require transplant, and therefore get transplanted in adult centers now. When we first started doing lung transplant in about 1990, when I was in St. Louis, cystic fibrosis was a common indication, because children under 18 with severe cystic fibrosis would come to St. Louis for transplant. In Philadelphia, we see less of that and more pulmonary hypertension and congenital heart disease. What drew you to the lung transplantation field? I started out as a congenital heart surgeon. Congenital heart surgeons all have to be adult heart surgeons fi rst. I did adult and congenital heart surgery at St. Louis Children’s Hospital. I was recruited to come here to take over from William Norwood, a very well known surgeon, who went to Europe to start a new program. When I came to Philadelphia, there wasn’t a lung transplant program in
this area. So I started a pediatric lung transplant program when I came in 1994, having started the program in St. Louis in 1990. I think my interest in lung transplant came from patients I saw in St. Louis who had no real, good option for repair of their heart, because they didn’t have good lungs to push blood into. There are certain types of congenital heart disease where that’s the case, where you could repair the heart defect if you had pulmonary arteries to pump blood into. But children who don’t have that became progressively more cyanotic. I saw some of these children and I thought, 'If we could just put new lungs in, we could fi x the heart.' So that’s what got me interested initially, and then that expanded to all the other potential reasons for lung transplant, of which there are many. What are some of the differences in quality of life after a successful pediatric lung transplant? Quality of life depends on the patient’s condition before the transplant, but the majority in the pediatric population
are extremely debilitated. They often have cystic fibrosis. They’re chronically infected. They have poor lung function. They have very little exercise tolerance. Patients with PH may have heart failure also. So the quality of life prior to transplant is very poor. The waiting times are so long for lung transplant that most children deteriorate significantly while waiting in the hospital. They’re sometimes waiting in the hospital for a year or more. So the quality of life after transplant, while vastly improved, takes a while for them to recuperate. I think what people often don’t realize is that if you’re sick for months and months prior to lung transplantation, there’s a lot of rehabilitation necessary. Even after a successful lung transplant, it’s not like patients are going home in a week. Many of them have to stay in the hospital for months while they’re literally recuperating and rehabilitating themselves from being chronically ill for the previous several years. Continued on page 26 Patient room (left) and art therapy (below) at The Children s Hospital of Philadelphia.
All photos courtesy of The Children's Hospital of Philadelphia.
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