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!)

New vocabulary words

The second hospital stay lasted 5 days and was also really boring.  I tried almost everything on the room service menu. More than once.  I watched 2 entire seasons of The Big Bang Theory.  I hemmed a quilt for a customer.  Outside, it went from fall to winter.  The kids came to visit just about every night and enjoyed the playroom for family visitors.  My mom held down the fort at home and cleaned and did errands.  They put up a Christmas Tree. My dad came on Wednesday morning and mom flew home on Wednesday night, effectively taking turns so that someone would still be able to help us manage our kids and house amidst our continuing crisis. Chris went to work.  Hannah lost weight.

The first bit of progress was that the MRI was normal.  That was a relief.  Dr. Schramm reported that in her scope, he found:

1. The esophagus was closed much like the first time.
2. The airway was particularly narrow.
3. She had a tracheolaryngeal deep groove. Not quite a cleft, but deep enough to take note, but unclear on whether it was relevant.
4. One of her vocal chords looked sluggish. This could be a function of being anesthetized, or it could indicate another abnormality.
5. She had some secretions in her lungs, indicating some aspiration of saliva or mucous.

Dr. Schramm wanted to give the botox a good 2-3 weeks to be fully effective before deciding if it had failed or not.  It was apparent that the dilation had been effective for about a day.  At this point it was important to figure out how to feed Hannah at an appropriate rate for growth, but in a way that would not cause problems. She got an NG tube (down the nose) for feeding. Bolus feeding means a bunch of food at once, and resembles how people actually eat: having a meal and getting full and then waiting until you get hungry again.  For Hannah that would look like getting a 75 mL bottle (through the tube) over the course of half and hour, and then again in 3 or 4 hours.  It gives the stomach the chance to stretch and feel full and feel empty.  You know, regular hunger cues.   But unfortunately the bolus feed made her choke and regurgitate through her mouth and nose on account of the reflux.  Also the feeding tube itself keeps the esophagus open somewhat creating a conduit for things to come back up.

The alternatives to bolus feeding include an NJ tube, which bypasses the stomach and puts food directly into her upper intestine, or a continuous feed with the NG tube.  I want Hannah to be able to have food in her tummy and not have to always be hungry, so I was a big fan of the second option. It was decided that the best way to get her the needed calories was to use a smaller volume of breastmilk and mix in a little bit of powdered formula to keep the calories high enough. The final goal was to see Hannah gain some weight before we could go home. At this point she was almost a full pound below her birthweight (her birthweight was 8 lbs 12 oz).

At one point an Occupational Therapist from the NICU stopped in to see if Hannah's swallow also had a behavioral component that could benefit from swallowing therapy, and if trying different thicknesses and nipple flows would affect her differently. The OT Deanna would prove to be an excellent advocate and source for us.

I don't know why it took us 5 days to get this much figured out and I can't even remember what progress we made each day.  Nevertheless on Thursday the 7th we went home.  I missed the kids choir concert by only a couple hours, which was a major disappointment to me.  I got home around 6pm and had to wait for home healthcare to come and bring me a feeding pump and show me how to nourish my now 15 day old baby through a tube in her nose.



Lexie loved the family play room at the hospital.

My instructions were to follow up with the pediatrician and make sure Hannah was gaining weight.  I also had to look forward to another swallow study and then an appointment with Dr. Schramm in a week and a half.  Somehow I got it in my brain that that time frame was a point at which things could maybe go back to normal, and therefore we'd only have the feeding tube for a couple weeks.  How do I get these crazy ideas in my head, anyway?

The Walk of Shame

When we went home on Friday, I thought Hannah was all better.  She was able to nurse normally and it was great! At this point, I had somewhat of an emotional melt-down.  I was so relieved that she was ok and that we had identified the problem, yet processing in hindsight the horrifying reality of what could have been. Seeing that the immediate crisis was past, I finally had the luxury of ugly crying and releasing all that pent-up fear, worry, relief, and exhaustion. At the same time I suspected that maybe we weren't out of the woods and was afraid that we'd have to go back to the hospital, with all its uncertainty, waiting, and emotional exhaustion.

