January-March 2018

Sickle Cell Disease: Prevalence, Clinical Presentation and Prognosis in Tribes; Current Concepts and Unanswered Question

Vaishali Patel1*, Hitesh Rathod2

1 Assistant Professor, Medicine Department, Medical College Vadodara, 2Associate Professor, Forensic Medicine Department, G.M.E.R.S Medical College Gotri, Vadodara


BACKGROUND: Sickle cell Disease is one of the most common severe monogenic disorder in the world. It is characterized by intermittent Vasoocclusive events and chronic Hemolytic Anemia. Patients clinically present with early and severe anemia requiring Blood Transfusion and Septicemia which are the most common complications. This review focuses on the recent initiatives to understand various phenotypes, role of immunization and Penicillin Prophylaxis, role of Hydroxyurea, awareness and central programs. Although more work is needed to develop effective treatment that specifically target pathophysiological changes and clinical complications of Sickle Cell Disease. MATERIAL AND METHODS: This is prospective study of 231 patients of Sickle Cell Disease conducted at Medical College, Vadodara during July 2014 to January 2017. All patients were subjected to personal biodata, detailed clinical history and examination with written consent. All were sent for routine blood investigations, and special investigations like sickling test, Hb electrophoresis, chest x ray PA view, ECG, PFT, CT Scan. RESULTS: In my study, most common age group was 12-30affecting males and female equally. In addition, persons from lower socioeconomic group had more complications. Most patients had positive family history with significant past history of pain crisis and blood transfusion. Painful vasoocclusive crisis and dactylitis are very common in Indian patients but chronic leg ulceration is rare. Few patients were put on Hydroxyurea therapy. CONCLUSION: Sickle cell disease is serious and life threatening disease. All should have thorough understanding of disease, its complications and treatment. Simple measures like vaccination in childhood, adequate oral fluid intake with electrolyte, during vasoocclusive crisis, and avoidance of extreme temperature reduce the number of patients with vasoocclusive crisis .Premarital counselling and prenatal diagnosis also help to reduce the morbidity and mortality related to disease. Although central and state government are now supporting the establishment of centers for the diagnosis of patients and comprehensive care.

Keywords: Sickle Cell Disease, Prevalence, Clinical Presentation, Prognosis.

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