Survey Finds Fewer Than 3% of US Sickle Cell Patients Receive Red Blood Cell Exchange

The finding comes from a nationally representative survey of 100 US-based healthcare providers who each actively manage at least one patient with sickle cell disease. Red blood cell exchange discards a patient's damaged red blood cells while simultaneously mixing the remaining plasma, platelets and white blood cells with red blood cells from a donor before returning them to the body.

Providers reported a range of barriers to administering the treatment. The most common were challenges coordinating between different medical departments, a limited supply of donated blood and a general lack of familiarity with the procedure. Only 5 percent of the providers surveyed reported no barriers to delivering the therapy.

Among patients, a major concern identified in the survey was whether red blood cell exchange would be covered by their health insurance.

Sickle cell disease, also called sickle cell anemia, is an inherited disorder affecting hemoglobin, the protein that carries oxygen in red blood cells, according to the National Heart, Lung, and Blood Institute. Normal red blood cells are disc-shaped and flexible enough to pass through blood vessels; in someone with sickle cell disease, the cells are crescent- or sickle-shaped, making them less flexible and allowing them to block blood flow to the rest of the body. The disease affects more than 100,000 people in the United States and 8 million worldwide, and primarily affects people of color. Of those diagnosed, 90 percent are non-Hispanic Black or African American, while an estimated 3 percent to 9 percent are Hispanic or Latino.

Dr Aaron Haubner of the University of Kentucky College of Pharmacy, the lead author of the research, said patient access to "new therapies" for sickle cell raises multiple problems, describing the treatments as "exciting" but "out of reach for most patients."

"Patients with sickle cell disease benefit from coordinated access to hematologists, transfusion and apheresis specialists, nurse educators, care coordinators, and other support services," Haubner said. "In many communities, outside of dedicated centers of excellence, these resources simply aren't available." He said the best way to expand access to transfusion care is to increase the number of comprehensive sickle cell disease centers nationally, which are designed to bring together the expertise and services needed for long-term disease management and preventive care.

Dr Edward Donnell Ivy, chief medical officer at the Sickle Cell Disease Association of America, pointed to an obstacle patients themselves can act on: asking about different treatments. Barriers include patients not being aware that they should ask about certain therapies, he said, adding that it is also important for patients to have doctors who know how to treat sickle cell disease and understand its risk factors. "There's a gap between the number of providers who understand how to treat sickle cell disease," Ivy said.

Ivy described the financial burden of the disease as a significant barrier, noting that roughly 80 percent of sickle cell patients are on Medicaid and face socioeconomic vulnerabilities. Geography also plays a part, he said. Many sickle cell patients live in rural areas and have access only to rural hospitals, and because the disease primarily affects African Americans, it is concentrated in southern states that tend to be more rural, leaving patients with less access to the equipment needed. "And these rural hospitals may be less likely to have access to these therapies," he said. Ivy added that healthcare providers need to be aware of the National Alliance of Sickle Cell Center guidelines and that a network of providers able to discuss treatment strategies should be created.

Dr Shannon Kelly, medical director of the apheresis program at UCSF Benioff Children's Hospital Oakland, said her hospital often receives referrals of patients from other centers because those institutions cannot provide the level of care certain patients need. "We get referrals to my hospital from other large institutions, where even they don't have a dedicated apheresis program that provides automated red blood cell exchange," Kelly said. "The issue is more that this therapy is not at enough institutions and hospitals." She said some patients travel a considerable distance to receive transfusions at her hospital, a hardship for people already living with a chronic disease.

Editor's Summary

The survey of 100 US providers found that fewer than 3 percent of sickle cell patients are recorded as receiving red blood cell exchange despite 91 percent of providers reporting access to it. Reported obstacles include coordination between departments, limited donated blood and unfamiliarity with the procedure, while patients worry about insurance coverage. Researchers and clinicians say expanding comprehensive sickle cell centers and rural access would be needed to close the gap.