Jonathan D’Cunha, MD, PhD, FACS, Chief of Lung Transplantation at UPMC talks about CF and the change in the transplant system.
Interview conducted by Ivanhoe Broadcast News in July 2018.
I wanted to start by asking you a little bit about Cystic Fibrosis, can you tell me what it is and what it does?
Dr. D’Cunha: Cystic Fibrosis is an inherited disease that children are afflicted with when they’re born. It’s a genetic defect in one of the chloride transmembrain transporters which really means that they have problems with mucus throughout their lives. And in a number of different organs that secrete mucus the lungs being one of them and a very important one obviously. They’re susceptible to problems with clearance of that mucus. It’s almost like when you have a bad cold where you’re coughing up sputum and then you get over it because it’s a viral infection or something of that nature. This is what chronically those kids and young adults live with, because they can’t clear their mucus do to this genetic defect. They get chronic infections and once they start getting infections they get resistant infections to different antibiotics and things and it’s progressive over time. What ends up happening to lungs is because of the infection they get destroyed and the function deteriorates. And although we’ve made considerable progress in the managing that over time, it’s really a chronic illness which the lung disease catches up with, it used to be in the teens years and now it’s in the young adults and older years.
Do most patients get to the point where transplant becomes their only option?
Dr. D’Cunha: Yes. And lung transplantation is really the only viable option for those patients when their lungs progress and they’re at the end stage of their lung disease. And obviously we try to hold off as long as possible before we offer them a lung transplant because it is a big operation and you’re on immunosuppression for the rest of your life. However having said that the scales tip at some point, in the young adult years or a little bit later where the infections just catch up with you and the patients really don’t exchange oxygen well and suffer from that.
In terms of transplantation how much of a need for organ donation is there, how long can someone be on that list? What kind of a process is it to get on to the list and then get to the point where you receive an organ?
Dr. D’Cunha: With lung transplantation being the cure for these types of lung diseases, and there’s many different types of lung diseases but just talking about CF in particular we want to get patients referred early and evaluated early so that we’re prepared if their lung disease progresses to get on the list and get transplanted because it is a process to get evaluated for lung transplantation. It’s also very important during that time for families and loved ones to learn about what it means to take care of a transplant patient and support them after surgery for the years to come. It’s a learning process always. They come here when they get their evaluation process and they’re with us for about a week learning about things. And that’s just the tip of the iceberg in the learning process. It’s almost like going to a mini course in college. They come in and they get taught, they get educated they meet you know pulmonologists, surgeons, social workers, financial people, pharmacists, everyone in the program, they try to get educated on things. Once their disease progresses and they get listed then it’s a wait on the wait list depending on their score which dictates they get a score based on their severity of illness. With the higher the score representing the more severe oxygen needs, etc. So that’s very important. Once they get listed they wait for donor organs which are short supply.
How many people on any given day are on the wait list for lungs?
Dr. D’Cunha: For example, here at UPMC we have a wait list that runs anywhere between thirty and forty people at a time and the national wait list is obviously much larger than that. There are patients waiting all over the country for donor lungs. One of the difficulties with organ donors is the shortage that’s out there and in particular lungs are affected because when a donor becomes available it’s the only organ that’s exposed to the environment by it being open to air. And so if you have a hundred donors really only fifteen to twenty percent of those donors are useful for a lung donation.
Can you speak to the change, first of all how those organs are allocated, how it’s decided who gets placed where on the list and then the changes in the allocation system.
