Winter 2008: Dynamic Forces
A Blood Bond
Patients with sickle cell anemia find hope through a center designed just for them
By Mary J. Loftus
After a lifetime of describing her pain, Michelle Jones, who was diagnosed with sickle cell anemia when she was two years old, is exceptionally good at explaining what acute episodes of the disease feel like. “It’s like there’s a rope wrapped around my waist that’s attached to two tugboats, and the boats are pulling in opposite directions,” says Jones, a substitute teacher from Lawrenceville. “I would curl up in a fetal position on the cool tile in my bathroom. I was already feverish, so the coolness was the only thing that gave relief.”
As a child, Jones would never admit to her mother when she was feeling ill, preferring instead to keep playing with her friends despite recurrent high fevers. “I would run past her, and she would actually feel the heat radiating off me.”
Now that she’s an adult, her sickle cell crises usually manifest as jaw and chest pain, making it excruciating even to draw a deep breath.
Sickle cell anemia afflicts millions around the world—especially those of African, Mediterranean, and Indian descent—and about eighty thousand people in the United States, mostly African Americans. The inherited disorder affects the blood’s hemoglobin, which produces stiff, misshapen red blood cells that deliver less oxygen and can disrupt blood flow, resulting in joint and organ damage and potential clots and strokes.
But Jones—who, like many with the disease, refers to herself as a “sickler”—remains optimistic, due in part to advances in treatment and the specialized care she receives at the Georgia Comprehensive Sickle Cell Center at Grady Memorial Hospital in Atlanta, the nation’s first twenty-four-hour acute sickle cell center. “Actually, I’m doing great,” she says. “I may not be able to run a marathon, but I have a full life.”
Jones travels, volunteers, teaches, and raises her twelve-year-old, Jessica, who was tested for sickle cell anemia at birth and was found to have the trait but not the disease. Part of her well-being stems from taking vitamins such as folic acid, essential in producing red blood cells, and a promising new medicine, hydroxyurea, recently approved to treat sickle cell after it was found to reduce the frequency and intensity of attacks.
Sickle cell crises are extremely painful and can be life-threatening. They require immediate treatment and possible hospitalization, which is why an around-the-clock facility designed specifically for sickle cell patients is so vital. In fact, the Grady center was created in response to the death of a sickle cell patient who succumbed while waiting for treatment in an emergency room during an acute crisis. “We have our own admissions, emergency room, and short-stay center,” says James Eckman, medical director of Grady’s Sickle Cell Center since it opened in 1985. “You can get admitted in ten to fifteen minutes, versus three hours in an ER. And the more quickly you treat the pain, the more likely it is to be controlled.”
Eckman, an Emory professor of medicine and of hematology and oncology at the Winship Cancer Institute, was honored in 2006 by the Georgia General Assembly for his sickle cell work. “With few resources, Dr. Eckman obtained grant funding to support the salaries of a genetics nurse, a social worker, and a clinical nurse specialist in psychiatry as the beginning of a multidisciplinary team to treat sickle cell disease,” the resolution reads.
The Grady center has cared for more than two thousand patients, from pediatric to adult, and participates in clinical research projects. “We provide all of our patients’ health care needs, from primary care through crises,” Eckman says. “This disease puts such a burden on someone’s life, we want to facilitate as few visits to health care facilities for them as we can.”
In 1998, Emory doctors from the Departments of Pediatrics, and Hematology and Oncology, performed the first successful “unrelated donor” cord blood transplant to cure high-risk sickle cell disease. Twelve-year-old Keone Penn of Snellville, who had sickle cell disease since birth, had a stroke at age five and received regular blood transfusions through a chest catheter for anemia. When severe complications and pain continued, his doctors began considering stem cell transplants. When no matching donor was found, they decided to try a cord blood stem cell transplant at Children’s Healthcare of Atlanta at Egleston—the first ever performed on a sickle cell patient.
Penn had chemotherapy to eliminate his own bone marrow cells before receiving a transfusion of blood donated to a New York cord blood bank. His entire blood system changed from type O to B as he took on the blood type of the anonymous donor. After a difficult recovery during which he developed graft-versus-host disease, Penn’s new stem cells began producing normal red blood cells. A year after surgery, he was considered cured and graduated from high school in 2004.
In May 2007, Georgia Governor Sonny Purdue signed into law the Saving the Cure Act, which authorizes the Georgia Newborn Umbilical Cord Blood Bank to collect and store stem cells. Penn—who inspired the law—was there for the signing. Each time he speaks to the press, he says “thank you” to the anonymous donor who provided his own cure.
A blood marrow transplant remains risky and costly, however, leaving most sickle cell patients with the need for constant maintenance of a chronic disease. With proper care, patients can expect to have long, full lives.
As Eckman spoke with Jones on a recent afternoon in the clinic, he told her that the oldest sickle cell patient he is caring for is eighty-two.
“Many patients are now living into their seventies and eighties,” he said.
Jones smiled back at him. “Oh, I am planning on it. My grandmother just turned ninety-one. We’re long-lived in my family.”