The Population-based Haemoglobinopathy Registry (PHR) is a crucial tool for effectively planning and overseeing haemoglobinopathy prevention and treatment initiatives within a city, state, or nation. It functions as a vital repository of information, facilitating a more profound comprehension of patients’ health statuses, treatment methodologies, and adherence, thereby enhancing surveillance and pinpointing deficiencies within the local healthcare infrastructure. A collaborative effort between IPH and JSS co-PIs conducted a scoping review to identify knowledge gaps for fortifying health systems in managing Sickle Cell Disease (SCD). This review highlighted the absence of care models tailored to specific tribal populations and the dearth of long-term data on health outcomes and associated conditions among Indian tribal communities. This initiative aims to establish a comprehensive PHR encompassing the three most prevalent haemoglobinopathies (SCD, thalassemia, and Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency). By doing so, it aims to ensure the delivery and monitoring of appropriate and accessible haemoglobinopathy services to the community while simultaneously enhancing the capacity of the public health system to sustain these efforts over the long haul.

 The screening for haemoglobinopathy within a cohort of 10,000 individuals will begin concurrently with the integration of individuals identified through the National Sickle Cell Anemia Elimination Mission’s screening program by the District Health Office (DHO) of Chamarajanagar into the Personal Health Record (PHR). Spearheaded by JSS Medical College (JSSMC), the PHR initiative, in collaboration with the DHO and the Center, will prioritize the implementation of key protocols to ensure confidentiality and restrict access for security purposes. The registry will collect comprehensive data across six essential dimensions, including demographic details, diagnosis information, laboratory and clinical parameters, treatment records, and linked health services, enabling holistic health management and surveillance. WP-3, in collaboration with other projects within the CTRITH, will identify and monitor individuals diagnosed with Sickle Cell Disease. As part of the state sickle cell disease technical expert committee in collaboration with the state government, the registry will also include patients identified from other districts.

Once a patient is identified for the registry, we will gather all necessary patient information and offer screening for family members, including extended family, at the household level. Patients will receive counseling on various aspects of the disease, including inheritance, recurrence risk, manifestations, complications, and prevention strategies. Each patient will also receive a helpline number from VGKK hospital for further assistance or queries regarding their condition. Additionally, patients will undergo annual follow-ups unless there are additional events throughout the year. During these follow-ups, we will collect specific event-related data if there are any events related to their condition. Otherwise, we will conduct a detailed review of their health history and treatment, along with a specific physical examination.

Acknowledging the inadequacy of an effective system for haemoglobinopathy care within the current healthcare framework, we intend to establish a comprehensive haemoglobinopathy care model at VGKK Tribal Hospital and a mobile health unit. This model will cater to the needs of research participants, offering essential hemoglobinopathy care services such as intravenous fluid therapy, pain management, supply of folic acid tablets, necessary vaccinations, penicillin prophylaxis, timely management of infections and diarrheal disorders, as well as hydroxyurea therapy when indicated. Moreover, for patients with complex conditions such as chest syndrome, pregnancy-related complications, strokes, and others, a triage system will be in place for prompt referral to appropriate medical facilities.

In collaboration with RIAL and the District Health Officer (DHO), guided by emerging research findings, we aim to co-create a haemoglobinopathy care model tailored to the district’s specific needs. This process will involve active participation from stakeholders and community members through participatory methods. We plan to utilise a theory of change approach to continuously refine and adapt the care model to suit the local context.

Drawing from the expertise and experience of JSS Medical College in establishing haemoglobinopathy care centres in Primary Health Centers (PHCs) catering to tribal populations, we aim to leverage this knowledge to successfully implement and sustain the proposed care model.