Dharmendra Kumar was 15-years-old when his knees began to stiffen. Fingers, too, lost their flexibility and started to fold inward from the tips. He was in tenth standard then, and suddenly found it impossible to hold a pen, let alone scrawl across a piece of paper and write. He dropped out of school. That was not a contentious decision. The son of a migrant labourer, eldest among five brothers and sisters, he was hoping to study only for a couple more years. With a salary of Rs 6,000, his father, Ashok Singh, was the only earning member in a family of eight. Dharmendra had to start working soon.
Frantic visits to hospitals in Delhi began. Among others, Dharmendra went to the Sanjay Gandhi Memorial Hospital in Sultanpuri, ESI Hospital in Punjabi Bagh and Lokmanya Jai Prakash Narayan Hospital near the Delhi Gate. But no one was able to diagnose the physiological disorder he was suffering from. Since there was no prescription, the family’s only financial consideration was the money required to visit all these hospitals. It was a worry, but not the kind that required a loan and so he kept going. In 2008, the following year, he reached the All India Institute of Medical Sciences (AIIMS) with his father. After a series of tests, the doctors found that he had Lysosomal Storage Disease (LSD).
LSDs, a list of 45 metabolic disorders, are ultra-rare diseases—afflicting one in a hundred thousand people across the world. Along with all the countries of the European Union, the United States, Canada, Mexico, Australia, Japan, Singapore, Taiwan, Thailand, Malaysia, and South Korea have policies for the treatment of rare diseases at the expense of the state. Neighbouring countries, such as Pakistan, Sri Lanka and the Maldives have also begun financially supporting the patients of these diseases. But in India, as Dharmendra would find out, conditions are not so benign.
Each of these disorders is caused by the deficiency of a specific enzyme in the body. Seven out of the 45 LSD disorders are currently curable by Enzyme Replacement Therapy (ERT). Of these seven, treatment for only four is available in South Asia. Mucopolysaccharidosis Type I (MPS-I), which Dharmendra suffers from, is one of them. It is caused by the lack of alpha-L-iduronidase, an enzyme that catalyses the hydration process in the body cells. The commercial equivalent of iduronidase is a drug named aldurazyme, which is sold by the global pharma giant Genzyme, and has been available in India since 2007. But one vial costs Rs 39,500. Dharmendra required it on a weekly basis for his ERT, which, the doctors said, would “last for years.”
He returned home and effectively put the idea of treatment behind him. This was not a contentious decision either. The treatment required a loan that the family could never avail. For the next two years, Kumar did little else than loiter around the one-room house in the labour colony of Sultanpuri. His fingers still folded; his knees stiffened further. Consistent with the symptoms of MPS-I, and perhaps compounded by the conditions of his neighbourhood, he developed a respiratory problem. The body entered a state of arrested development. De-hydrated due to the missing enzyme, his cornea acquired a smoky glaze. All he saw was fading away.
Two years later, in 2010, Dharmendra’s youngest brother, Vishal, who was born in 2008, started to complain of fatigue and rigidity. Well versed with the symptoms of LSD by now, and aware of the fact that it often blights siblings, the family took him straight to AIIMS. Tests followed and the doctors found that Vishal, too, was suffering from MPS-I.
The treatment would cost the same, but, this time, Ashok Singh learned from the doctors, of a Lysosomal Storage Disorders Support Society (LSDSS). LSDSS was formed in 2010 by several parents of children suffering from LSDs across the country, and it helps provide treatment to ailing children through donations and information. Genzyme, for example, treats 120 children in the country under its “Global Compassionate Program.” A few public sector units and the armed forces also have schemes, though mostly for their employees.
The Employees’ State Insurance Corporation (ESIC), one of the earliest social security schemes passed in India, is the state’s medical insurance for those earning Rs 15,000 or less. It is an autonomous body that falls under the Ministry of Labour and Employment’s purview, and is governed by the ESI Act of 1948. The scheme was inaugurated in Kanpur, in 1952, by the then prime minister, Jawaharlal Nehru. Ashok Singh, employed by a textile company, became a beneficiary in 2009.
