by Emily G, Year 11
Pulmonary fibrosis is a devastating and incurable lung disease. It steals breath, mobility, and time, yet it remains largely unknown to the public. Unlike cancer or heart disease, pulmonary fibrosis rarely makes headlines. It is a silent killer - its symptoms subtle at first, its damage irreversible. I know this not just as a writer or researcher, but as a daughter. My father passed away just a few months ago due to pulmonary fibrosis. He was diagnosed far too late, despite having visited doctors repeatedly. By the time we had a name for the illness, the disease had already taken most of what it came to claim.
This article is both a tribute to his strength and a call to action.
We must speak louder about pulmonary fibrosis, because silence, in this case, is deadly.
WHAT IS PULMONARY FIBROSIS?
Pulmonary fibrosis (PF) is a chronic and progressive lung disease in which the tissue deep in the lung becomes fibrotic (scarred) over time. This scarring thickens the walls of the lung, making it increasingly difficult for oxygen to travel through the bloodstrem. The result: patients slowly suffocate - not dramatically, but persistently, day by day, breath by breath.
PF is an umbrella term, often used to describe various types of institial lung disease (ILD). One of the most common forms is Idiopathic Pulmonary Fibrosis (IPF) - "idiopathic" meaning the cause is unknown. But, PF can also be caused by autoimmune diseases, certain medications, long-term exposure to environmental toxins, or radiation therapy.
Symptoms often include:
- Chronic dry cough
- Shortness of breath, especially during activity
- Fatigue
- Weight loss
- Clubbing (widening/rounding) of fingers, toes and nails
These symptoms develop gradually, and are often mistaken for aging, asthma, or more common lung diseases such as COPD. That is part of what makes PF so dangerously elusive.
THE DIAGNOSTIC CRISIS
Early diagnosis of PF is both essential and rare. In my father’s case, it came tragically late. His
breathlessness was dismissed. His cough misattributed. By the time he had a CT scan, irreversible scarring had already set in.
Diagnosing PF typically requires a combination of:
- (high resolution) CT scans
- Lung function tests
- Lung biopsy
- Sometimes bloodwork to rule out autoimmune causes
But many general practitioners in the UK are not trained to suspect or detect PF early. The condition often masquerades as something less serious. The result is a lost window - months, sometimes years, when interventions might have slowed its progress
TREATMENT AND MANAGEMENT: Managing the unmanageable
There is no cure for pulmonary fibrosis. Treatments aim to slow the progression, not reverse it. Two antifibrotic drugs - Pirfenidone and Nintedanib - have been approved in both the UK and the US. They do not stop fibrosis, but they can slow the scarring process. Other treatments include:
- Supplement oxygen therapy
- Pulmonary rehabilitation
- Corticosteroids or immunosuppressants in some cases
- End-of-life care planning
The only definitive intervention is a lung transplant. But this option is only available to a small minority of patients, depending on age, health status, and location.
UK vs USA: A STARK AND UNJUST DIVIDE
Though treatment protocols for PF are similar in the UK and the USA, the outcomes are not. On average, patients in the US live longer after diagnosis than those in the UK. A major reason? Lung transplants.
In the United States, transplants are more accessible, aggressively pursued, and supported by more comprehensive systems. The UK, under the strain of an overstretched NHS, has far fewer transplant opportunities. Even patients who qualify are often deemed too “high-risk” or placed on long waiting lists, with limited organs available.
This disparity is not merely statistical - it’s moral. It speaks to who gets a second chance and who is quietly ushered toward the end without one.
WHY AWARENESS MATTERS
The cruel irony is that awareness could save lives. If more general practitioners knew how to identify PF symptoms early, if more patients knew the risks, and if more funding were available for research, we could dramatically improve life expectancies and quality of life.
Instead, many families - like mine - are left asking "Why my family?" and "Why didn't we know sooner?"
PF is not rare. Over 70,000 people in the UK are living with some form of pulmonay fibrosis, with over 6,000 new cases diagnosed every year. Yet public understanding remains shockingly low.
A PERSONAL REFLECTION
My father was strong, intelligent, and gentle - a man who didn't complain even when he could barely breathe. Watching his world shrink as the disease progressed was one of the most heartbreaking experiences of my life. Stairs became Everest. Sleep became suffocation. His voice, once soft and steady, grew forced and thin.
By the time we fully understood what he had, there was little we could do when the medication he was given wouldn't work, but manage his decline with dignity. I don't want another family to feel that powerlessness.
WHAT MUST CHANGE
To confront pulmonary fibrosis head-on, we need:
- Earlier diagnostic pathways in primary care
- Better GP training to identify PF symptoms sooner
- More lung transplant funding and capacity in the UK
- Public awareness campaigns, like those for cancer and heart disease
- Increased research funding into both treatments and causes
We cannot change the past, but we can reshape the future for others facing this disease.
RESOURCES AND SUPPORT
If you or someone you love is affected by PF, you are not alone. Support is available through:
- Action for Pulmonary Fibrosis (UK)
- British Lung Foundation
- Pulmonary Fibrosis Foundation (USA)
- Local PF support groups and online communities
- Transplant assessment clinics (ask your respiratory consultant)
CONCLUSION
Pulmonary fibrosis may be a silent disease, but we cannot afford to be silent about it. My dad's story is one of thousands. If this article has made you pause, question, or feel, then perhaps it has already done some good.
I write this not just as a grieving daughter, but as someone who believes that awareness is power. Power to act. Power to diagnose earlier. Power to push for better care. And power to hope.
A key reason why my dad continued to fight every single day was hope.
In memory of my father, and in solidarity with every person fighting for breath - you are not invisible.
