For tribal communities in Tamil Nadu, thalassaemia is an unending battle

It is a festival day in Nonanganur, a tribal settlement at the foot of the Northern Sheveroy Hills in Papireddipatti taluk in Dharmapuri district, and the world around three-year-old Dhanalakshmi seems joyful. But reality rudely intervenes, when her grandmother whisks her away from the other playing children to swallow a handful of medicines, in preparation for a blood transfusion two days later.

In Dhanalakshmi’s life, as in her parent’s, these monthly blood transfusions are a constant. Without them, Dhanalakshmi could suffer slow development as she grows up, and may even die.

This is the world of hundreds of children in Tamil Nadu like Dhanalakshmi, who suffer the unlucky fate of being born with thalassaemia. While a near total lack of awareness about the condition is increasing the frequency of children born with it, the high levels of poverty in their families often deprives them the chance of winning over it and leading happy lives.

What is thalassaemia?

Thalassaemia is a hereditary condition, passed on from parents to their children, in which certain gene mutations affect the body’s ability to produce haemoglobin. This, in turn, affects the supply of oxygen carried by the blood to the various organs of the body.

Thalassaemia is classified into different types depending on the type of genes affected, and the severity of deficiency or absence of important protein chains. Thus, thalassaemia is classified as alpha- or beta-thalassaemia and as minor or major in severity. Depending on the type and severity of thalassaemia, patients might suffer symptoms ranging from weakness, fatigue and pale skin to heart problems, stunted growth in children, and weak or brittle bones. In the most severe form of alpha-thalassaemia, infants are often stillborn or survive for only a short period after birth.

Dhanalakshmi, the three-year-old child suffering from thalassaemia major.

The inherited disease is passed on to 25 % of the offspring when both parents are carriers of the mutated gene. Awareness plays a major role in the prevalence of thalassaemia which is currently lacking in several populations, say medical experts.

The high cost of living with thalassaemia

While people with less severe forms of thalassaemia do not require frequent treatment, patients suffering from thalassaemia major require monthly blood transfusions to stay healthy. However, the frequent blood transfusions result in an overload of iron, which affects liver and kidney functioning. This means that they are put on additional regimen of drugs to minimise the side effects of the transfusions. The annual cost of treatment per child is Rs 1,25,000 for blood transfusions and medicines.

In a small number of cases, if a proper donor is found, the patient can also undergo a stem cell transplant to treat thalassaemia. Where the transplantation is successful, this frees the patient from the need for repeated blood transfusions. However, the cost of a stem cell transplant can run up to Rs 15-18 lakh, placing it clearly out of reach of poor families that most need this treatment. Even if families can afford the treatment, the difficulties of finding a suitable donor, and the complications in the procedure that arise in some cases, make this an infrequent cure.

A significant health crisis

Thalassaemia represents a significant health crisis for India. According to studies, 10% of the children born with thalassaemia around the world are born in India.  According to a 2018 Union Ministry of Health and Family Welfare study, India has the largest number of children living with thalassaemia major in the world. There are almost 42 million carriers of the beta-thalassemia trait. While the prevalence of carriers averages 3-4% (4%  in TN) across India, a higher frequency has been observed in certain tribal and other communities.

A National Health Mission-funded survey has been underway among tribal adolescents in TN since November 2017. However, data from the survey is unavailable except in Krishnagiri and Nilgiris. In Krishnagiri district, where the survey is being conducted in two tribal dominated blocks, samples of 2,227 children, out of a target of 6,655 children, have been tested, and 99 have been found to be carriers (beta thalassaemia). Dr S Ragavendra Kumar, a district training officer of the Department of Health Services, says that the screening has also been extended to pregnant women in the targeted community. In the Nilgiris districts, where tribal communities comprise 5% of the population, tests on 800 samples have revealed a prevalence rate of carriers of about 10-15%. According to Dr Ramasamy, director of the National Adivasi Welfare Association, which is conducting the survey in Nilgiris and Coimbatore, the above estimate will alter as the survey progresses.  

