she's well, she now tolerates her feedings beautifully!). But even with that, I'm limited on volume for cardiac reasons and still can't catch her up enough - - averaging out a month, she's short roughly 300 calories per day. Thankfully, this doc (unlike another in the same practice) completely agrees with the way we handle her feedings during a migraine. He, too, is concerned with how hard she can retch and the risk of her slipping the repairs that were surgically made last summer. He urged me to try a calorie booster that was unsuccessful in the past (she vomited and retched for hours) to see if she'd do better now. I was skeptical, but said I'd try it over a weekend so that she'd not miss school if it made her miserable. Guess what?! It worked! We've been giving it to her for a week without issue... Woo Hoo!
We will see him in another month to check Gracie's weight and height. The goal is to get some weight on her... she's had a bit of linear growth since the fall, but still only weighs 29.5 pounds.
Neurology
I brought my records of Gracie's cycles (for the last year... I have records since she was just a few months old), a list of the symptoms, video and pictures. For the umpteenth time I expressed my belief that this appears to have a cranial nerve and sinus related component. (She does a lot of pulling on her face - especially her mouth, her color changes drastically, she covers her eyes and/or presses them, she has a increased secretions - - saliva, not mucous - - through the whole thing, etc.) He didn't really want to look at any of it... not in an unsympathetic way... just that there isn't much he can do other than mess with her meds. He'd like to get an MRI of her brain and take a close look at the nerves, etc. but Gracie has a pacemaker and it's simply out of the question. She had one as a baby, but her nerves were so small then. A CT of her brain won't show us anything in enough detail (with regard to nerves - - she's had three brain CTs in her life and we already know her brain structure is not normal), so, once again, he made some medication adjustments. (We are looking into pursuing another go at alternative medicine, though.) We will call him in two months to let him know if the pattern has changed at all. This is so very frustrating, not to mention heartbreaking... as this central nervous system/neurological piece of Gracie's medical drama has the most impact on her... it affects her nutrition (which, thankfully, is the best it's been since 2007), but more than anything, it affects her quality of life... every 16-21 days.
Opthamology
Gracie has a cataract in her right eye... which is, thankfully, her non-sighted eye. We will follow-up with the doctor in April to determine when to do a lensectomy, though she did say she'd like to wait as long as possible. The biggest concern with Gracie's right eye is that it is so small - - the orbital is smaller than normal, but the eye is even smaller than that - - and therefore, more prone to irritation and infection. The opthamologist stressed how fragile that eye is and mentioned that there is a possibility that, at some point, her right eye may have to be removed if it doesn't remain healthy. Our first opthamologist had mentioned this to us years ago, so I was not shocked by the statement. And, even though it is a "possibility down the road," I'd be lying if I said I didn't feel my stomach drop just a bit at the thought. In the wise words of my mama, I will "not borrow trouble." There are plenty of "for sure things" on my plate (and Gracie's) right now.
Orthopaedist
Gracie's scoliosis is slightly worsened (not too surprising given how sick and inactive she was for many months last year). The rotation in her pelvis has also increased and has caused her to sit even more crooked. The bracing "contraption" that the doctor ordered for her hips last year is not a safe option as it forces Gracie to lay flat on her back - - not good for a kiddo with a trach who needs to often roll to her side to clear her airway (not to mention how incredibly uncomfortable it is). She has had other braces for her scoliosis in the past, but they restrict her mobility so much that everyone (including the ordering docs) feel that it's better to give her the option to move and gain strength as those activities will also help with slowing the deterioration of the curve. We go back in April.
ENT
Fluid in her ears... as always. That's why she can't wear BTEs (Behind the Ear hearing aids). My bigger concern during that visit was the irritation under the right side of her trach. Ever since the pulomonologist increased her trach size (and thus the shape of her trach) we've dealt with a very irritated spot... so bad that it became a Grade 3 pressure ulcer and her ENT was just as displeased as we were. Much to the chagrin of the doc that wanted her trach size increased, I have since put back in the smaller trach to relieve the pressure on her skin and, thankfully, the nasty ulcer quickly healed. Now we need to see pulmonology to find out what they want to do. The only thing I know is we won't be putting Gracie through that kind of discomfort again... they'll have to figure out another way to protect her floppy airway.
And, speaking of trachs... My sweet girl has become a trach yanker in the last week. (John and I have always felt so lucky that Gracie has been a "perfect trach child" - - never really tugging at it or pulling too much on her ties. Those days, sadly, seem to be over.) What does "trach yanker" mean, you ask? Well, last Friday, she was in the family room laughing hysterically and I realized she sounded "different." I came out from the kitchen to find her trach out and her hand over her stoma as she's blowing air against it, cracking up and quite pleased with her little self. Then, this past Friday night she did it again. And, yesterday, she did it four times. For those of you who don't know, this is NOT good. Trachs must be sterile (or clean) and once it's out, it shouldn't go back in without proper cleaning. I have four more trachs to clean today. She just cracks up at me when I sternly sign "no" and put in a new trach. What's terrifying about this, more than the germ factor (which is huge, of course), is the fact that while the trach is out, something could easily enter that stoma and get lodged in there and/or inhaled into her lungs. She also has a floppy airway and the trach helps support that and keep it open. And, she can't swallow her secretions, so if the stoma closes up enough (which can happen quickly) that I can't even get our smaller, emergency trach in, she could choke or drown on her own saliva. If only she understood this... perhaps she wouldn't think it is so hilarious. Grrr.
Cardiology
Pressures remain stable, volume is still within acceptable (non-surgical) ranges and though her mitral regurgitation is moderate, she is no longer showing any signs of borderline heart failure. Great news!
We will continue to see him every three months to monitor the pacemaker in between the phone monitoring we do at home. He said, "I don't think we need to echo her again for a year unless she is symptomatic or starts having more "normal" activity for a child her age." I took this with a grain of salt, chuckled at him a bit and asked him to knock on wood because in the spring of 2006, we heard something very similar from her cardiologist. By July she was in early stages of heart failure and by December 2006 she was having her third open heart surgery. (By "normal activity" he means walking, running, etc. It might sound weird to say I hope he needs to echo her sooner, but with respect to "normal activities," I can only dream he'd need to!)
Dentist
Both kids went last week. We feel so lucky to have two kids who truly enjoy going to the dentist! :-) Unfortunately, we learned that Gracie's enamel didn't form properly on her permanent molars (probably due to how sick she was during the time the enamel formed on those teeth), so we will be having to keep a close eye on those... plus she is such a grinder, which doesn't help matters. She also has terrible recession of her gums where her bottom two front teeth are, so we will also be keeping a close eye on that as, sadly, some grafting will probably be in her future. He said that it's not anything we are or aren't doing... just the way it is for her. Bummer.
That's it... for now! Isn't that enough?!
Coming soon:
Follow-ups on most of these appointments, plus endocrinology, infectious disease, pulmonology and audiology (which has been cancelled four times since last June due to hopsitalizations or illness). Good grief!
Monday, September 6, 2010
Labor of Love
Taking a break from the "'S' is for..." series for the Labor Day holiday and, instead, focusing on a truly inspiring story about person-centered care, community involvement and acceptance, but mostly about selfless love and exemplary parenting. It's a story about a labor of love.
Before we had children, John and I used to talk about our children and their futures... dreams for kids who hadn't even been conceived. We'd talk about college and say that if our children got into Harvard, we'd want to able to pay for it for them, even if it meant working four jobs each. When Gracie was born, the Harvard dream didn't vanish entirely, but it has, obviously, evolved over the years. This story, for me, is about Gracie's Harvard.
The fact that we don't dream about an Ivy League campus for our daughter doesn't mean we don't have dreams for her; it doesn't mean that we've given up on her potential - - not that our spunky, fight with all her might girlie would let us even if we tried! John and I came to the realization a long time ago that Gracie will not likely live a truly independent life. We also agree that we don't want her, as an adult, living in an institution. Finding a way for her to be happy, have as much independence as possible, go to school, work, be social, safe and cared for while, at the same time, not burdening Preston with the responsibility of his sister's care (unless he makes that choice for himself), requires planning. It is a huge dream... and though different, of course, it's just as huge as Harvard.
Please click the link to read the article and watch the video. It will be a well spent five minutes of your day... VIDEO: A home for Jacob - The Morning Sun News
Watching the video was an incredibly emotional experience for me. There are so many similarities between Jacob and Gracie and regardless of their age difference, I can see her in him. I see what Jacob's parents have created for him and picture Gracie there. I hear their concerns and considerations for Jacob and they echo ours for Gracie. It's important to note, especially if you only watch the video and don't read the article, that Jacob is never left alone. He has caregivers at all times when he is not at school or work, including through the night - - funded, in part, through the Michigan choice waiver program which allows his parents to use a portion of the funds that would otherwise be spent to institutionalize him on hired help within his own home setting.
John and I have had the honor and pleasure of meeting Tim and Nancy Hartshorne (the parents featured in the article and video) in 2005 and again in 2007 at the International CHARGE Syndrome Conferences. They are experts on the subject of CHARGE as both parents and professionals. They generously share that knoweldge with other parents so that we may better understand how to help our children grow to their fullest potential, whatever that may be. We can all, regardless of our children's abilities, learn a lot from them about selfless, respectful and loving parenting.
Before we had children, John and I used to talk about our children and their futures... dreams for kids who hadn't even been conceived. We'd talk about college and say that if our children got into Harvard, we'd want to able to pay for it for them, even if it meant working four jobs each. When Gracie was born, the Harvard dream didn't vanish entirely, but it has, obviously, evolved over the years. This story, for me, is about Gracie's Harvard.
The fact that we don't dream about an Ivy League campus for our daughter doesn't mean we don't have dreams for her; it doesn't mean that we've given up on her potential - - not that our spunky, fight with all her might girlie would let us even if we tried! John and I came to the realization a long time ago that Gracie will not likely live a truly independent life. We also agree that we don't want her, as an adult, living in an institution. Finding a way for her to be happy, have as much independence as possible, go to school, work, be social, safe and cared for while, at the same time, not burdening Preston with the responsibility of his sister's care (unless he makes that choice for himself), requires planning. It is a huge dream... and though different, of course, it's just as huge as Harvard.
Please click the link to read the article and watch the video. It will be a well spent five minutes of your day... VIDEO: A home for Jacob - The Morning Sun News
Watching the video was an incredibly emotional experience for me. There are so many similarities between Jacob and Gracie and regardless of their age difference, I can see her in him. I see what Jacob's parents have created for him and picture Gracie there. I hear their concerns and considerations for Jacob and they echo ours for Gracie. It's important to note, especially if you only watch the video and don't read the article, that Jacob is never left alone. He has caregivers at all times when he is not at school or work, including through the night - - funded, in part, through the Michigan choice waiver program which allows his parents to use a portion of the funds that would otherwise be spent to institutionalize him on hired help within his own home setting.
John and I have had the honor and pleasure of meeting Tim and Nancy Hartshorne (the parents featured in the article and video) in 2005 and again in 2007 at the International CHARGE Syndrome Conferences. They are experts on the subject of CHARGE as both parents and professionals. They generously share that knoweldge with other parents so that we may better understand how to help our children grow to their fullest potential, whatever that may be. We can all, regardless of our children's abilities, learn a lot from them about selfless, respectful and loving parenting.
Wednesday, June 2, 2010
Glimpses of our girl and the Upper GI
Good news... Gracie has been spotted! We've seen glimpses of our giggly, sassy girl and we sure are happy to see her when she appears! She woke up this morning happy, begging for a bath and asking to get dressed for school. Granted, it was 3:30 in the morning, but still! She had been fasting (for the Upper GI) since about 9:45pm and was, therefore, in a fabulous mood.
Upon arriving at the hospital, she waited with me to check in and then took off, wheeling herself through the lobby to the elevators. Despite my best efforts to explain to her that we were there for pictures of her tummy, she was expecting therapy with Ally or Hema and was furious with me when I told her it was my turn to drive her wheelchair. She was not happy about waiting around, but then was a total trooper during the study itself. Once she understands she's not staying at the hospital, it's amazing how well she does for various procedures - - especially considering all she's already been through and what she's currently going through. She handled the barium well, just a tad over an ounce, but then was extra junky for about thirty minutes afterward.
The radiologist informed me that she definitely does not have SMA (Superior Mesenteric Artery Syndrome) - - that is good news. She did not, during the brief study, reflux any of the barium - - also good news. (She has not shown signs of reflux for the past three Upper GI studies or her last two Gastric Emptying Studies, though many doctors assume the increased secretions following feedings are reflux related. We think otherwise... it's more about her body's response to the discomfort and digestive process. She has great difficulty processing fat and protein, no matter how broken down the formula is. Dr. Grant indicated on Friday that what we describe, coupled with her surgical history, doesn't sound like reflux to him either.) The radiologist confirmed that her Nissen Fundoplication is slipped - - we have known that for years. He confirmed she has caused a Hiatal Hernia - - we have known that for years as well.
Previously, Gracie's team determined that surgery to repair the hernia was not indicated, as the hernia is not that severe. We will see what Dr. Grant and Dr. Idries think... especially since Dr. Grant discussed Gracie's Fundo with me at length on Friday. While he doesn't dismiss the idea of her abdominal migraines cycles and migraine headaches at all, one of his opinions is that Gracie's body has been reacting to the Nissen Fundoplication all these years. The intolerance to feedings and excessive, clear, stringy secretions that she produces for hours on end are common reactions he has seen in children who were, as it turned out, not good candidates for a Fundoplication. Gracie's Fundo was done at seven weeks (due to repeated vomiting and reflux which caused aspiration pneumonia), so we have no way of comparing the before and after. We do know this... the cycles are predictable and come in waves, so while the issue with the Fundo could be exacerbating the problem, we don't believe it is the only problem. And really, we still don't know what the problem is.
For now, unfortunately, we are supposed to just wait and continue what we've been doing. Dr. Idries will get our messages when she returns from vacation tomorrow and, hopefully, she will conference with Dr. Grant and then call us before the appointment next week. Yesterday, following her second feeding of the day, she slept for four straight hours and then slept intermittently until she "snapped out of it" a few hours after the fast started. She's asleep right now, and has been, since her second feeding of the day. (She handled her first feeding following the fast pretty well. She sat at the table and colored with Preston and we practiced tracing some lines and circles. Once she had that next feeding, she checked out again.) The abdominal migraine cycle is over, thus the continuous, excruciating pain has subsided. John and I decided that we will back off of Gracie's feedings during the day (go back to her regular "school/therapy" schedule) and send her to school tomorrow and Friday. We made this decision based upon the fact that if we aren't feeding her around the clock, she is herself again. She deserves the chance to have a couple of good days at school and then I will resume the 'round the clock "torture" over the weekend - - all of this contingent, of course, upon whether we hear from the doctor sooner rather than later.
So, we are in holding pattern of sorts... waiting to discuss all options and risks, waiting for answers to our many questions and waiting to schedule her admission.
And, again... a huge thank you to everyone for the emails, texts, voicemails, blog comments and FB messages... we can feel all the love and support... and we are so grateful. We wish we had time right now to get back to each of you personally... we hope you understand why we haven't caught up with all of you yet!
Upon arriving at the hospital, she waited with me to check in and then took off, wheeling herself through the lobby to the elevators. Despite my best efforts to explain to her that we were there for pictures of her tummy, she was expecting therapy with Ally or Hema and was furious with me when I told her it was my turn to drive her wheelchair. She was not happy about waiting around, but then was a total trooper during the study itself. Once she understands she's not staying at the hospital, it's amazing how well she does for various procedures - - especially considering all she's already been through and what she's currently going through. She handled the barium well, just a tad over an ounce, but then was extra junky for about thirty minutes afterward.
