Sickle cell disease in Indian tribal population: Findings of a multi-centre Indian SCD registry.

BACKGROUND: Sickle cell disease (SCD) registries provide crucial real-world data on demographics, epidemiology, healthcare, patient outcomes, and treatment efficacy. This paper presents findings from the Indian SCD Registry (ISCDR) on clinical manifestations, crisis episodes, disease management, and healthcare utilization in patients with SCD from 12 primary health centres (PHCs) in six tribal districts of India. METHODS: The ISCDR was introduced along with a three-tier screening process. Its Android-based application incorporates two electronic case report forms for patient data collection over one year. This paper presents a year's data from the ISCDR's 324 patients with SCD. RESULTS: Patients with SCD, aged one to 65 years, exhibited varied clinical manifestations. Most patients (85.2 %) were unaware of their SCD status before enrolling in ISCDR. Moderate to severe anaemia was prevalent (66.05 % and 30.56 %, respectively). Pain was a common complaint (80.86 %; CI: 76.17-85.00), while symptoms of stroke included sudden severe headaches (34.57 %; CI: 29.40-40.02). Common splenic sequestration symptoms included stomach pain (42.90 %; CI: 37.44-48.49) and abdominal tenderness (13.27 %; CI: 9.77-17.46), as a sign. Healthcare utilization was high, with 96.30 % receiving treatment and 83.64 % consuming hydroxyurea. Hospitalization occurred for 38.27 % (CI: 32.95-43.81), and 12.04 % (CI: 8.70-16.09) had blood transfusion during last year. CONCLUSIONS: ISCDR serves as a dynamic digital database on SCD epidemiology, clinical aspects, treatment and healthcare utilization. Notably, many patients lacked prior awareness of their SCD status, underscoring the need for improved awareness and care management. Integrating the registry into the national programme can streamline treatment implementation, prioritize management approaches, and optimize individual benefits.

Antihyperglycemic and antihyperlipidemic effects of fruit extract of Hodgsonia heteroclita (Roxb.) Hook. f. & Thomson in diabetic mice.

ETHNOPHARMACOLOGICAL RELEVANCE: Hodgsonia heteroclita has been known as an important traditionally consumed medicinal plant of North-East India known to have antidiabetic properties. This study aims to investigate the effects of the ethanolic fruit extract of Hodgsonia heteroclita against hyperglycemia and hyperlipidemia by using streptozotocin (STZ) treated diabetic mice. MATERIALS AND METHODS: The fruits of H. heteroclita were collected from the various parts of Kokrajhar district, Assam India (Geographic coordinates: 26°24'3.85″ N 90°16'22.30″ E). Basic morphological evaluations were carried out by the Botanical Survey of India, Eastern circle, Shillong, who also certified and identified the plant. Hexane, chloroform, and ethanolic extracts of the fruit of H. heteroclita were investigated for α-amylase inhibition assay as a rapid screening tool for examining anti-diabetic activity. The efficacy of ethanolic extract at a dose of 100, 200, and 300 mg/kg body weight was tested for 21 days in STZ-induced diabetic mice. The body weight, fasting plasma glucose and serum lipids, and hepatic glycogen levels were measured in experimental animals to examine the antihyperglycemic and antihyperlipidemic efficacy of the extract. Both HPTLC and LC-MS analysis was performed to examine the phyotochemicals present in the ethanolic extract of H. heteroclita. RESULTS: It has been observed that treatment with the ethanolic extract dose-dependently reduced the plasma glucose levels, total cholesterol, low density lipoprotein-cholesterol, very low-density lipoprotein-cholesterol, triglyceride, and increased the body weight, liver glycogens and high-density lipoprotein-cholesterol in STZ treated diabetic mice. HPTLC demonstrated the presence of triterpene compounds and LC-MS analysis revealed the presence Cucurbitacin I, Cucurbitacin E, and Kuguacin G as the triterpene phytoconstituents. CONCLUSION: The present study demonstrated that ethanolic fruit extract of H. heteroclita improved both glycemic and lipid parameters in mice model of diabetes.

Stigma of sickle cell disease among Indian tribal population: A multi-centric qualitative study.

