Simon medical, Mama’s take

Todd and I have been meaning to post something like this for a while now, explaining Simon’s current and historical health situation to any and all who may be interested. The question was who should write it, and in what spare time. I guess I win? Todd promises to give his take at some point.

Let me start by saying that apart from having some medical concerns, Simon is a Truly Exemplary Child, in Practically Every Way. He is an excellent sleeper (quote from the pediatrician), he loves sitting still on my lap and watching things. He loves books and will sit through several Dr. Seuss tomes without even wiggling. He is happy to be held by anyone, family or stranger, and gives out full-face smiles with flirtatious regularity. The only reasons he cries (if you can call it that) are hunger and fatigue, which are fairly easy to recognize and ameliorate. Basically, tempermentally, I have the Best Baby Ever. Sorry if that makes you jealous. Actually, not really.

After Simon’s newborn exam in the hospital, the attending pediatrician let us know that he had been born with a cleft palate and Pierre Robin Sequence, which began a cascade of medical interventions and treatments, as well as
substantial parental education. At the time, I wasn’t sure exactly what a cleft palate was, so I will attempt to share what I’ve learned with you as well, Dear Reader.

At some point in the first trimester, the left and right sides of the hard and soft palate fuse along the midline. When this doesn’t happen, a hole is left, extending from some point behind the teeth toward the back. Simon’s cleft is bilateral (on both sides of the midline), and rather wide. We’re told it lies more to one side than the other, but not being a professional, I can’t really see it.

When we look in his mouth, instead of seeing the roof of his mouth, we see up into his sinus cavity. Now, in thirty years of life, it never occurred to me to stop and ponder: I wonder what’s inside my face and how it all fits together. Now I sort of know, but it’s really hard to describe, and you don’t really want me to. Part of the inside of your face is kind of lumpy and foldy and weird. When he was a little bitty guy, he had these strange lumps of flesh behind his upper gums in the back: his uvula (which was split in two). As he’s grown and his mouth has changed shape, these have been pressed toward each other more, so that the cleft appears a bit narrower. Ultimately, Simon will need a surgery (palatoplasty) to repair the cleft.

One challenge for me early on was that Simon’s cleft makes it impossible for him to suck since his mouth and nose are connected through an extra hole. It would be like our trying to suck through a straw that has a hole in it. Before Simon was born, I had been planning to breastfeed him, and it was kind of a shock to be told by a lactation consultant that he would never be able to nurse for nutrition. Looking back, it was the best thing someone could have told me, since it took away the pressure of trying to get my child to do something he wasn’t physically capable of doing. At the time, however, it was really difficult. I pumped milk for him for about two months, and fed it to him with the special “Haberman Adaptive Feeder.” He still uses the Haberman, but we’ve switched to formula for a variety of reasons. It hurt my feelings a little that he didn’t seem to notice the difference between milk and formula, but, well, whatever.

There can be lots of different reasons that a baby is born with a cleft palate, but missing from this list is “being a bad and negligent mother,” which took me some time to appreciate. After all, I had spent 40 weeks (and five extra days!) growing this child in my body, so it seemed natural that any deficiency in his physiology could be wholly attributed to what I had put into my body, what position I had slept in, how much stress I may have been under, had I worried too much about something, not had the right vitamin, not listened to enough Beethoven, exercised enough, or was it the two ounces of wine I had in February? No. No, no, no. Sometimes, it just happens. It took me about three months to believe that.

In Simon’s case, the cleft appears to be due to the Pierre Robin Sequence (PRS), where for some reason (genetic or as some consequence of intrauterine environment), the growth of the lower jaw is restricted, which causes the tongue to be thrust upward and backward in the mouth, which
prevents the palate from closing.

One big question that had to be addressed was whether Simon’s PRS was caused by some underlying genetic syndrome, or whether it was just a fluke. So, we saw a geneticist, who recommended this and that screening, which all came back negative (with the caveat of a frustratingly high false negative rate). As far as we can tell, he is a “normal XY male.” The screenings did not mention his status as a Truly Exemplary Child.

Simon’s small jaw caused him to have a lot of breathing difficulties early on. The same reason that he had the cleft in the first place (the tongue pushing up and back into his mouth) caused some breathing obstruction when he was lying on his back, the recommended sleeping position for babies. He often struggled to get air in, which made Todd and me freak out pretty much all the
time. He sleeps on his tummy now to allow his tongue to be pulled forward by gravity, but as he’s grown, he’s gotten better at positioning himself in a way that allows him to breathe freely. We also think (but can’t verify it quantitatively) that his jaw is growing, which is our dearest hope, since it would pull his tongue forward to where it’s supposed to be.

One concern with the breathing difficulties that we hadn’t anticipated involved weight gain. Simon was spending a lot more calories breathing than a typical baby would. Plus, genetically, he was predisposed to being really tall and skinny. So his doctors were concerned that he wasn’t getting enough calories to maintain a healthy weight (he was until very recently off the bottom of the chart in “weight for length” despite being off the top of the chart in length). On top of this, he developed acid reflux, making eating a crying, screaming challenge. Thankfully, this is all under control now thanks to infant-dose Zantac and high-calorie-density food. He’s plumping up quite nicely, something neither Todd nor I did until we were in college, if we can even say that.

Right now, we’re looking at one, maybe two, maybe three surgeries for Simon. The most obvious one is the palate repair, which is normally done around twelve months of age, but may be pushed out to fifteen to eighteen months because the surgeon wants to be sure his jaw has grown enough to allow a nice clear airway before doing the surgery.

Most children with a cleft palate also have ear tubes placed to allow fluid to drain out of the middle ear. The muscle that allows us to open and close our Eustachian tubes is connected across the palate (so Simon’s doesn’t connect to anything). As a result, fluid is collecting in his middle ear, which is harmless at the moment (but increases the chances of developing ear infections) except that it seems to be impairing his hearing a bit. The surgeons usually do that at the time of palate repair, but Simon’s ear tubes might be placed in a separate surgery if indeed his palate repair is done later. Overall, no biggie except for the general anesthesia.

The surgery that Simon’s surgeons are trying to decide if he needs, and the one that is causing his mama the most anxiety, is the one that would lengthen his lower jaw, pulling the jaw and tongue forward, fixing the breathing obstructions. We (and I really do mean “we”) have completed two sleep studies to gather data to evaluate whether this surgery is necessary at this time. The first one was inconclusive, the second one better, and we’re waiting for the follow-up appointment with the sleep doctor to give us the interpretation of the results. My guess is that they’ll tell us Simon is doing “okay” but that we need to keep an eye on him.

All this to say: Simon has some special medical needs, but mostly I don’t even think about it because he is doing so well in every other way. He’s charming and smiley and likes to read books and be thrust directly upward into the air to play Flying Boy. Since he’s our first child, I don’t have any real point of comparison, nor do I have reason to stop to think that not all babies need the special Haberman bottle, nor do they daily spit up some food through their noses, or have monthly meetings with the cleft palate team or sleep studies out the wazoo.

He just seems normal. And wonderful and snuggly and Very Bright at Math. And I’m really thankful for that.