Fibrosis File

Matt from Florida recently asked if there are any “specific” exercises that target the conditioning needs unique to those with pulmonary fibrosis (PF).

Mark Mangus answers, The main problem or limita­ tion to exercising with pulmonary fibrosis is shortness of breath. You will have to do extra work to move sufficient air against the stiff lungs of PF, especially with exertion. You have the same conditioning needs with regard to building strength and endurance as those with COPD. So cardiovascular conditioning with longer duration, sustained exercises like biking, walking and elliptical gliding are of advantage. As well, you have the same strengthening needs. Resistance exercises, like weights and similar higher intensity, shorter duration exercises are also of advantage.

Pursed lip breathing is a big help to improve your oxygenation and exert better control over your in­ creased drive to breathe – which is driven by the difficult mechanics of breathing and low blood oxygen levels. Exercises like inspiratory and expiratory muscle resistance training and abdominal breathing (almost always called diaphragmatic breathing) are of no value, nor are they indicated.

Pursed lip breathing allows stale air to leave the lung instead of getting trapped.

image099-2Practice pursed lip breathing when you are not short of breath. This way, when you need to regain control, you will be able to do so without panic!

First try and relax – especially your neck and shoulder muscles.

  • Breathe in for two seconds through your nose, this will distribute the air more evenly.
  • Breathe out for four seconds (or twice as long as you breathe in) through pursed lips – as if you were going to blow out a candle.

Fernando Padilla Makes Transplant History

Last November, Fernando Padilla had PF and on the lung transplant list. The scar tissue in his lungs required that he use high flow oxygen. When a donor was found, Fernando learned he’d make history by being the first man in the U.S. to get donor lungs that were still breathing. The procedure was done at Ronald Regan UCLA Medical. The lungs are put in a high­ tech box where they are revived to a warm, breathing state. A machine circulates blood and oxygen through it, allowing the lungs to be outside of the body for eight hours or possibly more. Today, the 57­year­old Fernando is grateful to be spending time with his family!

image102-1Daughters of PF Event on September 20

This September, during National Pulmonary Fibrosis Awareness Month, the Coalition for Pulmonary Fibrosis (CPF) invites you  to  Washington,  DC  to  attend an exciting meeting that will bring together the Daughters of PF patients past and present for the first time. It will give you the opportunity to participate in an exciting day of in­person motivation, fellowship and training to fuel your efforts to fight PF in your local area.

The Daughters of PF event on September 20 (with a reception the night before) is free for all Daughters members. Also, if you are able, join the CPF on Capitol Hill for meetings with members of Congress and their staffs on September 18–19. Let the CPF know if you are able to attend by emailing [email protected] org to provide your name, address and phone number and which events you will be attending. If you have questions, please call at 1­888­222­8541, ext. 704.

Recent Pulmonary Fibrosis Research Results

  • A paper recently published in the New England Journal of Medicine finds that an important genetic risk factor for PF can be used to identify those who may develop the problem.
  • Based on preliminary research data, people with idiopathic pulmonary fibrosis (IPF) who took medication to treat acid reflux fared better than those who didn’t. The data suggests reflux drugs may provide a positive benefit for those who have symptomatic reflux as well as the less evident form of reflux in which you may not notice