Cystic Fibrosis: Transition into Adulthood
Guest: Dr. Patrick Flume - Pulmonary, Critical Care, Allergy & Sleep Medicine, MUSC
Host: Dr. Linda Austin – Psychiatry, MUSC
Dr. Linda Austin: I’m Dr. Linda Austin. I’m talking, today, with Dr. Patrick Flume, Professor of Pediatrics and Medicine, as well Director of the Cystic Fibrosis Center here at MUSC. Dr. Flume, in an earlier podcast, we talked about CF generally. You mentioned at the end of that podcast that, now, kids are transitioning into the adult clinic. What are some of the special challenges for CF kids as they become adults?
Dr. Patrick Flume: The greatest challenge is getting them to accept responsibility for their own care. They need to recognize that the therapies they’re involved in are beneficial to them and not just another challenge in their day. And what we ask them to do is quite a lot, in terms of medications and therapy. It’s both expensive and time-consuming. They have to deal with some of the issues many of us did not have to face during adolescence. For example, when I went off to college, I didn’t care much about what my insurance was. But it’s critical for them to know, so they can afford their medication. They need to contact their caregivers to make sure that they’re getting the services that they need.
Dr. Linda Austin: I would imagine, also, going off to college, away from parents, they need to monitor their health in a much more mature way than most kids have to think about.
Dr. Patrick Flume: They do. And what we find as we work toward that epiphany of recognizing that they need to take ownership of their care, there are many who actually thrive on the independence that they achieve. So, a big part of our transition program is that, early on, before we move them to the adult clinic, we begin to see the child, or the adolescent, on their own, without their parents in the room, in part, so they will take ownership. They’ll tell us about their medications and therapies. But they also need to tell us the symptoms they’re having without interruption from their parents. And when they get that opportunity, of being treated like an adult, their opinion is respected; we’re listening to them, it’s amazing how much they can thrive.
Dr. Linda Austin: I would imagine that it’s challenging for their parents, also, who have probably built their lifestyle around being concerned about their child.
Dr. Patrick Flume: It’s very difficult, and many are reluctant to step out of the room when we ask them to, because they have a great deal invested in these kids. But what they have to learn, as every parent has to learn, is that they have to let go of their kids and let them become independent, and hope that they’ve done their job in teaching them the best they can.
Dr. Linda Austin: I work with college students a lot and I’m struck by how different the culture is from, certainly, when I was in college, and even the culture from everywhere else, with late-night hours, hard partying, too much alcohol, illicit drugs. I mean, that is part of the college scene these days. Is that particularly hard for kids with CF?
Dr. Patrick Flume: It can be. The unique problem that they have is maintaining their therapies, which takes time and effort. They have to make sure they get their calories. So, skipping meals is not an option. They need to be careful about this need to be thin. It’s too easy for them to lose weight if they don’t take their digestive enzymes. And that’s a particular issue in the adolescent females. We also need to work toward providing them with therapies that they can do independently. They may not have the ability or luxury of having someone else help them with their chest physiotherapy. So, we have to teach them techniques, or use devices, that allow them to do their therapies on their own.
Dr. Linda Austin: I would guess that making that transition doesn’t happen overnight. I would guess that it’s a process that occurs over several years. Is that right?
Dr. Patrick Flume: It’s true. Actually, we define transition as the process, and transfer as the event. So, in our center, the transition begins at birth or, really, the first time that we meet the family. So, when a new child is diagnosed or is transferred to our center, and the pediatric program is doing their evaluation, we, on the adult side, will come over to meet the family, and meet the patient, not to do an evaluation, but to convey one critical message. No matter what they hear, or what they read, never forget that we take care of grownups. So, they should always have that hope and promise. With proper care, these kids will, in fact, grow up. They will become independent. We will work with them in getting their education, a job, family, and the like.
Dr. Linda Austin: Let’s talk, now, about some of the medical issues in adults with CF. How is it different, if it is different, from childhood CF?
Dr. Patrick Flume: It’s different in a number of ways, mainly in terms of severity. The lung disease is chronic and progressive. So, typically, they’re going to have worsening lung disease. So, generally, we’re using more therapies; more expensive therapies. We still focus on nutrition. But rather than focusing on growth, we’re trying to maintain nutritional health. With good nutrition, comes good lung function. They go hand in hand.
In our clinic, a multidisciplinary clinic, physicians, nurses, physical therapists, respiratory therapists, dietician, social worker, pharmacy, all, are trained in the area of CF and work together. We meet, often, to talk about our patients and try to find out what their needs are and help steer them. At one time, it was thought that the adult patients wouldn’t have the same needs as in pediatrics, but I’ve actually come to believe that their needs are even greater.