By Saturday evening, I realized that not much had changed from Hannah's initial condition.  While I could see some improvement in her ability to swallow right away, it hadn't lasted for more than a day or so.  She still had trouble catching up on breathing while I was nursing her.  Her Owlet baby monitor would signal a red alert that her oxygen was low while I was holding her in my arms. The low oxygen parameter for the Owlet is fixed at 80%, which is much lower than the hospital's 86.  

Overnight, Chris and I decided to try feeding her by bottle with a super slow-flow nipple so that she would not get overwhelmed by a high volume or overactive let-down, and we could moderate how much and how quickly she was eating.  He would give her the bottle for 5 seconds, and then we would wait 1-2 minutes for her to swallow, breathe and recover, then start again.  Just like before, she would try swallowing and stop breathing and start losing color.   We came home Friday with the ability to feed Hannah orally, but by Saturday, I could tell that it was not working anymore.  Somehow the dilation and botox procedures were not effective. She would need some kind of intervention for her to be able to eat without aspirating or she would starve and fail to thrive, and without going back to the hospital, there was no way to get that need addressed immediately.

What a fabulous view

So Sunday morning at 7am Chris dropped us off at the Emergency room after being home less than 48 hours.  I was very disappointed, but I knew that we needed to go back and keep working on a solution for Hannah. That walk down the hallway back into the peds ward was disappointing: I had just been there days before, and I now knew these people, had just said goodbye to them, and here we were again, right back where we started from.

Being retired, my parents have the luxury of being able to hop on a plane at a moment's notice, which my Mom did.  She even went to church first and fulfilled her obligations there before going to the airport, and was in our hospital room by dinner time.  I'm so glad she was able to come back and be "me" at home while I was at the hospital with Hannah.

Dr. Schramm stopped in to check on Hannah. He said he was disappointed to get the text that we were back, but not surprised.  One of the things we had learned along the way was that conditions like Hannah's are sometimes associated with an underlying neurological condition called a Chiari malformation. Babies under 30 days old have to be kept in the hospital for observation for 12 hours following an MRI, so we initially had planned to wait for her to be 31 days old.  Now that we were back anyway, it was the logical next step.

Monday turned out to be one of those wait-all-day-for-something-to-happen kind of days.  The MRI schedule was booked solid plus had 4 emergent cases.  Because of her size and age, Hannah would have to be fully anesthetized for it, and since she would be put under, Dr. Schramm decided to do another esophageal scope.  So we had to wait for MRI, anesthesia, and OR all at once, and of course she had to have an empty stomach while we waited.  We had no idea if or when all these things would align perfectly.  Sometime around 2pm some nurses came into our room and said, "It's time to go right now."  That's why I never left her bedside: wait all day, and then not even have time to go to the bathroom before heading to surgery again.

Trying out oxygen while she sleeps.

Meanwhile, back on the ranch...

The day Hannah was born, Lexie (2 1/2 years old) had full-blown croup, with a 103 degree fever, diarrhea, cough, and lethargy.  My mom took her to the pediatrician and Lexie got a steroid treatment, which started working almost immediately.  Regardless, she was still symptomatic when Hannah and I came home from the hospital 2 days later. It added another dimension to having a newborn at home: the risk of her getting the rest of us sick.

My mom had been with us for a week and a half and had gone home (to Chicago) on Monday.  On Tuesday, the day everything hit the fan, Lexie still had a fever and diarrhea, Audrey was starting to cough, and Leah (11 years old, 6th grade) had stayed home from school with a high fever and cough.  I always feel so bad leaving her home alone when she's sick, because she gets lonely and bored, and I would rather be home with her to keep her from getting too discouraged.  She is the most conscientious tween and doesn't want to let her teacher down or miss choir practice or get behind on school work.  So she stayed home because in my professional opinion as her Mother, she was too sick to go to school, but I was running all over creation with Sandy getting medical attention for Hannah.  By afternoon when I had decided to see another doctor, I called Chris and told him it was time for him to come home early, and arranged for someone to stay home with Lexie until he could get back, which would be about 45 minutes.

