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National Sickle Cell Anaemia Elimination Mission. Need for a design re-think !!

National Sickle Cell Anaemia Elimination Mission. Need for a design re-think !!

Yogesh Jain and T. Sundararaman.
A 18 month old girl with sickle cell disease and extreme pain due to dactylitis.

The National Sickle Cell Anaemia Elimination Mission  launched by the Union government in July 2023 focuses on “Eliminating sickle cell disease as a public health problem in India before 2047 “ as well as addressing the significant health challenges posed by sickle cell disease. 1

Sickle cell disease (SCD) is an inherited blood disorder, that results in modest anaemia, often referred to as sickle cell anaemia. This condition alters the shape of red blood cells, causing them to become rigid and sticky, potentially leading to either slowing or blocking of blood flow that affects organs and causes acute painful episodes. The disease is associated with a significant risk of early childhood mortality, primarily due to infections like pneumonia. Survivors may face chronic organ damage, resulting in a notable reduction in life expectancy.

SCD is a chronic single-gene disorder requiring the inheritance of two SCD gene copies, one from each parent, for manifestation. Individuals with only one copy, known as carriers or having the sickle cell trait, exhibit no disease symptoms. The carrier frequency of the SCD gene varies widely, ranging from 1% to 35%. By the Global Burden of disease estimates , an estimated 82,500 births with SCD occur annually in India 2, and the prevalence is estimated at   1.28 million cases, making it a significant public health concern. Additionally, β-Thalassemia, another inherited blood disorder, is also prevalent in India, with carriers averaging a frequency of 3-4%.

Implemented in 17 high-focus states across the country, this Sickle Cell Anaemia Program (SCAP) aims to improve the care and prospects of all sickle cell disease patients while reducing the prevalence of the disease. The 17 states are- Gujarat, Maharashtra, Rajasthan, Madhya Pradesh, Jharkhand, Chhattisgarh, West Bengal, Odisha, Tamil Nadu, Telangana, Andhra Pradesh, Karnataka, Assam, Uttar Pradesh, Kerala, Bihar, and Uttarakhand.

The program is to be executed in mission mode as the National Sickle Cell Anaemia Elimination Mission. The Mission goal is to eliminate sickle cell genetic transmission by the year 2047, showing a long-term commitment to eradicating the disease. The main strategies outlined the Operational Guidelines issued by the Ministry of Health and Family Welfare3 are:

  1. Primary prevention strategies: involve creating awareness, providing pre-marital and pre-conceptional counselling, genetic counselling and testing interventions in high prevalence districts, and health promotion activities with widespread community involvement.
  2. Secondary prevention and Screening: approximately 7.0 crore people under 40 years of age in the next 3 years to identify those with sickle cell carrier state and those with disease.
  3. Sickle cell genetic status cards: Issued to all those screened. They would be encouraged to match this card before deciding on marriage to reduce incidence in next generation.
  4. Holistic management and continuum of care: for those affected through primary to tertiary care support, advanced diagnostics, and treatment, Ayushman Bharat enrolment giving them free access to services, The integration with AYUSH, patient support, and rehabilitation.
  5. Prioritize tribal communities in India: the mission prioritizes its intervention in the high prevalence and tribal states.

The official press release at the mission launch highlighted the Prime Minister distributing Sickle Cell Genetic Status Card to the beneficiaries and that he “kickstarted the programme with distribution of about 3.57 crore Ayushman Bharat Pradhan Mantri Jan Arogya Yojana (AB-PMJAY) cards”4. In the same meeting, the health minister also urged that people should match Sickle Cell Genetic Status Cards before marriage, to ensure that the disease is not transferred to the next generation:

As per the Mission dashboard as of February 20th, 2024, 1.98 crores have been screened, and of these 6.10 lakhs have sickle cell trait (meaning a single copy of the gene- also known as heterozygote) and 1.09 lakh have been found to have the disease (two copies of the gene – also known as homozygote) and in another 2.84 lakhs the disease is suspected after the first test, but the confirmatory second test is awaited.

While the renewed focus on a neglected illness like SCD is welcome, there are major unsettled ethical and effectiveness concerns with the current strategy-design.

  • The idea that we can eliminate a genetic disease.


Eliminating selected diseases has become a goal since we eliminated smallpox forty years ago.  We have targets for eliminating poliomyelitis, tuberculosis, malaria, kala azar, filariasis in our calendars. But all these illnesses are infectious diseases and are amenable to intervention at the causative agent and the transmitting environment besides the host for us to maintain hope in disease elimination. SCD is an autosomal recessive genetic disease whose elimination is only possible by preventing births of people with this disease. Even if there were no new cases each year, since the heterozygote state will always continue, the gene remains in the population. Even the elimination of a marriage between a man and a woman both having sickle cell trait is a near impossible goal and is fraught with many ethical, operational, and social issues. Mitigating the effects of this disease and ensuring the highest quality life outcomes for people who suffer is a more reasonable goal, which is what all other countries in the world aspire towards.

