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.