Chronic complications and quality of life of patients living with sickle cell disease and receiving care in three hospitals in Cameroon: a cross-sectional study

Yannick Mboue-Djieka,Anne M. Andong,Cavin E. Bekolo,Eveline D. T. Ngouadjeu,Simeon-Pierre Choukem,Vincent S. Verla,Daniel Nebongo

doi:10.1186/s12878-017-0079-7

Abstract

BackgroundSickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. Although sub-Saharan Africa bears 75% of the global burden of SCD, there is a paucity of data on these complications and their effects on the QOL. We aimed to record these chronic complications, to estimate the QOL, and to identify the corresponding risk factors in patients with SCD receiving care in three hospitals in Cameroon.MethodsIn this cross-sectional study, a questionnaire was used to collect data from consecutive consenting patients. Information recorded included data on the yearly frequency of painful crisis, the types of SCD, and the occurrence of chronic complications. A 36-Item Short Form (SF-36) standard questionnaire that examines the level of physical and mental well-being, was administered to all eligible participants. Data were analyzed with STATA® software.ResultsOf 175 participants included, 93 (53.1%) were female and 111 (aged ≥14 years) were eligible for QOL assessment. The median (interquartile range, IQR) age at diagnosis was 4.0 (2.0-8.0) years and the median (IQR) number of yearly painful crisis was 3.0 (1.0–7.0). The most frequent chronic complications reported were: nocturnal enuresis, chronic leg ulcers, osteomyelitis and priapism (30.9%, 24.6%, 19.4%, and 18.3% respectively). The prevalence of stroke and avascular necrosis of the hip were 8.0% and 13.1% respectively. The median (IQR) physical and mental scores were 47.3 (43.9–58.5) and 41.0 (38.8–44.6) respectively. Age and chronic complications such as stroke and avascular necrosis were independently associated with poor QOL.ConclusionsIn this population of patients living with SCD, chronic complications are frequent and their QOL is consequently poor. Our results highlight the need for national guidelines for SCD control, which should include new-born screening programs and strategies to prevent chronic complications.

Highlights

Sickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life
The World Health Organization (WHO) estimates that 300,000 children are born with SCD each year, 75% of whom are in sub-Saharan Africa (SSA); they state that the burden of the disease could be reduced by simple careful management and prevention programs [3, 4]
The median (IQR) age at which the diagnosis of SCD was confirmed was 4.0 years (2.0–8.5); 113 (64.6%) participants were below the age of 21 and needed consent by proxy, 21 being the age of majority in Cameroon

Summary

Introduction

Sickle Cell Disease (SCD) is associated with chronic multisystem complications that significantly influence the quality of life (QOL) of patients early in their life. The World Health Organization (WHO) estimates that 300,000 children are born with SCD each year, 75% of whom are in sub-Saharan Africa (SSA); they state that the burden of the disease could be reduced by simple careful management and prevention programs [3, 4]. The disease morbidity and mortality improved in two small samples of patients in Nigeria and Angola using these simple but cost-effective interventions recommended by the WHO [5, 6]. Data especially those focusing on QOL are still scanty or inexistent in most SSA countries

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