Glossary of Terms

Here are all the new words I've learned in the past 3 1/2 months.  There might be a pop quiz later. 😉

She's such a happy girl!

Achalasia-failure of smooth muscle fibers to relax, which can cause a sphincter to remain closed and fail to open when needed. Most often associated with cardiospasm of the lower esophageal sphincter, but the term is not exclusive.

Atresia-absence or abnormal narrowing of an opening or passage in the body

Aspirate-condition in which food, liquid, saliva, or vomit is breathed into the airway (this is bad!)  Can cause aspiration pneumonia.

Bolus-the mass of food or liquid in a single swallow. Also refers to the administration of food or medicine in a single dose or preparation given all at once.  "Bolus feeding" means administering food all at once rather than continuously. We still haven't been able to get to bolus feeding with Hannah.

Botox-Not just for plastic surgery. It's an injection that paralyzes a muscle but is only temporary, lasting about 3 months. We tried Botox for Hannah at 8 days old and it didn’t work 🙁

Bronchoscopy- an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes

CP-abbreviation for cricopharyngeas, the sphincter muscle at the top of the esophagus

Dilation- to make something bigger. Think "dilate" pupils at the eye doctor, or dilating during labor

Lexie just loves her baby sister!

Dysphagia- symptom described by difficulty swallowing (not to be confused with dysphasia, which is a speech disorder)

Endoscope- flexible tube with a light used to examine the digestive tract

Endoscopic- procedure using natural body openings and an endoscope

Enteral- involving or passing through the intestine, either naturally via the mouth and esophagus, or through an artificial opening. Any kind of tube feeding is considered enteral feeding.

Esophagram- When the upper GI tract is coated with barium, the radiologist is able to view and assess the anatomy and function of the esophagus, stomach and duodenum through fluoroscopy

Esophagoscapy-Using an endoscope inserted down the throat to examine the esophagus.

Fistula-an abnormal or surgically made passage between a hollow or tubular organ and the body surface, or between two hollow or tubular organs. When Hannah was first hospitalized, we thought she might have had a TEF (tracheoesophageal fistula, or a connection between trachea and esophagus)

Fluoroscopy- imaging technique that uses X-rays to obtain real-time moving images of the interior of an object

Laparoscopic- procedure using a small incision and an endoscope

LES-Lower esophageal sphincter

GI- gastrointestinal

Hospitalist- a dedicated in-patient physician who works exclusively in a hospital.

Hypertrophy-increased size of muscle. You've probably heard the antonym "atrophy" when a muscle has lost its strength

Manometry- a test to assess motor function of the upper esophageal sphincter, esophageal body and lower esophageal sphincter (but probably not for babies)

MBS-Modified Barium Swallow- a radiologic examination using fluoroscopy in order to evaluate swallowing function in mouth, throat, and upper esophagus, usually just referred to as a swallow study.  The test may evaluate ability of the patient to swallow liquids in a variety of thicknesses.  A Barium Swallow, on the other hand, focuses on the propulsion of liquid through the esophagus and into the stomach.

Motility-ability to move food spontaneously and actively through the digestive tract

Myotomy-surgical procedure to cut a muscle

NG tube-a nasogastric feeding tube going through the nose to the stomach for enteral feeding. Naso- refers to nose and -gastric refers to stomach.

NICU-Newborn intensive care unit

NKDA-abbreviation for "no known drug allergies"

NPO- Abbreviation for latin term nils por os, meaning "nothing by mouth" Hannah has been NPO since she was 6 days old.

OT-Occupational therapist

Owlet- baby monitor that wraps around baby's foot to monitor oxygen and heart rate. Gives parents peace of mind but doctors hate it

Oximeter- Device using infrared light to evaluate pulse rate and oxygen rate. Pulse oximetry is a noninvasive method for monitoring a person's oxygen saturation.

Peristalsis-series of involuntary wave-like muscular contractions that push food down the esophagus

Secretions- mucus and saliva. Anything your nose and mouth produce on their own that has to be swallowed

She actually hates tummy time.

SLP-Speech language pathologist. In our case, also a feeding therapist. Our SLP is helping Hannah maintain her oral skills and will help us teach Hannah to eat by mouth after she has her surgery.

UES-Upper esophageal sphincter

Polyhydramnios-excess of amniotic fluid during pregnancy. In our case, caused by baby's inability to swallow in utero

Stricture- narrowing of the esophagus (or other bodily passage)

Zebra-Shorthand for the aphorism coined in the late 1940s by Dr. Theodore Woodward, professor at the University of Maryland School of Medicine, who instructed his medical interns: "When you hear hoofbeats, think of horses not zebras". It means an obscure and rare diagnosis, also called fascinoma.

