Who says waiting is a game?

So many tubes and still wide-eyed.

Every day is long and the weeks are an exhausted, chaotic blur. My body aches and my neck is tired of holding up my head. Sunshine and fresh air is rare in 20° inversion weather. Winter seems like it will be eternal, flu is a constant threat, we rarely leave the house, two year olds are tiny, manipulative terrorists, and we have serious cabin fever. So it surprised me when I realized it's already been a month since we saw Dr. Smith in Salt Lake!  We had another visit with him this week and have a little bit of a game plan. We're a little disappointed that Hannah's growth slowed down: only 2 ounces in 10 days. At the last few checkups she was gaining an ounce a day with the same number of calories.  Definitely something to bring up to the pediatrician.

Dr. Smith was pleased to hear that we are consolidating Hannah's feeding schedule and that she is tolerating the tube feeding well at a higher volume per hour (now up to 23 ml/hour! Baby steps, people...) She hasn't been spitting up or throwing up, and there is no evidence that she is aspirating to her lungs.  We do keep her elevated except during diaper changes, and she takes zantac to reduce any pain from reflux.

This is what survival mode looks like. Netflix is my babysitter
and she keeps changing her mind about what she watches.
Please don't judge me...

Incidentally, I recently learned that acid reflux medication doesn't actually reduce the reflux, it just makes it less painful. I can tell that Hannah is more fussy when I've missed a dose, and she does cough and sputter more if she is flat on her back for too long.  Also, the narrowing in her esophagus works slightly to her advantage in blocking reflux from coming up. All babies have reflux to some extent because the lower esophageal sphincter (LES) is immature. He said there is no way to know at this point if she has abnormal LES reflux or decreased motility in her esophagus below the CP (cricopharyngeal) bar. We can cross our fingers and hope not! He does not think we need to meet with a GI specialist.

A rare moment of sleeping without
being held. Gorgeous quilt by Aunt Barbara.

Dr. Smith ordered a pulse-oximeter test overnight to see how she does without oxygen. That would certainly simplify things at home to not have a 25 foot lead all the time, or a 20 lb tank whenever we want to leave the house. We'll know within the next few days if she passed. I peeked at the oximeter a few times overnight and I didn't see it get below 89% so I think she'll be fine.

Both her reflux and oxygen have been normal since we've been home from Primary Children's after getting over influenza. When she was sick she definitely needed the oxygen, and she also had more mucus and saliva secretions that made her cough and sputter and regurgitate more. Some days I have to suction white foamy secretions more than others and I get a little worried that she is getting sick again. Fortunately she hasn't had any more red alerts on the Owlet or a temperature for several weeks. That certainly gives us some peace of mind.

Every hospitalist I've talked to gets a grumpy face when I mention the Owlet monitor. The majority consensus of the doctors I've talked to think the Owlet is inaccurate, gives too many false alarms, and creates more parental worry than it alleviates. They are of the opinion that parents should be watching the baby rather than the monitor, and have told me that they very rarely send even NICU babies home with an oxygen monitor. But in the hospital and at home when we are testing her breathing without oxygen, what do they use? An oxygen monitor. I've pointed out that sleeping parents aren't watching their babies all night, so how else would they know the baby had a problem? And they can tell that I am a hands-on parent who doesn't just watch a monitor. The Owlet has legitimately alerted us that our baby needed emergency medical attention that we otherwise would not have sought on more than one occasion. Does everyone need to go out and buy one? No. But I truly think I was divinely inspired to. If you can afford it, or if your infant is medically fragile in any way, I would recommend it.

Snuggles always.

Back to our appointment with Dr. Smith. The game plan right now is to continue consolidating feeds, meet with a feeding therapist (he used Speech Therapist interchangeably) and come back on March 5 for a swallow study. If her esophagus shows improvement, we will try some oral feeds, and if not we will try botox and dilation again. I still had an ugly cry at the thought of the myotomy being off the radar for now, maybe even 2 or 3 months away or longer, but give me a couple of days and I will regroup and muster some more courage from the place where courage comes from but never seems to be in surplus. Courage is like the meal and oil Elijah promised the widow woman that never gets empty and never gets full. There is just enough for right now.

