Juxtaposition can be an odd thing. This week I gave a diagnosis to a man who had been struggling with his breathing for a while, and who required very detailed tests before I was able to tell him what his condition was. The same day, I heard on the news that Keith Chegwin – a stalwart of children’s television in the late ‘70s and early ‘80s – had died at the age of 60 from the same illness, known as Idiopathic Pulmonary Fibrosis, or IPF.
First described in 1933 its exact cause remains unclear (hence the word ‘idiopathic’, meaning ‘of unknown cause’) and it is a rare condition, affecting around 2 in every 10,000 people although it does appear to be occurring more frequently now than in previous decades. The progressive scarring that typifies it is thought to be due to excessive inflammation of the tiny air sacs in the lungs, possibly as a consequence of an excessive healing response to cell damage. Many possible triggers have been suggested for this such as cigarette smoke – it is more common in smokers and ex-smokers – pollution, viral infections and even stomach acid but any age can be affected and is slightly more common in men.
Symptoms typically are slowly progressive and insidious, with shortness of breath always being the first one to be noticed. This is often put down to lack of fitness or ‘getting older’ but continues to worsen over time and may be associated with a dry cough as well as fatigue and swelling at the base of the nails, known as clubbing. As the condition develops, the heart can come under strain due to rising pressure in the blood vessels of the lungs, leading to the condition heart failure that in turn causes more shortness of breath and fluid retention.
One of the problems with IPF is that it can mimic other lung conditions and so to diagnose it, a high-resolution scan of the chest is usually required, often with a biopsy of lung tissue for confirmation. Unfortunately, once the diagnosis is made there is at present no cure and so treatment aims to suppress symptoms wherever possible but as in the case of Chegwin a premature death may still occur. The aim is usually to try to slow or reduce the impact of inflammation in the lungs, so options range from steroid treatment, oxygen therapy and smoking cessation all the way up to lung transplants, increasingly being viewed as an option in younger patients as this has been shown to improve survival in people for whom it is suitable. Newer treatments such as the drugs pirfenidone and nintedanib have also been shown to slow the progression of IPF but are not a cure.
The progression of IPF is highly variable, with some people being stable for years or even decades, whereas others succumb to the condition very quickly and at the time of diagnosis it is unfortunately almost impossible to say how a particular individual is going to be affected by it in the future. With an average life expectancy of 3 to 5 years after diagnosis however, access to specialist testing is more important than ever and the search for the Holy Grail of a cure continues. Unfortunately for Chegwin, it was not found in his lifetime.