Love and Loathe

This photo... while a simple x-ray to you, speaks volumes to me. 
It is an image I both love and loathe.
It is Gracie's spine and was taken on March 19th, 2015.

I love it because it is possible. 
Five years ago (and all the years before that), no one would have believed that this x-ray of Gracie's spine (and more) would be possible.  Five years ago, she was clinging to life, battling four rounds of septic shock in a matter of five months.  Five years ago, the doctors told us (more than once) to prepare for the worst.  That her little body couldn't take much more.  Five years ago, I sat at Gracie's bedside and though I begged her to fight, I also gave her permission to stop.  Actually, if I am being totally honest, I also begged her to stop because it felt so selfish to beg her to fight.  I prayed (though I don't really know to who or how) to let her rest, to take her if that meant that the constant pain, sickness and suffering would go away.  If you don't understand how I could think that about my own child, I don't really care.  You haven't walked in my shoes and you sure as heck haven't walked in hers.  Until your child has coded (on multiple occasions over several years), until you've left the room to simply go to the bathroom and come back to find a crash cart and eleven people working on her, until you've been told to expect the worst time and time again, until you've read and signed DNR paperwork, you can't possibly understand where we were five years ago... or nine years ago... or nearly twelve years ago.  But, that's another post on its own... back to why I love this image.  I love it because it is possible. 

I love it because it shows, in all her glory, how bionic Gracie is. 
She is totally bionic (and she'll be more bionic soon).  Don't be jealous that you're not bionic... she earned this multi-million dollar, one of a kind body.  She fought harder than anyone I know to keep that bionic body.  But in this image, you can see evidence of battle wounds, equipment, hardware... the whole shebang is there, not covered by anything.  The only thing you can't see here are the dozens of scars... and the scars are a lot harder to look at than her x-ray image... the inside is just cool and super bionic.  My daughter is badass. 

I love it because it shows her perfect little child-sized muffin top.
Gracie has fought hard for every pound and every inch of growth her entire life.  I have counted every calorie and every milliliter of hydration that has entered her body since the day she was born.   Just two and a half years ago she was 29 pounds.  Today, she is a healthy 52+ pounds (and counting).  She is growing and gaining and that is nothing short of a miracle.  The amount of tears shed over her feeding issues could solve the drought in California... the number of times she begged (and still does) not to eat, the GI issues, the central nervous system issues, the autonomic issues... the list is long and dense.  Those little rolls of chub are a medal - - a badge of honor.  And they are an insurance policy... they represent the extra weight she needs to be able to put up her next big fight.  And that, I am sad to say, brings me to all the reasons I loathe this image.

I loathe this image because it shows the imperfection in Gracie's spine... the imperfection that must now be repaired. 
It shows her scoliosis.  It shows her hip rotation.  It shows evidence of her kyphosis.  It shows what still needs to be fixed.  The curve, while bad, isn't as bad as many.  But what these conditions are doing to her heart and lungs and general well-being are, without question, bad. The damage that has been done cannot be undone by straightening her out (she won't ever be totally straight).  What we do, by straightening her spine - - fusing it with hardware and keeping it permanently straight (and making Gracie more bionic) - - is prevent further deterioration.  We stop additional damage to vital organs and nerves. We give her body the best chance possible to continue this epic fight it has so valiantly, so tirelessly, fought. 

She needs this surgery.  We've known that since she was a baby.  It's not news.  We've been prolonging this for quite some time.  Two years ago her new surgeon (whom we've now grown to really love and respect) recommended we do it that summer.  We asked for some time to let her grow, feel as well as she can (by Gracie's standards, of course), gain some weight... just time.  He, of course, had to go by our decision, as it is ultimately ours.  But he did so while expressing that if the curve progresses, the damage does, too.  Our decision was the right one.  She has gained a ton of weight, has grown a few inches, and, until just six months ago, her back remained fairly stable.  But in the last six months there's been change.  I knew it before I saw the x-ray.  I could see it in her posture.  The time has come.  And I am terrified. 

I loathe this image because it brings to the surface every emotion I've been suppressing regarding this surgery.  Until last Thursday, I was able to tuck those emotions into a compartment called "not today."  My "not today" section is rather large, but it is also useful.  It allows me to be in survival mode when needed, dealing with only the things I have to "today" and leaving the rest to reside safely there until I have time to stress over them, worry about them, harp over them, be angry about them... whatever the case may be.  It's where I stick the crap I don't need to worry about at that given moment.  It's how I stay (somewhat) sane.  I cannot suppress this anymore. This has permanently come out of my "not today" compartment and is screaming loud and clear at me every waking moment.  And surgery won't be until September... it gives us plenty of time to do all the pre-op clearances (and there are many, after all, you don't become bionic and badass without seeing a whole team of specialists) and give Gracie her summer to be in the swimming pool as much as possible.  But, even with six months to go, these emotions are out of that safe compartment and I am terrified on so many levels.

How do we make her understand that she will go in feeling "fine" and come out miserable for weeks, even months, on end?  How do we help her comprehend the gravity of the facts - - that she will spend approximately two weeks in the PICU, she will be on pain medication for another six weeks, she will be forced to wear a brace, she won't be able to go to school, she will need rigorous physical therapy?  We can't.  Not fully.  She won't understand and that just breaks my heart.

How do we subject her to the numerous risks... so many... too many... too grim... to list here.  I can't wrap my head around any of it.  I can't bear to think of the what-ifs.  But, we also can't bear to think of the what-ifs if she doesn't have the surgery.  It is all terrifying and I loathe it.

