Abstract
To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.
Highlights
The term sickle cell disease (DF) is used to denote a group of genetic disorders in which there is a predominant hemoglobin (Hb) variant called HbS, which originates from a point mutation resulting in the substitution of glutamic acid by valine at position 6 of the β-globin(1)
A broad guiding question was adopted for this study: what evidence is available in the literature about health-related quality of life (HRQoL) assessment in adults with FD, which scales were used, and of which, which have already been validated in Brazil? The bibliographic survey was carried out from August 2015 to January 2016, in the electronic databases MEDLINE (Medical Literature Analysis and Retrieval System Online), CUMED (National Center of Information of Medical Sciences of Cuba), LILACS (Latin American Literature and the Caribbean in Health Sciences) and SciELO (Scientific Electronic Library Online)
Guiding question: what are the available evidence in the literature on the evaluation of HRQoL in adults with FD, which scales were used and, of these, which have already been validated in Brazil?
Summary
The term sickle cell disease (DF) is used to denote a group of genetic disorders in which there is a predominant hemoglobin (Hb) variant called HbS, which originates from a point mutation resulting in the substitution of glutamic acid by valine at position 6 of the β-globin(1). The most frequent forms of FD are: sickle cell anemia, homozygous and more severe form (HbSS); HbS hemoglobinopathy in association with another Hb variant (HbC) and Sb - thalassemia (Sb - tal), which presents the HbSβ - tal or HbSβ +-tal subtypes(2). This mutation produces changes in the structure of HbS that, under conditions of low oxygen concentration, undergoes polymerization. These polymer chains inside the erythrocytes cause a change in their shape, loss of characteristic deformability, acquiring the shape of sickle (falcization). Patients usually present several complications, which begin in childhood, as a result of repeated vaso-occlusive episodes, chronic inflammation and hemolysis(3)
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