Sickle Cell Anemia - What is All the Buzz About?
The Union Budget of 2023 focused on sickle cell anemia. Why is this disease so important?
The Union Budget was launched today by Nirmala Sitharaman, the finance minister. Having gone through a pandemic that exposed the inadequacies of our health system, the country was looking forward to a budget aimed at strengthening our health system. The establishment of 157 new nursing colleges is welcome news but the buzz has been around the Modi government’s announcement of launching a program to eliminate sickle cell anemia.
What is Sickle Cell Anemia?
Sickle cell anemia is caused due to sickle cell disease, a genetic disorder. A mutation in the hemoglobin reduces the oxygen-carrying capacity of the blood. This mutation also distorts the shape of the red blood cell which can then block blood vessels and lead to serious complications. Because of the mutation, the cells are prone to early breakdown leaving behind fewer healthy red blood cells in the person.
Malaria has been known to be one of mankind’s oldest and deadliest diseases. It is now known that sickle cell anemia forced the human body to evolve resulting in various mutations. One of these mutations is known to cause sickle cell disease.
A person who carries a single copy of the mutated gene is called a carrier of the disease and a person having 2 copies of this mutated gene is known to have sickle cell disease.
How common is Sickle Cell Anemia in India?
Sickle cell disease is the most common genetic disorder worldwide with India having the most number of cases in the southeast Asian region. In India, the state of Madhya Pradesh is estimated to have the highest disease burden with more than 10 lakh people carrying a single or double copy of the gene. This disease is more commonly seen in our tribal populations with a high occurrence of malaria. Based on a nationwide screening conducted by the Ministry of Tribal Affairs and Indian Council of Medical Research (2016-18), 1,13,83,664 persons were screened out of which about 8.75% (9,96,368) tested positive (Trait –9,49,057 and Disease – 47,311).
Can Sickle Cell Anemia be eliminated?
Sickle cell disease is a genetic disorder which means that a person is born with it. It is possible, however, to prevent it through genetic screening of couples from endemic areas during pre-marital counseling or during prenatal checkups. Once a person develops the disease, a drug called Hydroxyurea helps in controlling the disease by preventing the distortion of red blood cells. This further prevents blockage of blood vessels by distorted RBCs. It also increases hemoglobin and thus reduces the need for frequent blood transfusions. This makes controlling the disease more likely as compared to eliminating it.
A newer modality of treatment called gene therapy works by correcting the genetic mutation and thus has the potential to cure the disease. However, this modality of treatment is still under trial. Even if it is made available in India, It is expected to be unaffordable for a majority of Indian patients.
What are the current programs focusing on Sickle Cell Anemia?
Under the Anemia Mukt Bharat Program, screening for hemoglobin disorders like Sickle cell anemia is a strategy to tackle the burden of anemia in the country. However, this program has failed to provide any data on the number of sickle cell disease patients identified through the screening. The status of anemia has also worsened over the past two National Family Health Surveys thus highlighting a significant scope for improvement in this program’s execution.
In 2016, the government released Guidelines for Prevention and Control of Hemoglobinopathies in India. This document meticulously laid down the steps to be taken to control sickle cell anemia - screening, infrastructure development, healthcare worker training, availability of safe blood, access to treatment, the establishment of surveillance system and registry, research and finally improving community understanding of the disease and its prevention. However, no data have been reported over the past years on the steps taken to control sickle cell disease using these guidelines.
The Right of People with Disability Act 2016 recognized a person with sickle cell disease as a person with a disability thus making them eligible to receive the Disability Certificate and the benefits it offers. However, due to stringent criteria, people with sickle cell disease find it extremely difficult to get a disability certificate from the authorities.
The Ministry of Tribal Affairs under Mr. Arjun Munda launched the Sickle Cell Disease Support Corner in 2020 to bridge the gap between patients and health care services in tribal areas. Sickle Cell Corner aims to create a self-registration system for people with Sickle Cell Disease and Sickle Cell Trait. In addition to voluntary registration, this portal provides resources and information about SCD. The dashboard in this portal helps to understand the scale and distribution of SCD. However, as of 1st Feb 2023, the dashboard on the portal’s website was not found.
Is this ‘Mission’ a need of the hour?
Sickle cell disease is indeed a major public health concern and needs urgent attention. However, reinforcing pre-existing strategies would be more beneficial as opposed to introducing of new implementation plans. The primary health system, as seen during the pandemic, is extremely deficient. Strengthening access to care alone will address a majority of the concerns of the people living with the disease. The National Sickle Cell Disease Control Program lays down the following objectives - a)Provision of affordable and accessible care to all SCD patients b) To ensure the quality of care for SCD patients and c) To reduce the prevalence of SCD. The program lists various strategies to reach its goal of eliminating sickle cell disease as a public health problem by 2047.
With India aiming to achieve Universal Health Coverage by 2030, two out of the three objectives should ideally be achieved by 2030. Reducing the prevalence of the disease might not be impossible but is definitely extremely difficult keeping in mind that we currently are still relying on the tribal population census from 2011 with no patient registry to tell us the current extent of the problem. Besides these issues, sickle cell disease being a genetic disease would require population-based screening to reduce its incidence, a strategy that will turn out to be highly expensive in the long run.
With just 2.1% of GDP being allotted to health, does India have the financial liberty to focus on a genetic disorder when people are still dying of tuberculosis? With non-communicable diseases picking up and cancer more commonly diagnosed do we have the infrastructure and competent human resources to adequately manage the growing burden of these diseases? While we still are in the midst of a pandemic, are we prepared to face another wave let alone another pandemic?
Written by Parth Sharma.
This article was originally published in April 2023.