Lung transplant at right time only cure for idiopathic pulmonary fibrosis that killed Ustad Zakir Hussain

New Delhi: Idiopathic Pulmonary Fibrosis, the condition that led to the death of tabla maestro Zakir Hussain, is an extremely debilitating disease — a virtual death sentence.

The only definitive cure is lung transplant and that too if done at the right time.

Dr Avdhesh Bansal, a senior consultant in the department of respiratory and critical care at the Indraprastha Apollo Hospitals, said anti-fibrotic medicines and oxygen can delay the progression of the disease, prolonging life by seven-eight years.

“There is no cure for idiopathic pulmonary fibrosis. Lung transplant definitely offers a cure in selected patients but has to be done at the right time, taking into account the severity of the condition, age and other health factors. Transplant also has a life of five to six years,” he said.

In the Indian scenario, patients have been seen to be living for at least 10 to 12 years from the onset of the disease.

“However, every patient’s body and system behaves in a different manner and there is no definite rule,” the doctor said.

As for what causes the disease, Dr Bansal said in almost 50 per cent of the patients the cause is not known which is why the term “idiopathic” is used.

But in the other 50 per cent of cases, patients suffering from Rheumatoid arthritis, Systemic sclerosis or lupus can get Idiopathic Pulmonary Fibrosis (IPF).

In IPF, normal tissue in the lungs gets replaced by fibrotic tissues, making it difficult for oxygen to pass from the lungs to the blood.

“The lung volume reduces. This means it starts contracting, making breathing extremely challenging,” Dr Bansal explained.

There are multiple types of lung fibrosis with the commonest one being IPF, which happens in almost 50 per cent of patients.

“The disease most commonly happens at the age of 50 years. But it can happen at any age. In the Western countries it is said that if you have a definite diagnosis, your life span is about seven to eight years after the diagnosis,” Dr Bansal said.

Dr Saurabh Mittal, assistant professor in the Department Of Pulmonary, Critical Care and Sleep Medicine at AIIMS, Delhi, said besides advancing age, smoking is one of the primary risk factors for IPF.

“Also, if one’s parent or a sibling has IPF then they are automatically at a higher risk of getting the disease. So there is a genetic predisposition to the disease as well,” Dr Mittal said.

Certain occupations in which there is exposure to fumes and dust also increase the risk of pulmonary fibrosis including IPF. Gastroesophageal reflux disease is also predisposed to IPF.

Dr Mittal said environmental pollution, especially PM 2.5, can worsen the symptoms of the disease.

Listing the precautionary measures, Dr Mittal said one should avoid smoking and occupational exposure to dust, fumes or chemicals.

It is a disease which usually worsens with time and responds poorly to treatment, the doctor added.