Pregnancy often brings a surprise or two with it, but the news Siobhan White received while expecting her son, JJ, set the family on a course they never planned. Routine prenatal testing led to a diagnosis of sickle cell disease for both White and her new baby.
Shortly after his birth in August, 2006, JJ was referred to Children’s of Alabama to be seen by pediatric hematologist Dr. Tom Howard. Because several relatives were afflicted, the family was aware of sickle cell disease but soon found there was much to be learned, according to Siobhan’s mother and JJ’s grandmother, Dorothy Turner. “It was a journey at the very beginning to learn all about it and how it affected JJ,” she said. “We were given videos to watch during clinic visits and took quizzes. The nurses would meet with us to discuss any of our answers that were incorrect. They made sure we understood.”
As often happens in sickle cell disease, little JJ began experiencing pain crises with high fever and severe, debilitating joint pain. The episodes required visits to Children’s emergency department two or three times a month.
When he was 2, he underwent transcranial Doppler (TCD) screening, which tracks blood flow through the brain. The test showed that JJ was at a high risk of stroke so Dr. Howard began treating him with regular blood transfusions, a highly successful therapy. JJ has not experienced another pain crisis since then and his risk of stroke has decreased as well. Dr. Howard is hopeful that he can soon transition the youngster to an oral medication that will keep him stroke-free without the transfusions. “JJ is an example of TCD being able to reduce the incidence of stroke by identifying patients at risk and treating them accordingly,” Dr. Howard said. “We’ve been able to prevent strokes in 90 percent of the thousand patients we treat.”
Like other children with chronic illness or other special health needs, sickle cell disease requires that JJ receive a bit of extra attention while at school to help prevent a pain crisis or other complications. “When he started school, we had to educate the school,” said his grandmother, an intermediate school teacher who recently retired after 37 years in the classroom. “We told them he must be careful about extreme temperatures during P.E. class and we have to be made aware of illnesses going around the school because even minor things can make him really sick.”
Otherwise, the youngster with the wide grin is like most any little boy his age. He plays video games during his treatments, enjoys an occasional stack of pancakes and seems to like school, although he might not admit to it. His grandparents, both retired, help get him to his clinic appointments while his mom and dad are at work. “We feel we’ve been blessed,” Dorothy said. “To look at him, you’d never know anything is wrong. The doctors and nurses are well-equipped to care for him.”