A pediatrician talks about a young boy with sickle cell disease
The story that I thought of to tell was about a patient I had in the
pediatric intensive care unit. He was a six-year-old boy with sickle
cell disease who was sent to us with what sure sounded like sepsis, and
when he got to us, certainly was. He was in septic shock – he was
really quite ill.
And we noticed from the beginning that his hands and feet were turning purple. We worked on him mightily, and he stayed pretty much comatose for the first three or four days that he was in the ICU. And during this time it became clearer and clearer that no matter what we were doing that he was going to lose his hands and feet; they were becoming increasingly black as purpura fulmanans was fulminating. And so we brought in all sorts of consultants who would stand over his bed and talk about what the best thing to do for this was, first of all trying to prevent him from losing them, and secondly when it was clear that he was going to, how to manage that part of the issue.
Then one morning, his mother was sitting by his bedside, and he awoke from his coma and was sort of, maybe a little bit like you see on soap operas when somebody has been in a coma and flutters their eyes, and then their eyes pop open. But he didn’t do any of that. His eyes popped open, he looked at his mother, and he said “Ma, they’re gonna cut off my hands”.
And actually I was there, and the resident was standing there with me, and we both looked at each other in horror because we had been talking so freely over his bedside without any sort of recognition that he would, he would know this. But the wonderful thing about this story is not only that I’ve learned to be very circumspect around comatose children, and so did the resident, I’m sure, but the way his mother handled it. Her ability to respond to him in such a positive way. She said, “Well, you’ve been really sick and your hands and feet suffered probably more than anything else, and they may have to do that, but they’ll give you new ones.” And he said, “Oh, okay.” The resident and I left the bedside with tears in our eyes.
This is a really special boy – he did end up losing his hands and feet – we saved his life though and saved his brain. He was still a normal kid, he just didn’t have hands and feet. He came back into the hospital the next time on the orthopedic service to have the initial procedures done to prepare him for prostheses. He was a brilliant six year old, excited about being there, couldn’t wait to get his new feet; he was going to have something done on one of his arms to split the radius and ulna and make it in like a pincer thing – and then going to have an artificial hand on the other side.
And this little boy had always wanted a keyboard, he wanted to play music, so his parents had gotten him a keyboard. He’s sitting up in the bed, this six year old, with a keyboard in his lap and these stumps of arms, playing music he had composed. And saying how good he felt, how excited he was about starting school that September. And the resident who had been with me when he woke up came back to see him, too, and we just marveled at the resilience of a six-year old who was just going get on with being a kid who didn’t have hands and feet. He was a remarkable child, and he taught us all not only about being careful what you say around comatose people, but he also taught us a whole lot about resilience.