But the thing that terrifies me the most, is the potential psychological ramifications for Gracie.  She has a way of moving her body, of exercising, of just "being" that is very specific.  We call a lot of what she does "Gracie Yoga."  Her little "bionic" body might not be able to walk independently, but she sure can do some truly amazing things with it.   Her core, even with all the new, extra padding around her middle, is stronger than mine... and dare I say, yours!  (If I was smarter, I would spend 30 minutes a day copying what she does... I could make an ab workout podcast and become an internet sensation!)  Often, the way she moves her body, provides her comfort, stability and the proprioceptive input she so badly needs given all her other sensory deficits.  Her way of moving and positioning is the very thing that she can do to self-soothe. But, all this positioning and work that she does will be impossible once she has the surgery.  I will say it again... "Gracie Yoga" will no longer be possible once she has her spine surgery.  While she is the most resilient person I know and the most strong-willed, taking that power and comfort from her and replacing it with, what will be for a while, unbearable discomfort and total lack of knowledge of her own body, sounds barbaric and brutal - - because it is. 

There is nothing John nor I can do to prepare her for that.  There is nothing we can do to prepare ourselves for that.  We can't know what anything will look like on the other side of this surgery.   My "not today" compartment has been ripped open and too much stuff came flying out. I am terrified to my very core... and that, I loathe.

Pacemaker Change

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. 

Celebrating a Stable Spine

In a stunning and miraculous turn of events, Gracie's scoliosis has remained stable (her kyphosis deteriorated slightly) over the last four months.  This was so unexpected - - so unexpected, in fact, that I literally almost feel hung over from the (wonderful) letdown of anxiety and emotions. 
These findings, coupled with her linear growth and weight gain has resulted in her surgeon saying, "She bought herself some time."  Today started with us thinking we were setting a date for a complete spine fusion and ends with at least six more months surgery free.  We will take it!

This is not something we take lightly... we know that this surgery will happen, it's just a matter of when.  We chose, despite popular medical opinion (well, her current team's opinion... our SoCal team had varied opinions), to postpone last year when her surgeon recommended we move forward at that time.  Gracie needed time to just "be" and not deal with another complex invasion of her body.  We were afraid, that at her weight, it wouldn't be as safe and healthy to go through that kind of trauma.   The urgency, in our opinion, was to do everything within our power to increase her caloric intake, manage her nutrition better and get some weight on her... something that had been impossible her entire life.  I made it my mission (not that I hadn't before), but there was even more at stake... buying time for her growth before stopping all growth through her spine.  It was the right decision for her on every level. 

SICK of 2014 already

This is Gracie on December 28th, buried under blankets and hooked up to her Vest machine. 
She was miserable... for three days.

Gavin on December 29th. 
We literally fell like dominoes.

We rang in the new year with the violent, three day stomach flu... ALL FIVE OF US.  Happy Puke Year.  It was awful.  The I-don't-know-how-anyone-got-taken-care-of kind of awful.  It started with Gracie, but, with her, we never know if it's "just Gracie" or if there is something going on.  She couldn't eat without retching, she had a low-grade fever, she wanted to be under blankets... she was miserable.  Gavin and Preston had colds and, with that, Gavin always gets the croup.  So, I didn't really think Gracie had the stomach flu.  But, then Gavin projectiled all across the kitchen two days later (and then everywhere else), I knew for sure.  Then I got it on the 30th and John and Preston later that same night.  On New Year's Eve, I vaguely remember crawling to the fridge for fluids, handing stuff to Gavin in a sippy cup and literally falling asleep next to him halfway on our tile and family room rug.  It was brutal.  BRUTAL.

Then Gracie's fractured ankle.

Now... everyone except Gavin has the flu.  Influenza Type A.  Also BRUTAL.  Preston was the first to fall victim (along with several kids from his class).  His fever hung around 101.4, even with meds, and climbed fast with the slightest window in between.  Despite wiping every surface in the house multiple times, a nasty form of "ick" invaded our home again... perhaps because this boy cannot keep his hands out of his mouth trying to wiggle teeth!?  Ha!

My poor little man.

Then I got it, then John, and despite my most valiant efforts, Gracie.  And that, I am sad to say, landed her in the hospital - - mostly for observation and the need for oxygen at night.  That was our first experience inpatient at Children's Oakland and, though the staff was nice and our wonderful pulmonologist even stopped by (after a meeting, he wasn't even on call), the "accommodations" are less than desirable.  Eeek.  She has a long road of healing before she can get back to school, but at least they let me take her home where she is comfiest.

Gracie's aide, Kaitlyn, dropped this by before we got home from the hospital. 
She is still so sick, but at least she is home in our little make-shift hospital.

Gracie's awesome team made this for her from her entire class!

Fractured Fashonista

Check out our little Diva's left foot.
Yep, that's a cast - - and cute toes, of course!

 On January 2nd, Gracie's ankle was fractured in two places following a massage and stretching with her nurse.  While her nurse clearly should have used less pressure and force, it wasn't entirely her fault.  Gracie has low bone density in her lower extremities due to lack of weight bearing and nutritional issues for the better part of nine years. 

You can see the lack of bone density here... her bones should be very bright and glowing instead of faded and almost transparent in places.  The ER doc and radiologist told me to be prepared for more of these as she ages.  Ugh. 

The good news is that is wasn't vascular - - her pediatrician had me rush her to the ER on the 2nd thinking she had a blood clot (DVT) rather than a break.  I had called in requesting an x-ray order because she suddenly wouldn't weight bear as we were leaving for a park outing.  He wanted us to be seen.  So along with Gracie's nurse, I rushed all three kids into the pediatrician to then have him tell me under no uncertain terms to rush her down to Children's in Oakland... 40+ miles away.  "Don't even stop at home first," he said.  (I did... I needed stuff for my kids and I needed to get Gracie's nurse back to her car.)

We rushed down to Oakland, John abruptly left work and met us alongside the freeway where we quickly exchanged the boys.  Gracie's nurse had been kind enough to offer to watch the boys in the lobby of the hospital until John got there, but he literally flew through multiple freeways to meet me. 
When I got Gracie to the ER, they were expecting us (those who had been waiting for hours were not so friendly and we had lots to say about us getting right in... including screaming racial slurs at us and the nursing staff who pulled us to the back immediately.  Sometimes I am so grateful Gracie is deaf and oblivious to that part of life.)  Anyway, once they examined her, they all seemed very underwhelmed and said they figured it was a fracture.  I said I figured so, too, but that the pediatrician literally freaked when he saw her leg swelling straight up past her knee and felt how hot it was.  Thankfully, he was wrong.