30 Seconds From Tears

Getting ready for her swallow study.

Today I'm 30 seconds from tears when I remember that Dr. Smith is going to do Hannah's surgery this month!

Since Hannah was diagnosed, I have pretty much been 30 seconds from tears at any moment. Do something nice for me, and in 30 seconds I'll be crying.  Remind me how tired I am, I'll cry.  Ask me how I'm really doing, I'll cry. Remind me how lucky we are that she's alive, I'll cry. Think about how long she might be on a feeding tube, I'll cry.  Watch her choke on her spit, I'll cry. Ask me how scary it was to see my newborn turn blue, and you guessed it, I'll cry.

But today I'm crying happy tears!  I went into our swallow study very skeptical that anything had changed in Hannah's esophagus, and I was prepared with a laundry list of reasons to use to persuade the doctor to do the myotomy sooner rather than later.  He walked in to the exam room and looked at her swallow study and said, "It looks like the stricture in her esophagus isn't getting any better. I think we should go ahead and do the myotomy."  Happy Dance!  Happy Tears!  I am so relieved!  Now I feel bad that I was questioning his concern for Hannah.  He's definitely in our corner!

She's smiling because the doc says
she can have surgery!

Hannah's swallow study looked just like the first two: pooling above the upper sphincter, with barely anything getting through. She also had some silent aspiration, meaning she had some liquid go down her trachea and didn't even cough. This is obviously dangerous; if she had aspirated at home, we would not have known it.  There was no aspiration during her last swallow study on December 18, which leads me to think that perhaps her cricopharyngeus has become more constricting over time, as opposed to potentially relaxing over time as has happened in a few other cases.

Dr. Smith will do the surgery through an incision on her neck, and it should take about 2 hours.  He said he'd want her to stay overnight in the hospital for 2 nights just to make sure she doesn't have any post-op infection and to make sure she can eat.  Recovery is about 2 weeks and then we will have a follow-up swallow study.  I've read studies where babies with CA started breastfeeding in the recovery room following a myotomy (and reading that made me cry, of course). While that would be amazingly wonderful, it is very likely that Hannah has lost the oral skills to breastfeed.  I will let myself be pleasantly surprised if she can breastfeed right away, or even at all.

Lexie loves holding her baby sister.
She walks around saying "Hannah
so cute" several times per day.

The SLP who did her study indicated that she has a very disorganized suck.  With the exception of 2 swallow studies, Hannah has not taken any food by mouth since she was 2 weeks old, so it wouldn't surprise me if she forgot how.  She's also been on an almost continuous feed that whole time. This presents a few challenges.  Hannah gets 644 mL per day (22 oz), over the course of 20-22 hours.  Her tummy is so small that I'm not sure she can take more than an ounce at a time. Bolus feeding by bottle might not deliver enough calories, so the NG tube will be left in place to make up the difference as needed.  Another complication is that she has been on an almost continuous feed for so long that she doesn't know hunger.  Not only will she be relearning how to suck/swallow/breathe, but she'll also have to learn hungry vs full and that we're not trying to starve her.  It might be just like having a newborn again!

When I started writing this post, I didn't have a surgery date.  I was told it would be either March 16 or March 23.  I just got a call that she is scheduled for March 16. And then I started crying happy tears again. 

Have I mentioned I am So Happy!!!!

Family Fast

Hannah's prayer hands.

We invite our friends and family to join us in a special fast for Hannah this coming Sunday, March 4.  Hannah's surgery date is still undetermined, and we are asking for prayers on behalf of her doctors and surgeons to choose the best course of action for her optimal health and recovery.

Prayers for Hannah's body to be strong and resilient to ward off infections or surgical complications, and to be responsive to medications and treatments.

We are asking for prayers for our family. We've had the sickest winter we've ever experienced, and it's especially scary with a vulnerable baby in the house. Two kids have had croup, we've had coughs and fevers and throwing up. Kids have missed school, I've had to cancel appointments, and Chris has had to use sick leave to stay home when I wasn't able to.

11 lb 1 oz today!

Prayers for mom and dad. This has been an incredibly stressful time for us. So far we have managed to keep ourselves healthy, but it is exhausting to run a household of seven under normal circumstances, and we are often overwhelmed and discouraged. Whether we have stayed sane is yet to be seen.

Prayers for momma.  I'm really hoping for good news on Monday of a surgery date soon (maybe in the next couple weeks?), but I should be praying and hoping for the best possible outcome for Hannah even if it takes longer than that. Perhaps what I need is greater faith in the Lord's timeline.  And if the doctor's plan and the Lord's plan don't match my plan, I need some serious courage and strength to accept it.

