”Multidisciplinary Research Project of screening and detection, profile, follow-up and management including preventive and health education strategy of Sickle Hemoglobinopathy in a tribal village adopted by Sumandeep Vidyapeeth and also in survey camps as well as in patients and their relatives coming at Dhiraj Hospital with risk factors, symptoms and complications pointing towards Sickle and other Hemoglobinopathy.”
Hemoglobinopathy is a global disorder. Sickle-cell disorders and Thalassemia which was commonly found in the tropics and subtropics are now common worldwide due to migration. Approximately 7% of the world population carries these disorders and that up to 400,000 children with severe hemoglobinopathies are born each year.WHO suggests that screening and genetic counseling for Hemoglobinopathy should become one of the fundamental parts of health care in most countries. It is also suggested that it becomes basis for more comprehensive community services and becomes point of entry to genetic approaches into health system. WHO also recommends global development of these services as cost effective programme, which can integrate treatment services with carrier detection and genetic counseling. However, as per Modell B.and Darlison M, “service development can be unexpectedly challenging, because it requires inclusion of genetic approaches in health systems”.6 Sumandeep Vidyapeeth is committed for integrating curative and preventive health services and vision for comprehensive services to their community stake holders which are people of Gujarat, Madyapradesh and Maharashtra especially of tribal community belt wants to start this research work as nodal entry to global vision.
This project is aimed to find out clinical spectrum and complications of Sickling disorders and other hemoglobinopathy of tribal population of Gujarat & India and to compare it with tribal groups of African origin, whose reports are available. The hypothesis on which this research focus is Sickle and other hemoglobinopathies of tribal population of Gujarat & India has different clinical spectrum, complications and requires different approach in relation to prevention and treatment.
The study may result into an outcome to generate need based comprehensive service approach for screening, treatment and counseling of patients and their relatives such that these inherited condition’s morbidity and mortality can be decreased in future. Not only can that by proper counseling prevalence of this inherited condition also decrease.
- To find out profile and prevalence of signs, symptoms, complications, co-morbidities, hematological, biochemical, genetic, radiological and other spectrum of sickling disorders which may include Sickle cell disease (SCD), Sickle cell trait (SCT), and Sickling disorders associated with other hemoglobinopathy. (Sickle beta Thalassemia,) (…..will be referred as sickling disorder group now onwards in the project)
- To find out profile of infective complications especially prevalence of malaria in sickling disorders patients.
- To screen neonates, school children, tribal community in adopted village, relatives of pediatric (parents and sibling) and adults (spouse and children) of sickling disorders patients,
- To find out special problems like orthopedic, critical care and to find how best they could be managed.
- How best preventive, therapeutic and counseling and need based investigational facility can be created,
- To see the natural history of sickling disorders patients by follow up and longitudinal study.
Type of Work: Prospective, Observational and Descriptive as well as cohort and longitudinal; Community as well as Institutional based: Epidemiological and Clinical study which is, Hypothesis based EviGenCHIP research.
- Methodology: Duration of study: Study can be of two phases and will start simultaneously. First phase sample collection:-One year which may continue depending on available resources and research interest.
- Second phase study which is Continuous and Longitudinal: – will continue at least for 5 years to start with, which may continue depending on available resources and research interest.