Sickled but Not Silent: Community Screening and Awareness of Sickle Cell Disorders among Tribal Adolescents in Nashik District, Maharashtra

INTRODUCTION:

Sickle cell disease is a lifelong, debilitating public health problem impacting the quality of life. It is a monogenic inherited blood disorder (hemoglobinopathy) caused by changes in β globulin gene (HBB) resulting in formation of abnormal hemoglobin and red cells become stiff and sickle shaped.1,2 Blockage of blood capillaries restricts the blood flow and oxygen supply leading to development of chronic pain, severe anemia, infection, kidney and cardiovascular complications, high risk of infection, complications during pregnancy etc. Inheriting two hemoglobin S (HbS) genes causes sickle cell disease (SCD) while person with one HbS gene develops sickle cell trait (usually with no symptoms but can transmit the disease to next generation).^1,3

According to the Global Burden of Disease Study (GBD) in 2021, nearly 7.7 million people worldwide had sickle cell disease and about 515,000 newborns were diagnosed with the disease. The global SCD birth rate was reported to be 382 per 100,000 live births. The global cause specific death and total deaths due to SCD were 34,400 and 376,000 respectively in the same year. Western and Central Sub-Saharan Africa and India reported highest SCD disability burden. India accounts for 82,500 newborns with SCD cases representing 16% of global incident cases while the total number of sickle cell disease across all ages was 1,280,000.⁴

According to 2011 Census, the tribal population (scheduled tribes) in India was around 104 million constituting 8.6% of total population.5 The tribal population in Maharashtra state comprises of 10.5 million (~10%) of total tribal population of India. One of the districts in Maharashtra, Nashik hosts nearly 1.6 million tribal population estimating around 14.9% of total tribal population of Maharashtra.⁶

The sickle cell disease is a significant health concern in tribal population as they face vulnerability due to limited accessibility to healthcare facilities, low awareness and poor socio-economic conditions. It is also observed that SCD is prevalent among tribal population. The highest prevalence of sickle cell gene in some tribes of Maharashtra ranges from 20% to 35%.⁷

National Sickle Cell Anemia Elimination Mission by the Government of India was launched on July 1, 2023 which targeted on eliminating the disease by 2047. Around 60 million people have been screened out of targeted 70 million (aged 0-40 years) for the disease with around 0.2 million SCD cases and 1.67 million carriers and highest incidences were reported in the states of Odisha, Chhattisgarh, Madhya Pradesh, Maharashtra and Gujarat. The mission focuses on creating awareness, affordable and accessible services, counselling and holistic management for the disease.⁸

Early detection of sickle cell disease and sickle cell traits is important for reduction of death, preventing complications and improvement of quality of life. Screening in high risk populations helps in identifying the disease and that will enable timely supportive care, preventive treatment and counselling.9 The study was conducted among adolescents aged 12-16 years as this age group faces additional stress due to disease progression and it provides a critical window for early diagnosis, timely management and creating awareness to reduce stigma ultimately decreasing disease severity and burden.10 Therefore, the present study was conducted to assess prevalence, clinical symptoms and complications, evaluate community awareness and provide insights for planning early interventions and education programs. By focusing on a vulnerable and often neglected age group, this study will provide valuable insights that may help in understanding the epidemiology of SCD in tribal Maharashtra and may support clinical decision making and policy initiatives for early diagnosis and care.

MATERIALS AND METHODS:

It was a cross sectional observational study conducted among adolescents 12-16 years of age in tribal villages of Nashik District, Maharashtra, India during July 2024 to June 2025. The study aimed to assess the prevalence of Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT) among the study population along with associated factors and level of awareness.

The study area, Nashik had 15 administrative blocks, of which 9 were tribal blocks. According to data and reports from the National Sickle Cell Disease Control Programme provided by state health authorities, 7 tribal blocks with reported SCD were considered for study. Four (4) villages were selected from each of these blocks using simple random sampling leading to 28 villages for the study.

Adolescents both male and female aged 12-16 years of age, permanent residents of the selected villages and given written informed assent and parental/guardian consent were included in the study. The adolescents who had received blood transfusion within preceding 2 weeks of the study, already diagnosed with SCD/SCT, moved out of the village during the study and declined to participate were excluded from the study. Those already diagnosed with SCD/SCT were included for counselling and referral services, though not as part of the study.

