Health-related quality of life and drug therapy problems in patients with sickle cell disease on hydroxyurea at Kenyatta National Hospital.

Abstract

Background: Sickle cell disease is an inherited genetic disorder caused by presence of a mutated form of hemoglobin. It is estimated that 312,000 babies are born with sickle cell disease every year, with 90% of these births occurring in Africa. It has a high mortality rate with 50-90% of babies born with the disease dying before the age of five. Multiorgan involvement and painful crises are the hallmarks of sickle cell disease. Studies have revealed that patients with sickle cell disease have a lower quality of life compared to the general population. Hydroxyurea is the standard therapy for the management of sickle cell disease. Objective: To determine the health-related quality of life and drug therapy problems with hydroxyurea use in patients with sickle cell disease at Kenyatta National Hospital. Methods: A cross-sectional study was conducted at the Kenyatta National hospital hemato-oncology clinic. Sixty-seven participants were eligible. Enrolled participants answered questionnaires designed to collect information on the socio-demographic and medical characteristics. The World health organization Quality of Life (QoL) tool was used to determine the health-related quality of life score. Data was entered into Epi-info version 7, and STATA version 13 was used for data analysis. Descriptive data analysis was employed to summarize key variables. Linear regression analysis was done on pooled data to identify the most important determinants of quality of life and to control for confounding. Results: Participants had a median age of 20.7 (±7.2) years, 52% were male and had experienced several sickle cell disease-related complications with acute chest syndrome (65.7%) being the most prevalent. The most frequently reported clinical symptoms included headache (44%) and painful crisis (38.8%). Non-compliance (86.6%) was the most prevalent drug therapy problem. In the quality of life assessment, psychological health had the highest score of 68.2%. Positive family history (p=0.030) (6.58 CI 0.66, 12.50) was significantly associated with better overall quality of life scores. Vomiting (p=0.099) (4.77 CI -10.46, 0.93) and chest pain (p=0.697) (1.71 CI -10.43, 7.02) were associated with lower overall quality of life scores. Conclusion: The main drug therapy problem was non-compliance. Health-related quality of life among sickle cell patients was suboptimal across all domains. Strategies to contain drug therapy problems such as non-compliance should be encompassed in the management of patients. Effective management of pain and complications associated with sickle cell disease will improve the health-related quality of life of patients.