Early awareness key to managing symptoms
Myiesha Demery has come a long way. Born in the Virgin Islands, she moved to Boston when she was 5 years old in part because of her medical condition.
She was diagnosed with sickle cell disease — both her parents carried the trait — but doctors in St. Thomas could offer little in the way of treatments. She was told to simply eat foods that were thought to boost the oxygen in her blood.
Quite naturally, her condition worsened and the painful flare-ups occurred as often as three times a week.
“It can be sudden,” she said. “It can start in one spot and quickly spread to every joint in my body within 30 seconds.”
When asked to give the pain a rating between one and 10, she could only laugh. “Our pain far exceeds that,” she explained.
Not everyone with sickle cell disease experiences such acute pain. The symptoms range from very mild to requiring frequent hospitalizations.
Most common are the signs of anemia — fatigue, dizziness, headache, shortness of breath and cold hands and feet. Sickle cell disease has multiple complications. In the hand-foot syndrome, the sickle red blood cells block the small blood vessels of the hands and feet, causing pain, swelling and fever. Infections, such as pneumonia or the flu, are common. People with sickle cell disease are at higher risk for stroke, damage to the retina of the eye, development of gallstones, leg ulcers and delayed growth in children.
The damage is significant. According to a report by the Centers of Disease Control and Prevention, roughly 75,000 hospitalizations between 1989 and 1993 were due to sickle cell disease at a cost of $475 million.
A more recent report published in the April 2009 issue of the American Journal of Hematology estimates that the yearly cost of medical care exceeds $1.1 billion. The authors cautioned that this estimate does not include lost productivity and premature mortality.
Fortunately, it is often possible to avoid some of the problems. Regular checkups are important, and yearly flu shots and other immunizations can reduce the risk of infections. Hydration — drinking eight to 10 glasses of water every day — and not overdoing physical activity can go far in preventing crises, or painful episodes.
Notable advances have been made. Early diagnosis is essential for prompt treatment, and there is good news on that front. All 50 states, in compliance with a recommendation from the U.S. Preventive Services Task Force in 1996, now require screening for sickle cell in newborns. In Massachusetts, the Newborn Blood Screening Program run by the University of Massachusetts Medical School mandates screening for the disease within 48 hours of birth.
Timing is important. For the first few months of life, fetal hemoglobin is the main oxygen transport system for all infants. This inborn protection dissipates at about four to six months and instead of making normal hemoglobin, people with sickle cell disease make abnormal sickle hemoglobin. It’s been found, however, that the initiation of daily penicillin from the age of 2 months to 5 years can reduce the incidence of infections, particularly pneumonia, which had been common and deadly in children with sickle cell disease.
Most significant is the introduction in 1995 of hydroxyurea, an anti-cancer drug that has proven effective in preventing or reducing complications of sickle cell.
In rare cases, sickle cell disease can be cured through bone marrow transplantation.
The improved approach appears to be working. In a recent study published in the Journal of Pediatrics, the researchers found that the mortality rate for black children diagnosed decreased by 68 percent from 1983 to 2002. The researchers assume that this trend will continue.
Lifestyle changes are also imperative. For her part, Demery has learned to live with her disease. “I try to drink a lot of water,” she said. “And exercise helps.”
She walks regularly at Jamaica Pond. She is learning to control her stress — another trigger. Though she has chronic pain in her lower back and hips and takes prescription medications, she said she tries not to rely too heavily on the pain meds. “Sometimes it can make matters worse,” she said.
She also has learned to pace herself in her day-to-day life to keep the painful flare-ups at a minimum.
Although her condition has improved, Demery admits she still has flare-ups every two to three months.
In spite of everything, she looks at the positive. “I’m still a very productive person,” she said. “Although I am not healthy a lot, I work with what I have.”
She has completed a bachelor’s degree in biology and is planning to pursue a master’s degree in women’s health at Suffolk University in the fall. She is also an event planner and amateur photographer.
But she has become a bit of an advocate as well. In September, in commemoration of Sickle Cell Awareness Month, she is starting a support group for women with sickle cell disease. Demery feels there is a need to educate people about the disease.
“Without a strong support group, people can fall through the cracks,” she said.
|Myiesha Demery, 32, came to Boston from the U.S. Virgin Islands at the age of 5 for treatment for sickle cell disease.