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Managing Pulmonary Fibrosis Symptoms—Part 1

Managing Pulmonary Fibrosis Symptoms—Part 1

Pulmonologist Amy Hajari Case, MD discusses best practices for managing PF symptoms in a presentation with the American Thoracic Society.


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Effectively managing pulmonary fibrosis symptoms is critical to maintaining functionality, activities of daily living, and quality of life for every person living with the lung disease.

In an hour-long video presentation hosted by the Pulmonary Fibrosis Foundation (PFF) and the American Thoracic Society (ATS), Amy Hajari Case, MD, FCCP, a PFF Senior Medical Advisor for Education and Awareness, gave a thorough overview of managing symptoms that are common to many kinds of pulmonary fibrosis.* The video was recorded on September 23, 2020 during “Pulmonary Fibrosis Week at the ATS,” a collaborative program between the ATS Public Advisory Roundtable (ATS PAR) and the PFF.

Part 1 outlines the following topics covered in the first half of the video by Dr. Case:

  • A brief explanation of pulmonary fibrosis (PF)
  • What a PF patient experiences
  • Breathlessness
  • Treating breathlessness
  • Cough

(The PFF reminds listeners that the information presented is for educational purposes only and is not a replacement for professional medical advice by a personal physician.)

‘What is pulmonary fibrosis?’ (3:21)

Listeners are first given a brief explanation of PF, often used interchangeably with the term interstitial lung disease (ILD).

  • PF means “progressive scarring of lung tissue.”
  • PF can occur in more than 200 very specific conditions, which could be either:
    • confined to the lungs, 
    • throughout the body (frequently auto-immune in which the immune system negatively impacts the lungs), or 
    • exposure-driven (environment, job-related, adverse effects of medication, or cigarette smoke).
  • PF affects the “spongy” part of the lung designed to absorb oxygen into the bloodstream (versus obstructing the airways, as in COPD).
  • This restrictive lung scarring can lead to “very stiff” lungs, breathing problems, and low oxygen levels.

‘The PF patient experience’ (5:23)

Dr. Case relates common real-world patient experiences:

  • Patients often say they feel and can breathe fine so long as they sit still.
  • They complain their oxygen tank is too heavy to manage.
  • They long to get rid of their cough.
  • They lack energy, leaving them unable to interact with family.

So PF symptoms—breathlessness, fatigue, cough, deconditioning from lack of activity, depression, and anxiety have many impacts on quality of life, which can be compounded by other conditions (comorbidities) and side effects of medications.

Breathlessness (dyspnea) (7:49)

Shortness of breath is “one of the most common symptoms” in PF and the result of impaired oxygen and carbon dioxide exchange due to scar tissue buildup in the space, or interstitium, between the air sacs (where this gas exchange occurs) and spongy lung tissue. The lung’s interstitium is the area that can become inflamed and/or scarred in PF, making it more difficult to breathe as the disease progresses and the lungs become stiffer.

Since most people are typically born with more lung function than needed at rest, some lung function can be lost before it is felt at rest. Once moving and large muscles are engaged that require significant oxygen, and stiffened lungs need to work harder, breathing becomes difficult.

Breathlessness is often a downward spiral in PF. It begins with impaired breathing during moderate exercise that leads many to avoid exercise and become deconditioned. As the disease progresses, people tend to further avoid physical exertion in an attempt to avoid breathlessness..Eventually, they become weakened to the point where basic activities of daily living are difficult and cause shortness of breath. (A detailed chart at 20:39 illustrates this.) 

Many factors can contribute to breathlessness:

  • Low blood oxygen (hypoxia) (which may or may not be present with dyspnea)
  • Stiff lungs
  • Being overweight
  • Deconditioning
  • Comorbidities like high blood pressure in the lungs (pulmonary hypertension) that can further complicate gas exchange

Treating breathlessness (12:20)

Though treating breathlessness is “very challenging,” there are measures that can be taken:

  • Supplemental oxygen can be given to PF patients with low blood oxygen at rest or with exertion to compensate for the effect of scar tissue on oxygen transfer. Oxygen therapy (OT) can decrease and, in some users, prevent shortness of breath. OT should be used during activity to minimize or prevent breathlessness (the lungs cannot “store” oxygen) in order to maintain functionality and avoid deconditioning. (OT is not addictive—we are all addicted to oxygen—and it does not increase the lung’s need for oxygen.) OT may also:
    • reduce risk of pulmonary hypertension,
    • lower increased heart rate from low oxygen,
    • increase activity when limited by low oxygen, and
    • improve sleep during low oxygen.
  • A fan, while simple, can sometimes be effective at relieving shortness of breath in acute situations (for instance, in helping to relieve the stuffiness of wearing a mask during the current pandemic).
  • Pulmonary rehabilitation, a structured group exercise program (usually a set number of sessions), can help stop the downward spiral of breathlessness and deconditioning and improve quality of life with physical activity of major muscles which use a lot of oxygen. At the same time, it can reduce anxiety and fatigue, increase social involvement, and give PF patients more control. “Pulmonary rehab” consists of:
    • aerobic exercise and light resistance training with weights or bands
    • controlled breathing exercises,
    • nutritional support,
    • psychosocial support,
    • disease education and self-management (healthy lifestyle tips, OT, etc.), and
    • assessing individual outcome.
  • Breathlessness-related anxiety, or anxiety-provoked breathlessness, can be lessened with treatment to help stop the cycle.
  • Opiates can sometimes be prescribed to reduce breathlessness, usually in more advanced disease after other measures have been attempted.

Cough (23:12)

While breathlessness is often considered by people with PF as the most bothersome disease symptom, chronic dry cough is a close second.

What causes coughing in PF? In a simplified explanation, nerve endings sensing triggers to coughing extend from the lungs to the brain and brainstem, producing a cough reaction. 

  • In people without PF, common cough triggers could be acid reflux, aspiration (food, liquid, or stomach contents getting into the lungs), postnasal drip, allergies, asthma, smoking, and ACE inhibitor (blood pressure) medications, for instance. People with PF can have these triggers and will be more sensitive to them, having trouble controlling their cough.
  • People with PF also have specific cough triggers
    • PF can cause damaging disease changes such as a pulling on the airways by surrounding scar tissue making the airways wider (called traction bronchiectasis) and fibrosis-related changes to airway mucus.
    • Lung stiffness and scar tissue by themselves can trigger coughing.
    • Additionally, people with PF can have concurrent lung diseases like COPD (chronic obstructive pulmonary disorder) or emphysema that can trigger coughing.

*Pulmonary Fibrosis Foundation. (2020, September 29). Symptom Management in Pulmonary Fibrosis [Video file]. Retrieved from https://www.youtube.com/watch?v=GpJHgsQZdcQ

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