Life and Breath: Stories of IPF
Dr. Charlene Fell
Dr. Charlene Fell’s first experience with idiopathic pulmonary fibrosis (IPF) came early on in her medical career. It was during her fellowship in respirology at the University of Calgary that she was assigned a patient who changed the course of her career, and ultimately inspired her to dedicate her research efforts to unraveling the mystery of IPF. Dr. Fell remembers that first patient as a remarkable man with a gentle spirit, who was in the prime of his life. “I was so frustrated that I couldn’t do more to help him,” recalls Dr. Fell.
IPF is a serious disease that causes severe scarring of the lungs and makes it difficult to breathe. Its cause is unknown. The average survival time from diagnosis is three to five years. An estimated 5,000 to 9,000 patients are living with IPF across the country. There are currently no treatments available for IPF in Canada. However, Dr. Fell is optimistic that IPF patients will have access to effective treatment options and better diagnostic tools in the not too distant future. “There are a lot of exciting things happening in the field right now,” she says. Dr. Fells cites cutting edge research being done by a team of Canadian researchers at the Firestone Institute in Hamilton as one example. The researchers are working on developing biomarkers for IPF – the basis for a blood test for diagnosing the disease. In addition, she notes that several large-scale clinical trials are showing promise and could lead to the development of drug therapies within the next five years.
Dr. Fell is chair of the Canadian Thoracic Society Pulmonary Fibrosis Interest Group, which comprises respirologists, clinician scientists, researchers and pathologists with an interest in finding better ways to treat people with IPF. Dr. Fell is particularly excited about the group’s work in creating a national database of IPF patients. The database will be instrumental in facilitating research on the disease. The group also plans to develop Canadian IPF clinical practice guidelines to help better diagnose and treat the disease. Diagnosing IPF is a detailed process. It requires a careful medical history and input from specially trained radiologists and pathologists. A correct diagnosis is important because there are more than 200 forms of pulmonary fibrosis.
On average, Dr. Fell sees an estimated 100 to 120 patients with pulmonary fibrosis per year in her clinical practice. Just three or four of these patients a year will undergo a lung transplant. Although lung transplant is currently the only effective treatment for IPF available in Canada, the process of finding suitable lungs and then undergoing the actual procedure can be long and difficult. The average wait time for new lungs is two years. Most patients are supported with oxygen therapy to help them breathe and pulmonary rehabilitation. Some patients are able to participate in clinical drug trials.
“We need greater awareness about IPF,” says Dr. Fell. Indeed, awareness of IPF remains low compared with other diseases which have high profile fundraising events to raise money for research. Dr. Fell says awareness is slowly growing and she is hopeful that this will make a difference in the lives of those with IPF and their caregivers. “Raising awareness about IPF among the general public is a crucial first step in generating more research dollars into the disease – and eventually finding a cure.”
Judy Riedel, Lung Transplant Support Group Leader
They range in age from teens to seniors, a mix of men and women. Some are students. Others are professionals. They come from all walks of life – united by a common thread – all have been affected by serious lung disease. They are either waiting for, or considering undergoing a lung transplant. A few have already had the procedure. Some are caregivers.
Led by the Manitoba Lung Association’s patient educator Judy Riedel, the Lung Transplant Support Group is a lifeline for its members. Once a month the members meet to share information and offer each other emotional support. Their stories are powerful and at times heartbreaking. Their courage is inspiring. “I wasn’t sure what to expect when I started the group,” says Riedel, a registered nurse. “Lung transplant is a complex journey, but I quickly learned that the need for emotional support is overwhelming for these patients.”
Riedel started the group almost a year ago. Members must be referred from the Lung Transplant Team at Winnipeg’s Health Sciences Centre hospital. For those too sick to attend in person, telephone support is available. The group also has an active online community via Facebook – this forum is limited to Lung Transplant Support Group members to ensure a comfortable forum for sharing.
The assessment for lung transplant is a long process with many steps. Candidates must be assessed by a team of health professionals to determine whether lung transplant is a viable option. “You have to be sick enough to qualify, but well enough to undergo the transplant,” explains Riedel. This paradox is frustrating and draining for both the patient and their families. Once the complex approval process is completed the heart wrenching waiting game begins. Some group members have died waiting for new lungs. “That’s incredibly hard. It’s like losing a family member.” The average wait time is about two years.
