Weight Check

All of our babies have been tall and skinny in their first 2 years-around the 75th percentile for height and 25th percentile for weight.  Hannah was fairly big at birth (8 lb 12 oz), but her growth took a serious hit with three hospitalizations and two respiratory viruses before she was six weeks old.  She finally regained her birthweight at six weeks, and was growing fairly steadily for a few weeks.  The goal is to gain about an ounce a day, and Dr. Smith wants her to be at least 11 pounds before doing the myotomy surgery.

On January 12 she was 9 lb 7 oz.  But at her checkup on January 22, she weighed 9 lb 9 oz, which was only 2 ounces in 10 days, and on February 5 she was 9 lb 12 oz. So while she had been growing an ounce a day for a while, she had now only gained 5 ounces in 3 weeks. 😱  At birth she was in the 50th percentile, and now she's in the 5th percentile! This is obviously very concerning.

Hannah has a really hard time whenever we try to increase the amount she gets in her tummy.  She gets really fussy and starts to regurgitate when we go up even 2 mL/hour.  It almost seems like she's in pain.  She gets a really wet cough, and there is the concern that she will aspirate into her lungs; aspiration pneumonia is not uncommon in babies with CA. Whenever we increase by 1 mL, I have to give her an entire day to adjust to the higher volume.  Mind you, 1 mL is a fifth of a TEASPOON!  This is why she has been getting a continuous feed 24 hours a day rather than bolus feeding, which more closely mimics natural eating habits like getting a 3 ounce bottle over a 15-20 minute period every few hours.

He just can't make a normal face!

Last month the doctor and I were working on trying to consolidate Hannah's feeding schedule away from 24 hours a day.  So in mid-January, I stopped Hannah's food pump for one hour a day and increased the volume on the other 23 hours so that she would still get the same total calories per day. I decided to eliminate the 2am hour, hoping she wouldn't even notice.  We did this for 3 1/2 weeks, increasing her hourly volume and adding 1 off-hour each Friday.  We were down to 20 hours a day at 25 mL per hour with breaks at 2am, 8am, 2pm, and 8pm. We did make the mistake of not increasing her daily caloric intake in that time, which is why she stopped gaining weight. So since her weight plateaued, on February 5th we went back to 24 hours a day and increased the volume to 27 mL/hour.  Now her total volume per day is almost 650 mL rather than 500.  Within a week she had already started gaining an ounce a day again. What a relief!

She's such a snuggle bug

This week, Dr. Henderson called me and recommended that I take Hannah to another pediatrician who has more experience with special cases like Hannah's.  Dr. Henderson has been incredibly helpful. First of all, he took me seriously on the Day Everything Hit The Fan (November 28) and got Hannah admitted to the hospital.  Every time I had nursed Hannah and she had turned purple, I got this panicky feeling that something was wrong.  It gave me anxiety and I was starting to dread feeding time because it was so scary for me to see her stop breathing.  At the same time, when she wasn't eating, she was perfectly fine, so I was puzzled and unsure of how seriously I thought it should be addressed.  Surely, she doesn't need to be hospitalized!  The hospital is for babies who are sick, and Hannah wasn't sick.  The first doctor told me it was nothing but reflux, but Dr. Henderson saw the warning signs that something more was wrong and escalated her case.  At the time I thought the hospital was going to be overkill and I was going to be embarrassed that nothing was wrong.  I'm so glad Dr. Henderson took me seriously and followed his instincts.  He very well could have saved her life. Dr. Henderson also does shifts at the hospital where we've been several times and was proactive about getting us the Synagis shot.  He's friendly and personable and has answered all my questions or consulted with other doctors and specialists if he didn't know the answer.  It has been nice to have the continuity of care, but I appreciate that he recognizes our case might be out of his scope. So while it is hard to switch doctors and say goodbye, I think it will be a good move.

We got in to see Dr. Johnson the very next day (yesterday).  I had made an appointment to see him right after New Year's, but had cancelled it in order to keep our continuity with Dr. Henderson.  So Dr. Johnson had already read Hannah's chart and knew all about her.  Right now the primary concern is getting Hannah to grow, and this involves finding the right feeding regimen.  Most normal babies are self-directed in how much food they take in.  They cry when they are hungry and eat until they are full, and it is just right for growing. Oh, how I miss this dynamic!  My baby gets fed constantly, a tiny bit at a time, and I have no way of knowing if she is getting enough or not.  I went to see Dr. Johnson with a list of questions about calories, ideal growth, bowel movements, and transitioning to bolus feeding, and he addressed all my questions before I even asked them!

