Talking Like a Pirate May Be Fun, Cupcakes May Be Delicious, But IPF Is a Killer

What we do know is that IPF can affect adults of all ages, and that it often kills with devastating speed: barely half those diagnosed with IPF are still alive three years later, and just one in five will survive five years.

Last week was World IPF Week. Vying for public attention with such essential diary dates as 'National Cupcake Week', 'World Reflexology Week', and - most prestigious of all - 'International Talk-Like-A-Pirate Day', you may not have noticed.

However, World IPF Week - an awareness week for the little-known lung disease Idiopathic Pulmonary Fibrosis (IPF) that causes a progressive scarring of the lungs making it more and more difficult to breathe - is one we really should have been paying attention to.

After all, talking like a pirate may be fun. Cupcakes may be delicious. But IPF is a killer. And every year, it kills more and more people. Back in the 1960s, IPF deaths in the UK numbered in the hundreds annually. Nowadays it kills over 4,000 people a year - around twice as many as die from diseases like skin cancer, and on a par with conditions such as leukaemia.

And the reason for this dramatic increase in mortality? No one knows. With research into IPF considerably underfunded, the causes of IPF and its striking rise remain a mystery.

Instead, what we do know is that IPF can affect adults of all ages, and that it often kills with devastating speed: barely half those diagnosed with IPF are still alive three years later, and just one in five will survive five years. Indeed, just last week, during World IPF Week, we were contacted by the wife of a very dear BLF supporter who was diagnosed with IPF in 2011. Three years ago, he was the picture of health: young, fit, not a smoker. Last week he lost his life following an unsuccessful lung transplant to overcome his terminal IPF. He was just 39 years old.

So what can we do to tackle this disease, which kills so seemingly indiscriminately, and is rising so inexorably?

Unfortunately, most expert opinion is that the UK is currently worryingly ill-prepared to deal with the growing impact of IPF. However, we do now have a vision of what needs to be done.

Developed with IPF clinicians, patients and carers from around the country, the BLF have created the UK's first IPF 'patient charter', outlining exactly what is required to bring the fight against IPF in the UK into the 21st century.

Some of the solutions outlined in the charter inevitably require investment. We will not find new treatments or a cure for IPF without investing in research. And, as is the case with many serious health problems, IPF patients also need disease-specific care provided by a range of appropriately-trained staff, coordinated through specialist networks and made available from diagnosis through to end-of-life. This costs money to provide.

Yet, many of the improvements we need are far less cost-intensive. Better patient information resources are relatively cheap to provide, and can help us avoid some of the dreadful stories we've heard, such as of individuals simply being told to "Google it" when they asked for more information about their condition.

Making sure the fast-acting nature of the disease is recognised, by prioritising IPF patients appropriately on lung transplant waiting lists, is another relatively simple way in which we can help cut the number IPF deaths.

Overall, the aims of the charter will require effort and investment to achieve, but they are not unrealistic. Indeed, much of what we are calling for is merely for IPF to be fought with a level of investment and effort closer to that given to other conditions that kill similar numbers of people, such as leukaemia. After all, when it's you or a loved one that's affected, what's most important is not the name of the condition you have, but how you're going to fight and survive it.

Sadly, one of the main reasons we are not anywhere near that level of parity is that, unlike leukaemia, very few people have heard of IPF. There isn't the same clamour for UK governments and NHS leaders to fight the growing impact of IPF as there is with other, better-known (although no more deadly) diseases.

This needs to change. IPF has been on the increase for decades: we now desperately need rates of awareness to catch up and overtake. World IPF Week may be over for another year. The fight to help raise awareness and find a cure goes on.

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