Management of sickle cell disease

International Conference and Exhibition on Traditional & Alternative Medicine

December 09-11, 2013 Radisson Blu Plaza Hotel, Hyderabad, India

N. A. Alli

Accepted Abstracts: Altern Integ Med

Abstract :

Sickle cell disease (SCD) is an inherited haemoglobin disorder that is caused by a mutation in the beta globin gene. Approximately 72 million subjects worldwide are afflicted with this disorder, 55 million of whom reside in sub-Saharan Africa. Homozygous sickle cell disease is characterized by vaso-occlusive episodes and end organ damage, and carries a high mortality during early childhood in resource poor settings. Management of SCD requires a team of health care practitioners including internists, surgeons and allied health care professionals. The sickle gene has arisen independently more than five times globally. Its high prevalence along the equatorial belt in Africa and Asia is strongly linked to the selective survival advantage conferred to the heterozygous state against malaria. This selective advantage enjoyed by sickle cell trait has led to persistence of the sickle gene despite the high mortality of the homozygous state (balanced polymorphism). The prevalence of the sickle gene in South Africa is <1%, not unexpectedly so, since South Africa falls outside the malarial belt. However, lifting of travel restrictions post the first democratic election in 1994 has led to an influx of people from other parts of Africa, paralleled by an influx of the sickle gene. Consequently, health care workers have noted a steady increase in the number of outpatient as well as admission cases of SCD, prompting a candid re-assessment of our readiness at managing the condition viz., resources, infrastructure and expertise. In addition, there are significant cost implications that could place health budgets under strain, placing a need to educate health care workers and patients, as well as create awareness among the public at large on the subject. Learning institutions, educational platforms and media are well placed to achieve this objective. Owing to the chronic nature of the disease and challenges faced in resource limited settings, standardization of management is desirable. Management of SCD will be discussed under the following headings: 1) acute crises, 2) complications, 3) end organ damage, 4) preventive measures. The only chance of a cure is bone marrow transplantation which has its inherent risks and limitations, although gene therapy is beginning to show some promise.