The bad news is this fracture is likely the first of many to come due to the fragility of her bones.  They put on a soft cast in the ER and by the time we drove all the way back home (at 10 something at night), Gracie had torn it off.  She got her hard cast on Monday and literally cried for four straight hours before conceding and giving into the fact that it won't come off.  We all know how this girl likes to be sock/shoe/clothes-less the minute she gets home... she was fit to be tied.  Now, though, she accepts it and is standing in it, doing her "Gracie Yoga" and pretty much back to normal.  And, in true Gracie fashion, she handled the pain like a champ.  Unless it was touched by someone other than her, she didn't really complain of pain hardly at all.  She even cruised furniture a bit before she was casted.  OUCH! 

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:

"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.

Still battling the scale... Reconsidering TPN

We saw the GI doctor last week and Gracie was in a fantastic mood, wheeling herself all around the clinic to look at the pictures of all the kids on the walls. She was bossy, focused and insistent that she see every single picture. If I tried to redirect her toward the parking lot elevator, she pushed my hand away and wheeled as fast as she could in the opposite direction. I love when she is like that (even when it’s frustrating)!  Unfortunately, that was the best part of last Tuesday's clinic visit.

Her doc was less than thrilled with the fact that she has lost two more pounds (as am I, obviously, and I didn't need a clinic visit to know that she is, once again, going in the wrong direction). Last year, after the summer of horror, we had her up to 33 pounds. As the space between ends and another round of feeding issues begins (due to abdominal migraines, sinus infections, respiratory infections, gastritis and general intolerance at times), we're fighting to keep Pedialyte in her... calories are not even an option. If we were to hospitalize her every time she is in this state, she would be inpatient more than out.  And, they wouldn't be doing anything for her that we can't do at home - - or that we haven’t already tried numerous times. Needless to say, we are constantly chasing her weight and trying to build back up to her highest weight.  As months pass, the "high" number we are chasing starts to get lower and lower. That is, a year ago she was 33 pounds and after every illness we would do our best to pump extra calories in and try to get her back up to that weight before the next issue set in. Six months ago we were chasing 31 pounds and doing so fairly well. Last Tuesday (the 16th), she was 29.5 pounds and having a great day... two days later, she was down for the count, retching continuously for four days, barely tolerating Pedialyte and barely sleeping. It was a nasty abdominal migraine. I refuse to weigh her until I have five solid days of full feeds back into her little system... but I know she dropped more. Yesterday was the first time in nine days that her intake was over 1,000 calories… her goal is 1300.

Her current weight and constant battle with feedings prompted the GI doctor to suggest another round of home TPN. (If you don't remember how that went last year, you can read more about her summer in the PICU caused by her issues with having a central line by reading all the posts between June, July and August of last year… it wasn’t pretty.) The whole team agreed at the end of our summer of horror that it will be incredibly difficult to get a line in her again, let alone keep her infection free. Every line placed last year became infected no matter where she was... home or hospital. And, the infections (and yeast) were so bad, she was septic four times and in septic shock once. It's not a place any of us want to revisit. Yet, we can't continue on this path of weight loss either. Unfortunately, her body fights so hard and burns so many calories for her heart to function, for her breathing, etc., that when she is well, she often burns more than we can get into her. So, when she is sick, you can imagine how hard her body works to fight infection or pain on top of the regular work it does. 

The issues are deep. In her eight-plus years, we’ve tried every med, every type of feeding and feeding tube, multiple alternative medicine approaches and she has had surgery to repair every possible issue. The problem is, the roots of Gracie’s feeding issues are Central Nervous System (CNS) and Neurological and, at this point, there is nothing left to try.   What will we do? I have no idea. We go back in three months… or sooner if things get any worse. For now, we are continuing to ride it out, and do our best to beef her up in the space between.

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!

"What?!"

"What?" Gracie signed, as I stood near the photographer trying to get her to look at the camera and smile on picture make-up day.

Since Gracie was scheduled to get her picture taken right when I dropped her off on make-up day, I stayed to help. It was comical... my girl wanted to turn around on the little chest they have the kids sitting on. She wanted to smile but turn her head completely one way or the other - - never front and center. So, when she opened her mouth wide and signed "What?" at me (which she does often) and I heard that the photographer got it, I knew that would be the picture I chose. Turns out there were two I liked, but this one, is by far, my favorite. It is so quintessentially Gracie and makes me smile, even chuckle, when I look at it.

We continue to deal with infection type illnesses (on top of abdominal migraines) and CHOC's Infectious Disease team has been following Gracie closely. We have become regulars at the lab, going nearly every week since October for multiple vials of blood at a time. The last visit, Gracie wheeled into the room, stuck out her arm with confidence and tapped the crook of her elbow with her forefinger. She is "trooper" defined - - she even watches the whole process and is clearly more bothered by the tourniquet than the needle itself.

Thus far, we've learned enough to create more of a mystery. In October, Gracie had strep-pneumo (Streptococcus pneumoniae) which lead to her pneumonia and another ICU stay. This is something she was immunized for as a toddler. We would expect that with the recent exposure and the immunization, she'd have at least some antibody response. You or I would... at least something. But, Gracie didn't. Zero. Nada. Zip. The Infectious Disease team is puzzled and ordered more lab work to test her antibodies to everything else she has been immunized for in order to see what her responses are. From those results, we will (hopefully) come up with a plan. The expectation is that Gracie has some sort of autoimmune disorder, but if one thing is certain with her, it's to expect the unexpected. I'll post more as we learn.

Back to the good stuff... Gracie's longest stretch of "space between" has been two weeks and five days. Since the last post about the shopping carts, we've had two periods of good space and, as it turned out, that longest stretch had perfect timing. Though John took a tremendous amount of leave over the four month period when Gracie was in the hospital a total of 48 days, he still had a two week vacation slated during Thanksgiving and the week prior. We tried to get him back on the schedule for one of the weeks, not wanting to use up more of his leave in case it is really needed. Turns out, it was needed... for all the right reasons. The long "space between" for Gracie fell directly into the time John was off... and we enjoyed every bit of it. (She lost four days the first week of December due to an abdominal migraine cycle, but has been back "in the game" since Monday the 6th.)