All eligible adolescents in the selected villages were included in the study adopting total enumeration sampling. Total 2465 adolescents were surveyed and screened for the study. This was conducted in four structured phases such as line listing of all eligible adolescents in the selected villages, on site data and blood sample collection for screening of SCD/SCT followed by counselling and referrals along with community health education. Based on the findings and analysis, public health recommendations were made.

Approval from Institutional Ethics Committee (IEC) was obtained and all ethical guidelines were followed during the study period. The written informed assent from the participant and written informed consent from parents/legal guardians were obtained before participation in the study. Anonymity of the participants was ensured and no personal identifiers were collected.

After initial data collection about demographic factors and awareness parameters, initial screening was done using blood solubility test. This test was used as a first line screening test owing to its cost-effectiveness, immediate result, high sensitivity (93.8%) and specificity (100%).11 Positive samples were further subjected to high performance liquid chromatography (HPLC) or Hb electrophoresis for definitive diagnosis and differentiating between SCD, SCT and other hemoglobinopathies.

Structured proforma was used to collect demographic details, symptoms and detailed history. Standardized reporting formats as per state health department guidelines were used to enter laboratory results. Awareness of parents or guardians about SCD was assessed using self-administered questionnaire to understand the disease origin, transmission, symptoms, complications and preventive measures. The questionnaire was tested on 10% of the participants different from study group and finalized accordingly. Respondents were classified as aware if they correctly answered more than 50% of the questions and unaware if they scored less than 50%. Documentation about Information, Education and Communication (IEC) activities, counselling sessions, community education and health cards was maintained.

The participants who were identified as carriers/diagnosed with SCD/SCT were subjected to personalized counselling along with their parents/guardians. Color coded cards-red for SCD, yellow for SCT and white for negative results were provided. The detailed guidance on preventive aspects was given including hydration, avoidance of cold and strenuous activity, infection prevention and necessary immunization.

Data were entered into Microsoft Excel and analyzed using SPSS version 25. Descriptive statistics such as frequencies, percentages and mean were used to describe demographic and clinical variables.

RESULTS:

Out of total 2465 adolescents screened, 114 individuals (4.62%) were identified as carriers (HbAS) and 6 individuals (0.24%) were confirmed sufferers (HbSS), bringing the total prevalence of sickle-related hemoglobinopathies to 4.87%. Additionally, 171 adolescents (6.94%) were found to have other hemoglobin disorders such as β-thalassemia trait, α-thalassemia trait, HbD, and persistent fetal hemoglobin variants. The combined burden of hemoglobinopathies in this adolescent tribal population was found to be 11.81%. These findings indicate the possibly high genetic load of hemoglobin disorders in tribal areas of Nashik district.

A total of 120 out of 2465 adolescents were found to be either carriers or sufferers of sickle cell disease and trait with 57 (47.5%) males and 63 females (52.5%). (Figure 1)

Figure 1: Distribution of Male and Female Carriers and Sufferers of Sickle Cell Disease and Trait

Notably, 93 (77.5%) adolescents were asymptomatic, highlighting the silent progression of SCD/SCT; followed by anemia (26;21.6%) and pain episodes (4;3.3%).

The caste wise distribution of carriers & sufferers showed predominance of caste Kokana (72;60%) followed by Mahadev Koli (21;17.5%), Warli (19;15.8%) and Bhil (8;6.6%).

Tables 1A, 1B and 1C depict the prevalence of sickle cell disease, sickle cell carriers and other hemoglobinopathies among adolescents across different blocks, PHCs and villages in Nashik district.

