Analysis of patients adherence to sickle cell disease treatment during the pediatric-adult transition

Abstract

Sickle Cell Disease (SCD) is a classic example of hemoglobinopathy, characterized by sickle-shaped erythrocytes, due to a mutation in the hemoglobin beta chain. In Brazil, it is estimated that 4% of the Brazilian population has the sickle cell trait and that 25,000 to 50,000 people have the disease. The main manifestation of sickle cell disease is the painful crisis caused by vaso-occlusion. The objective of this study was to present interventions that corroborate for better adherence to the treatment of adolescents and young adults with SCD during the pediatric-adult transition. A systematic review of the literature was performed based on primary studies from searches of articles based on LILACS and Medline databases. Even though the pediatric-adult transition in sickle cell disease is the subject of research in several studies, it is clear that it is rarely experienced in practice. This deficiency consequently leads to lower adherence to treatment, exposing this age group to a greater risk of complications from the disease. Even with so many tools available, the pediatric-adult transition is still poorly addressed and, when addressed, late and poorly planned. It is extremely important that this topic be the subject of discussions between the doctor, caregivers and the child during routine consultations.