Dr. D’Cunha: Yeah. Last Thanksgiving there was a huge change to the lung allocation system and it’s one that has sent a ripple effect through the transplant community, in particular to the lungs but also to now other solid organs. Prior to Thanksgiving of last year we had donor service areas where the lungs were allocated first to the area based on severity of illness. And then they went out to the national pool after that, after the local centers passed on the lungs. So what happened was there was actually a lawsuit last fall around the time of Thanksgiving where one of these lines that was drawn there was a sick patient across the border. For example, there would be a sick patient in New Jersey but the lung donor was in New York and that patient didn’t have access to that particular organ which was only separated by a bridge to be honest with you. And so UNOS, the United Network of Organ Sharing having the final rule being that the sickest patient should receive the organs. There was a lawsuit and basically over night the organ allocation system was changed to say that the sickest patients within a designated area that being two hundred and fifty nautical miles of the donor hospital should be all put in the same pool to receive organs. That was transformative for lung allocation. Meaning that if you can be assured that if you’re sick within that region you would be competitive for receiving those donor organs which are in short supply.
What did that mean for Jenna? She’s from Rochester which I’m not sure about nautical miles but it’s a little bit of a drive to get there, it’s about a four hour drive. What did that mean for Jenna and her family?
Dr. D’Cunha: What it meant for Jenna is being listed at a center like ours, a high volume center, it meant that she’s in a pool of patients and her score was very high because she was significantly ill, she was in the hospital going through a lot. So when she has a high score she’s competitive to where a donor became available. And it’s really the two hundred and fifty nautical miles is from the donor hospital. And so even though she’s from Rochester she came down here for the expertise that we offer in particular from a multidisciplinary collaborative approach that we provide for all transplant patients here but in particular for lung transplant. When a donor became available we were in a good position to go out and access that donor and she got benefitted from her score putting her at an advantage in the new system. At a high volume center where we have the resources to evaluate organs continually it also put her in a good position because what we do we have the ability to fly out to evaluate many donors. Sometimes simultaneously to make sure we’re not passing on any organs available. And that’s an advantage in this day and age when you compare things to centers that do lower volumes of lung transplantation. That doesn’t mean those centers don’t have an important role because it’s a big deal for a patient to move from hours away. Sometimes from across the country to come live in Pittsburg while they wait for their for their lung. So the local centers definitely have a very valuable role in lung transplantation it’s just in this circumstance Jenna and her family decided to move here and they benefitted from this new system.
Jenna told me she got her transplant on Thanksgiving Day.
Dr. D’Cunha: Correct.
Is that just a stroke of luck that she was here and needed it and this ruling became available, did that open up the opportunity for this organ and can you speak to that a little bit?
Dr. D’Cunha: Yeah it did because her score then all of a sudden was higher. It was high to begin with because of her severity of illness but now instead of just being high on the local area, what she benefited from was where the donor hospital that is within two hundred and fifty miles you’ve got concentric circles and now she’s in a pool that’s much bigger. And that’s the goal of the new system is to make sure the sickest patients get access to organs to reduce the chance of them dying while they’re on the wait list.
Can you tell me a little bit about Jenna, she’s up, she’s walking she’s working, what are your impressions of this young lady?
Dr. D’Cunha: From knowing Jenna from before the transplant, just from the listing process and her being in the hospital the thing that struck me was first of all there’s an energy around her from all her support system. It’s quite remarkable. I didn’t know about all the depth of the fund raising and the support that she has. And then her vibrant energy you could just tell that she’s a go getter and these are the patients that you love to transplant because when you give them a lifesaving organ they’re going to pay it forward afterwards. And you could just feel that she was a fighter, she’s a go getter and she’s going to do well with the gift that she’s been given. And that’s certainly the case today there’s no doubt about it. She’s really done remarkably well.
Is there anything I didn’t ask you that you would want people to know?
Dr. D’Cunha: So I think the other thing I would comment on is the wait list time with the new system. And what we’ve noticed is a huge change in the wait list time. So before for example, prior to Thanksgiving patients on our wait list would wait somewhere between a hundred and seventy five and two hundred days on the wait list? And now our wait list when you get listed for transplant, you’re transplanting people with in twenty five to thirty five days currently. So the new system is really changed. And you might say well why, what happened with everything. It’s really because the patients for example at our center that we list, they often times come here because they’re getting very ill, there are a lot of inpatient transfers and their scores are very high because they’re on a lot of oxygen. And so they become very competitive for those donor organs when they become available.
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.
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