Patient file: pulmonary fibrosis

What were you told about living with pulmonary fibrosis?
I truly don’t recall being told anything about living with pulmonary fibrosis (PF). It seemed to be assumed that I already knew, or that someone else had told me. I think someone should spend time talking about the diagnosis and its consequences. However, I have also heard some very negative experiences from those who have been told and have been extremely frightened. Okay, it’s not great, but it’s not all doom and gloom. I have a lot of life left in me yet!

How did you cope with the diagnosis?
It was all very vague really. I saw the PF consultant at Wythenshawe Hospital, but I still don’t recall PF being mentioned. I had gone to have some bloods taken and the consultant followed me to ask me to consider a pirfenidone trial. I was given written information to take home and consider. Of course, I googled pirfenidone to find out its effects and side effects and, though I wasn’t looking for it, I saw it was for PF. I also saw the prognosis and life expectancy.

I was shell-shocked and kept it to myself for a while, to make sense of it. I suppose it was a mixture of shock and disbelief and I kept thinking of my children and seven grandchildren. The youngest was only months old and I began calculating how old they’d be when I ... you know, and that was the most upsetting. From my nursing background (I retired around 2016 after frequent absences caused by my RA and difficulty stabilising my treatment), I was very aware of the value of support groups. Therefore, I searched for my local group. That was my best move and where I have gained most of my information. As I learned more, my disbelief turned into anger. I was furious that treatment, not a cure, was limited to idiopathic pulmonary fibrosis (IPF), and even that was restricted to when a patient’s lung function had deteriorated to 80% and was to be stopped when it reached 50%. People like me, with a known cause, such as RA, or asbestosis, or sarcoidosis, or farmer’s Lung, didn’t meet the criteria at all.

I was livid and I think that was what focused me and helped me to cope. No way was I taking it lying down. I had fought to improve things for cardiology patients for much of my later working life so, being a bit bolshy, I was ready for a fight if necessary.

What care have you received?
I was followed up regularly at Wythenshawe Hospital as part of the pirfenidone trial. I am convinced I was on the drug, not the placebo because I was so nauseous. I nearly threw it in more than once, but I persisted. I completed the trial in April 2020, just as the pandemic and lockdowns started to take hold. That was fortunate, as the trial was then discontinued. I was also on prednisolone, which I have tried to reduce, but even now I am taking 10mg daily. The pulmonary support group was also central to my ‘care’, as there was input from so many professionals, including the consultant from Wythenshawe Hospital, research doctors, pharmacists and dietitians. Clinic follow-ups were restricted to telephone reviews.

Is there anything you would change about your treatment?
The obvious thing I would change is for all patients with PF to be prescribed an antifibrotic from diagnosis. Like others in my position, I have reluctantly bought a supply of nintedanib from India which is very uncomfortable for me, but I felt I had no other choice. My respiratory consultant and rheumatologist are aware, and my GP kindly monitors me. Hopefully, I will be prescribed the drug now that NICE [the National Institute for Health and Care Excellence] has seen fit to change its rule. But there are so many issues to address beside drug therapy. Treatment and management of PF is poor for lots of complex reasons.

Although I consider my GP practice to be very good, there was very little awareness of PF and all its different causes. Consequently, diagnosis and referral to specialists is often late or very late. Few people can do what I did and call on former colleagues. Primary care is unable to request CT scans, which are essential for a diagnosis; coding is poor, leading to a lack of accurate data and false perceptions of the scale of the problem. Plus, the level of research is woeful.

How does having pulmonary fibrosis affect your day-to-day life?
I think I have adapted to live my life as normally as possible. I have continued to have my shopping delivered after lockdown and I pace myself. I am looking to get a cleaner. I don’t have the energy I used to have, and bending down is a problem. My husband, children and grandchildren are central to my usual week, and I am determined to continue to take the three-year-old to and from nursery for as long as I can. While I can’t have adventurous days out, I do go swimming regularly, which doesn’t seem to limit me. The children often come with me, and I feel very normal in a pool, which is great, although I don’t really understand my lack of breathlessness. I am also very conscious of avoiding infection, which can be difficult with young children.

What are the key symptoms you experience?
I do get breathless, mainly walking up inclines or after walking only short distances, of maybe 100 yards. I also have spinal stenosis, which causes sciatica and limits my walking. Unfortunately, where I live is anything but flat, so I am limited. I do cough, which I know is troublesome for many, but so far it’s not a big issue for me. Fatigue can be a major issue and sometimes I must just go to bed. Fatigue is also a feature of RA and can be overwhelming.