Introductions

Big eyes!

Hi! I'm Hannah. I'm 2 months old!  I was born the day before Thanksgiving. I have a crazy family and some crazy sisters and a crazy brother. They are so LOUD but I'm used to the noise. During my naps I sleep better when it's noisy. Sometimes they get along and sometimes they don't, but they all seem to love me and want to hold me and protect me. I like it when they stroke my head. They give me silly nicknames like Hannah Bear, Sugar Plum, and even Miss Hannigan. Daddy calls me weird things like Miss Dump-a-Load.

My daddy is really silly.

Every once in a while those crazy parents stick this thing in my nose and all the way down.  It's awful!  I scream and scream and eventually they stop. Then I don't mind it anymore. I have a feeding tube because when I try to swallow, it doesn't go down right, but it'll get fixed soon and then I'll be as good as new.  For now my tube puts milk in my belly and I never feel hungry. I probably don't even know what hungry is! Because of my tube I hardly ever spit up.

My big sister Audrey

I went to the hospital a bunch of times, and I rode in an ambulance.  At the hospital they poked me with sharp things a lot and made me cry. I especially didn't like it when they sucked boogers out of my nose, but it did help me breathe better. There were a lot of noises and beeps but I got used to it.  I much prefer being at home.

I really like to be swaddled nice and snug, and I love my binky.  Sometimes my mom dips my binky in her magic milk and I can taste it. It is so yummy!  I wish I could have more.

I can sleep alllllllll night long. My mommy brings me to her warm dark room and puts me in my cradle all bundled and then turns on the soothing vibration that helps me fall sound asleep. Sometimes she puts a bean bag on me to make me think there is someone putting their hand gently on my chest. Then I go to sleep for 8 hours and I don't mind that its quiet.

My big brother Samuel.

I really enjoy my bath. It is warm. When I get out I'm cold at first but then I feel so warm and sleepy and safe after I get dressed in fresh jammies. I also like to poop and burp and be held. I ESPECIALLY like to be held. It is warm and comforting to be in someones arms and hear their heart beat and their breathing. It makes me feel safe. Sometimes all those other crazy people all want to hold me at once and it gets a little too crowded. I like to be around my family during the day. When they are eating at the table I like to sit in my vibrating bouncer chair nearby or sit on someone's lap so I can be near all the action. Sometimes my mommy holds me like a football while she eats and that's cool.

I don't like being in my car seat. Its boring and nobody holds me and it's not warm. There's nothing to see or do.

My big sister Lexie

My latest trick is smiling and watching other people's mouths. They get really happy when I smile at them and I get happy when they smile at me. I can almost hold up my head, but it's hard work. There's so much to see that I open my eyes really wide to take it all in, and everybody likes that and thinks I'm cute. I know that I am loved.

Whew!  Now I'm exhausted from all this chatting. Now I get to go to sleep.  See you next time!

My biggest sister Leah!

Holding Pattern

Always wide-eyed!

Hannah is growing! Friday, January 5th she was 8 lbs 14 oz (finally surpassing her birthweight) and a week later, on the 12th she weighed in at 9 lbs 7 oz. That's more than an ounce a day! Hooray!

Hannah is a good candidate for the RSV prophylactic shot.  Even though she isn't a preemie, she qualifies based on the criteria that she has a muscular condition impairing her ability to clear mucus secretions.  The doctor told me that there has been an RSV outbreak this week especially, which makes me nervous because I took her to the grocery store on Thursday. We are crossing our fingers and praying that she stays healthy and we don't have another hospital stay! We are hopeful that the insurance company will approve the RSV vaccine. Even though it is astronomically expensive for a shot, it is much cheaper than another hospitalization.

I also talked to the doctor about Hannah's feeding and oxygen. She's doing really well with a very low dose of oxygen, and for 15 minutes without it, her sats dipped into the high 70's/low 80's.  So we're going to keep her on oxygen for now.

I shall call her squishy and she shall me mine.
And she shall be my squishy!

