Impact of Anemia & Hemolysis in Sickle Cell Disease - Episode 13

Advice for Sickle Cell Disease Management

September 30, 2021
Sophie M. Lanzkron, MD, MHS

,
Jeffrey D. Lebensburger, DO, MSPH

,
John J. Strouse, MD, PhD

Sophie M. Lanzkron, MD, MHS; Jeffrey D. Lebensburger; and John J. Strouse, MD, PhD, share key takeaways and closing thoughts for the management of sickle cell disease.

Transcript:

Sophie M. Lanzkron, MD, MHS: For take-home messages, the first and foremost is that people with sickle cell disease want to live normal, active lives. They don’t want to spend their time in the hospital. If you’re seeing them in front of you, there’s a reason, and there are always strategies to try to improve the overall quality of life for people with sickle cell disease. We should provide them with all of the opportunities available to try to make their lives better. There has been a lot of structural racism that has affected how patients with sickle cell disease are treated, both in the emergency department and in lots of places. We have to overcome that structural racism and make sure our patients have every opportunity to live a long healthy life.

Jeffrey D. Lebensburger, DO, MSPH: It is so important for hematologists and primary care providers who take care of patients with sickle cell disease to first, establish the trust. Many of our patients suffer from what they perceive to be bias against them when they come in for crisis. Please take the time to listen to them and to get to know them because once you can establish that trust, we can really improve the lives of our patients with sickle cell. Second, sickle cell disease-modifying therapies are needed. We can no longer think that sickle cell pain is simply, “Am I in a painful event, or am I not in a painful event?” Please understand the importance that the organ complications over time are the life-limiting complications of sickle cell. Getting patients to accept sickle cell disease-modifying therapies from an early stage of life is vital.

Third, understand the impact of sickle cell on quality of life. Please pay attention to schoolwork, how sickle cell affects their relationships, how it can affect them holding a job, and view our patients in the whole because sickle cell impacts every aspect of their lives. In several cases, we’re the only providers they have to turn to for addressing this multi-socioeconomic impact of sickle cell. Please look out for the entire patient, not just the sickle cell pain event.

John J. Strouse, MD, PhD: It’s important that we take a life span perspective when we treat people with sickle cell disease. Many of the complications that we see in adulthood start in pediatrics, but don’t necessarily cause problems for children. But that doesn’t mean that they aren’t important. I’ll take the kidney as an example. We start seeing a lot of microscopic proteinuria, the microalbuminuria in children with sickle cell disease as adolescents. It can be almost a third of adolescents who have sickle cell anemia who develop microalbuminuria, but they don’t have renal insufficiency as children. Many of them still have hyperfiltration, where their creatinine levels are lower than normal. As those patients move into their 20s and 30s, we see the development of chronic renal disease, and with that, worsening anemia and disease progression in other ways. It’s important that we intervene when people with sickle cell disease are children because that’s where these end-organ complications start.

Transcript edited for clarity.

x