Through the doctors at AIIMS, he got in touch with Manjit Singh, the Delhi state coordinator for LSDSS. “Most of the people don’t have any idea what to do when their children are inflicted by LSDs,” Manjit Singh told me when I met him on 6 April. “Many times they do not even get diagnosed, but even when they do, they don’t know what to do. Unless they are very rich, they can’t afford the treatment and with rare diseases they find themselves completely clueless. It is quite a hopeless situation.”
Through LSDSS, Ashok Singh found out that the ESIC should be covering the expenses for the treatment of his son. The process of claiming the insurance cover began in mid-2011. A file had to be created, with medical and birth certificates, expense sheets, and recent photographs. Signatures and stamps of doctors and hospitals had to be procured. Ashok Singh did all this at the expense of unpaid leaves. With all requirements fulfilled, the file was submitted to the local ESIC office in Paschim Vihar. From there, the dossier went to the ESIC central office in Panchdeep Bhavan, where it lay stacked with dossiers of insured patients from all over the country. More than a year passed before the authorities ascertained that Vishal Kumar is a medically insured person suffering from MPS-I and required money for his treatment. In November 2013, Ashok Singh received a cheque, credited in the name of AIIMS, for Rs 31,94 lakh for six months of treatment. Vishal got his first ERT on 31 December 2013.
ERTs tend to be more effective when administered from an early age. Younger bodies are more adaptable to the enzyme equivalent, and later, the therapy will not cure mutations caused over time by the lack of it. But there were still more delays. The ESIC-approved medical dispensary—where ESIC patients can avail free medicines—the family were told, had trouble procuring vials of aldurazyme. This was the story every few weeks. What was supposed to be a six-month long therapy lasted for about a year. For Vishal, who was then six years old, there were visible signs of recovery nonetheless: his body started growing again; his height increased, the size of his liver reduced, pain in his joints went away, and his flexibility returned. But by the end of 2014, the money ran out. For the entirety of next year, he did not receive any treatment.
Dharmendra, meanwhile, received no treatment whatsoever. While preparing a file to claim the medical cover along with Vishal, the orthopaedic doctor at the ESI hospital refused to sign off on his papers. The cost of ERT varies with the weight of the patient, and Dharmedra’s body required a lot more aldurazyme than Vishal’s. “Your brother’s treatment alone would cost over 30 lakh rupees, you can only apply for the claim after he gets his,” Dharmendra was told by the doctor. In July 2015, he filed a petition in the Delhi High Court with the help of Ashok Agarwal, a senior lawyer who represents LSD patients deprived of their medical cover by ESIC pro-bono.
After another series of letters, requests and complaints, Vishal’s treatment resumed in January this year. The ERT showed positive effects, but once again, it was interrupted in February. This time, the problem was not the supply of aldurazyme. It was told it was because he had crossed the maximum yearly limit of his medical cover. So far as Ashok Singh knew, there was no such limit. Upon asking, Singh was pointed towards a new clause inserted by the ESIC in a notice titled “Decisions of Medical Services”: “Upper limit on the expenditure for procedures … would be Rs 10 lakh per beneficiary per year.”
The new clause is not only insufficient for the treatments such as ERT for LSDs, a kidney transplant or several surgeries, it is also a violation of five Articles of the Constitution. Agarwal notes in the petition filed in the name of Vishal and his father in February this year: Article 21, protecting life and personal liberty; Article 38, which secures a social order for the promotion of welfare of the people; Article 39, a list of principles of policy to be followed by the State; Article 41, that asks the state, in its “economic capacity,” to make provision for securing the right to work, to education and to public assistance in cases of, among others, sickness and disability; Article 47, asking the State to raise the level of nutrition and to improve public health; all contradict to the said clause. “It defeats the purpose of the ESI Act itself,” Agarwal said when I met him on 5 April.
Towards the end of March, the court directed ESIC and AIIMS to resume Vishal’s treatment and directed AIIMS to also come up with a treatment course for Dharmendra. Following the ruling, the brothers were called to AIIMS by their doctors on 9 April. “They did some tests on Vishal,” Dharmendra told me later. “But I was asked to wait in the waiting room.” He anticipates a repeat of the entire process; saying in a weary tone, “We have to go back in a couple of days again.” The binding clause, for the time being, will remain inked in the ESIC policy.
Atul Dev is a staff writer at The Caravan.