BACKGROUND: Sickle Cell Disease (SCD) is the most prevalent hemoglobinopathy, impacting around 5% of the global population. The Indian tribal population, which has been a key focus of the Indian SCD program, can experience health-related stigma due to the multidimensional impact of the disease. This preliminary qualitative inquiry delves into the lived experiences of individuals and synthesizes domains to identify the sources of stigma. METHODOLOGY: The study's framework for developing the stigma tool was rooted in Bronfenbrenner's Ecology of Human Development. The study was implemented in five tribal-dominated districts of India and involved in-depth interviews with sickle cell disease (SCD) patients and their caregivers to explore their stigmatizing experiences. RESULTS: The analysis revealed four overarching themes and several subthemes explaining the type of stigma, its source, and factors contributing to stigmatization. First, the study focused on elements associated with perceived stigma, such as disclosure, self-isolation/refusal to participate, and self-judgment. The second theme pertained to the internalization of stigma. The third theme addressed experienced stigma concerning the disease's impact on day-to-day events, and the fourth theme explored the support system patients needed. The framework highlighted the varying degrees of stigmatizing components within different aspects of patients' ecology. CONCLUSION: Our study highlights the importance of addressing stigma at various levels. Policies, programs, and healthcare interventions must target stigma across these levels. Culturally adaptive tools for identifying stigma, implementing appropriate interventions, and improving healthcare participation are essential for enhancing the quality of life and reducing the disease burden.

Indian sickle cell disease registry for surveillance and patient management: Development and implementation.

In India, sickle cell disease (SCD) predominantly occurs in indigenous (tribal) people, who are about 104 million. However, screening and diagnosis seldom happen. This situation necessitates developing a comprehensive SCD care model, including a registry. This paper describes the development and implementation of the Indian SCD registry (ISCDR) in six tribal-dominated districts of India. The ISCDR was created in two components-(i) an Android-based mobile/tablet application, (ii) a dashboard/admin panel for patients' data management and retrieval. Data capture involves two electronic case report forms (CRF), that is, the primary form (CRF-1) and the repeat visit form (CRF-2). CRF-1 is completed as soon as the patient is found positive and captures the patient's information, including medical history, diagnosis, symptoms, precipitating factors, hospitalisation history and treatment received. Issues related to quality, security and data-sharing were addressed. After the screening system was functional, ISCDR was initiated. In 12 months, data of 324 SCD patients and 1771 carriers were entered. The study demonstrates the feasibility of establishing an SCD registry in India. It collects systematic longitudinal data on SCD patients, which are essential for programme planning and management. Further, it is feasible to scale up and integrate with other health management databases.

Iodine Status, Thyroid Disorder and Feto-Maternal Outcome among the Tribal Pregnant Women of Eastern Himalayas.

Introduction: Iodine deficiency and thyroid disorder during pregnancy have adverse effects on fetal and neonatal outcomes. Objective: To assess iodine status and thyroid functioning during pregnancy and to evaluate the feto-maternal outcome. Methods: Urinary iodine content (UIC) is determined by arsenic cerium catalytic spectrophotometry method and thyroid hormone analysis was carried out by chemiluminescence assay. Fetal and neonatal outcomes were obtained from hospital records. Results: Among the considered tribal pregnant women 56.75% had insufficient urinary iodine and 24.5% had a thyroid disorder. Thyroid disorder was more common in pregnant women with urinary iodine concentration (UIC) <99 µg/L than UIC >150 µg/L (56.75% vs 41.5%). Pregnant women with UIC<99 µg/L had a higher incidence of anemia (86.36%), gestational diabetes mellitus (GDM) (3.33%), and preeclampsia (5.71%) than UIC >150 µg/L. The fetal outcome with UIC <99 µg/L had a higher incidence of low birth weight (9.09%) and preterm births (1.9%). Stillbirths were distributed equally among different UIC groups. The neonatal outcomes with UIC <99 µg/L between 150-249 µg/L had a higher incidence of respiratory distress (5.23%). Hypothermia was equally distributed among different UIC groups. Subclinical hypothyroid had a high prevalence of anemia (62.96%), preeclampsia (3.7%), and GDM (6.17%) respectively than the euthyroid group. The fetal outcome with low birth weight (LBW) (9.87%), stillbirths (3.7%), and preterm birth (8.64%) was more common in the subclinical hypothyroid than in the euthyroid group. Among the neonatal outcomes respiratory distress (6.17%) and hypothermia (4.93%) were more common in subclinical hypothyroid than euthyroid pregnant women. Conclusion: Insufficient maternal iodine and thyroid disorders during pregnancy were associated with adverse pregnancy outcomes.

