Danielle Jamison can’t remember a time when she didn’t live in pain. The 36-year-old Colleton County resident was an infant when she was diagnosed with sickle cell disease, a rare blood disorder that causes damage to blood vessels and organs.
“I was about 4 months old when my parents noticed I was getting very puffy,” Jamison says. “My arms and legs were swollen and I wouldn’t stop crying.”
They rushed her to the Medical University of South Carolina (MUSC) in Charleston, an hour and a half from their home in the small, rural community of Islandton. Tests revealed she had sickle cell anemia, the most severe form of the disease.
It was then her parents learned they both carried the sickle cell gene or trait, resulting in a 1 in 4 chance their children would have the disease. Jamison’s older sister inherited the trait but does not have the disease. Her younger sister wasn’t so lucky. She also suffers from sickle cell anemia.
Read More: Sickle Cell Anemia Symptoms: What You Need to Know
“As children, one or the other of us was always sick,” says Jamison, who has spent most of her life in and out of hospitals. “My mother had to quit her job to take care of us. I remember being in the hospital with my sister in the same room and my mother sleeping in a rollaway cot between us.”
As many as 4,500 people in South Carolina — the vast majority of them of African-American descent — are afflicted with sickle cell disease, a group of disorders that cause anemia, shortness of breath and fatigue. In sickle cell patients, the hemoglobin inside red blood cells is damaged, causing it to twist and break down. When these sickle-shaped cells clump together and block small blood vessels, it can trigger a severe attack called a sickle cell crisis.
Days or Weeks of Painful Crisis
Jamison describes the pain crisis this way: “It’s like when you stub your toe and a sharp pain shoots through your entire body — only imagine it 10 times worse. The pain won’t stop. It’s a constant throbbing that’s excruciating, and it can last for two to three days or weeks.”
During these painful episodes, tissues and organs are deprived of oxygen, which can lead to serious complications, including organ damage, heart failure and stroke. In her freshman year at Virginia State University, Jamison had to have emergency surgery to remove her gallbladder. She also suffered from avascular necrosis in the hip, which results from a loss of blood supply to the bone. A common complication of sickle cell disease, the necrosis led to the deterioration of the bone, requiring hip replacement surgery.
Any number of things can trigger a crisis, including stress, overexertion, dehydration, cold weather and sudden temperature changes, like swimming in cold water on a hot summer day.
In her early years, Jamison was treated by a Beaufort Memorial pediatrician at Beaufort Pediatrics. As an adult, she sees a hematologist, an internist who receives extra training in disorders of the blood, bone marrow and lymphatic system. Seven years ago, she found a hematologist who specializes in sickle cell disease and it changed her life.
Dr. Julie Kanter-Washko had just joined MUSC as director of sickle cell disease research.
Local Access to Life-Changing Treatment
“She got me on a new regimen,” Jamison says. “It made a huge difference.”Jamison couldn’t have been more thrilled when Dr. Kanter-Washko opened a sickle cell clinic at Beaufort Memorial in the spring of 2018. The clinic offers ongoing disease-modifying therapy with individualized treatment plans based on the latest evidence-based practices. Therapies include hydroxyurea management, blood transfusions and acute pain management, as well as additional research opportunities.
“It’s so convenient,” says Jamison, who still lives in Islandton. “It’s a 45-minute drive rather than an hour and a half to Charleston. And I feel so much more at home there. I’ve been going to Beaufort Memorial since I was a child.”
Jamison is one of 45 patients currently treated at the hospital’s sickle cell clinic. Although Dr. Kanter-Washko recently moved to Birmingham to serve as director of the University of Alabama Adult Sickle Cell Clinic, she continues to visit the Beaufort clinic every three months to see her patients and offers telemedicine appointments as needed.
Read More: Managing Sickle Cell Disease
In January 2018, Dr. Kanter-Washko offered Jamison the opportunity to participate in a clinical trial studying the drug Adakveo, which had been shown to decrease pain crisis in nearly half of patients. The drug, approved by the FDA in November 2019, is administered intravenously once a month.
“After three or four doses, I started to notice a difference,” Jamison says. “I had a lot more energy and was able to control my pain at home with my prescribed medications. I never imagined I would have this kind of quality of life. I haven’t been admitted to a hospital in three and a half years.”
Annemarie Gault, NP-C, the Beaufort Memorial sickle cell clinic’s certified nurse practitioner, has seen similar results with three of her other patients on Adakveo.
“It has greatly reduced ER visits and hospital stays,” she says. “But every patient is different even if they have the same disease. Other new medications are coming out that also show promising results.”
Jamison, who remains in the Adakveo study, continues to go to Beaufort Memorial’s sickle cell clinic once a month for lab work and pain management.
“Annemarie is so awesome,” Jamison says. “She is just as caring and passionate about her sickle cell patients as Dr. Kanter-Washko.”
For more information on Beaufort Memorial’s sickle cell clinic or to make an appointment, call 843-522-5351.
