Abstract

There is much variability in the expression of sickle cell disease (SCD) and recent works suggest that environmental and social factors may also influence this variability. This paper aims to use geographic information systems technology to examine the association between socio-environmental exposures and health outcomes in all persons who have attended or currently attend the Sickle Cell Unit in Jamaica. Rural patients presented for clinical care at older ages and had less annual visits to clinic. Persons travelled relatively long distances to seek SCD care and those travelling longer had less health maintenance visits. Urban patients had a higher prevalence of significant pain crises (69.4% vs. 55.8%, p value<0.001) and respiratory events (21.2% vs. 14%, p value<0.001). Prevalence of leg ulcers did not vary between rural and urban patients but was higher in males than in females. Females also had lower odds of having respiratory events but there was no sex difference in history of painful crises. Persons with more severe genotypes lived in higher poverty and travelled longer for healthcare services. Persons in areas with higher annual rainfall, higher mean temperatures and living farther from factories had less painful crises and respiratory events. The paper highlights a need for better access to healthcare services for Jamaicans with SCD especially in rural areas of the island. It also reports interesting associations between environmental climatic exposures and health outcomes.

Highlights

  • The term sickle cell disease (SCD) includes a variety of pathological conditions resulting from the inheritance of the sickle haemoglobin (HbS) gene either homozygously or as a compound heterozygote with other interacting abnormal haemoglobin genes

  • About 43.6% of patients were registered at the Sickle Cell Unit (SCU) before their 5th birthday and another 35.8% registered between 5 and 18 years of age

  • Wide variability exists in expression of SCD between individuals, and at least some of this variability is attributable to social factors including the environment

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Summary

Introduction

The term sickle cell disease (SCD) includes a variety of pathological conditions resulting from the inheritance of the sickle haemoglobin (HbS) gene either homozygously or as a compound heterozygote with other interacting abnormal haemoglobin genes. It is the most common monogenic hereditary disorder, affecting millions of persons worldwide and is especially prevalent in persons of African and Asian descent [1]. Jamaica is a Caribbean country where the prevalence of an abnormal haemoglobin gene is ~15% and one out of 150 babies born has a sickle haemoglobinopathy [2].

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