In our true "seize the moment" mindset, we have made every effort to capitalize on the moments that truly allow us to celebrate the "space between" and, even if just briefly, forget about the space to come. Whether it's the little things - - something Gracie did at school, sweet things that Preston says, milestones reached, or the regular, day-to-day stuff that makes me smile simply because it's regular, day-to-day stuff (like the shopping cart)... or the big things (like our family trip to visit my brother and his family in Arizona for Thanksgiving), there has been much to celebrate.

In addition to her remarkable resolve at the lab (which, while sad on the one hand because she is so familiar with the process, is actually really happy in that she is tolerant, she understands and she communicated clearly), Gracie has been feisty, full of energy and making progress on all levels. She has, in recent weeks...

- started back to therapy (Occupational and Physical Therapy... twice a week each, after school. Hopefully, after the start of the year, she'll resume VitalStim.) She is working hard and though she's lost strength and stamina in some areas, she has increased in others. Her (vastly) improved nutritional status has made a tremendous positive difference, but has also caused a need for her to get reacquainted with her body, as it is 25% heavier and she is growing.

- has started doing her vest therapy in all kinds of positions... sometimes for postural drainage reasons, which is really quite smart! She also has been scooting on her bottom (more than her back), pulling herself up to stand more, requesting to stand more and cruising furniture (to the point that I'm afraid to step away!)


- wheeled herself around the Aquarium of the Pacific in Long Beach for the first time. She had a ball! She does, however, have a lot to learn about following directions (she LOVES being in control of where she goes and when, much like a toddler!) and the personal space of other people. She will just wheel herself right up to someone and wrap her legs around them or touch them if she wants to get more acquainted! Cute, but also not necessarily appropriate!


- come home with this note in her communication book...

"Hi Kristi,
Gracie worked well today. She did great in APE. Gracie caught the ball. Betty Lou (her aide) was holding her by her hips."

Do you realize, for Gracie, how much coordination that takes standing, even with someone supporting her hips? So cool!

- signed "wrong" at school when she was seated at the table with her friends for a painting activity. "Wrong" can mean that something is wrong with her, like pain. It also can mean "no." On this day (and others since), it meant that something was wrong with the situation. Gracie didn't get a paint shirt put on (because she often protests extra layers over her clothes and then wants to remove everything!) and all of her peers had theirs on. She did not stop signing "wrong" until they figured out that she just wanted a paint shirt like her friends!

- added to her bath time routine. After walking into the bathroom with hand-held assistance, she will climb the two steps of the stool, grab the counter (I will then let go of her) and she will reach over, turn on the light (perhaps several times!) sign, "light on, light off," admire herself in the mirror, brush her teeth (if I set it up for her), turn on and off the water, brush her hair, etc. SO CUTE!!! She will then, with hand-held assistance, carefully search for the step down and make her way to the tub - - all wonderful use of the vision she has and her awareness of her safety and the depth of the steps.


- really turned into a diva! Little Miss LOVES getting dolled up and especially enjoys getting her hair curled. (And, she still won't keep her clothes on! Grr!)

Pa's Pumpkin Patch

On Tuesday afternoon, we took the kids to "Pa's Pumpkin Patch" in Long Beach. Aside from sitting in her chair for about 90 minutes at a birthday party last Saturday, it was Gracie's first big outing since being discharged from the hospital. It's not too far from our house... and is on the corner of Pacific Coast Highway and 2nd Street... so it is a "patch" of parking lot! But, the kids loved it. And, because of that, so did we! It is strange, though, to hear traffic from PCH and literally smell the salt water while picking out a pumpkin!

When Gracie was discharged last Thursday, they said she could resume all normal activity. I chose to keep her home, let her build some strength and, as I mentioned in a previous post, allowed myself to get us into a routine with her new marathon of treatments. As many of you know from facebook, Gracie went back to school Wednesday, had a good day and then spiked a low-grade fever that evening. During the night, her fever persisted and her secretions increased with a vengeance. The likelihood that this development is "bacterial" in nature is slim... she is on two heavy-duty, broad spectrum antibiotics. Her labs taken on Tuesday afternoon looked improved from discharge, so that is also a good sign, however, the labs weren't ordered to look for any fungal issues (like the yeast she developed in her PICC line over the summer) which are always a possibility when on antibiotics so much and for so long. Thankfully, we have a scheduled appointment with Pulmonology (our third appointment of the week) today (Friday), so we can get a better handle on what the deal is... I hope.

We almost decided not to go to the pumpkin patch on Tuesday - - we were going to wait until Thursday. I'm so very glad that we seized the moment and enjoyed that very small space between.

I took a ridiculous amount of pictures... and so many turned out cute that I had a hard time choosing just a few for the blog. So... enjoy the slideshow!

I have to mention a few highlights that aren't captured in photos (so I don't forget!)...

- Gracie, upon seeing the sea of pumpkins as we entered the gate, signed, independently... "Jack-o-lantern." And, while they were still very much "pumpkins," I was floored and so very proud. We had told her we were going to the pumpkin patch, we had never once signed "Jack-o-lantern."

- Preston, was terrified of the man who ran the little train ride... he had a long, Santa-like beard, with a spider dangling from it. Preston could barely look at him to say thank you for the ride. Let's just hope, for the sake of the Christmas tree patrons, he removes is eight-legged friend come November.

- Gracie was signing up a storm in the petting zoo, mostly prompted, but after we'd been in there a bit, several were unprompted and correct!

- Preston loved the House of Mirrors. LOVED. Gracie and I stayed outside and I heard this terrifying "thud," which I thought for sure was going to be followed by crying and a bloody nose, but instead, the most infectious laughter erupted. John and Preston rounded the corner inside and I could see them... both of my boys, giggling like crazy. Then, bam! It happened again. More giggling! When they finally made it out, we all cheered for joy (for the fun and no broken noses!) and Preston wanted to go right back in!