We feel very blessed by all the medical interventions that have been available to sustain Hannah and that she has been able to stay healthy over the past few months.  She has not taken food by mouth since she was 6 days old, and she is now over 3 months old, and is finally over eleven pounds. What a miracle!  And somehow this sweet little girl manages to be happy and smiley despite all her challenges and always having a tube through her nose and into her stomach.

Sweet big sister

The first Sunday of each month in the Church of Jesus Christ of Latter-Day Saints (Mormon church) is called Fast Sunday.  Church members refrain from food and drink for 2 consecutive meals and donate what would have been spent on food to the poor and needy. The purpose of fasting is to show humility to the Lord, to pray and meditate and repent, to seek spiritual guidance, and ask for needed blessings. The pangs of physical hunger remind us of our continual need for spiritual nourishment.

I'm not actually physically able to fast right now since I'm nursing (or rather, pumping), but I am planning to dedicate my day to scripture study, prayer and meditation, and I'm going to refrain from my favorite guilty pleasures of Mtn dew and sweets and idle time-wasting on my phone.  It's not quite the same as a physical fast, but it will be a sacrifice for me in a way that I'm able to make.

We are using this Sunday to direct our prayers towards Hannah's recovery and all who are involved in her care, and we invite you to join us in any way you are comfortable, whether it be prayer, fasting, cosmic thoughts and good vibes, or anything else you can think of. Since I wrote my post a couple days ago feeling discouraged about the surgery being a moving target, I've been gaining confidence that I might actually be able to present my reasoning to the doctor without bawling my eyes out, and that it might actually work, and that if he doesn't agree to do the surgery sooner rather than later, I'm going to find a surgeon who will.  This is me advocating for my child.

Dangling Carrot

But those eyelashes!

Finally March is tomorrow.  Hannah will see Dr. Johnson for a weight check on Friday, and will get the second dose of synagis. Next Monday (!) is her swallow study and follow up with Dr. Smith.  I am so glad that it is finally here! I feel like each day and week is an eternity of waiting.  She was 10 lbs 12 oz on Monday, so I'm confident that by next Monday she will have reached the 11 lb milestone Dr. Smith was waiting for.

The game plan when we last saw Dr. Smith was to look at her throat and see if there was any improvement. If so, we would start oral feeding.

Come inside Jenny's brain for a moment.
Why would Hannah's esophagus get better on its own?  In some cases, the cricopharyngeal (CP) bar went away on its own.  If the sample size is only 5, and 2 out of 5 got better on their own, that isn't very impressive.  If 3 out of 5 got better on their own, that's more than 50%.  But still not impressive with such a small pool of data. And I don't usually ever win stuff, so I'm going to say that the odds are not in our favor.  I hear Hannah try to swallow every day. She does 5 or 6 loud swallow attempts in a row, with huge confused eyes, followed by a big grunt and sigh. Because it doesn't work. So yeah, I don't think it got better on its own.

Dr. Lexie at your service

And if it didn't get better on its own, Dr. Smith said he would do the dilation and botox again.
Ok, last time we did that, the dilation worked for about a day. After a day she couldn't eat again, and we had to go back to the hospital and get a feeding tube. And then we had to wait three whole weeks to see if the botox ever started working.  If Dr. Smith sees that the swallow study is no different than before, he'll say, "Lets do dilation and botox next week." which puts us in the middle of March. Let's say the dilation also works for a day or not at all, and Hannah still has a feeding tube. Let's say Dr. Smith wants to wait three weeks again to see if the botox worked, that brings us to the beginning of April. Let's say we get to the beginning of April and do another swallow study, and Hannah's esophagus STILL doesn't work, THEN will Dr. Smith do the myotomy? 

In my mind the myotomy surgery is a dangling carrot that keeps moving away from us every time we move forward a month or a week or 2 kilograms.  Every step is followed by, "Check back in 3 weeks to see if it worked." I feel like it will never happen.  Maybe I'm wrong and it's not as far off as I think and I'm being unfair.  But I am just so tired and I am so worried and I want my baby to get better right now. Yesterday. A month ago.

