The First Leap to Adult Care for a Hispanic Sickle Cell Disease Patient: Lessons for Adequate Transition of Care

Introduction: Transition from pediatric to adult medical care can be a high-risk period for youth with Sickle Cell Disease (SCD). Morbidity and mortality increase for patients with SCD in the United States after the age of 18 years. We present a case of a 21-year-old Puerto Rican female with SCD who presents for her first adult hospitalization due to an acute pain crisis complicated by acute chest syndrome (ACS).

Case Description: A 21-year-old Hispanic female with a diagnosis of SCD, with history of multiple pediatric hospitalizations due to pain crises, recurrent splenic sequestration, early childhood stroke, and ACS, presented to our adult hospital with acute and constant back pain. The patient admitted poor compliance with medication and with medical follow-up with her pediatric hematologist for the past two years. Her last visit to the hematologist was four months prior, during which a formal transfer to the adult hematology service was discussed. By the time of admission, the patient had never met with an adult hematologist. Her physical exam was remarkable for tenderness to palpation over the upper back, with later progression to dyspnea and fever. Laboratory findings were remarkable for a hemoglobin (Hgb) of 5.8mg/dL (baseline 8-9mg/dL). Serial chest X-rays showed progressive bilateral opacities. The patient was transferred to the medicine intensive care unit due to hypoxic respiratory failure and started on high-flow nasal cannula. The case was discussed with her primary pediatric hematologists, who recommended ACS management with aggressive intravenous fluids, pain medications, and antibiotics. She also received blood transfusions for a goal Hgb of 10mg/dL. The patient recovered, and prior to discharge, she was transferred to adult Hematology and Internal Medicine care.

Discussion: This case highlights the poor transition of a Hispanic young adult with SCD to adult care. In Puerto Rico, there is a predominantly Hispanic population with poor access to healthcare resources. This population has worse health outcomes when compared with other Hispanic subgroups. Currently, Puerto Rico does not have a transition of care clinic or a protocol for transitioning patients with chronic conditions to adult care. The challenges these patients experience has yet to be studied, and standardization of transition from pediatric to adult care in Puerto Rico needs to be discussed.

Conclusion: This case of an adult with SCD complicated by ACS shows the importance of transition of care for a pediatric patient into adulthood. Transition of care should be a part of the routine care for every child starting at approximately 12 years of age until the transition is complete. Minority groups have less access to quality health care. Understanding the challenges faced by these populations is essential to coordinate a structured process of transition of care that allows compliance and access to the needed services.