More questions than answers

Search your memory for the phrase "This has been the longest week of my life."  I could say that for just about every week since October.  That last one, two, three weeks of pregnancy, the first week in the hospital, the second week in the hospital, the week after that waiting for the swallow study to see if the procedures worked, etc.  I had this rosey notion in my mind that she would be all better (again) and that we could finally try to get to normal.  But, alas, this was not to be.  At least not yet.

Swallow study

Hannah really wants to swallow properly and tries really hard. She sucks on a pacifier and, given the chance, on a bottle with great determination and seems genuinely hungry. In my opinion, it appears that behaviorally she knows what to do, and that the problem is purely anatomical.  For the swallow study I was hoping and expecting to see her swallow all the way down and for the narrowing in her esophagus to be all gone. But no, the x-ray looks about the same as the first one 3 weeks ago. Deanna, the OT, was going to watch her swallow with several different nectar-thicknesses, hoping to develop a game plan about how to feed her orally, whether she needed a thickener or not. But she didn't even proceed with the varied thicknesses during the study. We saw the liquid pooling above the narrowing and then slowly coating the esophagus below, without enough volume present for proper peristalsis (peristalsis is the muscular, reflexive wave that pushes food down your esophagus after you swallow). Even watching in the next room I could see that it wasn't what we were hoping for.



Still shot of the swallow study.
The darker gray is the path of her esophagus,
and you can still see the muscle in the way.

Deanna's opinion about feeding was that Hannah should never have been sent home on a continuous feed in the first place, and that she hardly ever sends NICU babies home with a continuous feed. She was of the opinion that bolus feeding should have been adjusted by 15 or 30 minute increments, even if its 2 hours rather than 1 hr 45 min for a specific volume. In the hospital, our nurses had started Hannah on a 30 minute bolus feed, but when she started regurgitating from her mouth and nose, we went directly to continuous rather than trying to condense that same volume over a longer period of time first.

On Wednesday we followed up with Dr. Schramm.  He pointed out that Hannah wasn't aspirating to her lungs, which is something I hadn't thought to look for in the study. This is definitely good news. But he also shared my disappointment that the botox hadn't been effective, even given 3 full weeks. Dr. Schramm offered to try the dilation procedure again, but I was hesitant to put any hope in that since it hadn't worked for more than about a day the first time. Other than that, he said there wasn't much more he could do for us, and didn't want to do the myotomy yet given that Hannah was still so little.

I had so many questions: If we did the dilation again, how likely is it to be effective this time if it wasn't the first time? How many dilations is ideal? What is the ideal age and size for her to have the myotomy surgery? How often is the myotomy definitive and successful? What is recovery like? Is the lower sphincter also causing problems?  Feeding? Aspirating? Prognosis?.  The answers are just not there. There isn't enough evidence from other cases, because its just that rare of a condition. The data that Dr. Schramm had included 5 participants. I decided to go see a specialist at Primary Children's Hospital in Salt Lake City, and fortunately they could see us the very next day.

Thursday morning we saw Dr. Henderson for a check-up. I asked him about nutrition, how to get Hannah the needed calories for growth, and what is the best way to get her accustomed to bolus feeding, rather than continuous feeding. To me, moving to bolus feeding is progress, and I REALLY was feeling desperate to see more progress and faster growth. He was of the mind to keep her continuous feeding as is and to not rock the boat. She is growing, even though it is only an ounce a day. He extended the home healthcare for NG tube feeds by 1 month.

Then we went straight to SLC for a meeting with Dr. Smith at Primary's.  He didn't have any more answers than our first surgeon had, and I left that appointment feeling equally discouraged.  He did a soft scope of her throat to look at the top of the esophagus.  Dr. Smith was much more conservative about treatment than I would have hoped, and he's only seen 5 cases like Hannah's in his 25 years of practicing medicine. He was inclined to consult with a nutrition specialist, a GI specialist, a Speech Language Pathologist, and to come back in a month. He said he would want her to be at least 11 pounds before doing surgery. He also proposed doing the botox and dilation again, and said he didn't have enough information to know if the myotomy would be curative, and whether she could out grow her condition.  He was interested in transitioning Hannah to bolus feeding so that her stomach can stretch and she can learn normal hunger cues. This was what I wanted, but now I was confused to have 2 different opinions about feeding in the same day.




When you keep pulling out your NG tube, you get your sleeves sown shut!

At first I saw Dr. Smith's analysis as passive and slow, and felt like he wasn't an expert since he had only seen 5 cases ever. But I was corrected by others who pointed out that his attitude is very team-oriented, and shows that he is not so cocky as to think he should do the procedure immediately without gathering more information and consulting with other specialists. He IS an expert in his field. He does pediatric ENT surgeries every day, and it was unfair of me to say he isn't an expert based on not seeing this super-rare condition more. Being conservative in his treatment plan shows that he has my daughter's best interests in mind. After all, she is only 4 weeks old, has already been anesthetized twice, and isn't even back to her birth weight.  (It was also unfair of me to believe that going to see a specialist in another city would have a better outcome.)

So on one hand, I want to do what's best for Hannah when the timing is right, but on the other hand the surgery she needs seems to be relatively curative and I feel like we're just prolonging the inevitable by trying other less effective means repeatedly (if we were to do the botox and dilation again).

At first I was disappointed by this week, having a swallow study, 2 weight checks, 2 specialists, 3 different medical opinions about her feeding, and coming out with just as many questions as before and no answers. But I have to take a step back and look for the progress that is there and recognize the medical professionals doing their jobs and working together. I'm learning to take deep breaths and realize that the myotomy is best for Hannah only when she is big enough and strong enough and when the timing is most ideal, whenever that may be. For now, I can acknowledge that she is healthy and slowly gaining weight finally, and that is positive progress.  She has so many people in her corner.

 

Overheard from the next room Audrey saying to the baby, "When I'm at school, when I'm at home, when I'm having screen time, when I'm cleaning up, when I'm sick, when I'm asleep, I'll always be alive and I'll always love you."