Throw away your expectations

I've had several people tell me lately that Hannah is strong and that she is a fighter. My response has been, "Well, she has no other choice!"  I am becoming strong and I am certainly fighting for her. Our whole family is fighting for her.

Christmas week she fought coronavirus. New Years' Eve she had influenza A.  We had our 3rd hospitalization Dec 30-Jan 2 at Primary Children's Hospital in SLC. After she had a febrile seizure the first night there (!!!), the doctors wanted to make sure she didn't have a bacterial infection, so they did a lumbar puncture and a 36 hour culture of CSF (cerebrospinal fluid).  Fortunately they didn't find anything, and her urine and blood cultures were also normal. That was a big relief.

New Years Day her fever was gone and her respiration and oxygen levels improved significantly.  She had a consult with a neurologist who didn't find anything alarming either (she already had a normal MRI). We were able to come home on Tuesday, the 2nd, with Hannah on oxygen (.06-just a whiff). If you have been counting, that is her 4th time coming home from the hospital. So now she has a feeding tube with backpack and a nasal cannula with oxygen tank. Certainly more baggage than baby!

Dr. Schramm told me that he "pulled back the reins" on any surgery the moment he found out she had had a seizure. I hadn't realize how truly sick she was, again thinking that the surgical myotomy was the answer to her apneic episodes, bradycardia, and cyanosis, and that was why we were going to Primary Children's. I thought that surgery was on the horizon and getting closer with each hurdle.  But the last two weeks have brought us 2 illnesses and a hospitalization that had little or nothing to do with her esophageal problem. I also learned this week that the surgery might not be done until she is symptom-free for 6 weeks, ensuring no inflammation in her throat. So again, I am learning to adjust my expectations. Better yet, to have no expectations.

I never thought that a Speech Language Pathologist would have much to offer an infant that doesn't speak, but apparently there is quite a lot!  A SLP stopped into our hospital room to spend some time with Hannah and teach me about how to keep her orally stimulated while she isn't able to eat by mouth.  She showed me ways to interest Hannah in sucking on things, putting her hands in her mouth, tasting different textures, even smelling and licking flavored chapstick off her lips. She also told me to do skin-to-skin with her so that she is familiar with that area (Chris says this is good for husbands too). The hope is that she will someday be able to nurse again (and of course have normal eating habits later!), and that in the meantime she can maintain her interest in oral exploration, have positive, reinforcing experiences with her mouth (that doesn't include tests and tubes!), and continue to salivate with stimulation. Its really good that Hannah likes a binky. This is a whole world I never considered! The SLP pointed out to me that Hannah can manage her own saliva, otherwise she would be drooling constantly.  Try going a whole minute without swallowing and you'll see.

Finally starting to fill out!

We saw Dr. Henderson today and Hannah is 8 lbs 14 oz-past her birthweight!  Hooray!  She's finally got some padding now on her arms and legs, and her face is rounding out again like when she was a newborn.  It took 6 weeks to get back to her birthweight, and right now she's gaining about 1 ounce a day. Dr. Henderson can see that I am eager for progress such as faster growth, transitioning to bolus feeds, weaning off oxygen, and ultimately having surgery. But he pointed out that we do have progress: Hannah is alive, she's healthy, she's home, she's growing, she's tolerating the NG tube feeds. Even though its a hard holding pattern to be in and it feels like we'll be here forever, her progress is measurable and important.

I am letting go of my expectation to be off oxygen after a certain amount of time.  She is doing great with the oxygen, and her Owlet doesn't alarm at night. I sleep very well because I don't have to worry about whether she is breathing. What a difference!

I am letting go of my expectation to wean soon from the continuous NG feeding to bolus feeding. Right now she is growing steadily, not coughing or choking on any reflux and therefore aspirating. She is comfortable and actually sleeps through the night. Do you know many 6 week olds who sleep through the night?  Eventually we will transition to a regular feeding schedule and it might be a lot like having a newborn again. I'm preparing myself for that possibility sometime in our future.

I am letting go of my expectation that each swallow study will show improvement. If it does, I will be pleasantly surprised.

I am TRYING REALLY HARD to let go of my expectation that Hannah can have surgery as soon as she reaches 11 lbs. Given her weight gain, that would be the middle of February.

I am letting go of the expectation that having surgery will solve all of her problems. She could have severe reflux that the hypertrophy(narrowing) is actually protecting her from; she could have more motility problems in her esophagus that need correcting; she could (heaven forbid) get sick again.

I am letting go of the expectation that even if the myotomy does work, we will ever have Normal. We still have recovery and swallowing therapy and re-introducing bottle- and breast-feeding.

I am letting go of my expectation that she will breastfeed again. Fed is best of course. 

