April 2022

Help us get nintedanib for all who need it

CPFF has provided a simple online tool to make it easy for you to contact your provincial representative and ask for expanded coverage for nintedanib, so all PF patients who need it have access.

We are very pleased to share the news that Alberta became the first province on April 1, 2022, to list on their drug formulary and fully reimburse the use of nintedanib (Ofev) for all chronic fibrosing ILDs (interstitial lung diseases). And today we learned that Quebec will also expand its coverage of the drug, effective immediately.

The medication nintedanib has been used to successfully treat one type of interstitial lung disease (ILD) – idiopathic pulmonary fibrosis (IPF) – in Canada since 2015 and has been listed and fully reimbursed under all provincial health plans across the country for the treatment of IPF, since 2018.

CPFF (Canadian Pulmonary Fibrosis Foundation) has been advocating for some time for the provinces to expand the listing and coverage of nintedanib for all individuals with a chronic fibrosing ILD. “I want to thank Alberta and Quebec for leading the way in broadening coverage beyond those with IPF to others who could benefit from this anti-fibrotic medication,” says Sharon Lee, Executive Director of CPFF.

Now is the time for the other provinces and territories to list and fully reimburse the use of nintedanib (Ofev) for all chronic fibrosing ILDs. If you live outside of Alberta or Quebec, please join us by sending a letter to your MPP/MLA. It is easier than ever to do so. Click on this link and complete the online submission – the email message is already done for you – and your call for action will be sent directly to your provincial or territorial representative automatically. No need to look up your representative’s email address.

“As a member of the PF Community, and as a voter, your email to your elected official cannot be ignored,” says Lee. “ This simple action, can make a huge difference to those currently not able to access anti-fibrotic medications in their province or territory. Please help them.”

CPFF has alerted all other provincial and territorial ministers of health of the recent addition of nintedanib to Alberta and Quebec’s formularies and is urging them to follow suit.

CPFF made patient submissions to CADTH (Canadian Agency for Drugs and Technologies in Health) and INESSS (the corresponding Quebec agency), which subsequently  recommended in February 2022 that nintedanib (Ofev) be reimbursed for the treatment of chronic, progressing fibrosing ILDs under certain conditions. Earlier this year, Boehringer Ingelheim (Canada) Ltd., the maker of Ofev, and the pan-Canadian Pharmaceutical Alliance (pCPA) completed negotiations for the pricing of nintedanib, (Ofev) for treating chronic fibrosing ILDs.

Being a PF caregiver has it challenges and rewards

Beth Garbutt has found ways to cope and thrive as a care partner to her husband Rob, who lives with IPF.

Asked what advice she’d give to other caregivers living with people with pulmonary fibrosis (PF), Beth Garbutt, 61, has a few tips: “Join a support group. Learn more. Ask questions. And, don’t Google PF. Instead, visit credible websites like www.cpff.ca for accurate information.”

Beth’s husband Rob Garbutt, now 63, was diagnosed in 2011 with IPF and for about eight years he was able to continue with most of his activities, until in 2019, he was hit really hard with an exacerbation and hospitalized for a week. His condition has worsened since that time and he has now been waiting for more than a year for a lung transplant. The couple live in Winnipeg and transplant surgery will take place in Edmonton.

Beth’s assistance has been vital a couple of times – after Rob got out of hospital and in preparing for a lung transplant. “After the exacerbation, there were so many medications and he was so sick,” says Beth, who still works full-time. “It was very stressful.” She helped him with organizing his medications; colour-coding the pill bottles and setting up a medication journal. There was also plenty of paperwork for insurance purposes and once he quit working, more paperwork for employment insurance and other benefits too. She handled most of it for him.

Once he was stable again, Rob was able to care for himself and drive to appointments himself. He keeps as active as he can, taking along his oxygen when he rides his recumbent bicycle with his dog Maggie and golfing with a cart. These days others need to do some of the more strenuous household chores he used to do, like cutting the grass at the house and the cottage.

“But, he can help with the vacuuming – a bit at time,” says Beth, “and helps with some of the other housework. It’s a team effort.”

The other time Beth’s support was critical was in making the decision to proceed with a lung transplant and going through the “medical boot camp” assessment process. “I went with Rob to his specialist appointments, to listen and process the information. It can be overwhelming. Together, we sifted through the pros and cons and made the decision to proceed with the transplant assessment.”

This process involves an exhaustive series of medical appointments to assess his physical condition. “There were so many calls with doctors and nurses and appointments to schedule and then so many tests. Sometimes three, and once even six, in one day.”

Since it’s been more than a year since his first transplant assessment, with no transplant surgery, Rob and Beth will go through the medical boot camp once again, in case there have been any changes in his physical condition or in their situation, that would impact his chances of a successful outcome.

In between and during these times of “crisis,” Beth most importantly provides emotional support. In some ways, she thinks the COVID-19 pandemic has been helpful. Beth has been able to work from home for 18 months and public health restrictions made it easier to avoid exposure to all viruses as they wait for the call from the transplant centre in Edmonton.