All the kiddos.  I think they like her.

It was really good to learn the "formula" of how many calories she needs in order to gain weight and catch up: 110 calories per kg of weight. Now I can adjust her daily calories without having to ask a doctor how. I suppose I could have googled it, but I didn't think to. Dr. Johnson also ordered some blood work to check that all her internal systems are working properly and that her nutrition levels are balanced.  With regards to not being able to increase her hourly volume very quickly and without discomfort, he had a couple recommendations.  First, Hannah gets breastmilk with a little formula mixed in for added calories, and he switched us to a new formula that is easier to digest. Secondly, he prescribed a medication, Reglan, that helps the gut digest faster.  Hopefully these two changes will help her stomach empty a little faster and allow us to increase the hourly volume faster. Our first goal is to get to 32 mL an hour for 20 hours a day. The second goal will be to have 2 hours on and 1 hour off throughout the day, which is more like 41 mL/hour for 16 hours, but probably higher because she will weigh more by the time we get to that point.

Third, he ordered an ultrasound to check Hannah's pyloric muscle, at the bottom of her stomach. Babies who have pyloric stenosis have projectile vomiting as the main symptom. But Hannah doesn't ever have a "full" belly, and the cricopharyngeal bar blocks most of her reflux from coming up: she hardly ever spits up at all. So it is possible that she does have pyloric stenosis without that tell-tale symptom, and it would explain why she can't increase volume without significant discomfort. Incidentally, pyloric stenosis is an overly large sphincter muscle, just like her overly enlarged upper esophageal sphincter, and the surgery to correct it is also called a myotomy, a pyloromyotomy. It seems not a coincidence to me if she were to have both. Maybe she has some kind of disorder where her sphincter muscles are too big.  My mom says birth defects come in threes.

I put on pants! Survival mode here.
Celebrating the little things, people...

If she does have pyloric stenosis, she would have immediate surgery to correct it: same day or next day.  Our surgeon, Dr. Smith is hesitant to do Hannah's myotomy until she is bigger because of the risks associated with anesthesia.  But if we were going to be anesthetizing her to do the pyloric correction, couldn't we also do her cricopharyngeal myotomy while we're at it? I asked Dr. Johnson as much and he said yes and said he would call Dr. Schramm in Provo for his help.  I like that he is on the same wavelength as me: he seems to agree that the myotomy is Hannah's best option and had asked why we hadn't done it yet. Well, this was kind of exciting news for me and also a little overwhelming: Here it was 1:00pm on Valentine's day, and my kids are almost home from school.  I'm about to go get an ultrasound that could determine if we have surgery in the next 24-48 hours, surgery that I have been hoping for for months. And also, it would mean another chaotic balancing act while we have a hospital stay. Do I go home and pack a bag just in case I don't go back home? And oh, yeah, I've been trying to potty train Lexie this week (two year olds are so stubborn!).

Unfortunately, the ultrasound was inconclusive.  After trying for the better part of two hours, we were not able to get a good shot of the pyloric area with food movement, partly because the volume in Hannah's stomach is so small, partly because breastmilk is harder to see on an ultrasound, and partly because she had a lot of gas bubbles in her stomach and was really fussy.  (This baby does not burp!) So for a few hours I had an unsettled eagerness and anxiety that we might have imminent surgery, and then a huge let down when we didn't have an answer at all.  Talk about a roller-coaster! The next step will be to do a full study of the lower stomach and duodenum to put to rest or confirm the theory of pyloric stenosis.  We've already had various tests to look at her anatomy, but nothing below the stomach. I'm on pins and needles waiting for a call to go to radiology today, or maybe it won't happen until next week.

In all, I'm glad we have Dr. Johnson and that he has the same concerns as me and some new answers for us.  I'm glad he is up to speed on Hannah's condition and is proactive about testing. Hannah has enough calories and I have a game plan to keep her growing steadily.  And I'm preparing myself that any day could turn out completely different than how it starts.