Madhya Pradesh sickle cell mission: An initiative for screening, counseling and treatment of sickle cell disorders among tribal communities.

Abstract

Sickle cell disease (SCD) is a structural hemoglobin disorder that causes anemia in affected individuals. In addition to inhibiting a person’s natural growth, it causes extreme pain crisis, acute chest syndrome, high fever, and periodic swelling in different parts of the body. It is a hereditary disease that can be passed on from an infected parent to a child. In India, it is mainly reported among the tribal population of central and southern India.[1] In addition, 27 of the 45 districts of Madhya Pradesh fall into the sickle cell zone, and the pervasiveness of sickle hemoglobin (HbS) fluctuates between 10% and 33%. Four tribal districts of the state namely Alirajpur, Anuppur, Chhindwara, and Dindori constitute around 75% of the existing cases of sickle cell anaemia according to the annual report 2020–2021.[2] In these districts, children and pregnant women have died due to inadequate access to facilities and improper diagnosis or miscarriage. Vector borne diseases especially malaria is highly prevalent in the tribal population due to them residing in the forest areas. Malarial parasites affect only the red blood cells (RBC). To prevent malaria, RBCs have to undergo a change in shape from round to sickle shape but due to the change in the shape of RBC, the RBCs before reaching the age of 90–120 days undergo senescence. The primary treatment, hydroxyurea, has several side effects, including an increased risk of infection and tumor development. In addition, this drug is ineffective in one-third of the patients. As a result, new medicines for the successful treatment of sickle cell anemia (SCA) with low-adverse effects must be developed. Thus, new genome editing technologies like clustered regularly interspaced short palindromic repeats_(CRISPR)-Cas9 complex may be utilized to make precise alterations to SCA patients’ genomes and create a corrected gene version. Gene-corrected autologous CD34+ hematopoietic stem/progenitor cells obtained from a cohort of Indian SCA patients will also be generated and characterized, In non obese diabetic (NOD) scid gamma (NSG) mice, the functional efficiency of gene-corrected human stem cells (HSCs) will be verified.[3] The recipients will also receive customized Hemoglobinopathy genetic counseling cards developed by a doctor couple of Bhopal district. The Hemoglobinopathy Genetic Counseling Card, also known as GIPCI (Genetic Inheritance Prediction Counseling Identification), is a specially designed card for the prevention of sickle cell anemia and thalassemia that accurately predicts the monogenic disorder that will occur in the new born of a husband and wife.[4] The mission is divided into three parts: Screening, sickle patient treatment, and system strengthening. Pregnant women (door-to-door), school-aged children, and first-degree relatives of positive individuals will be actively screened. The project management team will conduct door-to-door screenings of pregnant women, school-aged children, and family members of positive cases. If a pregnant woman tests positive for an illness or characteristic, her husband is also tested. This screening is a time-consuming process that requires three team members (one auxiliary nursing midwifery (ANM), one lab technician, and one resource person). The resource person organizes daily screenings and arranges for resources (screening consumables, data collection, vehicle arrangements, etc.). Pre-screening counseling, sample collection, and antenatal care (ANC) check-ups will be handled by ANMs, and all tests will be performed by lab technicians. Two to three milliliters of blood will be transferred to the district hospital for this test. High Performance Liquid Chromatography (HPLC) test will be done on the staples in the district hospital.[5] If appropriate screening and diagnostic facilities are provided, patients will be diagnosed and, with appropriate facilities for counseling, treatment, and management, these affected patients can lead normal lives to contribute to their personal and agricultural growth and development. The genetic counseling card will play a pivotal role in early detection and timely intervention. If the couples match before marriage, the card immediately shows how likely it is that their children will develop the disease. In addition, a sickle cell couple can recognize the possibility of the disease in the next child as well. It will play an important role in family planning as well as awareness creation among tribes. Various key stakeholders at state level can also engage in dialogues based on primary results of intervention and suggest changes or modifications to expedite the mission. This step taken by the Honorable Prime Minister and the Madhya Pradesh Government will be of cardinal concern as tribal health in Madhya Pradesh has seen a setback due to SCDs in the tribes that are yet untouched and intervention programmes such as this mission will acquire a detailed picture of the disease and role of various interventions in disease prevention. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.