Health-related quality of life of patients with sickle cell disease aged 8-17 years at Kamuzu Central Hospital, Malawi.

BACKGROUND

Sickle cell disease (SCD) is characterized by both acute and chronic complications that affect the daily lives of patients and lower their quality of life.

OBJECTIVE

To describe the health-related quality of life (HRQoL) and the associated factors in children aged 8-17 years with SCD attending the pediatric hematology clinic at Kamuzu Central Hospital (KCH) in Lilongwe, Malawi.

METHODS

A mixed-methods cross-sectional study was conducted at KCH. Patient data were collected with the aid of a standardized case report form. HRQoL was assessed using PedsQL™ Sickle Cell Disease Module by the child's report. Associations between HRQoL scores and independent variables were evaluated by a linear regression model. In-depth interviews were then carried out and the qualitative data were analyzed using content thematic analysis.

RESULTS

A hundred and sixty-three children with SCD were enrolled and 52.1% were females. Their median age was 11.2 ± 2.7 years. The mean global HRQoL score of the children was 62 ± 17.3. The highest scores were in the treatment domain (72.5 ± 15.1) while the lowest scores were in the emotions domain (55.2 ± 28.7). The mean pain score was 58.8 ± 16.3. The factors associated with low HRQoL scores were pain (β-coefficient -6.97 CI (-3.07,-15.58); p value .034) and low hemoglobin levels (β-coefficient 2.29 CI (.65-3.91); p value .006).

CONCLUSION

The HRQoL of this population is low. Pain and low hemoglobin were significantly associated with low HRQoL scores. Adequate treatment to control pain and increase the steady-state hemoglobin may improve the HRQoL of children with SCD. Interventions to address low emotional scores are recommended.