Sickle Cell Disease: A Reality Check from A Prevalent Zone In India

Author(s): Dinesh Pendharkar*, Garima Nirmal, Neha Gupta, Abhishek Raj

Background: Sickle cell disease is a major cause of morbidity and mortality in large tribal populations of India. States have created facilities for its diagnosis and for the supply of medication. The symptoms of acute crisis necessitate frequent medical visits, blood transfusions, and management of pain crisis

Methods: A cross-sectional prospective research design was used that involved the completion of questionnaires on socio-demographic characteristics and clinical characteristics. One hundred and fourteen individuals with Sickle cell disease who went to centers for outpatient visit were analyzed.

Results: The median age of the cohort was 19 years with Male: Female ratio of 1.5:1. Siblings were screened for 45.6% of individuals. Thirtysix (32%) individuals had multiple episodes of veno-occlusive crisis in the past 1 year. Only 16% of patients required >10 rounds of transfusion. Seven patients reported avascular necrosis of femur head. One hundred four patients with SCD were on Hydroxyurea and the majority of them (80%) had hemoglobin F in the desirable range of > 20%.

Conclusions: This study describes the high usage of hydroxyurea in the patients with sickle cell disease and 32% patients having multiple episodes of VOC in past 1 year

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