What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis is a chronic lung disease in which scar tissue (fibrosis) forms in the lungs without a known cause. This scarring makes the lungs stiff, reducing their ability to expand and take in oxygen.

The term "idiopathic" means the exact cause is unknown — though risk factors include age, genetics, smoking, and certain environmental exposures.

IPF typically affects adults over 50 and worsens over time, eventually leading to respiratory failure. While there is currently no cure, early diagnosis and targeted treatment can slow the progression.

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Understanding IPF progression: Is there a staging system?

Unlike cancer, IPF doesn't have a universally accepted staging system. However, doctors often describe its progression in terms of mild, moderate, and severe disease, based on:

• Lung function tests (e.g., forced vital capacity [FVC], diffusing capacity [DLCO])
• Oxygen needs
• Imaging findings
• Physical activity tolerance
• Symptom severity

Let’s look at how these stages commonly unfold.

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Stage 1: Early/mild IPF

• Symptoms: Mild shortness of breath during exertion, dry cough
• Lung function: Slightly reduced (FVC > 70% of predicted)
• Oxygen levels: Normal at rest and during activity
• Daily life: Most activities are still possible
• Treatment goal: Slow progression with antifibrotic medications (e.g., nintedanib or pirfenidone), monitor lung function, stop smoking, and start pulmonary rehab

This stage can last for months or even years, especially with early intervention.

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Stage 2: Moderate IPF

• Symptoms: Noticeable breathlessness with daily tasks, fatigue, more frequent coughing
• Lung function: Moderately impaired (FVC ~50–70%)
• Oxygen levels: May dip during exertion
• Daily life: Climbing stairs, walking long distances becomes harder
• Treatment goal: Optimize therapy, manage comorbidities, introduce supplemental oxygen if needed, and continue rehab

Emotional support becomes more important at this stage, as physical limitations start to affect mental wellbeing and independence.

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Stage 3: Severe IPF

• Symptoms: Shortness of breath even at rest, low energy, persistent cough, potential weight loss
• Lung function: FVC below 50% of predicted
• Oxygen levels: Low at rest, requiring continuous oxygen
• Daily life: Basic tasks like dressing or eating may cause breathlessness
• Treatment goal: Focus shifts to palliative care, managing symptoms, and maintaining comfort

Patients may begin to consider lung transplantation, depending on age, health, and eligibility. However, this option is limited and not available to all.

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Stage 4: End-stage IPF

• Symptoms: Severe shortness of breath at all times, frequent respiratory infections, anxiety due to hypoxia, potential heart strain (pulmonary hypertension)
• Lung function: Critically low
• Oxygen: Required 24/7, often at high flow
• Quality of life: Mobility and independence are significantly reduced
• Treatment goal: Prioritize dignity, emotional support, and advanced care planning

Palliative care and hospice may be introduced to manage physical and emotional pain and support the family through the end-of-life process.

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Emotional impact across all stages

IPF doesn’t just affect the lungs — it affects identity, relationships, and mental health.

Patients may feel:

• Isolated due to physical limitations
• Frustrated by lack of a cure
• Anxious about the future
• Sad or angry about changes in lifestyle

Psychological support, peer groups, and clear communication with care teams are essential to navigating these complex emotions.

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Is there hope after diagnosis?

Yes — while IPF is progressive, its course can vary. Some people live many years with proper support, early antifibrotic treatment, and lifestyle changes.

Others, especially those diagnosed late or with aggressive progression, may decline more rapidly. But even in these cases, supportive care can ease discomfort and improve emotional wellbeing.

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When medicine ends — is there another option?

For individuals facing late-stage IPF with no further treatment options, some explore cryopreservation.

Cryopreservation is not a treatment — but it is a way to preserve the body and brain after legal death in hopes that future technologies may one day offer revival and repair.

Tomorrow.bio offers:

• Cryoprotectant perfusion to minimize tissue damage
• Long-term storage in liquid nitrogen
• A secure, monitored storage facility in Europe

For those looking beyond current limitations, it offers a final act of choice and possibility. You can book a consultation or visit our website to learn more.

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About Tomorrow.bio

At Tomorrow.bio, we are dedicated to advancing the science of cryopreservation with the goal of giving people and pets a second chance at life. As Europe’s leading cryopreservation provider, we focus on rapid, high-quality standby, stabilization, and storage of terminal patients — preserving them until future medical technologies may allow revival and treatment.

Our mission is to make cryopreservation a reliable and accessible option for everyone. We believe that no life — human or animal — should end because current medical capabilities fall short.

📧 Contact us at: hello@tomorrow.bio
🌐 Visit our website: www.tomorrow.bio
🤝 Schedule a consultation: Book a call

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