The Vest

Gracie came home from CHOC late Thursday afternoon. Friday, the 22nd, was a buzz of supply deliveries, new equipment set ups and general household chores which seem to multiply like fertile little rabbits while she's inpatient. Amongst the new equipment/supplies in our small, efficient home hospital, is "The Vest" - - a fabulous (if your child likes it - - which both of mine do... more on that below) airway clearance machine that literally vibrates the mucous right out of the lungs (or at least loosens it enough to be coughed or suctioned out).

Used every four hours while she was in the hospital, The Vest was very effective. The home treatment regimen is essentially the same as at the hospital, and thus, has me pretty overwhelmed. Gracie has so many treatments per day that it's taken me a few days to get a handle on a schedule that I could manage and make sure she tolerated. Her current regimen entails using the vest while taking a nebulizer treatment of Xopenex & Mucomyst. She stays on the vest for another neb treatment of Dnase, comes off the vest and gets a treatment of Pulmicort and then another of Tobi - - TWICE a day. In between these "marathon" treatments, she gets additional vest treatments with Xopenex every four hours. All of this, coupled with feedings, her regular meds and her added two-week regimen of both Cipro and Zyvox (both of which yield lovely diapers at least every hour) and you have a pretty busy kiddo (and mama) who hasn't even left the house! Add in three doctor appointments this week, a lab draw this week and three appointments next week and, well, you get the idea! It's worth it, though.

The Vest, it turns out, could be the big hero for Gracie, given all the bugs she tends to regularly host, plus the ones she is so easily picking up. Because her sinuses are chronic and she doesn't swallow, the bacteria that she harbors in those wide open sinuses easily travels to her lungs when she micro-aspirates. The hope is that the use of the vest will keep things broken up and moving out. Thankfully, she really, really loves it... all that input is like a dream for her! She happily signs for it (almost on schedule) by patting her chest (which is her sign for the vest) and then signs "trach medicine." It's quite possible this might trump her love for baths!

Preston, who did not visit her in the hospital this time, was fascinated by the new "shaky shake" machine that was delivered and wanted to know what it was all about. I explained to him that it is definitely not a toy, but also wanted him to get an idea what it was like for Gracie. Little did I know that videoing his three minute experiment would provide me with hours of enjoyment. John and I can't help but giggle when we watch him in this. I didn't include the whole thing... I think it's mostly funny to us because we're his parents! In any case, hope this little clip brings you a chuckle, too!

PICU again... Pneumonia again

Unfortunately, Gracie is back in the PICU, having been admitted late Sunday night/early Monday morning. She got sick on Thursday night with what I assumed to be nasopharyngitis and, despite trying to ride it out at home in our "makeshift hospital," she continued to get worse. I took her to the ER on Sunday night figuring that she had developed pneumonia... which she had.

She had a rough first day in the hospital, as she was very agitated and working so hard to breathe. She was inconsolable, combative and fixated on getting out of the bed and room. Thankfully, the "alien" who overtook my daughter for about ten hours on Monday has not resurfaced. She is now far calmer and more comfortable, though certainly still breathing fast. Her chest Xray looks the same today as it did Sunday in the ER, but her CRP has dropped from 45 to 35 (normal is 1.0 or less) and that's good news, as the drop is a sign that she is responding well to the antibiotic cocktail that Infectious Diseases has ordered.

The space between

Taken September 30th... a good day.

If there is one thing that is certain when it comes to Gracie's health, it's that things can turn on a dime. We've learned that we can't count on this hour being the same as the next, we can't make plans - - even carefully laid ones - - without them often being shifted to "Plan B" or even "Plan C." We cherish the quickly moving moments, hours and days that she feels well and we nurse her through the moments, hours and days when she doesn't - - those days that our hearts are heavy and time seems to stand so very still.

The pattern is nothing new, so you'd think I'd be fully accustomed to it. But, during the trauma of the summer and 42 more days in the hospital, I guess I somehow allowed myself to hope the pattern would change - - that maybe, after all she'd been through, the reward would be more stability for my chronically ill daughter. That is not the case.

Since her discharge in August, she's had three ear/sinus infections and two cycles of abdominal migraines. The most recent abdominal migraine started on Sunday and lasted through Thursday; the sinus symptoms seemed to have started Saturday... but it's really so hard, with these repeated problems, to tell what is actually coming first. Are the sinus symptoms a result of the migraine brewing or is the migraine a result of the sinus/ear pain? Or, are the sinus symptoms just environmental allergies flaring up (because everyone else in the family was irritated by their allergies)? I am not certain we will ever know. And this time, we had another factor involved because of an attempt to change formula.

At her GI visit two weeks ago, the doc was happy with her tolerance, but not happy that she is losing weight... we are back to that pattern, too. Only this time, she is not losing nearly the calories as she was before. Her bad days now yield more calories than her worst days before surgery. Because of the weight loss, the doctor upped her calorie requirement, figuring that being sick so much is messing with her metabolism. He also wanted me to change her formula. I was not happy with that idea and I expressed my concerns. He heard me, but still wanted to try - - his hope was that all previous issues were solely related to the complications of her her stomach and liver being so tightly fused, the hernia, etc. He wanted me to bump her calories and once that was successful, try a whole day on the new formula.

She easily handled the addition of the calories and once she was successfully there for a week, I added in the other formula. I only did half and half... for one feeding... so she only had a total of three ounces of the new formula. One ounce into the second feeding, she was white as a ghost, clammy, had beads of sweat under her eyes, was gagging and writhing around in agony. I cut the feeding pump off and she proceeded to have crazy, major diarrhea about twelve (not exaggerating) times in a four hour period... straight-out-the-diaper-no-matter-how-tight-it-was kind of diarrhea. Poor sweet girl... and poor mama for cleaning it up and dealing with the guilt of trying the formula when I knew the doctor's logic was not at all logical.

