PILOT 5

Implementing Hydroxyurea Therapy among Youth with Sickle Cell Disease: A Needs Assessment

Investigator: Aimee Hildenbrand, PhD

Mentor: Melissa Alderfer, Ph.D.

Aimee Hildenbrand, PhD is a postdoctoral research fellow in the Nemours Center for Healthcare Delivery Science. Her research is mentored by Drs. Melissa Alderfer and Anne Kazak and focuses on three primary areas: (1) pain and pain management in youth with acute and chronic medical conditions, including those with sickle cell disease; (2) child and family adjustment to pediatric injury and illness; and (3) the development and evaluation of interventions for pediatric medical traumatic stress. In addition, Dr. Hildenbrand provides psychology services for youth with cancer, childhood cancer survivors, and children and adolescents with chronic pain through the Division of Behavioral Health. She is also an Associate Fellow in the Center for Injury Research & Prevention at the Children’s Hospital of Philadelphia and a member of the Editorial Board for the Journal of Pediatric Psychology.


Youth with SCD are at risk for significant morbidity and mortality and impaired health-related quality of life. Hydroxyurea (HU) is one of few disease-modifying treatments available for children with SCD. Research has established the efficacy and safety of HU therapy among infants, children, and adolescents with SCD. Specifically, HU therapy is associated with significant reductions in vaso-occlusive pain episodes and acute chest syndrome, fewer hospitalizations, reduced healthcare expenditures, and improved health-related quality of life, growth, and survival. As a result, the National Heart, Lung, and Blood Institute advocates that HU be offered to all children with SCD ages 9 months and older, regardless of clinical severity, to reduce SCD-related complications. Despite national guidelines and strong evidence of efficacy and safety, uptake of HU has been slow in pediatric SCD care. Recent estimates suggesting that 40-65% of children with severe SCD receive HU, with significant variability across providers and institutions. Enhancing the implementation of HU therapy among youth with SCD is vital to improve outcomes among this at-risk population. A critical first step for developing evidence-based implementation strategies is to conduct a formative evaluation to better understand barriers and facilitators of HU treatment.

Using the Consolidated Framework for Implementation Research and a multi-method, multi-informant approach, this study seeks to improve implementation of HU therapy at our children’s hospital by achieving the following specific aims: (1) Examine the proportion of pediatric SCD patients who have been offered and prescribed HU; and (2) Identify barriers and facilitators of HU treatment for pediatric SCD patients. Electronic medical record (EMR) review will be used to examine the proportion of SCD patients offered and prescribed HU across the Nemours Children’s Health System. Patient demographic and clinical factors associated with HU will be examined to identify potential determinants of current practice. Formative qualitative interviews with Nemours/AI duPont Hospital for Children stakeholders including patients, parents, and providers will be conducted to identify barriers and facilitators of HU therapy. The proposed study will launch a program of research on developing, testing, and disseminating implementation strategies for HU treatment, with the ultimate goal of reducing this critical research-to-practice gap and decreasing the burden of SCD for children and families.

Publications:

https://www.ncbi.nlm.nih.gov/myncbi/browse/collection/56790105/