Kenneth McCurry, MD, Director of Lung and Heart Lung Transplantation at Cleveland Clinic and a staff Cardiac Surgeon, talks about the rare occurrence of a combination lung and liver transplant surgery.
Mr. Fealy had Alpha-1 Antitrypsin Deficiency; can you describe how that affects the body?
Dr. McCurry: Alpha-1 Antitrypsin Deficiency is a genetic disease; it’s an inherited disease and it comes in a couple of different forms depending on how many gene mutations a particular patient has. Basically it results in many patients, progressive failure of the lungs from what we call obstructive lung disease. It’s sort of a similar process to what happens to the lungs when people smoke. But this happens as a result of a genetic problem and the absence of smoking. Unfortunately it can also affect the liver as well and lead to cirrhosis; progressive sorts of failure and fibrosis of the liver which Mr. Fealy was affected by both.
How common or rare is it to need both a liver and lung?
Dr. McCurry: It’s pretty uncommon. There are a variety of indications and reasons that we do combined organ transplantation. Sometimes its lung/liver, sometimes lung/heart, more commonly; occasionally even lung/kidney. Out of the patients who we do lung/liver on probably the most common indication is Alpha-1 Antitrypsin Deficiency or A1AD. But the majority of people who have that disease can get by with just a lung only because usually their liver is still working pretty well, so it’s really a small minority of patients. We occasionally do lung/liver transplantation for other problems. For example, someone may have pulmonary fibrosis or some sort of other disease and have some other secondary second disease that involves the liver. It’s actually a bit shall I say controversial in the United States. It’s a very complex operation to do both; it can be very difficult and requires centers with a lot of expertise on both the lung side and the liver side to perform it technically and then to manage the patients afterwards. We’ve actually had even some difficulty with patients that we thought there was a very clear cut indication to do both a combined lung and liver yet their insurance company has refused to allow that to be done. And unfortunately we have had a few patients not survive as a result of their insurance company declining to allow it to proceed forward.
So in Mr. Fealy’s case was his worse than the normal patient you see that’s why he was able to get the lung/liver?
Dr. McCurry: Yeah his was very advanced. We commonly do lung transplants on patients with some liver disease. So if the liver disease is rather mild or even moderate and the lungs are severely damaged, most of the time we can get those patients through with a lung only and then the hepatologist, the liver medical doctors, will sort of wait and see what happens to the liver later on down the road. The majority of those people do well for a long period of time. In his particular circumstance his liver disease was so advanced that we felt that if we did his lung transplant alone that there was a very high chance that the liver might fail as a result of the stress of the operation. So that’s why we made a joint decision between the lung program and the liver program to proceed with the combined organ transplant. He had a lot of manifestations of liver disease, he had what we call ascites which is fluid in the belly, he had big collaterals in the belly that result from what we call portal hypertension which has to do with the pressures being very elevated as a result of the cirrhosis.
Mr. Fealy was singing a few days after his transplant, can you talk about how his recovery went? What was life like for him before the transplant, what was he going through?
Dr. McCurry: He was as you can imagine very short of breath because of his lung disease. He was also suffering from his advanced liver disease as well, and then he had fluid in his belly so it was swollen and making him uncomfortable. All of those things led to a very poor quality of life and his mortality rate without a transplant would have been quite high over the next year or so if we hadn’t been able to get the organs for him.
What factors make a double organ transplant more difficult than a single organ?
Dr. McCurry: It’s a much bigger operation, each in isolation are quite big. But to do both, typically we do them sequentially, so in this particular circumstance we did the lungs first and then the liver team did the liver transplant second. The time involved, the technical expertise involved while we’re doing the lung transplant; the liver is still on ice in a cooler so it’s a longer period of time that the liver is ischemic and without blood supply. Under these circumstances we need to proceed pretty expeditiously and quickly with the lung transplant so the liver transplant surgeons can proceed with the liver transplant. The combination of those two things and the logistics make it technically difficult. The management afterwards is also quite difficult. In the case of a lung/liver transplant though things are pretty well matched up in terms of how we manage fluid in the body and immunosuppressive medications and things like that. In the case of say a lung and a kidney when we occasionally do those two, the management afterwards can be sort of diametrically opposed. Because for the lungs we typically try to keep patients quite dry without much extra fluid in their bodies and the kidney transplant surgeons typically want the exact opposite so it can be quite difficult to manage patients.