We are still working our way back to full feedings, but she is perky again and I'm grateful. She went to school for a few hours on Thursday and Friday. Hopefully she can hold onto this space for a while - - feel well and do the things she loves... go to school and therapy, see her friends, play outside. I'm amazed, as always, by her resolve. She passes through those painful, dark places all too frequently and comes out the other side with her spirit seemingly unscathed. I, on the other hand, am not unscathed and try to recover from the heartbreak and exhaustion of those lows. As I shared with a dear friend the other day, I tell myself to exhale, for lately I seem to be spending a lot of time holding my breath, knowing that the next downturn is looming around the corner. Instead of focusing my energy and worry there, I try to capitalize on the days Gracie feels well and fully live in the space between.

More from the impromptu cell-phone photo shoot in front of the house... so cute and sassy in her skirt from the Clayton family!

I ♥ this picture... such a real moment of conversation between my sweet peas!

'S' is for... Sick Day

Gracie's sick. Not the scary, hospital kind of sick, "just" sick... again.
And she missed school today because of it. Bummer.

I've suspected something big was brewing the whole week as she's been needing tons of sensory input, won't keep her clothes or shoes on even in the car and has had that "look" in her eyes. Sinusitis, Tracheitis and still an ear infection that had been percolating the week she got home. Ugh. The tracheitis is frustrating because she is currently mid-cycle on her bi-monthly TOBI treatments (an inhaled antibiotic to help with pseudomonas, tracheitis and overall lung function). So, she's been prescribed an additional antibiotic again.
She's uncomfortable, clingy, her nose is running, her eyes are running, her oral and trach secretions are non-stop. We can literally "tip her over and pour her out." I quote the lyrics from "I'm a Little Teapot" because that's actually what John does sometimes when she's like this - - he helps her drain. We sing it to her (she can't hear us, but Preston can and he'll do the little dance for her). It sounds a little barbaric, but it gives her a tremendous amount of relief much faster than constant suctioning. Gross, I know.

She had a bevy of appointments this week.

Surgical follow-up: He is very pleased (as are we) with how she's doing from that standpoint. Her weight-gain is good (31.6 lbs and not much is fluid retention) and she's tolerating her current feeding regimen well - - something we didn't dare to dream possible. (With this current illness, I've had to slow her feedings down considerably because she is coughing so much which makes her prone to gagging and is hard on the fundo.)

Cardiology: She is "stable." He is pleased that, with all her body endured since May, her heart managed to come out the other end without worsening. The echo looks "good for Gracie... which means it's unchanged."

Infectious Disease: She was not pleased about my suspicion that Gracie was getting sick, but feels that Gracie's issues are likely not stemming from an immune disorder. She has ordered a full immunological work-up to be certain and we will complete those labs once she's well and we have endocrine labs to combine into one draw.

Pulmonology: He prescribed the antibiotic. We discussed the different ways we can treat this recurring issue, which are limited due to her resistance to so many meds. He reiterated some things from the hospital... He is pleasantly surprised with how her lungs look on the Chest CT - - he had expected them to look much worse. He paid us a high compliment again, saying that her status is clearly the result of diligent, constant care. He also stated that, because of her swallowing issues, this problem is not ever going to be solved, rather "managed." She will always be a junky trach and she will always (and probably more frequently now) be fighting bugs as her lungs continue to deteriorate over time.

On the horizon: ENT, GI, Neurology, Ortho, Endocrinology, Audiology.
Good grief.

Post-Op Update

First, thank you so much to everyone for all the love, support and prayers. We truly appreciate, as always, all the blog comments, text messages, voicemails and FB messages... they help give us strength.

Gracie's surgery went on as scheduled Wednesday afternoon. The surgery itself was much more difficult and took longer than anticipated. Though it came as no surprise to us, they found getting a new central line placed to be quite challenging. It took three attempts to get it done. Next came the actual surgery itself, which was, to quote her surgeon directly, "... a very, very difficult case." Dr. Lam and his team worked tirelessly to complete the surgery laparoscopically, and for that we are incredibly grateful. It means a much easier recovery for Gracie and far less risk of infection.

As it turned out, Gracie's liver and stomach were completely fused together by adhesions, so before he could actually begin repairing the hernia and re-doing her fundoplication, he had to separate the liver and stomach. Once he did that, he "took down" the hernia and, interestingly enough, found her fundo hadn't slipped, rather was fully intact and shoved up into her chest. He reinforced the fundo, all the while, the surgical assistant holding her liver up with the probes in order to keep his line of vision clear for working. He and his team were exhausted... and he wasn't ashamed to admit it. She was finally rolled out of the OR around 6pm... about three and a half hours after they started.

Our only objective has been to keep Gracie's pain managed and, of course, carefully monitor her vital signs. Her little body has been put through the ringer from this major surgery - not to mention the last two months - and we just want her to rest and heal. Unfortunately, she had tremendous breakthrough pain and, for the first day, needed hourly morphine to stay comfortable. She was also on Ativan, Tylenol and Benadryl (to control the itching from the morphine). It was absolutely heartbreaking to watch her writhe in such agonizing pain. She required a blood transfusion on Thursday. Her chest X-ray mid-day Thursday looked "fuzzy" and today's looked even "fuzzier." She retained a lot of fluid and required an extra dose of IV lasix to try and get rid of it (aside from the twice daily IV lasix she is already on). She required IV albumin because that is low (which has been her trend her whole life, regardless of her nutritional status) as is her prealbumin. She has been running a constant fever of 102 without Tylenol on board. Her CRP and white count are high... the CRP is expected to be high following surgery, however, hers was exceptionally higher today. All of this is pointing toward another respiratory infection. What's so unsettling is that she was so well covered with two broad-spectrum antibiotics for the last ten days. Based on preliminary culture results, they added a new antibiotic today and will make adjustments once the culture has had another 48 hours to grow.