I am letting go of my expectation to get back to work anytime soon. I put my Etsy store on hold and stopped taking on other sewing work before Thanksgiving, with the hope to return by Christmas. I simply don't have time for that. Hannah and the other kids are a full-time job right now.

I am letting go of expectations. 

My baby is alive and I am happy to prepare her tube feeds every four hours, to pump 5 times a day, to snuggle her and play with her and change her diapers, to plug in her binky every 2 minutes. I'll do it again tomorrow. If I just take it one day at a time, I can do it. If I look ahead and worry that I will be doing this for 6 months I will break down in tears, so I'm not going to do that.  

Today, tomorrow, the day after that: I can do this.

Deep breath. 

Ready....go. 

Fast Track

We do our holidays in the hospital this year, so why should New Years Eve be any different?

Hannah's oxygen monitor kept going off all morning yesterday, even during daylight when she was awake. Her color was just not right. She looked gray and sickly. So I took her to the emergency room around 2:30. While there her oxygen dropped into the 50's and her heart rate dropped into the 80's.

The pediatric hospitalist from upstairs (who of course remembers us) consulted, and got in touch with our surgeons, Dr Schramm at UVH and Dr. Smith at Primary Children's.



Car Seat + Stretcher for ambulance ride

Aside from going home and continuing with the status quo (which was no longer ok with me!), we could 1. Be admitted at UVH and Dr. Schramm would try the dilation again, or 2. Be transferred to Primary Children's Hospital in Salt Lake City where we can consult with more specialists.  At Primary's Hannah could be seen by a neurologist and a GI specialist. Dr. Smith said that if any other problems could be ruled out he would consider doing the myotomy early, before getting to 11 pounds as he would have preferred.

Dr. Schramm said if we chose to stay at UVH, he would consider doing the myotomy but it was up to me and my comfort level. A week and a half ago I would have jumped at that offer and signed on right away, but I'm learning to wait for reason, assess the options more calculatedly, and have patience in the timing that is best for Hannah. I am glad I have learned what I have in the past weeks, because I now realize the benefit of meeting with these specialists before having the surgery.  If I had my way, she would have had the myotomy a week ago, and what if there were more problems that arose afterward? I would not have been prepared for that.

When we came home from the hospital after the first dilation procedure, I though Hannah was all better, and she wasn't.  When we came home on the feeding tube, I thought we would have that for 2 weeks, have a perfect swallow study a week later, and be off the feeding tube and finally have Normal, and I was also wrong. So I'm learning not to get my hopes up or try to rush the process.  If I keep expecting that each intervention is the final step, I will keep being disappointed. I am now accepting that this process may be much longer than we would have wanted, and to brace myself for the long haul.  And I've learned to be okay with that.

Hannah's esophageal condition is so rare that there just isn't enough evidence to tell us what the right course of action is and when.  When I ask the doctor if the myotomy is the definitive solution, he says "We don't know."  When I ask how many dilations is ideal, he says "We don't know." When I ask what age is the ideal age for the surgery, he says "We don't know."  And its not because he isn't an exceptional surgeon, its because there just isn't enough information, research, and evidence. And its also a relief to realize that this particular surgeon is not too cocky to admit that. Each medical journal article talking about this condition has at most 5 cases to talk about.

So we are at Primary Children's.  We came by ambulance (no lights or sirens though), so I can cross "Ambulance ride" off my bucket list.  We got here at 7pm and settled in.  While she doesn't look sick, she is. Her apneic episodes are more likely a result of the coronavirus rather than a complication of her swallowing problem.  At midnight her heart rate was over 200, her fever was 102*, and she had a febrile seizure that lasted 10 minutes.  They did blood work and a lumbar puncture. Today, results and specialists. Right now the respiratory virus is by far the bigger concern than her esophageal narrowing.

I'm so glad I am here.  At home I was worried for her life.  Here am I not worried because I know she is in the best possible hands.  I don't worry that she will stop breathing.  I would not have known how to handle a high fever like that or a seizure at home, and I would have wished I were here. Now I am hopeful that we are on the Fast Track to meeting with specialists and getting answers.



So the take home message here is do not delay going to the Emergency Room.  Do not wait for your baby to turn blue or stop breathing. Do not wait for your baby to have a seizure at home. When in doubt, GO!  If you dread going to the hospital because its expensive and stressful and its a holiday, DON'T!  If you are concerned about paying extra copays because you are overreacting and its probably nothing, DON'T.  It's just money, and your baby's life is worth more than that.

Night Vigil

This week has been brought to you by caffeinated soda and the Owlet baby monitor.

