Our Crazy Day!

Well, Luke and I are home, and he is doing well. Again, after much preparation and worry, Luke didn't have the Laryngotracheal Reconstruction (LTR). This is probably good news. As many of you know, Luke doesn't just have one airway issue, but many. He was trached at 6 days old because of his choanal atresia (CA) (his nose was completely blocked by bone). Then later it was discovered that he had tracheomalacia and laryngomalacia (floppiness of his trachea and larynx) and subglottic stenosis (a narrowing of his airway just below the glottic region). Since he already had the trach it was hard to know how much each of these issues affected his breathing because the trach bypassed them. So first, Luke's ENT, Dr. H., has been working on Luke's nose, which Dr. H. described as the worst case of CA he has ever treated. Luke has had more than a dozen surgeries on his nose - 2 major, the rest one-day. The good news today is that Luke's nose has stayed open and looks better than it ever has. We may finally be close to a resolution of that issue. Dr. H. did go ahead and dilate Luke's nose today since he was already in the OR.

So then we look at what else may be causing Luke's difficulty breathing. Until today every time Dr. H. has seen Luke's airway it was red and inflamed. Today there was some irritation, but it looked better than ever before. And with the reduction of inflammation Luke's subglottic stenosis was much better. It would still be on the small side, but not small enough for Luke to need the LTR to make his airway bigger. Another reason to have an LTR is if there is a collapse in the trachea caused by having a trach. This is what I thought happened when Luke's decannulation attempt went so badly. But Dr. H. didn't find any significant collapse when he did the scope today. So, as far as I understand, unless something new happens, Luke won't have an LTR at all.

If there isn't a collapse then we have to think about which of Luke's other issues could have caused the decan to fail the way it did. And we are left with the laryngomalacia (LM). Most of the time kids are able to outgrow LM without the need for a surgical repair or a trach. But when LM is severe the epiglottis can contract and cut off the airway. This wouldn't be constant and would be worse on the inhalation than the exhalation. Times it would be worst would be when upset and when lying on your back. With Luke it would explain why when he started crying hard during the decan his airway closed off (looking like a collapse), why when he is capped he does fine when he is awake (except when he is crying and obviously can't catch his breath), but when he is asleep (on his back) he has a noticeable occlusion - he can wear his PMV while asleep though with no problems (with this he is breathing in through his trach, rather than his mouth). LM is associated with aspiration, reflux and poor weight gain. Kids usually outgrow it by 24 months. Luke is 35 months and hasn't outgrown it yet. The surgical repair of LM is supraglottoplasty. It involves taking away some of the excess (floppy) tissue of the epiglottis. And this is what Dr. H. would like to do on Luke. It is a quick surgery and fairly common. He would probably come home the same day. We would then cap for a few weeks and try to decannulate again. The problem is that once the tissue is taken away it can never be added back again. And with kids who aspirate it can worsen aspiration permanently. If aspiration is bad enough, you cannot eat by mouth and you may have to have a trach to be able to suction the aspirated fluids. This is what makes this simple surgery a not so simple decision. Luke's last swallow study was last Spring. At that time he was still aspirating, but we don't know now if he is or not. We have scheduled a swallow study for Monday afternoon, and depending on the results of that, we will be able to weigh the risks of surgery for Luke. If they seem too great, then we will probably wait, keep the trach, do repeat swallow studies and see if either the aspiration improves or the LM does. If we decide on the surgery it should be scheduled for the end of next week.

If anyone has any insights or advice we would love to hear it.