Can you talk about how Mr. Fealy’s recovery went?
Dr. McCurry: Yeah, he did extremely well. As I recall the breathing tube came put pretty quickly, his liver worked great so both organs, the lungs and the liver, both worked fine and he was in the ICU for a relatively brief period of time, so he’s made good progress. He’s still got a ways to go, there are a few issues I think that we’re dealing with, but that’s not at all uncommon. I think in the big scheme of things in terms of the complexity of the transplant he’s done quite well.
What types of considerations will the patient have to deal with now after his transplant?
Dr. McCurry: As with any organ transplant recipient, the big issues after a transplant are really sort of three. There’s always a risk of infection, related to the immunosuppressive medications. There’s always a risk of rejection, and then there’s sort of complications or side effects of the medications. So any time we give someone an organ transplant the body recognizes that organ as not being part of themselves and we have to use medications that we call immunosuppressive medications to sort of lower the immune response against the organs. And that diminishes the likelihood that the organs would be destroyed but at the same time increases the risk of infectious complications. So a big part of what we do after the transplant is sort of trying to balance how much of those medications we give to sort of achieve the desired level of immune suppression but not to over immuno suppress so the risk of rejection is higher. There’s also a lot of potential anyway for side effects of the medications, they commonly cause diabetes and can sometimes cause kidney dysfunction and lead to other sorts of complications like that. What I always counsel potential transplant recipients is that receiving a transplant in many ways becomes another chronic disease that they have to deal with. Because it requires coming back frequently to see us to have these things managed. But hopefully it’s with a much better quality of life and hopefully a much longer life than they would have been able to live otherwise.
As a surgeon what’s it like for you to see a patient who was so ill be able to have a second chance at life?
Dr. McCurry: I mean the reward from a personal standpoint is tremendous. It’s very fulfilling to be part of this process; it’s why I got into this field in particular with regards to lung transplantation. Years ago I saw a number of patients suffer and die as a result of their lung disease and became very interested in prospects for lung transplantation. At that time the field was sort of just beginning to evolve and emerge, so we have many patients who come in and they’re extremely debilitated, short of breath, can’t walk fifty feet without getting short of breath. And I had some personal experience with that in the past as well. So to be able to see people enjoying their lives afterwards and living longer lives is very rewarding. There’s actually something called the transplant games as well which was actually here in Cleveland in the last year or two. And that’s an amazing thing because you see all different organ recipients but in particular the lung recipients obviously from my standpoint. These people were very short of breath, on oxygen before their transplant and now they’re out racing doing hundred meter yard dashes and the four hundred meter race and various other things like that so it’s pretty spectacular.
Can you talk about the importance of organ donation?
Dr. McCurry: Obviously a transplant starts with the organ donor. And it’s a tragic situation when someone dies; it’s what we in the medical field work to try to prevent; to save lives. But when someone dies, the gift of an organ donor is tremendous because many people can be helped and unfortunately there’s still a shortage of organs that are available primarily because everyone that could be an organ donor is not an organ donor. As a result of that, particularly with regard to lungs, for about every two patients that we transplant we have one patient die on our waiting list. So even though about two thousand or so patients benefit from a lung transplant every year in the United States, there are a lot more that could benefit. So I would certainly encourage everyone to consider being an organ donor and to talk to their family members about that because it’s something very good that can come out of a very tragic and unfortunate situation.
Is there anything we didn’t cover that you think is important to talk about?
Dr. McCurry: I don’t things so; we brought up organ donations, very important. There are some differences, lung transplant is still in many ways sort of an orphan disease or an orphan procedure. There are about two thousand lung transplants that are done every year in the United States or about sixty five to seventy or so (it varies year to year,) lung transplant programs in the United States. So if patients have some sort of lung disease and their pulmonologist has considered transplantation as an option, there is a significant difference in availability of lung transplants around the country. So they should always consider pursuing options at a larger center perhaps that may be at a remote distance from them if they’re turned down or if it’s not possible to get a lung transplant locally.
END OF INTERVIEW
This information is intended for additional research purposes only. It is not to be used as a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or accuracy of physician statements. Procedures or medicines apply to different people and medical factors; always consult your physician on medical matters.
If you would like more information, please contact:
Andrea Pacetti
Sign up for a free weekly e-mail on Medical Breakthroughs called First to Know by clicking here
