What is a flare-up of pulmonary fibrosis? 

Some people with pulmonary fibrosis have acute exacerbations or a ‘flare-ups’ where there is an abrupt decline in how the lungs function. This sudden worsening of symptoms can happen with little to no reason.  After a flare-up, some people will recover to a certain degree and others will never be the same or continue to get worse. 

What are the signs of a flare-up? 

• A worsening in cough, shortness of breath, fatigue 
• Fever 
• Phlegm (sputum production) 
• General feeling of being unwell 

How is a flare-up treated? 

There is limited research on proven and effective therapies for a flare-up of pulmonary fibrosis. Many doctors will prescribe anti-inflammatory medication (systemic corticosteroids). Most of the research supports treating the worsening of symptoms and low oxygen in the blood (hypoxemia) with supplemental oxygen. Equipment to help people breathe (mechanical ventilation) may be considered to treat lung (respiratory) failure. Some pulmonary fibrosis therapies (nintedanib and pirfenidnone) may help prevent the development of a flare-up of pulmonary fibrosis. 

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? 

• Very rapid breathing 
• Sucking in skin above the breastbone and between the ribs 
• Being really tired/lethargic (because of the work of breathing) 
• Finding it hard to speak - you can’t finish a sentence 
• Nostrils flaring out 
• Pale, grey, sweating 
• Chest pain 
• Blue lips or fingers 
• Confusion 
• Drowsiness 
• Extreme 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. 

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 website registration is required to access resources)   

End-of-Life Care in Advanced Lung Disease 

For those with chronic lung disease, there often is a decline in health over time.  For some lung diseases, there will be more symptoms and acute exacerbations (a sudden worsening of breathing due to complications such as infection or causes that are not known) that are associated with an increased risk of dying. Death is a natural stage in the cycle of human life. Discussing advance directives, goals of care, palliative care, and hospice care, are important conversations in the end-of-life care of any illness. End-of-life care should be discussed as early as possible. 

What is end of life care? 

End-of-life care is an active, caring approach that treats, comforts, and supports those who are living with or dying from chronic life threatening conditions. End-of-life care is sensitive to personal, cultural and spiritual values, beliefs and practices and includes support from families and friends. It also includes the period of bereavement. 

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 

The goal of palliative care is 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 

Palliative care does not only focus on your physical needs but also your mental, emotional and spiritual well-being. It will not speed up or postpone death. 

* Note: End of Life care and Palliative care definitions are from Canadian Advocacy for Canadians, 2000). 

Common fears of dying: 

• 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 treatment plan. But there may come a time when you are unable to express your treatment wishes to healthcare providers. This is why it is important to plan ahead and make sure your loved ones are aware of your wishes and can act on them if you are unable to. 

• Discuss your wishes with your friends and family or whomever may make decisions on your behalf if you are unable to do so. 
• Write down your wishes and speak with your healthcare providers and financial or legal professionals. Work together to develop an “Advance Care Plan” or “Healthcare (Advance) Directive”. 
• Discuss and identify a substitute decision maker. This person can make decisions on your behalf if you are unable to do so. 
• Review your treatment options with your doctor. 

To learn more visit Advance Care Planning's website for great information on talking to your loved ones.