Sickle Cell Disease: A Care Challenge. Narrative Review

Abstract

Introduction: Sickle cell disease or sickle cell anemia (SCA) is an autosomal recessive inherited disease characterized by the production of hemoglobin S (HbS), which causes medical complications such as vaso-occlusive episodes, chronic anemia, and organ damage. This disease represents a global public health challenge requiring comprehensive medical care and effective management strategies.  Objective: To analyze recent scientific literature on sickle cell disease to identify main care needs, healthcare barriers, and current debates from an integrative perspective.  Method: A qualitative literature review was conducted following the PRISMA-ScR extension guidelines. Eligibility criteria included original articles and narrative reviews published between 2013 and 2023, in Spanish and English, with a clinical, epidemiological, or pathophysiological focus. The search was conducted using terms such as "sickle cell disease," "hemoglobin S," and "sickle cell disease." Five researchers participated in the selection, applying methodological quality criteria using the CAsPe tool. Results: From an initial 1,886 publications, 15 studies were selected after the screening process. The findings were organized into two thematic areas: identified care needs (pain management, crisis, preventive treatment, transfusions, and psychosocial support) and barriers to healthcare (limited access, lack of professional knowledge, socioeconomic barriers, and stigmatization).  Conclusions: Sickle cell disease presents complex challenges that require a multidisciplinary approach. Significant gaps in the healthcare system were identified, especially in resource-limited countries, highlighting the need to improve professional training and the fundamental role of nursing in the comprehensive care of patients and families