Despite the continuing restraints of a second year of the pandemic, this year’s virtual Pulmonary Fibrosis Awareness Month campaign in September raised more than $43,000 (as of October 8) and had hundreds taking part in various online activities.
Thanks to all of you, participation and connections were made across the country. You walked almost 10,000 kms, well beyond a journey across the country (which is 6,818 km) in the Hope Breathes Here Clarke Walk.
Watch our short thank you video for inspiration and a pat on the back – available now here.
This year we had a spectacular line up of 17 new webinars delivered by experts from across the country, speaking on topics you told us you wanted to know more about. We listened and acted on your suggestions. Several sessions addressed palliative care, in general, and more specifically for certain provinces. There is a session for the newly diagnosed, as well as webinars with updates on innovations, technologies and drugs for ILDs, and many other topics of interest to our PF Community.
This newsletter includes four articles with highlights from just four of the 17 new sessions. We encourage you to read the articles and then watch and listen to any, or all of them, at your convenience for a fuller picture of the topic. And check out the other 13 webinars now available online. No need to register, just click and watch. We’ll also be bringing you more webinar highlights articles in the next two issues of this newsletter.
CPFF is very grateful to all of our presenters who volunteer their valuable time and expertise to share their knowledge with the PF Community.
You can still visit www.hopebreatheshere.com where you can find greetings and proclamations from a number of elected officials from across the country, as well as photos of various landmarks, which were lit in red and blue during September in recognition of Pulmonary Fibrosis Awareness Month.
You’ll also find touching tributes of those now living with the disease and those who have passed; stories of strength and resilience despite the challenges of this terrible disease. You can still add your story, or a tribute to a loved one, by visiting www.hopebreatheshere.com. Go to the Stories section and click on the Share your Story button.
CPFF is grateful to the sponsors of the Hope Breathes Here Clark Walk: Respiratory Homecare Solutions, Telford Land & Valuation Inc., Springborough Endodontics and North Calgary Dental Specialists.
Join Drs. Angela Cheung and Margaret Herridge for this live webinar on Friday, October 15 at 5 p.m. EST titled “CANCOV Study: The Impact of COVID-19 on Lung Health.” Registration is quick and easy and you’ll be emailed a link for the Zoom call.
The Canadian COVID-19 Prospective Cohort Study (CANCOV) is a platform observational study that provides a comprehensive evaluation of early to one-year outcomes in patients with COVID-19 and their family caregivers. Get early insights from this study on the impact of COVID-19 on lung health in this live webinar with Dr. Angela Cheung and Dr. Margaret Herridge.
If you cannot make the live session, the webinar will be recorded and posted within a few days at www.hopebreatheshere.com to view at your convenience.
Dr. Angela M. Cheung is a Professor of Medicine and the KY and Betty Ho Chair of Integrative Medicine at the University of Toronto. She is an Internal Medicine specialist and senior scientist at the University Health Network and holds a Tier 1 Canada Research Chair in Musculoskeletal and Postmenopausal Health. She obtained her MD from Johns Hopkins University and completed her training in General Internal Medicine at the University of Toronto and Beth Israel Deaconess Hospital in Boston, and her Ph.D. from Harvard University.
Dr. Margaret S. Herridge is a Professor of Medicine, Critical Care and Pulmonary Medicine at the University Health Network; Senior Scientist in the Toronto General Research Institute; Director of Research for the Interdepartmental Division of Critical Care Medicine, University of Toronto. She obtained her MD from Queen’s University at Kingston, completed her clinical training in Respiratory and Critical Care Medicine at the University of Toronto and obtained her MPH at the Harvard School of Public Health. Dr. Herridge was recently quoted in an October 8 Globe and Mail article on the struggles of patients with “Long COVID.”
In a common sense and empathetic manner, Kamara Tayo-Jones discussed coping strategies for loss, change and stress in her webinar presented on September 14 and it is now available for viewing at anytime.
Tayo-Jones is a social work therapist at the Wellness Institute in Manitoba, holds a Master of Social Work degree and is a registered social worker with the Manitoba College of Social Workers. Her clients include those living with chronic illnesses.
Everyone in our PF Community should find something they can relate to in this informative and thoughtful presentation. We only wish Kamara could have an individual therapy session with each of us! We are fortunate that we have her webinar to view at any time – or several times, whenever we need to re-visit her encouraging messages.