Post-transplant patients and their families also benefit from the support group. The road to recovery following a lung transplant is often difficult. Anti-rejection medications can cause serious side effects and rehabilitation for some can be very slow. The support group forum offers the chance to ask questions based on current medical information, problem solve and adjust day-to-day activities to improve quality of life. The information also helps those on the wait-list to better understand what to expect post-operation.
Riedel is passionate about patient education. She has been with the Manitoba Lung Association since 2003 and prior to that worked in a hospital as a patient educator. She is a natural fit for this role. Her warmth and gentle spirit are complimented by a depth of professional skill and knowledge. Riedel says the support group has taught her to never underestimate the power of hope and human connection. “It’s just so easy to love these people.”
John: A brother’s story
* Names in this story have been changed to respect the privacy of the family.
John and his family of five siblings were no strangers to filling the role of caregiver. The close-knit family had rallied together many years before to support their brother Max, 51, who was diagnosed with schizophrenia as a teen. With family support and the right medical treatment Max has been able to live independently and enjoy a full life. In 2005, however, Max began losing weight and feeling tired and short of breath. After several doctor’s visits, Max was finally diagnosed with idiopathic pulmonary fibrosis (IPF). It marked the start of a long journey to help Max navigate the medical system and find information and support.
Max uses oxygen full time to breathe. He’s been on the lung transplant list for roughly two years. “Max is a bit of a special case,” explains his brother John. “He has a rare blood type and he’s a small man so finding the right set of lungs is proving to be a bit of challenge.” IPF varies from person to person. In Max’s case the disease has progressed fairly slowly. He has been hospitalized about twice a year with lung infections. John says because awareness of IPF is so low, it’s difficult sometimes to communicate Max’s needs to hospital staff when he has a breathing emergency. He suggests patients carry a letter or card from their lung specialist explaining the condition and what to look for. “It would make the treatment process so much smoother to have a simple tool.”
John also advises having a caregiving plan and schedule so others can fill in the gaps. He stresses the importance of setting limits and being realistic about what you can do. He also suggests attending a support group. He and Max attend a lung transplant support group once a month together. The group offers hope and emotional support for both caregivers and patients.
John tries to spend as much time as he can helping Max, taking him to appointments, monitoring his condition and including him in family activities. He is inspired by his brother’s courage and positive outlook. Caregiving is rewarding, but it’s not always easy. Balancing a career and family obligations with the demands of caregiving is sometimes a challenge, John admits. It’s a juggling act that can lead to burnout. “You have to know when to ask for help yourself,” says John. “You don’t have to be a superhero. ”
Ken Peagam’s story
Ken Peagam, 67, has always loved the simplicity of a long walk outside. The combination of fresh air and exercise is just good for the soul somehow. Ken and his wife Linda enjoyed a walk together almost daily – whether around their Fort Qu’Appelle, SK, neighborhood or on a sandy Florida beach in winter. But three months ago Ken’s daily walk became suddenly – and increasingly – difficult. The retired military firefighter felt short of breath. “When I got home from my walk I’d have to sit for a while to catch my breath.”
Soon even a short walk outside left him feeling exhausted and breathless. At his wife’s urging he went to his local emergency department. After a 10-day stay in a Regina hospital and a series of tests he learned he had idiopathic pulmonary fibrosis. Two months later the diagnosis is still fresh. “This all just so new to me still…I feel like I don’t know what’s coming at me next.”
Ken is currently undergoing testing to determine his suitability for a lung transplant. Every week Linda takes him to several different medical appointments. The process is long and involved. Pre-screening for a lung transplant requires a detailed physical exam that includes several blood tests, chest x-rays, heart and lung tests, among others.
Ken must use oxygen 24/7 to help him breathe. His daily walks have been replaced with a daily ride on a motorized scooter. But he seems to take this in stride and remains thankful for small blessings. “I miss walking, but my scooter gives me independence…I can still get outside.” Ken is also grateful for the support of his family and friends. He is a proud father of three adult daughters and 11 grandchildren. Visitors come often to the Peagam home.
Like many others with IPF, Ken hadn’t heard of the disease before his diagnosis. He wonders if his career as a military firefighter might have triggered the disease. He fought many training fires during the course of his career in which aviation fuel, furniture, oil and chemicals were burned in a fire pit. “We wore a hood and no mask so we breathed in everything,” recalls Ken. “Did it cause my disease? I don’t know, but I do wonder.” IPF is a mystery. The exact cause of the disease is unknown. Researchers are working to unravel the mystery of IPF and its possible causes including occupational risk factors. Ken hopes his story will inspire further research – and eventually a cure.
