Them bones, them bones, them jaw bones

When Mr. A was born, nothing was said about his jaw. It was brought up later, when discussing Pierre-Robin Sequence (PRS), but no one could decide whether he had it or not. So, not much fuss was raised over his probable micrognathia. When he was just over 2 weeks old, he was transferred to the children’s hospital, where the doctors went about trying to find every possible thing wrong with my son. (No, really. Name an organ or system, and they examined it, usually multiple times, usually with high-tech equipment. I grew to hate the neonatologists and the NPs as well, as they never had any answers or seemed to want to “fix” anything, just to order more and more studies.)

Luck of the draw gave us Dr. M as an ENT–he was on-call for NICU the day they decided to delve into “dysmorphic facial features”. He offered an opinion on Mr. A’s jaw: micrognathia, but not very severe. He gave A his first (of many) bed-side scope, and said his tongue did fall back, but not so much that he was worried about it. He recommended side-lying, which we were already doing for reflux, and said he’d talk to us again after the sleep study. At this point, Mr. A had been on room air for about a week. The sleep study revealed both central and obstructive apnea, and they decided to put him back on O2 (just 1/8 of a liter, and we were discharged on 1/16) as he was dipping down into the mid- to high-eighties more than they’d like. Dr. M came by as promised, and said he didn’t think it was A’s tongue causing the obstruction, but perhaps something else in his airway, and said he would like to do a rigid broncoscopy when A had surgery to fix his intestines and insert his G-tube.

When your child is operated upon by multiple surgeons during one “go”, each surgeon comes out to talk to you after finishing their part. And so it was, that on A’s one-month birthday, Dr. M told us he didn’t really see anything to explain the obstructive apnea. He said his trachea was slightly narrow (but qualified this with, “and he is still very small”–true both in terms of weight (about 4.5 pounds) and age (still should be in utero)), his larynx might be small (again, same modifiers apply), but that he didn’t really know. It certainly didn’t seem like his jaw was to blame, and so jaw surgery was taken off the table. When the days passed and they were unable to extubate A, Dr. M scoped him again, and again offered the same opinion, but did say he would consider it. Luckily, we were able to get him back on the nasal cannula and airway fears were swallowed up with fears that his intestines would never work again. (One charming neonatologist even informed me that “sometimes kids with syndromes just get worse, so…” despite the fact that non-functional GI tracts are not in any of the (sparse) literature regarding A’s chromosomal deletion.) (His intestines just needed some time, and work just fine, thankyouverymuch.)

Fast-forward 3 months, to Mr. A in the PICU. He had had a Nissen, hernia repair, and ear-tubes (hi, Dr. M! Hi, 2nd surgical scope!), and was once again proving difficult to extubate. Dr. M gave him at least 2 scopes. He also developed the habit of popping by to say hi, particularly when there was going to be an extubation attempt. Dr. M was there when he was finally successfully extubated, and helped with suggestions of how to encourage A to breathe (positions, nasal trumpets, etc.) And when A was still on BiPAP (and high settings at that), and everyone wanted to trach him, Dr. M stopped by again, for another scope and a discussion.

This scope is the one that is the deal-maker, by the way. I watched him examine every angle–in deeper, shallower, left, right, all the while telling the nurse to thrust his jaw forward, release, thrust, release. The “thrust” mimics jaw distraction–giving more room in the lower jaw where the tongue can go, instead of falling back. But Dr. M did not see any difference whatsoever in the thrusted vs. relaxed positions. He told me he did not think jaw surgery would help A. He said his jaw really wasn’t that small, and didn’t seem to affect his tongue, which didn’t seem to be causing the problem.

This was probably day 28 or 29 of what should have been a 3-4 day stay. I had been told all sorts of dire things about my son. One charming attending told me, when I asked him what exactly he meant by saying he was “worried” about Mr. A, that: “The sickest children in 6 states come to this hospital. The sickest of those are in the PICU. So when I say I’m worried about him, I mean he is the sickest of the sickest of the sickest.” Thanks, asshole, but what I really meant was: are you telling me you think he will die. Anyway, suffice to say I was a wreck. I liked one fellow an awful lot, and had explained to him how A grew tachypneic when he was angry or upset. When they would attempt to wean him from BiPAP, they always turned things back up because his respiratory rate would sky-rocket. That’s because, on CPAP, you’re allowed to control your own rate of breathing, whereas on BiPAP, you’re really not, very much. They would tell me that he would never be able to sustain that rate, and turn things back up, as Mr. A had a sign declaring “CRITICAL AIRWAY” over his bed. (He’s hard to intubate, and they didn’t want to do an emergency intubation. Though some of those trach-happy residents probably did…) But I knew he was just mad. I mean, he developed a pressure sore from the BiPAP mask that I pointed out, and that you can still see, almost 8 months later (though much, much less obvious). He was in pain. He was mad. He was detoxing from the extremely high doses of sedatives he’d been on while intubated. Oh, and he had fought off ventilator-aquired pneumonia and central-line-aquired bacterimea.

I wasn’t able to properly advocate for A, and I’ll never forgive myself for it. But Dr. M, Fellow G, and I worked out a plan. We would risk the emergency intubation and just go to CPAP until he started to desat. We would give it the night. If things didn’t look stable, we would do jaw surgery in hopes of avoiding a trach. Dr. M was largely responsible for getting the attending to agree to this plan.

And by morning, A was on minimal levels of CPAP, ready for highflow. Less than a week later, he was home.

So, obviously, when we had our pre-surgery consultation in January, I was anxious. I wanted to be sure this stay won’t be like the last (surgical) one. We worked up an excellent extubation plan, and I once again asked about his jaw. Dr. M said he was extremely glad we hadn’t done the surgery, and that A’s jaw had done a lot of catch-up growth, and he no longer considered it clinically significant. He said it might not totally catch up, but that would be a cosmetic issue we could address after (or during) puberty if we desired.

So I thought that was the end of the jaw.

Ha.

This tale is dragging. Suffice to say, Dr. M strongly disagrees with Dr. S. We’re siding with Dr. M, as he knows A’s airway better and has looked and looked and looked at the jaw issue. I’m nervous we’re making the wrong decision, but the surgery is extremely painful and required 2 separate operations, plus then the palate repair, and I just don’t want to subject A to more pain and suffering and put off repairing his palate (and thus ending the constant food/vomit/everything-in-sinuses and maybe, possibly, hopefully, help him learn to speak) any longer.

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1 Comment

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One response to “Them bones, them bones, them jaw bones

  1. Anne D.

    I hate that these things aren’t just black and white for parents. I am sure you will make the right decision. You know Mr. A the best and it sounds like Dr. M knows him well, too. Hugs, Mama!

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