Sibllings, Rajakumar and Monisha, both of whom are sufferring from thalassemia major, seen with their monther.

When poverty and thalassaemia come together

In the districts of Dharmapuri, Salem and Namakkal, numerous stories have emerged of  poor tribal families struggling to manage the medications and treatments required by children living with thalassaemia.

In Bodhakkadu village in Papiredipatti Taluk in Dharmapuri district, for instance, lives eight-year-old Karunakaran. He was diagnosed with thalassaemia major when he was six months old and had been getting regular blood transfusions at Apollo Hospital in Chennai, till he received a stem cell transplantation one year ago. “Except for a recent episode of giddiness, he is quite normal and is able to go to school regularly,” says his mother. Her woes have not ended, however, as she struggles to provide post-surgery medications for her son. Although she owns two acres of land, water scarcity has forced her to leave the land fallow. And the death of her alcoholic husband three months ago has left her, her two daughters and son with little family income. She depends on MGNREGA work and seasonal work as a coolie on private farms to sustain the family. Amidst this scarcity, providing for Karunakaran’s medications is a herculean task.

In the neighbouring village of Kariathadhanur, Rajakumar, 14, and his sister Monisha, 12, are both living with thalassaemia. The children of Malayali tribal parents, the siblings are both small for their age, a known manifestation of the disease. Their teeth are also stained due to fluoride contamination, as Papireddipatti block struggles with both fluoride contamination and a large number of people with thalassaemia.

While Rajakumar has undergone bone marrow surgery, Monisha goes to Chennai every month for blood transfusion at Apollo, Chennai. Their father Ramu, owns less than one acre of land, which remains fallow due to water scarcity, and has had to borrow huge amounts of money and sell off family possessions to fund the children’s treatment. Besides the costs of Monisha’s monthly trips to Chennai for transfusions, Ramu requires Rs 5000 every month for post operation medicines for Rajakumar. Unfortunately, Rajakumar has developed acute diabetes and hypertension, and has been put on insulin. Together, the medical expenses of the two children add up to an unmanageable amount for the family.

Such stories are frequently heard from the families of tribal children in Sitheri Hills (considered the thalassaemia capital of Tamil Nadu) in the neighbouring Harur taluk, and the Sheveroy hills and Kolli hills in the neighbouring districts of Salem and Namakkal. These children of low-income tribal parents flock to the Voluntary Health Services (VHS) hospital and Apollo Hospital every month for blood transfusions. Any failure to receive the transfusions in time leaves the children with crippling tiredness and can lead to many further complications.

After NGOs and activists took up the issue, the state government established blood transfusion facilities in the Harur taluk hospital itself. However, Dr Ramesh Kannan, the Chief Medical Officer at the hospital, says that the families prefer to go to a bigger hospital in places like Chennai, rather than avail of facilities in Harur.

Karunakaran who has undergone bone marrow treatment seen with his mother.

Parents, however, blame the hospital authorities, claiming the latter demands that the patients’ families should bring in a donor or pay for the blood. More importantly, the hospital procedure at Harur requires children to be admitted a day in advance, and they are discharged a day after the procedure. This means that the parents lose nearly three days of work for each transfusion.

In the hospitals in Chennai, however, the transfusion procedure is a one-day affair. They typically take an overnight train to Chennai, and reach the hospital in the morning and complete the procedure in a day. Another overnight train lets them reach their villages the next morning and return to work that day. In the process, they lose only a day’s wages. Parents in these hill areas have even begun to group together, taking multiple children to Chennai in turn, so that the other parents will not have to be absent from even a day’s work.

In the Salem GH, the medical officer at the blood bank says that about 20 thalassaemia patients from the tribal areas of Belur, Yercaud and Mettur receive regular transfusions without being charged.  However, in most of the other districts with extensive tribal pockets such as Dharmapuri, the hospital deans or resident medical officers have no data about such services being availed.