Table 1 A: Block/PHC/Village-wise Prevalence of Sickle Cell Disease, Carriers and Other Hemoglobinopathies Among Tribal Adolescents (Part I) Blocks: Baglan and Dindori

Block	Name of PHC	Name of Village	Actual no. 12-16 yrs population	No. of +ve samples	Village wise Incidence	No. of Sickle Cell Carriers	Carriers Incidence	No. of Sickle Cell Sufferers	Sufferers Incidence	No. of Other Hemoglobinopathies detected	Other Hemoglobinopathy Incidence
Baglan	Aliyabad	Jaad	96	8	8.33%	8	8.33%	0	0.00%	5	5.21%
	Aliyabad	Bhimkhet	114	7	6.14%	6	5.26%	1	0.88%	8	7.02%
	Kelzar	Bundhate	123	4	3.25%	3	2.44%	1	0.81%	12	9.76%
	Kelzar	Tatani	91	4	4.40%	3	3.30%	1	1.10%	8	8.80%
	Block Total		424	23	5.42%	20	4.72%	3	0.70%	33	7.78%
Dindori	Nanashi	Palasvihir	152	4	2.63%	4	2.63%	0	0.00%	13	8.55%
	Nanashi	Mahaje	113	2	1.77%	2	1.77%	0	0.00%	10	8.85%
	Pandane	Ahiwantwadi	128	5	3.91%	5	3.91%	0	0.00%	10	7.81%
	Pandane	Chausale	145	1	0.69%	1	0.69%	0	0.00%	9	6.21%
	Block Total		538	12	2.23%	12	2.23%	0	0.00%	42	7.81%

Table 1 B: Block/PHC/Village-wise Prevalence of Sickle Cell Disease, Carriers and Other Hemoglobinopathies Among Tribal Adolescents (Part II) Blocks: Igatpuri and Kalwan

Block	Name of PHC	Name of Village	Actual no. 12–16 yrs population	No. of +ve samples	Village wise Incidence	No. of Sickle Cell Carriers	Carriers Incidence	No. of Sickle Cell Sufferers	Sufferers Incidence	No. of Other Hemoglobinopathies detected	Other Hemoglobinopathy Incidence
Igatpuri	Kaluste	Kurungwadi	59	0	0.00%	0	0.00%	0	0.00%	2	3.39%
	Kaluste	Shenwad Bk	100	0	0.00%	0	0.00%	0	0.00%	6	6.00%
	Vaitarana	Walvihir	53	0	0.00%	0	0.00%	0	0.00%	3	5.66%
	Vaitarana	Pimpalgaon Bhatata	44	0	0.00%	0	0.00%	0	0.00%	0	0.00%
	Block Total (Igatpuri)		256	0	0.00%	0	0.00%	0	0.00%	11	4.30%
Kalwan	Dalwat	Virshet	63	2	3.17%	2	3.17%	0	0.00%	5	7.94%
	Dalwat	Shingashi	46	2	4.35%	2	4.35%	0	0.00%	2	4.35%
	Jaidar	Sule	47	3	6.38%	3	6.38%	0	0.00%	4	8.51%
	Jaidar	Pratapnagar	108	6	5.56%	6	5.56%	0	0.00%	11	10.19%
	Block Total		264	13	4.92%	13	4.92%	0	0.00%	22	8.33%

Information, Education and Communication (IEC) activities were carried out in each selected village through 25 schools, 15 college seminars and 24 Gram Sabha sessions. Distribution of 2547 brochures and 2282 pamphlets, along with organization of 22 school rallies were conducted. Mass media and social platforms such as WhatsApp groups, community notice boards and loudspeaker announcements were also used to reach wider audiences. The goal was to educate adolescents, parents and village leaders about the nature, symptoms, complications and prevention of SCD. Notably, Pandane, Kelzar and Chinchohol villages showed highly active participation with multiple outreach formats.

Table 1 C: Block/PHC/Village-wise Prevalence of Sickle Cell Disease, Carriers and Other Hemoglobinopathies Among Tribal Adolescents (Part III) Blocks: Peth, Surgana and Trimbakeshwar

