Abstract
In a tribal area of western India, a non-governmental organization implemented a comprehensive sickle cell disease (SCD) program at a secondary level hospital. In a cohort of SCD patients registered during December 2015 to June 2017, we assessed rates of lost to follow-up (LTFU) during the follow-up period using routinely collected data. We compared the uptake of proven interventions and indicators of disease severity from one year prior to registration until the end of the study (June 2018). Of 404 patients, the total follow-up duration was 534 person-years (PY). The rate (95% CI) of LTFU was 21 (17.5–25.3) per 100 PY. The proportion of people who received the pneumococcal vaccine improved from 10% to 93%, and coverage of hydroxyurea improved from 3.5% to 88%. There was a statistically significant decrease in rates (per 100 PY) of pain crisis (277 vs 53.4), hospitalization (49.8 vs 42.2), and blood transfusion (27.4 vs 17.8) after enrollment in the SCD program. Although clinical intervention uptake was high, one quarter of the patients were LTFU. The study demonstrated significant reductions in disease severity in SCD patients.
Highlights
Sickle cell disease (SCD) is a common genetic condition characterized by the tendency of red blood cells to become sickle-shaped and block capillaries, leading to vaso-occlusion and anemia
The specific objectives of the study were (a) to describe the socio-demographic and clinical profiles of patients registered with sickle cell disease, (b) to determine the time taken until patients became lost to follow-up and the associated factors, (c) to determine the proportion of patients receiving clinical interventions as per the standard treatment guidelines, and (d) to describe the clinical outcomes, including number of hospitalization episodes, number of pain crises, number of blood transfusions, rate of severe anemia, and complications due to sickle cell disease at different time points
A total of 404 SCD patients were registered during the reference period, including 247 (61%) females with a mean (SD) age of 19 (9) years
Summary
Sickle cell disease (SCD) is a common genetic condition characterized by the tendency of red blood cells to become sickle-shaped and block capillaries, leading to vaso-occlusion and anemia. Sickle-shaped red blood cells often become trapped in the spleen, leading to a serious risk of death before the age of seven years from a sudden profound anemia or due to an overwhelming infection [1]. SCD is more common among tribal communities than the general population [5,6]. In India, tribal populations constitute 8.6% of the total Indian population, about 68 million people [7]. The prevalence of sickle cell heterozygous (SCD trait) and SCD varies, with rates of about 1–40% and 1–12%, respectively, in Indian tribal populations [5,6,8]
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