Aspectos básicos da fisiopatologia e de cuidados às pessoas portadoras de doença falciforme

Abstract

Sickle cell disease is a structural hemoglobinopathy, being one of the most common inherited diseases in Brazil and worldwide, with dominant character, transmitted from parents to children. Its historical-geographical origin is reported to the African continent, and currently sits on every continent due to population migrations. The disease results from a genetic alteration, and entails a structural change in the protein hemoglobin formation, emerging others different from hemoglobin A, which is the one normally found in adults without sickle cell disease. The abnormal hemoglobins most commonly found are the C and S. Structural change along with other tissue factors determines the sickling of red blood cells, leading to occlusion of blood vessels (vessels-occlusion) and the early death of red blood cells (hemolysis chronic). This study presents a literature review on the pathophysiology of sickle cell disease, as well as the basics on the care that should be provided to people living in various situations of your lives, for example, in childhood, adolescence and pregnancy. It also discusses situations of acute and chronic complications of the disease and the interventions required in each case, which should be adopted by primary care teams. Finally, it presents the literature on problems faced by carriers of the disease, late diagnosis as history, unfavorable socioeconomic conditions and unknowns of the primary care team about the disease.