By Wednesday I was living and sleeping and showering at the hospital.  I was afraid to leave and miss something important.  We were in full on crisis mode, and so many friends and loved ones wanted to help, but I didn't know what I needed.

Fortunately Chris was able to take a sick day on Wednesday to stay home with Lexie.  On Thursday he had work meetings he couldn't miss and I had to coordinate people to help get my kids the right places at the right time.  This is where I got to call in all those proffers for help.  Chris got the kids out the door to school, dropped off Lexie at a friends house, and went to work.  Leah was again home sick and I didn't want her to be home alone, but I also didn't want her to get my friend's kids sick.  I rallied friends to check in on her throughout the day, and they came through for us with flying colors. A friend picked up milk and pizzas and came over to have lunch with her. Others dropped in to check or keep her company.  Leah and Samuel had a concert the following day, and I recruited one friend to finish making Leah's black skirt, and another friend to pick up some black concert pants and socks for Samuel at Kohl's.  All week friends and ward members showed up with gifts and dinners and rides for our family.   I think all our friends now know our garage door code! 

There is no way we could have met all our obligations without the outpouring of love and support from our friends and church community, which I hope any church community could provide, regardless of denomination.  We are Mormon, and each family has Priesthood brothers and Relief Society sisters assigned to watch over and provide spiritual support and fellowship. Our ward (congregation) jumped into swift action to provide the compassionate service we needed.

On Friday, Leah was again too sick to go to school and missed her concert anyway.  She was especially discouraged because the next day, Saturday was her birthday. No one wants to be sick on their birthday!  I knew I absolutely did not have the time or mental energy to plan a Harry Potter birthday party, and so I asked my Relief Society President, "Remember how you said I could ask you to help with anything I needed?  Well, I need you to plan a Harry Potter birthday party."  She and another sister came through for me and started making arrangements for a party the following week.

For the second Friday in a row, Hannah and I came home from the hospital in time for lunch.  Saturday was Leah's 12th birthday and we got to be together as a family. We went to an art museum where Leah's work was on display, then went out for pizza. Everything felt almost normal, but I was leery that Hannah was really not out of the woods yet.

On being in the hospital

Part 3.5

One thing I learned right away about the hospital experience is that everything takes forever, and "soon" means any time in the next 3 hours. Or it could be in 5 minutes.  "The doctors and residents will be rounding soon" means between 9am and noon, and "you're next" might still be in 45 minutes.  Waiting for a procedure takes all morning, and waiting for the results takes all afternoon. Leaving for surgery will happen 2 hours after the scheduled time. And if you're waiting to take that shower or hit up the cafeteria, something will happen the moment you step out of the room. 

As a parent, you don't want to miss anything happening or the chance to ask questions when the doctor does pop in. It makes you feel somewhat paralyzed. The inability to leave, change gears, even take a nap or take 15 minutes to pump without someone walking in.

My constant view

For me, feeling helpless and worried sick about my child put me in such a funk that I didn't want to do anything at all. I didn't feel like reading.  I didn't feel like sleeping.  I didn't feel like playing a game on my phone.  I didn't feel like watching TV.  I didn't even think to eat or feel hungry.  Nothing.  And with time and progress crawling at a snail's pace, it was SO BORING.

Also, it was hard not to look around and see dollar signs piling up on every piece of equipment, every morsel of food or medication, every disposable plastic thing. Every time the nurses scanned something before using it or administering it, I knew it was getting added to our bill.  And what is the base room rate at this B&B going to turn out to be anyway?  Try not to think about it, Jenny.  Does it matter how much it costs?  Does it matter if I will ever be able to pay it off? No.  It doesn't.  Does Hannah matter?  Yes. She does.

