Comparative assessment of quality of life among adolescents with sickle cell disease and sickle cell trait: evidence from Odisha, India.

The present study aims to assess the quality of life (QOL) of adolescents with sickle cell disease (SCD) and sickle cell trait (SCT) in hard-to-reach regions in Koraput district of Odisha state. 387 adolescents with sickle cell genes (HbSS = 52, HbAS = 135, HbA = 200) were selected through their medical records from southern parts of Odisha. A validated and pretested QOL scale was modified to assess the QOL. The questionnaires were modified by aiming to describe the proportion of adolescents who feel restricted in different domains, measuring the extent within each domain, and finding an aggregate score of QOL. Furthermore, to explore the expenditure on health, 552 households were selected randomly, of which 72 families had HbS individuals. This study found a significantly lower health-related QOL in adolescents with SCD. However, most psychosocial sub-domains, for instance, worry about the illness, frequency of angry days, feeling jealousness toward other normal adolescents, and negative feelings of sadness on some days, are similarly affected in adolescents with SCT and SCD. The overall QOL of SCD individuals is more affected (percentage of affected mean score = 60.93%), followed by SCT individuals (35.63%). Healthy adolescents' QOL is relatively unaffected (13% were affected). The yearly frequency of blood transfusion received (1.7 ± 0.4) and hospitalization (2.1 ± 0.9) was significantly higher in adolescents with SCD. The healthcare expenditure was significantly higher (3.6% to 81.3% of the family income) in families with HbS than in families without HbS (0.8% to 19.2%) (p < 0.05). The overall QOL was affected in both SCD and SCT adolescents. The focus should be given equally to both SCD and SCT individuals, in spite of only SCD individuals.