Tayo-Jones first outlined the learning goals of the session:
Describe what stress is and how it can affect your health.
Recognize the causes of stress and why managing stress is important.
Identify lifestyle changes that you can make to manage stress.
Discuss strategies that can help you make those changes and keep motivated.
She then moved through each area offering specific suggestions along the way. We can all relate to the feeling of stress, of being overwhelmed, but chronic stress activating high levels of the hormone cortisol, has real impacts, both physical and mental. These can include: feeling exhausted, high cholesterol, high blood pressure, high blood sugar, digestive issues, heart disease, depression of the immune system, heartburn, depression and anxiety, low self-esteem, difficulty managing time and making decisions.
It is important to remember that unresolved stress can aggravate an existing illness, such as pulmonary fibrosis, and can affect individuals living with the disease as well as those who care for them.
Kamara describes coping strategies as: “What we think and do to adjust to our new lives, so that we can regain our quality of life, reduce discomfort and rebuild our sense of self-worth.” The following are highlights of some of her recommendations:
Learn all you can about your health condition, your medications and equipment, from reliable sources, such as your health care team and recommended websites.
Avoid predictable stressful situations. Learn to set healthy boundaries on your time, energy and space. You don’t have to do everything you used to, or those things that cause you stress.
Change negative thinking to a realistic mindset. Instead of saying “No, I just can’t do that. It’s too hard. Say: “I’ve done things just as hard before. I can do this.”
Create a support system and connect with them. CPFF has support groups across the country where you can share ideas, feelings and tips for coping. Your family, friends and health care team are also there to help. Let them. Keep in touch with people, by phone, online or in person. Help someone else. It just feels good to connect with others. Volunteer or take up a new activity.
Organize and manage your time and energy. Set SMART goals that are specific, measurable, achievable, realistic and timely. For example: This week I will practice my breathing exercises for five minutes each day after lunch.
Take good care of yourself. Self-care is NOT selfish. Exercise, good nutrition and sleep are the cornerstones of feeling better. Do what you can. Consult a dietitian if necessary and set up a good sleep routine. (Listen to CPFF’s Nutrition and Pulmonary Fibrosis webinar and check out the exercise resources on our website.
“Taking care of yourself is different for everyone,” says Kamara. “For some it is getting a massage or pedicure, and for others it is decluttering or watching the sunset.
“Whatever you choose, self-care activities help you feel calm, connected to something or someone and in control. It is a way for you to invest time in meeting your own needs. It is intentional and will boost production of your feel-good hormones.”
Kamara described specific activities that can increase your body’s production of what she calls the “hormones of happiness”: oxytocin, dopamine, endorphin and serotonin.
She also described various relaxation techniques that can take as little as five minutes a day, from stretching, deep breathing and progressive muscle relaxation to meditation, visualization and mindfulness. She even leads the audience through a short relaxation session during the webinar. For a short list of her recommended websites about sleep, self-care, mental health and mindfulness meditation, see our Helpful Links webpage under Living well with PF.
Yes, generic anti-fibrotic medications are essentially the same in terms of safety and efficacy as the brand name medication, but the process to come to market is much swifter and far less costly, and as a result the cost of generic drugs are substantially less than brand name drugs.
In her timely webinar on September 13, Dr. Charlene Fell talked about the differences between generic drugs and brand name drugs and concerns individuals may have about switching. This is of interest to our PF Community with the recent introduction of generic pirfenidone (brand name Esbriet) in Canada. See the article in the September 2021 issue of our newsletter. Dr. Fell is the Director of the University of Calgary Interstitial Lung Disease Program and Chair of CPFF’s Medical Advisory Board.
In her easy-to-understand fashion, Dr. Fell leads the audience through several points, including: “What are generic drugs?, Are they as effective as brand-name drugs?, Are the side effects of generic drugs worse, or better?, as well as answering questions specific to generic pirfenidone and Esbriet.
Most of us are familiar with generic over-the-counter drugs: when Tylenol is sold on a shelf next to a generic brand of acetaminophen and Advil is available next to a generic brand of ibuprofen. Cough, cold and allergy medications, among many others, are also sold as brand names as well as generic brands, often labelled with a retail brand, such as Life brand products available at Shopper’s Drug Mart.
Medications, whether over-the-counter or prescription, generic or brand-name, must be approved by Health Canada as safe and effective.
