Soccer, street hockey, computer games and television are all typical trappings of 7-year-old boys. Though they love all of these things, brothers Nicholas and Toby are anything but typical. First of all, it's nearly impossible to tell the adorable identical twins apart. What's far less obvious is that both of the boys have a rare and severe form of hemoglobin H disease, a type of thalassemia.
Thalassemia affects the ability to produce hemoglobin. Found in red blood cells, hemoglobin is a protein that carries oxygen throughout the body. Thalassemia can cause severe anemia and lead to a wide range of other medical issues. Diagnosed soon after birth at Dartmouth-Hitchcock Medical Center, Toby and Nicholas were referred to Children's Hospital Boston at age two when the family moved to southern New Hampshire.
Healthy, Active and Doing Well
Today the twins are healthy, active and doing well. Their parents and medical team have worked to find opportunities to make managing the illness simpler and less disruptive. According to Melody Cunningham, MD, Director of the Thalassemia Program, one advance they have benefited from is a clinical study of a new oral iron chelator. The boys require blood transfusions every four weeks and chelation therapy to remove excess iron that can cause serious organ damage. Chelation is usually performed at home, through a pump connected to a needle placed under the skin. The process takes at least ten hours, and in the boys' case, was necessary five nights a week. Now Nicholas and Toby take the new chelation medication mixed in water each morning, eliminating the hours-long chelation process.
Other improvements have reduced the amount of time the family needs to spend at the hospital. Transfusion patients typically come to the hospital the day before the procedure to have blood drawn for testing. Since the boys live more than an hour away, this wasn't possible. Instead, they came in early on the day of transfusions to have blood drawn, then waited an hour or two for results before beginning transfusions, which take up to 4 hours. Jennifer Braunstein, the nurse practitioner in Children's Thalassemia Program, arranged for Evelyn, the twins' mom, to be trained to draw blood. Now she draws it at home a day or two before the transfusions and express mails it to Children's blood bank, reducing the time the twins are away from school and the activities they enjoy.
A Center of Excellence
The Thalassemia Program at Children's is one of five centers in the country designated for clinical excellence by the National Cooley's Anemia Foundation. It is also part of a five-center network chosen by the National Institutes of Health to foster clinical research in the illness. "Children's offers the top doctors in thalassemia, and that's reassuring," says Evelyn.
"With a chronic condition like thalassemia lots of side issues come up. We can get them all monitored and addressed here," says the boys' dad, Ben. As well as being followed by a hematologist who specializes in thalassemia, Nicholas and Toby also see an ophthalmologist, audiologist, cardiologist, and other health professionals as necessary.
Toby and Nicholas seem unfazed by it all. Active and energetic, they are spending some time on Cape Cod, celebrating their seventh birthday in July, enjoying things like sports and television, and looking forward to starting first grade.
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