Abstract

BackgroundSickle cell disease is the commonest genetic disorder in Jamaica and most likely exerts numerous effects on quality of life (QOL) of those afflicted with it. The WHOQOL-Bref, which is a commonly utilized generic measure of quality of life, has never previously been utilized in this population. We have sought to study its utility in this disease population.Methods491 patients with sickle cell disease were administered the questionnaire including demographics, WHOQOL-Bref, Short Form-36 (SF-36), Flanagan's quality of life scale (QOLS) and measures of disease severity at their routine health maintenance visits to the sickle cell unit. Internal consistency reliabilities, construct validity and "known groups" validity of the WHOQOL-Bref, and its domains, were examined; and then compared to those of the other instruments.ResultsAll three instruments had good internal consistency, ranging from 0.70 to 0.93 for the WHOQOL-Bref (except the 'social relationships' domain), 0.86–0.93 for the SF-36 and 0.88 for the QOLS. None of the instruments showed any marked floor or ceiling effects except the SF-36 'physical health' and 'role limitations' domains. The WHOQOL-Bref scale also had moderate concurrent validity and showed strong "known groups" validity.ConclusionThis study has shown good psychometric properties of the WHOQOL-Bref instrument in determining QOL of those with sickle cell disease. Its utility in this regard is comparable to that of the SF-36 and QOLS.

Highlights

  • Sickle cell disease is the commonest genetic disorder in Jamaica and most likely exerts numerous effects on quality of life (QOL) of those afflicted with it

  • Sickle cell disease (SCD) is the commonest genetic disorder in Jamaica with the sickle hemoglobin (HbS) gene being present in about 10% of the population

  • Median survival is calculated at 53 years for men and 58.5 for women [5]

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Summary

Introduction

Sickle cell disease is the commonest genetic disorder in Jamaica and most likely exerts numerous effects on quality of life (QOL) of those afflicted with it. Sickle cell disease (SCD) is the commonest genetic disorder in Jamaica with the sickle hemoglobin (HbS) gene being present in about 10% of the population. It includes a variety of pathological conditions [1] and affects the individual throughout their life cycle. In Jamaica, SCD has become a significant indirect cause of maternal mortality [2] and contributes as a causative factor to 0.7% of cases of chronic renal failure [3] It has been presented as one of the 10 most common causes of sudden death in Jamaica accounting for 2.5% of cases [4]. Median survival is calculated at 53 years for men and 58.5 for women [5]

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