Updated June 19, 2017
The question of how many children with sickle cell disease (SCD) also have asthma is unknown, notes Johns Hopkins Children’s Center pediatric pulmonologist Christy Sadreameli. But the answer is important, she adds, because SCD patients who also have asthma tend to have more frequent acute chest syndrome episodes, painful crises, hospitalizations and higher mortality than those who do not have asthma.
“Sickle cell patients with asthma may die at a younger age,” Sadreameli says, “and we are trying to understand how to prevent this.”
Better detection will mean earlier treatment and improved asthma outcomes, says Sadreameli, which is why she launched a pilot one-year asthma screening program at Johns Hopkins Children’s Center. Thanks to a grant from the Thomas Wilson Foundation for a portable spirometer and other expenses, Sadreameli conducted the screenings in the hematology clinic. The screenings, which include a pulmonary function test and a questionnaire, should also enhance understanding of the relationship between the two disorders, Sadreameli says, and hopefully lead to more effective asthma treatments.
“There’s not a whole lot in the literature on this subject and no one really understands whether sickle cell disease patients’ risk of higher morbidity and mortality is modifiable with good asthma care,” Sadreameli says. “Does treating the asthma help you have less pain than someone with untreated asthma? We don’t know. However, we do know a lot about how to treat asthma, so we want to provide the best care we can for our patients who are affected with asthma and sickle cell disease.”
Another unresolved issue is the impact of tapering down a common asthma medication – fast-acting systemic steroid – to stop severe asthma attacks. Sadreameli cites a rebound effect of acute pain and complications for some sickle cell patients when steroids are withdrawn.
“Sometimes doctors are a little more hesitant to give oral steroids to the sickle cell patient having an asthma attack, but if it’s bad enough you have to give it,” says Sadreameli. “For me, that’s all the more reason to keep the asthma under control with long-term medications so oral steroids are needed less frequently.”
The asthma should continue to be treated by the pediatric pulmonologist, and the sickle cell disease by the pediatric hematologist, but there needs to be more coordination of care by the two specialists, stresses Sadreameli, who sees a substantial number of pediactric patients with sickle cell disease and lower airway issues. The sickle cell patient with asthma should be seen by a pulmonologist at least every six months, she adds, and have pulmonary function testing yearly. For more information, call 410-614-0050. For appointments with Pediactric Pulmonology, call 410-955-2035.