In the small, single-room house of the Vadakottathara Iruala settlement in Attappadi, seven-year-old Amritalakshmi’s smiling photograph now rests where she once slept beside her siblings.

Her mother, Kaliyamma, recalls the quiet struggles her daughter endured. “When stomachache struck her, she would bear it silently as much as she could. When she became too sick, her nose would bleed, and she would miss school for five or six days. If she were at school when the pain hit, the teachers would send someone to call us from work to bring her home. The night before she died, she lay down as usual, placing her leg on my husband’s belly, and said, ‘Father, I will become a doctor. No one else should suffer like me.’

“She had fed her younger siblings before going to sleep. Later, she asked for water—I told her to get it herself, and she must have. She usually woke up late in the morning, but that day she didn’t. Her siblings got ready, but she was still in bed. When I tapped her back and called her, her neck fell back… and I realised my daughter was already gone,” her mother recalls.

This little girl, who had long-held dreams, tragically became another victim of the net of death cast by sickle cell disease. The drawings and name she had etched on the wall of her house are beginning to fade. She had even shown her talent as an actress in the short film titled ‘Sivakami.’

Vellankeeri and Kaliyamma have seven children, three of whom, including the late Amritalakshmi, suffer from sickle cell disease, while three others are carriers. Only their middle daughter is free from the condition. Both Vellankeeri and Kaliyamma are carriers of sickle cell disease. In Attappadi, the death of a child like Amritalakshmi is tragically routine, seen as just another occurrence that takes place every few months.

Sickle cell disease has a history of one and a quarter centuries. The highest incidence of sickle cell disease in the world is reported in the sub-Saharan region of Africa. Sickle cell patients and carriers are also among those who have migrated to the US from Africa and to countries such as Greece, Turkey, Saudi Arabia, and Cyprus. As per the statistics, 29 million people suffer from this disease. Among all the African and Afro-American children born in a year, one in 13 are sickle cell disease carriers, and one in every 365 is born with the disease, according to a study conducted by the National Heart, Lung, and Blood Institute based in America.

India now has the second-highest number of sickle-cell anaemia patients in the world. Sickle cell disease was first reported in India in 1952 among tribal people in the northern region of the Nilgiri Hills in Tamil Nadu. The main reason for sickle cell disease is a genetic mutation that originally happened in human bodies as a defence against malaria. At that time, people living in plantations and forest regions were often killed by malaria. Over time, effective vaccines and medicines were developed, making malaria less deadly, but sickle cell disease continued to be transmitted genetically. Thus, the genetic mutation that was once a boon has become a bane today.

The highest incidence of sickle cell disease in the country is reported in Andhra Pradesh, Odisha, Maharashtra, Madhya Pradesh, Tamil Nadu, Rajasthan, Gujarat, Karnataka, and Kerala. The surveys in the health sector reveal that, while the disease existed only among the tribal sections in earlier days, now it is being spread to other sections as well. India has one of the largest tribal populations in the world. 8.6% of the total population of India is tribal people. It means 67.8 million people. Statistics say that 40 per cent of all sickle cell patients are children. These children, born into pain and suffering, are the future hope of a major section of society who are marginalised. Though it is a fatal threat that affects so many people, the disease has not received the urgent attention it deserves from the authorities.

Sickle cell disease in Kerala

Sickle cell disease in Kerala is mainly reported among the tribal people in Palakkad, Wayanad, and Malappuram districts. As per the existing official statistics, among the 45 patients in Nilambur, Malappuram district, five are children. Among 224 patients in Attappadi, 80 are children. In the last screening conducted in Wayanad district, 1234 persons were found to be affected by the disease. Among them, 471 were children. 43 per cent of those who have the disease suffer from severe pain and other complications(SICKLECELL CRISIS) of the disease.

What is sickle cell disease?

Sickle cell disease is caused by a strange disfiguration that happens to red blood cells owing to genetic reasons. The blood cells that turn into the shape of a sickle gradually lose their ability to carry oxygen. They create blocks in veins, thus pushing the patients to severe pain and other physical troubles. It may also cause the sudden death of the patients. When small veins are blocked, internal organs would not receive sufficient oxygen. It may lead to a stroke or heart attack. It can also cause damage to the brain, eyes, kidneys, liver and lungs.

Normal blood cells have a lifespan of 120 days. But in sickle cell patients, blood cells live for only 20 to 60 days. As the body cannot produce new blood cells at the same speed as the cells being damaged, anaemia is common among sickle cell patients. The intensity and symptoms of the disease differ from person to person. Major symptoms include severe pain in limbs, chest, headache, difficulty in breathing, anaemia, tiredness, constant fever, hepatitis and inflammation in the gall bladder.

Diseases in children

The disease is transmitted to children from parents who are either sickle cell patients or carriers. These children are constantly affected by bacterial infections. As veins are constantly blocked, the growth of body parts and organs is arrested. In Kerala, the condition of the patients worsens during the monsoon season. It’s their bodies’ inability to resist cold and the lack of facilities that lead to worsening the condition.

Eradication or treatment?

While presenting the Union Budget 2023, Finance Minister Nirmala Sitharaman announced a national commitment: sickle cell disease will be completely eradicated in the country by 2047 (National Sickle Cell Elimination Mission).

As of August 2024, 4,11,98,030 crore people have undergone screening to detect sickle cell disease. Among them, 11,27,307 people were found to be carriers of the disease, and 1.61 lakh people are sickle cell patients. India has the second-highest number of children currently suffering from sickle cell disease.

The number of babies born each year with the gene for sickle cell disease, including both those with the disease and carriers, ranges from 50,000 to 60,000. The constitution states that free and universal education is the fundamental right of children. However, the question remains: who is responsible for failing to protect their right to a healthy life in this country?

 

Read tomorrow: The scathing blow of sickle cell disease on Attappadi.