I'm so glad that Chris has had the week off.  We've been taking turns on "Night Watch" for Hannah.  It started the night before Christmas Eve, when Hannah's Owlet baby monitor kept signaling low oxygen (5 times in an hour, 8 times total). The second half of the night, she would cough and sputter in her sleep, and then choke on her mucous and have white foamy spit. We are getting uncomfortably comfortable with this process: She coughs and chokes, we hold her upright and pat her back while she tries 5 or 6 times to swallow, her eyes huge and confused.  We give her a few seconds to try to sort it out, then blow a puff of air in her face to startle her and help her get breathing again.

This overnight vigil has us fairly terrified. I was really sad at the prospect of spending Christmas in the hospital, but I had this gut feeling we were headed back there. (I had already spent Thanksgiving in the hospital, seeing as Hannah was born the day before).  But at about 4am on Christmas Eve, I got this crazy idea to have Christmas a day early in case Hannah and I had to go back to the hospital.  The kids were surprised and very confused that Santa had come a day early.  Audrey is on to us, and said that Santa couldn't have come because he brings cereal and puts beef jerky in Dad's stocking. She was certain he would really come the actual night of Christmas Eve. The bigger kids just kept looking at me like I was crazy, and trying to figure out why. (Haha, good luck with that one kids...you'll never solve me!)

The same thing happened again that night, with Chris and I taking turns holding a sleeping baby upright all night long, and thinking that this is ludicrous and certainly not a practical or sustainable way to keep a baby alive. I would stare at her in her cradle with a flashlight and watch her O2 drop on the monitor, waiting for the alarm to go off.  I wanted to see if she was turning blue, if she would recover on her own, and if it was upsetting to her (It wasn't-she slept right through). It looked exactly like Chris's sleep apnea to me.

The on-call nurse from the pediatrics office said to go to the emergency room if she stopped breathing, so to the emergency room we went.  My mom gave me this tidbit of reason: "If she needs to go to the hospital, take her, regardless of the date or hour. Dad had his appendix out on Christmas one year because that's the day he got appendicitis."

I stopped unpacking my hospital bag last time we came home, so that its mostly ready if we ever have to go back. Kinda sad, huh? Well fortunately, we did not have to stay! Dr. Henderson was actually on staff Christmas morning, which was great, because he knows Hannah's case very well. I had already decided to keep him as Hannah's Primary Care Physician (PCP). The ER staff monitored her oxygen (which of course was great by now), and did a swab for respiratory infections. The protocol was to keep her on the Owlet oxygen monitor at home and watch closely for lung retractions, indicating trouble breathing. (Her other breathing problem is already explained by the cricopharyngeal bar, and we already know there is nothing we can do about that at this point).

I asked about the suction clinic (Outpatient Respiratory Clinic).  Unfortunatley it doesn't sound like the kind of thing that would help Hannah. Its for respiratory infections, to get the mucous from the back of the nasal cavity. Hannahs problem is that she can't swallow her own saliva, and it's much lower than the nose-she would need to be suctioned every 20 minutes.

Soooo close.
I feel like she is trying to smile at us!

We came home with a new resolve to wash hands better and sneeze and cough into face masks rather than the ether around us.  Hannah did test positive for coronavirus, and by the symptoms, I'd venture to say that everyone else in our house has it, too. 

I have a theory about her breathing.  During the day, Hannah is in a pack and play in the living room, surrounded by light and noise. She wakes and naps lightly throughout the day.  At night she is snuggled tightly in a dark, quiet room, and can sleep much more deeply. That is why she has possible apneic episodes and doesn't wake up when she coughs and chokes.

Those two nights were the worst and fortunately the following nights were mostly better. She tends to sleep fine for the first half of the night, and then has more low-oxygen alerts and coughing spasms toward the 4-6 am hours.

I spoke with Dr. Schramm on the phone Thursday and he said there's not much we can do differently at this point. She's too little for surgery, repeating the dilation procedure is no guarantee of improvement, and the next level of care would be having her  intubated in the PICU for breathing.

We are tired. The status quo is not ideal. In fact it's terrifying. She de-sats while I'm holding her in my arms.  We wish there was some kind of intervention that would make this easier while we wait for Hannah to get bigger. In the mean time, we are going to consult with a GI specialist, a nutrition specialist, a speech language pathologist, do a motility study on the rest of her esophagus called manometry, and possibly a sleep study for apnea.

Finally getting some chubby-ish rolls on her legs.

I'm so glad I spontaneously bought the Owlet monitor one day when I was pregnant. It was not something I had ever previously considered spending money on. Ever. But now I think I was truly inspired, and that it showed up for sale on my Facebook feed at just the right moment on a payday.

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."

Home Again

We came home from the hospital (again) on December 7. My friends organized a fabulous 12th birthday party for Leah.  My parents eventually had to leave.  The kids went to school. Chris went to work. We had cinnamon toast for breakfast, lunch, and dinner on more occasions than is necessary to admit.  Lexie realized she was shafted as littlest and compensated by being the loudest and trying to be the neediest.

