Managing Sickle Cell Disease
November 29, 2017
"Having a primary care physician makes it easier to keep track of the disease. With a chronic condition like sickle cell, it's really important because things can go off the rails anytime."
Despite having painful joints, Leah-Zaire Liburd didn't let her sickle cell disease stop her from climbing trees as a kid and cheerleading in high school. But at the age of 25, after having the first of her two children, the inherited red blood cell disorder began to play havoc with her health.
Beaufort Memorial now offers a Sickle Cell Clinic to serve patients in Beaufort and Jasper Counties.
Teaming up with a Primary Care Provider
Every three to four months, she would become so fatigued and short of breath she would need blood transfusions to boost her red blood cell count. After four years in and out of the hospital, she decided to quit her job and focus on improving her health.
Liburd moved to St. Helena Island and began being seen by a provider at Beaufort Memorial Lady's Island Internal Medicine. She was put on new medication that has helped keep her hemoglobin in check.
"I had been taking a holistic approach and self-monitoring," she said. "Having a primary care physician makes it easier to keep track of the disease. With a chronic condition like sickle cell, it's really important because things can go off the rails anytime."
The Facts on Sickle Cell Disease
Some 4,500 residents of South Carolina live with sickle cell disease, the most common inherited blood disorder in the U.S.
Parents can pass along all kinds of wonderful traits to their children. The mutation for sickle cell disease is not one of them. This inherited gene produces a defective kind of hemoglobin—the molecule in red blood cells that takes oxygen from the lungs to all parts of the body.
Shaped like a sickle rather than the usual circle, the mutated red blood cells get stuck in the walls of tiny blood vessels, clogging blood flow. They also die early, which causes a constant blood cell shortage. Patients with the debilitating disease can suffer severe joint pain, infections, organ damage and stroke.
Most patients are diagnosed at birth during routine newborn screening tests. The usually begin to show symptoms around 5 months of age.
How to Stay Ahead of Sickle Cell Disease
To date, the only potential cure for sickle cell disease is bone marrow transplant. Because the surgery is very risky and can have serious side effects, most sickle cell patients simply manage the disease to relieve pain and prevent complications.
Treatment varies based on the individual's symptoms. It can include blood transfusions, intravenous therapy and medications to alleviate pain. For patients with severe sickle cell disease, hydroxyurea may be prescribed to reduce painful episodes (called crises) and the need for blood transfusions, and to prevent acute chest syndrome, a life-threatening condition that can cause chest pain, coughing, difficulty breathing and fever.
Staying well hydrated is critical to avoiding the onset of a crisis. Patients are advised to drink eight to 10 glasses of water each day. If they become dehydrated, they may receive large volumes of fluids intravenously.
This Beaufort Memorial patient story first appeared in the Winter 2017 issue of Living Well.