A message from CPFF Board Chair Kirk Morrison
This has been another very challenging year as we dealt with the continued anxiety and restrictions of the pandemic, especially for those of our vulnerable PF Community. Emotionally it has taken its toll, with isolation from friends and family, restricted access to health care and daily activities, and in some cases, the loss of loved ones. I want to assure you that CPFF is here to support you and to offer hope to the members of our community, even in these stressful times.
Throughout 2021, we continued to support one another and accomplished so much. We were able to fund our 22 support groups to continue to meet virtually, a lifeline for so many. We were also able to hire Roberto Zapata as an additional resource for the groups.
Just as scientists and researchers were able to deliver effective vaccines to help us deal with COVID-19, CPFF invested $220,000 to fund two, Robert Davidson fellowships and two, PF research projects, to advance our knowledge of ILDs and train more specialists.
We commissioned a national patient and caregiver survey and with the results produced the Breathless for Change report, which identified the needs and priorities for our community. We successfully advocated for the approval of nintedanib for non-IPF patients.
And once again, through the Hope Breathes Here campaign, we promoted September as Pulmonary Fibrosis Awareness Month. We produced 17 educational webinars and videos during the month and held our national Walk for PF, in partnership with the Clarke family, during which the PF community collectively walked over 9,944 km to raise awareness and funds to support research.
Thanks to Sharon Lee, our Executive Director, her staff, as well as our Board of Directors and our Medical Advisory Board for all of their work during 2021. We were fortunate to welcome to our Board Dr. Holly Smith, Verity Pringle and Ray Protti, all from B.C., and Mark Ashcroft of Ontario. Taipan Chowdhury of Alberta and Stephen Heather from B.C. joined the organization as our first Volunteer Committee (of the Board) members.
Finally, thanks to all of you in our PF community, including the more than 5,000 of you who subscribe to the newsletter, for your support and engagement throughout the year. Continue to take care of yourselves, your loved ones and your community, and all the best to you and your families over the holidays. I look forward to working with you in 2022!
Another holiday season is here, and we’re still dealing with COVID-19 and its nasty variants. Restrictions have relaxed in many places this year because fortunately many people are now double-vaccinated. Even if you are vaccinated, it is still best to adhere to public health guidelines and wash your hands often, avoid large crowds, stay away from others who are sick and wear a mask in public, indoor spaces. In regards to holiday gatherings, check if all guests will be vaccinated, so you can make an informed decision about attending.
CPFF recommends that those living with PF, as well as their carers and family members, get a third COVID-19 vaccine – or booster shot – as soon as you are eligible.
Along with a COVID-19 booster, don’t forget to get your flu shot and keep up-to-date with your pneumonia vaccines as well.
Since vaccines are administered by province, vaccine programs vary across the country. Check out our COVID-19 Helpful Links page for links to vaccine information in your province or territory.
A second company, Jamp Pharma, has begun manufacturing and distributing generic pirfenidone, in 267 mg. capsules and in 267 mg. and 801 mg. tablets. It is now available in most provinces, and will be available in all three forms across the country by the end of January 2022. You can read about Jamp’s patient support program by downloading this document with Jamp’s patient contact information.
In September, Sandoz Canada was the first company to manufacture and distribute generic pirfenidone, replacing the brand product Esbriet, when its patent expired. Sandoz also has a patient support program, including compassionate care. You can read about it in our September newsletter.
“There have some been growing pains around supply with generic pirfenidone,” reports Shane Shapera, Director of the Interstitial Lung Diseases Program at the Toronto General Hospital. Sandoz did not anticipate the volume of drug that would be required and supply is currently limited. They are able to provide the drug for everyone who is currently on their version of generic pirfenidone. However, due to supply shortages, they are not accepting any new patients into their program until they can increase production, which they estimate will take until about February 2022.
Jamp is not reporting any supply shortages. So, those who are just being started on pirfenidone between now and February 2022, should have their respirologist prescribe it from Jamp. For patients who are about to start on pirfenidone for the first time, if they are told that there is inadequate supply, then they should ask their respirologist to move the prescription from Sandoz to Jamp. Alternatively, they can wait until February to start on drug if they would like to stay with Sandoz.
Another pharmaceutical company is expected to release a third version of generic pirfenidone in January, which should help ensure a robust supply going forward.
If you are having any issues getting your pirfenidone, please contact your prescribing respirologist and have them follow-up with Jamp or Sandoz to ensure you get your medication.
