We are aiming to beat IPF
Dr George Chalmers says progress is being made.
Idiopathic pulmonary fibrosis, otherwise known as IPF, is a chronic and progressive lung disease. It’s uncommon but you couldn’t call it rare, as around 15,000 people in the UK are living with it - and the numbers are rising.
Idiopathic means that the cause of the disease is not known - but its effects are clear: a scarring and stiffening of the lungs leaving people breathless and tends to gradually worsen. Other symptoms can include a persistent dry cough, tiredness, loss of appetite and weight loss.
As the disease progresses, many people will need to have oxygen at home, and portable oxygen to allow them to get out. The thickening and scarring of the lungs results in a decline in lung function, reduced quality of life, and ultimately death.
Around 5,000 people across the UK will die of this disease this year. People with IPF can expect to survive around just 3 years from diagnosis, with only one in 5 making it to over five years. Unfortunately, there's no cure for IPF, other than for the small number of patients who are able to receive a lung transplant.
5 years ago there was little prospect of an effective drug treatment for IPF, but we are starting to see the first signs of light at the end of what has been a very dark tunnel. Although we can’t yet repair the damaged areas of the lung, emerging treatments are showing encouraging effects in slowing the progress of the disease and improving symptoms.
We now have a drug available and being prescribed in the UK, and another which may be available to NHS patients within 12 months. Both these drugs have been shown to slow the rate at which the disease progresses, and there is evidence for improvements in physical function, and more prolonged survival with this new 'anti-fibrotic' treatment.
As with all powerful drugs, there are side-effects and not all patients will benefit from treatment, but they are effective treatments for this serious condition and a big step forward. These are not a cure for IPF though - that remains a challenge for the future. It's heartening to know there are on-going studies of novel drugs that carry with them the hope of treatments which will stop the disease and its debilitating symptoms, and perhaps even reverse the damage to the lungs due to IPF.
“Thank goodness it’s not cancer!” – an understandable emotion, but unfortunately the condition is as serious as many cancers. Seeing my patients reminds me of the daily struggle that IPF brings. Simple tasks become difficult, and then impossible, as the disease limits them from doing normal every day activities.
We are familiar with the call to 'beat cancer' – let’s aim to beat IPF. Maybe not tomorrow, but one day.