Feasibility of population-based screening of sickle cell disease through the primary health care system in tribal areas of India.

OBJECTIVE: To describe the development and implementation of a population-based screening programme for sickle cell disease (SCD) implemented in 12 SCD-endemic and tribal-dominated primary/community health centres (PHCs/CHCs) across six districts of India. SETTING: India reports a huge burden of SCD, especially among indigenous (tribal) communities. However, there is no state-led SCD programme in many places, and systematic screening is absent. This situation necessitates developing a model of population screening. METHODS: This programme was meant to screen all people and was carried out in three tiers. The first tier was a symptomatic survey carried out by community health workers. Regular health workers then screened those referred by sickle cell solubility test at sub-health centres as the second tier. The third tier was confirmation by haemoglobin electrophoresis at PHCs/CHCs. Communities were mobilised and prepared to accept the screening. Capacity building of health facilities was ensured through training and supply of equipment and material. RESULTS: Initial observation based on six months' data revealed that out of the 110,754 tribal population of 12 PHCs/CHCs, 8418 (7.6%) were identified in the symptomatic survey. Subsequently, 9416 people, including the above 8418, underwent the solubility test, and 2607 (27.7%) were found to be positive. Of these, 1978 (78.9%) underwent electrophoresis. About 64.2% were found to be positive for sickle haemoglobin (233 (18.4%) SCD and 1036 (81.6%) SCD trait). CONCLUSIONS: The study demonstrates the feasibility of establishing a population-based screening programme in the primary health care system. It is easy to implement in tribal habitations as part of the proposed national SCD/haemoglobinopathies programme.

Strengthening Health System and Community Mobilization for Sickle Cell Disease Screening and Management among Tribal Populations in India: An Interventional Study.

Sickle cell disease (SCD) affects 5% of the global population, with over 300,000 infants born yearly. In India, 73% of those with the sickle hemoglobin gene belong to indigenous tribes in remote regions lacking proper healthcare. Despite the prevalence of SCD, India lacked state-led public health programs until recently, leaving a gap in screening and comprehensive care. Hence, the Indian Council of Medical Research conducted implementation research to address this gap. This paper discusses the development and impact of the program, including screening and treatment coverage for SCD in tribal areas. With a quasi-experimental design, this study was conducted in six tribal-dominated districts in three phases - formative, intervention, and evaluation. The intervention included advocacy, partnership building, building the health system's capacity and community mobilization, and enabling the health systems to screen and manage SCD patients. The capacity building included improving healthcare workers' skills through training and infrastructure development of primary healthcare (PHC) facilities. The impact of the intervention is visible in terms of people's participation (54%, 76% and 93% of the participants participated in some intervention activities, underwent symptomatic screening and demanded the continuity of the program, respectively), and improvement in SCD-related knowledge of the community and health workers (with more than 50% of net change in many of the knowledge-related outcomes). By developing screening and treatment models, this intervention model demonstrated the feasibility of SCD care at the PHC level in remote rural areas. This accessible approach allows the tribal population in India to routinely seek SCD care at their local PHCs, offering great convenience. Nevertheless, additional research employing rigorous methodology is required to fine-tune the model. National SCD program may adopt this model, specifically for community-level screening and management of SCD in remote and rural areas.

Sickle cell disease-related knowledge and perceptions of traditional healers in tribal communities in India: implications on sickle cell disease programme.