Tummy time

On Valentine's day, we were looking for pyloric stenosis. If a baby has pyloric stenosis, they would have corrective surgery within a day or two of being diagnosed.  Pyloric stenosis is an enlarged sphincter muscle at the bottom of the stomach, and prevents food from going through. The procedure to correct it is called a pyloromyotomy. So really, pyloric stenosis is almost the exact same problem as Hannah's except in a different place, and has almost the exact same surgical fix. If babies have surgery immediately to correct pyloric stenosis so that they can take food by mouth and grow, why wouldn't they also correct a cricopharyngeal "stenosis" immediately? Her problem not only prevents her from eating, it also compromises her breathing, which some would argue is kind of important.  And likewise, do they ever wait six months to see if a baby's pyloric stenosis goes away on its own? I think not!  This doesn't seem fair.  Just because ours is more rare, we get half a dozen swallow studies and months on a feeding tube, and so much waiting and wondering and worrying.  He only sees us and thinks about us once a month, but we do this every day and I think about that 3-weeks away appointment Every Day.

Every day and week and month that she is on a feeding tube she loses oral stimulation.  She loses elasticity in her stomach.  She has no hunger cues. She can't eat at the pace and volume she wants to set, that her body tells her to. She is 3 months old and should have tummy time every day and start pushing up and rolling over.  All of our babies have started crawling around 6 months old. How is she going to be mobile with a feeding tube in her nose? She would pull it out constantly.

The long-term alternative to an NG tube is a G-Tube, which is a tube through the abdominal wall directly into the stomach. Babies who still need enteral feeding after becoming mobile generally get a G-Tube that is easier to maintain than an NG tube. But it has to be surgically placed and requires general anesthesia or deep sedation. If we are withholding the myotomy surgery because of the risks of surgery and anesthesia to an itty bitty baby, it would seem counter-intuitive to surgically place a G-tube rather than to just do the dang surgery already and eliminate the need for tube feeding.

Every time she coughs and doesn't quite clear everything in her throat, I worry she is going to aspirate to her lungs or stop breathing. Every time she sputters up white foamy mucus and saliva, she chokes and screams, and I jolt out of bed, hold her upright and make sure she isn't turning blue.  Every time I lay her flat for a diaper change or for tummy time I worry that she will choke on her own spit.  Every time we leave the house or I hear someone sneeze I worry she will get sick again.

Oh yeah, that reminds me that Dr. Smith mentioned that he wouldn't do throat surgery if the baby has had a respiratory infection within six weeks because surgery is riskier if the throat is already irritated or inflamed. So here we are, sitting on our hands during an incredibly dangerous winter hoping that this baby doesn't get sick again, and if she does it will add at least 6 more weeks to the timeline I mentioned above.  Again, see the part about the baby needing to become mobile in the near future...

Its also inconvenient for me in a whole bunch of other ways, but I'm trying to focus on why surgery NOW is best for Hannah and not just me.

Maybe I will tell Dr. Smith everything I've just told you and he'll say "You're right! Lets do surgery tomorrow!" but I highly doubt it.

Still manages to smile even with
a tube in her nose!

Never a Dull Moment

Last Wednesday, Hannah had an ultrasound of her pyloric muscle to determine if she has pyloric stenosis. The ultrasound was rather fuzzy and we couldn't get a very clear picture, but a radiology consultant said "It's probably fine" and that was that.  I think we are still going to get a follow-up GI study done in the near future, because "probably fine" isn't super definitive in my opinion. We also had her nutrition levels checked, called a prealbumin blood test. It checks protein and nutrient levels, as well as organ functions. Gratefully, the test showed normal function and nutrition aside from low iron. We're going to give her an iron supplement and continue with her established feeding regimen.

I didn't take enough pictures this week

This Wednesday I was concerned that Hannah might be getting sick. She has a really wet cough and since her cough isn't very productive, I always worry she will aspirate to her lungs. There is a Facebook group, albeit small (about 20), of parents with babies who have CA, and many of them had aspiration pnemonia multiple times. Hannah does have some wheezing on both sides of her lungs but the doctor said it's not aspiration, and Hannah tested negative for RSV (Hallelujah!!)  We also got a one-week standing order for the suction clinic, which provides BBG suctioning outpatient at the hospital.  If I'm not able to clear her nose and throat with a bulb syringe or my NoseFrida, that is a logical next option for us. We went there once about a month ago, and it was very helpful. (PS The NoseFrida is awesome!) Her oxygen was excellent and she does not have lung retractions or a fever.  As long as she stays that way, we'll be OK. A respiratory infection will most likely delay her surgery by a month or more when the time comes.  It's so hard to keep the sicknesses at bay in a house full of people!  I have 3 kids in public school and a two-year old thumb-sucker, so who knows what germs are lurking around in here!

