Wednesday, May 11, 2011

IPF - a killer baffling doctors. And the only warning sign is feeling out of breath...

Having been diagnosed with IPF (Idopathic Pulmonary Fibrosis) myself I can emphasize with Lawrence Matz as he goes through the worry and apprehension of knowing that without a lung transplant the future is very bleak. Nine years following my transplant I feel great and don't think about breathing but I can tell you that when you have IPF that's all you think about. Prior to receiving my new lung I couldn't even bend over to tie my shoes or plug a cord into a wall outlet.

Lawrence Matz has idiopathic pulmonary fibrosis (IPF). Even the exertion of shaving is enough to leave him gasping for breath
IPF is responsible for the deaths of around 5,000 people annually, claiming more lives than leukaemia or ovarian cancer


When businessman Lawrence Matz started feeling breathless, he put it down to cutting back on his visits to the gym.

A consultant reassured him that by resuming his fitness regime — three visits a week to the gym and weekends on the golf course — he would soon be back to normal.

But the breathlessness got worse — walking around the house left him panting. So two months later, Lawrence, 57, went to his GP. He diagnosed asthma and gave him an inhaler. It did no good.

Finally, three months later, he was referred to a chest specialist. An X-ray revealed the cause of his problems: Lawrence has idiopathic pulmonary fibrosis (IPF), a condition that leads to thickening and scarring of the lungs, making it harder and harder to breathe.

Until recently, the condition was relatively rare, but a report last week, published in the medical journal Thorax, said the numbers affected by it are increasing.

The amount of new cases diagnosed each year in this country shot up by over a third between 2000 and 2008, to 15,000 sufferers.

IPF is responsible for the deaths of around 5,000 people annually, claiming more lives than leukaemia or ovarian cancer.

The disease typically progresses quickly and more than half of those who develop it die within three years of their diagnosis.

For Lawrence, the disease has brought a brutal change to his life. Just two years ago, he put in long hours at his fashion business. Now even the exertion of shaving is enough to leave him gasping for breath.

‘I have a wonderful wife and family, but my quality of life is not very good and if I was an animal they would put me down,’ he says bleakly. ‘It’s all been such a huge shock.’

What is alarming about the condition is that doctors have no idea what causes it. Each year, thousands of previously healthy individuals like Lawrence are developing this illness for which sadly there is no cure.

Professor Neil Barnes, consultant respiratory physician at Barts And The London NHS Trust, says: ‘It is more common in men than women, but no one knows why and in around 4 per cent of cases it runs in families — so there is a genetic link, but only a very mild one.

‘Smokers are slightly more at risk and have a 30 per cent increased chance of getting it.

‘At one point it was thought that antidepressants might be to blame, but that was discounted. Another theory was it might be related to working in a dusty atmosphere. The only thing we know for sure is that it becomes more common the older you are.’

PF begins with damage to the alveoli — the tiny air sacs within the lungs. Normally, the oxygen that you breathe in passes through the alveoli walls into tiny blood vessels and is then carried away to be used around the body.

However, with IPF, the alveoli become inflamed. As the body tries to heal the damage, the alveoli walls and surrounding lung tissue stiffen and become clogged with scar tissue. As a result, not as much oxygen can be absorbed into the blood. Patients become breathless with minimal exertion. Some may also develop a nagging cough.

The condition can progress slowly in some and rapidly in others, but either way little can be done to help.

‘Sometimes steroids and immunosuppressants are given to try to reduce the inflammation, but the evidence that these work for this condition is poor,’ says Professor Barnes.

‘The normal course for these patients is they end up needing to be on oxygen either all day or when they move around, and some may go on to require a lung transplant. If they don’t have a lung transplant, more than half of those diagnosed die within three years.

‘Around 80 per cent of those who get a lung transplant end up living beyond three years.’

As it is only just becoming more common, not all GPs are used to spotting the condition either. ‘For a GP to diagnose IPF is hard,’ says Dr Kevin Gruffydd-Jones, the Royal College of GPs spokesman on respiratory issues.

‘If a patient comes to see them and complains of shortness of breath, there is so much else it could be — asthma or bronchitis for example. They need to be referred to a specialist and to have an x-ray to make a proper diagnosis.’

Professor Barnes says: ‘It is not uncommon for it to be picked up by chance when someone comes in for investigation into another condition such as a heart problem and then an X-ray shows the tell-tale scarring on the lungs.’

Lawrence, from Finchley, North London, who is married to Gloria and has two grown-up step-children, saw three medics before his condition was diagnosed.

‘In June 2009, I went to a friend’s 50th birthday in Israel and noticed I was getting breathless even when I walked the 20 yards from the pool to my room,’ he says.

‘I hadn’t been going to the gym as much as normal and had been having the odd puff on a cigarette when I was out with friends — and initially put it down to that. I stopped smoking, but things didn’t improve.

'I went to see a heart specialist who told me I was fine and that it was a fitness issue. But I was still huffing and puffing, so in August, I went to my GP who said he thought I had asthma, and gave me an inhaler.

‘That made no difference, so in October, I decided to see a private GP and she sent me for a chest X-ray that day.’

The next day, the doctor broke the news Lawrence had ‘something on his lungs’ and referred him to a private chest specialist.

‘When I went to see the specialist and he said I had idiopathic pulmonary fibrosis, for a second I thought: “Thank God it’s not cancer”,’ says Lawrence.

‘Then he told me there was no cure. When I got home, me and my wife Googled the condition and I soon realised that in a way, what I have is worse than cancer. There is nothing they can do for my condition.’

Lawrence was put on to 15 pills a day — a mixture of steroids and immune suppressants.

‘They couldn’t really give me a prognosis,’ he says. ‘For a year, I was stable — there was no way I could play tennis, but I could get round the golf course and I still went to work. Then in November, I started to go downhill and in January this year my condition nose-dived.

‘Now, any exertion, even talking, can make me run out of breath. I am on an oxygen machine most of the time, even when I sleep. When I go out, I have portable canisters I can take with me.

‘For Gloria, it has been a terrible shock. She’s used to me being so fit and strong and now I have to rely on her to help me all the time.’

NICE is assessing a drug called pirfenidone as a possible treatment. This helps reduce inflammation and scar tissue in the lungs.

Lawrence, meanwhile, is hoping he is suitable for a lung transplant.

‘If they say no, then I may go to the U.S. to see if I can get one there,’ he says. ‘I try not to look ahead. In the early stages, I kept thinking: “Tomorrow I will wake up and feel better.” Now I know that is not going to happen.’

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