“That’s an excellent question!” – You will hear this a lot. Do not get frustrated, it’s not your doctor’s fault if he or she does not have the answers. Through fundraising and support, advances in research will help answer those questions.
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What causes Pulmonary Fibrosis?
What are the symptoms of PF?
What is the prognosis?
Should I maintain an active lifestyle?
Can I travel?
What about alternative medicines?
What can I do to help myself?
Build and maintain a close circle of family and friends. If you are placed on the transplant list their support will be especially important.
How can I help someone who is suffering from PF?
What can I do to protect myself from the smoke and haze of wildfires?
*There is very limited evidence on the use of respirators as an individual-level mitigation approach during wildfire smoke events. The evidence found is equivocal. The effectiveness of respirators as a public health intervention has not been fully evaluated.
The BC Centre for Disease Control has issued a paper on protection from the effects of wildfires which can be accessed through the following link: http://bit.ly/1Zc2J65
This paper has a number of cautions and lots of information and I recommend that all PF patients read it for their own protection. Here two important pieces of information from that paper:
*To provide individual-level respiratory protection during a smoke event, the personal protective equipment must be able to filter very small particles and it must fit, providing a firm seal around the wearer’s face to minimize hazardous agents from entering the respiratory system around the edges of the respirator. Personal protective equipment that provides respiratory protection includes respirators that either filter air or supply fresh, clean air.
* Respirators with HEPA (P100) filters offer the highest protection, but may be less comfortable as they provide more resistance to breathing. P100 respirators may be slightly more expensive than the N95 respirators, for which retail price is between $13 and $25/box of 20 units3
Surgical masks do not provide any protection from these PM and thus should not be used for long periods of exposure to smoke and haze.
Essentially it is recommended that PF patients stay indoors as much as possible and if they must go out a facemask with HEPA (P100) Filters or N95 Filters should be used as protection. PF patients should not spend much time outdoors during periods of haze and smoke from wildfires. Remember that such haze can extend over 500 km from the wildfire source.
* Source BC Centre for Disease Control paper
On May 27th, 2015, CPFF partnered with Ward Health to host an online Question and Answer session on the global burden of pulmonary fibrosis.
An expert panel answered a variety of questions about the challenges of early and accurate diagnosis, current treatment options, clinical trials, exercise, incidence of the disease, and others.
Please take a minute to scan the questions and answers to find what you need, or start from the beginning and go through all the Q & As. The information is current and very informative.
If you have additional questions, please send them to sharon@cpff.ca
Panel of Expert Respondents
Dr. Martin Kolb: Dr. Kolb, Associate Professor of Pathology and Molecular Medicine at McMaster University, and Director of The Firestone Institute for Respiratory Health at St. Joseph’s Healthcare in Hamilton Canada.
Professor David G Taylor: David Taylor is Professor Emeritus of Global Health and Pharmaceutical Policy at UCL London, UK
Read Professor Taylor’s article “Pulmonary Fibrosis – Care for Patients, Benefit all.”
Robert Davidson: Robert Davidson is President and Founder of the Canadian Pulmonary Fibrosis Foundation, a registered charity dedicated to providing education and support for people affected by pulmonary fibrosis.
— A special thank you to Dr. Martin Kolb and Professor David Taylor for taking the time to share their expertise and provide answers to these important questions. Thank you as well to the many individuals from across the globe that took the time to ask a question. Pulmonary fibrosis can be an isolating disease and therefore CPFF is extremely grateful to Ward Health for providing the opportunity to engage with a global community of people affected by PF. It is events such as this online Q&A, programs such as our CPFF patient support program and other awareness activities that help bring people together and to let them know that they are not alone and that together we can drive awareness to this little known, yet fatal disease. Increased awareness leads to more funding for research that will one day lead to a cure.
How can someone with PF exercise when it is difficult to breathe?
I do a tremendous amount of coughing, from the time I get up until I go to bed. Is this common? My Mother and My sister had this and they did not cough like I do.
Is the risk of developing IPF increasing?
Does Pulmonary Fibrosis only affect people when they get older?
Answered by Robert Davidson – – While most patients are between the ages of 50 to 70 at diagnosis there a number of younger patients although theirs tends to be as a result of other factors such as scleraderma.
I have been offered an opportunity to enter a clinical trial. Are there risks? What happens if my symptoms get worse?
Can societies across the world afford better therapies for disorders like Pulmonary Fibrosis?
All the medicines ever discovered currently cost the world between 1 and 2 per cent of our gross annual income. This proportion has been roughly stable for decades, in large part because once new medicines become established products they can be made and distributed as low cost generics. Human labour, by contrast, is always relatively expensive. Few people say they want unchecked competition to drive down the wages of doctors, nurses and other health and social care workers to poverty levels. At the same time it is in patient and wider public interest terms right to try to ensure that enough is paid for new medicines like better treatments for IPF to encourage continuing high risk investment in ongoing innovation.
Crackles or velcro-sounds, are these unique to IPF or even to PF? Are they suggestive of other conditions? If not a sound associated with COPD, then this should be further information for the doctor that the patient doesn't have COPD?
I have been told that I should be referred to an ILD (interstitial Lung Disease) unit to confirm the diagnosis and find out what treatments and trials might be available. Is this necessary?
How does NSIP differ from IPF? Any studies going on for NSIP? Do I need biopsy for definite diagnosis? What is prognosis?
Can new treatments cure Pulmonary Fibrosis?
Answered by Robert Davidson — There are treatments that can help with some of the symptoms of the disease. These would include exercise, prednisone and perhaps NAC. There are also two treatments approved in many countries that slow down the progression of the disease. One is esbriet (pirfenidone) manufactured and distributed by Hoffman La Roche and Ofev (nintedanib) manufactured by Boehringer Ingelheim. Both these drugs are prescribed to patients with mild to moderate forms of IPF. Ofev is not yet approved by Health Canada but that is expected this year.
My GP believes I have IPF and is referring me to a respirologist, what is involved in the full diagnosis? I have heard that a lung biopsy may be done. Is that dangerous as it may cause more scarring?
Why is there so little known about PF?
What is pulmonary fibrosis and what causes it?
People with diagnosed Pulmonary Fibrosis have relatively short life expectancies. Some forms of the condition are associated with other lung disorders such as COPD (chronic bronchitis) and appear to be triggered by smoking or breathing in other known lung irritants. But the most common type, Idiopathic Pulmonary Fibrosis IPF), is of unknown cause, albeit biomedical advances are now throwing light on the fundamental mechanisms underlying its occurrence.
Answered by Robert Davidson – PF is a disease characterized by progressive and irreversible scarring of the lungs. As the lungs become more scarred it becomes more difficult for patients to properly exchange gases (Oxygen/CO and CO2) and the patient suffers from shortage of breath and the oxygen saturation declines affecting other organs.