Idiopathic Pulmonary Fibrosis: Rare Disease Behind Zakir Hussain’s Death

Dr Pavan Yadav, Lead Consultant - Interventional Pulmonology & Lung Transplantation, Aster RV Hospital throws some light on Idiopathic Pulmonary Fibrosis due to which we lost a legend of Classical Music World Shri. Zakir Hussain

Imagine your lungs are like balloons, helping you breathe in oxygen and release carbon dioxide. Now, imagine if these balloons became stiff and couldn’t expand properly. That’s what happens in a condition called Idiopathic Pulmonary Fibrosis (IPF).

IPF is a serious disease that causes scarring (fibrosis) of the lungs. Over time, this scarring makes the lungs stiff and less efficient at supplying oxygen to the body. The word “idiopathic” means we don’t know exactly what causes it, and “fibrosis” refers to the scarring itself.

IPF mostly affects people over 50 years old and is slightly more common in men than women. While it’s rare in young people, anyone can develop it.

The frustrating part about IPF is that doctors often don’t know the exact cause. However, in some cases, it could be linked to:

• Genetics: Mutations in certain genes.

• Environmental factors: Long-term exposure to toxins.

• Injuries to the lung: Repeated small injuries that heal abnormally, causing scarring.

The symptoms of IPF often develop slowly, and people might mistake them for signs of aging or other lung problems:

1. Shortness of Breath: Feeling out of breath, even during simple activities like walking or climbing stairs.

2. Chronic Dry Cough: A persistent cough that doesn’t go away.

3. Fatigue: Feeling extremely tired, even after resting.

4. Chest Discomfort: A feeling of tightness in the chest.

5. Clubbing: The tips of the fingers may become rounder and wider.

Yes, IPF is a serious and progressive disease. Over time, the scarring worsens, and the lungs struggle to deliver enough oxygen to the body. This can lead to complications like respiratory failure, heart problems, or lung infections. Unfortunately, without treatment, IPF can be life-threatening, with most patients living 3-5 years after diagnosis.

Absolutely! Early diagnosis is key to managing IPF. While it cannot prevent the disease, catching it early allows doctors to:

1. Start treatments to slow the progression of lung scarring.

2. Improve the patient’s quality of life through oxygen therapy, exercise, and medications.

3. Plan for advanced care options like lung transplantation.

Unfortunately, there is no permanent cure for IPF. Once scarring occurs in the lungs, it cannot be reversed. However, there are treatments that can slow down the disease, reduce symptoms, and help patients live longer, healthier lives.

• Drugs like pirfenidone and nintedanib can slow lung scarring. These are game-changers in IPF treatment and are widely prescribed today.

For patients with low oxygen levels, supplemental oxygen can help them breathe easier and feel less tired.

This involves supervised exercise programs and education to improve lung function and overall well-being.

For those with progressive disease in advanced stages, a lung transplant can be a life-saving option.

While IPF is a challenging disease, hope is not lost. Early diagnosis, timely treatment, and lifestyle changes can make a big difference. If you or someone you know has unexplained shortness of breath or a persistent cough, don’t ignore it—visit a doctor. Remember, the sooner you act, the better your chances of managing the disease effectively.

Just like a balloon needs to be flexible to work properly, our lungs need to stay healthy to keep us alive and active. Let’s not take them for granted!