Disease primarily affects Black patients in rural and Southern states
In red blood cell exchange, a sickle cell patient’s damaged red blood cells are discarded while the patient’s remaining plasma, platelets, and white blood cells are mixed with red blood cells from a donor before being returned to the body. The procedure is effective and widely available in US hospitals, but a national survey of 100 healthcare providers who actively manage at least one sickle cell disease patient found that fewer than 3% of patients had been recorded as receiving it, according to The Guardian, which reported the survey findings Sunday.
The gap between access and use is the survey’s central finding. While 91% of providers reported having the technology available, the providers identified several barriers to delivering it. 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% of providers surveyed reported no barriers in delivering the treatment. Among sickle cell patients, the survey found, a major concern was whether red blood cell exchange therapy was covered by their health insurance.
Dr. Aaron Haubner of the University of Kentucky College of Pharmacy, the survey’s lead author, emphasized the gap between the existence of treatments and patient access. “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.”
Haubner said the best way to expand access to transfusion care was to grow the number of comprehensive sickle cell disease centers nationally. “These centers are designed to bring together the expertise and services needed to support long-term disease management and preventive care,” he said.
Dr. Edward Donnell Ivy, chief medical officer at the Sickle Cell Disease Association of America, identified additional barriers, including a gap in patient awareness. “[Other] barriers include patients not being aware to ask about certain therapies, but it is also important for patients to have doctors that are knowledgable about how to treat sickle cell disease, about the risk factors too,” Ivy said. “There’s a gap between the number of providers who understand how to treat sickle cell disease.”
Ivy pointed to the financial impact of the disease as a significant obstacle, noting that roughly 80% of sickle cell patients are enrolled in Medicaid and face socioeconomic vulnerabilities. Geography, he said, compounds the problem. “A lot of sickle cell patients live in rural areas and so only have access to rural hospitals. If you look at the distribution of sickle cell disease, it primarily affects African Americans who tend to be in states in the south that can be more rural, and so have less access to some of these equipment,” Ivy said. “And these rural hospitals may be less likely to have access to these therapies.”
Ivy said providers needed better awareness of existing treatment guidelines. “We need to make sure healthcare providers are aware of the National Alliance of Sickle Cell Center guidelines, and creating a network of providers than can discuss treatment strategies,” he said.
Dr. Shannon Kelly, medical director of the apheresis program at UCSF Benioff Children’s Hospital Oakland, said her program often receives referrals from other large institutions. “We get referrals to my hospital from other large institutions, where even they don’t have a dedicated apheresis program [involving a centrifuge machine that processes blood from the patient’s body] that provides automated red blood cell exchange. The issue is more that this therapy is not at enough institutions and hospitals,” Kelly said.
Patients who do reach programs like UCSF’s often travel significant distances, Kelly said. “We see patients who do travel from some distance to receive their transfusions with us. It’s a hardship on them because already they have a chronic disease that can be debilitating and then they have to travel a long distance,” she said.
Kelly said expanding red blood cell exchange to more sites was a question of maintaining clinical competencies. “Ideally this would be a therapy that could be offered at more sites, but if you don’t have a decent number of sickle cell patients in your area it’s very difficult to maintain the competencies of this therapy. It would be very hard for some rural centers to maintain a program like this,” she said.
Sickle cell disease, also called sickle cell anemia, is an inherited disorder that affects 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, making them flexible and easy to pass through blood vessels; in sickle cell disease, the cells are crescent-shaped, less flexible, and can block blood flow. The disease affects more than 100,000 people in the United States and 8 million worldwide.
Among US patients, 90% are non-Hispanic Black or African American, while an estimated 3% to 9% are Hispanic or Latino, according to the National Heart, Lung, and Blood Institute.