Mortality in Sickle Cell Disease -- A Population Based Study in an
Aboriginal Community in the Gudalur Valley, Tamil Nadu, India
Abstract
Background: Sickle cell disease (SCD), the commonest monogenic disorder,
affects more than 300,000 births annually, with 44,000 in India. While
the clinical phenotype of SCD is considered to be milder in aboriginal
populations in India, there is a paucity of data on outcomes. To
determine the severity of SCD in this population, we studied mortality
rates and causes of mortality in a longitudinal cohort of patients with
SCD in a remote aboriginal community in India receiving community-based
comprehensive care. Procedures: Causes of death in this cohort from
January 2008 to December 2018 were analyzed. Details were collected from
hospital records and in case of deaths at home, by utilizing the WHO
verbal autopsy questionnaire. Results: The cohort consisted of 157
patients belonging to the Paniya, Betta Kurumba, Kattunyakan, and Mullu
Kurumba tribes. During the study period, there were 22 deaths, all from
the Paniya tribe. Twelve deaths (54.5%) occurred in the hospital and
the remaining at home (45.5%) reflecting a crude mortality rate of 140
per 1000 population. 25% of deaths occurred in the 6-18 age group.
There were no deaths in the 0-5 age group. The median age of death was
25 years, which was 20 years less than in the non-SCD aboriginal
population. The leading causes of death were acute chest syndrome,
anemia, and sepsis among the SCD patients and stroke and suicides in the
non-SCD aboriginal population Conclusion: SCD is a severe disease among
the Gudalur Valley’s aboriginal population with a significant risk of
premature mortality.