Stem cell transplant: an elusive treatment

Paediatric Haematologist Dr Revathi Raj, who treats several children with thalassaemia at Apollo Hospital, says that TN is far ahead in terms of treatment facilities and government support for thalassaemia. The stem cell transplant surgery, which costs Rs 15.4 lakh, is reimbursed to the tune of Rs 9 lakh through the Chief Minister Comprehensive Medical Insurance, she points out. Often, about 50% of the remaining amount is arranged through NGOs or independent donors, while the cost of incidental expenses of about Rs 1-2 lakh has to be borne by the family.

However, even where such financial support is available, children with thalassaemia are not always able to avail of the treatment. In Dhanalakshmi’s case, for instance, the cord blood and haematopoietic stem cells of five donors, including that of family members, have not been a match. Hence, her surgery has been put on hold indefinitely. Her father Govindaraj served at the Indian border in the paramilitary force, but was terminated from his job and lost his secure income due to the long leaves he had to take to be with his daughter during the monthly transfusions and tests at the VHS hospital.

According to Dr Revathy, matching stem cell donors are available for only about 50% of patients with thalassaemia. This despite that fact that the Chennai hospitals where she works are networked with the world’s largest stem cell registry, with 25 million donors. “90% of those who undergo bone marrow transplant can lead a normal life. For others regular transfusions and medication support will keep them functional,” she says.

Stigma and lack of awareness

Dr Revathy says that even as efforts are on for the country to achieve a thalassaemia-free status by 2025, the lack of awareness about the condition is a major obstacle.

“If anything is to be said about the condition, it is the lack of awareness. The government should focus attention on campaigns targeting pregnant women and those about to get married. If they are found to be carriers by pre-natal and pre-marital screening they can take precautionary steps to prevent a child being born with thalassaemia major. Counselling of carrier couples is necessary for them to take precautions or avoid giving birth to children with thalassaemia major,” she explains.

Complicating the problem is a sense of stigma that attaches to the condition. “Voluntary screening is still too difficult to carry out due to stigma. It is better to counsel couples to get screened, and if they are found to be carriers, they could be made aware of the risks of bearing a child,” she adds.

Dr. Kuralarasan who works with an NGO called PPDC, which is conducting the national thalassaemia survey in Dharmapuri district, says that he found several cases of stigma during the screening process. “A 14-year-old girl attempted suicide when she was tested as a carrier. According to the survey mandate, family members of children found positive should also be screened. But they become greatly perturbed if a child tests positive, and they resist until they are patiently made to understand that carriers can live without any symptoms and must be careful to avoid marrying a carrier,” he explains.

He adds that one major shortcoming of the survey is that it doesn’t take account of the large-scale migration of tribal populations to towns and cities, where the national screening is not being carried out. Hence, there is the possibility of the frequency of the disease increasing among the urban population, unless a population-wide screening is carried out.

A scheme for financial aid needed

For the families of those already living with thalassaemia, further financial aid from the government is crucial. Some help comes from agencies such as the Association for Women, Agriculture and Rural Development (AWARD). In Dharmapuri District, P Selvaraj, secretary of AWARD, says that after a long period of sustained campaigning, the government has granted free train passes to the families of thalassaemia patients, so that they may avail of treatments at outstation hospitals. However, more government schemes are required to offset the financial strain due to loss of work and the high amount of incidental expenditure, he argues.

For families such as Dhanalakshmi’s, this lack of support can be particularly crippling when other members are also in need of medical aid. Dhanalakshmi’s mother, for instance, suffers from a spinal problem. However, her father Govindaraj says that Rs 5,000 gets deducted from his Chief Minister’s Comprehensive Medical Insurance Scheme for Dhanalakshmi’s blood transfusions and medication. This means that he is left with little insurance security for his wife’s treatment.

“We have been asking the government to give out monthly cash doles, as in the case of HIV patients. But the state government has not taken a decision yet on this. We tried to arrange monthly support for a few families through private donors, but we could not sustain it,” says Selvaraj.

Even as World Thalassaemia Day was observed on May 8, vulnerable families in TN’s tribal areas still struggle with this difficult condition. A more proactive response from the government is the need of the hour.