A Team-Based Approach to Treating Unmet Needs of Sickle Cell Patients

Sickle cell anemia is a complicated disease, affecting more than a person's physical well-being.

Patients with sickle cell anemia have a large burden of medical complications, including unmet mental health needs. Their disease-profile is complicated, and their condition requires consistent care," says Dr. Temeia Martin, an MUSC Health physician who is board-certified in psychiatry and internal medicine.

As medical director of MUSC Health's Rena N. Grant Sickle Cell Center, Dr. Martin oversees and works side by side with nurses, nurse practitioners, and physician assistants to provide treatment and care for patients from the Lowcountry and around the state. Her dual training in internal medicine and psychiatry has prepared her well to manage her patients' conditions, which can range from mild to severe and for which cure is not widely available.

"When I was in training, I didn't think people could really heal without having their mental health needs addressed in addition to their physical health," she says. "I want to provide whole patient care."

The center, which is the only patient centered medical home in the state certified to treat sickle cell patients, recently observed its 10th year caring for patients. The CDC describes the PCMH model as a team-based approach to delivering high-quality, cost-effective primary care that coordinates patient care across the health system. The PCMH model has been associated with effective chronic disease management, increased patient and provider satisfaction, cost savings, improved quality of care, and increased preventive care.

The goal is to provide and maintain access for patients who need acute care, Dr. Martin says. "Patients come in 5 days a week for IV infusions, pain medications, or hydration. It helps them avoid the ER and a hospitalization, and it provides quality care and consistency of care. Patients have regular appointments with the same provider, and they don't have to wait as long to get an appointment."

Sickle cell anemia is an inherited blood disorder. According to the CDC, approximately 100,000 Americans and more than 4,000 South Carolinians have sickle cell anemia, characterized by abnormal blood cells that are sickle-shaped and sticky and block blood flow throughout the body. The disease has several different forms, depending on its genetic makeup. The most common and most severe is HbSS in which someone inherits a gene from both parents, Dr. Martin says. Two other common subtypes are HbSC and sickle beta-thalassemia. HbSC and beta-thalassemia complications usually don't develop until later in life, but all forms are severe and life-limiting, Dr. Martin says.

Although sickle cell anemia is often regarded as a disease that affects only African Americans, other ethnicities can have sick cell anemia. People from Mediterranean, Hispanic and Caribbean countries also can be affected. "It all depends on your parental heritage," she says.

Because it is present at birth, sickle cell anemia can be diagnosed with a simple blood test, meaning therapy can begin early in life, delaying the onset of symptoms and complications.

No two patients are alike; symptoms and complications include extreme pain, infections, and stroke. And, despite therapy, life expectancy has not changed: The average is 42 for men and 48 for females. Dr. Martin's oldest patient is 70, a happy achievement she attributes to treatment and care. "The better and more consistent their care, the more we can manage their complications, improve their quality of life, and prolong their life, especially during childhood and adolescence."

A bone marrow transplant is the only cure for sickle cell disorder but is associated with a significant risk of death and is not widely available to patients. Gene therapy, however, has shown promising results in clinical trials, and newer therapies that have become available in the last few years have increased treatment options and produced fewer side effects.

In addition to hydroxyurea, a standard treatment for many years, L-glutamine and crizanlizumab have been shown to reduce the number of pain episodes that require hospitalization, and a third, voxelotor, increases hemoglobin levels.

"These therapies are the first new ones in over 50 years, and we're excited that they're available to our patients," Dr. Martin says. "For years, hydroxyurea, which is a form of chemotherapy, was the only treatment, and many patients could not tolerate it. These new therapies have fewer side effects and offer greater options to the patients."

In addition to expanded therapies, Dr. Martin wants to see more providers join the ranks of physicians treating sickle cell patients. We're trying to shift the attitude among health care providers so we can have primary care physicians who can manage sickle cell disorder," she says. "It's a common misconception that only hematologists can take care of sickle cell patients. A very competent PCP or internist can manage sickle cell anemia as well, and we hope more will begin helping patients manage their disease successfully."

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The Adult Sickle Cell Patient-Centered Medical Home is located in Rutledge Tower, on the MUSC Charleston campus. For more information or to make an appointment, call 843-876-0888.