I'm Aware That I'm Rare: George B. Mallory, MD
George B. Mallory, MD is a pediatric pulmonologist at Texas Children’s Hospital. In 2001, he joined the Baylor College of Medicine pediatric faculty and founded the TCH Lung Transplant Program. Since 2005, Dr. Mallory has also been medical director of the region’s only pediatric pulmonary hypertension program. In this episode, Dr. Mallory discusses his experience with lung transplantation in children with pulmonary hypertension and cystic fibrosis.
My name is George Mallory. I’m a pediatric pulmonologist, currently working at the twilight of my career at Texas Children’s Hospital in Houston, a member of the faculty at the Baylor College of Medicine.
I’ve had a really unique perspective on pulmonary hypertension in children in that I started in lung transplantation almost 28 years ago, in the era where there were no therapies. And so many of my very first patients referred at St. Louis Children’s Hospital for lung transplantation when it was brand new, had pulmonary hypertension. And I still remember … if I can give you a precious vignette, just a wonderful story. A 23-year-old from St. Louis with Down syndrome, who had been evaluated at the world famous adult lung transplant program across the street, Barnes Hospital, where the senior surgeon, a great man, but not well educated in Down syndrome said, “We’re not going to transplant this young woman. It would be a waste of resources.”
Well, in 1990 the pediatric world had come to accept that people with Down syndrome deserved absolutely equal access to healthcare resources, surgical services. With informed consent, we evaluated her. She had had a heart defect that either been overlooked or ignored 20 years before, and she had developed pulmonary hypertension in the form that we call Eisenmenger’s syndrome. She was a delightful young woman, great family. Missouri Medicaid was not covering lung transplant at this point. The hospital made a deal with them, and they ended up, for the next six months … she actually got transplanted within a month … the family sold pretzels on street corners in St. Louis to try to make money to help cover the bills. Well, they never got around to raising enough money. But she got a single lung transplant, one lung, and she lived 19-1/2 wonderful years. So she was the longest living recipient of a lung transplant at St. Louis Children’s for many years, and I just thought what a rich irony. It was pulmonary hypertension before there were any therapies and a very unlikely person. But I learned a lot from her.
I’ve had this unique experience of being involved, very involved in lung transplantation, being significantly involved in the world of pulmonary hypertension that has changed so much in the last 15 years. We’re not doing many lung transplants in childhood for pulmonary vascular disease at this point, probably no more than 10 to 15 individuals in the U.S. under the age of 18 per year; whereas, even though we’ve made all these advances in therapy, probably 100 to 200 babies, children, and adolescents who are dying of pulmonary hypertension.
The cystic fibrosis community has made a recent discovery that probably too many individuals who are dying of cystic fibrosis, even in this optimistic period, without ever really having a chance to really think through or be evaluated for lung transplant. I’m on a committee with the Cystic Fibrosis Foundation, and I’m going to borrow their terminology. I think it’s time to probably normalize the idea that children with pulmonary hypertension and their families should be educated in a serial way about what the menu is if the child’s disease continues to progress. It includes new, still only somewhat tested procedures, like the Potts shunt. We keep looking for new medications, and I’m very hopeful about that, but if it is end-stage, lung transplantation is an option that I think families should be told about.
Some good, loving, responsible families and teenagers who must assent to it say, “No, thank you.” And there are worse things than dying, but I believe that lung transplantation is an option that should be considered. I had an opportunity to access one of my well connected PH mothers who has been involved with many other PH mothers on social media, and I asked her to help me understand some of the reasons that she and other parents might consider the least attractive parts of lung transplantation. And she was very generous to share. I think she sent out a communication and she got a lot of feedback. I filtered through that feedback and presented many of those perceptions. I think there are some very legitimate concerns.
There are only six or eight pediatric lung transplant centers in the United States, some of whom are very low volume, and if a child got listed at one of those programs, some of those programs have a very high what we call wait list mortality. The chance of getting on the list may be fairly decent, but some programs are just not very aggressive at going after lungs, and kids die on the list, and that’s a real eventuality that I think most parents and kids shudder at.
On the other hand, there were reasons that were provided by other parents that I really think were a product of misinformation. One mother said, “Well, you know the average life expectancy is only one and a half years.” That’s never been true. In our own program, we’re up to an average life expectancy of six and a half years, but I’ve learned that there are some parents who don’t understand what that really means. That doesn’t mean that you’re guaranteed six years. It doesn’t mean that at six years your lungs are going to suddenly fall apart. It means that half of the patients are still going to be alive at six years. And many of those patients are going to be in excellent health.
Most of our patients recover and get out of the hospital within two weeks after a transplant operation. They’re already starting to feel well, despite a major operation. They’re on a new set of medications, but the good news is that … and this was another misconception … the side effects of immunosuppression, in terms of how they make you feel on a daily basis, are actually considerably more benign than some of our PH medicines, especially the prostanoids. We are able to reduce some of those medications over time.
I feel very good about not only the work I’ve done, but the stories, the lives that I’ve seen. And I can tell you and your listeners that over my almost 28 years in this field, I’ve probably been involved in more than 400 pediatric lung transplants, and I can’t think of even five families that told me that they regretted having made the decision. It doesn’t mean there were always great outcomes, but lung transplantation is an incredible partnership. We have a wonderful multidisciplinary team in our own center, and I know there are great teams in other centers.
As I move towards the end of my career, I’m just delighted to continue to still be involved with pulmonary hypertension, to be involved with lung transplantation. I hope the good Lord keeps me around long enough that I can continue to see improvements. Life expectancy has gotten better in PH. It’s gotten better in lung transplant. We’re using more and more techniques and different medications to enhance the lives of the patients entrusted to us.
In the most recent era, we’re still talking about somewhere between 20 and 50% of newly diagnosed pediatric PH patients are going to die at 10 years. If it’s a child diagnosed today, I want your readers to know we don’t have the curve for patients, because there are going to be newer medicines. But what that means is, need to pay attention that we’ve successfully slowed down the disease. We haven’t defeated it.
And to talk about lung transplant is not to sign a contract. It’s to have your options open. I think that bringing one’s child to a transplant center … not for fun. Not if it’s years and years away. We’re too busy to see a nice cute kid who’s doing fine, who has mild pulmonary hypertension. That would be a waste of resources. But to be able to look into it, to be well informed, make sure that you hear what is really considered to be an accurate reflection of what the state-of-the-art is, you need to know what could happen, both on the good end and on the bad end, and ask good questions. I’m always thinking of word pictures. When you go to a restaurant, you don’t to just pick the first thing on the menu. You want to kind of look at what’s there and then maybe ask some questions and consider it.
My name is George Mallory and I’m aware that I’m rare.
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