If you are interested in learning more about generic drugs in general, and generic anti-fibrotics in particular, read about a recent webinar by Dr. Charlene Fell in our October newsletter or watch her video.
Latest webinar explains how new x-ray technology could detect PF sooner
Imagine a chest x-ray that provides three images, instead of just the usual one, using the same level of radiation, as well as similar time and staff resources, as a standard chest x-ray. One image is the same as the current image generated; the second shows only the bones; and the third shows only soft tissue. These multiple images should result in earlier detection of many lung conditions, including pulmonary fibrosis.
In a November webinar, presenters Karim S Karim, Founder and Chief Technology Officer of KA Imaging and Dr. Patrik Rogalla of the University Health Network (UHN) explain how this new imaging, called dual-energy subtraction (DES) X-ray or single exposure dual-energy X-ray, improves on current imaging technology.
Dual-energy x-ray technology was actually invented in the 1970s and uses physics to remove bones or soft-tissue from an image. By focusing on different types of energy inside the X-ray beam, it can differentiate these structures providing a clearer view.
“Unfortunately, early versions had plenty of drawbacks. It was not portable, it could take frontal images, but not side ones. It was expensive and it didn’t always produce good images,” says Karim. “Today’s version has overcome all of these issues and enables a more accurate diagnosis than a standard x-ray.”
Throughout the presentation, Dr. Rogalla shows a number of examples of the three images and talks about how the additional two images can clarify or rule out possibilities, that the image reader, a radiologist or physician, may have from only a single image.
“Dual-image x-ray is not a replacement for high-resolution CT (HRCT) scans, which is the standard of care for a pulmonary fibrosis diagnosis,” says Dr. Rogalla, but along with other symptoms such as cough and breathlessness, the three images may better indicate that PF is a possibility and that a follow-up CT scan should be ordered.”
A study is currently underway at Princess Margaret Hospital in Toronto to investigate the “Diagnostic value of dual-energy chest X-ray in immunocompromised patients to rule out pneumonia.” Initial results (with 33 participants) indicate that the additional bone and soft-tissue images improve the diagnostic accuracy. In addition, agreement among several radiologists on a diagnosis is higher with dual-energy chest x-ray than with the current single x-ray image.
“This indicates to us that there is a greater confidence in the diagnosis with this x-ray technology, than the current method,” says Dr. Rogalla.
Overall, single exposure dual-energy x-ray enables new opportunities for early detection of pulmonary disease. According to Dr. Rogalla, it offers more confident, accurate image interpretation; saves time and benefits patients in radiology, emergency and critical care departments; requires no additional procedure or dose – the same as chest X-ray; and leverages existing X-ray equipment and clinical techniques.
We know that one of the biggest challenges of pulmonary fibrosis is obtaining a timely diagnosis. Most patients wait close to a year, many wait much longer, before receiving a diagnosis. During this time, without treatment, patients’ lungs continue to deteriorate.
A chest x-ray that can lead to further investigation and a CT scan sooner, will be a great benefit to those eventually diagnosed with PF by giving them access to earlier treatment.
For those already diagnosed with PF, the new dual-energy x-ray may help detect pneumonia or other lung conditions during an acute exacerbation.
While studies of the new x-ray have not been done on PF specifically, they may well be undertaken in the near future and researchers will be looking to recruit study participants. CPFF will keep you posted on future investigations.
You can watch the complete video of this presentation by visiting www.hopebreatheshere.com, under education forums. Other videos on a variety of topics relevant to those living with PF and their families can be found there as well.
Newly diagnosed? This video is for you
Navigating a new diagnosis of pulmonary fibrosis with Dr. Mathew Binnie, answers many of the questions you likely have as someone who has just been diagnosed with PF, or as a family member. Questions like: What is pulmonary fibrosis and how did I get it? What does this diagnosis mean for me and my family and how will this disease affect me? This video will give you some answers. And even if you’ve been living with disease for some time, you may find some new and useful information.
Dr. Binnie, an assistant professor at the University of Toronto and staff physician at the Toronto Lung Transplant Program, has been caring for people living with interstitial lung diseases and undergoing lung transplants for some time and understands some of the questions you may have and shares some of his insights.
