Fibrosis File

The paperback book, Breathing Should Never Be Hard Work: One Man’s Journey with Id- iopathic Pulmonary Fi- brosis, is now image074-1available at

Robert Davidson was diagnosed with IPF in October 2007 after having difficulties with the fitness test nec- essary to be a soccer referee. He tells his journey with the disease that led to a double lung transplant in January 2010.

Mr. Davidson is also the founder  of  the Canadian Pulmonary Fibrosis Foun- dation ( where you will find many resources as “One Breath at a Time” exercise video for PF along with many other educational and support offerings to help you cope with your daily struggles with pulmonary fibrosis.

Esbriet (pirfenidone) is an approved med- ication used to manage the symptoms of Idiopathic Pulmonary Fibrosis (IPF) and prevent it from getting worse. It works to reduce inflammation and tissue scarring. Analysis from clinical trials reveal that de- creasing the dose of the drug can effectively reduce the number of side effects without affecting the benefits Esbriet brings. Side effects may include nausea, vomiting, loss of appetite; stomach pain, heartburn, upset stomach; diarrhea; headache, dizziness, tired feeling; weight loss; cold symptoms such as stuffy nose, sneezing, sore throat; joint pain; or sleep problems (insomnia). The results were published in the journal BMJ Open Respiratory Research.

 Updated international guidelines to help medical professionals diagnose IPF were recently published in the American Journal of Respiratory and Critical Care Medi- cine. Specialist Dr. Ganesh Raghu noted, “Diagnosing IPF is challenging because these symptoms are non-specific: they occur with all other interstitial lung diseases and with other respiratory problems. Because drugs may slow the progression of IPF, an early and accurate diagnosis is essential for prompt and appropriate treatment for this disease.” The guidelines were outlined in the article, Diagnosis of Idiopathic Pulmonary Fibrosis: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.

The Pulmonary Fibrosis Foundation (PFF) ( has launched its Oxygen Information Line to provide in- formation and resources to help people using supplemental oxygen and their caregivers. The organization set up the line to give advice about which oxygen delivery system to use, how to manage flow settings, and how to ensure safety.

The Oxygen Information Line, 1-844- 825-5733, is available Monday-Friday, 9 am to 5 pm CST. The PFF also provides a Quick Start Guide for Supplemental Oxygen and the Traveling with Supplemental Oxygen Guide, both available for download. A new video on how to use supplemental oxygen can be found on the PFF’s YouTube channel.

An exacerbation of any illness is a sud- den worsening of the disease or what most people think of as a “flare up.” Boehringer Ingelheim published a video available on You Tube to help you determine if you are having an exacerbation of your IPF. You can view it at

 Hope on the Horizon

Pliant Therapeutics announced its poten- tial therapy PLN-74809 was designated an orphan drug by the FDA, an act that helps to advance its development as a possible treatment of IPF. Orphan designation pro- vides companies exploring possible therapies for rare diseases with financial incentives to possibly speed their development. Pliant intends to move PLN-74809 into clinical trials early image077in 2019.

Also granted fast track designation is FibroGen’s investigational compound pam- revlumab. Recent study results showed that, over 48 weeks, intravenous deliv-  ery of 30 mg/kg pamrevlumab halted lung fibrosis (scarring) progression in people with mild-to-moderate IPF, in comparison to placebo. This correlated with improved lung function, as measured by forced vital capacity.

Types of Insterstitial Lung Disease

  • Idiopathic pulmonary fibrosis
  • Cryptogenic organizing pneumonia (COP)
  • Desquamative interstitial pneumonitis
  • Nonspecific interstitial pneumonitis
  • Hypersensitivity pneumonitis
  • Acute interstitial pneumonitis
  • Interstitial pneumonia
  • Sarcoidosis
  • Asbestosis

Common Symptoms of Pulmonary Fibrosis

Shortness of breath is the most com- monly associated symptom of pulmo- nary fibrosis (PF). It begins with short- ness of breath during physical exertion but as the disease progresses, people find they have difficulty breathing when doing simple tasks.

A persistent cough is often present, but because coughing is associated with many different lung diseases, it is not automatically linked to PF. People with PF experience a dry cough.

Fatigue is much more than just feeling tired and cannot usually be remedied by a good night’s sleep.

Weight loss in pulmonary fibrosis tends to occur as the disease progresses.

Shallow or fast breathing is a com- mon symptom which may be due to low oxygen levels in the blood.