The BetsyG-Spot

A few weeks ago, I wrote about my son’s (then) upcoming echocardiogram and the anxiety that surrounds this quarterly event (Echoes of Anxiety). It took quite a while to get any result at all (for no good reason, really, as I usually hear something immediately) and all I got was “it is better,” which didn’t tell me much.

Since that post, Matthew and I have been downtown two more times to visit the pulmonologist and to have an additional exercise tolerance test. To refresh you, Matthew somewhat inexplicably has pulmonary hypertension, which means there is too much blood pressure in the pulmonary artery, which goes into the heart. The pressure is supposed to be something like 25%; his is now down to 50% from 55% after he’s been on medication for about six months. This is considered moderate pulmonary hypertension, but the danger is that it typically gets worse, and that’s just not good.

It’s been a year since Matthew’s diagnosis. He initially presented with no symptoms at all, except his hands had turned blue. It’s still unclear what the relationship is between his blue hands and the pulmonary hypertension (go ahead—google…you won’t find anything about pulmonary hypertension and blue hands). The hands essentially ended up being a neon sign reading, “Look inside!”

At the time of diagnosis, the pressure was about 55%, but his exercise tolerance was excellent. The only problem was that his blood oxygenation (SATs) went down with extreme exercise, something he didn’t even notice. This is not what you’d expect from someone with his level of pulmonary hypertension—you’d expect much worse. After they started him on medication, even though the echo showed very little improvement, his exercise tolerance impoved. So at now 50% pressure, he is awfully close to normal in his exercise tolerance. Fifty percent pressure and normal exercise tolerance is anything but a normal combination.

The new exercise tolerance test that Matthew had last week was a more accurate measure of heart function. I wasn’t in the room, but he was on a bike and apparently got his heartrate up to 190 while they measured both his SATs and the gasses that were expelled from his lungs. The technologist said the results looked good—pretty close to normal—and that his SATs were fine. Bizarre.

I was thinking about how weird it was that Matthew seemed so normal when, internally he is so…not…normal (the inflated pulmonary artery is one of the more normal abnormalities going on inside him). If he’s not having the expected symptom—exercise intolerance—how bad could this thing really be?

Oddly, that evening the pulmomologist called to tell me the pulmonary hypertension guys at Mass General held essentially the same viewpoint. Matthew should be a very sick boy, and he should be getting worse. A year ago they were prepared to give him highly toxic medications (with the liver being the organ affected by these toxins) so they could improve his condition enough for him to be retransplanted. (There was no point being careful about a liver that was going to come out, and yes, they really do make decisions that way.) But he continues to live a very normal life with a deadly condition, with no signs of worsening. So the conclusion they came to was…leave it be. They had been thinking of putting him on a less toxic medication (similar to the very toxic one) which still had a small chance of causing damage to the liver. But the risks of that drug seemed to be greater than the risk of leaving him be and continuing to follow him.

So that’s what they’ll do: continue to follow his condition, make no medication changes, and hope that this truce the various organs in his body seem to have made holds.

Thank you everyone for your concerns and support.