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Living with IPF: treatment and care strategies

Idiopathic pulmonary fibrosis (IPF) is a progressive and incurable lung disease, but treatment can slow its course, relieve symptoms, and improve quality of life. This article explores available idiopathic pulmonary fibrosis therapy options and supportive care strategies to help patients breathe easier and live better.
4 minutes
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MAY 2 2025
|
Medical
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Fibrosis
Alessia Casali

What is idiopathic pulmonary fibrosis?

IPF is a chronic lung disease where scar tissue (fibrosis) builds up in the lungs without a known cause, making it harder for oxygen to pass into the bloodstream. As scarring worsens, lung function declines, leading to:

  • Shortness of breath
  • Persistent dry cough
  • Fatigue
  • Chest discomfort
  • Unexplained weight loss

IPF typically affects people over 50 and progresses at varying rates, with no known cure — but with proper therapy, patients can maintain a meaningful quality of life.

Goals of IPF therapy

The main goals of therapy are to:

  • Slow disease progression
  • Relieve symptoms
  • Improve lung function and endurance
  • Prevent complications and hospitalizations
  • Support emotional and physical well-being

Since no single therapy is effective for everyone, a personalized approach is essential.

Medication options

Two antifibrotic drugs are currently approved to treat IPF:

1. Pirfenidone (Esbriet)

Reduces scarring and inflammation in the lungs. Clinical trials show it slows lung function decline.

2. Nintedanib (Ofev)

A tyrosine kinase inhibitor that also helps reduce fibrosis. It is taken orally and may cause gastrointestinal side effects.

While these medications don’t cure IPF, they are often the first line of therapy and can significantly extend stable phases of the disease.

Supplemental oxygen therapy

As IPF progresses, patients may experience low blood oxygen levels, especially during physical activity or sleep. Oxygen therapy helps prevent complications like heart strain or fatigue and improves energy levels.

Types include:

  • Portable concentrators
  • Home oxygen systems
  • Nocturnal (nighttime) oxygen support

Your doctor will recommend oxygen based on blood tests and oxygen saturation levels.

Pulmonary rehabilitation

Pulmonary rehab is a key part of IPF management and includes:

  • Supervised exercise training
  • Breathing techniques
  • Education about lung health and medications
  • Emotional support and coping tools

Studies show pulmonary rehab improves endurance, reduces breathlessness, and helps patients feel more in control.

Managing symptoms and complications

IPF may lead to complications such as pulmonary hypertension, respiratory infections, and acute exacerbations (sudden worsening of symptoms). Ongoing care may involve:

  • Regular checkups and imaging
  • Vaccinations (flu, pneumonia, COVID-19)
  • Antibiotics during infections
  • Cough suppressants or corticosteroids (short-term use)
  • Managing GERD or acid reflux, which can worsen symptoms

Good symptom management can extend comfort and reduce hospitalizations.

Lung transplantation

For eligible patients under age 65 with rapidly progressing IPF, lung transplantation is a potential treatment. While not suitable for everyone, it can significantly extend life expectancy and quality of life.

Evaluation includes:

  • Psychological readiness
  • Overall health
  • Social support network
  • Willingness to follow complex post-op care

Lung transplants carry risks and require lifelong immunosuppression, but for some, they offer renewed hope.

Emotional well-being and mental health

Living with IPF often involves anxiety, depression, and grief over changing health and independence. Don’t hesitate to seek mental health support — therapy, medication, and support groups can help you cope more confidently.

Connecting with others through foundations like the Pulmonary Fibrosis Foundation can also offer a strong sense of community and understanding.

Planning for the future

Though treatment can extend quality life, IPF is a terminal disease. Preparing for the future may include:

  • Advance care planning
  • Discussions about end-of-life care or palliative support
  • Choosing someone to act as a healthcare proxy
  • Talking with loved ones about your wishes

These conversations can bring peace of mind and reduce anxiety for both patients and families.

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