- NO COMPLAINER: Aaliyah Madyun.
Aaliyah Madyun of Little Rock is a petite 17-year-old who takes care not to overdo exercise, get cold or get dehydrated. She says hers is a normal life, and that she takes part in all the “usual activities,” though she’s home-schooled partly for reasons to do with her health. She does not complain.
She has sickle cell anemia, but she makes it seem like nothing. Yes, she had a “crisis” about three months ago, had to go to the hospital and be hooked up to IV painkillers, and it took a week to resolve, but it was the first one she’d had in a year and a half. She shrugs it off. But asked how painful her crisis was on a scale from one to 10, she looked at her brother and they laugh. “Ten or higher,” she says. “We’re glad she’s strong enough [to take it],” he says.
Aaliyah is downplaying a disease that can cause bouts of excruciating pain as well as stroke, pneumonia, meningitis, blood poisoning and kidney failure. Sufferers can be jaundiced, have swollen hands, suffer from gallstones and leg ulcers, have orthopedic problems. They may need regular blood transfusions or apheresis (the replacement of sickled cells with healthy red cells) and all the complications that come with those procedures. Because narcotics are used to control pain, it can bring on drug dependency in adults.
One in 58 babies born in Arkansas this year will have sickle cell trait. That doesn’t mean they’ll have sickle cell anemia; it does mean that they carry a recessive gene that, paired with another, will cause anemia in their descendants. Arkansas now requires every newborn be screened for the trait. The anemia causes problems from the beginning of life, so parents need to know. Family compliance with medical instructions and understanding therapies are crucial to managing the disease in children. Genetic counseling is crucial to reducing the incidence of the blood disorder.
On Friday and Saturday, Sept. 8 and 9, Arkansas Children’s Hospital and the University of Arkansas for Medical Sciences will address these issues at its first sickle cell educational conference for doctors, nurses and sickle cell families. Hematologist-oncologist Suzanne Saccente, Aaliyah’s doctor, will speak along with Allan Platt of the Georgia Comprehensive Sickle Cell Center in Atlanta, who is the author of “Hope and Destiny: A Patient’s and Parent’s Guide to Sickle Cell Trait.” The conference will be held in the Children’s Hall on the ACH campus.
The red blood cells in people with sickle cell anemia respond to immune system stresses by changing shape from round to angularly bent — Dr. Saccente said her children are more familiar with bananas than sickles and that’s what they liken the shape to. Banana-shaped red blood cells stack up and block blood vessels and capillaries, cutting off oxygen to target tissues and causing inflammation and pain. Cold weather, dehydration, too much exercise — anything that increases viscosity in the blood — can trigger a crisis. The mutation in the hemoglobin gene that causes the trait is an adaptation that protects against malaria, which is why it’s found in people from Africa, India and countries bordering the Mediterranean, where malaria, a mosquito-borne disease, is common.
Five years ago, Aaliayah was hospitalized for a week after an infected spider bite caused her leg to swell with cellulitis; she could not walk. She required two blood transfusions before her immune system could battle the infection. She won the fight, but infections are the leading cause of death in children with sickle cell.
Aaliyah’s never had a stroke, and so she does not require the once-a-month (or more) apheresis that 6-year-old Ashley Minix has undergone at Arkansas Children’s Hospital since she was 2 years old. The procedure, which takes several hours, replaces 75 percent of Ashley’s cramped blood cells with healthy donor cells. That means that she’s able to avoid pain crises that children who haven’t had strokes will endure, an irony that was not lost on Ashley’s mother, Keili Minix.
Minix has a second daughter with sickle cell anemia — Jordan, 10 — and until Jordan became a participant in the SIT (Silent Infarct Transfusion) study that Saccente is participating in, she didn’t qualify for apheresis. As part of the study, however, Jordan had a brain MRI, and it showed she’d suffered a silent stroke — one with no visible effects. She was randomly selected in the double blind study to be part of the group that would get apheresis.
Saccente is also participating in the ASSERT trial of a new drug to reduce pain in sickle cell patients; “the beauty of it is it’s not a narcotic,” Saccente said. The drug works by blocking potassium release.
Another drug has made life easier for patients who, because of repeat transfusions, have too much iron in their blood. Exjade is a once-daily oral medicine to help rid the body of iron. Until Exjade was approved, all patients with iron overload had to be hooked up to a chelation pump 10 to 12 hours six to seven days a week — forever.
While transfusion is the standard of care in treating sickle cell pain, some people refuse blood. Their fears are not completely unfounded, Saccente said; there could be agents for yet-to-be recognized diseases in donated blood. Too, while the hospital screens blood to find the best match for donors and patients, just matching blood type is not enough. Ashley, for example, has created what is called a “warm antibody” in her blood as a reaction to antigens in donor blood; the protein breaks down red blood cells, counter to the goals of the apheresis.
What would help Ashley, and other sickle cells anemics who get transfusions or apheresis, is blood donated from African-Americans, whose blood would contain fewer of the antigens found in blood from people of European descent. Keili Minix, a legal secretary whose flexible schedule allows her to make two trips every month with her daughters to Children’s Hospital for their monthly blood work and follow-up apheresis procedures (and her son to football practice four times a week — “my friends think I should be a basket case,” she said), may soon volunteer for a new job: to get the word out to the African-American community that blood donors are needed. She plans to start at her church, Gaines Street Baptist, where her husband, Jim Minix, is pastor.
For more information on the conference, call 682-9900. Admission is free for the public, $10 for med students and $25 for health professionals.