I had to un-sew Hannah's sleeves so she can flex her hands rather than make a fist constantly, and so that she can explore putting her hands in her mouth. I get serious anxiety whenever I think she's about to pull her NG tube out or the tape is coming loose. We are getting good at putting it back in but it is stressful for me and Hannah.  We probably have to put it back in and re-tape her face about once a week.  She is steadily gaining weight while getting 27 mL/hour through her NG tube. I am trying to go up incrementally, but she's picky about it, and her wet cough showed up right when I tried to go to 28 mL, so I went back 2 days later. I'll try again soon. 

Only one in diapers...that's a relief.

I gotta say, five kids is really busy and a lot of work.  And so loud. All my peeps want dinner every single night (the nerve!) but often complain about what is served, dishes are constantly piling up, laundry is an endless, eternal pain in the neck, and caregiving is exhausting. They have homework, church and school activities, I have a weeknight church assignment, we have several doctors appointments each week. We all have emotional needs. I'm pumping 4 times a day.  Hannah wants to be held constantly and seems to wake up the moment my head hits the pillow for a power nap. Last weekend I was pretty much running the show solo while Chris recovered from a minor surgical procedure, and I was also potty training Lexie, finally having a breakthrough on day seven.  (5 kids is plenty...I'll let you conclude what kind of surgery he had).  And did I mention the sheer volume of laundry? 

Our 2005 little red Honda is on its last legs (I guess cars don't have legs), and so Chris has been riding the train to and from Draper and leaving the car at the Draper train station to only go the 5 miles to and from his office. So rather than 250 miles a week commuting, he drives 50 miles. We just have to make that car last a little longer until we can replace it. But this means a longer commute, leaving earlier in the morning and getting home later, plus only one car at home to get us all those places I mentioned. 

I think its funny that they each have a
facial apparatus and tube of some sort. Haha!

I know they are Worth It and this is what I signed up for, but being Busy with a capital B is really hard. I have a to-do list ten miles long and some of it will probably never get done. I'm self-medicating with snickers bars and Mountain Dew.  Lets me honest, this momma needs therapy and anti-anxiety meds, and that's two more appointments to keep.  I wish I had more time to read, but whenever I have down time it's something mindless like Facebook, Netflix, or a game on my phone. I realized this morning that Leah will be a senior in High School when Hannah starts kindergarten.  This circus show is only getting started!  I keep thinking it should be getting easier to manage, but its only going to get harder and it will be like this for a long time even when Hannah is better.

I need a serious change of heart and mind, because I'm going to be doing this for many years to come, with increasingly busy kids with new and varied challenges. I have to figure out how to do this with a better attitude and more patience, more organization, etc. 

At least somebody likes the snow...

So far I have a few strategies for dealing with everything.  I have a notebook with all of Hannah's health information, growth progress, feeding changes, doctors appointments, phone numbers, and more... Doctors always seems surprised or impressed when they see me taking notes or referring to my notebook. I have a really good, no-fold laundry sorting set-up (which works great when I actually get around to it).  The kids are expected to put away their own clean, sorted laundry and help with taking out garbage and loading and unloading the dishwasher.  I'm recommitting to menu-planning to save on groceries and shopping frequency. Leah and Samuel are old enough to babysit the other kids and a sleeping Hannah if I need to run a quick errand. Chris is using FMLA leave a few morning hours per week in order to help get the kids out the door and allow me time for a shower.  I'm praying more (did you know that you can pray that a two-year old will pee in the potty and then she will?). I'm going to therapy and taking anti-depressants. I try to get out of the house for girls game night or a $1 movie. I got a cute new haircut.

I always feel like I need to end my posts on a positive note, so here goes. Hannah is healthy and growing. The other kids are (mostly) staying healthy and progressing in their respective spheres, whether it is a science test, times tables, reading new sight words, or going potty and moving to a big-girl bed. We are trying to still have meaningful experiences with them. Chris and I are re-learning how to manage our hectic household, although probably not very gracefully.  We have been incredibly blessed by friends and family and strangers, and countless prayers. My sister's homeschooling Co-op had a bake sale and decided to donate their proceeds to our YouCaring fundraiser, and they are in Illinois! My friend Stephanie donated her time and talents to take our family portraits. Ward members and friends have been babysitting and providing dinners and even helping clean my house as needed (a Ward is what we call our local church congregation).

Although fortunately no one has said this to me, I've often heard people say of someone else's trying circumstances "I could never do that" or "I don't know how you do it" and what I really think it means is "I don't want to have to do that" or "I'm glad I don't have to." And to be honest, I really wish I didn't have to either, but I tried not to ever say or think it because I knew in my heart that if I needed to I would Rise to the challenge and become a Warrior for my child.  And here I am clumsily working on my Warrior badge.

Shoot.  Someone just threw up. Gotta go.