It’s been mostly just the two of them during the pandemic. Friends have checked in and helped out as needed. Their son Andrew works in Vancouver and their daughter Maegen recently had their first grandchild, a boy.

Beth is back to work in the office some days now and her co-workers have been very supportive, wearing a mask around her and inquiring if Rob has received that important call from Edmonton yet. PF altered their future plans with Rob retiring five years earlier than planned and Beth now planning to keep working longer than anticipated.

As a caregiver, Beth knows it’s important to take care of her own physical and mental health. She’ll often spend Saturday and Sunday mornings painting watercolours. And she exercises each morning on the recumbent bike at home. Both she and Rob are involved with the CPFF Winnipeg Support Group and have found it especially helpful in supporting one another through COVID-19. Rob has shared his story in a recent CPFF documentary and as a spokesperson for CPFF advocacy and awareness campaigns.

An offshoot of the group is just for caregivers and meets one evening a month. “It helps to realize that there are other people going through a similar journey, although everyone’s journey is different. We ask and answer questions and cry and laugh together. And we understand one another. It’s hard for caregivers when it’s all about the person with the disease all the time.”

Throughout her journey as a PF caregiver, Beth has learned about the power of positive thinking. “I just don’t let myself go to that dark place,” she says. “I focus on the life-affirming things and activities in my life, like my new grandson and making a new bucket list.”

Rob and Beth have made plans for the future after the transplant. “We’re going to travel out west and go downhill skiing again.”

Editor’s Note: April 5, 2022 was National Caregiver Day, recognizing caring Canadians. For more information and resources for caregivers, visit Carers Canada.

Thank you to all who took part in our Rare Disease campaign

Thank you to everyone who participated in our Rare Disease campaign which raised more than $7,300 and earned more than 338,000 impressions.

From February 28 until March 31, the Canadian Pulmonary Fibrosis Foundation (CPFF) conducted its Rare Disease campaign to raise awareness and funds to help advocate for more equitable access to diagnosis, treatment, support and opportunity for people living with pulmonary fibrosis (PF).

“In all respects, the campaign was a great success, especially in terms of building awareness,” says Sharon Lee, Executive Director, CPFF.

The campaign generated more than $7,300 in donations, significantly increased CPFF community engagement, grew CPFF social media followers and built awareness with new audiences who haven’t previously engaged with the organization.

The campaign garnered more than 345,000 interactions, or impressions, with campaign- related content, including web pages, social media channels and YouTube. Dozens of people sent cards both digitally and by mail to honour the people they know living with PF, or to remember those who have passed away due to the disease.

CPFF’s new documentary Breathless for Change, the documentary trailer, and video clips with “rare individuals” were viewed more than 66,000 times. Several others shared stories of courage and resiliency on the website.

“I’d like to thank everyone who participated in our Rare Disease campaign in every way, whether making a donation, sending a card, responding to a social media post or sharing their story online,” says Lee. “It is always a privilege to honour members of our brave and committed PF Community.”

How did I get pulmonary fibrosis?

European webinar offers some answers to several questions about PF, including: “How did I get pulmonary fibrosis?”

It’s a question most people diagnosed with pulmonary fibrosis ask. Could it be a reaction to inhaling wood or metal dust? Could it be from cleaning out the bird cage each week? Or is it related to my advanced rheumatoid arthritis?

These questions and many more are ably answered in a recent webinar hosted by the European Pulmonary Fibrosis Federation.

Guest speaker Dr. Simon Hart, Hull York Medical School/ University of Hull, gives us an easy-to-understand overview of the risk factors of pulmonary fibrosis, the process of fibrosis and the symptoms of the disease. He also discusses the tests used by health care professionals and explains how they inform diagnosis and treatment. The presentation is mainly aimed at patients and their families, although health care professionals may also find it interesting. The webinar is moderated by EU-IPFF President, Steve Jones.

Dr. Hart begins by stating that while many genes may be involved in pulmonary fibrosis, there is no single gene, or genetic test, that indicates whether someone will get PF. And only about one person in 10 with PF has, or had, a relative with the disease.

Environmental factors, even in the distant past, may induce an immune response that can trigger PF, such as inhaling smoke, wood or metal dust, or asbestos. Inhaling bird dander or mould can also trigger such a response.

Reflux, which can occur silently, without any symptoms, may also be a risk factor. “This means you could be inhaling something that should be in your stomach, rather than in your lungs, causing a fibrotic reaction in the lungs.”

Some people with connective tissue diseases like rheumatoid arthritis may eventually develop pulmonary fibrosis. And, some medications used to treat cancer can also cause PF.

However, the single biggest risk factor is age, according to Dr. Hart. Most people develop PF as older adults, and it is that factor, probably in combination with the other risk factors already mentioned, that put people most at risk for developing pulmonary fibrosis.

