Shortage of essential drug hurts patients with Wilson’s Disease

Wilson’s Disease is a genetic disease which causes the copper to build in several organs of the body. The liver is the main organ to store copper and when it is loaded, copper accumulates in the brain and the cornea of the eye. If left untreated, Wilson’s Disease can be fatal. The treatment includes a decoppering agent, D Penicillamine, which helps in the excretion of copper. However, doctors say that the drug is no longer available as manufacturers have stopped producing the drug either due to high price of raw materials or due to government’s decision to slash prices.

The present shortage has raised alarm bells among experts, whose young  patients are in the early stages of treatment. 

Dr Srinivas S, paediatric gastroenterologist, Apollo Children’s Hospital and Kanchi Kamakoti CHILDS Trust Hospital., says, “There are several children at the centres in need of the drug. I have been talking to pharma companies’ representatives and specialists all over India and there seems to be a country-wide shortage of the drug. We don’t know whom to get in touch with for sorting out the issue. The other option is sourcing the drug from other countries, but that is neither reliable nor affordable.” 

Though public hospitals like the Institute of Child Health has the drug available at the moment, doubts prevail about how long the stock will last. Dr D Nirmala, Head of Department, Paediatric Gas troenterology at the hospital, says, “Being a referral centre, we see a number of patients with the disease from across the state and the rest of the Southern region.”

Dr Naresh Shanmugam, paediatric hepatologist, Global Hospitals, says, “There is a huge question mark on the future of the new cases that are undergoing the first line of treatment involving the drug. Zinc is also an integral part of the medication, but D-Penicillamine is the essential drug. A sudden withdrawal of the drug can lead to liver failure.” 

Dr Srinivas says that in extreme cases, the only option left is transplant. “It would be unfortunate, if they have to undergo transplant in the absence of medication,” he says. On the neurological side, there are more than one reasons to worry. Dr Halprashanth DS, neurologist, says, “Abruptly stopping medicines can cause neuro-psychiatric problems. Many develop abnormal movement, tremors, abnormal posturing, get depressed and even become mute.” 

Meanwhile, a source from the Tamil Nadu Chemists and Druggists Association said that the drug is unavailable across pharmacies and that the reasons are yet to be known.

Wilson disease is hereditary. In order to have the disease, a person must have inherited two defective genes, one from each parent. 

Sometimes symptoms of Wilson disease resemble hepatitis. Alternatively, some patients have an enlarged liver and spleen and liver test abnormalities. 

As physical symptoms such as slurred speech, failing voice, drooling, tremors or difficulty in swallowing as psychiatric disorders such as depression, manic behaviour or suicidal impulses.