Government’s Plan of Management for Hemoglobinopathies
Hemoglobinopathies are a group of genetically inherited blood disorder in which there is either abnormal form of haemoglobin or decreased production of haemoglobin. Most common in India are variants of sickle cell disease and thalassemia. Sickle cell disease (SCD) progresses to cause a debilitating systemic syndrome characterized by chronic anemia, aplastic crisis, vascular crisis and chronic organ damage. Thereby this disease causes significant reduction in life expectancy.
If a male and a female carrying the abnormal gene trait have a baby, there is a 25% chance that their baby will be suffering from the blood disorder. For instance, sickle cell disease cuts 30 years from a patient’s lifespan. There is no cure available so the government hopes screening will prevent those carrying the trait from having babies together.
In the union budget of 2023-24, Finance Minister Nirmala Sitharaman announced to launch a mission to eliminate sickle cell anemia by 2047. This entails the goal to reduce disease occurrence with continuous measures of prevention of gene transmission to the new generation. An entire family and its successors could be saved with every patient that underwent screening and effective counselling.
Achieving this goal would involve screening at least seven crore people in the age group of 0-40 years in multiple phases by 2025-26. A budget of ₹542 crore has been proposed by National Health Mission for the massive exercise. The focus will be on awareness creation, universal screening of major population in the affected tribal areas and counselling through collaborative efforts of central ministries and state governments.
Under National Health Mission, Government of India supports the states for prevention and management of sickle cell disease as per their annual PIP proposals. The Ministry had also released technical operational guidelines for prevention and control of hemoglobinopathies in 2016 including sickle cell anemia.
With barely two weeks left in the fiscal year, the Health Ministry has completed a miniscule 1% of its ambitious target to scan one crore people for sickle cell disease in 2022-23. The Ministry is starkly behind schedule, having screened just a little over one lakh people this year, according to official data accessed by The Hindu from the National Health Mission’s portal for sickle cell disease.
The World Health Organization has clearly outlined the goals for control of hemoglobinopathies – “provide affordable and adequate therapy for those affected, while at the same time reduce the number of births of children with the disease through strong political, administrative and financial support.”
Keeping these guiding principles in mind, the vision of the National Health Mission is to provide optimal treatment to those affected and prevent the birth of children with disease through carrier screening, genetic counselling and prenatal diagnosis. Prevention and control will be achieved through the following strategies –
1) Carrying out awareness, education and screening programmes in the community and schools.
2) Establishing laboratories for carrier screening for hemoglobinopathies and new born screening for sickle cell disease at the district level.
3) Screening pregnant women and their husbands to prevent the birth of children affected with thalassemia major or sickle cell disease
4) Establishing prenatal diagnostic centres in Medical Colleges in the States where required.
For instance, State Hemoglobinopathy Mission has been established in Madhya Pradesh to tackle the challenges in screening and management of sickle cell disease. A pilot project launched by Honourable Prime Minister on 15th November 2021 for screening in Jhabua and Alirajpur district of M.P and 89 tribal blocks included in Second Phase of Project. As reported by state, total 993114 persons have been screened. Out of whom 18866 have been detected HbAS (Sickle Trait) and 1506 (HbSS sickle diseased). Further in terms of treatment, support is given under NHM for capsule hydroxyurea, free blood transfusion for all Sickle cell patients (men & women) as per State’s proposal.
State government has established Integrated Centre for Hemophilia and Hemoglobinopathies in 22 Tribal District for treatment and diagnosis of patients. It has been effective and proposed in all states especially in the tribal districts the guidelines comprising:
1. Free diagnosis and testing and analysis of hemophilia and thalassemia disease.
2. Free exercise physiotherapy and free modalities (Muscle Stimulator, IFT, Ultrasound, TENS, Weight Cuffs, CPM) given to needy patients depending on condition.
3. Free counselling of patients about life planning for better health, pre and post-marital counselling of youth, ANC mother counselling, counselling of parents of new born and children of paediatric ward, giving benefits of various schemes of Govt. for the hemophilia, thalassemia Patients
4. Free CBC test, HPLC test, coagulation profile test (PT, APTT daily on-demand basis).
5. Providing free factor VIII, VWD, IX, FEIBA, VII injection to patients with haemophilia
6. Providing free oral iron chelation tablets (Desirox 250 mg, Desirox 500 mg, Kelfer 250 mg, Kelfer 500 mg) to patients.
7. Free blood transfusion services to thalassemia patients from blood bank.
8. Awareness to the community through monthly activity of awareness camp.
9. Monthly free screening test camp for factor VIII, IX.
GUIDELINES FOR MANAGEMENT OF AFFECTED CHILDREN
“Based on rights of patients for access to care :
• To provide optimal care to all patients of thalassemia and sickle cell disease by establishing day care facilities for transfusion and monitoring with the help of State health departments at the District Hospitals.
• To ensure availability of safe blood to children with thalassemia, strengthen existing Blood Banks to provide facilities for component separation and leuco-depletion, and help the States to set up new Blood Banks or blood storage facilities where there are none; Promote voluntary blood donation to fulfil the blood requirements.
• Provide financial support for obtaining medicines for iron chelation-an essential component of management without which blood transfusions given over the years will lead to iron-overload.
• Developing and implementing protocols for early diagnosis and intervention in cases of Sickle Cell Disease (SCD) and Thalassemia major (TM). New born screening for SCD – in order to provide timely intervention with prophylactic penicillin and vaccinations (see Section C on Management) an targeted screening of children with anemia, to identify those having thalassemia trait or disease. Referral for Hematopoietic stem cell transplant (HSCT), also termed Bone Marrow Transplant (BMT) and facilitate establishment of more HSCT centres,
• Inform the community about appropriate treatment and management options and making these available through public health facilities.”
The new initiatives and vision under the National Health Mission provide an efficient framework for prevention and management of hemoglobinopathies. This includes guidelines foradequate therapy for those affected helping them lead better lives and prevention through carrier
screening, genetic counselling and prenatal diagnosis.As of now there is a massive load of the hemoglobinopathy disease burden on India. We as citizens could be a part of awareness camps and mass media campaigns to aid the government’s plan for management of hemoglobinopathies for the coming years.
“Prevention is the Cure”
References :
www.pib.gov.in
https://nhm.gov.in
https://dmgwh.in/services/integrated-centers-for-hemoglobinopathies-hemophilia/
thehindu.com
Image source :
https://www.getdoc.com/wp-content/uploads/2019/06/Sickle-Cell-01_wellness_spring2019.jpg
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