The radiologist informed me that she definitely does not have SMA (Superior Mesenteric Artery Syndrome) - - that is good news. She did not, during the brief study, reflux any of the barium - - also good news. (She has not shown signs of reflux for the past three Upper GI studies or her last two Gastric Emptying Studies, though many doctors assume the increased secretions following feedings are reflux related. We think otherwise... it's more about her body's response to the discomfort and digestive process. She has great difficulty processing fat and protein, no matter how broken down the formula is. Dr. Grant indicated on Friday that what we describe, coupled with her surgical history, doesn't sound like reflux to him either.) The radiologist confirmed that her Nissen Fundoplication is slipped - - we have known that for years. He confirmed she has caused a Hiatal Hernia - - we have known that for years as well.
Previously, Gracie's team determined that surgery to repair the hernia was not indicated, as the hernia is not that severe. We will see what Dr. Grant and Dr. Idries think... especially since Dr. Grant discussed Gracie's Fundo with me at length on Friday. While he doesn't dismiss the idea of her abdominal migraines cycles and migraine headaches at all, one of his opinions is that Gracie's body has been reacting to the Nissen Fundoplication all these years. The intolerance to feedings and excessive, clear, stringy secretions that she produces for hours on end are common reactions he has seen in children who were, as it turned out, not good candidates for a Fundoplication. Gracie's Fundo was done at seven weeks (due to repeated vomiting and reflux which caused aspiration pneumonia), so we have no way of comparing the before and after. We do know this... the cycles are predictable and come in waves, so while the issue with the Fundo could be exacerbating the problem, we don't believe it is the only problem. And really, we still don't know what the problem is.
For now, unfortunately, we are supposed to just wait and continue what we've been doing. Dr. Idries will get our messages when she returns from vacation tomorrow and, hopefully, she will conference with Dr. Grant and then call us before the appointment next week. Yesterday, following her second feeding of the day, she slept for four straight hours and then slept intermittently until she "snapped out of it" a few hours after the fast started. She's asleep right now, and has been, since her second feeding of the day. (She handled her first feeding following the fast pretty well. She sat at the table and colored with Preston and we practiced tracing some lines and circles. Once she had that next feeding, she checked out again.) The abdominal migraine cycle is over, thus the continuous, excruciating pain has subsided. John and I decided that we will back off of Gracie's feedings during the day (go back to her regular "school/therapy" schedule) and send her to school tomorrow and Friday. We made this decision based upon the fact that if we aren't feeding her around the clock, she is herself again. She deserves the chance to have a couple of good days at school and then I will resume the 'round the clock "torture" over the weekend - - all of this contingent, of course, upon whether we hear from the doctor sooner rather than later.
So, we are in holding pattern of sorts... waiting to discuss all options and risks, waiting for answers to our many questions and waiting to schedule her admission.
And, again... a huge thank you to everyone for the emails, texts, voicemails, blog comments and FB messages... we can feel all the love and support... and we are so grateful. We wish we had time right now to get back to each of you personally... we hope you understand why we haven't caught up with all of you yet!
Monday, May 31, 2010
She lost an entire week
Thankfully, the worst of the cycle appears to be over. Gracie was a little better today - - she was not crying as much, she didn't bite her fingers or hit her face and we weren't having to suction her continuously as she dealt with her feedings. Still, though, she slept a lot. So, while she's better as far as the excruciating pain goes, she still missed nearly the whole day. Don't get me wrong... sleeping all day without pain is way better than sleeping all day with pain. But in the big picture, sleeping all day is no way to spend the day. With the exception of her doctor appointments (which she cried through or "checked out" of), she has literally missed an entire week.
We've been asked by several people what made this cycle so bad compared to others she's had recently. The answer is not simple... and I am not sure I know the whole answer. What I do know is this: the fastest way to help her out of the cycle pattern is to give her body a break and stop all intake for several hours and then once we re-start, move very slowly with Pedialyte and then every twelve hours or more, build up her calories. Sometimes it takes a few days to successfully get back to full-strength formula. The problem is, that in between cycles or illness, we are not able to get much weight on her. When she does gain a bit of weight, she inevitably hits a wall (like she did this week) and we end up starting over. We can't keep chasing a weight that is an unhealthy weight to start with. We also can't keep causing her so much discomfort during illnesses or abdominal migraine cycles (or regular migraines) by force-feeding her. I am sure that's what caused this current cycle to drag on and on. And, it doesn't seem like the medication increase has done much of anything, but, the only way to really tell is to resume a regular schedule (like school, therapy and FUN) and see how she does.
Thus, we are in big debate about what to do next and when. Gracie is scheduled for an Upper GI series on Wednesday, but can't see her regular GI doctor until the 8th. We aren't thrilled to be waiting that long to discuss (and move forward with) the next step. We can continue doing what we've been doing for a while, which is to balance out and time her feedings so that she can "live" - - enjoy her days and tolerate school/therapy as best as possible. (Even though she spends a great deal of her day at school, they only give her approximately 1/8 of her daily intake. When they give her more (and often lately when they give her the normal amount), she sleeps at school - - or she takes a long time to recover and doesn't want to sit up and work/be engaged.) The problem with continuing like this for another week or two is that we will maintain her current weight (maybe... if she doesn't have a cycle again in the meantime) and we are still causing her discomfort. Her current weight is not an acceptable weight. Force feeding her around the clock to the point that she can't function through the "living" parts of her day is not acceptable.
So, I will call tomorrow to find out if we'll be able to discuss Wednesday's Upper GI results before next Tuesday and plan for the central line placement (and all that entails). We have a million questions written down... many of which surround the care for the site and the specific risks to Gracie regarding potential infection due to her trach and the different "bugs" she already harbors in her respiratory system. Having the line is, we feel, her only choice. She needs to be rescued from this pattern and her body's reaction to anything traveling through her digestive system. We can't wait and trouble shoot any more. She will need heart surgery soon... and she can't have surgery at her current weight. Her scoliosis could be contributing to the discomfort as well... and if that is the case, spine surgery might be sooner than later.
Over the last heartbreaking, painful week, we've questioned ourselves repeatedly... trying to make decisions for the right reasons. We have been analyzing what we are doing for her and making sure that these decisions are truly in Gracie's best interest. That is, to say, that we are making decisions to do things for her and not just to her... for her, not for us.
******************************************************************
A huge, heartfelt thank you to our wonderful friends, Jackie and Dave, and their son, Danny, who had Preston over to play (all day) both Saturday and Sunday. Knowing Preston was having a great time and being well taken care of gave me such peace of mind and allowed me to tend to Gracie's needs without feeling like I was neglecting him. As he has other times before when they've kindly helped us out, he had a blast with them. Over the two days they played at their house, went to My Gym, Pretend City, the Tot Lot at the beach, and a barbeque. So this morning, when Preston woke-up, he wanted to know what time Jackie was coming to pick him up!
Other friends and neighbors have been so thoughtful and brought us enough food to feed an army! We had homemade lasagna, french toast casserole, pizza, chicken salad... so much food... from people who knew that we were still able to cook, but they didn't want us to have to think about it. So nice... especially since John worked all weekend.
Thank you to everyone, again, for all of your love, support and prayers. I will do my best to update as we get more information about lab & test results, answers to our questions and the next step in the plan.
We've been asked by several people what made this cycle so bad compared to others she's had recently. The answer is not simple... and I am not sure I know the whole answer. What I do know is this: the fastest way to help her out of the cycle pattern is to give her body a break and stop all intake for several hours and then once we re-start, move very slowly with Pedialyte and then every twelve hours or more, build up her calories. Sometimes it takes a few days to successfully get back to full-strength formula. The problem is, that in between cycles or illness, we are not able to get much weight on her. When she does gain a bit of weight, she inevitably hits a wall (like she did this week) and we end up starting over. We can't keep chasing a weight that is an unhealthy weight to start with. We also can't keep causing her so much discomfort during illnesses or abdominal migraine cycles (or regular migraines) by force-feeding her. I am sure that's what caused this current cycle to drag on and on. And, it doesn't seem like the medication increase has done much of anything, but, the only way to really tell is to resume a regular schedule (like school, therapy and FUN) and see how she does.
Thus, we are in big debate about what to do next and when. Gracie is scheduled for an Upper GI series on Wednesday, but can't see her regular GI doctor until the 8th. We aren't thrilled to be waiting that long to discuss (and move forward with) the next step. We can continue doing what we've been doing for a while, which is to balance out and time her feedings so that she can "live" - - enjoy her days and tolerate school/therapy as best as possible. (Even though she spends a great deal of her day at school, they only give her approximately 1/8 of her daily intake. When they give her more (and often lately when they give her the normal amount), she sleeps at school - - or she takes a long time to recover and doesn't want to sit up and work/be engaged.) The problem with continuing like this for another week or two is that we will maintain her current weight (maybe... if she doesn't have a cycle again in the meantime) and we are still causing her discomfort. Her current weight is not an acceptable weight. Force feeding her around the clock to the point that she can't function through the "living" parts of her day is not acceptable.
So, I will call tomorrow to find out if we'll be able to discuss Wednesday's Upper GI results before next Tuesday and plan for the central line placement (and all that entails). We have a million questions written down... many of which surround the care for the site and the specific risks to Gracie regarding potential infection due to her trach and the different "bugs" she already harbors in her respiratory system. Having the line is, we feel, her only choice. She needs to be rescued from this pattern and her body's reaction to anything traveling through her digestive system. We can't wait and trouble shoot any more. She will need heart surgery soon... and she can't have surgery at her current weight. Her scoliosis could be contributing to the discomfort as well... and if that is the case, spine surgery might be sooner than later.
Over the last heartbreaking, painful week, we've questioned ourselves repeatedly... trying to make decisions for the right reasons. We have been analyzing what we are doing for her and making sure that these decisions are truly in Gracie's best interest. That is, to say, that we are making decisions to do things for her and not just to her... for her, not for us.
******************************************************************
A huge, heartfelt thank you to our wonderful friends, Jackie and Dave, and their son, Danny, who had Preston over to play (all day) both Saturday and Sunday. Knowing Preston was having a great time and being well taken care of gave me such peace of mind and allowed me to tend to Gracie's needs without feeling like I was neglecting him. As he has other times before when they've kindly helped us out, he had a blast with them. Over the two days they played at their house, went to My Gym, Pretend City, the Tot Lot at the beach, and a barbeque. So this morning, when Preston woke-up, he wanted to know what time Jackie was coming to pick him up!
Other friends and neighbors have been so thoughtful and brought us enough food to feed an army! We had homemade lasagna, french toast casserole, pizza, chicken salad... so much food... from people who knew that we were still able to cook, but they didn't want us to have to think about it. So nice... especially since John worked all weekend.
Thank you to everyone, again, for all of your love, support and prayers. I will do my best to update as we get more information about lab & test results, answers to our questions and the next step in the plan.
Friday, May 28, 2010
Today's GI Visit
Let me first say how much everyone's thoughts, prayers, love and support mean to John and me. We've received so many messages on FB, emails, voicemails, texts and messages here on the blog. This is a very difficult time... the most difficult since her extended time in the PICU in 2004, but for very different reasons, of course. We can feel all of you supporting us, loving us and our sweet girl. "Thank you" seems like such a small phrase, but please know that it is said from the bottom of our hearts. We wish we could get back to each and every one of you individually, but right now we are focusing on taking care of Gracie, Preston and ourselves.
As for how Gracie is feeling, she is essentially the same as she's been since Monday evening - - miserable, lethargic, disengaged, sleeping a lot, crying a lot, gagging, retching, so many secretions (a response to pain and something entering her stomach and running through her digestive tract) that I literally don't turn off the suction machine as I switch back and forth from suctioning her trach and mouth.
She was seen this morning by the Assistant Chief of Gastroenterology, Dr. Grant. He is one of two docs that our team up north recommended, but he doesn't take new patients, so this was our first visit with him. I was happy to finally get to see him, but obviously not by the circumstances. He spent a considerable amount of time with us, listening, asking questions, listening some more. He really heard me. Not that her current doctor hasn't heard me, but Dr. Grant heard a lot today, all at once, and really tried to take it in before he jumped to asking questions - - which is what a lot of docs do... try to provide a solution before they've really listened to the whole problem. (We've been to several doctors for these issues over the years... she's been seen by two doctors at Sutter Memorial, a doctor at UC Davis, a Fellow at Stanford and now three different doctors at CHOC.)
Anyway, we left there with a game plan: labs ordered, an urgent Upper GI study ordered (to check things out and to rule out a condition (SMA) that is caused when the body is so thin, the intestinal tract and spine collide causing an obstruction, thus excruciating pain) and her Neurontin increased significantly (this will likely make her out of it, but that's what we need to get nutrition in without causing so much distress). The bottom line is that we need to find the problem... and we might not have time to find it (since we have spent seven years trying to find it already) before her little body needs to be rescued nutritionally. The rescue would be to put in a central line so we can give her TPN (IV nutrition). This decision is one we've been headed toward for a long time, but it's not a decision to be taken lightly... for reasons that I am, quite honestly, too tired to detail here now. We will do this, most likely, within the next two weeks if we can't force feed her through the pain and discomfort successfully (and have her keep it all in). I am not overly optimistic about giving her meds that make her a "zombie," nor are we okay with her being shut-down like she's been this week (and so many other weeks of her life)... having her go through her days disoriented and checked-out is not a good solution. It's not a way to live.
This afternoon, when Gracie and I got home and I went to feed her, she had about 10ml of old blood that rose in her G-tube. She needed to be suctioned, so I clamped the tube for a second and she proceeded to throw-up more old blood. I immediately gave her Carafate (a med that helps heal the stomach lining), called the doctor (who had already left for the day) and finally got a call back this evening. This is not the first time that Gracie's gastritis has flared up, but it's the worst it's looked in over three years. She still has a tremendous amount of old blood in her stomach (which is shocking considering I have had her tube vented all week and haven't seen any new blood). So, I figured they'd want to admit her, but they don't - - it's a three-day weekend and unless the blood starts looking fresh (bright red) or she starts looking/acting worse (how much worse can she get?) or dehydrated, they'd rather her be able to be miserable at home than in the hospital. Dr. Grant ordered Carafate (um... already doing that, Dr. Grant...) and asked me to keep a close eye on her intake, output and behavior (um... already doing that, Dr. Grant). He wants to be kept apprised of any changes and be paged if we decide the ER is necessary. I guess I should be flattered that they know I am doing everything here that they can do there (aside from IV fluids if she'd need them), but I really just wish they could start the labs and studies now, instead of next week.
The central line is, as I've said before, not to be taken lightly. It is the last option we have. Even as we put her through this current set of tests and labs, the consensus is that's what Gracie will be getting within a couple of weeks.
Knowing we're headed to our last option to solve a problem that can't be identified is a scary thing...
As for how Gracie is feeling, she is essentially the same as she's been since Monday evening - - miserable, lethargic, disengaged, sleeping a lot, crying a lot, gagging, retching, so many secretions (a response to pain and something entering her stomach and running through her digestive tract) that I literally don't turn off the suction machine as I switch back and forth from suctioning her trach and mouth.