Block	Name of PHC	Name of Village	Actual no. 12–16 yrs population	No. of +ve samples	Village wise Incidence	No. of Sickle Cell Carriers	Carriers Incidence	No. of Sickle Cell Sufferers	Sufferers Incidence	No. of Other Hemoglobinopathies detected	Other Hemoglobinopathy Incidence
Peth	Kumbhale	Hatrundi	91	5	5.49%	4	4.40%	1	1.10%	10	10.99%
	Kumbhale	Dhondmal	94	12	12.77%	11	11.70%	1	1.06%	9	9.57%
	Karanjali	Usthale	60	0	0.00%	0	0.00%	0	0.00%	0	0.00%
	Karanjali	Borvat	62	0	0.00%	0	0.00%	0	0.00%	2	3.23%
	Block Total (Peth)		307	17	5.54%	15	4.89%	2	0.65%	21	6.84%
Surgana	Barhe	Thangaon	108	4	3.70%	4	3.70%	0	0.00%	6	5.56%
	Barhe	Bhegu	46	2	4.35%	2	4.35%	0	0.00%	2	4.35%
	Bubli	Shribhuvan	85	3	3.53%	3	3.53%	0	0.00%	8	9.41%
	Bubli	Waghdhond	21	0	0.00%	0	0.00%	0	0.00%	1	4.76%
	Block Total (Surgana)		260	9	3.46%	9	3.46%	0	0.00%	17	6.54%
Trimbakeshwar	Chinchohol	Deodongara	107	7	6.54%	7	6.54%	0	0.00%	6	5.61%
	Chinchohol	Goldari	116	15	12.93%	14	12.07%	1	0.86%	2	1.72%
	Thanapada	Kharshet	80	4	5.00%	4	5.00%	0	0.00%	6	7.50%
	Thanapada	Dalpatpur	113	9	7.96%	9	7.96%	0	0.00%	3	2.65%
	Block Total (Trimbakeshwar)		416	35	8.41%	34	8.17%	1	0.24%	17	4.09%
Total Block			2465	120	4.87%	114	4.62%	6	0.24%	171	6.94%

Table 2: Awareness of Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT) Among Parents/Guardians Across Tribal Villages (N=2465)

Sr No	Block	PHC	No. of Villages	No. of Parents / People Surveyed	Aware of Sickle Cell Disease (>50% correct answers)	Not Aware (<50% correct answers)
1	Baglan	Aliyabad	2	210	48%	52%
	Baglan	Kelzar	2	214	51%	49%
2	Dindori	Nanashi	2	265	60%	40%
	Dindori	Pandane	2	273	65%	35%
3	Igatpuri	Kaluste	2	159	65%	35%
	Igatpuri	Vaitarana	2	97	60%	40%
4	Kalwan	Dalwat	2	109	60%	40%
	Kalwan	Jaidar	2	155	65%	35%
5	Peth	Kumbhale	2	185	60%	40%
	Peth	Karanjali	2	122	61%	39%
6	Surgana	Barhe	2	154	62%	38%
	Surgana	Bubli	2	106	65%	35%
7	Trimbak	Chinchohol	2	223	65%	35%
	Trimbak	Thanapada	2	193	62%	38%
Total District	—	—	28	2465	60.64%	39.36%

Out of 2465 respondents,1495 (60.64%) were aware about the sickle cell disease while remaining 970 (39.36%) gave less than 50% answer showing limited knowledge. (Table 2)

A key component of this study was to ensure that adolescents diagnosed as either carriers or sufferers of SCD received post-diagnostic counseling. This was crucial for disease understanding, precautionary practices and access to follow-up care.

DISCUSSION:

In present study, total 2465 adolescents aged 12-16 years were screened from 28 tribal villages of Nashik district. The overall prevalence of sickle related hemoglobinopathies was found to be 4.87% (120 out of 2465) out of which 114 (4.62%) were identified as carriers (HBAS) and 6 (0.24%) were sufferers. Additionally, 171 (6.94%) adolescents had other hemoglobin related disorders thus the total hemoglobinopathies account for 11.81%. Around 93 (77.5%) adolescents with sickle related hemoglobinopathies were asymptomatic at the time of screening.

