Idiopathic Pulmonary Fibrosis: What Killed Ustad Zakir Hussain

'It's almost like the tissue of the lung has a cancer spreading to it, which is causing scarring and rapidly progresses.'

Dr Lancelot Pinto's first reaction on hearing that Ustad Zakir Hussain had died of idiopathic pulmonary fibrosis was total surprise.

As a world-renowned tabla maestro, who often used his voice, he never exhibited signs of the disease in public, or used oxygen,and that Dr Pinto termed impressive for someone suffering IPF.

Epidemiologist and consultant pulmonologist and respirologist, attached to the P D Hinduja Hospital and Research Centre, Mahim, north central Mumbai, Dr Pinto unveils some lesser-known facts about this "rare disease," about which so little is still known.

"It's almost like the tissue of the lung has a cancer spreading to it, which is causing scarring and rapidly progresses," Dr Lancelot Pinto tells Vaihayasi Pande Daniel/Rediff.com

'Idiopathic' pulmonary fibrosis implies pulmonary fibrosis or thickening/stiffening/scarring of lung tissue without a cause, correct?

That's right. Interstitial lung diseases arethe broad umbrella under which this disease comes; IPF is one of the classifications.

The thought process is when you see somebody with an interstitial lung disease, you do a whole workup in terms of all the standard known causes.

If everything comes up negative -- and there are certain patterns that you see on a scan -- you label the person as having idiopathic pulmonary fibrosis.

If you cannot find a cause, it means the fibrosis was not due to smoking or acidity (GERD), then it becomes idiopathic?

Acidity and smoking are both hypothesised associations. They're not proven beyond a reasonable doubt to be causative. They are associations that do exist. Neither of them has been attributed as a sole cause.

The prevalence of people who smoke among those with IPF is higher than those who don't. But I don't think any animal models have shown a direct link between exposure to cigarette smoke and development of fibrosis.

That's the problem. I don't think they're convinced of calling smoking as a positive agent. It's a contributory agent.

Same with acid reflux -- it has been very controversial. There's a school of thought that believes that there's no connection.

There was a study done, where they tried to do something called a fundoplication (a surgical procedure to treat GERD) to help, and it was a negative study. It didn't prove to benefit.

Then idiopathic pulmonary fibrosis is different from pulmonary fibrosis?

Pulmonary fibrosis is a layperson term for an interstitial lung disease of a certain fibrotic nature, where there is scarring in the lungs. Scarring is the layperson's term for fibrosis.

Fibrosis basically means possibly no active inflammation, but scar tissue is being formed in the lungs. Interstitial lung disease encompasses a whole bunch of diseases.

If you talk about causes, there are, there are connective tissue diseases which have ILDas part of them -- there's Sjögren's syndrome, and something called systemic sclerosis.

Very often rheumatoid arthritis can have ILD associated. If you find those causes, then you would call it a CTD-associated, or connective tissue disease-associated lung fibrosis.

Then you have a bunch of drugs that can cause fibrosis and you would say chemotherapy-associated fibrosis.

You have exposure to pigeons, for example. Hypersensitivity pneumonitis, which is common in India, is again one of the causes of fibrosis.

Once you've searched really hard, not found a cause and there's a certain CT pattern which matches what's seen in IPF, when you look at a scan, you then label it as idiopathic.

If the cause of the pulmonary fibrosisis possibly GERD or smoking, it will also get classified as IPF too?

The problem is we don't consider smoking or GERD as a cause of fibrosis. As of today, we know of it as an association, but we don't think of it as a cause.

So even if someone smokes, their pulmonary fibrosis would be yet labelled idiopathic pulmonary fibrosis?

If they meet the criteria -- their CT has a certain appearance.

If you can't find a cause after you've searched reasonably extensively for it, both clinically, as well as based on the person's history, plus you have done blood tests, plus you would do radiology.

Despite looking for all causes, you don't find it, then you would label it as IPF.

Basically, IPF is a death sentence?

No, not really. Once upon a time it was. We have anti-fibrotic drugs, which slow down the rate of progression.

About 20 years ago, once diagnosed with IPF, the median survival was about two years. That has increased significantly since these drugs have come into play.

There are two drugs already in place and there are a couple of more drugs in the research phase right now.

But it'll get you eventually?

Considering the fact that most people get diagnosed in their late 50s, early 60s, death will theoretically get to you eventually anyway too.

I don't mean to be cavalier about it, but it's one of those chronic diseases, diagnosed later in life, which used to have a very bad prognosis, but it's not so bad.

A lot of us have seen patients now surviving five-six-seven years, with a very slow degree of progression with some of these newer drugs.