She was seen this morning by the Assistant Chief of Gastroenterology, Dr. Grant. He is one of two docs that our team up north recommended, but he doesn't take new patients, so this was our first visit with him. I was happy to finally get to see him, but obviously not by the circumstances. He spent a considerable amount of time with us, listening, asking questions, listening some more. He really heard me. Not that her current doctor hasn't heard me, but Dr. Grant heard a lot today, all at once, and really tried to take it in before he jumped to asking questions - - which is what a lot of docs do... try to provide a solution before they've really listened to the whole problem. (We've been to several doctors for these issues over the years... she's been seen by two doctors at Sutter Memorial, a doctor at UC Davis, a Fellow at Stanford and now three different doctors at CHOC.)
Anyway, we left there with a game plan: labs ordered, an urgent Upper GI study ordered (to check things out and to rule out a condition (SMA) that is caused when the body is so thin, the intestinal tract and spine collide causing an obstruction, thus excruciating pain) and her Neurontin increased significantly (this will likely make her out of it, but that's what we need to get nutrition in without causing so much distress). The bottom line is that we need to find the problem... and we might not have time to find it (since we have spent seven years trying to find it already) before her little body needs to be rescued nutritionally. The rescue would be to put in a central line so we can give her TPN (IV nutrition). This decision is one we've been headed toward for a long time, but it's not a decision to be taken lightly... for reasons that I am, quite honestly, too tired to detail here now. We will do this, most likely, within the next two weeks if we can't force feed her through the pain and discomfort successfully (and have her keep it all in). I am not overly optimistic about giving her meds that make her a "zombie," nor are we okay with her being shut-down like she's been this week (and so many other weeks of her life)... having her go through her days disoriented and checked-out is not a good solution. It's not a way to live.
This afternoon, when Gracie and I got home and I went to feed her, she had about 10ml of old blood that rose in her G-tube. She needed to be suctioned, so I clamped the tube for a second and she proceeded to throw-up more old blood. I immediately gave her Carafate (a med that helps heal the stomach lining), called the doctor (who had already left for the day) and finally got a call back this evening. This is not the first time that Gracie's gastritis has flared up, but it's the worst it's looked in over three years. She still has a tremendous amount of old blood in her stomach (which is shocking considering I have had her tube vented all week and haven't seen any new blood). So, I figured they'd want to admit her, but they don't - - it's a three-day weekend and unless the blood starts looking fresh (bright red) or she starts looking/acting worse (how much worse can she get?) or dehydrated, they'd rather her be able to be miserable at home than in the hospital. Dr. Grant ordered Carafate (um... already doing that, Dr. Grant...) and asked me to keep a close eye on her intake, output and behavior (um... already doing that, Dr. Grant). He wants to be kept apprised of any changes and be paged if we decide the ER is necessary. I guess I should be flattered that they know I am doing everything here that they can do there (aside from IV fluids if she'd need them), but I really just wish they could start the labs and studies now, instead of next week.
The central line is, as I've said before, not to be taken lightly. It is the last option we have. Even as we put her through this current set of tests and labs, the consensus is that's what Gracie will be getting within a couple of weeks.
Knowing we're headed to our last option to solve a problem that can't be identified is a scary thing...
Thursday, May 27, 2010
Gut Wrenching
It is a terrifying and heartbreaking thing to watch your child and her body refuse the very thing she needs to live.
Since the day she was born, Gracie has struggled to tolerate feedings. Whether it's calories or just hydration, getting what she needs into her little body has been difficult. In the early years, we'd be able to "force feed" her, literally making her be on the pump every single second she could bear it in order to keep the proper hydration and nutrition in her.
For the better part of the first three years of her life, she struggled with abdominal migraines/cyclical vomiting syndrome on a very predicable schedule... every 11 to 17 days and they'd last anywhere from two to five days (sometimes seven). She'd be truly down for the count... intolerant to light or movement. She'd tolerate a cool washcloth on her forehead, but not much else. For the first day or two, we'd literally have to stop all caloric intake and just give Pedialyte... but usually, she'd need to have a break from anything at all for at least 12-24 hours and it would take a week to get her back up to full strength formula. We learned how to manage it as best as we could - - knowing at what point she'd need to be admitted for IV fluids, knowing the signs that she couldn't manage an increase in the richness of the formula... all of it was down to a science. Finally, we found a combination of meds that seemed to help manage the intensity and frequencies of the cycles. As she got older, she started having fewer abdominal migraine symptoms and presented more like regular migraines. Again, through trial and error, we found a combination of meds that helped keep it all at bay. She's never been truly "cured," but the severity and frequency improved drastically.
It's no secret that the last handful of months have been a struggle in our house health-wise. Since January, we've not gone two weeks without someone having something. Nearly every family I know with more than one child has had a somewhat similar winter and spring, so I didn't pay too much attention to the details of the recent patterns (that is to analyze them - - I still make note of everything related to Gracie... charting little notes each day related to her feedings, especially), until now. Going back on my "Gracie calendar," it's seems pretty clear that the cycles are back. Whether they are actual abdominal migraines or reaction to the pain from her recurrent ear and sinus infections is not clear... though I think it's likely a combination of both. Or... is it that the increased intolerance to feedings creates increased, tenacious secretions which, in turn, cause fluid in her ears and sinuses to build up thus causing the infections. One thing is certain, the pattern is about every 16-22 days and it lasts about five days. It's most definitely the reason that we can't get weight on her and struggle to maintain.
This is not a new problem - - it's always been a problem. Her highest weight was at four years of age... 30.5 pounds. Since her pacemaker generator replacement in August of 2008, she has dropped from 28 pounds to 23. She's teetered between 24-26 over the last several months and now is back down to 23. We are scared. She is wasting away. Her face is so thin that her cheek bones are protruding slightly and you can actually see the bones around her eye sockets. Her pacemaker is clearly visible on her left side.
On Monday afternoon, a feeding caused Gracie to become absolutely hysterical. Monday night she was so upset that she required constant suctioning for the better part of three hours. She was clearly in pain - - hitting herself in the face, pulling on her lips, biting her fingers to the point of bruising. We had to vent her G-tube the whole night and stop all fluid intake of any type to help her calm down and relax. She was so worked up she spiked a fever of 103. On Tuesday, we struggled to keep her hydrated (she'd already missed so much the night before) and with every small bolus, she was sweaty, cried, retched, gagged and hit herself in the face. She'd require nearly constant suctioning for about 45 minutes afterward and then, a switch would turn and she'd be fine again... until the next time she needed fluid. Forget about hooking her up to the feeding pump for a slow feed... she absolutely hates being tethered to it on a good night and becomes self-injurious if connected while awake.
It's heartbreaking to see her like this... we are helpless... she is in pain, we can't help her. When you have a baby, feeding him or her is such an intimate connection... it's nurturing, it's loving and the very thing that sustains your baby's life. Gracie's body does not easily take in that nutrition. Her desire to eat orally is cruelly contradicted by her body's inability to swallow... and yet, even if she could eat, her body can't process it.
We want to have her admitted, but are so hesitant. Her GI doctor is on vacation... the GI doctor we saw previously said to solve the problem we should just force feed her. Not a solution. That's what we've been doing her whole life during these times... forcing fluid into her. When she stops violently retching, gagging and reacting to the betrayal she feels and the pain she's experiencing, she literally just passes out from exhaustion. She checks out - - sleeps - - in an effort to escape the pain. She has literally missed yesterday and today. No, she's not dehydrated because we are forcing fluid and nutrition into her, but she's not present. Our child has her best days when she is not getting anything at all - - no formula, no water. It's an unthinkable pain we are experiencing as her parents... feeding her is torture. Yet, so is not feeding her.
Knowing she is in the beginning stages of heart failure again only compounds this issue... she needs surgery soon, but cannot possibly have it without weight gain. We ask for your love, positive thoughts, prayers, support... whatever you have in you to give and send Gracie's way as we try to navigate this problem, yet again, with doctors who barely know her or us.
Since the day she was born, Gracie has struggled to tolerate feedings. Whether it's calories or just hydration, getting what she needs into her little body has been difficult. In the early years, we'd be able to "force feed" her, literally making her be on the pump every single second she could bear it in order to keep the proper hydration and nutrition in her.
For the better part of the first three years of her life, she struggled with abdominal migraines/cyclical vomiting syndrome on a very predicable schedule... every 11 to 17 days and they'd last anywhere from two to five days (sometimes seven). She'd be truly down for the count... intolerant to light or movement. She'd tolerate a cool washcloth on her forehead, but not much else. For the first day or two, we'd literally have to stop all caloric intake and just give Pedialyte... but usually, she'd need to have a break from anything at all for at least 12-24 hours and it would take a week to get her back up to full strength formula. We learned how to manage it as best as we could - - knowing at what point she'd need to be admitted for IV fluids, knowing the signs that she couldn't manage an increase in the richness of the formula... all of it was down to a science. Finally, we found a combination of meds that seemed to help manage the intensity and frequencies of the cycles. As she got older, she started having fewer abdominal migraine symptoms and presented more like regular migraines. Again, through trial and error, we found a combination of meds that helped keep it all at bay. She's never been truly "cured," but the severity and frequency improved drastically.
It's no secret that the last handful of months have been a struggle in our house health-wise. Since January, we've not gone two weeks without someone having something. Nearly every family I know with more than one child has had a somewhat similar winter and spring, so I didn't pay too much attention to the details of the recent patterns (that is to analyze them - - I still make note of everything related to Gracie... charting little notes each day related to her feedings, especially), until now. Going back on my "Gracie calendar," it's seems pretty clear that the cycles are back. Whether they are actual abdominal migraines or reaction to the pain from her recurrent ear and sinus infections is not clear... though I think it's likely a combination of both. Or... is it that the increased intolerance to feedings creates increased, tenacious secretions which, in turn, cause fluid in her ears and sinuses to build up thus causing the infections. One thing is certain, the pattern is about every 16-22 days and it lasts about five days. It's most definitely the reason that we can't get weight on her and struggle to maintain.
This is not a new problem - - it's always been a problem. Her highest weight was at four years of age... 30.5 pounds. Since her pacemaker generator replacement in August of 2008, she has dropped from 28 pounds to 23. She's teetered between 24-26 over the last several months and now is back down to 23. We are scared. She is wasting away. Her face is so thin that her cheek bones are protruding slightly and you can actually see the bones around her eye sockets. Her pacemaker is clearly visible on her left side.
On Monday afternoon, a feeding caused Gracie to become absolutely hysterical. Monday night she was so upset that she required constant suctioning for the better part of three hours. She was clearly in pain - - hitting herself in the face, pulling on her lips, biting her fingers to the point of bruising. We had to vent her G-tube the whole night and stop all fluid intake of any type to help her calm down and relax. She was so worked up she spiked a fever of 103. On Tuesday, we struggled to keep her hydrated (she'd already missed so much the night before) and with every small bolus, she was sweaty, cried, retched, gagged and hit herself in the face. She'd require nearly constant suctioning for about 45 minutes afterward and then, a switch would turn and she'd be fine again... until the next time she needed fluid. Forget about hooking her up to the feeding pump for a slow feed... she absolutely hates being tethered to it on a good night and becomes self-injurious if connected while awake.
It's heartbreaking to see her like this... we are helpless... she is in pain, we can't help her. When you have a baby, feeding him or her is such an intimate connection... it's nurturing, it's loving and the very thing that sustains your baby's life. Gracie's body does not easily take in that nutrition. Her desire to eat orally is cruelly contradicted by her body's inability to swallow... and yet, even if she could eat, her body can't process it.
We want to have her admitted, but are so hesitant. Her GI doctor is on vacation... the GI doctor we saw previously said to solve the problem we should just force feed her. Not a solution. That's what we've been doing her whole life during these times... forcing fluid into her. When she stops violently retching, gagging and reacting to the betrayal she feels and the pain she's experiencing, she literally just passes out from exhaustion. She checks out - - sleeps - - in an effort to escape the pain. She has literally missed yesterday and today. No, she's not dehydrated because we are forcing fluid and nutrition into her, but she's not present. Our child has her best days when she is not getting anything at all - - no formula, no water. It's an unthinkable pain we are experiencing as her parents... feeding her is torture. Yet, so is not feeding her.
Knowing she is in the beginning stages of heart failure again only compounds this issue... she needs surgery soon, but cannot possibly have it without weight gain. We ask for your love, positive thoughts, prayers, support... whatever you have in you to give and send Gracie's way as we try to navigate this problem, yet again, with doctors who barely know her or us.
Wednesday, February 17, 2010
Growth Hormone Deficient
Gracie update: Within 48 hours of starting Cipro, she is definitely resembling herself again. However, as much as Cipro knocks out bugs, it also knocks out her gut and gives her a horrible diaper rash... the side effects are brutal for her and, thus, we only give it as a last resort. Unfortunately, it's nearly the only antibiotic that works on her bugs. She had a good day at school, but is not tolerating her feedings well at all and is still pretty weak.
Two weeks ago, Gracie's Endocrinologist called to discuss the lab results from the day of her heart cath.
The good news - - unlike they initially thought the day they drew her blood, they did, in fact, draw enough to run all labs the Endo ordered. So, no trip to the lab this month. Good.
Other good news - - Gracie's Vitamin D and Calcium levels were normal. I was floored. I thought for sure, given the difficulty we have getting nutrition in her, she'd be deficient in both areas. She's not. That's good.
Now... for the rest of the news - - Gracie's growth factors are both low. They always have been, but not in the "we need to treat" range, rather in the "they are low and we'll monitor every two years" range. One is low, but not low enough to justify treatment on its own. The other growth factor is very low... normal is 1.5... Gracie's is 0.7.
So, what does this mean? For the moment, it means we wait. No treatment action will be taken at this point until we spend the next several months getting all of her specialists to weigh in on the pros and cons from their perspective. Then we'll put everything together, including our own opinions along with the advice and wisdom of parents who've already walked this road, and try and make the best decision for Gracie.
At this point, what we do know is this:
- She is clearly very tiny... which we've known for a long time, obviously.
(She's between 37.5"-38.5" depending on how stretched and straight she is and she's 25 pounds. Despite every effort, we cannot keep weight on her. Her heaviest weight was at age 4 and she was 30.5 pounds. Her highest weight in the last 16 months is 26.5 pounds. However, she is incredibly small regardless of her nutritional status. I've kept an obsessively accurate log of her caloric intake and, in theory, she should still be bigger in weight and height.)
Her doctor (whom I like very much) kindly and gently made a point to tell me that the decision is ultimately ours as her caregivers. She wants us to consider, first and foremost, how we would handle Gracie getting bigger as we continue to get older. Obviously, our dream is that Gracie will progress with her ability to stand, walk and transition independently. We won't give up believing that she can do it. Likewise, we won't give up believing in her ability to make great strides and live a more independent life someday. As time moves further and further along though, we also have to consider much more seriously the fact that these things might not happen for a really, really long time... or perhaps not at all. It doesn't mean that we aren't hopeful for our daughter; our dream is for her to reach her fullest potential - - whatever that may be. As her parents, we give her every tool to which we have access (and fight for access to those tools we don't have) and we don't allow a definitive line to be drawn in the sand about anything regarding her potential. That being said, we are keenly aware of what reality will look like if those things don't happen.
This piece of the "do we want her bigger?" decision is easy for us. We are not considering ourselves... we will figure out how to manage if she gets too big for us to easily care for as we age. If she's smaller, then it's obviously much easier for us to carry/transition/help her with all mobility tasks. But... we don't want to keep her small for our sake when there is still hope that she'll be independently mobile. There is plenty of equipment to help us with those things and if we need to hire help someday when we're older, we will. And, in all reality, even though she has a lot of very tall family, there are some shorter women, including me... I'm 5'4" (according to my driver's license!) so Gracie wasn't necessarily destined to be huge... and likely wouldn't have tremendous growth with growth hormone treatment.