Other similarities between generic and brand-name drugs, include:
- They must have the same medicinal content (or active ingredient)
- They must come in the same form, i.e. capsule, liquid, tablet
- They must be available in the same dose
- They must be absorbed by the body in the same manner, having the same ‘bioavailability.’
Differences between generic and brand-name drugs, may include:
- They may have different “fillers,” or ingredients added to the medicinal content used to manufacture the drug
- They may look different, having different shapes or colours.
Most importantly, generic brands cost substantially less than brand-name medications, because generic manufacturers do not incur the expenses for the research and development of new drugs, including expensive clinical trials.
It is for this reason that makers of brand-name drugs receive a patent for a specific length of time to sell their drugs exclusively, to offset some of these costs, which can be hundreds of millions of dollars. In 2020, the average cost to develop a single new drug, including all the early research into potential molecules that were found not to be effective, was estimated at $985 million USD. (JAMA 2020;323(9).844-853. doi.10.1001/jama.dodo.1166)
There will also be more price reductions as other generic versions of the same drug come onto the market. For instance, the first generic version of pirfenidone will cost 25 per cent less than Esbriet, the brand name version. The second generic version will cost 50 per cent less and the price of the third generic version will be 75 per cent less. (Hollis A. CMAJ. 2015 Oct 6; 187(14): 1033–1034. doi.10.1503/cmaj.150395)
Recently, Sandoz Canada released the first generic version of pirfenidone in Ontario and it will be available soon in Alberta and then likely in other provinces. There are also two other manufacturers that will offer generic pirfenidone in the future.
Patients should be informed by Roche Canada if their prescription for Esbriet is being replaced with one for the generic pirfenidone. Sandoz Canada has confirmed that they will be providing compassionate supply of pirfenidone for anyone who is enrolled in their patient support program, who initially gets coverage either from the provincial payer or private insurance and subsequently loses their coverage.
Just as with Esbriet, the generic pirfenidone manufacturers will offer a patient support program. And you will still need to get the required blood and other tests through your health care professional to monitor for side effects and to continue to receive coverage for generic pirfenidone.
As for side effects, “with very little difference between the brand-name Esbriet and the generic pirfenidone, we can extrapolate that there should be very little difference in effectiveness or side effects,” says Dr. Fell.
For more details on generic anti-fibrotics, spend an hour viewing the complete webinar by Dr. Charlene Fell. You may also wish to view the other 16 webinars launched this September as part of CPFF’s Hope Breathes Here campaign for Pulmonary Fibrosis Awareness Month.
While they may not be as dramatic as Star Trek’s Dr. McCoy (Bones) sliding a handheld gadget over someone to determine a diagnosis or simply laying them on a table to treat almost any aliment, Dr. Giovanni Ferrara’s webinar described some recent innovations underway in ILD management that help put the patient at the centre of care.
With a warm and thoughtful manner, Dr. Ferrara of the University of Alberta, begins his presentation with an overview of his session, including: the complexity of the multi-disciplinary approach to ILD; new ways to look at pulmonary function tests and the six-minute walk; Patient-reported outcome measurements (PROMS) and patient-reported experience measurements (PREMS) and hopes for the future.
Complexity of multi-disciplinary approach to ILD
“The days of the single physician instructing a patient what to do are over,” says Dr. Ferrara. Today, a legion of health care professionals make up the ILD care team, including the general practitioner, respirologists, radiologists, pathologists, respiratory therapists, nurses, social workers, rehab therapists, palliative care specialists and many more.
“No one person can deliver the optimum care anymore,” says Dr. Ferrara. ILD care requires coordination and many resources, with the patient at the centre of the circle of care.
Care involves everything from patient education to advance care planning and from drug treatments to ongoing testing and assessments to monitor disease progression. In some cases disease management may include a lung transplant, which is an option for only about 15 to 20 per cent of patients.
And while anti-fibrotic drugs are slowing disease progression, survival for diseases like IPF is an average of three to five years from diagnosis until transplant or death; with the unpredictability of acute exacerbations affecting survival.
“The best supportive care for these patients have to include targeted interventions that improve quality of life,” says Dr. Ferrara, “and have to identify personalized solutions to the patient’s needs.”
New ways to look at PFT (pulmonary function tests and the 6-minute walk test)
The pandemic has made it difficult, or risky, at times for patients to visit ILD clinics for regular assessments, including pulmonary function tests such as forced vital capacity (FVC) done in “the box” and the six-minute walk test. And for more remote patients this has always been an issue.
