Hematology and Oncology

At the Intersection of Sickle Cell Disease and Asthma

A child uses an inhaler. (Stock photo)

While many people know of sickle cell disease (SCD), it may be surprising to learn that there is a high prevalence of co-occurring asthma among children with SCD. Brandi Pernell, DNP, has been researching social determinants of health that impact young patients with a double diagnosis.

“While I was pursuing different strategies for improvements among the asthma population, I discovered a connection between environmental stress and asthma,” said Pernell, an assistant professor in hematology and oncology at Children’s of Alabama and the University of Alabama at Birmingham (UAB). “When you look at the general population in comparison to the sickle cell population, you’re going to see that more children with sickle cell have a diagnosis of asthma because these two conditions have some overlapping inflammatory physiological pathways. That in itself is going to increase the risk of having asthma along with sickle cell.”

Asthma affects oxygen levels, especially among those who are undiagnosed or who are diagnosed but whose disease isn’t fully managed, Pernell said. And low oxygen is a risk factor for red-cell sickling. Patients with both asthma and SCD experience higher rates of pain and acute stress as a result.

Pernell estimates that she provides clinical care for 80 to 90% of young patients in the area who have both SCD and asthma, as well as any patient hospitalized with acute chest syndrome, an acute lung complication. “I have an acute care follow-up clinic where I see anybody who has been hospitalized with acute chest syndrome within four weeks of discharge to make sure that they have focused sickle cell management and any asthma management that needs to take place to reduce the risk of it happening again,” she said.

Another major component of care, Pernell believes, is patient education. She works with community-based organizations and the Sickle Cell Disease Foundation to provide health education. She also gives patients and their families handouts with up-to-date information for disease management. “I try to employ a multimodal approach to education, but I think that the best method is still face-to-face interaction,” she said. “After all the clinical care is done, I take time to see if there are any questions from patients. I explain why I’m recommending this therapy or why this therapy is so important and how it works exactly in your body—because I think that improves adherence if you understand why you need it—or what can happen if you don’t take a medicine or how it could further impact your health in a negative way.”

Support for her patients is also multi-modal. Pernell relies on assistance from a social worker who can lock in community services as needed. She also works with the Children’s of Alabama school liaison to ensure patients have comprehensive support. “By school age, about 30% of children with sickle cell will have suffered a silent stroke, which can impact the child neurocognitively and lead to a decline in academic performance,” Pernell said. She and the liaison also make sure that each patient has a 504 plan in place for important classroom accommodations, such as being able to maintain hydration and avoid temperature extremes to avoid a sickle cell crisis.

The research Pernell has undertaken looks closely at social determinants of health and those factors within a patient’s control that can improve symptoms. “We are focusing on lifestyle interventions,” she said. “Are there dietary changes (that can reduce symptoms?) Are there different stress reduction tactics that can be taken or things prenatally that moms can do?”

These and other research questions are also guided by input from the patients themselves. Pernell believes that patients, as the ultimate end users of any research discoveries, must be invested in the research planning process and study design. “We need to make sure that the outcomes that we’re pursuing are the outcomes that the patients themselves care about.”

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