- Typically, with Gracie's numbers, an MRI of the brain would be ordered to get a good look at her brain, specifically the pituitary gland. While the labs are very informative, more information about the brain would give clarity as to exactly how deficient she truly is. Gracie's last MRI was done in August 2004. There were some abnormalities noted about the pituitary gland, but her doctor would prefer to get another MRI done now that she's nearly seven. Unfortunately, because of her pacemaker, she cannot have an MRI. So, we base it on the labs, her size and the MRI from 2004.
- With the Orthopedist, we need to discuss the fact that Growth Hormone is contraindicated for patients with scoliosis (because with more rapid growth can come faster and more severe curvature). He might not consider the scoliosis piece to be too much of a concern as Gracie is already slated to have spine surgery someday... when she is bigger, ironically. Perhaps his biggest concern about her spine will be how much/how fast the curve deteriorates rather than if it deteriorates.
There is also a potential side effect (though not common) for hip issues. Since her Orthopedist is already concerned about her potential for hip growth plate separation (thus the full-body brace that's been casted... that experience is another post in and of itself), he might be less inclined to support GH treatment for that reason alone.
- We need to discuss with the Cardiologist, too. She will, when she is bigger, need an additional open heart surgery to replace her mitral valve. It is our assumption that her Cardiologist would be in favor of this treatment for that reason. Obviously, with growth, she'd be more closely monitored to be certain that her heart can handle the work for her increasing body size. (But, really, we are not talking about rapid growth. If we treat, we'd start incredibly slow and monitor her very closely with all of her specialists.)
- With GI, we need to discuss what the risks would be if we do treat with GH and yet continue to have a difficult time increasing her tolerance for caloric intake. How would she be affected? Would we definitely need to go the route of TPN (IV nutrition) and bypass her digestive system entirely? This is something we continually debate for several reasons... one of which is the big concern about her psychological tolerance of being continuously attached to an IV... she most certainly would not tolerate it.
- Another possible (though, again, not common) side effect is for increased brain pressure and headaches... sadly, all too familiar territory for Gracie.
That's "it," in a nutshell. We ask, we listen, we think... and at some point in the next handful of months, we make a decision.
Two weeks ago, Gracie's Endocrinologist called to discuss the lab results from the day of her heart cath.
The good news - - unlike they initially thought the day they drew her blood, they did, in fact, draw enough to run all labs the Endo ordered. So, no trip to the lab this month. Good.
Other good news - - Gracie's Vitamin D and Calcium levels were normal. I was floored. I thought for sure, given the difficulty we have getting nutrition in her, she'd be deficient in both areas. She's not. That's good.
Now... for the rest of the news - - Gracie's growth factors are both low. They always have been, but not in the "we need to treat" range, rather in the "they are low and we'll monitor every two years" range. One is low, but not low enough to justify treatment on its own. The other growth factor is very low... normal is 1.5... Gracie's is 0.7.
So, what does this mean? For the moment, it means we wait. No treatment action will be taken at this point until we spend the next several months getting all of her specialists to weigh in on the pros and cons from their perspective. Then we'll put everything together, including our own opinions along with the advice and wisdom of parents who've already walked this road, and try and make the best decision for Gracie.
At this point, what we do know is this:
- She is clearly very tiny... which we've known for a long time, obviously.
(She's between 37.5"-38.5" depending on how stretched and straight she is and she's 25 pounds. Despite every effort, we cannot keep weight on her. Her heaviest weight was at age 4 and she was 30.5 pounds. Her highest weight in the last 16 months is 26.5 pounds. However, she is incredibly small regardless of her nutritional status. I've kept an obsessively accurate log of her caloric intake and, in theory, she should still be bigger in weight and height.)
Her doctor (whom I like very much) kindly and gently made a point to tell me that the decision is ultimately ours as her caregivers. She wants us to consider, first and foremost, how we would handle Gracie getting bigger as we continue to get older. Obviously, our dream is that Gracie will progress with her ability to stand, walk and transition independently. We won't give up believing that she can do it. Likewise, we won't give up believing in her ability to make great strides and live a more independent life someday. As time moves further and further along though, we also have to consider much more seriously the fact that these things might not happen for a really, really long time... or perhaps not at all. It doesn't mean that we aren't hopeful for our daughter; our dream is for her to reach her fullest potential - - whatever that may be. As her parents, we give her every tool to which we have access (and fight for access to those tools we don't have) and we don't allow a definitive line to be drawn in the sand about anything regarding her potential. That being said, we are keenly aware of what reality will look like if those things don't happen.
This piece of the "do we want her bigger?" decision is easy for us. We are not considering ourselves... we will figure out how to manage if she gets too big for us to easily care for as we age. If she's smaller, then it's obviously much easier for us to carry/transition/help her with all mobility tasks. But... we don't want to keep her small for our sake when there is still hope that she'll be independently mobile. There is plenty of equipment to help us with those things and if we need to hire help someday when we're older, we will. And, in all reality, even though she has a lot of very tall family, there are some shorter women, including me... I'm 5'4" (according to my driver's license!) so Gracie wasn't necessarily destined to be huge... and likely wouldn't have tremendous growth with growth hormone treatment.
- Typically, with Gracie's numbers, an MRI of the brain would be ordered to get a good look at her brain, specifically the pituitary gland. While the labs are very informative, more information about the brain would give clarity as to exactly how deficient she truly is. Gracie's last MRI was done in August 2004. There were some abnormalities noted about the pituitary gland, but her doctor would prefer to get another MRI done now that she's nearly seven. Unfortunately, because of her pacemaker, she cannot have an MRI. So, we base it on the labs, her size and the MRI from 2004.
- With the Orthopedist, we need to discuss the fact that Growth Hormone is contraindicated for patients with scoliosis (because with more rapid growth can come faster and more severe curvature). He might not consider the scoliosis piece to be too much of a concern as Gracie is already slated to have spine surgery someday... when she is bigger, ironically. Perhaps his biggest concern about her spine will be how much/how fast the curve deteriorates rather than if it deteriorates.
There is also a potential side effect (though not common) for hip issues. Since her Orthopedist is already concerned about her potential for hip growth plate separation (thus the full-body brace that's been casted... that experience is another post in and of itself), he might be less inclined to support GH treatment for that reason alone.
- We need to discuss with the Cardiologist, too. She will, when she is bigger, need an additional open heart surgery to replace her mitral valve. It is our assumption that her Cardiologist would be in favor of this treatment for that reason. Obviously, with growth, she'd be more closely monitored to be certain that her heart can handle the work for her increasing body size. (But, really, we are not talking about rapid growth. If we treat, we'd start incredibly slow and monitor her very closely with all of her specialists.)
- With GI, we need to discuss what the risks would be if we do treat with GH and yet continue to have a difficult time increasing her tolerance for caloric intake. How would she be affected? Would we definitely need to go the route of TPN (IV nutrition) and bypass her digestive system entirely? This is something we continually debate for several reasons... one of which is the big concern about her psychological tolerance of being continuously attached to an IV... she most certainly would not tolerate it.
- Another possible (though, again, not common) side effect is for increased brain pressure and headaches... sadly, all too familiar territory for Gracie.
That's "it," in a nutshell. We ask, we listen, we think... and at some point in the next handful of months, we make a decision.
Sunday, December 20, 2009
Medical This 'n That
CHOC Rocks & Pop Rocks
Gracie's back at VitalStim! Yay! This wonderful news brought to you, not by my efforts battling insurance, but by the "powers that be" at CHOC and the (what I can only imagine to be) tireless efforts of the therapists in the Rehab department. As it turns out, many patients were having difficulty getting their insurance to cover the electrodes that are used as part of the therapy. (While the electric stimulation is part of the treatment, there are several modalities of treatment occurring at the same time - - all of that was covered, just not the electrodes themselves because the "stim" is considered experimental or investigational.) Several weeks ago (yes... so behind on updates!) I received a call from one of Gracie's therapists telling me that CHOC had made a decision (following the efforts of some of the therapists to find a solution) to cover the electrodes so that these patients can have access to the therapy. Problem solved - - CHOC Rocks!
A few weeks ago(er... several weeks ago), Gracie experienced Pop Rocks at VitalStim! How cool is that?! Never in a million years would I have thought that Miss Gracie would have that opportunity (I didn't even know they still made them, but even if I did know, I wouldn't have ever thought she could have them!)
When her therapist, Ally, placed a piece on her tongue, Gracie got a look on her face like, "What is that...? And, can I have more?!" Talk about a multi-sensory experience! I had a few, too, and was instantly transported to my childhood. Preston was with us, so he got to try a little taste, as well. Just like Gracie, he wanted more!
October Was A Rough Month... So I Made Some Changes
October proved to be a rocky month for Miss Gracie. I keep a daily log of her calorie and volume intake as well as notes regarding headaches, retching, etc. Not once during the month did she meet her full caloric goal and she had more "rough" days/nights than not. Not good. She saw her GI doctor on the 5th and she made some dosage changes in her meds in the hopes of helping her cope with feedings better. If anything, the exact opposite seemed to have occurred. (In fact, my fuller-faced girl - - as seen in her school picture - - has dropped over a pound since it was taken.) Because it appears that her feeding trends worsened with the dosage change, I have dropped the meds back down to the previous dosage to see if that is the culprit. Interestingly enough, it was also her "off" month of her inhaled antibiotic. Not convinced that has anything to do with this (it really shouldn't, in theory), but will certainly keep an eye on next month's patterns just in case.
She missed school the Monday after Halloween (even though her fever had been gone for 24 hours) because she was coughing so hard and requiring excessive suctioning.
Since I reduced her dosage (and since she's been back on her inhaled antibiotic), she has been having much better days. Time will tell as I continue the "experiment."
(Updated following the experiment... she's still much better than she was in October and she's off of the inhaled antibiotic again.)
Behavioral Pediatrician
We finally saw the behavioral pediatrician in October. Our main objective was to discuss the behaviors we have been calling "OCD" - - stripping her clothes off and refusing to wear any at home, repeatedly wanting lights turned on and off... no rhyme or reason (in our minds), wanting the TV on or off, wanting certain items removed from her sight line (again, in our minds, no rhyme or reason), always putting all of her clothes under the couch when she undresses... the list goes on and on. School hasn't reported any of these behaviors, but often when she comes home, she comes "unglued" and has many requests (demands!). After talking it out with the doctor (who totally passed the "coffee test," Hannah, she commented that it sounded much more sensory than obsessive. It was like getting smacked upside the head... I know to always consider sensory issues first, but somehow, had gotten wrapped up in the repetitive nature of the behavior that I forgot to consider the source of the behavior. Uh... DUH! Experts (David Brown) have been to our home, met Gracie multiple times and we have discussed these very issues on a smaller level. She's older now and the behaviors have evolved, but so has her level of input during the day. This all coincides with increased activity level (school every day... therapy several times a week, etc.), so it's no wonder that her coping mechanisms (behaviors) have changed. I shared the conversation with the doctor with Gracie's team at school and we made a plan for more proprioceptive input and a more varied and rich "sensory diet" than what she'd been getting. So far, so good. Although, she still won't wear clothes at home... even when it's freezing cold outside! She can still wiggle her way out of every contraption we've tried, so for now, we are letting it go. She has very little control in her life... so if this one thing (at home only!) makes her happy, then what's the harm?
Cardiology
Gracie saw the cardiologist on Veteran's Day. Since she's likely getting closer to needing her mitral valve replaced, he decided he wanted to get a better picture of what everything looks like - - measurements, pressures, etc. - - so he has scheduled a heart catheterization for January 19th. The other issue is that Gracie's pacemaker generator is using the battery far faster than anticipated at her replacement surgery in August 2008 - - and faster, again, than what was indicated at the last pacemaker interrogation. This is because she is completely dependent on it and, most likely, because scar tissue has continued to form around the external leads on her heart. Because she is so small, her pacemaker is in her lower left side rather than implanted in her heart. Thus, the leads (wires) that run from the pacemaker to her heart attach on the outside of her heart rather than the inside. This, by nature of the design, is less efficient than the pacemaker that is implanted directly in the heart. The catheterization will give a better picture of the tissue around those leads, as well.
We are nervous... it's "routine," but nothing is ever "routine" when it comes to your child. And, though we've experienced so many procedures and surgeries (including multiple open heart) with Gracie, this is her first time in the cath lab.
Gracie's back at VitalStim! Yay! This wonderful news brought to you, not by my efforts battling insurance, but by the "powers that be" at CHOC and the (what I can only imagine to be) tireless efforts of the therapists in the Rehab department. As it turns out, many patients were having difficulty getting their insurance to cover the electrodes that are used as part of the therapy. (While the electric stimulation is part of the treatment, there are several modalities of treatment occurring at the same time - - all of that was covered, just not the electrodes themselves because the "stim" is considered experimental or investigational.) Several weeks ago (yes... so behind on updates!) I received a call from one of Gracie's therapists telling me that CHOC had made a decision (following the efforts of some of the therapists to find a solution) to cover the electrodes so that these patients can have access to the therapy. Problem solved - - CHOC Rocks!
A few weeks ago(er... several weeks ago), Gracie experienced Pop Rocks at VitalStim! How cool is that?! Never in a million years would I have thought that Miss Gracie would have that opportunity (I didn't even know they still made them, but even if I did know, I wouldn't have ever thought she could have them!)
When her therapist, Ally, placed a piece on her tongue, Gracie got a look on her face like, "What is that...? And, can I have more?!" Talk about a multi-sensory experience! I had a few, too, and was instantly transported to my childhood. Preston was with us, so he got to try a little taste, as well. Just like Gracie, he wanted more!
October Was A Rough Month... So I Made Some Changes
October proved to be a rocky month for Miss Gracie. I keep a daily log of her calorie and volume intake as well as notes regarding headaches, retching, etc. Not once during the month did she meet her full caloric goal and she had more "rough" days/nights than not. Not good. She saw her GI doctor on the 5th and she made some dosage changes in her meds in the hopes of helping her cope with feedings better. If anything, the exact opposite seemed to have occurred. (In fact, my fuller-faced girl - - as seen in her school picture - - has dropped over a pound since it was taken.) Because it appears that her feeding trends worsened with the dosage change, I have dropped the meds back down to the previous dosage to see if that is the culprit. Interestingly enough, it was also her "off" month of her inhaled antibiotic. Not convinced that has anything to do with this (it really shouldn't, in theory), but will certainly keep an eye on next month's patterns just in case.
She missed school the Monday after Halloween (even though her fever had been gone for 24 hours) because she was coughing so hard and requiring excessive suctioning.
Since I reduced her dosage (and since she's been back on her inhaled antibiotic), she has been having much better days. Time will tell as I continue the "experiment."
(Updated following the experiment... she's still much better than she was in October and she's off of the inhaled antibiotic again.)