My experience was to pump every 4 hours and then wash my pump supplies.  The nurses brought me drinks or snacks.  I ordered 3 meals a day, but they took 45 minutes so I had to plan ahead before I actually felt hungry, and then sometimes my tray would come and I wouldn't even have an appetite to eat it for an hour or two.  I gave the nurses my dirty dishes.  I exchanged pleasantries with the housekeeping staff when they emptied my trash.  I surfed the TV channels for something better than Family Feud.  I held the baby and got tangled in her IV, feeding tube, and oximeter cords.  I watched the baby's heart rate and oxygen levels.  Then I gave the nurses my dirty dishes and started all over again.  I perked up whenever a nurse came in for vitals or a doctor stopped in to give me a 5 minute update on the progress for the day's goals (of which we would likely accomplish only one or two because everything takes so dang long).  The nurses were fun to talk to.  We often chatted about regular stuff, including McDreamy vs. McSteamy. (even though Grey's doesn't remotely resemble actual medicine, every nurse is privy to this debate and has an opinion.)  

Each step of the way I met professionals whose whole days were spent doing just one step of this process: taking meal orders over the phone, delivering meal trays, sweeping our room, escorting hospital beds or cribs from their room to the surgical wing, processing patients for surgery in the pre-op holding room, updating family members in the waiting room, guarding the door to the pediatric wing, and so on.  And many more processes I was not even aware of.  

Nurses came and went on 12 hour shifts, reporting for their work day and then going home.  Everyone has a very specific job and they only encountered Hannah and me for a brief moment, and we became a short-lived blip on their dutiful work-day.  Because its a day at work.  And everyone I encountered, regardless of their role, was genuinely concerned for my child's wellbeing and recovery.  I'm incredibly grateful for all the well-oiled cogs that make up our modern, first-world hospital system.  I may only cross paths with them briefly, and some of them play smaller roles than others, but they are all essential and important. 

This is nothing like Grey's Anatomy

On Thursday the surgeon suggested doing the balloon dilation and the botox injections together, which I had also considered.

The nurses turned off the baby's feeding tube some time in the middle of the night so that she would have an empty tummy for anesthesia and surgery. Poor little one. She might have been on an IV for nutrients and hydration, but that doesn't do much for an empty tummy. Hannah was surprisingly peaceful nonetheless. She certainly loved the binky and being tightly wrapped.

Super Scary Form

Surgery was scheduled for 1pm. So naturally at 3pm we finally got started. Wheeling a ginormous crib and IV pole for an itty bitty 8 day old baby down the hallways and elevators to surgery was truly surreal and foreign. First we went to "holding" and signed all the Scary Papers and met the anesthesiologist. Then she was wheeled one direction and I had to go the other direction.  The waiting room had a screen showing patient number and surgery status...kind of like waiting for a flight to arrive.

I'm so grateful to my visiting teacher and friend Kari who came with me and sat with me during surgery so that I would not be alone. I was a ball of nerves.  It was refreshing to talk to someone that wasn't a doctor or nurse, and to remember that it was still just a Thursday and that real life was still swirling all around outside of the hospital.

Esophagus before and after dilation.
Top picture shows intubation tube going down trachea.
Cricopharyngeal muscle is below the esophageal opening.

The official name of Hannah's surgery was "esophagoscapy with balloon dilation and botox injections."  The surgery took less than an hour and Hannah seemed much the same as before, albeit very sleepy. (Her cry sounded a little different afterward too...less like a screechy, wounded velociraptor and more like a newborn, but that could be completely unrelated to surgery).  Dr. Schramm gave me some pictures of the procedure and said "Here are the most expensive baby pictures you'll ever have." 

I was told that as soon as she was able to nurse successfully twice we could go home. And Hannah nursed like a champ, as if we hadn't missed a single step.  Yay!  Hannah was all better! We went home Friday before lunch.  It turns out I was probably a little overeager to go home after 3 days in the hospital, because we wouldn't be home for very long!

Looking for Zebras

Wednesday morning we did an esophagram. Its a real time x-ray taken while the patient swallows a barium contrast in order to watch the progress of her swallow and see the anatomy of her esophagus and upper GI tract.