He began his presentation by acknowledging the contributions of two “extraordinary individuals,” that he got to know: Robert Davidson, the founder of the Canadian Pulmonary Fibrosis Foundation (CPFF) and Barbara Barr, a CPFF Board member. Both had lung transplants, both lived for quite some time with PF, and both made substantial contributions to building awareness of PF and supporting people affected by the disease. While both have passed away, their legacies live on.
Dr. Binnie explains briefly what PF is, and the various forms of interstitial lung diseases. “While we know a lot about the cells and molecules involved, we usually do not know why an individual develops pulmonary fibrosis,” he says.
It is likely a combination of repeated injury to the lungs from various things – dust, cigarette smoke, reflux, autoimmune disease and/or infections – and a defective or exhausted repair response. When the repair process fails, scar tissue is deposited instead. In addition, genes and the environment interact and both can play a role in the same individual. “There is still so much we need to learn about why some individuals develop PF and others do not.”
Dr. Binnie also covered a wide range of possible questions the newly diagnosed may have about hereditary PF, pulmonary rehab, drug treatment options, reflux, management of symptoms such as breathlessness and cough, oxygen use, diet, lung transplantation and palliative care.
According to Dr. Binnie, the most important question of all is: “What can I do?” The following are some of his suggestions.
- Stay positive. Look after yourself – mentally, physically, emotionally and spiritually – and make the best of your situation.
- Realize that you are not alone. Educate yourself about your type of PF by consulting your health care team and credible online resources, such as cpff.ca. Reach out to a pulmonary fibrosis support group, to find others dealing with similar issues and challenges and share information. (During the pandemic, support groups are meeting online.)
- Write down your questions. Make sure your doctor takes the time to address them. If necessary, insist they do so.
- If you smoke, stop now.
- Protect yourself from infections. Practice the public health measures we’ve all become used to during the pandemic; avoid crowds, others who are sick, wash your hands often. And, get your vaccines for flu, pneumonia and COVID-19.
- Get fit. This cannot be underestimated. Enroll in a formal pulmonary rehab program, if possible, which has many benefits beyond the physical. There are also online resources to help with this. Staying fit will have an impact on your symptoms and your capabilities as the disease progresses.
- Appreciate that everyone’s PF journey is different. The trajectory of your experience tends to become apparent over time. Be aware that things can worsen abruptly.
- You may need to deal with supplemental oxygen, lung transplant, and palliative care in the future.
- Do things that are important to you. You may have more limitations in the future, so why not do it now?
CPFF is dedicated to providing people living with PF and their families with reliable information. In addition to Dr. Binnie’s presentation, you can find numerous videos featuring PF experts addressing a wide range of relevant topics to help you live your best life. Visit www.hopebreatheshere.com under educational forums. Additional videos are also available on the CPFF website, including presentations on COVID-19 and PF.
Limited spaces available for free online exercise program
An online, one-on-one exercise program is now available at no cost for a limited number of pulmonary fibrosis patients. You need to enroll by the end of December 2021 to take advantage of this opportunity.
The home-based program, developed by WillKin, is a 10- session exercise and education program, delivered remotely each week, one-on-one with a specialist, accredited kinesiologist. WillKin specializes in developing programs for patients living with chronic health conditions and connecting kinesiologists with patients and providing tools to support this relationship.
You will need to be referred to this program by your respirologist if you are deemed suitable. You must also have good Internet connection, a computer or tablet with a working webcam and a small and clear area in your home (approx. 4m x 4m) to exercise safely. Once WillKin receives the referral form from your respirologist, they will contact you to explain the program and schedule your first session.
It is possible that further complimentary spaces will be available, once this first 10-week session is complete.
This program has been developed with input from those with pulmonary fibrosis. This past summer several members of the pulmonary fibrosis community participated in a survey to understand the thoughts and experiences of people living with PF, and the health care professionals who work with them, on at-home and remote exercise programs. A report on the results is now available.
The Canadian Pulmonary Fibrosis Foundation, along with RESPIPLUS™ and the Quebec Lung Association (L’Association pulmonaire du Québec ), helped to recruit participants for the survey, and the report was made possible thanks to the support of an unrestricted grant from Boehringer Ingelheim.
It must be noted that the data was collected between waves of the COVID-19 pandemic. Some areas had partial restrictions lifted, but people’s fears of the virus were still present and significant impacts on clinical settings were still present.
Even if you were not involved in the survey, you may find the results of interest. And health care professionals, who care for people living with PF, may also find some of the results helpful.