Behavioral Pediatrician
We finally saw the behavioral pediatrician in October. Our main objective was to discuss the behaviors we have been calling "OCD" - - stripping her clothes off and refusing to wear any at home, repeatedly wanting lights turned on and off... no rhyme or reason (in our minds), wanting the TV on or off, wanting certain items removed from her sight line (again, in our minds, no rhyme or reason), always putting all of her clothes under the couch when she undresses... the list goes on and on. School hasn't reported any of these behaviors, but often when she comes home, she comes "unglued" and has many requests (demands!). After talking it out with the doctor (who totally passed the "coffee test," Hannah, she commented that it sounded much more sensory than obsessive. It was like getting smacked upside the head... I know to always consider sensory issues first, but somehow, had gotten wrapped up in the repetitive nature of the behavior that I forgot to consider the source of the behavior. Uh... DUH! Experts (David Brown) have been to our home, met Gracie multiple times and we have discussed these very issues on a smaller level. She's older now and the behaviors have evolved, but so has her level of input during the day. This all coincides with increased activity level (school every day... therapy several times a week, etc.), so it's no wonder that her coping mechanisms (behaviors) have changed. I shared the conversation with the doctor with Gracie's team at school and we made a plan for more proprioceptive input and a more varied and rich "sensory diet" than what she'd been getting. So far, so good. Although, she still won't wear clothes at home... even when it's freezing cold outside! She can still wiggle her way out of every contraption we've tried, so for now, we are letting it go. She has very little control in her life... so if this one thing (at home only!) makes her happy, then what's the harm?
Cardiology
Gracie saw the cardiologist on Veteran's Day. Since she's likely getting closer to needing her mitral valve replaced, he decided he wanted to get a better picture of what everything looks like - - measurements, pressures, etc. - - so he has scheduled a heart catheterization for January 19th. The other issue is that Gracie's pacemaker generator is using the battery far faster than anticipated at her replacement surgery in August 2008 - - and faster, again, than what was indicated at the last pacemaker interrogation. This is because she is completely dependent on it and, most likely, because scar tissue has continued to form around the external leads on her heart. Because she is so small, her pacemaker is in her lower left side rather than implanted in her heart. Thus, the leads (wires) that run from the pacemaker to her heart attach on the outside of her heart rather than the inside. This, by nature of the design, is less efficient than the pacemaker that is implanted directly in the heart. The catheterization will give a better picture of the tissue around those leads, as well.
We are nervous... it's "routine," but nothing is ever "routine" when it comes to your child. And, though we've experienced so many procedures and surgeries (including multiple open heart) with Gracie, this is her first time in the cath lab.
Monday, September 21, 2009
Perspective
"It's all relative to your own experiences," I often tell my friends and family after they've said, "I don't know how you do it." Usually this is said in reference to the frequent curve balls life throws at our daughter and us. I constantly remind them that they'd take the same steps if in similar shoes. Sometimes they'll qualify statements about their own sleepless night because they know how sleep deprived I've been for the better part of six years. I am always telling them not to do that... of course they're tired if they're used to getting more sleep.
It's all relative - - experiences create your perspective. Learning about the experiences of others can change your perspective - - not necessarily because another's situation is more difficult or dire, but so many times because of the way in which others so gracefully and positively handle such situations.
Several months ago, Hannah (my friend and sister on this journey), wrote a poignant post on Perspective. I encourage anyone reading this post to click the link and read it (or re-read it if you've previously had the pleasure). It's the kind of post that speaks to my very core - - as do many of hers. I revisited it today because in the last handful of days, several of my friends have emailed, texted or called to say that after catching up on our blog, they've once again been able to take a step back from their own chaos and what they think is currently a big deal in their lives and re-group. They kindly say that our situation - - Gracie's life and how we function as a family in spite of all she endures - - gives them perspective on their own.
Perhaps "perspective" is one of the reasons so many of us blog. We share, we glean, we learn, we grow - - our perspectives shift as we read about and share in the lives of others. In the same way that my friends view our lives, I gain new perspective each day by following the lives of the Nelson Family. They are unmet friends in CHARGE and their daughter, Moriah (who has had a place in our blog sidebar for quite some time now), has been in the hospital for ten months. What the three of them have been through during this time is unfathomable. Yet, through it all, they've shown unwavering faith and have handled themselves with the utmost grace. They are amazing people... amazing parents... and they have a beautiful daughter who is such a sweetheart and clearly a fighter.
While we've weathered a lot with Gracie, what Moriah and her parents have endured is unimaginable, even to us. Through their experiences (that they so openly and honestly share with the blogger world), they've taught us and continue to teach us so much about courage, faith, love, what matters and what doesn't. They give us and teach us perspective daily.
* * * * * * * * * * * * * * * * * * * * * * * * * * * * * *
Moriah's medical issues continue to stump the doctors and her condition has taken yet another downturn. Whether you pray, send positive thoughts or meditate... please direct some of this energy to the Nelson family.
It's all relative - - experiences create your perspective. Learning about the experiences of others can change your perspective - - not necessarily because another's situation is more difficult or dire, but so many times because of the way in which others so gracefully and positively handle such situations.
Several months ago, Hannah (my friend and sister on this journey), wrote a poignant post on Perspective. I encourage anyone reading this post to click the link and read it (or re-read it if you've previously had the pleasure). It's the kind of post that speaks to my very core - - as do many of hers. I revisited it today because in the last handful of days, several of my friends have emailed, texted or called to say that after catching up on our blog, they've once again been able to take a step back from their own chaos and what they think is currently a big deal in their lives and re-group. They kindly say that our situation - - Gracie's life and how we function as a family in spite of all she endures - - gives them perspective on their own.
Perhaps "perspective" is one of the reasons so many of us blog. We share, we glean, we learn, we grow - - our perspectives shift as we read about and share in the lives of others. In the same way that my friends view our lives, I gain new perspective each day by following the lives of the Nelson Family. They are unmet friends in CHARGE and their daughter, Moriah (who has had a place in our blog sidebar for quite some time now), has been in the hospital for ten months. What the three of them have been through during this time is unfathomable. Yet, through it all, they've shown unwavering faith and have handled themselves with the utmost grace. They are amazing people... amazing parents... and they have a beautiful daughter who is such a sweetheart and clearly a fighter.
While we've weathered a lot with Gracie, what Moriah and her parents have endured is unimaginable, even to us. Through their experiences (that they so openly and honestly share with the blogger world), they've taught us and continue to teach us so much about courage, faith, love, what matters and what doesn't. They give us and teach us perspective daily.
* * * * * * * * * * * * * * * * * * * * * * * * * * * * * *
Moriah's medical issues continue to stump the doctors and her condition has taken yet another downturn. Whether you pray, send positive thoughts or meditate... please direct some of this energy to the Nelson family.
Wednesday, September 16, 2009
Chronic
Chronic... it's the word that reverberates in my head. I can hear it over and over - - much like it's skipping on my old white record player with the penny taped on the needle arm. No matter how hard I try to stop it, it won't stop repeating. When I close my eyes, I can see the word in print. I've read it in countless articles, written it down time and again in notes and have even used it here on the blog a time or two. It is a word - - the word - - that is used to describe so many of Gracie's medical conditions... Chronic Gastritis, Chronically Ill, Chronic Migraines, Chronic Middle Ear Pathology (ear infections), Chronic Pain and last Wednesday, a new one - - Chronic Lung Disease.
I was not, in any way, prepared for the later. I still am coming to grips with Chronic Pain - - that one makes me cry on a regular basis... having only recently come to terms with the fact that Gracie's pain is, indeed, chronic. While I've known that in the back of my mind, it's an entirely different thing to actually use that word and truly comprehend the meaning. Despite the effort I will make until I breathe my last breath, her pain will likely never go away. She copes remarkably well with all that she endures - - I am quite certain that if I were in her shoes, I would not.
There are several well-written and informative articles about pain in CHARGE Syndrome. Some of them can be found in two previous issues of CHARGE Accounts - - Spring 2007 and Summer 2007. Considering we've discussed this topic at length with professionals over the years and the fact that I read those articles when they were published two years ago (more than once), one would think that I'd have already come to grips with the idea as it applies to Gracie. But, when it comes to pain and your child, there's not a lot of room for acceptance.
Then, on the ninth, during Gracie's (ridiculously long) pulmonary clinic appointment, the word was presented again. Perhaps I was easily rattled because we had pulled into the parking garage at the hospital at 2:15 and the nurse practitioner didn't come into the room until well after 5:00. Perhaps it was because when the doctor finally came in (a colleague of our regular doctor), I was freezing from sitting in the over-air conditioned room (Gracie liked it, though), had been trying to keep Gracie occupied and happy for hours (she actually coped with the wait better than I did) and had to use the restroom for over an hour. Perhaps it was because I just thought we were there to have a check-up, get some paperwork signed for school and discuss a few "housekeeping" items. I am not sure it was any of these things or a combination of all of them... but I didn't handle the information well. It was a good thing that the nurse practitioner was in the room the while the doctor was and that she wrote down everything for me... because I missed half of the conversation - - a fact I discovered upon reviewing the notes with the nurse.
While Gracie is not formally diagnosed with Chronic Lung Disease, the doctor feels we have to treat her as having it. (Whatever that means... if it's serious enough to treat her for it, then it might as well be a formal diagnosis). She has primary aspiration - - which means that she aspirates her own saliva - - (as opposed to secondary aspiration which is refluxing, then aspirating). This is not news, of course. In spite of being trached, she continues to micro-aspirate her saliva into her lungs. For over two years, we have treated her with an inhaled antibiotic (a month on, a month off) to ward off the many trach "bugs" that are so common (and that seem to be very attracted to Gracie). Over the past several months, though, I have noticed that on the off months, she is getting more and more symptomatic of the bugs long before the medication is scheduled to restart. I shared this with the team last Wednesday. The consensus is that Gracie isn't developing a resistance to the med, rather she is not getting her lower lobes cleared out enough and is, therefore, harboring that bacteria all the time. The antibiotic, the (extremely proactive) doctor feels, is only masking the symptoms and the continuous micro-aspiration is doing permanent damage to her lungs.
As a result, she's prescribing a new medication regimen and CPT (Chest Physiotherapy - which is vigorous percussion of the chest wall). On months that she's on the antibiotic (which she believes will be through the spring months) she will have a total of six breathing treatments a day. The regimen is: Xopenex (to open her lungs) via nebulizer, followed by CPT (about 20-25 minutes), thorough suctioning to clear out all mucous brought up with the CPT, followed by Pulmicort via the nebulizer and then, finally, the antibiotic med via the nebulizer. Twice a day.
This is a lot... and I am not complaining because I will do whatever needs to be done... though I am really concerned with the amount of time this will take (Gracie's already getting a total of an hour of treatments a day). On the months that she's on the antibiotic, with the new plan, it will now take well over an hour. Twice a day. This on top of school, feedings, therapy, FUN... etc. Luckily, she loves her nebulizer and the CPT. But so far, she's not overjoyed about that hour and fifteen minute stretch at a time. We've tried it... and I absolutely have to give her a break. This is not the optimal way to deliver these meds or the therapy... there is a method to the doctor's madness. But, we also have to live in the real world. For the first few days I was emotional and angry about the whole thing. I had left that office feeling like I was going to be harming my daughter if I wasn't able to fully comply, yet also harming her if I force the full-length treatments twice a day. What I've come to realize, though, is that there are going to be days that I can't get all of it in... that I have to skip a med or a little of the CPT. We have to live in the real world... and we have to live.
And that brings me to the better part of this post... the "Other Chronics" in our lives! For instance, Chronically Amazed and Chronically Surprised!
On Saturday, my very intuitive daughter somehow knew that Mommy was in need of a pick-me-up and during the day's first very lengthy set of breathing treatments and CPT, she started pushing herself into a sit... all by herself... over and OVER!!! She pushed up on all fours, walked her hands up to her knees and pushed back into a sit. She then corrected her positioning, sat there very pleased with her achievement, got herself down and did it all again... at least thirty times! And she has done it many times every single day since!
Then, that night, she cruised from one thing to another for the first time...
And then, as a sweet reward while Preston napped on Sunday, Mommy gave her a well deserved, very luxurious pedicure... complete with a warm-lotion leg and foot massage!
The photo quality isn't great... these were all taken with my cell phone and the lighting was terrible. But they are actual moments captured - - the moments that made me refocus from last Wednesday's appointment and concentrate on all of the Good Chronic in our lives.
I was not, in any way, prepared for the later. I still am coming to grips with Chronic Pain - - that one makes me cry on a regular basis... having only recently come to terms with the fact that Gracie's pain is, indeed, chronic. While I've known that in the back of my mind, it's an entirely different thing to actually use that word and truly comprehend the meaning. Despite the effort I will make until I breathe my last breath, her pain will likely never go away. She copes remarkably well with all that she endures - - I am quite certain that if I were in her shoes, I would not.
There are several well-written and informative articles about pain in CHARGE Syndrome. Some of them can be found in two previous issues of CHARGE Accounts - - Spring 2007 and Summer 2007. Considering we've discussed this topic at length with professionals over the years and the fact that I read those articles when they were published two years ago (more than once), one would think that I'd have already come to grips with the idea as it applies to Gracie. But, when it comes to pain and your child, there's not a lot of room for acceptance.
Then, on the ninth, during Gracie's (ridiculously long) pulmonary clinic appointment, the word was presented again. Perhaps I was easily rattled because we had pulled into the parking garage at the hospital at 2:15 and the nurse practitioner didn't come into the room until well after 5:00. Perhaps it was because when the doctor finally came in (a colleague of our regular doctor), I was freezing from sitting in the over-air conditioned room (Gracie liked it, though), had been trying to keep Gracie occupied and happy for hours (she actually coped with the wait better than I did) and had to use the restroom for over an hour. Perhaps it was because I just thought we were there to have a check-up, get some paperwork signed for school and discuss a few "housekeeping" items. I am not sure it was any of these things or a combination of all of them... but I didn't handle the information well. It was a good thing that the nurse practitioner was in the room the while the doctor was and that she wrote down everything for me... because I missed half of the conversation - - a fact I discovered upon reviewing the notes with the nurse.
While Gracie is not formally diagnosed with Chronic Lung Disease, the doctor feels we have to treat her as having it. (Whatever that means... if it's serious enough to treat her for it, then it might as well be a formal diagnosis). She has primary aspiration - - which means that she aspirates her own saliva - - (as opposed to secondary aspiration which is refluxing, then aspirating). This is not news, of course. In spite of being trached, she continues to micro-aspirate her saliva into her lungs. For over two years, we have treated her with an inhaled antibiotic (a month on, a month off) to ward off the many trach "bugs" that are so common (and that seem to be very attracted to Gracie). Over the past several months, though, I have noticed that on the off months, she is getting more and more symptomatic of the bugs long before the medication is scheduled to restart. I shared this with the team last Wednesday. The consensus is that Gracie isn't developing a resistance to the med, rather she is not getting her lower lobes cleared out enough and is, therefore, harboring that bacteria all the time. The antibiotic, the (extremely proactive) doctor feels, is only masking the symptoms and the continuous micro-aspiration is doing permanent damage to her lungs.
As a result, she's prescribing a new medication regimen and CPT (Chest Physiotherapy - which is vigorous percussion of the chest wall). On months that she's on the antibiotic (which she believes will be through the spring months) she will have a total of six breathing treatments a day. The regimen is: Xopenex (to open her lungs) via nebulizer, followed by CPT (about 20-25 minutes), thorough suctioning to clear out all mucous brought up with the CPT, followed by Pulmicort via the nebulizer and then, finally, the antibiotic med via the nebulizer. Twice a day.
This is a lot... and I am not complaining because I will do whatever needs to be done... though I am really concerned with the amount of time this will take (Gracie's already getting a total of an hour of treatments a day). On the months that she's on the antibiotic, with the new plan, it will now take well over an hour. Twice a day. This on top of school, feedings, therapy, FUN... etc. Luckily, she loves her nebulizer and the CPT. But so far, she's not overjoyed about that hour and fifteen minute stretch at a time. We've tried it... and I absolutely have to give her a break. This is not the optimal way to deliver these meds or the therapy... there is a method to the doctor's madness. But, we also have to live in the real world. For the first few days I was emotional and angry about the whole thing. I had left that office feeling like I was going to be harming my daughter if I wasn't able to fully comply, yet also harming her if I force the full-length treatments twice a day. What I've come to realize, though, is that there are going to be days that I can't get all of it in... that I have to skip a med or a little of the CPT. We have to live in the real world... and we have to live.