  • The conflation of sickle cell disease with Adivasis.


Surely, the Adivasis have it in higher proportion than what it would be if the disease was uniformly distributed. The sickle gene emerged and underwent selection in humans around 30,000 years ago, providing protection against severe falciparum malaria-related deaths in a heterozygote state. As falciparum malaria is most prevalent in forest and forest fringe ecosystems, regions like the central Indian belt from Gujarat to Bengal and the northeastern states have a higher prevalence of the sickle gene, with a significant proportion of adivasis residing in these areas. While it’s accurate to state that nearly half of individuals with sickle cell disease may be adivasis, it’s crucial to avoid categorizing sickle cell disease solely as an Adivasi illness. This characterization not only misleads but also poses a risk of fostering stigma and discrimination, as the disease affects both adivasis and non-adivasis.

  • Our diagnostic plan is not in place, and the hope that is invested in the Point of Care Diagnostic (POC) may be premature!!



The diagnosis of sickle cell disease traditionally has been a two-stage process- a screening test done at the point of care, which is cheap and easy to do, and then a confirmatory test like a hemoglobin electrophoresis, which would be done in a referral laboratory. Inefficient logistics management in our public health systems, make stock outs of the cheap reagents for the solubility screening test a common occurrence in many states. The larger problem is the near absence of any confirmatory testing facility for the great majority of those screened positive. In the last few years, we have had the availability of a point of care (POC) test, as a rapid diagnostic kit, that combines the two stages and thus give the treating team a confirmed diagnosis. These POC tests are now increasingly being used in the public health system- both as a screening+ confirmatory test, and as a confirmatory test once the solubility screening test is positive. There is much hope that this will be a game changer in the diagnosis and care of sickle cell disease and if so, that would be welcome. Nevertheless, there is insufficient field experience with these, and numerous field reports[1] raise concerns about the reliability of test results, with published data still unavailable.

  1. An unaddressed need for demystifying treatment of SCD and decentralizing treatment initiation and management with hydroxyurea to  primary care providers

We have had a cheap drug like hydroxyurea for the last 20 years that is recommended for almost everyone with sickle cell disease for a lifetime. This controls the disease in over 90% of patients. But its widespread use is still to happen, with many physician administrators and physicians showing reluctance in its use. Regular uninterrupted supplies of this drug and ensuring that the patient can access this drug at the level of primary healthcare provider would be a game changer. Besides hydroxyurea, medicines for pain crisis, and antibiotics to treat infections and pneumococcal vaccines which all those with the disease must take are also priorities that are essential for good comprehensive care and preventing deaths. This should be a top priority for the entire health system.

Sickle cell disease is now included as a disability about which there is little to no knowledge among health professionals and public. More importantly even the benefits of people with disabilities are not readily available, for example reservation for jobs and for education. The emphasis on enrolment in PM-JAY seems to imply that this is largely a tertiary care intervention, when on the contrary it is a condition which is best managed at the primary care level, except  during a sudden exacerbations. And such exacerbations can be almost completely prevented.

  1. The focus on blood transfusion has to be brought down, even as a measure of the effectiveness of our management plans.

Sickle cell disease patients had traditionally been given a tablet of folic acid and frequent blood transfusions, especially when they had painful crisis. If the disease is managed correctly with hydroxyurea, and analgesics and antibiotics when needed, the proportion of sickle cell disease who require blood transfusions can be brought down to less than 10%. At any rate, blood is a precious therapeutic agent, which should only be used when essential. We can be confident that once we put in place a rational management strategy for all sickle cell disease patients, unwanted and inappropriate blood transfusions would stop.

  1. Importance of a Community Based Approach : for a chronic disease demanding daily treatment and for decades,.

The duration of most chronic diseases like diabetes, epilepsy, hypertension, severe mental disorders, and sickle cell disease is often life long, and thus treatment and regular follow up is needed for many years and decades. To maintain compliance and continuity of therapy for these illnesses is frequently a challenge. For example, it is known that compliance with treatment of hypertension and its adequate control is less than 20% in institution-initiated care. Community based approach to care for chronic diseases is mandatory if we wish to maintain a reasonable continuity of care. This means facilitating formation of peer support groups of patients with this disease, availability of drugs and follow up care close to people’s homes and larger role for community-based health workers. These features are   almost completely missing in the present design of the sickle cell disease program.

The Odisha government has taken the initiative of providing transport support to the tune of INR 500 per month for patients with SCD, which is worth emulating by other states.

[1] Personal communication from several sites in Chhattisgarh with  author, YJ.