With all that she has going on it becomes more difficult to assess her pain vs. discomfort from what might be brewing. Now that she is 48 hours post-op, we are trying to stagger some of her meds to keep her relaxed and pain free, yet at the same time, not wanting to snow her to the point that we can't really tell how she is. It's a fine balance and exhausting, especially today when she had a day nurse who insisted on questioning both John and me on every thing we asked for and how we're doing things. She was nice enough, but not a great team player and I have already asked the charge nurse that today's nurse not be assigned to Gracie again. (We have only done that two other times in Gracie's entire hospitalization history.) Poor rapport with the nurse just made an already difficult day more challenging. Thankfully, tonight she has Katie, a great nurse, who has taken care of her several times before and whom I was very relieved to see at 7:30 this evening.

Tomorrow they will start clear fluids on a drip through her suspended G-tube at a rate of 10cc an hour. I am so anxious about this as she has been gagging, retching and super junky with her trach secretions all day. I am trying not to pass any judgment about the success of the surgery - - or the new trach size - - until more time has passed and she has a chance to heal, but it's already concerning that her oral and trach secretion production is relatively the same when the docs expected that the combination of these two things should result in a vast improvement. I will be patient, though and let time tell. Please wish our Little Miss luck as she resumes fluids in her tummy for the first time since total gut rest.

Hard Core Advocating - Surgery Wednesday 8/11

I will preface this post by stating something that's already well known... Gracie is a trooper. She continues to blow through IVs and is currently on number eight. She did "well" today (always a qualified statement), all things considered.

That being said, it was a very difficult day that involved numerous phone calls to doctors, case managers, the surgeon's office and the surgeon himself. There were a lot of tears... tears of anger, sadness, frustration, irritation and more anger... you name a reason to cry and I cried for it today. (It is quite possible that I had a lot of tears that needed crying, as I've not had a good, solid uninhibited sob in a while.) At the end of the day, I cried tears of relief, as, thankfully, we got surgery scheduled for tomorrow instead of next week or later. It's just so unfortunate that it took hard core advocating to get there.

I'll back up a bit. When Gracie was discharged on July 22, we (i.e. John, the GI team, the attending physician and I) had not wanted Gracie discharged. We all felt as though she would end up back in the hospital before we could get surgery accomplished - - we just wanted to move forward with surgery... the sooner, the better. She had been cleared by everyone, but the surgeon did not want to do it that admission. We were told he was going on vacation... he was not... and it doesn't, at this point, matter why or how that piece of info was incorrectly communicated. Bottom line is we took her home, did some more surgery related soul searching, got it scheduled, she got sick again (everyone feels that would have happened in that amount of time regardless of where she was) and she missed her scheduled surgery date last Friday.

Because of the infection, surgery was cancelled. But, once her cultures were negative, we were told that the surgeon's office was working on getting Gracie on the schedule with the assumption that she'd be fully cleared. Since Friday, everyone (and I mean everyone, BUT the surgeon, whom we haven't yet seen this admission) had been saying that it was looking like Wednesday, yet at the same time, she never was officially on the schedule.

Cut to today... I called John at the hospital to find out if he'd heard anything. Not a word. It was agreed that he'd ask every single person who walked into the room until he got an answer. In the meantime, I called the surgeon's office directly. They informed me that Gracie wasn't on the schedule and I asked that they have the scheduler or surgeon call me directly. I needed an answer, not the runaround. Back at the hospital, John was informed that the scheduler was out sick. Minutes later the surgeon's office called me back and said that the schedule was really full this week and he (the surgeon) was trying to get her on for next week. She ended her little speech with, "Okay?" To which I said, "No... have the surgeon call me directly, please. We are not 'okay' with her sitting in the hospital waiting for surgery for at least another week."

In the meantime, in an effort to convince the surgeon that this needed to get accomplished sooner than later, I continued my telephone tirade by leaving messages for the GI doc, the Pulmonologist and the Case Manager on the PICU. I word-vomited on all their voicemails about the situation. I knew that they were on our side, but whether or not they'd have clout with the surgeon at this point was questionable. I asked the case manager to set-up a care conference. The pulmonologist ended up rounding on Gracie to check on her new trach (more on that below) and John told her what was going on. She said she'd go to the OR herself and talk to the surgeon. We don't know that she actually did. No sooner had she left Gracie's bedside did the surgeon call me to say that she was NOT having surgery this week. He went on to say that the schedule was full and that Gracie's case was not emergent enough to bump anyone else. He also said that there was no way that any of his Nurse Practitioners would have said that Wednesday was a possibility. He said that information never came from his office and that he was sorry we had been so terribly misinformed. At that point, my tears were free flowing and there was no way I could keep myself from sounding hysterical. I pleaded with him about the fact that she continues to blow through IVs, that we can't feed her through her G-tube or she'll start having another set of issues prior to surgery... not to mention the increased risk of infection just for sitting in the hospital... what did he expect us to do? She can't leave her room because she is in isolation, she is depressed and begging to go home... what are we supposed to do for her? He said to put in another central line to avoid the IV issue... I reminded him that Interventional Radiology would require another anesthesia - - wouldn't it be better to just put her under once? He literally raised his voice in frustration with the fact that we'd been given any sort of hope that this week was possible. He said that by calling me himself about this he was already going above and beyond what he does for most parents. I told him it doesn't matter what he does for others, I needed him to hear me about my child. He said he would try to add her to Monday's schedule, but that next week was already full as well.

By the time we got off the phone, I was sobbing, Preston (yes... all of this in front of Preston because no matter what I did, he followed me all through the house) was crying, too, and I had to explain to him that mommy was so very mad that she was crying and she was really sorry if he was scared. Later he told me, "I was so mad that you were so mad that I cry, too, Mama." So sweet, but I feel so guilty. I just couldn't hold it together, though, no matter how hard I tried. After I got off the phone with the surgeon, more hysterical calls to the John, my mom, the GI office and the case manager. About an hour later, the surgeon called me back, claimed to have moved mountains and said that he got an OR time for tomorrow (Wednesday) at 2:15pm. He said that he would be doing a lot of extra driving from one hospital to another to get it done... for which I am grateful, but not at all sorry. I have no idea what or who actually triggered him to get Gracie on the schedule ASAP, nor do I care. The point is, it's done and she doesn't have to sit and wait another week or more. I know he is a good man... I know he is an excellent surgeon. I'd like to think that once he had five minutes to really think about the situation, he just did the right thing.