And that brings me to the better part of this post... the "Other Chronics" in our lives! For instance, Chronically Amazed and Chronically Surprised!
On Saturday, my very intuitive daughter somehow knew that Mommy was in need of a pick-me-up and during the day's first very lengthy set of breathing treatments and CPT, she started pushing herself into a sit... all by herself... over and OVER!!! She pushed up on all fours, walked her hands up to her knees and pushed back into a sit. She then corrected her positioning, sat there very pleased with her achievement, got herself down and did it all again... at least thirty times! And she has done it many times every single day since!
Then, that night, she cruised from one thing to another for the first time...
And then, as a sweet reward while Preston napped on Sunday, Mommy gave her a well deserved, very luxurious pedicure... complete with a warm-lotion leg and foot massage!
The photo quality isn't great... these were all taken with my cell phone and the lighting was terrible. But they are actual moments captured - - the moments that made me refocus from last Wednesday's appointment and concentrate on all of the Good Chronic in our lives.
Wednesday, August 19, 2009
Head, Shoulders, Knees & Toes (Really... Ears, Throat, Heart & Head)
Audiology
In late July, Gracie had a very long, thorough audiological evaluation... again. With our move late last year, this new group is her sixth audiology group to try and get to the bottom of her hearing loss and the best way to aid her.
At our first visit in April, they did a booth test.
The report reads, "The results indicated a profound hearing loss, bilaterally."
Four sedated ABR tests over Gracie's lifetime show "a profound sensorineural hearing loss, bilaterally."
However, booth tests and social hearing behaviors up until late 2005 showed (sporadic) moderate hearing loss in the right ear and a severe-profound hearing loss in the left ear.
Additionally, we still notice sporadic "hearing behaviors" where Gracie will turn to sound that we know she's hearing - - not feeling. The interesting thing is that these are both high and low tones... which doesn't make much sense given her ABR results over her lifetime.
When we went to the CHARGE Syndrome Conference in 2005, we learned about Auditory Neuropathy and we were convinced at the time that Gracie had this. With Auditory Neuropathy (AN), a patient might have hearing, but sounds is like an out of tune AM station. There are tests that can be done to diagnose this, but Gracie never was a clear-cut case (she's not "clear cut" on anything!) so we never got a confirmed diagnosis... only "suspicions" of AN.
She has, as is often very typical of kids with CHARGE, "a history of chronic middle ear pathology." This is, I am thoroughly convinced, due to the fact that her ears are so "wide open" that her saliva drains out her ears when she has tubes. Her left ear is much worse... it literally pours out on her "junkier" days.
So, back to the appointment in July. This time... a non-sedated ABR was done to rule out and/or confirm Auditory Neuropathy/Dys-Synchrony. "Today's results indicate a profound hearing loss, possibly mixed, bilaterally, with concerns about Auditory Neuropathy in the left ear." So, again, not definitive, but AN pops up again and this time it's actually documented.
In a nutshell, Gracie is profoundly deaf... and even if she weren't, her left ear would cause great difficulty in discerning the sounds due to the AN. And now we are left (yet again) with the big issue of how to aid her... whether she can be aided... whether she'd benefit from a cochlear implant. This is not the first time we've discussed an implant. It's also not the first time that we've struggled with whether it's the right decision. While so many are "pro" implant, I continue struggling with the idea.
Over the years, with certain audiology groups (not our current), it has often felt a bit like, "they have great insurance, what's the harm in giving it a shot?" situation. While I know this isn't entirely true, I also know that there are so many things pointing to the "not implanting" column that no one can really refute. They can only say that there is "a possibility she could, with intensive training, access sound." The "possibility" is what makes me sleepless, stressed and ties my stomach in knots.
Gracie isn't (and has never been) a true aid wearer. She can't wear her BTEs (Behind The Ear aids) because of constant fluid in (or draining out of) her ears. She wears a BC (Bone Conduction Aid) during structured activities while sitting, but will NOT wear it any other time. In theory, a bone conduction aid shouldn't really be helping her (as her loss is primarily sensorineural, not conductive). She doesn't seem to mind the BC aid in those structured activities, but I have always questioned whether she just enjoys the vibro-tactile input rather than gaining any sense of sound from the aid. The fact that she doesn't seem to benefit from the BTEs when she can wear them (which is almost never) is a huge issue. We don't know if she'd even benefit from those aids because she constantly has a fluid problems. What would lead anyone to believe that a child who isn't an aid wearer would be a candidate for an implant? Furthermore, do we believe that Gracie can be trained/taught to use the implant even if it worked...? It's not a matter of just turning it on and "voila!" It's an intensive, complicated process. Does she have the processing skills and cognitive skills to use it? We know she's a smart little cookie, but we also realize how delayed she is and how affected she is by her language processing issues.
Another concern is her facial palsy. Gracie's cranial nerve involvement is so complex... she's "classic" CHARGE... no swallow at all, right-side facial palsy, all inner ear issues, no true blink on either side, her nerves are so small/narrow in some areas that they can't actually confirm presence or completion on a CT. Thus, "opening her up" for a look-see isn't all that appealing to us. Then there is the risk of damaging the good facial nerve on her left side - - a risk that is so frightening to me... she'd have bilateral palsy and lose all facial expression.
As I consider everything, it feels like moving forward with the surgery would be doing something to Gracie rather than something for her.
But, there is still the big, yet very unlikely, "What if it works?" that won't let me be...
Vital Stim
For years, we've tried to get Gracie cleared for Vital Stim. This is a swallow rehabilitation therapy using electric stimulation. Previously, the pacemaker company and her cardiologist weren't comfortable with her receiving the therapy as there wasn't much data. And even if we'd gotten their approval, there weren't any Speech Pathologists in our area who were certified or willing to become certified. They all stated that there wasn't enough data regarding the use of Vital Stim in pediatrics.
When Gracie had her latest swallow study done in the spring, I mentioned the therapy to the speech pathologist who informed me that many of the SP's at CHOC (Children's Hospital Orange County) were certified in administering Vital Stim. She also mentioned that she was fairly certain there were other patients with pacemakers. So, my phone calls started and... now... after nearly four years of hoping and trying... Gracie is approved by the pacemaker company, her cardiologist and our insurance for Vital Stim! She was put on the waiting list and now has her first session next Tuesday.
We are cautiously optimistic (because we like believing there is a miracle left for her swallow), but are also realistic. Vital Stim has not had a great track record for kids with CHARGE Syndrome. There are some successes out there, but most of those kids have a lesser cranial nerve involvement than Gracie.
This is (as of today's technology and medicine) our last remaining effort to work on her swallow. We have tried everything... even Botox into her salivary glands... without success.
Our goal is for her to be able to better manage her secretions... and swallow them enough to avoid aspirating. A great success would be if she'd have enough ability to swallow that she'd tolerate a PMV (Passy Muir Valve - a speaking valve worn over the trach). As of right now, she's not able to wear one due to the "bubble bath" of secretions that sits in the back of her throat and blocks the ability to visualize her vocal cords in a nasal endoscopy. An even greater success with Vital Stim would be that she'd have a chance at decannulation (getting her trach out).
Cardiology
She had a routine check-up and a pacemaker interrogation. Turns out Gracie is using up her battery life on the new generator much faster than initially anticipated. The battery life is estimated at 3.5 years... less than half of what we'd expected. She is completely pacer dependent and this, coupled with the additional work her heart does because of her mitral valve regurgitation, is causing her to use up the battery quickly. She will eventually have to have an artificial valve placed, but we are hoping to do that once she's older and bigger, thus minimizing the need for multiple valve replacements as she grows. She will be on Coumadin (a blood thinner) and require monthly labs once this surgery occurs. Our hope now will be that we can replace her generator and the leads at the same time, rather than having to have an additional surgery a year later to replace the leads.
We go back for another echo in November to check the mitral valve regurgitation. The hope is that it remains stable and her heart doesn't look enlarged from the extra work it does despite the pacemaker.
Neurology
Gracie had an appointment with her new neurologist a couple of weeks ago. Yes - - another new one because the one we saw when we moved here has left the practice. Thankfully, I really like the new one, so it all worked out fine. Unfortunately, though, the appointment was incorrectly scheduled as a return-patient (only 15 minutes instead of 45 - - and my opinion, when it comes to our "complicated" kids, 15 minutes isn't sufficient for a return-patient anyway), so we have to go back again to give the doctor time to get to know Gracie and her history better. I was just grateful that he wanted to take that time.
Good news: He wrote the prescriptions for her new wheelchair and walker :-)
Yay for mobility and independence!
Next medical update just around the corner, will include: Dentist, Vital Stim, GI, Pulmonology, Behavioral Pediatician... it never, ever ends!
In late July, Gracie had a very long, thorough audiological evaluation... again. With our move late last year, this new group is her sixth audiology group to try and get to the bottom of her hearing loss and the best way to aid her.
At our first visit in April, they did a booth test.
The report reads, "The results indicated a profound hearing loss, bilaterally."
Four sedated ABR tests over Gracie's lifetime show "a profound sensorineural hearing loss, bilaterally."
However, booth tests and social hearing behaviors up until late 2005 showed (sporadic) moderate hearing loss in the right ear and a severe-profound hearing loss in the left ear.
Additionally, we still notice sporadic "hearing behaviors" where Gracie will turn to sound that we know she's hearing - - not feeling. The interesting thing is that these are both high and low tones... which doesn't make much sense given her ABR results over her lifetime.
When we went to the CHARGE Syndrome Conference in 2005, we learned about Auditory Neuropathy and we were convinced at the time that Gracie had this. With Auditory Neuropathy (AN), a patient might have hearing, but sounds is like an out of tune AM station. There are tests that can be done to diagnose this, but Gracie never was a clear-cut case (she's not "clear cut" on anything!) so we never got a confirmed diagnosis... only "suspicions" of AN.
She has, as is often very typical of kids with CHARGE, "a history of chronic middle ear pathology." This is, I am thoroughly convinced, due to the fact that her ears are so "wide open" that her saliva drains out her ears when she has tubes. Her left ear is much worse... it literally pours out on her "junkier" days.
So, back to the appointment in July. This time... a non-sedated ABR was done to rule out and/or confirm Auditory Neuropathy/Dys-Synchrony. "Today's results indicate a profound hearing loss, possibly mixed, bilaterally, with concerns about Auditory Neuropathy in the left ear." So, again, not definitive, but AN pops up again and this time it's actually documented.
In a nutshell, Gracie is profoundly deaf... and even if she weren't, her left ear would cause great difficulty in discerning the sounds due to the AN. And now we are left (yet again) with the big issue of how to aid her... whether she can be aided... whether she'd benefit from a cochlear implant. This is not the first time we've discussed an implant. It's also not the first time that we've struggled with whether it's the right decision. While so many are "pro" implant, I continue struggling with the idea.
Over the years, with certain audiology groups (not our current), it has often felt a bit like, "they have great insurance, what's the harm in giving it a shot?" situation. While I know this isn't entirely true, I also know that there are so many things pointing to the "not implanting" column that no one can really refute. They can only say that there is "a possibility she could, with intensive training, access sound." The "possibility" is what makes me sleepless, stressed and ties my stomach in knots.
Gracie isn't (and has never been) a true aid wearer. She can't wear her BTEs (Behind The Ear aids) because of constant fluid in (or draining out of) her ears. She wears a BC (Bone Conduction Aid) during structured activities while sitting, but will NOT wear it any other time. In theory, a bone conduction aid shouldn't really be helping her (as her loss is primarily sensorineural, not conductive). She doesn't seem to mind the BC aid in those structured activities, but I have always questioned whether she just enjoys the vibro-tactile input rather than gaining any sense of sound from the aid. The fact that she doesn't seem to benefit from the BTEs when she can wear them (which is almost never) is a huge issue. We don't know if she'd even benefit from those aids because she constantly has a fluid problems. What would lead anyone to believe that a child who isn't an aid wearer would be a candidate for an implant? Furthermore, do we believe that Gracie can be trained/taught to use the implant even if it worked...? It's not a matter of just turning it on and "voila!" It's an intensive, complicated process. Does she have the processing skills and cognitive skills to use it? We know she's a smart little cookie, but we also realize how delayed she is and how affected she is by her language processing issues.
Another concern is her facial palsy. Gracie's cranial nerve involvement is so complex... she's "classic" CHARGE... no swallow at all, right-side facial palsy, all inner ear issues, no true blink on either side, her nerves are so small/narrow in some areas that they can't actually confirm presence or completion on a CT. Thus, "opening her up" for a look-see isn't all that appealing to us. Then there is the risk of damaging the good facial nerve on her left side - - a risk that is so frightening to me... she'd have bilateral palsy and lose all facial expression.
As I consider everything, it feels like moving forward with the surgery would be doing something to Gracie rather than something for her.
But, there is still the big, yet very unlikely, "What if it works?" that won't let me be...
Vital Stim
For years, we've tried to get Gracie cleared for Vital Stim. This is a swallow rehabilitation therapy using electric stimulation. Previously, the pacemaker company and her cardiologist weren't comfortable with her receiving the therapy as there wasn't much data. And even if we'd gotten their approval, there weren't any Speech Pathologists in our area who were certified or willing to become certified. They all stated that there wasn't enough data regarding the use of Vital Stim in pediatrics.
When Gracie had her latest swallow study done in the spring, I mentioned the therapy to the speech pathologist who informed me that many of the SP's at CHOC (Children's Hospital Orange County) were certified in administering Vital Stim. She also mentioned that she was fairly certain there were other patients with pacemakers. So, my phone calls started and... now... after nearly four years of hoping and trying... Gracie is approved by the pacemaker company, her cardiologist and our insurance for Vital Stim! She was put on the waiting list and now has her first session next Tuesday.
We are cautiously optimistic (because we like believing there is a miracle left for her swallow), but are also realistic. Vital Stim has not had a great track record for kids with CHARGE Syndrome. There are some successes out there, but most of those kids have a lesser cranial nerve involvement than Gracie.
This is (as of today's technology and medicine) our last remaining effort to work on her swallow. We have tried everything... even Botox into her salivary glands... without success.
Our goal is for her to be able to better manage her secretions... and swallow them enough to avoid aspirating. A great success would be if she'd have enough ability to swallow that she'd tolerate a PMV (Passy Muir Valve - a speaking valve worn over the trach). As of right now, she's not able to wear one due to the "bubble bath" of secretions that sits in the back of her throat and blocks the ability to visualize her vocal cords in a nasal endoscopy. An even greater success with Vital Stim would be that she'd have a chance at decannulation (getting her trach out).
Cardiology
She had a routine check-up and a pacemaker interrogation. Turns out Gracie is using up her battery life on the new generator much faster than initially anticipated. The battery life is estimated at 3.5 years... less than half of what we'd expected. She is completely pacer dependent and this, coupled with the additional work her heart does because of her mitral valve regurgitation, is causing her to use up the battery quickly. She will eventually have to have an artificial valve placed, but we are hoping to do that once she's older and bigger, thus minimizing the need for multiple valve replacements as she grows. She will be on Coumadin (a blood thinner) and require monthly labs once this surgery occurs. Our hope now will be that we can replace her generator and the leads at the same time, rather than having to have an additional surgery a year later to replace the leads.