With regards to nutrition, she's doing well at the amount of calories she's getting. She's been on a 24 hour continuous feeding schedule since December 5. So of course she doesn't ever get hungry.  How many 7 week old babies do you know that sleep 10 hours every night!? But of course the 24 hour feeding schedule must come to an end at some point. Starting last Friday, we are condensing her feeding into 23 hours, and giving her a one-hour break. Then after a week, we will condense her feeding into 22 hours, and so on, so that she can gradually get used to a larger volume in her tummy and alternately feel hunger. She'll still be getting the same volume and number of calories, but will have 1 hour breaks throughout the day. We expect this might be hard for her, and for us it might be a lot like having a one week old baby again.  But it's inevitable and we are glad to make some progress!  I decided to take out an hour in the middle of the night, from 2 to 3am, theorizing that she is sound asleep and might not notice. If she can keep her sleeping-all-night status during this transition, we'll all be much happier.  So far so good!

First smiles captured on camera!

Developmentally, Hannah is right on schedule. She started smiling this week and it is such a sweet, happy smile! She doesn't seem to mind having tubes and tape all over her face. She is always wide eyed and intently focused on the people around her. She wants to make eye contact and is trying to imitate what we do with our mouths.

Daddy making silling faces

As for Mom and Dad, we are surprisingly ok. While the current holding pattern is not ideal in the long term, it is safe and doable. We both know how to take care of Hannah and meet her immediate needs. We are confident that she is getting the right amount of food and that she is going to keep breathing. What a relief! It's such a difference from a couple weeks ago when she was getting sick and we were afraid to go to sleep in case she stopped breathing. We are sharing the load and we are all together at home. We are taking shifts and having meaningful experiences with the other kids. We are effectively co-managing the chaos of a household of 7.  It's not clean or quiet, but like I said, it's a holding pattern. While sometimes we become discouraged by the chaos and exhaustion, we are more often upbeat than depressed.

A brief moment without tubes :)

 We are so glad that Hannah is stable and growing. While it is a lot of working lugging around an oxygen tank and food pump, and for me pumping 5 times a day, we are in a safe holding pattern and Hannah is making progress. We are doing our best to limit her exposure to more illnesses, with obsessive hand washing, staying home, and keeping Lexie away from germ-y places like the mall playground or the church nursery. Of course this also means that I won't get to leave the house much this winter, which feels pretty cooped up.  But Hannah is worth it.  We have been incredibly blessed. We feel the prayers from scores of friends and family and strangers and angels.

Passing the hat

My parents spent 2 years on a church service mission in Nicaragua, one of the poorest countries in Central America. People live on $2 a day and are lucky to have a house with corrugated metal walls. Their fences are tree lines. Their meal is rice and beans three times a day. Their livelihood might be reselling trinkets. They have almost nothing.

When individuals have a crisis or medical need and experience a financial burden beyond their capacity, they "pass a hat" around a room of friends and family members.  And even though they have very little themselves, they generously contribute what they can into the hat for the beneficiary. And the following week, the hat is passed for another friend or family member in need. While each contribution may be incremental, when pooled together with others, it adds up to a meaningful sum for the person receiving it, and helps to ease their burden.

I am not comparing our situation to the dire poverty of a third world country.  We have infinitely more. We have more food in our fridge and pantry than they have in a year. We have insurance. We have access to the best medical care in the world. We invest in the care of the most helpless of individuals and think about costs after the fact. What a blessing it is to live in such a society. But I do feel impressed to "pass the hat" nonetheless.

I find myself compelled to buy a hamburger for the homeless couple on the corner. I am compelled to donate to a friend's cancer fund. I am compelled to help a neighbor cover funeral costs. I want to contribute to a friend's IVF (in vitro fertilization) or adoption fund, and I donate to humanitarian aid when communities are hit with catastrophe. All worthy and meaningful causes.

