Left Behind and Lost in Transition

The transition of adolescents from pediatric to adult healthcare has been a long-recognized gap in our healthcare system. It affects all young adults but is especially impactful for those with complex childhood-onset problems. The negative impacts of transition have been demonstrated for many diseases, including young adults with type 1 diabetes, solid organ transplants, and congenital heart disease. The care of persons with sickle cell disease (SCD), in particular, suffers as young adults transition to adult care.

Few diseases suffer from such an intersection of racism, disease-specific negative attitudes, poor provider knowledge, and lack of specialist access. The impacts of these disparities are reflected in the rise in mortality as persons with SCD enter their early twenties. This parallels waning options for high-quality healthcare and decreasing utilization of hydroxyurea.

“The care of adults with SCD has been a major failing of our healthcare system and a manifestation of structural racism, particularly when compared to other rare genetic diseases such as hemophilia and cystic fibrosis.”

The landscape of adult care for persons with sickle cell disease is a desert. Depending on the state, approximately 30-50% of adults do not have access to a hematologist. In this context, care is often shifted to primary care physicians and the acute care setting, where providers are often not comfortable caring for adults with SCD and are unaware of current guidelines. 

Aside from a lack of knowledge, the attitudes that we as healthcare providers hold about persons with SCD impact their care. Despite multiple national organizations’ recommendations for rapid assessment of pain and treatment with IV opioids, people with SCD are often not believed and labeled “drug seekers” and face long waiting times in the emergency room. In fact, persons living with SCD may delay their own evaluation in the acute care setting due to fears of how they will be perceived and treated.

The care of adults with SCD has been a major failing of our healthcare system and a manifestation of structural racism, particularly when compared to other rare genetic diseases such as hemophilia and cystic fibrosis. What strategies can medical centers take? First and foremost, resources should be allocated to the development and sustenance of adult sickle cell disease programs. These may be stand-alone or integrated into a larger clinical space, such as a general internal medicine or hematology clinic. Essential SCD program personnel, as described by Kanter et al. (Blood Advances, 2020), include an SCD specialist, social worker(s), patient coordinator(s), dedicated nursing, and advanced practice providers. The team should have the capacity to provide a full spectrum of care, including managing pain, disease-modifying therapy, blood transfusions, and ensuring timely referral to other specialists. In addition to the benefits to persons with SCD, programs may have substantial benefits on health systems, including decreasing hospitalizations, ED visits, and lengths of stay.

For many adolescents and young adults with SCD, the process of a pediatric-to-adult transition is a “bridge to nowhere.” They are left in a vacuum, cared for by providers without specialized knowledge, and continue to suffer from progressive early morbidity and mortality. Though substantial progress has been made in preventing mortality in children, survival in adults continues to be limited. Recent data from California estimated the average age of death in the early 40s. Health systems must build up programs and expertise to support adults with SCD, acknowledging the wrongs committed and the influence that racism and bias have had on the care of SCD.