Sickle cell patients are missing out on an effective treatment despite widespread US hospital access to the technology, new research has found.
That treatment is called a red blood cell exchange, a procedure that discards a patient’s damaged red blood cells while simultaneously mixing the patient’s remaining plasma, platelets and white blood cells with red blood cells from a donor before returning them to the body.
But a new nationally representative survey of 100 US-based healthcare providers who actively manage at least one patient with sickle cell disease shows fewer than 3% of patients have been recorded receiving a red blood cell exchange even though 91% of surveyed providers report access to it.
The survey found that providers encountered barriers in administering the treatment, which most commonly included challenges coordinating between different medical departments, a limited supply of donated blood and a general lack of familiarity with the procedure. Only 5% of health providers surveyed had no barriers in delivering this type of treatment, the research found.
Among sickle cell patients, the survey found that a major concern was if red blood cell exchange therapy was covered by their health insurance.
Sickle cell disease, which is 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 the blood vessels; someone with sickle cell disease has crescent- or “sickle”-shaped blood cells, meaning they are less flexible and can block blood flow to the rest of the body.
The disease, which affects more than 100,000 people across the United States and 8 million people worldwide, primarily affects people of color. Of those diagnosed with the disease, 90% are non-Hispanic Black or African American while an estimated 3%-9% are Hispanic or Latino.
Dr Aaron Haubner of the University of Kentucky College of Pharmacy, lead author of the research, emphasized the various issues surrounding patient access to these “new therapies” for sickle cell, calling them “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,” he said. “In many communities – outside of dedicated centers of excellence – these resources simply aren’t available.”
He believes the best way to expand access to transfusion care is 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,” Haubner said.
Dr Edward Donnell Ivy, chief medical officer at the Sickle Cell Disease Association of America, also pointed out an obstacle that patients themselves have control over: asking about different treatments.
“[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 noted the financial impact of the disease as a significant barrier, disclosing that roughly 80% of sickle cell patients are on Medicaid and face socioeconomic vulnerabilities. He also noted that geography plays a part in care.
“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,” he said. “And these rural hospitals may be less likely to have access to these therapies”.
He continued: “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.”
Dr Shannon Kelly, medical director of the apheresis program at UCSF Benioff Children’s Hospital Oakland, said that her hospital often receives referrals of patients from other centers because they cannot provide the same level of care that certain patients need.
“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.
“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.
“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.”