So I am reconciling my pride and passing the hat for our own family. Many people have asked me what we need or how they can help us during this crisis. At first I didn't know what we needed. Over the course of the past months, I have learned that it is okay to ask for help.  I have asked people for rides, for meals, to pick up my library books, drop off a gallon of milk, watch my kids, hem a skirt, plan a birthday party, and more. And still people want to help. We are truly grateful for all the help and service we have received. If you want to help further by contributing financially to our family, please consider donating through YouCaring. Every contribution is meaningful to us. Thank you for blessing our family and for being a part of Hannah's story and our lives.

-Chris & Jenny

Hindsight

Polyhydramnios is a condition during pregnancy in which there is too much amniotic fluid.  According to the Source of All Knowledge (the internet), it happens in 1-4% of pregnancies. It is usually detected during a prenatal appointment if the mother's belly is measuring larger than expected or weight gain has increased rapidly, and it is usually detected in the 2nd or 3rd trimester. Sometimes it resolves on its own, and sometimes not. It is often a reason to do some testing or deliver the baby early. Other symptoms include edema (swelling), weight gain, discomfort, shortness of breath, heartburn, and more. If you haven't noticed, these are also symptoms of being 9 months pregnant!

Why am I telling you this?

Almost a family of 7. I was so huge!

WELL...during my delivery, when my doctor broke my water, I had GUSHES and GUSHES and GUSHES of amniotic fluid.  My doctor has been delivering babies for 30 years and he said he has NEVER seen that much amniotic fluid before. I even asked him if he was exaggerating; he wasn't. I thought I was just special! At the time, we were VERY relieved that my water had not broken anywhere outside of the hospital; cord prolapse is a serious concern with polyhydramnios, in which the umbilical cord descends into the birth canal ahead of the baby, cutting off circulation (haha...ahead. See what I did there?).  Also, while not nearly as significant of a concern, with that much amniotic fluid, I would have most certainly ruined my mattress.

Sometimes its not clear why a woman would have too much amniotic fluid, and she might even have the condition in more than one pregnancy. But sometimes there is a very good reason. Possible causes include gestational diabetes, decreased fetal kidney function, Rh incompatibility (Hannah and I are both Rh negative, so we can rule that out), neurological abnormalities, and...here's the kicker...a congenital birth defect that impairs the baby's ability to swallow.

I didn't learn this until our 3rd hospitalization, when a doctor at Primary Children's pointed out to me that Hannah had this problem even when she was in my womb. Light bulb! This random tidbit of my birth story has meaning! How did we not know this? Why did it take us 5 weeks to connect the dots?

Birth day! Nov 22, 2017

I vaguely remember measuring a little big at the last 2 or 3 appointments, but not big enough to cause concern, and my weight gain was not abnormal. Another clue might have been that I didn't feel as many sharp knee and elbow jabs as previous pregnancies, and the baby never dropped (think of a goldfish in a Ziploc bag full of water).

At my 6 week postpartum checkup today I told my doctor about Hannah's condition and the crazy road we've been on since she got here.  I asked how we could have missed this and what we would have done differently if we had detected it. Dr. Young was very surprised to find out that the baby had this problem and wanted to know all about what we've been through. He has delivered babies with a tracheoesophageal fistula before, but had never heard of cricopharyngeal hypertrophy. He said there was really no way we could have known, given that my measurements appeared to be within normal ranges.  My last ultrasound was at 33 weeks and there had been a normal amount of fluid, indicating that Hannah developed this condition after that.

During pregnancy, amniotic fluid is measured by the Amniotic Fluid Index (AFI), and a score of 8-18 is normal at full-term, with greater than 20-24 being classified as polyhydramnios. While we don't know the exact volume of amniotic fluid that I had, Dr. Young said that had it been detected, I probably would have had a score of 30-35!

If we had known about the polyhydramnios, not much about my delivery would have been different. Dr. Young would have had me do a non-stress test every week and we would have delivered a little early at Utah Valley Hospital where there is a NICU just in case. But since Hannah's symptoms didn't manifest for several days and there were no other indicators that the baby had a problem, its most likely that we would still have gone home soon after delivery with a clean bill of health for both of us.

Now I am processing in retrospect how it would have affected me if I'd known about the polyhydramnios. I would have worried a lot and googled every possible contingency under the sun. I might have been a nervous wreck. On the other hand, when we had problems nursing that first week I might have had a better idea of what was going on.