I hate that I had to be a stalker today. I hate that I was hysterical, especially when trying to talk to doctors. I hate that it all came down to this, because up until this whole fiasco, this stay had been truly wonderful as far as care is concerned. In the end, the surgeon is doing the right thing - - the thing that considers the whole patient and the whole situation, not just the surgery itself. It's just so infuriating that it had to get to the point that it did. The wonderful case manager, Jo, reminded me that I was doing my job as Gracie's mama... that it wasn't as though I woke up today with the agenda to get under the surgeon's skin. She's right... my focus is my daughter and I only did what was in her best interest. Like me, she is sorry that it had to come down to some serious "pit-bulling," and yet I shouldn't apologize for it at all. I appreciate that reminder so much.

Please keep our sweet girl in your thoughts and/or prayers Wednesday as this surgery, while "routine," has no guarantee of being the end-all solution for Gracie. Furthermore, it is a 'gut revision' and, as such, does not have an easy recovery at all.

On another note, the resident and attending in the PICU did an excellent job getting all tests and procedures done in order to clear Gracie for that Wednesday target date. On Sunday she had her echocardiogram to check for any issues following the yeast infection in her blood. On Monday she had an abdominal ultrasound, a bronchoscopy, a CT of her lungs and an Opthalamolgic Exam which included dilation of her eyes. It was, needless to say, a very busy day. Her echo, ultrasound and CT came back negative as did the eye exam.

Unfortunately, the bronch showed that her tracheolmalacia has worsened and as a result, the pulmonologist changed her trach to a larger, longer temporary one and has ordered her a custom trach which we should receive next Monday. Essentially, Gracie's airway is so floppy, it was easily collapsing just below her old trach. In theory, this contributed to her inability to manage her secretions, her violent coughing jags, and her gagging. The longer trach will (or should) help keep everything open. So far, though, it hasn't made a difference... she is gagging more, actually, as she tries to adjust to the way it feels. Her secretions haven't yet lessened either. It's a "wait and see" game for now... they may have to adjust the trach again, but until she gets through surgery, there isn't any way of really knowing whether it has helped or if it needs some tweaking.

So many things going on... steps forward almost guarantee a step or two backward. Hopefully, over the next week, we gain more steps than we lose.

In the PICU... AGAIN... line sepsis

Gracie was home exactly eleven days this time. By Monday evening, she had a fever that, despite Tylenol, continued to move in the wrong direction. A spike to 104.6 at 8:00pm earned her a trip to the ER. Within three hours of our arrival, she was spiking to 105.3 and was admitted back to CHOC. At 4am, she was 106 and completely out of her mind. It was terrifying. That fever, coupled with low blood pressure and increased work of breathing earned her a bed in the PICU.

Last night, we learned that her PICC line was positive for yeast. Here's the medical lesson of the day in layman's terms (for those of you who don't already know): We all have yeast active in our respiratory system. However, we also all have bacteria in our systems that keep that yeast from being problematic. When you are on antibiotics, the good bacteria gets killed off, thus leaving you susceptible to a yeast infection. Gracie ended up with yeast in her line... which was not surprising to the Infectious Disease doctor given her history and her baseline respiratory labs. But, since she's been on BIG GUN antibiotics for weeks and weeks - - actually months and months - - all of her good bacteria are gone and her system had no way at all to fight that yeast. She didn't stand a chance with this infection. (The fact that Gracie has all kinds of respiratory flora when she is not acutely sick was always a concern for us with respect to having a central line placed. She is trached - - her secretions are tenacious and actively coughed out - - she is just at a higher risk for infection.)

As a result of the yeast infection, her PICC line was pulled today. Additionally, her blood pressure never responded to the fluids given on Monday night, so on Tuesday morning, they started her on Dopamine to maintain her blood pressure. She continues to fight fevers, her respirations are high most of the time, she has an active (albeit small) bleed in her stomach that is starting to affect her hemoglobin levels. She looks like a pin cushion due to all the peripheral draws that have been required to get labs. She now has an IV in each foot (so she can have her hands free) and she has more labs to come in the next several days in order to know how the infection is responding to the anti-fungal medication. Once those numbers look good, she will have a full eye exam, liver work-up and echo to confirm that the yeast didn't cause any damage or adhere to her pacemaker. Still so much to go through... and once she gets over this hurdle, there is another set waiting for her...

Gracie is very sick... very. Despite our experience with CHOC during her last admission (they've been wonderful so far this time), we had, after thoughtful consideration, scheduled surgery for this Friday. That has been cancelled, obviously. She hasn't been well since the day that PICC went in. And in looking at the bigger picture, she hasn't been well without antibiotics for more than fourteen days since January. Getting the PICC to give TPN was a huge step to "save" her... to keep her from starving. Even with the TPN and full gut rest, she is miserable from a GI standpoint. Receiving full nutrition and proper hydration made her gain weight, yes, but her body isn't handling that nutrition and hydration well at all. Her trach and lung health are constantly compromised. She gags and retches just trying to manage her saliva and we all know how uncomfortable gagging and retching is... imagine doing it all the time? With all of this, we are left wondering what will happen now that the line is out, wondering whether the surgery will really make that big of a difference since it won't solve so many of her other GI issues, wondering when enough is enough for our sweet girl.

She spent the better part of her first five years miserable. Yes, she has "great" days, but they are "great" days for her... and that means at some point, her day has hellish moments... even hours. It means that every day there is pain. She has now spent the last six months sick, fighting illnesses and constantly losing. We are walking the tightrope between the belief that she can be cured and the harsh reality that nothing is really helping. John and I have been asking ourselves tough questions with regard to Gracie's health and quality of life for years. Because of the last few months, we've had to try and answer those unfathomable questions... and the bottom line is that they don't yield any good, clear-cut or pain-free answers.