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!