One thing to look for was a tracheoesophageal fistula, which is an abnormal connection between the trachea and esophagus.  Presence of milk in the trachea would explain why Hannah's airway closes and she loses consciousness when eating.  The attending doctor said TEF's are extremely rare and unlikely.  He said its like looking for zebras in The United States...you just aren't going to find them.

Sure enough, no fistula. There was however the acid reflux as the first doctor had said the day before.

But Hannah's swallow was far from normal.  I watched the radiology techs literally scratch their heads during the esophagram.  I didn't know what normal looked like, so I had no idea what to expect.

The pediatric ENT surgeon, Dr. Schramm visited our hospital room to tell us what the x-ray had found and give us the next steps. The x-ray showed a cricopharyngeal bar- the sphincter muscle at the top of Hannah's esophagus was abnormally large, and failing to contract and relax properly during the swallowing process. The muscle is in the way, creating a significant narrowing of her esophagus, which was causing milk to pool above the bar rather than drain down at the appropriate rate.  As the milk pools it can spill over and go down the trachea, which tries to stop it by closing or spasming, which stops her breathing. That's why she passed out and turned blue whenever she tried to swallow.

This condition is called cricopharyngeal achalasia.  And guess what? Its way more rare than a fistula. Zebras, indeed!  I like to think of it more as a Space Monkey.  Dr. Schramm had seen this particular condition only a few times, and usually as an acquired condition in elderly people.  He consulted with a colleage at Primary Children's, and he had only seen 5 neonatal cases in his 25+ years of practicing medicine.

The treatment options we were given are 1. Botox 2. Balloon dilation 3. Myotomy

I know what you're thinking: botox!? Isn't that for facelifts?  Botox is used to paralyze a muscle, and in this case would be used on the cricopharyngeus to keep it from spasming and allow it to relax and get out of the way.  A dilation means making something wider. A balloon is inserted into the esophagus and "inflated" to open up the esophageal canal. And a mytomy is a procedure in which the muscle is cut. These options were given in order of least invasive to most invasive.

After getting the results, we were given time to process and consider the options. Surgery was scheduled for 1:00pm the following day, even though we weren't yet sure which procedure we would do.  Hannah was allowed to get some nutrition through an NG tube, but only for a little while, as she had to have an empty stomach for surgery.

At about this point I was feeling relieved to know that I hadn't been imaging that there was a problem, and I was so glad to have a name to go with it!  But it is kind of weird to be in the hospital with a baby who isn't actually sick-she looks and sounds just fine, and as long as she isn't trying to eat, she is. But she really isn't fine.  The advantage to having a rare and bizarre congenital condition (if you can call anything about this advantageous), is that the only hits on google searches are for medical journals rather than buzzfeed or a mommy forum.

On Exemplary Friends and Second Opinions

Having successfully breastfed 4 babies, I knew something was wrong when Hannah started turning purple during feedings and stopped breathing for 15-25 seconds after nursing for only a few seconds. This started pretty much as soon as my milk came in.  She would also slurp and swallow lots of air. She had the suck, swallow part figured out, but not the breathe part. We also have an Owlet baby monitor that alerted for low oxygen and low heart rate several times at night and during feedings.

My friend Sandy brought me lunch on Monday and offered me a ride to see a lactation specialist the next day, since one of our cars was broken down.  So on Tuesday morning, when Hannah was 5 days old, we saw a lactation specialist at the WIC clinic. She told me that Hannah had a slight lip tie on top, and also gave me a few ideas about helping her latch better to not swallow so much air. She had never seen a baby stop breathing and turn blue quite like that though, and was very concerned, calling in two other people to get their input. They told me to go straight to the doctor, which I did.

The regular pediatrician that we see listened to Hannah's heart and lungs, and I described her non- breathing episodes (she wasn't hungry at this point, and I was also a little hesitant to nurse in front of the 30-something, handsome pediatrician). His initial opinion was acid reflux, and he prescribed a medication for it and sent us on our way.