A systematic review and meta-analysis of Indian studies showed that national prevalence of sickle cell trait was 5.9% and sickle cell disease was 1.17%. Contrary to the present findings the prevalence of SCT and SCD in the state of Maharashtra was reported to be 14.48% and 2.06%.12 In another study by Colah et al showed that the prevalence for SCT ranges from 0% to 35%. These findings were comparatively higher than the present study.13 However, the SCD prevalence observed in present study aligned with finding (0.8%) of a systematic review and meta-analysis conducted among adolescents.14 The prevalence of present study was on the lower side of the reported range, this may be attributed due to differences in tribal composition, age group used in study, local environmental and social conditions.¹²

There were more females who were carriers and sufferers compared to males in present study. Similar observations were seen in other studies.^15,16

The overall prevalence of 11.81% of all hemoglobinopathies was found to be in 291 adolescents. Findings from other studies by Mohanty et al.17 and Ganesh et al.18 reported prevalence ranging from 8.7% to 14.7% which nearly aligned with the present study.

In present study, the sickle carriers and sufferers were highest in Kokana tribe (60%) followed by Mahadev Koli, Warli and Bhil. Contrast to present study, the sickle cell hemoglobinopathies were most commonly seen in Bhil, Madia, Pawara, Pardhan and Otkar in study by Nagisetty S et al.7 The systematic review and meta-analysis showed that prevalence of SCD and SCT was more marginalized tribal communities compared to non-tribal population.¹⁴

Among carriers and sufferers most of the adolescents were asymptomatic (77.5%) followed by signs of anemia and pain. In a study, nearly 87% SCT patients were asymptomatic, only 13% had symptoms including anemia, hemolytic crisis and pain which was consistent with present finding. As SCT and SCD are inherited blood disorders, mostly symptomless. The disease is transmitted from parents to their children may sometimes lead to severe complications including death.19 Contrasting finding was reported in a study where the children with SCD were admitted to the hospital due to severe pain, infection and acute chest syndrome.²⁰

Nearly two third parents or guardians of adolescents were aware about SCD in present study. Similarly, Das et al. found that around 74% participants were aware about the disease.21 However, another study reported only 32% awareness level showing marked difference between findings.22 The variation between studies may be due to differences in study location, population, literacy levels and health education and awareness programs.

The IEC activities and counselling using color coded cards was an innovative idea conducted to improve the awareness regarding disease, timely screening and reduction of stigma among people. This aligns with the goals of the National Sickle Cell Anemia Elimination Mission which emphasizes on screening and counselling.

Limited generalizability due to cross-sectional design and focus on few tribal communities as these might not represent the diverse genetic and socio-economic backgrounds of other tribal communities. However, the study provided an important insight into the prevalence of sickle cell disease and related hemoglobinopathies while also creating awareness and need for screening to help prevent future complications. This study will help by identifying high risk communities and may guide in designing specific programs like systematic screening using HPLC/electrophoresis in tribal schools and communities and providing a baseline for future longitudinal research to study long term impact as well as allocating dedicated resources for SCD.

REFERENCES:

1. World Health Organization. Sickle-cell disease [Internet]. 2025 [cited 2025 Oct 14]. Available from: https://www.who.int/news-room/fact-sheets/detail/sickle-cell-disease

2. Dolai TK. Sickle cell prevalence in the universe problem in 2024. J Hematol Allied Sci. 2024 June 20;4(1):1–2.

3. Government of Maharashtra. National Sickle Cell Elimination Mission | Public Health Department | India [Internet]. [cited 2025 Oct 14]. Available from: https://phd.maharashtra.gov.in/en/scheme/sickle-cell/

4. Thomson AM, McHugh TA, Oron AP, Teply C, Lonberg N, Vilchis Tella V, et al. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021. The Lancet Haematol. 2023 Aug 1;10(8):e585–99.

5. Ministry of Tribal Affairs. Year End Review 2022: Ministry of Tribal Affairs [Internet]. [cited 2025 Oct 14]. Available from: https://www.pib.gov.in/www.pib.gov.in/Pressreleaseshare.aspx?PRID=1887716

6. Office of the Registrar General & Census Commissioner, India, The Ministry of Home Affairs. Census tables | Government of India I District wise scheduled tribe population [Internet]. [cited 2025 Oct 14]. Available from: https://censusindia.gov.in/census.website/data/census-tables

7. Nagisetty S, Thatoju PK. Sickle cell disease burden among tribal population of India: a narrative review. Int J Community Med Public Health. 2024 Oct 29;11(11):4537–45.