  1. Mass screening Vs Selective screening: Need for a public health perspective.

Mass screening should be considered when all those who are already diagnosed, have been put on treatment, and those who are symptomatic for sickle disease get their correct diagnosis. The next priority should be 100% antenatal screening, followed by 100% newborn screening to ensure early detection of the disease.

This suggested prioritization of activities is based on public health logic. Those who ask for treatment, and those who are sick and need treatment should be looked at first. While we also build the system’s capacity to handle an increasing number of patients with SCD, we will be better situated to go for screening activities to identify more who need treatment. Mass screening may also throw up so many sickle cell disease patients for which the system should be prepared to put on treatment. If we are not prepared for that, we would fail the basic ethics of screening activities- to put the screened positive people on treatment. Under five mortality is very high in SCD, but this can be reduced by 90 percent if detected early and put on appropriate care. 100% antenatal screening and newborn screening would help detect SCD early and these children would not be picked up in the mass screening approach.

  1. Urgent need of attention to ethical issues in Mass Genetic Screening linked to Premarital Counseling in Central India


A robust approach necessitates bottom-up methodologies, incorporating community perspectives through high-quality dialogues in central India to gauge their readiness for both mass screening and premarital counseling. This is crucial due to the potential stigmatization associated with SCD and sickle trait positivity.

Mass screening, particularly of older children and adolescents, aims to facilitate premarital counseling, preventing unions between individuals with sickle cell trait positivity. Engaging with communities harboring sickle genes is essential, seeking their collective consent for mass screening, considering the potential stigma, vulnerability to social risks and discrimination associated with sickle trait positivity.

Given the potential challenges, particularly in an unequal world where sickle trait positivity may lead to unjust discrimination especially against women. Such community-based medical interventions, falling in the realm of population genetics require careful consideration and forethought before they are introduced.

Rethinking premarital counseling for preventing marriages between two individuals with sickle trait positivity is essential. Despite attempts in various countries such as Italy, Greece, Saudi Arabia, Iran etc, premarital counseling has not proven effective in preventing such marriages, except for one instance in Cyprus, a small country with a homogeneous religious community.


  1. National Sickle Cell Anaemia Elimination Mission. Accessed February 23, 2024.
  2. GBD 2021 Sickle Cell Disease Collaborators. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021. Lancet Haematol. 2023;10(8):e585-e599. doi:10.1016/S2352-3026(23)00118-7
  3. Operational Guidelines for National Program For Prevention and Management of Sickle Cell Disease-Ministry of Health and Family Welfare, 2023.pdf.
  4. Prime Minister launches National Sickle Cell Anaemia Elimination Mission from Shahdol, Madhya Pradesh. Accessed February 23, 2024.
Acknowledgement: Shalini Singh for peer review and suggestions.

Note : This article, the fourth of our Conversations over Health Policy series  is posted at the website:  The earlier three “conversations” are also available in the website.

Comments (5)

Good article. Congrats. The main to head start the program is a barrier of absent POC diagnostic. The Hb electrophoresis is a slow diagnostic, yet doable on Ayushman Bharat platform. I had developed an early SCEP program for indigenous population in Odisha in 2012 as a part of Tribal Health program, but it was slow to start. Jai Ho,
Prof Dr Subhash Hira
Global Health & Infectious Dz expert
Member, draft committee of Health Mission India.

Great article! I completely agree with all the points. I don’t understand why people working on the ground are not consulted before making such programs and policies. This program seems to be completely disconnected from reality. Another point to note is that a program already exists where sickle cell disease is covered – Anemia Mukt Bharat. Why can’t that program be strengthened?
It is also important to highlight that the Rights of People with Disability Act 2016 includes SCD. But people struggle to get a certificate. How is this program going to address that?

Some points mentioned here also apply to multiple other programs. For example, for TB, the government is pushing for TM Mukt Panchayat but there are not enough testing facilities in place. Similarly for cervical cancer, a recent article in the Hindu was advocating for a separate program for cervical cancer. The existing program is failing, there are not enough skilled people to screen, and if screened positive then not enough facilities to treat.

India needs less of vertical initiatives and more investment in building a strong public health system as that forms the backbone of all these programs.

Thank you for writing this article and initiating this discussion!

Very useful information to guide the disease control organisations.
Appropriate this effort.

Thank you for this detailed piece on addressing sickle cell disease. As you rightly pointed out, there is a lot of hope with Point of Care testing. However, how to operationalize the use of POC tests, is still unclear. If there are clearer guidelines, it will be helpful.

This is a thoughtful and apt description of the potential of comparing, mass screening and point-of-care diagnosis as effective tools for eliminating Sickle Cell Anaemia from India. The cautionary note on the risks associated with a targeted approach during mass screening is particularly insightful, drawing parallels from public health history. The shift to a ‘target-free’ approach in family welfare after 50 years underscores the need for a broader perspective on public health. The question remains: when will we extend this understanding to other health programs and move beyond the limitations of the target-centric approach?

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