Background: Sickle cell anaemia (SCA) is a serious, multisystem, genetic disorder
affecting millions of children worldwide. The disease causes numerous complications that interfere with the health-related quality of life
(HRQoL) of these children including an impact on educational, physical and
psychosocial development. Few studies have described the clinical spectrum and
quality of life of children with SCA living in a low-resource area. Objectives: This study aimed to determine the clinical spectrum and HRQoL among
children living with sickle cell anaemia
(SCA) in northwest Tanzania. Methods: This hospital-based cross-sectional study took place at Tertiary
and teaching hospital, Bugando Medical Centre, Mwanza Tanzania.The study enrolled children ages 2 - 12 years old with SCA attending the
Bugando Medical Centre sickle cell clinic. Health related quality of life was
measured using the Pediatric Quality of Life, Brief Generic Core Scale after
translating from English into a Swahili version. Important SCA complications
were assessed using a structured questionnaire. Results: From October
2016 to March 2017, 204 children were enrolled.
Participants presented at a median age of 6 years [IQR 4 - 9]. Among
children with SCA the most common clinical signs at the time of enrolment were
pale in 69.6% (142/204), jaundice in 65.9% (134/204), oxygen saturation <90% in 25% (51/204) and splenomegaly in 19% (39/204). Severe anaemia was
observed in 30.9% (63/204). A majority reported vaso-occlusive crisis (166/204,
81.4%), and very few had experienced a
prior stroke
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