Sixty percent of polyhydramnios cases are idiopathic (another new word; it means cause unknown), 20% are neurological and 20% are caused by congenital defects. Considering both of the latter causes would have been very nerve-wracking for me and there may have been copious tears. I purposefully chose not to do amniocentesis or other genetic testing during my pregnancy because all of my ultrasounds had been normal. It seemed like more reasons to worry, and the tests are not always accurate or necessarily definitive. We were incredibly blessed to have an uncomplicated delivery and that Hannah's problem was not immediately life-threatening.

Thanksgiving. My cup runneth over.

As it was, I was able to deliver at my preferred hospital in blissful ignorance and went home with my baby a day and a half later.  I had Thanksgiving dinner with my family, albeit a day late, and 4 foggy days at home with my now family of 7 (seven!) in a blissful, sleep-deprived euphoria. I snuggled my baby and nursed her (well, tried to). I slept in my own bed and showered in my own shower.  My postpartum recovery only lasted 6 days, but I am so grateful I got to have 6 days before everything Hit the Fan and we went into Crisis Mode. And as a tender mercy my recovery this time around was much faster and less painful than previously.  It would have been incredibly depressing for me if she was rushed straight to the NICU leaving me to go home without her, as I'm sure it is for the many people who experience it.

It is possible that Hannah might have more congenital defects than we already know about that could have been detected earlier with prenatal testing. Even though her MRI and neuro exam were unremarkable, its possible there is an underlying neurological problem to explain her hypertrophy. But I am okay with learning that incrementally. Maybe that makes me naive. But I am okay with how things have gone so far and to be honest, I wouldn't have it any other way. I still think she is perfect.

Perspective

by Ellen Sorenson

Hannah with
Grandma Sorenson

Watching Hannah’s journey to health, I keep reflecting on the lives of my ancestors. My grandmother lost four babies before my mother lived. Perhaps they died from the RH issues that led to the death of my own infant sister, or perhaps an infant suffered as Hannah does with an esophageal muscle constriction which left her unable to swallow or to breathe without choking. My grandmother told me that one infant son died because “he was just too smart; you could see it by looking into his eyes.”

Exceptional care at Utah Valley Hospital

In an earlier generation, there were no oxygen monitors to alert sleeping mothers when their babies needed air. Babies like Hannah simply died—suddenly when they couldn’t breathe or slowly because they couldn’t eat. There were no diagnoses. No barium swallow studies with x-rays to reveal a constricted throat. No manometries to analyze esophageal function. And there were no solutions—no carefully calibrated portable NG feeding tubes, no home oxygen canisters, and no drugs to counteract influenza and fevers and seizures. There were no surgical balloons, Botox shots, or myotomies to ease over-enthusiastic muscles.

Sadly, in much of the world today, little has changed. In our recent time in Nicaragua, we lived among wonderful people who lost newborn babies because of lack of intervention during labor and delivery and elderly parents to lack of access to dialysis treatments. We knew an eight-year-old who went to a dentist with a toothache, and the dentist pulled out a permanent molar. We saw people who were crippled because they did not have fractures set or who were bedridden or demented by severe anemia.

Volunteer groups providing meals in the Ronald McDonald
Family Room at Primary Children's Hospital

I am so grateful to live in a society that places the highest value on the life of one helpless infant and that has the resources to save her life. Hannah has interacted with dozens of caring nurses, doctors, ambulance drivers, technicians, and volunteers. Jenny and her family have been fed and sustained by the Ronald McDonald house, Boy Scout troops, and various church congregations. And behind the scenes there are untold hundreds of researchers, inventors, and manufacturers and distributors of medical supplies. There are unseen angels intervening from Heaven, friends nearby who offer dinners, babysitting, and rides to the doctor, and strangers across the country who pray for a little baby they have never met. There are truly Christ-like people who will surely reap great blessings. For Jesus taught, “In as much as ye have done it unto the least of these, my brethren, ye have done it unto me.”

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.