Author(s)

Zivonishe Mwazyunga^1*, Emmanuela E. Ambrose^1#*, Neema Kayange¹, Respicious Bakalemwa¹, Benson Kidenya¹, Luke R. Smart^1,2, Adolfine Hokororo¹

¹Department of Paediatrics, Catholic University of Health & Allied Sciences and Bugando Medical Centre, Mwanza, Tanzania.
²Division of Haematology, Cincinnati Children’s Hospital, Cincinnati, OH, USA.

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 PedsQL^TM and 163/204 (79.9%) children had high scores, indicating good HRQoL. On multivariate analysis, age ≥ 5 years (p-value < 0.001), haemoglobin < 7 g/dl (p-value = 0.001) and >3 hospitalizations 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.

Sickle Cell Anaemia, Health, Quality of Life, Children, Tanzania

Mwazyunga, Z. , Ambrose, E. , Kayange, N. , Bakalemwa, R. , Kidenya, B. , Smart, L. and Hokororo, A. (2022) Health Related Quality of Life among Children with Sickle Cell Anaemia in Northwestern Tanzania. Open Journal of Blood Diseases, 12, 11-28. doi: 10.4236/ojbd.2022.122002.