Abstract

Background

Sickle cell disease (SCD) is a genetic, potentially debilitating disease with global spread and public health concern commonly found in people of African origin. Nigeria has the highest burden of the disease in the world. The disease predisposes to high morbidity and mortality rate, increasing comorbid complications with advancing age and impaired quality of life. Understanding fine details of quality of life issues is important to guide the design of comprehensive care protocols. The Wilson and Cleary conceptual model of HRQL is a biophysiological model of health that lends itself as a tool to enhance our understanding of the relationships among the health concepts.

Aim

The aim of the study is to describe the health-related quality of life of people living with sickle cell disease and to characterise the predictors in adults with SCD.
Pertinent questions were answered in pursuit of the aim, they included:
1. What is the profile of HRQL in adults with SCD in the study area?
2. What are the factors that ssociate with the measures of HRQL?
3. How does the Wilson and Cleary model fit the HRQL data of the study population?
4. Are there relationships among the bio-physiological variables, symptoms, functional status, general health perceptions, individual characteristics and the overall quality of life as hypothesised by Wilson and Cleary?
5. What are the patterns and paths of the relationship of the HRQL determinants?
6. What is the relative importance of each determinant?
7. What is the utility score and its determinants in the population?

Methods

A cross-sectional design was used. Sociodemographic and patient-reported questionnaires were used to collect data from a population of 200 adults with SCD aged 18 years and older from two sickle cell clinics in Ibadan, Nigeria. The Wilson and Cleary conceptual model of health-related quality of life was empirically tested using structural equation modelling technique.
Results
Findings supported the Wilson and Cleary model indicating strong relationship between objective and subjective health. Adults with SCD have significantly impaired quality of life predicted by biological and physiological factors, symptoms status, functional status, general health perception and some characteristics of the individual. Symptoms status, indicated by bodily pain, depression, anxiety and somatic symptoms, was the most important contribution to reduced quality of life in the population.

Conclusion

The study underscores the need to include assessment of patients’ quality of life as part of measures of medical outcomes beyond the presence or absence of diseases. The insight gained from the study can be used by clinicians to understand how changes in specific clinical characteristics affect a patients’ overall health outcomes. A multidisciplinary approach is therefore required to manage the disease because interventions that focused only on biological and physiological variables without considering the patients’ experiences of their symptoms will not be fully effective. Routine investigation of the patients’ psychiatric status should be integrated in the disease management protocol to be targeted for immediate attention once traces are detected to improve the health-related quality of life of the patients.