Foto Friday 2, vol. 15 - Nothing ever goes according to plan...

As many of our family and friends know from Facebook, the last three weeks haven't gone as planned. Gracie was inpatient June 15 - June 19 and then again from June 22 - July 2. We have been home one week today!

Gracie was admitted June 15th to get her PICC line placed. Unfortunately, the PICC team was not able to get the line placed themselves and sge had to go into Interventional Radiology late the following afternoon and have it placed under general anesthesia. Both the Radiologist and the Anesthesiologist said that he barely got the line in... her veins are "difficult" and he was afraid the entire time he wasn't going to get it in. They both separately said they hoped she'd never need another one. Needless to say, that freaked us out.

Gracie did great during her stay, but due to very poor planning by a case manager (who also had horrible bedside manner), Gracie's discharge was delayed a day because things couldn't be coordinated with home care and supplies for her PICC. We got home on Saturday, June 19th. That extra day (I am sure of it) resulted in both of us blossoming with colds on Monday night and by Tuesday the 22nd, Gracie had a low grade fever which later blossomed into a scary high fever - 104.6 temporal with Tylenol and Ibuprofen - and yielded a visit to the ER Tuesday evening. (Because she has a PICC, we had to be certain that the fever wasn't due to a blood infection.)

I was certain she didn't have an issue with her PICC and knew that it was respiratory in nature - - which wasn't rocket science considering the awful "ick" she had in her trach. First diagnosis: Acute Tracheitis. They immediately began treating her with IV antibiotics and ran every lab you can imagine. Her blood pressure was exceedingly low so they were bolusing fluids into her like crazy. We were finally admitted to CHOC from the ER at 1:30 in the morning. At 5:30 in the morning, they still couldn't get her blood pressure up, so we were transferred to the PICU. Her blood pressure recovered fairly quickly after that, but I was panicked by the amount of fluids they'd given her on top of her TPN and how that might affect her heart. Her respiratory status was poor, but well managed with regular breathing treatments,so it didn't appear that she was harboring too much fluid in and around her lungs.

But, that wasn't all that Miss Gracie had up her sleeves. She decided to let the doctors see many of the things that I've been describing to them for months now. It was hard to tell if she was in the midst of an abdominal migraine cycle or not, considering how miserable she was, but they were certainly paying more attention to her symptoms. On Wednesday morning, she had a lot of old blood in her G-tube. By Thursday, it was frank blood... and a lot of it. On Friday, the 25th, they did an EGD with biopsy. (Esophagogastroduodenoscopy - an examination of the lining of the esophagus, stomach, and upper duodenum with a small camera - flexible endoscope - which is inserted down the throat). While they were amazed (as were her past doctors) at how friable her stomach is - - the scope gently touching the wall of her stomach causes a bleed, as does the tiniest bit of air blown on the lining, this is not new information to us. And like Gracie's previous doctors, they have no explanation or solution for this bleeding issue and moved her out of ICU with the idea to conference about all of her other issues and formulate a plan.

In the meantime, she began having tremendous amounts of frank blood from her trach, caused by the incessant, hard coughing and dry hospital air. Even the cool mist wasn't keeping it at bay. One of the labs came back with parainfluenza, but they didn't do anything new to treat that... it just had to run its course. Gracie had a second bleed 24 hours after the EGD and because of that, we were monitoring the blood in her stools, too. Still no explanations for the bleeds, but they finally understand that the amount of bleeding is more serious than they'd thought even with my previous (and frequent) explanations. Because of the bleeding, her blood levels were low and they were watching her hemoglobin closely. They were considering a transfusion last Monday, but decided she was asymptomatic and held off. Not sure that was wise... she had been lethargic and sleeping 16-18 hours a day since she came home but finally recovered from the lethargy Wednesday. She is still sleeping more than usual (during the day, unfortunately), but that is likely her body adjusting to the sudden weight gain and proper nutrition.

In the first few days, she dropped all the weight she had gained with the PICC... however, she now has recovered that weight and MORE! Woo Hoo! At the end of May, during her horrible abdominal migraine, she dropped to 23 lbs. She was 24.64 lbs on June 15th. As of yesterday, she was 29.04 lbs - - awesome! This is the heaviest she's been since August 2008 and only 1.5 lbs away from her heaviest weight ever... 30.5 lbs at age four. Her nails and hair are growing like gang-busters! The doctor will likely be reducing her TPN volume next week depending on how much more she gains. This is all great news...

However, we haven't resolved the neurological/nervous system issues... and probably won't. Most likely, we will only be able to manage with meds as best as possible. We also haven't determined the source for her literal passing out following feedings. It doesn't happen with every feeding, but it happens at least once a day and usually three times out of the five daytime feedings, regardless of being on the pump or a bolus. It is not a glucose issue (at least that we've caught)... which we monitored in the past for months and monitored during the recent hospital stay. She doesn't appear to have a dumping problem, at least that can be determined with the testing that has been done recently and in the past. (She had a TORTUROUS gastric emptying study during her stay. She has had several in her life, some incomplete because she vomited, but none as horrendous as this one. She was understandably furious about having her hands held down and being bound to a table for 90 minutes. She managed to work her way out of the contraption SIX times during the study... proving that size and health status have nothing to do with strength or will. My sweet girl was so happy to have her hands free at the conclusion of the study that she literally waved to every single person we passed on the way back to her room!)

The majority of our discussions with doctors surrounded Gracie's Nissen Fundoplication and her Paraesophageal Hernia. In the past, her doctors have felt that the hernia was "surgically unremarkable" and too small to be the source of much discomfort. Here, the docs feel that, although it is small, Gracie is symptomatic - - that is, she shows all the signs of being bothered by the defect, regardless of its size. Their hope is that by repairing that hernia and redoing the fundoplication, she will not have nearly as many secretions (as they will more easily be able to flow into her stomach) and that, perhaps, she will be more comfortable with feedings. There is also thought that her pacemaker leads could be making the problem worse when she's fed. In the past she has had diaphramatic pacing which worsened with feedings, so there is a chance that these issues are all tied together given that everything is sitting in the same region of her anatomy.

We spent a lot of time asking the GI team questions and met with the surgeon who was very kind to spend significant time answering even more questions. While we are now in favor of repairing the hernia, we are concerned about several things... the biggest concern being that the Nissen Fundoplication will slip again due to her neurological issues (the frequent retching) and she will be back in the same boat. Since they want her in optimal nutritional status before surgery, we have a few weeks to research, talk to other families (anyone with input, PLEASE COMMENT!) and conference with the team again. She will have surgery in mid-August, but at this point, we don't know exactly what surgery.

She was discharged with the following plan: gain weight and stay healthy.
The trachietis and respiratory ick is better, but because of the large amount of fluid she is getting, her lungs are not sounding so hot. She is getting round the clock breathing treatments and sounds much more coarse than she used to. (There actually was a benefit to the limited fluid she was getting before the PICC... her lungs and heart were operating more optimally... go figure.)

As we beef up our girl, the doctors are hopeful her improved nutritional status will change her tolerance to the G-tube feedings. John and I have been skeptical of that concept since we started discussing TPN... so far, unfortunately, we are right. She is sound asleep right now, as I fed her about forty-five minutes ago. The good news is, she is sound asleep at home. The other good news is that when she is awake, she is a happy, chubbier by the second, camper!