This is part one of a four-part series on Atrium Health's innovative, holistic approach to sickle cell disease care.
About a generation ago, nearly half of the children who had sickle cell disease didn't live past the age of 18. Recent decades, though, have brought advancements in treatments, medications and awareness. People with the disease now live longer and better lives than ever before.
But with the good news comes a new question: How can we continue to improve life for people who will spend decades facing this chronic illness? Atrium Health is at the forefront of tackling this challenge, offering a holistic, patient-centered approach to sickle cell disease care.
What is Sickle Cell Disease, and Who's at Risk?
Sickle cell disease is an inherited disorder that causes red blood cells to be misshapen. Healthy red blood cells are smooth and round, and they're able to carry oxygen throughout the body. But for people with sickle cell disease, red blood cells take on the shape of a C (like a farm tool called a sickle). This leads the red blood cells to stick to each other, forming clumps that block proper blood flow.
When red blood cells can't flow as they should, it can lead to chronic pain, stroke, infection and even organ failure. Sickle-shaped red blood cells also break down faster than healthy cells, causing a shortage of red blood cells known as sickle cell anemia. Sickle cell disease affects approximately 100,000 Americans, with a disproportionate impact upon people of African and Hispanic ancestry are disproportionately affected by the disease.
Suffering from a painful chronic disease disrupts daily life. People with sickle cell disease often require frequent doctor visits, hospitalizations and prescriptions to manage their condition. It can be difficult to go to work or school, and often comes with financial hardship given the level of care that's required. Yet this debilitating disorder often has no outward signs, so the people who deal with it also face stigma about the disease.
It's hard to have a chronic illness, says Ifeyinwa Osunkwo, MD, MPH known as Dr. Ify to her patients who leads Atrium Health's comprehensive sickle cell disease program at Levine Cancer Institute. Sickle cell disease will be there all of your life: 40, 50, 60, 70 years. However long you live, you have work extra hard to take care of yourself on an ongoing, regular basis.
Innovations in Sickle Cell Disease Treatments
During the past few years, Atrium Health has focused on improving outcomes and care for patients with sickle cell disease. The organization offers a comprehensive sickle cell disease clinic that connects patients with medical providers, a psychotherapist, a social worker, a case manager and dedicated nurses who can assist with all aspects of life with the disease.
We use a multidisciplinary model to determine our patients main issues, says Dr. Ify. Then we pull from system resources to help address each and every need that the patients have. It's not just about giving a pill, or a prescription for a medication – it's a holistic approach to managing the disease.
The only way to cure sickle cell disease is through a stem cell transplant, where a person with sickle cell disease has their stem cells replaced with healthy cells from a donor. And while this treatment has been around since the 1980s, it hasn't been widely available. In the past, donors had to be an exact match, meaning they're the patient's full sibling, with the same HLA type a system of genes that one in four siblings share and without sickle cell disease. These strict criteria meant that only 18 percent of eligible patients could get a transplant.
But through its participation in a national study, Atrium Health has learned that a transplant with a half-matched donor called a haploidentical transplant – can be effective as well. This could mean that almost every eligible patient could be matched with a donor.
Another common treatment option for patients who have severe or chronic organ complications is a blood transfusion. A donor's healthy red blood cells are given to the patient, reducing the amount of sickle cells in their blood. And while transfusions are now safer and more effective than ever, they still result in potentially harmful amounts of iron in the blood.
But recently, a safer option has emerged: exchange transfusions. Exchange transfusions draw sickle cell patients blood out as they receive non-sickle cell blood, decreasing the amount of sickle cells without creating excess iron. When given monthly, exchange transfusions can significantly reduce the risk of first and second strokes and improve quality of life for children and adults with sickle cell disease.
The exchange transfusion is a marvelous way of keeping the amount of sickle cells in the blood reduced, says Daniel McMahon, MD, director of the pediatric sickle cell program at Atrium Health's Levine Children’s Hospital (LCH). It's relatively quick and easy.
LCH has partnered with Atrium Health's Nephrology department to establish a program for regular exchange transfusions. The same principles that have guided nephrologists work with kidney disease patients are now helping sickle cell disease patients, too.
By investing in its sickle cell program, Atrium Health has learned more about what patients need and how best to deliver that care to them. The results have been undeniable and immediate and there's more to come.
Patient Spotlight: DeJuan Minnegan
DeJuan Minnegan is only 17, but hes already a veteran of sickle cell disease treatments. He's known since he was a small child that he had the disease, and he's been taking medications for most of his life.
Recently, DeJuan began to receive exchange transfusions at LCH. There is one downside to these transfusions for him the needles are bigger than regular transfusions, he says but the positives far outweigh those big needles. His sickle cell levels are lower, and he's even been able to stop taking several of his medications.
DeJuan knows that sickle cell disease impacts his day-to-day life. He knows it causes fatigue that slows him down. He imagines that without the disease, he would've played football like his friends. But overall, he says he's proud to have sickle cell disease. He's proud to manage his health, and he feels the support of his doctors at LCH and his teachers at school.
DeJuan aspires to own his own business one day. In the meantime, the high school junior offers words of support for other young people with sickle cell disease who are just beginning their treatments.
Don't be nervous. The doctor's got you, DeJuan says. It's going to help a lot.
His mother, Mary, credits the blood exchange for giving her son a better life, and credits the staff for helping their family navigate this chronic illness.
There are some people who work for this hospital who have really gone above and beyond to help my family, Mary says. Things you wouldn't believe, going way past the level of expectations. It's like a family environment.
Continue reading this series on sickle cell disease:
Part 1: Game-Changing Advancements in Care
Part 2: Improving Long-Term Care for Sickle Cell Disease
Part 3: Innovative - and Proven - Therapies
Part 4: An Integrated Pharmacy Model
Read More About Atrium Health's Sickle Cell Program and Download the eBook Today.
Learn more about sickle cell disease care at Atrium Health. Call us at 704-381-9900 to begin a conversation about our program and how we can support your health.
