Our daughter Hannah was born 11/22/17. She's our fifth baby and a welcome surprise caboose for our family. At 6 days old she was diagnosed with a rare condition called cricopharyngeal achalasia, also known as cricopharyngeal hypertrophy, bar, or narrowing. It is a congenital defect involving the upper esophageal sphincter muscle that is too large and impairs the swallowing process, kind of like a pinch in a hose.

At 8 days old Hannah underwent a procedure to dilate her esophagus and reduce the muscle with Botox injections, but unfortunately, the procedure was unsuccessful. On March 16 she had a myotomy of the muscle, and she is now able to swallow. After 4 months on a feeding tube, she is finally able to eat by mouth.

This is her story that is still being written.

Tuesday, January 9, 2018

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.

1 comment:

  1. Hindsight. You may recall I had some pregnancy drama with Sammys pregnancy (total bed rest for 4 months, etc). In hindsight, a birth defect saved his life. He had a 4 inch long umbilical cord. He could never have been born vaginally. And thats the only reason I didnt have a miscarriage on my family room floor when the drama first started 4 months before he was born. Instead I passed out. AND IT SAVED HIS LIFE. So, yeah...hindsight.
    Also, I didn't know I had West Nile Virus this summer until the worst, most scary neurological symptoms were past, so I totally get not wanting to know while it was happening because it would have just been scarier.
    Anyway, good attitude and perspective. Just keep loving the heck out of that little doll. We're praying for you all.

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