Preventive Care in Sickle Cell Disease

Ify Osunkwo, MD, MPH: Now, we didn’t touch on, early in the conversation, about the life expectancy of sickle cell disease. We have both pediatrics and adult hematologists here. In pediatrics, it’s beautiful, right? We do a great job. They take their hydroxyurea, they live to be 19, 20, 25 and then they move on to adult care. And you just mentioned that after 30, 40 years, it’s not so rosy. Julie and Matt, what do you guys see in terms of the mortality you see in your young adult population?

Julie Kanter, MD: As you mentioned, we are still seeing what I would call a spike in mortality in our 25-to-35-year-olds. I think there are a lot of reasons for that. The biggest issue it seems to me is difficulty in coping, in coping not just with the disease, but the independence that’s associated with being a young adult. Adulting itself can be hard.

Ify Osunkwo, MD, MPH: It is hard.

Julie Kanter, MD: Adolescence itself is its own disease. Just the idea that they’re going to have this disease and coming to some form of balance and acceptance, and acceptance that they’re not going to fight it but they’re going to learn to live with it. Just like anybody else in college, they can’t go out and party every night. And that can be a really difficult thought, that not only can they not party every night, but maybe they can’t party like their friends because that burden will increase their stress and cause them to end up in the hospital.

The other thing though that I think is crucial to bring up is a lot of my young adults are dying because they’re in hospitals where providers are not as familiar with the complications of sickle cell disease. I really want to reach out to providers and say, “If you ever need anything, please call me.” If you have a question or there’s a complication that looks different or you’re not familiar with it, that we want to make sure we get to these individuals to a center that can take care of them. I do think frequently they end up in community hospitals, and everybody wants to do the best by their patients, and they just might not be as familiar with sickle cell disease. That period of young adulthood is especially stressful, and so I think those patients are in the hospital more, and that poses more risks.

Ify Osunkwo, MD, MPH: Do you see that within the adult population?

Matthew Heeney, MD: I think there are not only disease-related factors that can potentially be contributing to that spike in mortality in terms of the accumulation, the inexorable accrual of organ damage that we talked about, coming to some sort of threshold. I also think there are other systemic aspects to it. Getting older is harder. What comes along with it now, job insecurity, school, housing insecurity, food insecurity, all those things come to a head in young adults, the loss of insurance, potentially. The definition of disability in our country really thinks more about static disability like a transected cervical spine as opposed to sickle cell disease where patients can be completely disabled 5% of the time, just long enough to lose a job, just long enough to fail out of school, but not long enough to get some benefits in terms of social security.

Ify Osunkwo, MD, MPH: That’s a very good point.

Matthew Heeney, MD: There are systemic aspects to it as well. It seems, from the young people I’ve spoken to, like serial abandonment. They lose their pediatrician, then they lose their pediatric hematologist. They have to go to a new care facility where they may not immediately kick it off with their new physician, and we beg them to give it a chance. We just beg them to give it a chance and to really solidify their new medical home because that’s what’s so important for their long-term success.

Ify Osunkwo, MD, MPH: By the time they come to you, they’re on the down spiral.

Biree Andemariam, MD: In terms of…?

Ify Osunkwo, MD, MPH: Disease progression, complication rates, accumulative damage from lack of care.

Biree Andemariam, MD: Well, I’m seeing a change though. Oh, you mean those who have been out.

Ify Osunkwo, MD, MPH: Out of care.

Biree Andemariam, MD: Out of care, not necessarily transitioning directly from the pediatric hematologist’s office. You’re absolutely right. I see 2 populations of patients. I see those who have been well tucked in by people like Matt in the pediatric programs who are on their hydroxyurea, who are on their transfusions for stroke prevention, who are all vaccinated, who know their history, who know their alloantibody status.

Then I have those adults who I’ve acquired through community outreach who are in their late 20s, 30s, 40s, 50s and beyond who are really in a bad way—retinopathy, partial blindness, if not complete blindness in 1 eye, bad avascular necrosis with serious pain, liver disease, pulmonary hypertension, you name it. They have somehow grown up thinking that not having providers available to take care of them has led to this problem. Thinking that, “Well, I’ll just live pain crisis to pain crisis, and when the pain crisis is over, I’ll get on with everything that was on hold.” But they don’t always know that things are happening under the radar, chronic organ damage is developing. We have a lot of improvement to do.

Ify Osunkwo, MD, MPH: We have a lot of work to do.

Julie Kanter, MD: I really think some of that needs to start more in pediatrics. As a lifespan provider, I’ve taken care of kids and adults, and I think it’s important that we really start the conversation about preventive care. Pain is important and it certainly affects quality of life, but understanding that this disease isn’t all about pain. And we have to address the issues that are going to cause mortality because when they’re still listening to us when they’re younger, we want to make sure they understand that.

Ify Osunkwo, MD, MPH: Make an impact.

Julie Kanter, MD: Exactly.

Ify Osunkwo, MD, MPH: Get through to them so that they have that, they kind of grow up with it.

Julie Kanter, MD: Exactly.

Transcript edited for clarity.