We go back for another echo in November to check the mitral valve regurgitation. The hope is that it remains stable and her heart doesn't look enlarged from the extra work it does despite the pacemaker.
Neurology
Gracie had an appointment with her new neurologist a couple of weeks ago. Yes - - another new one because the one we saw when we moved here has left the practice. Thankfully, I really like the new one, so it all worked out fine. Unfortunately, though, the appointment was incorrectly scheduled as a return-patient (only 15 minutes instead of 45 - - and my opinion, when it comes to our "complicated" kids, 15 minutes isn't sufficient for a return-patient anyway), so we have to go back again to give the doctor time to get to know Gracie and her history better. I was just grateful that he wanted to take that time.
Good news: He wrote the prescriptions for her new wheelchair and walker :-)
Yay for mobility and independence!
Next medical update just around the corner, will include: Dentist, Vital Stim, GI, Pulmonology, Behavioral Pediatician... it never, ever ends!
Wednesday, June 24, 2009
Numbers
1: The number of teeth Gracie has lost (or that was pulled because it was super loose!) this week.
4: The number of new(ish) phrases that Preston is saying that really crack me up. These are the results of watching the NBA playoffs, the US Open and hitting balls off his T in the backyard.
"Yay, made it! Right on!" (Said with two enthusiastic thumbs up!)
"Awww, almost! Try again!"
"Missed it! No hoop"
"Uh-oh, I'll get it, Mama!"
17: The number of years since I have felt this bad from a cold... I have a wicked sore throat, deep cough, low grade fever and scratchy man-like voice right now. I just hope that no one else in the house gets it.
23: The number of times (from her waking until she finally went to sleep late at night) I re-diapered and dressed my daughter on Monday because she insists on taking off everything. (And, no, I am not exaggerating.)
Also the number of times I felt like I was going to have a nervous breakdown.
6: The number of times I cleaned up "potty drama" due to the fact that my little exhibitionist feels the need to go the minute she's diaperless. Ugh.
3: The number of diaper pins now holding her shirt to her shorts so that she can't randomly undress.
4: The number of times she has, so far, outsmarted the pin idea and pulled her arms through her shirt and then pulled (stretched!) the neck of her shirt down to her waist and shimmied out of both shirt and shorts! Can't ever accuse her of being undetermined.
14: The number of meds (not number of doses) that Gracie is currently on. Good grief.
2: The average number of baths that Gracie gets in a day.
I joke about her being Miss OCD and fixating on a bath, but the truth is this has become a huge issue. She absolutely insists on getting in the tub. Once she's in, even if for just five minutes, she's fine after, but then an hour or two later starts asking again. We have decided (for now) to give in to the request if she truly cannot be redirected. (I have re-directed her right now by letting her lay across my lap while I type. She is scrolling through a slideshow on my cell phone!) Anyway, my theory is she is getting input from the water that she can't get from other types of stimulus, like brushing or joint compressions. I'll probably never know... but will definitely be bringing it up at our appointment with the Behavioral Pediatrician this summer... along with all the other obsessive behaviors that are getting worse by the day. (I also wonder if her new meds to help with her pain association during feedings are contributing to her increased OCD and anxiety.)
10 or 11: The number (age in years) that we should expect Gracie to need spine surgery. YAY!!! Great news! We saw the new orthopaedist on Tuesday (Hannah, he totally passes the coffee test!) and he feels that despite the severity of her curve and the obliquity in her pelvis when she stands and walks, Gracie doesn't need surgical intervention at this time (provided that everything progresses slowly and her lung capacity is not further compromised). He also stated that she doesn't need any kind of bracing for her scoliosis or her legs, as he feels that more than benefit her, these things would inhibit her muscle development and make her more apt to give-up working so hard on walking and standing... and she is, indeed, working hard!
3: The number of "catch-up posts" that I've started and not yet published - - one of which is about all of Gracie's hard work at therapy! Despite all of the obsessive behavior, there is a lot of good happening, and that is why I haven't yet become certifiable!
4: The number of new(ish) phrases that Preston is saying that really crack me up. These are the results of watching the NBA playoffs, the US Open and hitting balls off his T in the backyard.
"Yay, made it! Right on!" (Said with two enthusiastic thumbs up!)
"Awww, almost! Try again!"
"Missed it! No hoop"
"Uh-oh, I'll get it, Mama!"
17: The number of years since I have felt this bad from a cold... I have a wicked sore throat, deep cough, low grade fever and scratchy man-like voice right now. I just hope that no one else in the house gets it.
23: The number of times (from her waking until she finally went to sleep late at night) I re-diapered and dressed my daughter on Monday because she insists on taking off everything. (And, no, I am not exaggerating.)
Also the number of times I felt like I was going to have a nervous breakdown.
6: The number of times I cleaned up "potty drama" due to the fact that my little exhibitionist feels the need to go the minute she's diaperless. Ugh.
3: The number of diaper pins now holding her shirt to her shorts so that she can't randomly undress.
4: The number of times she has, so far, outsmarted the pin idea and pulled her arms through her shirt and then pulled (stretched!) the neck of her shirt down to her waist and shimmied out of both shirt and shorts! Can't ever accuse her of being undetermined.
14: The number of meds (not number of doses) that Gracie is currently on. Good grief.
2: The average number of baths that Gracie gets in a day.
I joke about her being Miss OCD and fixating on a bath, but the truth is this has become a huge issue. She absolutely insists on getting in the tub. Once she's in, even if for just five minutes, she's fine after, but then an hour or two later starts asking again. We have decided (for now) to give in to the request if she truly cannot be redirected. (I have re-directed her right now by letting her lay across my lap while I type. She is scrolling through a slideshow on my cell phone!) Anyway, my theory is she is getting input from the water that she can't get from other types of stimulus, like brushing or joint compressions. I'll probably never know... but will definitely be bringing it up at our appointment with the Behavioral Pediatrician this summer... along with all the other obsessive behaviors that are getting worse by the day. (I also wonder if her new meds to help with her pain association during feedings are contributing to her increased OCD and anxiety.)
10 or 11: The number (age in years) that we should expect Gracie to need spine surgery. YAY!!! Great news! We saw the new orthopaedist on Tuesday (Hannah, he totally passes the coffee test!) and he feels that despite the severity of her curve and the obliquity in her pelvis when she stands and walks, Gracie doesn't need surgical intervention at this time (provided that everything progresses slowly and her lung capacity is not further compromised). He also stated that she doesn't need any kind of bracing for her scoliosis or her legs, as he feels that more than benefit her, these things would inhibit her muscle development and make her more apt to give-up working so hard on walking and standing... and she is, indeed, working hard!
3: The number of "catch-up posts" that I've started and not yet published - - one of which is about all of Gracie's hard work at therapy! Despite all of the obsessive behavior, there is a lot of good happening, and that is why I haven't yet become certifiable!
Sunday, June 7, 2009
A priceless gift
For the kids' birthdays, our friend in CHARGE (and for life) and unbelievably talented photographer, Catherine, gave us a photo shoot.
Catherine is a gifted artist... and what she captured for us Wednesday afternoon is undeniably priceless. She posted a few of the photos to her own family blog on Friday (and more on Saturday) and sent me a link to view all 467 shots on Saturday.
John and I looked at all 467 photos Saturday evening and he is, just as I am, absolutely blown away by the gift she's given us. These aren't just photos.. they're art. They show the essence of our children... their spirits... their joy... their love for life, each other, for us and our love for them. Catherine captured that in a way that is indescribable and priceless. Their light shines through these photos (especially Miss Gracie) in a way that I have always hoped to capture, but with the clarity and artistic eye that I never could myself. The Gracie that John and I see and feel but often can't easily photograph - - her energy, her light, her life - - is shining bright in these photos.
At some point, I will share more of our favorites on our blog, but for now, feel free to have a sneak peek on her blog and also check out her photography site.
And if you're even remotely near the Southern California area, don't miss your opportunity to have Catherine capture amazing moments of your family.
Catherine is a gifted artist... and what she captured for us Wednesday afternoon is undeniably priceless. She posted a few of the photos to her own family blog on Friday (and more on Saturday) and sent me a link to view all 467 shots on Saturday.
John and I looked at all 467 photos Saturday evening and he is, just as I am, absolutely blown away by the gift she's given us. These aren't just photos.. they're art. They show the essence of our children... their spirits... their joy... their love for life, each other, for us and our love for them. Catherine captured that in a way that is indescribable and priceless. Their light shines through these photos (especially Miss Gracie) in a way that I have always hoped to capture, but with the clarity and artistic eye that I never could myself. The Gracie that John and I see and feel but often can't easily photograph - - her energy, her light, her life - - is shining bright in these photos.
At some point, I will share more of our favorites on our blog, but for now, feel free to have a sneak peek on her blog and also check out her photography site.
And if you're even remotely near the Southern California area, don't miss your opportunity to have Catherine capture amazing moments of your family.
Thursday, May 28, 2009
LONG Catch-Up: Potty Patrol, Birthday Boy, Humbled Thumbs, IEP, Outpatient Surgery
So it's been a while since I've posted... three words: busy, madness, exhausted!
I had been letting myself feel guilty about not posting on our blog, but since it's my blog and our life dictates how I spend my time, I decided to let that piece of guilt go! I did, however, miss so much that was going on with my blogger friends and 'family' - - some of it not so good and some of it so joyous and wonderful - - and am definitely feeling guilty about that.
Besides being a way for me to "journal" our life and keep a virtual scrapbook, my blogger network has become a wonderful support system and lifeline. Thanks to everyone who emailed, texted and called to check on the Flock... your concern is so sweet and really touched our hearts.
Now... on to business! I last posted on Preston's birthday... nearly three weeks ago. The day before his birthday was a pretty dramatic and somewhat traumatic day...
So... the IEP - - Nana to the rescue. Turned out that John, while he did get the day off, had court for a ticket he'd issued and couldn't attend the meeting... not really a day off. Just our luck. It went well... her services won't change from what was established in the initial IEP 30 days ago, but for the assessment and recommendation that's pending from the Adaptive PE Coach. Her goals have been completely re-written and while I feel that the goals themselves are good, I am still waiting to talk to her teacher a bit more for some clarification on measurements of success and for correction to the document itself regarding present levels. I will explain more about this once I am able to have a conversation with her teacher. I can't mention the IEP without shouting a very public, loud and heartfelt "Thank You" to Leslie, Katie's mom, for providing me with so much valuable and helpful information from Katie's IEPs and evaluations. Gracie and Katie are so similar - - CHARGE Sisters, if you will - - and Leslie's willingness to share information has been of phenomenal help to me. Thank you! :-)
The ENT did her tubes and the bronchoscopy. The bronch showed (by our estimation in comparing our photos of previous bronchs) a fairly happy airway - - the least red we've ever seen, actually. This is excellent news. There are structural anomalies, some that are quite interesting, but none that are compromising her airway and none that require surgical intervention - - aside from the granulation tissue he removed during the bronch. It's a beautiful airway that would work perfectly without the trach... but for the fact that she doesn't swallow at all.
There is a picture from the procedure that is looking down at her carina (the top of the area that "bridges" where the lungs start) and sitting on top of the carina and rolling into her lungs was a giant pool of bubbly saliva. Without the trach, Gracie would drown. Period. If we can't rehab her swallow enough to manage her secretions, she'll never get it out. Not surprising news... not new news, but still so sobering each time it's said.
As for the tubes in her ears... this one was up for debate until the day of surgery. We wavered back and forth because the tubes make Gracie crazy. Her left ear drains constantly and the feeling makes her so irritable. She's so "open" structurally on that side that her saliva actually drains out her left ear... thus it's no wonder that without tubes in her ears, she constantly has fluid in them. We had the ENT put in temporary tubes thinking that if it is too unbearable for her, we will have to take them out or hope they come out on there own quickly. We wavered also because of the fact that she's consistently testing with a profound loss in both ears now. Behaviorally, we haven't seen much response to sound in a while and when we do, it's to things that are incredibly loud or could be things she's feeling rather than hearing. The tubes are there to protect her eardrums from damage... but if she's deaf, what are we really protecting? Constant ear infections haven't plagued her... constant fluid has, though. It's a true catch 22... and we chose (since she was going under no matter what) to give it one more "go round" with the tubes. The (very) unfortunate thing regarding the tubes is that because of the drainage issue, Gracie can't tolerate her BTEs (Behind the Ear Hearing Aids). We've been trying to get her to wear these instead of her BC (Bone Conduction aid) as they provide more appropriate amplification, but it's been a struggle. If she'd have taken to those aids and seemed to be getting any benefit, then we'd have skipped the tubes altogether. It's just unfortunate that we can't really work on wearing those aids now.
The endoscopy was the thing we were most concerned about... the GI team needs to get a good picture of the status of her "gut" and they did. Hearing me talk about her gastritis and her profoundly negative reactions to feedings didn't really sink in until the new GI doc got a look-see for herself. Like her airway, her tummy was not as horribly red as it's been in the past. That's not to say that it wasn't red and bleeding - - it was. But it actually looked better... until she showed us the rest of the pictures. She blew a little air onto the surface of her stomach lining, went in further to get more views and when the camera came back to the stomach, it was horribly raw and bleeding... just from the air. Thus, you can only imagine what formula and meds do to it.
She spent several minutes going over everything with us and then discussing the things we've attempted in the past. I confessed that I'd been skipping her Prevacid because she seems to be uncomfortable when she gets it... it's like the little granules irritate her very raw stomach. She agreed with me and formally discontinued it. We added Nortriptlyne to Gracie's long list of daily meds in the hopes that it would help block out the pain she associates with feeds. So far, we've not noticed a big difference. We'll be patient. If by the weekend, we've not noticed a change, we are supposed to increase to twice a day. If that doesn't work, then she wrote for another medication to be added. If that doesn't work, we will go back to the G-J tube (bypassing her stomach entirely by feeding directly into her jejunum). She's had that tube before, it wasn't any better, really. And, while we're not keen on pumping her full of meds and making her groggy or foggy, we don't have much choice in the matter... she has to eat... she has to gain weight and be able to keep it on. Period. "Failure to thrive" doesn't begin to define how dire this situation continues to be for her.
The good news is that despite being rail thin, having a raw tummy and a drainy left ear, Gracie continues to get more comfortable at school. She looks forward to getting there and the minute I get her out of the car and into her KidKart (adaptive wheelchair/stroller), she is ready to leave me and get inside! I love that! Next week, we bump her days at school from three days to four! Yay... progress!
I had been letting myself feel guilty about not posting on our blog, but since it's my blog and our life dictates how I spend my time, I decided to let that piece of guilt go! I did, however, miss so much that was going on with my blogger friends and 'family' - - some of it not so good and some of it so joyous and wonderful - - and am definitely feeling guilty about that.
Besides being a way for me to "journal" our life and keep a virtual scrapbook, my blogger network has become a wonderful support system and lifeline. Thanks to everyone who emailed, texted and called to check on the Flock... your concern is so sweet and really touched our hearts.
Now... on to business! I last posted on Preston's birthday... nearly three weeks ago. The day before his birthday was a pretty dramatic and somewhat traumatic day...