Hannah has been on a feeding tube continuously (24 hours a day), and my job is to keep her alive and make sure she gains weight. We came home from the hospital (for the 3rd time since birth) on a Thursday, and the following Monday she had lost weight.  The regular pediatrician said he wasn't concerned, but was confident she would gain weight. But I was concerned, and so was the surgeon. So I guess that is strike 2 for the pediatrician? So my next task was to get her more calories (and find a new pediatrician).  I talked to a nutrition clinic over the phone on how I could increase her calories, whether by adding more formula to the breastmilk (usually formula is mixed with water), or by increasing the volume she receives per hour.

My baby has baggage already.

We did a lot of weight checks this week. This is the most unusual newborn experience: Hannah doesn't wake up hungry in the middle of the night.  She sleeps the whole night through, and I wake up to an electronic beep indicating that her feeding bag is empty. At some point, she will have to learn hungry vs. full because 24 hour feed is not realistic.

Its easier to stay home than try to go anywhere. I've been thinking of this week as a "holding pattern" while we wait for the next steps. At first I was hopeful that we'd have the feeding tube for 2 weeks while we give the botox more time to be fully effective, and then we'd have a great swallow study and she would be able to bottle feed with a slow-flow nipple to make sure she wouldn't aspirate. I was hoping and expecting things to somehow be "normal" with that timeline, but when home healthcare called to close out our case I told them please not to, because we aren't sure what will happen next week, and this girl still needs to grow.

I have appointments next Monday for a swallow study, and next Wednesday to follow up with Dr. Schramm.  There was some confusion about which doctor should do the swallow study-we have an Occupational Therapist we've been working with at UVH, but a SLP clinic at American Fork hospital called me to set up the study. I was very confused about which one I was supposed to do, as it seemed they both wanted to do the study slightly differently. I had the surgeon call them both and sort it out for me. I also have to decide which pediatrician to stick with as Hannah's primary care doctor here in Provo.

Meanwhile, I'm gathering information and finding resources in every way that I can.  I found a FB group for people with babies that have Cricopharyngeal Achalasia, and there are about 17 of us.  It sounds like most babies are not diagnosed until they are 6-12 months old. They start out getting treated for reflux (where we started, too), then the babies spend months failing to thrive and getting aspiration pneumonia repeatedly. By the time they find the problem, the babies are sick and have developed an aversion to FOOD!  I'm so glad we have been ahead of the game in getting this diagnosed so early-6 days old rather than 6 months.

I joined a regular Achalasia group (general swallowing disorders), and its nice to have people who have the same vocabulary about swallowing and understand the challenges we are experiencing. But for the most part, their achalasia is a disease and it is acquired, and the surgeries are different (lower esophageal sphincter, rather than upper), whereas Hannah's is congenital and will likely be all better once she can have the surgery.  The new friends I've made online were quick to suggest surgeons all over the world and tell me that I need a team to manage this condition. I agree that I need a team. In addition to ENT that we already have on board, it looks like we will have at least Occupational Therapy (OT), Speech Language Pathology (SLP), Nutrition, and Gastroenterology (GI) to consult with. But I am hesitant to buy into their claims that "Dr. So-in-so in Timbuktu is the ONLY one who can treat this!" and "You MUST go to Amsterdam and see Dr. Amazing!"  I am collecting a list of surgeons, but I can't see that it is necessary or realistic to cross the country to meet with a specialist. Even the specialists in Boston or at Stanford have only seen this particular disorder a handful of times.  All the research I have found about CA specifically (and not all the other achalasia problems) is done retroactively and has 5-12 cases in it.

No shortage of cuddles around here!

And friends.  My friends have been incredible resources for me. My BFF is married to a surgeon, so I call the Swensons regularly to get their input and moral support, as well as get a realistic down-to-earth medical opinion from someone I trust and doesn't charge a copay (Thanks you guys!)  Plus, its 2 hours earlier in Alaska, and I can call them at midnight and its only 10pm for them! And Jenn Petersen, whose son Zeke has was born with a heart defect, has been my "mentor" as I learn to navigate the hospital world and parent a critically needy child.  She told me something very valuable: There is not ONE doctor who is going to know everything about my child, her history, or her medical condition. I am the expert on Hannah, and I am the one who is going to know everything there is to know about her. I'm so glad she told me this. I have already learned so much, and often find myself telling doctors what I've learned from the other doctors. My mom also encouraged me to have a notebook that has all her information in it since birth.  All her weight checks, every change to her feeding regimen, all the questions to ask each specialist, followed by the answers, every phone call, etc. Its kind of like downloading all the information from my brain so I don't have to rely on my memory exclusively. (This tired momma is forgetful!)