Sickle cell disease (SCD) is a progressively debilitating genetic disease, and India is the second most affected nation in the prevalence of births with SCD. This SCD prevalence is high among Indian indigenous tribal communities, whose healthcare is pluralistic. Traditional healers are an essential part of tribal pluralistic care. This study aimed to understand the extent of SCD-related knowledge and practices of traditional healers and their willingness to participate in the SCD programme, which is primarily meant to screen and treat SCD. Following the grounded theory approach, data were collected by in-depth interviews with 40 traditional healers selected from five SCD endemic districts. Text data were coded through a deductive approach, and thematic content analysis was carried out. A few healers knew about SCD. However, almost all are aware of anaemia and its symptoms. Most healers were unaware of the cause of SCD and mentioned that malnutrition and anaemia are reasons for the recurrence of SCD-related symptoms. Most of the traditional healers did not give any treatment. Some gave symptomatic treatment and provided herbal medicines along with some rituals. Though some healers treated some of the typical symptoms of SCD like spleen enlargement, jaundice, swelling and pains in joints, they did not link them with SCD. All traditional healers expressed concern and said they support the government-run SCD programme. The programme should recognise the role and importance of traditional healers. Necessary education on SCD may be given to the healers. Such involvement and education empower the healers in appropriately guiding the people concerning SCD care.

Peripheral health workers' knowledge and experience related to sickle cell disease: an in-depth interview study in six tribal-dominated districts of India.

This study reports the extent of sickle cell disease (SCD)-related knowledge and management practices of peripheral health workers located in tribal areas of India. This formative qualitative study used a grounded theory approach and collected data through in-depth interviews. It was implemented in six districts endemic to SCD. From each district, four primary health centre (PHC) areas, predominantly inhabited by tribal communities, were included. The participants were 120 peripheral health workers, in two categories-regular health workers (RHWs) and community health workers (CHWs), working in 24 PHCs. Most of the RHWs and a little lesser number of CHWs were aware of SCD, and most of them knew it as a blood disorder. About half of the health workers reported that SCD is hereditary; however, some attributed it to malnutrition and considered it anaemia. Many health workers could tell some typical symptoms of SCD, including anaemia and severe pain. None of the PHCs offered management/treatment of SCD. And none of the health workers has any expertise in managing SCD patients. However, some health workers dealt with SCD cases and provided symptomatic treatment. Though several health workers said that SCD is preventable, comprehensive knowledge on prevention was lacking. Some health workers mentioned mass screening, counselling, and creating awareness. Thus, this paper reports the inadequacy of SCD-related knowledge and management practices among health workers. Health workers should know their roles and capabilities in managing SCD. They should be provided in-service, regular, and structured training programmes as part of the comprehensive SCD programme.

Compound heterozygosity for hemoglobin S and hemoglobin E in a family of Proto-Australoid origin: a case report.

BACKGROUND: Hemoglobin S and E are commonly occurring hemoglobin variants among distinctly separate tribal populations of Central and Northeast India, respectively. Combined heterozygosity for hemoglobin S and E or hemoglobin SE disease is a benign clinical condition with rare incidence. Reports of approximately 46 hemoglobin SE cases are available worldwide. We conducted a screening program to study the prevalence of hemoglobin variants among the tribal population working in the tea estates of Northeast India. A total of 551 subjects were screened, and complete blood count was performed. Based on their hematological profiles, hemoglobin typing was done for 218 subjects. CASE PRESENTATION: We describe a case of an adolescent male of Munda tribe diagnosed as double heterozygous for hemoglobin S and E. On screening of the nuclear family of the subject, the mother was found to have hemoglobin E disease and father as hemoglobin S trait. Both siblings of the subject were diagnosed as hemoglobin E trait. CONCLUSION: This is the first case of compound heterozygous for hemoglobin S and E to be reported from the tea tribes of Assam, India.

Inadequate community knowledge about sickle cell disease among the Indian tribal population: a formative assessment in a multicentric intervention study.

BACKGROUND: Sickle cell disease (SCD) is a serious public health problem, with >300 000 affected births worldwide each year. About 73% of the SCD-affected people in India belong to the tribal population. The Government of India is planning to implement a programme for SCD and hence people's knowledge of SCD is crucial. This paper reports the SCD-related knowledge among the Indian tribal population. METHODS: As part of a formative assessment before an intervention, data were collected from 9837 adults from 24 primary health centre areas of six states. Each community's knowledge of SCD was elicited through an interviewer-administered pretested questionnaire. Univariate and multivariate analyses were conducted. RESULTS: Overall, 32.1% (CI 31.2 to 33.1%) of participants had heard of SCD, 7.9% (CI: 7.3 to 8.4%) knew that SCD is hereditary, 19.4% (CI: 18.7 to 20.3%) knew that a blood test can diagnose SCD and 23.9% (CI: 23.1 to 24.8%) knew that SCD is treatable. Only 13.1% (CI: 12.4 to 13.8%) knew that SCD can be prevented. No more than 16% knew about any SCD symptoms. Multiple logistic regression revealed some predictors of basic knowledge (i.e. had heard of SCD). CONCLUSIONS: There is a gross inadequacy of knowledge about SCD in the Indian tribal population. This study warrants implementing a health education programme as a part of the SCD programme.

