Fibrosis File 

Susan Farrell, BS RRT, of Inova Fairfax Hospital in Falls Church, VA, recently spoke about rehabilitation for those with pulmonary fibrosis and other restrictive lung diseases such as sarcoidosis, LAM (Lymphangioleiomyomatosis) or NSIP (Nonspecific Interstitial Pneumonia.) She wants to promote the concept of self management with a reduction of dependency on others.

Susan gives you these recommendations:

  • Have a strong focus on pacing your activities and on energy
  • You want to focus on improving the quality of your
  • Oxygen is your friend, don’t resist using it! You should learn how to use a personal oximeter. Keep O2 saturation 90% or above.
  • Let others know your cough is not
  • There should be personal time and support for caregivers.
  • The interaction and support from other restrictive and fibrosis patients is exceptionally
  • Pulmonary Rehab will give you a sense of control over your circumstances and let you know you still have
  • Continued physical and functional gains are goals to long term participation in pulmonary

Research out of the University of Nottingham in the United Kingdom finds that patients coping with idiopathic pulmonary fibrosis (IPF) are three times as likely to experience severe coronary events, including heart attacks, than those free of the disease. Physicians suggest maybe patients should go on heart prevention treatments right away. Future strategies could include a trial of anticoagulant therapy.

Findings in The American Journal of Human Genetics found mutations in the SFTPA2 gene have been found in families with the inherited version of IPF. About one in 50 people with IPF have an inherited form of the disease, for which there is no cure beyond a lung transplant. The SFTPA2 gene normally produces the surfactant protein A2 in the fluid of the lungs, and it helps protect the organ from invading pathogens. The ultimate goal is to find or develop a medication that can stem the progression of pulmonary fibrosis.

As a public service, the Coalition for Pulmonary Fibrosis regularly provides information on active clinical trials on their web page They highlight industry-sponsored trials, IPFNet/NIH trials, and studies underway at individual medical centers that are actively seeking patients.