When you heard about Mr Hussain passing away from IPD, as a pulmonologist, what did you think?

Oh, he had fibrosis. I wasn't aware at all. That's impressive. He wasn't on oxygen or anything and he performed without oxygen.

He was performing right till November.

That's pretty impressive. Typically, what would happen for a person with idiopathic pulmonary fibrosis, as the fibrosis increases, their oxygen levels would drop and eventually you would get oxygen dependent. You would need oxygen 24 hours a day.

What can also happen is: You might have fibrosis that's not bad enough to need oxygen. But then a sudden viral infection, or something, tips you over, because your lung reserves are so low and it could have catastrophic consequences, just because you're standing so close to the edge.

Maybe his fibrosis wasn't what got to him. Maybe, something acute (secondary cause) tipped him over.

Given the fact you have weak lungs to begin with, then even a small pneumonia can kind of tip you over, which a person with healthy lungs would have possibly tolerated.

That's assuming that he was not needing oxygen. Lots of people who are prescribed oxygen, take their chances, don't use oxygen, because they think it's not going to look good and kind of power through. I don't know the details.

You mentioned there are diseases that hit at a certain age. We are all aware about how to protect ourselves from heart diseases and diabetes. How do you protect yourself from pulmonary fibrosis?

The strong association that we know about is smoking. There's a higher prevalence of smokers among people with IPF than the general population.

We don't put a causative link, but it definitely plays a role in some manner, which is whyone way to protect yourself is to not smoke.

If you're speaking purely about IPF, based on the fact that we don't know what causes it, you can't really tell anybody to do anything to prevent it.

So, the only vague connections are GERD and smoking. For GERD one should, I guess, take the regular precautions -- eat well, exercise well, sleep well and that kind of thing?

Smoking is an established connection. But GERD is controversial. There is a school of thought that believes that there's no connection at all.

Typically, what happens is, if your lungs are stiff and fibrosed, with every breath you produce what we call negative intrathoracic pressure. That could lead to GERD.

It's a chicken and egg phenomenon. We don't know whether the GERD is a consequence or it's a cause.

But as a thumb rule, if you're generally healthy, there's a less chance of getting these diseases?

Diabetes and heart diseases are what we call lifestyle diseases. You wouldn't classify IPF in that category.

They, in fact, they draw parallels between IPF and cancers. Even the drugs which are being used are very similar to the anti-cancer drugs used.

It's almost like the tissue of the lung has a cancer spreading to it, which is causing scarring and rapidly progresses. It's like cancers.

Very often, we don't know why. People who've never smoked, never done anything, get cancers. We don't really know what the cause of IPF is. It's a rare disease.

What are some of the symptoms? How does it sort of get diagnosed initially?

It's most important -- I've been trying to do this as much as possible, left, right and centre -- to make people realise, over and over again, that breathlessness is never a sign of aging.

Most people assume, 'Okay, my joints are painting, I'm getting older, I'm getting stiffer, so a little bit of shortness of breath is also part of aging process'.

But it's not! Being short of breath should never, ever, ever be ignored. If you've not exercised in forever and then you start exercising one fine day, you're going to feel a little short of breath.

But while doing the things that you've been doing all your life, if you suddenly start noticing that you're getting a little short of breath doing them, you should never ignore it.

The second other common way in which IPF would present would be a chronic cough. If you have a cough that goes on and on and on for days together, not two weeks, but you're talking about months, that's something that one should, shouldn't ignore as well.

It typically tends to be a dry, 24/7 irritant kind of a cough, as opposed to other diseases, where you have phlegm and you're trying to bring out the phlegm and therefore you're coughing.

This is just an irritant cough that's very often there almost throughout the day. And that's again, obviously something that shouldn't be ignored.

These are the two commonest ways in which it presents -- a chronic cough or shortness of breath.

For a pulmonologist, it's really very low hanging fruit. You put a stethoscope to the chest and 9 out of 10 times will know that the person has an interstitial lung disease, if they do.

So, the sensitivity of a pulmonologist picking it up tends to be very high because it's very easy to pick up. It's not a complicated diagnosis clinically, even before you do any tests.

We have this air quality problem in India. Would that be a cause too?

If it were that straightforward, you could just compare a country with supremely good air pollution with India, and you would see the prevalence of IPF to be much higher in India, than that country. There would have been a clear signal.

But we've not got that clear signal. India does not seem to have more IPF than the rest of the world, for example.

If we someday figure out what's causing it, it's likely to be some sort of a rare combination of exposures.

It's unlikely to be something very obvious, is my guess, as opposed to something like COPD, for example, which is fairly common.

Feature Presentation: Ashish Narsale/Rediff.com