There are some innovations now under investigation to make these test more available remotely, with the patient doing their own testing at home.
A recent UK study, had a number of patients use a home spirometer each day to test their own FVC each day, in addition to having the test done in the clinic every four weeks. The results using the two methods were quite similar. And while the home spirometer does not offer the full range of measurements, yet, it was quite accurate and also helped track FVC during exacerbations.
The one drawback was that over the course of a year, patient use of the home spirometer declined because of the lack of time, illness or forgetting to do the test.
“This shows that any new technology needs to be as user-friendly as possible and integrate easily into someone’s life,” says Dr. Ferrara.
Research is also underway to develop wrist and/or waist devices that collect the same data comparable to a six-minute walk test and then send results to specialists. These devices can track very well daily activities and artificial intelligence software can analyze the data, indicating the performance status of the patients.
Another European study, using a wrist “step counter” found that a decline in physical activity may be another way to measure disease progression, since they found that a decline in steps did not correlate to physiological measurements such as FVC and the six-minute walk test, but did indicate a decline in survival.
It must be stressed that these products have to be validated as “medical” devices, not “wellness” devices produced for general consumers. This implies that they have to be approved by Health Canada, through a rigorous validation process.
PROMS and PREMS
Patient-reported outcome measurements (PROMS) and patient-reported experience measurements (PREMS) questionnaires have been done increasingly remotely too, during the pandemic.
In a recent study, a newly treated group of IPF patients were randomly assigned to a standard care group or a home monitoring on top of standard care group for 24 weeks. The home monitoring program included such things as home spirometry, reporting of symptoms and side effects, a medication coach, patient reported outcomes, information and e-consultations.
While physical symptoms were reported to be worse in the home monitoring group, perhaps because they were tracking and reporting them more often, the home monitoring group reported feeling reassured, having better insights into their disease course, and had more accessible communication with the hospital. And, 95 per cent would recommend the program to others.
Hope for the near future
Dr. Ferrara expects to see a more frequent and improved use of PROMs and PREMs in the future, with patient concerns and barriers to access overcome by the new technologies. And in Rome and elsewhere, work is proceeding on oxygen concentrators that adjust to the patient’s need for oxygen automatically. And, Dr. McCoy may be not be so far away from us. In perhaps five to 10 years, we may have wrist monitors that listen to the lungs and send reports directly to a central monitoring system.
Whatever the future of ILD care holds, Dr. Ferrara says, “it is paramount that patients be involved in the process, so we can ultimately improve their quality of life.”
Most individuals living with pulmonary fibrosis would benefit from early access to palliative care, according to Dr. Doris Barwich, Medical Director, BC Centre for Palliative Care and Dr. Shalini Nayar, of Surrey Memorial Hospital and a specialist in both respiratory medicine and palliative care.
The two doctors presented their webinar “Intersects and access to palliative care” live on September 17. It is now available on the Hope Breathes Here website for viewing at any time.
Palliative care is not only for end-of life, but is a specialized form of healthcare for individuals and families who are living with a life-limiting illness that is usually at an advanced stage. The goal of palliative care is to provide comfort and dignity for the person living with the illness, as well as the best quality of life for both the individual and their family.
Palliative care addresses pain and symptom management and provides social, psychological, emotional and spiritual support, as well as providing support to caregivers and family members.
According to Dr. Barwich, between 62 per cent and 89 percent of those who die could benefit from palliative care, including nearly everyone who does not die unexpectedly. Unfortunately, few Canadians (15 per cent) have early access to palliative care in the community and 80 per cent of the time, when palliative care was provided in hospital during admission, the stay was unplanned or through an emergency department. In addition, most Canadians would prefer to die at home if they could receive the support they need and those who received palliative care at home were 2.5 times more likely to die at home than those who received regular home care.
A UK task force for lung health, found that people with lung disease often face as much pain and other mental health issues as cancer patients and that shortness of breath is a particularly distressing symptom for people with lung disease, as well as for their loved ones, and has a strong negative association with quality of life and prognosis.
In the last year of life, what is most important to individuals is having a realistic understanding of their expected symptoms, quality of life and disease progression. Palliative care supports can prepare the person experiencing the symptoms and their caregivers. A palliative care team can help with advance care planning (ACP), including identification of goals of care and an action plan for a crisis, which reduces stress and anxiety and often the number of visits to the emergency department.
