Out Again

Since my last post dealt with Trust landing himself back in the Peds unit at St. Joes, I thought I could write a follow-up post dealing with the outcome.

An few interesting developments came out of this latest visit. First, we may have a name for what's going on with Trust's throat and there may be a fairly quick solution for it. Second, our hospital has been bought by another in the area and the brand-new head of the pediatric department has shown some interest in an idea Dad's been developing for the last eight hospital visits.

In a nutshell, Trust strongly appears to have congenital epiglottic stenosis heightened by repeated intubations. Attached article should be a pretty concise description, but what that means is that the part of his throat just below his vocal chords seems to have a very narrow part. It could be narrow because his throat is formed in an oval instead of round, or the bones around it are too close together, or there is a membrane webbing partly covering it. Or a combination of the above. This causes his airway there to be so much smaller than usual that any sore throat with a little bit of swelling more or less closes his throat up. It doesn't take very much to severely cut down on throat space, especially since he's so little so there isn't much space there in the first place.

Congenital Subglottic Stenosis

Congenital subglottic stenosis is the third most common congenital anomaly of the larynx. It accounts for 15% of all cases. Males are affected twice as often as females (5). Congenital subglottic stenosis is usually associated with a small or malformed cricoid cartilage with or without thickening of the underlying submucous layer.

Patients with subglottic stenosis may be asymptomatic until an upper respiratory infection causes further narrowing of the airway. The patient may present with biphasic stridor and a barking cough may be noted. Many patients are diagnosed with recurrent croup prior to a final diagnosis of subglottic stenosis. With severe subglottic stenosis, patients will present with dyspnea and marked suprasternal and subcostal retractions. The patient's cry remains unaffected (2).

[This is all exactly true of Trust and we've read other articles that even more particularly describe his situation, including being intubated early in life and having a very hard time getting off the ventilator once it was put in - Trust spent three weeks in the hospital while we waited for swelling to go down in his throat so we could get that vent out.]

A history of recurrent croup may support the diagnosis of congenital subglottic stenosis. Subglottic narrowing may be noted on plain lateral and AP x-rays and the diagnosis is made with rigid bronchoscopy. The length and diameter of the stenosis is measured and congenital subglottic stenosis is diagnosed when the lumen diameter is less than 4 mm in a term infant or less than 3 mm in a preterm infant (1).

In the majority of patients with subglottic stenosis, respiratory problems resolve with growth of the child. However, endotracheal intubation and tracheostomy may be needed in patients with significant airway compromise.

[We already know Trust isn't this bad or he'd've needed a tracheostomy a while ago. He's what they call "asymptomatic", which means he doesn't have any trouble unless he gets a virus - "upper respiratory infection".]

Decannulation by age 3 to 4 years is usually possible when the subglottic space widens. Laryngeal reconstruction to enlarge the lumen of the stenotic airway has proven successful in severe cases of congenital subglottic stenosis (4).

The good news is membrane and possible scar tissue from the times he was on the ventilator can be cut by putting a scope down his throat and using a special laser to cut the webbing, which should open his throat up to full size and take away the need to keep having to rush to the hospital when he gets croupy. Or "stridorous", since real croup doesn't act the way Trust's does.

Apparently, there's already been a noticed correlation between Down's Syndrome and epiglottic stenosis, which is another point to consider.

At any rate, we are currently considering trying to visit a doctor in Cincinatti who's done a lot of work with kids like Trust who have problems with their throats like epiglottic stenosis.

Now, about the discussion with the hospital that has encouraging signs:

The doctor at St. Joes is willing to consider a way to provide us with something like call-ahead seating: a prearranged setup where the hospital has all Trust's information in a special file with detailed instructions of what do to when he shows up with the same croupy problems he always does. Doesn't sound like much, but it would relieve a big part of the pain of having to go in when he gets sick.

We can skip the emergency room diagnosis headache, call ahead to warn Peds we're coming in, go straight upstairs, and have all Trust's medicine waiting for him and the following routine all laid out and ready to go. No more fighting with the ER doc about not intubating him, no more trying to talk everyone into giving him the exact same course of medicine that worked so well the other half-dozen times we've had to do this, no more trying to convince people that he doesn't have pneumonia and doesn't need X-rays, no more trying to keep them from taking a million blood tests to try to figure out all kinds of stuff that doesn't exist...this would be a huge improvement!

Apparently the doctor had a friend who was dying of cancer several years back. Everything that could be done for her had been done and her goal was basically to spend as much time at home with her family as possible. Only problem was a side effect of the cancer was her lungs would fill up with fluid now and then. She just needed the fluid drained so she could be comfortable; but when it'd happen she'd have to rush to the hospital. Some doctors would drain the fluid and send her straight home, but others would want to admit her and run lots of tests to determine what she already knew: she was dying. So she would start calling ahead when she felt fluid starting to build up, asking the ER who was on duty and waiting for the right doctor to be in before she'd go in. The doctor we were talking to agreed with Dad there should be some way to provide people like her or Trust - who have chronic problems that cause certain side effects that need specific attention now and then - to skip the whole rigamarole at the front and come straight in for the stuff they need when they need it.

Mostly, we've sort of been thinking to ourselves for a long time that next time we have a cold, Trust won't need to go in. Eventually he'll just grow out of it, we thought.

Problem is, this has been going on for three years now and really shows no sign of changing soon. He could be seven or eight or maybe even ten or eleven before this stops, and it's about time we did our best to try to work out a system that makes everything a little easier on all of us. I think we've all come to the conclusion it's time to start looking at doing things instead of just waiting.

We need to get an official diagnosis of what the problem with his throat is so we can march in anywhere and say, "Okay guys, here's the story" instead of giving them kind of vague definitions of what we guess might be wrong.

We'd like to see if there really might be a chance of fixing the problem. And we may be able to get a way to skip three-quarters of the hassle that goes along with taking Trust in for medicine, which would make things a lot more relaxed when we do have to take a trip over there.

And in the meantime...Trust stood by himself for fifteen or twenty seconds today. If we keep seriously consider getting a video camera, he may be the first of all our kids to have his first steps recorded. When he finally walks, I think we should have a party!