Patient stories
To be diagnosed with idiopathic pulmonary fibrosis (IPF) is to be given a death sentence. You’re told you only have 3 to 5 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 when 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 the 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.
For more patient stories, please visit the
British Lung Foundation website:
https://www.blf.org.uk/
Interview with Stephen Jones
IPF patient and chair of the charity organisation Action for Pulmonary Fibrosis.
Can you please introduce yourself and your background?
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).
Tell us about your journey to the diagnosis of IPF
It took around five months to be diagnosed with IPF. I first went to my GP with a persistent cough. He changed my blood pressure medication, which seemed to stop my cough for six months but then it came back. I expected my doctor to change my blood pressure medication again, but he referred me to my local hospital for a chest X-ray, then a CT scan, and lastly a bronchoalveolar lavage, at a specialist centre. When the results came back, I had my diagnosis: IPF.
My journey was straight-forward and I was diagnosed fairly quickly, which isn’t always the case. Results from a 2018 survey by Action for Pulmonary Fibrosis of 776 patients in the UK showed that 35 percent were initially diagnosed incorrectly, while a study by EU-IPFF showed that 37 percent of patients across Europe were misdiagnosed. Misdiagnosis is very common among IPF patients because the disease can be mistaken for other respiratory conditions such as pneumonia and asthma. The average time to diagnosis in the UK is seven months, with some patients unfortunately remaining undiagnosed for over two years. For a disease with a survival prognosis of only 3-4 years from diagnosis, this is serious.
You mentioned that one of the initial symptoms of IPF is a cough. What’s the difference between IPF cough and what we know as ‘normal’ coughing?
For many people, IPF cough starts as a tickly, dry and persistent cough. Up to 70 percent of IPF patients experience cough during the disease but not everyone has it from the start. Over time, my cough became productive, with lots of phlegm: sticky phlegm is a particular problem as it’s more difficult to clear than watery phlegm. Coughing becomes very exhausting and impacts other parts of an IPF patient’s life.
Debilitating is the best way to describe IPF cough… it impacted all aspects of my life. At work, I found myself muting calls to allow myself to cough, or avoiding calls altogether. When I worked with clients, I had to pretend that there was nothing wrong, which was isolating. Before IPF, I used to like telling one-liner jokes, but I had to stop because my cough always seemed to get in the way. My family and friends were understanding but I know some patients are embarrassed by their cough and feel they can no longer meet people socially.
How did IPF cough affect your family?
My children were especially worried about me because, to them, a worsening cough was an indication of the developing disease. They were especially concerned when I spoke to them on the phone because I coughed so much. The cough was always there and very unpleasant. It affected my sleep and my family’s as well.
Is there an unmet need when it comes to managing IPF cough?
Yes, most definitely. Currently, there is no treatment available for IPF cough. However, there is some hope now, as we know that there are a few drugs in the pipeline which target IPF cough.
What are the best approaches people can use to relieve IPF cough?
I couldn’t find any medicines to relieve my cough. However, I found that riding my electric bike helped. It forced me to breathe more deeply, which loosened the phlegm and made my body feel more efficient. I found a special place on my cycle route where I used to stop for a while and force myself to cough it all up. It felt good for a few hours until the phlegm built up again.
What resources are currently available for IPF patients?
Action for Pulmonary Fibrosis, which I chair, currently has a network of around 80 support groups, which we aim to increase to 100 over the next two years. At first, I was reluctant to go to a support group. When I finally decided to attend a support group meeting, I was surprised at how much I enjoyed it. I became a born-again support group enthusiast! The benefits of the support group are that everyone understands what you’re talking about, we support each other, share experiences, and find out about the latest treatments and ways of managing symptoms.
My role as Chair of Action for Pulmonary Fibrosis also includes helping colleagues to organise patient information days, across the UK. We’re also in contact with around 4000 patients, directly and indirectly. We aim to provide a space for those diagnosed with pulmonary fibrosis, where they can connect with others and find support.
This narrative has been developed in collaboration with Action for Pulmonary Fibrosis to which Vicore provided a grant to the charity in order to raise awareness of IPF and support research within this field.