Danielle Jamison was 3 months old when she had her first sickle cell crisis. Now 32, she has struggled her entire life with chronic pain from the disease.
But in rural South Carolina, where Danielle lives, finding a doctor trained to treat her complicated health issues has often been difficult. To see a specialist, she must drive an hour to Charleston and back.
“I know other people who drive three or four hours,” she says. “They end up going to emergency rooms when the crises grow severe. It’s very difficult to get the treatment we need.”
Sickle cell patients across the state are hoping that a new program will address that challenge and become a model for other areas. Developed at the Medical University of South Carolina in Charleston, the SC2 project – which stands for Sickle Cell South Carolina – will use outreach and telemedicine to build a statewide network of clinics and care.
For health professionals, SC2 will offer consultations with specialists who know about the disease and best practices. For patients, it will pave the way to improved, comprehensive services.
A $670,000 grant from The Duke Endowment is supporting the effort.
“We have people living longer with sickle cell disease, but not enough doctors who know how to help them,” says Dr. Julie Kanter, director of SC2 and sickle cell research at MUSC. “The goal here is to make sure that everyone who has this disease in South Carolina gets the right care when they need it.”
Advances – and Challenges
First identified in 1910, sickle cell disease is a hereditary blood disorder that afflicts about 100,000 people in the United States, and as many as 4,500 people in South Carolina. Individuals with the disease don’t have enough healthy red blood cells to carry adequate oxygen throughout their body, which can lead to organ failure, stroke and extreme pain.
Not many years ago in the United States, most people with sickle cell disease died before their 20th birthdays. Today, with advances in newborn screening, education and treatment, many live into their 40s and beyond.
But despite improvements in childhood care, sickle cell disease remains life-limiting. Patients require comprehensive attention that includes blood testing, transfusions and close monitoring of medications. Only one drug, hydroxyurea, is FDA-approved to reduce the need for blood transfusions and the frequency of pain crises – and many physicians aren’t familiar enough with the drug (or the disease) to prescribe it.
At MUSC, Dr. Kanter believes more could be done. Trained as a pediatric hematologist oncologist, she now focuses on sickle cell disease care for all ages. She designed SC2 – which stands for Sickle Cell South Carolina – as a combination of in-person outreach clinics and telehealth. It will launch in the Lowcountry, where the need is highest, but plans call for expansion across the state.
Connecting to Expertise
The hub of the SC2 network is the Lifespan Comprehensive Sickle Cell Center at MUSC. With Dr. Kanter as director, a program coordinator will facilitate referrals and patient navigation; a social worker will link patients to local resources.
Partners for the first year are Palmetto Health Richland and Tidelands Health System, with Beaufort Memorial Hospital and Greenville Health System expected to join in the future. Each partner will provide clinic space and support from doctors and nurses on staff.
At Beaufort Memorial, Dr. Stacey Johnston says SC2 could offer the “perfect solution” to a daunting challenge.
“A lot of times, these patients fall through the cracks,” she says. “This model would bring specialized expertise right into our community and get them the access they need for better continuity of care. If we can help them get their pain better controlled as an outpatient, we’re hoping to see a decline in patients who are discharged, and then return to the hospital a week later when their pain has become severe again.”
Dr. Kanter has set clear goals for the project:
- Of the 800 enrolled patients, 60 percent will show improvement in pain management; 75 percent will have individualized care plans
- 200 new patients will be identified to begin hydroxyurea
- Up to 50 patients will be identified to begin transfusion therapy to reduce the risk of stroke
- Patients with three or more hospital visits a year will have a 50 percent decrease in days hospitalized
For Danielle Jamison, SC2 could mean fewer health crises, fewer trips to the emergency room, and increased access to quality care. Her sickle cell disease won’t go away, she says, “but this will give people like me a better chance to lead a fuller life.”
Stacy E. Warren
Program Officer, Health Care