I am a family doctor in Buffalo, New York. A young man is in my hospital with acute chest syndrome right now. It happens to people with sickle cell disease. Something starts it — a pneumonia, usually, but sometimes it’s a little particle of fat that breaks off from the bone marrow in a leg and travels all the way through the heart and into the lungs until it gets lodged in the terminal branches of the pulmonary artery like a smart bomb. Once it begins, the patient’s lungs choke on crumpling blood cells and they drown.
We’ve come a long way in the medical management of acute chest syndrome. For nearly all of human history, until the last few years, it was the death knell of the sickle cell patient — a harbinger. I would like to say that, with all our advancements, we’ve conquered this scourge, but the truth is that by the time people present with acute chest, they’re already on a clock, and the clock is winding down to zero. It has to be treated aggressively, quickly and assiduously. People still die. It remains the most common cause of death in people with sickle cell disease.
There are over three times as many people in the U.S. with sickle cell disease as with cystic fibrosis — yet cystic fibrosis receives as much as 10 times more research funding, a disparity long fueled by race and visibility. This kind of inequity doesn’t just persist by accident; it reflects a collective comfort with certain kinds of suffering, and certain kinds of dying.