In addition, several studies on hospice palliative care offered to people with IPF indicate that there is a great need for early integrative palliative care with advance care plans to improve the end-of-life care, says Dr. Barwich.
Dr. Shalini Nayar shares with listeners the “Bowtie Model” of where disease management intersects with palliative care (complete with cute dog photo wearing a bowtie). Pain and symptom management fall within both categories, with rehab and end-of-life care added to the palliative side of the bow.
She spoke about the three different journeys of illness leading to death – in a simplified manner to show the differences.
- The terminal illness trajectory (usually cancer deaths) is generally predictable with a short course of a few years with more rapidly progressive decline in the last few months of life.
- The organ failure trajectory is a longer course of slow decline with intermittent acute, life-threatening episodes and then a seemingly “sudden” death. Often this would occur over a period of two to five years. This would be common, for example, with lung or heart failure.
- The frailty trajectory is a long course of slow unpredictable deterioration with the ability to self-care decreasing over time, maybe even over as much as six to eight years. This would be consistent with the frail elderly and dementia.
She noted that most approaches to palliative care follow those facing the terminal illness trajectory, but it is clear that those facing the organ failure or frailty trajectory could also benefit from palliative care.
Which ILD patients might benefit from a palliative care approach? Dr. Nayar highlighted some ways to identify those who would. First, does the patient want or need supportive care? Would the health care team, or family members, be surprised if this patient were to die in the next year? Has the patient had more than two admissions to ICU for ventilation? Are they experiencing increased breathlessness? Have test results shown substantial reduction in lung function? Clinical indicators can include: a DLCO (diffusing ability of the lungs) less than 40 percent; oxygen levels less than 88 per cent during the six-minute walk, increased scarring on CT scans, and a decrease of more than 10 percent of FVC (forced vital capacity) over a set period of time.
There are challenges and misconceptions holding people back from requesting and providing palliative care. Patients are generally aware and state their preferences, but they are often waiting for health care professionals to begin discussions, especially about end-of-life care. It is worth noting that caregivers are often inaccurate about a patient’s quality of life.
In general, the public believes that “doing nothing,” means more discomfort.
The health care team can also be reluctant to suggest palliative care for many reasons, such as: unrealistic expectations of recovery; lack of awareness of options when a recovery is no longer possible; difficulty withdrawing or withholding care; a lack of experience or discomfort in breaking bad news; a lack of time, privacy or too many interruptions for discussions; and personal and professional experiences with illness and death.
There are medications that can ease pain and anxiety for ILD patients. Again, there are still misconceptions, both by patients and health care providers. The judicious use of opioids for pain management in pulmonary patients is safe and does not suppress respiration. Opioids can also ease breathlessness. Benzodiazepines can help relieve anxiety, which can be brought on by breathlessness and general uncertainty and fears.
In summary, Dr. Nayar acknowledges that the course of various ILDs is different for everyone, but that death can come at any time. We need to recognize that palliative care approaches should begin early for those living with pulmonary fibrosis. Advanced care planning can help to relieve anxiety for both patients and their families. Part of that plan should be the preferred place of death. Home palliative care is an option and patients should identify someone for shared decision making, in the event they are unable to make decisions for themselves.
You can learn more about palliative care and available services in your province, by listening to some of the four other palliative care education sessions recorded last month. They are now available on the Hope Breathes Here website. More information is also available at your provincial palliative care association and the Canadian Hospice Palliative Care Association.
The University of Alberta is seeking participants for its study: “AB-ILD pathway to improving ILD care in Alberta.” The study is a collaborative effort between ILD centers in Calgary and Edmonton. It aims to improve time to diagnosis and access to care for people living with idiopathic pulmonary fibrosis (IPF) and other interstitial lung disease (ILD), and their health care team.
As a first step, the researchers are looking to learn of your experience with your ILD diagnosis journey and understand the barriers and challenges you may have faced through this process.
Participants need to have IPF or another ILD diagnosed in Alberta, be over 18 years of age, able to speak English reasonably well, able to give consent and have access to a computer or a smartphone.
Participation involves taking part in one, online, focus group discussion for approximately 50-60 minutes.
If you or someone you know are interested in participating in this study, please contact: Dr. Charlotte Pooler RN, PhD, at 780-700-0468 (you can leave a voicemail) or Charlotte.Pooler@ualberta.ca.