Sickle-cell Disorder (also known as sickle-cell anemia or sickle-cell disease) is a common genetic condition due to a hemoglobin disorder, the molecule in red blood cell that delivers oxygen throughout the body. Sickle cell disease (SCD) is caused by a ‘mutation in the hemoglobin (HBB) gene causing a single amino acid Val, valine for glutamic acid replacement) leading to a disorder have atypical hemoglobin molecules called hemoglobin S, which can distort RBCs into a sickle, or crescent shape’. Sickle cell trait (SCT) is the condition, in which the person inherited the sickle cell gene sickle cell disease (SCD).
In context of Nepal, SCD is most common disorder among the indigenous Tharu population of western Terai region. A study done by Nepal Health Research Council titled “Prevalence of anemia among the reproductive age groups of Female” with a sample of 781 which shows 19 (2.4%) of the Tharu women were affected from sickle cell trait. Hence; Nepal Health Research Council (NHRC) with support from Ministry of Health and Population (MoHP) conducted the study on SCD in Tharu population of Bardiya districts to determine the population based prevalence of SCD and its relation to socio demographic factors, clinical symptoms in people aged 1-29 year and also provide Post test Counseling to the all the participants.
- Majority of the participants 4073 (20.4%) were of age group 11-15 followed by age group
16-20 years 3454 (17.3%)
- About 60% of the participants were female.
- Desauri 11902 (59.5%) and Dangaura Tharu 7943 (39.7%) were the most common
sub-group of Tharu ethnicity in this study.
Prevalence of Non-Sickling Hemoglobinopathy
(Out of 20000 population $428 (27%) had Non-Sickling Hemoglobinopathy among them
majority 4035 (20.2%) had Reduced A2 followed by Beta thalsssemia 1011 (5.1%)
Prevalence of Sickle cell disorder (SCD)
- Out of 20000 population 2256 (11.3%) have SCD among them 2111 (10.6%) of the participants were found to have SCT and 145 (0.7%) of the participants have sickle cell diseases.
- Among 2256 SCD population, sickle with Beta thalassemia was found in 232 (10.2%) followed by Sickle with D Punjab 6 (0.3%), Sickle with suggestive D Iran 6 (0.3%).
- Among 7982 males, 874 (10.9%) were affected by SCT and 75 (0.9%) were affected by SCD. Similarly, in 12018 female, 1237 (10.3 %) had sickle cell trait and 70 (0.6%) had SCD.
- Out of 11902 of Desauri sub-group of Tharu population, 1284 (10.8%) were affected by SCT and 82 (0.7%) was affected by SCD. Likewise out of 7943 Dangaura Tharu, 801(10.1%) had SCT and 61 (0.8%) had SCD.
- Majority of the participants complained of frequent fever 497 (23.5%) followed by
headache 313(14.8%) and abdomen pain 274 (13%) among participants with SCT.
- Participants with SCD complained of frequent fever 67 (46.2%), headache 54 (37.2%),
Extremities (hand and foot syndrome) 54 (37.2%) and joint pain 48 (33.1%).
- Post test counseling was provided to all the participants.
Credits: NHRC Press Releases
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