Improving the Capacity of Health System and Community for Sickle Cell Disease Screening and Management Among Tribal Population in India: Protocol of an Intervention Study.

Sickle cell disease (SCD) is one of the major public health problems in the world. In India, the burden of SCD is comparatively high in socio-economically disadvantaged tribal communities. Though efficacious interventions are available to manage SCD, they are not reaching to these communities and no comprehensive programme is in place in the health care system. Therefore, the Indian Council of Medical Research has initiated a nation-wide study to develop an effective intervention model for SCD patients in tribal areas through the government health care system. This intervention includes increasing awareness and preparing the communities for accessing the government health care system for SCD care, and improving the capacity of the primary health care systems including the training of the health care providers on prevention and management of SCD. The study adopted a quasi-experimental design with pre-vs. post-intervention comparisons of outcome variables within the interventional groups and with the control group. The study will be implemented in 6 districts which are endemic for SCD, spread across different geographical zones of India. In each district, four primary health centre (PHC) areas which are predominantly inhabited by tribal population will be selected. Of these four PHC areas, two will be selected randomly for implementing the intervention and the remaining two will be the control area. Information necessary for development and implementation of the intervention will be gathered during formative research, by using both quantitative and qualitative research methods. Intervention with an inclusive partnership and community mobilization will be implemented. The major steps in the implementation of intervention are partnership building with various health and non-health partners including the community. Capacity building and strengthening is another important component to enable the primary health facilities to screen and manage SCD patients. Primarily, sub-health centres and primary healthcare centres will be equipped with appropriate SCD screening techniques. All doctors in the system will be trained in advanced treatment and management issues. To improve the community's awareness and readiness, community mobilization activities will be conducted. An impact evaluation will be carried out at the end of the intervention by comparing the improvement of SCD management in intervention PHCs to that of the control PHCs. However, the process evaluation and necessary mid-term corrections will be made throughout the intervention period. Thus, an intervention model in terms of its suitability, replicability and sustainability for the tribal population will be developed and tested. The findings of this study are more suitable to use during advocacy and to replicate the model by the state health departments. This study develops and places an appropriate referral system for SCD patients at the PHC level. Improving the community's access to health care, improving the quality of care in government health centres and raising awareness among tribal communities are crucial to achieving through innovation. Taken together, these innovations would significantly contribute to better access to health care and management of the SCD patients of underserved tribal population.

Non-invasive assessment of adrenocortical function as a measure of stress in the endangered golden langur.

The golden langur (Trachypithecus geei) is an endangered primate endemic to northern India and Bhutan. The main stressors to the species are habitat degradation and fragmentation. Non-invasive fecal glucocorticoid metabolite (fGCM) analysis is a powerful tool for assessing stress associated with environmental disturbances in wildlife. However, interspecific differences in glucocorticoid metabolism require careful selection of the antibody used in their quantification. The goals of this study were to: 1) validate an enzyme immunoassay (EIA) to determine fecal GC metabolite (fGCM) concentrations in the golden langur and 2) compare fGCM concentrations between golden langurs living under different environmental conditions. We compared five enzyme immunoassays for determining stress related physiological responses in golden langurs by performing an adrenocorticotropic stimulation test in both sexes. Our validations identified an 11-oxoaetiocholanolone EIA detecting 11, 17 dioxoandrostanes as the most suitable assay for monitoring adrenocortical activity in the species. FGCM concentrations from semi-captive temple langurs were significantly higher than respective stress steroid concentrations in free-ranging or pet/zoo langurs. This study presents a validated practical method for non-invasive monitoring of adrenocortical function in this rare and declining species.