Sickle Cell Disease in India: Addressing Stigma and Healthcare Needs

What is Sickle Cell Disease (SCD)?

Sickle cell disease is a genetic blood disorder caused by a mutation in the hemoglobin gene. This mutation leads to the formation of rigid red blood cells that resemble a sickle, instead of being round and flexible. These abnormal cells can obstruct blood flow, resulting in pain, infections, organ damage, and fatigue.

Why is SCD a Concern in India?

India has the second-highest number of individuals affected by SCD, following sub-Saharan Africa. The disease is particularly prevalent in tribal communities, where access to healthcare is often limited. Misconceptions, low awareness, and social stigma exacerbate the challenges faced by these populations.

What is Social Stigma in the Context of SCD?

Social stigma refers to the negative attitudes and discrimination directed at individuals due to their illness. Common examples include:

• A girl with SCD facing rejection in marriage proposals because of her health status.
• Children with the disease being denied admission to schools due to myths that SCD is contagious.
• Workers concealing their diagnosis from employers for fear of job loss.

Challenges with Existing Global Tools to Measure Stigma

Before the Indian Council of Medical Research (ICMR) developed its own tool, existing stigma measurement scales from the USA, Ghana, and the UK did not align well with Indian contexts. Issues included:

• In India, family honor and marriage prospects are significant concerns that global tools overlooked.
• Language barriers and local dialects rendered foreign questionnaires unusable in many tribal areas.
• Indian patients often encounter stigma from traditional healers or caste-based discrimination, which is absent in Western contexts.

What is the ICMR-SCD Stigma Scale for India (ISSSI)?

The ISSSI is the first Indian-specific tool aimed at measuring various forms of stigma faced by SCD patients. It is also the fourth such tool globally, covering multiple dimensions such as:

• Familial stigma: A parent being blamed by relatives for passing on "bad blood."
• Reproductive stigma: Women with SCD being pressured to avoid pregnancy due to fears of complications or societal shame.
• Social disclosure: Young people not revealing their SCD status in friendships or relationships.
• Healthcare interaction: Patients experiencing rude behavior from medical staff or refusal of blood transfusions.
• Illness burden: Individuals being perceived as lazy or unreliable due to frequent hospital visits or fatigue.

Validation of the ISSSI

The ISSSI was developed using field data from six SCD-endemic districts:

• Andhra Pradesh (Alluri Seetharama Raju)
• Madhya Pradesh (Anuppur)
• Gujarat (Chhotaudepur)
• Odisha (Kandhamal)
• Karnataka (Mysuru)
• Assam (Udalguri)

These locations were chosen for their cultural, tribal, and geographical diversity. The scale was tested among patients and caregivers through interviews and observations.

Significance of the Lancet Report

The Lancet journal has reviewed and validated the ISSSI, calling it a robust tool for understanding stigma in Indian contexts. This international acknowledgment provides Indian researchers with a reliable, evidence-based framework for studies and policy formulation.

How Will ISSSI Benefit SCD Patients in India?

The ISSSI aims to:

• Offer data-driven insights into how stigma impacts patients' lives.
• Assist in designing more effective awareness campaigns.
• Guide healthcare providers in treating patients with greater respect.
• Empower tribal health initiatives to tackle real issues.
• Enhance the design of training modules for healthcare workers.

Government Actions to Combat SCD

The National Sickle Cell Anaemia Elimination Mission was launched in 2023, featuring:

• Screening 7 crore individuals by 2030.
• Mapping high-risk districts and creating registries.
• Providing genetic counseling for at-risk couples.
• Strengthening primary healthcare in tribal areas.
• Integrating services under the National Health Mission and TRIFED.

Remaining Challenges Despite New Initiatives

• A shortage of trained professionals in rural areas.
• Resistance to testing due to fear of stigma.
• Poor supply chains for essential medications.
• Irregular follow-ups in remote regions.
• A need for targeted Information, Education, and Communication (IEC) campaigns.

Community-Level Solutions

• Utilizing ASHA workers and local NGOs to increase awareness.
• Engaging tribal leaders to openly discuss SCD.
• Providing support groups in schools and workplaces.
• Promoting early marriage counseling with SCD education.
• Implementing mobile clinics for screening and consistent follow-up in tribal hamlets.