8. Ministry of Health and Family Welfare. India achieves Milestone of 6 Crore Screenings under National Sickle Cell Anemia Elimination Mission | Ministry of Health and Family Welfare | GOI [Internet]. 2025 [cited 2025 Oct 14]. Available from: https://www.mohfw.gov.in/?q=en/pressrelease-274

9. Colah RB, Mehta P, Mukherjee MB. Newborn Screening for Sickle Cell Disease: Indian Experience. Int J Neonatal Screen. 2018 Nov 13;4(4):31.

10. Howell KE, Pugh N, Longoria J, Shah N, Kutlar A, Gordeuk VR, et al. Burden of Aging: Health Outcomes Among Adolescents and Young Adults With Sickle Cell Disease. Hemasphere. 2023 July 13;7(8):e930.

11. Surve RR, Mukherjee MB, Kate SL, Nagtilak SB, Wadia M, Tamankar AA, et al. Detection of the beta s gene: an evaluation of the solubility test against automated chromatography and haemoglobin electrophoresis. Br J Biomed Sci. 2000;57(4):292–4.

12. Rao P, Raj EA, Natesan S, Gudi N. Prevalence of Sickle cell disease, Sickle cell trait and HBS-beta-thalassemia in India: A systematic review and Meta-analysis. Clin Epidemiol Glob Health. 2024 July 1;28:101678.

13. Colah RB, Mukherjee MB, Martin S, Ghosh K. Sickle cell disease in tribal populations in India. Indian J Med Res. 2015 May;141(5):509–15.

14. Nanda AK, Pradhan SK, Supakar S, Pradhan PC. Prevalence of sickle cell disease among children in India: A systematic review and meta-analysis. Int. J. Health Sci. 2022 May 31;6(S2):13083–99.

15. Trivedi DJ. Genotypic Pattern of Sickle Cell Anaemia among Tribal Community of Dahod: A Tertiary Hospital Based Study. (IJPRP [Internet]. 2024;13(3). Available from: https://rfppl.co.in/subscription/upload_pdf/djiraj-sir,-ijprp-1737184629.pdf

16. Singh V, Biswas AK, Baranwal AK, Asthana B, Dahiya T. Prevalence of hemoglobinopathies using high-performance liquid chromatography as diagnostic tool in anemic patients of tertiary care center of Western India. Asian J Transfus Sci. 2024 Jul-Dec;18(2):257–63.

17. Mohanty D, Colah RB, Gorakshakar AC, Patel RZ, Master DC, Mahanta J, et al. Prevalence of β-thalassemia and other haemoglobinopathies in six cities in India: a multicentre study. J Community Genet. 2013 Jan;4(1):33–42.

18. Ganesh B, Rajakumar T, Acharya SK, Devika S, Ramachandran V, Yuvaraj J, et al. Prevalence of hemoglobinopathies among Malayali tribes of Jawadhu hills, Tiruvannamalai district, Tamil Nadu, India: a community-based cross-sectional study. Hematology. 2024 Dec;29(1):2350320.

19. Khaled SA, Ahmed HA, Elbadry MI, NasrEldin E, Hassany SM, Ahmed SA. Hematological, Biochemical Properties, and Clinical Correlates of Hemoglobin S Variant Disorder: A New Insight Into Sickle Cell Trait. J Hematol. 2022 June 27;11(3):92.

20. Salman ZA, Hassan MK. Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq. Anemia. 2015 Oct 26;2015:195469.

21. Das A, Dixit S, Kumar Barik M, Ghosal J, Babu B, Bal M, et al. Knowledge and perception related to sickle cell disease among tribal community, India: A mixed-method study. J Natl Med Assoc. 2023 Aug;115(4):441–53.

Babu BV, Sridevi P, Surti S, Ranjit M, Bhat D, Sarmah J, et al. Inadequate community knowledge about sickle cell disease among the Indian tribal population: a formative assessment in a multicentric intervention study. Trans R Soc Trop Med Hyg. 2021 Dec 2;115(12):1434–44

Sickled but Not Silent: Community Screening and Awareness of Sickle Cell Disorders among Tribal Adolescents in Nashik District, Maharashtra

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Abstract:

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INTRODUCTION:

MATERIALS AND METHODS:

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REFERENCES:

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