New approach to reaching sickle cell patients across state

Julie Kanter, M.D., treats many sickle cell patients who haven’t seen a blood doctor in more than 10 years. “They’re on no disease-modifying therapy and have no idea what they need to do to better care for themselves,” she said.

As director of sickle cell research at MUSC, she finds the situation ridiculous. Many doctors do. But not all doctors develop a statewide solution that may soon become a national model. With the help of a grant from the Duke Endowment, Kanter hopes to build a statewide network of clinics specializing in the treatment of SCD. The South Carolina Sickle Cell Network will enhance access to care for people living with sickle cell disease (SCD) and help educate local providers on how to treat and manage the disease.

“Using a hub and spokes model, we’re going to create new, small SCD centers throughout South Carolina,” Kanter said. “These centers will be connected to the hub at MUSC both through outreach and telemedicine.”

A project like this hasn’t been tried before in South Carolina or anywhere else, and rural sickle cell patients stand to benefit  tremendously.

A hereditary blood disorder, SCD afflicts approximately 100,000 people in the United States, the vast majority them of African-American decent. Another 3 million carry the sickle cell trait. 

Those with the disease have sickle-shaped hemoglobin – the protein in red blood cells that carries oxygen throughout the body – and over time, it can damage blood vessel walls, decreasing blood flow to muscles, bones and organs and starving them of oxygen. SCD causes severe pain akin to the pain of frostbite.

Mamie Taylor, a patient poised to benefit from Kanter’s project, knows all too well how severe the pain can be. “It hurts very badly. I get sick when I get too cold, too hot or when a bad storm comes,” she said. “It’s painful and scary.”

Taylor lives in Andrews, South Carolina, and travels to MUSC at least once a month for a blood transfusion. The drive is two hours each way. She leaves early so she can arrive before 8 a.m. and often doesn’t head home before late afternoon.

Her experience is typical of many rural sickle cell patients, said Kanter. A chronic disease, sickle cell requires a sustained, comprehensive approach to care that includes blood testing, transfusions and close monitoring of medications. Anyone with SCD not lucky enough to live near a specialty clinic either must make a long drive or make do with potentially inadequate medical care at a local hospital. 

Because the disease’s main symptom is pain, many patients are stigmatized as drug seekers and don’t receive the proper treatment or pain medications at the proper doses. 

“Unfortunately, pain can’t be easily measured or treated, and even medical professionals lack proper education about SCD. I’ve had unfortunate experiences with many hospitals across the state,” Kanter said. “If patients just wanted drugs, they could get them cheaper and easier on the street.”

Another problem Kanter mentioned was the misdiagnosis of other diseases in patients with SCD. “Often, patients are told that whatever they walk in the door with is just a symptom of SCD, that there’s nothing the doctor can do. I have SCD patients now who have been diagnosed subsequently with rheumatoid arthritis, lupus, sarcoidosis. All these patients were initially told, ‘Oh, it’s just your sickle cell.’”

These are issues Taylor has experienced firsthand. Before she started seeing Kanter last March, Taylor said she was in and out of the hospital every week. Doctors didn’t listen. Her doses of pain medications were far too low to be effective, and she wasn’t receiving any long-term treatment for SCD. Many times, she said, doctors sent her home still in pain. 

“They didn’t know what to do,” she said. “The doctors said, ‘We don’t really deal with sickle cell patients.'”

Since Taylor started seeing Kanter, her condition is being managed much more effectively. She’s only been admitted to the hospital once in the past year.

“These patients have multi-system problems that don’t fit easily into the typical family medicine clinic,” Kanter said. “The way medicine is these days, patients may only get 10 minutes with their doctor. You can’t take an incredibly complicated patient that hasn’t been seen in 10 years and diagnose them in 10 minutes.”

Kanter said her biggest goal is to make sure any one living with SCD has access to a specialist. To meet this goal, her project will harness the efforts of stakeholders across the state – patients, physicians, community-based organizations and the Department of Health and Environmental Control – to coordinate care and set a common agenda. These groups will also establish measures of success to accurately gauge South Carolina’s progress in addressing this disease as a matter of public health. 

“The methodology is called collective impact,” she explained. “We want to merge what everybody’s already doing, set specific, measurable goals, and make sure we’re not duplicating efforts so that we can use our limited funding as well as possible.”

Currently, for example, managed-care organizations, hospitals and the Sickle Cell Disease Foundation all have their own case management programs for SCD. 

“These programs don’t share the same goals or the same measures of success,” Kanter said. “And often they’re spending money to do the exact same thing.”

Another aspect of the project involves partnering with regional health care providers to better serve rural areas of the state. Current partners include Palmetto Health Richland, Tidelands Health Systems, Beaufort Memorial Hospital and Greenville Health Systems. Kanter also hopes to find a partner in the Florence area. Each of these groups has agreed to provide substantial resources, including clinic space and nursing and physician support.

“These institutions have agreed that we need to improve the care of individuals living with SCD and are working with MUSC to develop a sustainable treatment model across the state,” Kanter said. “In Georgetown, for example, we’re working with Tidelands to establish a clinic that doesn’t exist yet. It will be run by an advanced practice provider with help and support from emergency room and Med-Peds (a specialty that combines internal medicine and pediatrics) doctors. I’ll come monthly to do comprehensive care and establish individualized treatment plans, and I’ll also be available when needed by telehealth for acute care.”

The Duke Endowment grant brings much-needed funding to a historically underfunded disease, Kanter said. “We’ve known about SCD for 106 years, and we have only one FDA-approved drug. That’s pretty pathetic.”

The reasons for this, Kanter said, are twofold. Compared to other genetic diseases such as cystic fibrosis, which affects predominantly people of Northern European ancestry, SCD suffers from a significant lack of funding. And because of a history of abuse in research, many African-Americans feel wary of participating in research studies, studies that may lead to the development of new drugs. The Tuskegee experiment, in which researchers with the U.S. Public Health Service intentionally infected poor African-American sharecroppers with syphilis, casts a long shadow to this day, Kanter said. 

That SCD, a complex disease in its own right, is further complicated by issues of race and poverty is a significant challenge, but not one that can’t be conquered. “The stigma of this disease can very easily become discriminatory,” Kanter said. “But I’m very grateful to the Duke Endowment and to the MUSC leadership for recognizing what an important project this is. We as an institution, and as a state, have an opportunity to become role models in improving care for sickle cell disease.”