Quality of life of individuals with sickle cell disease followed at referral centers in Alagoas, Brazil

Abstract

BackgroundSickle cell disease is a genetic, hereditary and chronic disease that affects the health of its carriers and might impair their health-related quality of life. ObjectiveThe aim of the current study was to assess the health-related quality of life of individuals with sickle cell disease followed at referral centers in Alagoas, Brazil. MethodsA total of 40 individuals with sickle cell disease aged 12 to 43 years old were evaluated by means of sociodemographic and clinical questionnaires, the Medical Outcomes Study 36-Item Short Form Health Survey and the Beck Depression Inventory. The latter was applied only to adults. ResultsMost participants were adults (62.5%) with a predominance of the SS genotype (85%) with pain being the commonest complication (95%). Mood disorder was found in 40% of the adults. The patients exhibited overall impairment of quality of life, which was more pronounced among the adults and under 15-year-old adolescents. Married adults exhibited less impairment of most quality of life domains compared to unmarried adults, and the adults with mood disorder exhibited greater impairment of all quality of life domains. ConclusionsThese results suggest that interventions that aim to improve vitality, pain, and mental health might contribute to maintaining high levels of quality of life in patients with sickle cell disease, especially among adults and under 15-year-old adolescents.

Quality of life in adults with sickle cell disease: an integrative review of the literature

“…Control case Level IV SCIELO Vilela et al (28) . Quality of life of individuals with sickle cell disease followed at referral centers in Alagoas, Brasil.…”

“…Socio-demographic and genotype aspects of study participants Among the 22 articles selected, the majority of the participants were: women in 21 (95.5%) (1)(2)(3)(4)(5)7,(13)(14)(15)(16)(17)(18)(20)(21)(22)(23)(24)(25)(26)(27)(28) , with HbSS / Sβº Tal genotypes in 19 (86.4%) (1)(2)(4)(5)7,(13)(14)(15)(16)(17)(19)(20)(22)(23)(24)(25)(26)(27)(28) ; unmarried children in 13 (59.1%) (1,(3)(4)(5)13,15,(20)(21)(22)(23)(24)…”

Mortality by sickle cell disease in Brazil

“…It is a chronic genetic pathology, which negatively affects the quality of life of individuals and their families 6, 9, 10. It exhibits autosomal recessive inheritance and, therefore, affected individuals are homozygous for Hb S (Hb SS).…”

Dynamics of sickle cell disease as one of the determinants of quality of life

“…The generic Medical Outcomes Study 36 - Item Short Form Health Survey questionnaire (SF-36), already translated and adapted to the Brazilian culture, examines components related to functional capability, physical aspects, pain, general health, vitality, and social, emotional and mental aspects. Due to its reproducibility and validity it has proved to be a suitable parameter in the assessment of rheumatoid arthritis (17) and other diseases (18) .…”