Potty Drama
I took Gracie to school, ran some errands with Preston, took him to "TOTnastics," came home to pick up a few things and meet the nurse that we have on Fridays (we only have nursing on Fridays...). Our plan had been to go pick Gracie up and head over to a regional park that we've yet to explore. When I unlocked the door, I immediately knew that Sophie (our beloved dog) had been naughty. I typically don't leave her in the house during the day, but there were people coming to look at some work that the landlords were having done and I didn't want her to be in the backyard. Anyway, I could smell the problem before I actually saw it. I'll just say this... when I walked into Gracie's room I literally screamed. Nicole, Gracie's nurse, thought I'd lost my mind. Then she saw it, too... the very light colored carpet in my sweet daughter's room looked more like a leopard print and smelled like a sewage plant. Get the picture? It was obvious she hadn't really been naughty... she was super sick... and it was horrendous. HORRENDOUS.
Needless to say, the park plans were scrapped and the moment we got back to the house after picking Gracie up, I started calling carpet cleaning companies to see who could come out on a Friday afternoon. Preston, thankfully, had fallen asleep in the car and once in his room, was oblivious to the whole situation for a couple of hours. Nicole kept Gracie busy while I spent two hours in her room trying to get a jumpstart on the massive clean-up. I found a company that could come out, but not knowing what time they'd actually arrive, I just couldn't let it all sit.
Needless to say, the park plans were scrapped and the moment we got back to the house after picking Gracie up, I started calling carpet cleaning companies to see who could come out on a Friday afternoon. Preston, thankfully, had fallen asleep in the car and once in his room, was oblivious to the whole situation for a couple of hours. Nicole kept Gracie busy while I spent two hours in her room trying to get a jumpstart on the massive clean-up. I found a company that could come out, but not knowing what time they'd actually arrive, I just couldn't let it all sit.
Since the whole house was due for a cleaning, I took the opportunity to have it all done in one shot. That turned out to be a huge mistake. Our old house had mostly hardwood, but I had the carpeted areas cleaned often and knew what to expect in the way of drying time. This company told me the same things... safe to walk on immediately, completely dry within four hours. Um... not so much. Not even dry 40 hours later. It was a fiasco. Imagine my daughter trying to scoot around on wet carpets... she is a little (a lot) compulsive about things, too... so keeping her on a Neat Sheet was out of the question... she wanted to check out the carpet. And if she laid on it for thirty seconds, I could literally wring out her clothes. She wouldn't sit in any of her chairs once she knew the floor was wet. Ugh. Despite my complaints and threats to cancel payment, the company wouldn't come back out to try and get out the excess water. Nor did they ever call me back on Saturday as I was promised. Meanwhile, I was stuck with sopping wet floors. We were up so late... we used our shop vac to try and suck up water, John went to the grocery store and rented a Rug Doctor, we had all the windows open and fans blowing... it was a wet mess.
Back to Sophie... poor doggie. She's our "firstborn" and we were quite concerned... it was a lot of diarrhea... A LOT. Something was obviously very wrong. The only thing that gave us a bit of comfort was that she still wanted to eat and drink. This, however, was not good for me. That meant she got sick... over and over. I spent the next SIX nights with her sleeping in the kitchen and me "sleeping" on the couch (in between running back and forth to suction Gracie) so I could let her out every 45 minutes. ARE YOU KIDDING ME? THAT is dog love. (She's 12 years old... it's too cold for her old joints to sleep outside and regardless of the temperature, she's always slept inside... outside at night would certainly have caused neighborhood disturbance in the way of howling or barking.) If I didn't hear her quiet pleas in the wee hours of the night (which at one point every night for six nights, I didn't) then she'd go all over my kitchen. I spent every morning for six mornings mopping, scrubbing grout and disinfecting my kitchen floor. Every morning... for six mornings. Because of the bout with doggie dysentery, I couldn't let the kids in the backyard... there's no way to protect them from that mess. And as luck would have it, this started on day three of John's 8-day stretch at work. So, I couldn't even get her into the vet with both kids in tow, it was impossible. Because she was eating and drinking, we decided to wait it out and hope that she had a bug of some sort. Those first few days, though, we were incredibly worried and even had to start having "the talk" about how much is too much for her to go through. Thankfully, she is fully recovered. But that was just the start of our potty drama...
Gracie has some "habits" that are a little less than ladylike... and one of them is to strip naked at the drop of a hat when she is ready for a bath. Thankfully, she only does this at home because she knows that is where bathtime occurs. Thank goodness for small favors! It doesn't matter what the time of day is... if she feels that it's bathtime, then she will get naked. You can put her clothes back on and tell her "no" but she will cry and kick and fight... and take them off again. (She also strips naked in the morning if she's awake for more than a minute or two... to0 long for Princess Gracie to be waiting to get dressed!) Inevitably, the moment she's naked, she feels the need to "go." I don't know if it's the fresh air hitting her nether region that gives her the urge, or if it's intentional. It's never much, so I'd like to think it's not intentional, but any amount is too much to have to deal with when she's begging for a bath and Preston is thinking it's pretty funny that she's naked and decides to strip his diaper off and go, too.
I've pretty much curbed his behavior. He gets that his sister does things that he's not supposed to do. He knows that what she's doing is naughty and I think he even understands the fact that his naughty behavior has far different consequences than hers. Nevertheless, this all started in the days that Sophie was in the midst of her illness and I was at my wits end. It was not pretty... on so many levels! For six days, no matter how much I scrubbed my house or myself, I never felt clean. As Preston would say, "Ewww, yuck!"
Back to Sophie... poor doggie. She's our "firstborn" and we were quite concerned... it was a lot of diarrhea... A LOT. Something was obviously very wrong. The only thing that gave us a bit of comfort was that she still wanted to eat and drink. This, however, was not good for me. That meant she got sick... over and over. I spent the next SIX nights with her sleeping in the kitchen and me "sleeping" on the couch (in between running back and forth to suction Gracie) so I could let her out every 45 minutes. ARE YOU KIDDING ME? THAT is dog love. (She's 12 years old... it's too cold for her old joints to sleep outside and regardless of the temperature, she's always slept inside... outside at night would certainly have caused neighborhood disturbance in the way of howling or barking.) If I didn't hear her quiet pleas in the wee hours of the night (which at one point every night for six nights, I didn't) then she'd go all over my kitchen. I spent every morning for six mornings mopping, scrubbing grout and disinfecting my kitchen floor. Every morning... for six mornings. Because of the bout with doggie dysentery, I couldn't let the kids in the backyard... there's no way to protect them from that mess. And as luck would have it, this started on day three of John's 8-day stretch at work. So, I couldn't even get her into the vet with both kids in tow, it was impossible. Because she was eating and drinking, we decided to wait it out and hope that she had a bug of some sort. Those first few days, though, we were incredibly worried and even had to start having "the talk" about how much is too much for her to go through. Thankfully, she is fully recovered. But that was just the start of our potty drama...
Gracie has some "habits" that are a little less than ladylike... and one of them is to strip naked at the drop of a hat when she is ready for a bath. Thankfully, she only does this at home because she knows that is where bathtime occurs. Thank goodness for small favors! It doesn't matter what the time of day is... if she feels that it's bathtime, then she will get naked. You can put her clothes back on and tell her "no" but she will cry and kick and fight... and take them off again. (She also strips naked in the morning if she's awake for more than a minute or two... to0 long for Princess Gracie to be waiting to get dressed!) Inevitably, the moment she's naked, she feels the need to "go." I don't know if it's the fresh air hitting her nether region that gives her the urge, or if it's intentional. It's never much, so I'd like to think it's not intentional, but any amount is too much to have to deal with when she's begging for a bath and Preston is thinking it's pretty funny that she's naked and decides to strip his diaper off and go, too.
I've pretty much curbed his behavior. He gets that his sister does things that he's not supposed to do. He knows that what she's doing is naughty and I think he even understands the fact that his naughty behavior has far different consequences than hers. Nevertheless, this all started in the days that Sophie was in the midst of her illness and I was at my wits end. It was not pretty... on so many levels! For six days, no matter how much I scrubbed my house or myself, I never felt clean. As Preston would say, "Ewww, yuck!"
Birthday Boy
Despite the fact that our house was quite soggy and the backyard was off limits, my little man managed to have a very fun 2nd birthday. He helped me make his cake... he poured, mixed, tasted cake batter even though I told him it wasn't yummy until it's baked, did dishes, licked spatulas, licked the beaters, shared with his sister, ate way too much frosting and loved every minute of it. 
Humbled Thumbs
A reason that I've been unreachable by cell phone and text is that we got new cell phones - - touch screen Blackberrys - - and while the whole idea of them is very cool, using them has not been, at least not for me. Turns out my previously speedy thumbs are now useless - - they are too chubby for the keyboard and I keep hitting the wrong keys. I never really thought my thumb tips were all that fat, so this has been quite humbling for my opposable digits! For a good ten days I played with that stupid phone trying to get the hang of it, all the while typing a total mess of a text message and not making any sense. One sentence was taking me five minutes. We got them because John's phone went kaput and the Blackberrys are buy one get one free right now. We are (or at least he is) trying to bring me out of the dark ages. I still carry a very fat, overloaded daytimer that holds so much important information that I am convinced the earth would literally stop rotating if I were to lose it. It weighs down my purse and takes up way too much room... but I haven't been able to part with it. So far, like with the palm pilot and other "toys" I've tried over the years, I have not yet been converted. To add insult to injury, neither of our phones are connecting to any of our bluetooth devices. I have a Jabra in the car... which I LOVE... but it's been sitting on my visor, so sad and neglected! Clearly, the issue is not with our phones or the devices, it's all operator error. We just haven't had the time to solve the problem. Grrr.IEP
As for Miss Gracie - - things in her world have been very busy. We had her IEP on Wednesday of last week. Thankfully, my mom came down to give us a hand so that John could attend with me this time. She was also here to help us on Thursday because Gracie had outpatient surgery and procedures at the hospital and we both really wanted to be there.So... the IEP - - Nana to the rescue. Turned out that John, while he did get the day off, had court for a ticket he'd issued and couldn't attend the meeting... not really a day off. Just our luck. It went well... her services won't change from what was established in the initial IEP 30 days ago, but for the assessment and recommendation that's pending from the Adaptive PE Coach. Her goals have been completely re-written and while I feel that the goals themselves are good, I am still waiting to talk to her teacher a bit more for some clarification on measurements of success and for correction to the document itself regarding present levels. I will explain more about this once I am able to have a conversation with her teacher. I can't mention the IEP without shouting a very public, loud and heartfelt "Thank You" to Leslie, Katie's mom, for providing me with so much valuable and helpful information from Katie's IEPs and evaluations. Gracie and Katie are so similar - - CHARGE Sisters, if you will - - and Leslie's willingness to share information has been of phenomenal help to me. Thank you! :-)
Outpatient Surgery
Is it terrible to say that it wasn't a big deal in the world of Gracie and surgeries? That is not to say that anesthesia isn't scary and that the discomfort and loopiness she experienced wasn't a big deal... it was, of course. But as far as "hospital time" goes, this was the way to do it. In at 5am, out by 9ish. She had a bronchoscopy, an endoscopy with biopsy and a new set of tubes in her ears.It was really strange to not know a soul at the hospital, to not know where we were once inside, not know the routine. Quite unsettling for two very "hospital seasoned" parents. Thankfully, Gracie was happy as a clam the entire time we waited in the ambulatory care unit. She hung out, watched some Disney Channel, signed her ABC's and then smiled as they wheeled her into the OR. She wasn't so thrilled when she woke-up... rightfully so. But, once back in the car, she was loopy, but happy.
The day was mixed... good and bad results. The bad was not a surprise, just confirmation to the doctors that I am not crazy and that what I have been telling them about her (because some doctors STILL haven't gotten records down here... unacceptable) is, in fact, the reality.
The day was mixed... good and bad results. The bad was not a surprise, just confirmation to the doctors that I am not crazy and that what I have been telling them about her (because some doctors STILL haven't gotten records down here... unacceptable) is, in fact, the reality.
The ENT did her tubes and the bronchoscopy. The bronch showed (by our estimation in comparing our photos of previous bronchs) a fairly happy airway - - the least red we've ever seen, actually. This is excellent news. There are structural anomalies, some that are quite interesting, but none that are compromising her airway and none that require surgical intervention - - aside from the granulation tissue he removed during the bronch. It's a beautiful airway that would work perfectly without the trach... but for the fact that she doesn't swallow at all.
There is a picture from the procedure that is looking down at her carina (the top of the area that "bridges" where the lungs start) and sitting on top of the carina and rolling into her lungs was a giant pool of bubbly saliva. Without the trach, Gracie would drown. Period. If we can't rehab her swallow enough to manage her secretions, she'll never get it out. Not surprising news... not new news, but still so sobering each time it's said.
As for the tubes in her ears... this one was up for debate until the day of surgery. We wavered back and forth because the tubes make Gracie crazy. Her left ear drains constantly and the feeling makes her so irritable. She's so "open" structurally on that side that her saliva actually drains out her left ear... thus it's no wonder that without tubes in her ears, she constantly has fluid in them. We had the ENT put in temporary tubes thinking that if it is too unbearable for her, we will have to take them out or hope they come out on there own quickly. We wavered also because of the fact that she's consistently testing with a profound loss in both ears now. Behaviorally, we haven't seen much response to sound in a while and when we do, it's to things that are incredibly loud or could be things she's feeling rather than hearing. The tubes are there to protect her eardrums from damage... but if she's deaf, what are we really protecting? Constant ear infections haven't plagued her... constant fluid has, though. It's a true catch 22... and we chose (since she was going under no matter what) to give it one more "go round" with the tubes. The (very) unfortunate thing regarding the tubes is that because of the drainage issue, Gracie can't tolerate her BTEs (Behind the Ear Hearing Aids). We've been trying to get her to wear these instead of her BC (Bone Conduction aid) as they provide more appropriate amplification, but it's been a struggle. If she'd have taken to those aids and seemed to be getting any benefit, then we'd have skipped the tubes altogether. It's just unfortunate that we can't really work on wearing those aids now.
The endoscopy was the thing we were most concerned about... the GI team needs to get a good picture of the status of her "gut" and they did. Hearing me talk about her gastritis and her profoundly negative reactions to feedings didn't really sink in until the new GI doc got a look-see for herself. Like her airway, her tummy was not as horribly red as it's been in the past. That's not to say that it wasn't red and bleeding - - it was. But it actually looked better... until she showed us the rest of the pictures. She blew a little air onto the surface of her stomach lining, went in further to get more views and when the camera came back to the stomach, it was horribly raw and bleeding... just from the air. Thus, you can only imagine what formula and meds do to it.
She spent several minutes going over everything with us and then discussing the things we've attempted in the past. I confessed that I'd been skipping her Prevacid because she seems to be uncomfortable when she gets it... it's like the little granules irritate her very raw stomach. She agreed with me and formally discontinued it. We added Nortriptlyne to Gracie's long list of daily meds in the hopes that it would help block out the pain she associates with feeds. So far, we've not noticed a big difference. We'll be patient. If by the weekend, we've not noticed a change, we are supposed to increase to twice a day. If that doesn't work, then she wrote for another medication to be added. If that doesn't work, we will go back to the G-J tube (bypassing her stomach entirely by feeding directly into her jejunum). She's had that tube before, it wasn't any better, really. And, while we're not keen on pumping her full of meds and making her groggy or foggy, we don't have much choice in the matter... she has to eat... she has to gain weight and be able to keep it on. Period. "Failure to thrive" doesn't begin to define how dire this situation continues to be for her.
The good news is that despite being rail thin, having a raw tummy and a drainy left ear, Gracie continues to get more comfortable at school. She looks forward to getting there and the minute I get her out of the car and into her KidKart (adaptive wheelchair/stroller), she is ready to leave me and get inside! I love that! Next week, we bump her days at school from three days to four! Yay... progress!
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