TITLE:
Health Related Quality of Life among Children with Sickle Cell Anaemia in Northwestern Tanzania
AUTHORS:
Zivonishe Mwazyunga, Emmanuela E. Ambrose, Neema Kayange, Respicious Bakalemwa, Benson Kidenya, Luke R. Smart, Adolfine Hokororo
KEYWORDS:
Sickle Cell Anaemia, Health, Quality of Life, Children, Tanzania
JOURNAL NAME:
Open Journal of Blood Diseases,
Vol.12 No.2,
May
23,
2022
ABSTRACT: 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 (5/204, 2.5%). Using a modified Likert scale, a total of 41/204 (20.1%) children had poor HRQoL indicated
by low scores on PedsQLTM and 163/204 (79.9%) children had high scores, indicating good HRQoL. On multivariate analysis, age ≥ 5 years (p-value 3hospitalizations per year (p-value = 0.008) were associated with poor HRQoL. Conclusion: SCA complications, negatively impact the HRQoL of
children living
with the disease. Severe anaemia, older age and frequent hospitalizations
were highly associated with poor HRQoL. Comprehensive management is needed
beginning at diagnosis to identify these children early and provide them with
adequate support.