Dr. Linda Austin: In the earlier podcast, you talked about CF being a disease of both the lungs and the pancreas. You mentioned the lungs, but is the pancreas increasingly a problem as well in adult life?
Dr. Patrick Flume: There are some features of CF that get worse with time, and there are some which are present throughout. So, in general, the child who has pancreatic insufficiency will have the same problem in adulthood. And it’s not something that necessarily becomes progressive. Having said that, another aspect of pancreatic disease is the development of diabetes, and this is something that becomes increasingly prevalent as they grow older. So, we screen our patients, starting in early adolescence, to look for the presence of some glucose intolerance, or features of diabetes. We have a higher number of patients with diabetes in the adult patient population.
Dr. Linda Austin: I’m getting the sense; I don’t know if this is correct or not, that the lungs are a greater problem than the pancreas in adulthood. Is that right, or wrong?
Dr. Patrick Flume: The primary reason that people die due to cystic fibrosis is because of lung disease. More than 85 percent of the deaths are due to respiratory failure. Their lung disease progresses and they just can’t survive. But I can’t stress enough that the nutritional status of the patient, which ties in with the pancreas, is absolutely critical in maintaining good nutritional health, in order to maintain good lung health. In the CF clinic, it’s lung disease, pancreatic disease, and nutrition. It’s GI disease. It’s diabetes. It’s osteoporosis. It’s a whole slew of medical issues that occur with chronic disease. And then there are the psychosocial ramifications of dealing with all of those problems.
Dr. Linda Austin: So, yes, it’s lung disease, but you really want to get the whole patient as healthy as possible. Now, you mentioned, though, earlier, that lung transplant is a possibility for some end-stage patients. Can you talk about that a little bit?
Dr. Patrick Flume: Lung transplant became a real possibility around 1990. And CF has become one of the major diagnoses that has benefitted from lung transplant. Lung transplant is performed because a person is going to die as a result of their lung disease. So, it’s not something that we introduce just because a person is out of breath or has an increasing cough. It’s because they’re going to die soon.
Lung transplant is not for everybody. And there are some patients who are not good candidates for physiologic reasons; an example being kidney failure, or cancer. And there are some who aren’t good candidates because they don’t have the psychosocial resources, or support system, to really get through such a challenging procedure. When a person is nearing the end of their life, we have to make that careful consideration in discussion with the patient and their family. Some of those patients, we refer them for transplant. Others, that are not good candidates, or they choose not to pursue transplant, we then have to begin talking about the next transition, which is preparing for the end of their life.
Dr. Linda Austin: What percent of patients are getting lung transplants now? Do you know that data?
Dr. Patrick Flume: From looking at our own data, of all the patients who have lung function that makes them eligible for transplant, about 50 percent of patients end up being referred. Of those that we don’t refer, for some it’s their own choice; they don’t feel that it’s the right option for them. For others, it’s not a good choice because of some other medical or social factor.
It’s important to understand that transplant is not a cure. It becomes an opportunity to prolong life, but comes with some risks. The median survival after transplant is about four to five years. So, although I’ve talked about patients who have had tremendous outcomes and live for many years, that’s not to be expected for everybody.
Dr. Linda Austin: For young women with cystic fibrosis, I would imagine that an important question is: can I get pregnant? Can you comment on that?
Dr. Patrick Flume: I can. First, let me start with the men. Ninety-eight percent have obstructive azoospermia, which means they make sperm but the duct is blocked, so they generally cannot father a child in the usual way. So, generally, we think of the males as being functionally sterile.
Women, it has long been thought that they have a reduced rate of fertility. A number of explanations for that have been proposed. I’m not sure if that’s true. What I can tell you is that they can, and do, get pregnant. It was previously taught that women with CF should not get pregnant because it would be adverse to their health. But that has not been our experience, or that of the rest of the country.
There are issues that are important. Those with severe disease, it’s probably not advisable to get pregnant. Their nutritional needs are going to be greater because the child is going to grow no matter what. One of the critical things we monitor is that they get their calories and grow. And then they need to remember that children require a lot of time and effort. Most mothers devote all their energy to their child, but must not forget their own health.
Dr. Linda Austin: And then, of course, there’s the genetic issue also. Is that right? They would pass on one copy of the gene?
Dr. Patrick Flume: That’s true.
Dr. Linda Austin: They need to understand the genetic implications of that.
Dr. Patrick Flume: And we often test the father of the child to see if he might be a carrier, to try to predict whether the child would have CF.
Dr. Linda Austin: Dr. Flume, thank you so much.
Dr. Patrick Flume: Thank you.
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