Researching Early Indicators of Heart and Lung Damage to Improve Outcomes for Children and Young Adults with Sickle Cell Disease

Acute chest syndrome, a serious respiratory complication of sickle cell disease, is the leading cause of death among young sickle cell disease patients. Currently, it is very challenging to detect in its early stages – and it is therefore difficult to treat until it has advanced to a more serious stage. Dr. Rhea Hans, a hematology fellow at Phoenix Children’s, one of the nation’s largest pediatric healthcare systems, is working to change that. Her ongoing research is designed to better identify and measure early predictors of acute chest syndrome, including hemolytic markers and damage to the heart and lungs. By identifying these indicators of early-onset respiratory damage, doctors may be able to intervene earlier, institute more effective preventative measures, and improve outcomes for children and young adults with sickle cell disease.

Dr. Hans’ research was awarded Agios’ first Hemolytic Anemias Clinical Fellowship Grant, an award funded by Agios and intended to increase the number of skilled clinicians committed to providing comprehensive care for individuals living with non-malignant hematological disorders, with a focus on hemolytic anemias. Currently, there is a dearth of clinicians focused on these diseases; in fact, a recent study in the journal Blood Advances showed that less than 5% of hematology/oncology fellows plan to focus their careers on non-malignant hematology. Supporting innovative research by skilled clinicians dedicated to these patient communities is vital to generate leaders able to provide training and support to other future clinicians in hemolytic anemia patient care, and ultimately help address the health inequities experienced by these historically underserved patient communities.

Dr. Hans’ interest in pediatric sickle cell disease began during her residency training in Newark, New Jersey, where there is a large sickle cell disease population. “Because the sickle cell disease patients came into the hospital so frequently, I got to know them the best,” says Dr. Hans. “I saw how difficult the disease was for them and the impact it had on their lives at home and at school. It’s disheartening that the disease has been known for so long, and some advances have been made, but there is so much more we need to do.”

To learn more about the sickle cell disease program at Phoenix Children’s, please visit their website here.