What is a flare-up of pulmonary fibrosis (PF) or interstitial lung disease (ILD)? 

A flare-up (also called an acute exacerbation) is a sudden worsening of PF or ILD symptoms. Lung function can decline quickly, sometimes without a clear reason. 

After a flare-up: 

• Some people recover to baseline
• Some people partially recover
• Some people may continue to worsen

What are the signs of a flare-up? 

• Increased shortness of breath 
• Worsening cough 
• More fatigue than usual 
• Fever 
• Increased phlegm (sputum) 
• Loss of appetite or weight 
• A general feeling of being unwell

How is a flare-up treated? 

There is limited research on proven treatments for flare-ups. Doctors may recommend: 

• Anti-inflammatory medications (such as prednisone) 
• Antibiotics if the cause is a bacterial infection  
• Supplemental oxygen to treat low oxygen levels (hypoxemia) 
• Mechanical ventilation in severe cases of respiratory failure 

Some pulmonary fibrosis medications, such as nintedanib and pirfenidone, may help reduce the risk of flare-ups. 

Should I seek medical help for a flare-up? 

If you have a sudden worsening of breathing, cough, sputum production, fever, loss of appetite or a general feeling of being unwell, make an appointment to see your doctor right away. 

What are the warning signs of a breathing emergency? 

These are important signs of a breathing emergency: 

• Very fast breathing 
• Struggling to breathe (skin pulling in between ribs or above the breastbone) 
• Extreme tiredness or lethargy 
• Difficulty speaking - can’t finish full sentences 
• Flaring nostrils 
• Pale, grey, or sweaty skin 
• Chest pain or pressure 
• Blue lips or fingers 
• Confusion or drowsiness 
• Severe shortness of breath 

If you or someone you love experiences a breathing emergency, call 911 and go to the nearest emergency department right away! The person having the breathing emergency should not drive. 

Be prepared for what to do if your symptoms worsen 

• Reduce infection-triggered flare-ups by staying up to date with recommended immunizations/vaccines 

• Discuss creating an action plan with your health care provider 

Learn more by downloading An Action Plan When your Symptoms Get Worse from Living Well with Pulmonary Fibrosis. (Note: Membership to this website is free, but a website registration is required to access resources)   

End-of-Life Care in Advanced Lung Disease 

With chronic lung disease, health may gradually decline over time. Some people experience more symptoms and flare-ups, which can increase the risk of death. 

Death is a natural part of life. Talking early about advance care planning, goals of care, palliative care, and hospice care is important.

What is end of life care? 

End-of-life care is an active, supportive approach for people living with or dying from serious illness. It focuses on: 

• Comfort, focusing on symptoms and pain 
• Support for client and family and friends 
• Respect for personal, cultural, and spiritual values 
• Bereavement (grief) support 

What is palliative care? 

Palliative care is a special kind of healthcare for both the individual (and their family), who is living with a life-threatening illness that is usually at an advanced stage. 

Goals of Palliative Care 

Palliative care aims to provide: 

• comfort 
• dignity 
• the best quality of life for both the person dying and their family 
• relief of pain, shortness of breath, and other symptoms as much as possible 

It supports physical, emotional, mental, and spiritual well-being. It does not speed up or delay death.

Common fears of dying: 

The following concerns are normal and important to talk about with loved ones and healthcare providers:

• Losing independence 
• Becoming a burden 
• Losing control 
• Not being able to breathe or suffocation 
• Not being able to communicate 
• Pain 
• Being easily forgotten 
• Losing self and dignity 

Advance Care Planning 

It’s important to have a voice in your healthcare decisions. However, there may come a time when you cannot speak for yourself.  Advance care planning helps ensure your wishes are known and respected. 

Steps to take: 

• Talk with your loved ones about your wishes 
• Write down your preferences 
• Speak with your healthcare provider 
• Consider legal and financial advice 
• Create an Advance Care Plan or Healthcare Directive 
• Choose a substitute decision-maker who  can speak for you if needed 
• Review treatment options with your doctor 

Planning ahead gives you and your family clarity, confidence, and peace of mind. To learn more, visit Advance Care Planning Canada Website, for helpful information and guidance on your planning.