The clinical course of idiopathic pulmonary fibrosis (IPF) may take several forms: slowly progressive, rapidly progressive, or periods of stability interposed with acute respiratory deterioration, which may require hospitalisation for respiratory failure.1
Disease progression can vary substantially from person to person, but in slowly progressive IPF (the classic clinical phenotype of IPF) lung function, as measured by forced vital capacity (FVC), will decline by 0.13-0.21 litres per year.1 This means patients will become increasingly breathless over time.2
What impact does IPF have?
IPF has a debilitating effect on patients and a poor prognosis.2 Given its irreversible nature, a diagnosis of IPF can be difficult for patients and their families to accept.3
“When I was diagnosed with idiopathic pulmonary fibrosis, my wife and I spent entire nights crying. After a while, we understood why – we were grieving the life, at that point, we knew we would never have.
Living with IPF is soul-destroying because you end up relying on your family for everything. Your breathlessness is a constant struggle to the point that even just a flight of stairs or lifting the grocery shopping are impossible tasks. In my case, my wife has become my carer and she does everything I should be doing; this often makes me feel inadequate and embarrassed, especially when people around you don’t know anything about IPF. She always makes sure I wear a scarf when it’s cold, because an infection could send me to the hospital and damage my lung function really badly, to the point that lung transplant might become my only choice.
In the mornings, my cough is so severe that it isn’t just awful for me, but also for who hears me coughing, even people sleeping in the room next to mine. When I cough in public, I can feel the stigma connected to cigarette smoking. I also get lots of looks because of my oxygen. Some people think I smoke 60 or 70 cigarettes a day. Mind you, I have never smoked in my life, so it’s quite ironic.
I work part-time from home, which is ideal for me. I am 58 years old, so I am too young to retire and I can’t quit because I need a salary. I can’t do everything my job would require, and it’s depressing, but the company I work for is very understanding.
I used to be very outgoing and the life and soul of the party but going out and meeting friends has become increasingly difficult; IPF has made me withdrawn and I feel very lonely. I might make plans to go to the pub on a Saturday night, but it is such a big effort that, I just give up and cancel, unless my wife drags me out of the house.
As I heavily rely on oxygen, if I go out, I need to make sure I have enough hours of oxygen. If I run out and I am not at home, I would be in great trouble. This makes everything even more complicated.”
The following websites offer support for people with IPF and other long-term, life-threatening diseases, for carers and for clinicians managing people with these conditions:
- Action for Pulmonary Fibrosis: https://www.actionpulmonaryfibrosis.org/ (including local support groups)
- British Lung Foundation: https://www.blf.org.uk/
- British Thoracic Society: https://www.brit-thoracic.org.uk/
- Hospice UK: www.hospiceuk.org/
- Lung Health Taskforce: https://www.blf.org.uk/taskforce
- NHS Choices: www.nhs.uk/conditions/idiopathic-pulmonary-fibrosis
- NICE guidelines (updated 2017): https://www.nice.org.uk/guidance/cg163
- Primary Care Respiratory Society: https://www.pcrs-uk.org/
- Pulmonary Fibrosis Trust: https://pulmonaryfibrosistrust.org/
1Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;183(4):431-440
2Raghu G et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183: 788–824
3Costabel U. The changing treatment landscape in idiopathic pulmonary fibrosis. Eur Respir Rev 2015;24(135):65-68