In his description of the process of fibrosis, age also seems to be a factor, as he talks about “old and exhausted cells” and the healing function of scarring that has gone wrong.

Another interesting moment in the webinar is when Dr. Hart addresses breathlessness. He mentions that it often is not tied to a lack of oxygen, but rather can be the result of “neuro-mechanical uncoupling” with the brain registering that the lungs are not inflating as they should, so people experience the sense of breathlessness.

He spoke of exploring ways to “re-train’ the brain to overcome this sensation and talked about how a hand-held fan blowing cool air on the face seems to bring some relief.

Overall, there is some very thoughtful information in this webinar from the other side of the world and some approaches that differ from those in North America. This webinar is one in a series of monthly sessions being offered by the organization in the lead up to the European Pulmonary Fibrosis Patient Summit taking place virtually in November 2022. Visit their website to be notified of upcoming sessions.

If you are interested in more information about occupational and environmental inhaled risk factors, you may be interested in reading a recent article in the March 2022 issue of this newsletter about a research project now underway at the University of Calgary, led by Dr. Kerri Johannson.

COVID-19 two years later

This UHN video discusses where we are today, how we’ve managed so far, what we’ve learned and how COVID-19 has impacted society and individuals, beyond the consequences of the disease itself.

When the latest UHN Virtual COVID-19 Q&A video was broadcast on March 10, 2022, it was almost two years to the day since the virus was declared a pandemic by the WHO (World Health Organization). Today, we’re learning to live with the virus.

This video by the University Health Network (UHN) Foundation in Toronto features Dr. Susy Hota, Medical Director of Infection Prevention and Control at UHN. Moderated by Christian Cote, the two discussed where we are today, how we’ve managed so far, what we’ve learned and how COVID-19 has impacted society and individuals, beyond the consequences of the disease itself.

On March 10, Dr Hota reported that case counts, along with hospitalizations and ICU admissions were stable and that positivity rates have been at 11 per cent for about a month. With testing restricted, Dr. Hota says scientists are focused on waste water sampling, along with hospitalizations and ICU admissions, to monitor the virus’ impact on the population and the health care system.

In Ontario, the lifting of most restrictions, including mask mandates in most settings, on March 21, means hospitalizations will likely rise, especially among the unvaccinated. Many in the health care field, including Dr. Hota, felt it was too soon to lift mask mandates, especially for schools as they headed into March Break.

Dr. Hota, recommends you assess the risk for yourself and others within your social circle to make decisions about exposure and what adjustments to make, like wearing a mask.

“Think about what will happen if you get COVID-19, even if you don’t need to go to the hospital. You will need to isolate, probably at home, and will not be able to do your normal activities and get the things done, you usually do. You will not feel well, maybe for a week or more. You may infect others more vulnerable within your home.

“For instance, if you have a big interview coming up, you may not want to go to a large, indoor sporting event in the days before the interview.”

As the weather improves, gradually ease into your social life, as you feel comfortable. Start with small gatherings outdoors, wearing a good-fitting mask. If you’re meeting others indoors, check on the vaccine status of others before you decide whether or not to attend.

Get vaccinated. Overall our vaccination rates are quite high, with about 85 per cent of those eligible (over five years old) having two shots. “But, it’s surprising how many people have not gotten a third-shot or booster, especially with the Omicron variants being so contagious,” says Dr. Hota.

As things open up, Canadian scientists are tracking the experiences of other countries who are ahead of us with easing restrictions. They are watching the trends in terms of natural immunity, immunity from vaccines and waning immunity.

How do we process the meaning of the last two years, Cote asked Dr. Hota? “We can look at how we’ve changed,” replied Dr. Hota. “We have become more focused on preventing illness. And we can look at how our behaviour has changed under long-term stress. And people are much more aware now of the role of public health and medical science,” she adds.

Dr. Hota also spoke about how our society is not as science literate as it could be. She adds that some of that is on scientists to improve how they communicate both simple and complex information and ideas to the public. Most importantly, we need to made more information accessible and make sure people know where to find credible information.

Looking to the future, Dr. Hota believes that the most likely scenario is that COVID-19 will evolve to be a more seasonal respiratory virus. And eventually, a combination COVID and flu vaccine will become part of our fall healthcare routine.

Until that time, the presenters urge everyone to get every COVID vaccine as it becomes available. Fourth shots are now being offered in some provinces for more vulnerable populations, like those with pulmonary fibrosis, older adults and those living in congregant settings, like nursing homes.

The next UHN video COVID-19 Virtual Q&A looks at the “Sixth Wave,” with Dr. Alon Vaisman, Infection Control Physician, University Health Network. Now, we must learn how to navigate social gatherings responsibly, mitigate infection control in public spaces and enlist best practices for keeping our most vulnerable communities safe. The livestream video was held Thursday, April 12, 2022 from 5 to 6 p.m. EST. You can watch the video after the live broadcast anytime.