Patient: Danny O’Rourke, 9
Doctor: Harold J. P. van Bosse, pediatric orthopedic surgeon, NYU Hospital for Joint Diseases
Patient’s mother: Karen O’Rourke
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Karen: At first, when Danny was born, I couldn’t tell anything was wrong. But when he started walking, you could see he was crooked. He actually walked before he could sit up, because he couldn’t balance himself—but I guess I just didn’t really think it was as bad as it was.
Van Bosse: He had congenital scoliosis and asymmetrically fused ribs, a variant of a genetic disorder called Jarcho-Levin syndrome. It thrust his spine to the side and gave him a severely deformed chest cavity; he was terribly off balance, like a wind had blown through him. Eventually, his lungs wouldn’t have enough room to grow, and if we couldn’t expand his chest, he would have become an adult with child-sized lungs. In the long run, he couldn’t survive like that.
Karen: When I realized how bad it was, it was a scary thing.
Van Bosse: When he was a year and a half, I did my first operation, fusing his spine, and I revisited that when he was 3. But this was years ago, and we didn’t have a whole lot we could do.
Karen: As Danny got older, there were times he couldn’t breathe. Every two months he’d have an asthma attack, and he was afraid to go to the doctor because he was worried he’d end up in the hospital again.
Danny: I started playing baseball when I was 6. My favorite team is the Yankees. I like Gary Sheffield, because he has the coolest stance. But it was difficult. When I’d run to first base, I’d get out of breath.
Van Bosse: I knew about a gentleman named Robert Campbell, who’s treated a number of kids with chest deformities. He came up with a device that helps expand the chest—the perfect thing for Danny. What stood in the way was FDA approval. But if you don’t do this, Danny’s condition could kill him, so the FDA gave us an exemption. We needed to find the right time for the procedure, but Karen wanted to let Danny finish baseball season first.
A device was hooked onto Danny’s second rib and spine—a jack to straighten out the spine. “We’re trying to grow his chest along with him.”
Karen: That’s the one thing I wasn’t going to take away from him. He made the all-star team.
Van Bosse: The surgery happened in September. We made a large incision on the back along the shoulder blade. We found where the ribs were glued together and cut across, separating them. Then we installed the device: It’s two rings on either end of a telescopic rod. The rings grab onto Danny’s third rib—that’s a high rib—and tenth rib. By opening up this rod and locking it in place, you’re able to open up the chest. A second device was hooked onto Danny’s second rib and the spine itself—a jack to straighten out the spine. Every six months you go back to the operating room and make a limited incision, extend the rod, then clamp it down. We’re trying to grow his chest along with him. Even during surgery, the anaesthesiologist could see a difference in how much pressure he needed to open Danny’s lungs. He’s breathing much better now.
Karen: He’s a lot straighter, too. His shirts used to fall off his shoulder because one was higher—now he’s like, “Wow, my shirts stay on!” He’s a 9-year-old boy … he’s self-conscious.
Van Bosse: I’ve noticed him become a lot more open, more expressive. It certainly hasn’t become all roses; he probably has a 6-year-old’s chest now in relation to his 9-year-old body, but before, it was a 3-year-old’s. He’s not ever going to have normal lung function, but if we can keep it where it is, we’re not doing too badly.
Danny: When I run to first, I can keep running. Next: College Student Going Blind In Both Eyes