To be diagnosed with idiopathic pulmonary fibrosis (IPF) is to be given a death sentence. You’re told you only have 2 to 4 years to live, and there is nothing much doctors can offer you.
I went to my GP with a tickly cough in spring 2016. Eleven months later, after misdiagnoses and long waits for hospital appointments, I was finally diagnosed. By then the other main symptom – breathlessness – had begun to kick in. I was finding it difficult to walk upstairs at home without resting and gentle slopes were becoming a problem. Tests showed that the lung scarring was getting worse.
Over the next two years, I became progressively more and more breathless and suffered an exacerbation. I was admitted to hospital with a chest infection, which ended up cutting my lung function by 10%. I now use supplementary oxygen to walk around my house and whwn I’m outside. I have just been told that I should now be on oxygen 24/7. Recently, I’ve found it difficult to shower or get dressed without help, and I now depend on my wife for almost everything. The future for me is not great but I try to stay positive. Any day is a gift to be enjoyed. However, it is difficult at times and I have had some psychological counselling.
I’m lucky not to have money worries. Sadly, this not the case for many of my friends living with IPF. The government does provide help, but it is often insufficient. When you have IPF many things, such as transport and oxygen when on holiday, cost more. This can be difficult for some people.
Being a member of our local pulmonary fibrosis support group helps me enormously. It’s great to meet and talk to other people living with the disease and to be able to support each other. Our group meets every month and is always full of laughter. It helps a lot.
One thing that bothers me is that I’m often too self-centred. It’s all too easy to be obsessed with one’s own problems and to forget the impact the disease is having on loved ones. My journey is difficult, but so is the journey my wife and children are having to travel. Over time, with increasing breathlessness, I’ve become less able to do things for myself. At the same time my wife, who was my companion and lover, has had to become my carer. As I’ve become more and more dependent on her, she has also lost her independence. And, as I worry about the future, so does she.
Currently there are two antifibrotic medicines, which have been shown to slow the rate of fibrosis for many patients. I take one of these. But more effective drugs are needed to stop this devastating disease in its tracks. These new medicines won’t come in time to help me, but I hope they will benefit future generations.
In future, with a better understanding of genetics and of the basic processes involved in fibrosis, more effective and personalized care tailored to the needs of individual patients, will hopefully be possible.
Patient story kindly provided by Action for Fibrosis through a grant from Vicore Pharma; patient details have been removed to respect confidentiality.
My name is Steve Jones. I am the Chair of Action for Pulmonary Fibrosis UK, a charity founded in 2013 by patients, family members and doctors specialising in pulmonary fibrosis.
I was diagnosed with IPF in 2008 and lived with the disease for eight years, before receiving a single lung transplant in 2016, at the age of 67. I’m very fortunate to have survived to enjoy my 70th birthday.
I’m also on the Executive Board of the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) and on the Council of the European Lung Foundation (ELF)…