By this point it was lunchtime and I had been away for several hours, leaving my oldest child Leah alone, home sick from school. I sent my visiting teacher over to my house to check on her. I had told her I'd be gone an hour.

I'll reiterate that this was already a stressful first week of life with a new baby: all of the other kids getting sick in succession, a broken down car, and my mom going back home the day before.

Sandy and I went and got some fast food lunch and let our 2 year olds play while we waited for the prescription, and then Sandy dropped me off at the mechanic to pick up my car and I went home.

Not half and hour later she called me to say that I needed to get a second opinion.

By this point it was almost 3:00. Leah was burning with fever and incredibly bored, Lexie was down for a nap, and the other kids would be getting off the bus soon.  I made an appointment to see another doctor within the hour and arranged for a ward member to come and sit with my kids until my husband could get home from work early.

The second doctor, Dr. Henderson watched me nurse Hannah so he could see her disrupted breathing, and see how her oxygen changed (by this time my concern for Hannah outweighed my hesitation to nurse in front of a good looking doctor).  Hannah took 25 seconds to inhale and her oxygen dropped to 72%.  The second time she stopped breathing for 16 seconds and her oxygen dropped to 67%.  I'm so glad that Dr. Henderson trusted my mommy instinct that this was more than just reflux, that I wasn't imagining that she was turning purple, and that this was definitely not normal or OK.  I felt that he was truly advocating for Hannah and me, and wanted to be aggressive at figuring out this puzzle.  Babies turning blue is definitely a big problem, people!  The clinic did a chest x-ray and sent me home, with the expectation of likely admitting Hannah to the hospital once a radiologist could look at her film.  It sounds ludicrous now, but after running around all day I remember thinking that it was a terrible day to forget deodorant.

I got home around 5:45 and had a quick bite to eat-a friend had dropped off some pizzas for us!  I hugged all the kids and packed a bag. At 6:15 the doctor called to instruct us to go straight to admitting at Utah Valley Hospital, and that Hannah should not eat anything. Hannah was given a priesthood blessing by my husband and some ward members, and she and I went to the hospital.

This was incredibly scary for me.  I had several trains of thought. On the one hand, I was worried that this was overkill: "What if this is nothing?  What if she's fine and the doctors think I'm a paranoid, helicopter mom?  What if we go to the hospital and do tests and put this new baby through all this fuss and get a whole bunch of medical bills for nothing!?"  And on the other hand I was terrified that something was really wrong and that we would lose her and this was really the beginning of the end.

I should pause and remind you that Hannah was only 6 days old (as of 6:45 pm), and I was therefore also 6 days postpartum, with all its glorious bells and whistles.   I was a hormonal train wreck, emotional, wounded, swollen, exhausted, and still had a fabulous epidural headache.  I had been on my feet running around all day for the first time since giving birth.  While the day before I had been in the groove of lying low, being waited on, resting, and getting a free pass from dishes and laundry and cooking, I was now in full-on caregiver mode and my recovery was officially over.

Once we were admitted to the pediatric ward, the first step was to rule out an atresia, or a dead end in her esophagus. After getting an IV into an extremely dehydrated newborn, the pediatric doctors inserted a tube down Hannah's throat and took an x-ray to make sure it was completely connected to her stomach. An atresia was unlikely given the fact that Hannah was not throwing up and was obviously getting nutrition since she had plenty of wet and poopy diapers, but it had to be ruled out nonetheless. No atresia.

It was incredibly hard for my momma heart to watch a team of half a dozen nurses and doctors poke and prod my new baby. It took 3 or 4 tries to get an IV going, and also, babies don't like having a tube put down their throat either. Would you?

By 11pm we were left alone to get some sleep for the night. Poor Hannah had IV fluids but an empty belly. I was impressed how surprisingly content she was despite feeling hungry and missing the comfort of nursing. She's such a tiny little peanut in that big scary hospital bed.

That pull-out chair in the back was my "bed" for 3 days.
If you are tired enough, you can sleep anywhere!