Lana Biro, Registered Respiratory Therapist and Certified Respiratory educator
When asked to describe the most rewarding aspect of Lana’s job as a certified respiratory educator with the Ontario Lung Association, Lana says "I like making a difference".
Lana works as part of a team of educators who staff The Lung Association’s helpline. A respiratory therapist with a background in critical care, Lana and the helpline team answer questions from patients and their families on a variety of issues related to lung disease, including idiopathic pulmonary fibrosis. Often those calling about IPF are newly diagnosed and upset with this diagnosis. Lana notes that awareness of IPF remains relatively low compared with other diseases. Many people have never heard of the disease until their diagnosis.
One of the first steps in coping with a diagnosis of IPF is getting educated about the disease. “Our IPF patients and their families have so many questions. This disease is such a mystery and it can be overwhelming,” explains Lana. Part of her role as a certified respiratory educator is to help patients better understand and manage their disease. Although there is currently no cure for IPF, there are strategies people can do to conserve energy and make day-to-day living easier.
IPF patients and their families also call the helpline for emotional support. Talking to someone who understands the disease and its challenges can literally be a lifeline.
Lana says more resources and research are needed to support those with IPF – and ultimately find effective treatments. She has been working with The Lung Association to develop new resources and online content based on her experience in supporting IPF patients.
Lana belongs to a special interest group on IPF to stay up-to-date on the latest IPF research and developments. “ I want to help solve the puzzle of IPF.”
Questions about lung health? Ask an expert. Our certified respiratory educators are ready to take your questions. 1 -866-717-2673
Dr. Nasreen Khalil, an IPF researcher: A story of persistence and dedication
One of the most important characteristics of an idiopathic pulmonary fibrosis researcher is persistence. Indeed, Dr. Nasreen Khalil has dedicated her research career to unraveling the mystery of IPF and finding effective treatments. The disease affects an estimated 30,000 Canadians and there is no known cure – yet.
It was during her respirology fellowship some 20 years ago that Dr. Khalil first became captivated by IPF. Previously viewed as an inflammatory disease similar to asthma or COPD, Dr. Khalil and her team discovered that IPF is actually a disease of the lung tissue. In fact, she was the first scientist to describe the association of a molecule called TGF-b1 with the development of pulmonary fibrosis. This groundbreaking discovery changed the course of her IPF research and led to the development of an agent that she hopes will one day be made into a commercially available drug to treat IPF. It also put her team of Canadian researchers on the map as leaders in IPF. She is currently based at the University of British Columbia.
It is her patients that keep Dr. Khalil working so hard to raise the profile of the disease, and pounding on doors for research funding. “It’s heartbreaking. I have one patient – she has only a quarter of her lung function left and she’s just struggling to live. Almost every year around spring she’s asks me: Is there anything you can do to keep me alive for just a bit longer-just until the summer?” The typical IPF patient is usually between 50 to 60 years old and male. The number of people being diagnosed with IPF is rising. Currently, 6,000 Canadians are diagnosed with IPF each year, and 5,000 Canadians die from the disease annually. The two hallmark signs of IPF are shortness of breath and a persistent, dry cough. “This disease is insidious,” explains Dr. Khalil. “Many people cannot pinpoint exactly when they noticed becoming short of breath – usually when doing regular activities.”
It hasn’t been an easy road – and there is still a long way to go before clinical trials and commercial development of her team’s drug. But step by step she is working to advance her discovery to give hope to those living with the progressive and fatal disease. Over the years, she has juggled raising a family, earning a living at her “day job” as a doctor, and finding the time and funding to conduct her research. She admits that: “There are times when I was so discouraged and tired and I thought about giving up, just stopping and having a regular life” But that feeling was fleeting. “One look at my patients and I see no choice but to keep moving forward; they are innocent victims of complex disease and deserve to have investigators and researcher to keep going; anyone can give up but not everyone can keep going.”
Ralph McFadyen's story
Ralph McFadyen, 69, says he’d never heard of idiopathic pulmonary fibrosis before his diagnosis six years ago. "I just had no idea what to expect – it was all new to me."
"The first sign for me was difficulty breathing," recalls McFadyen, who lives in Cartwright, Manitoba with his wife Leyah. Once an avid curler, he began suffering bouts of shortness of breath. Everyday activities like walking upstairs felt increasingly difficult. Eventually, a specialist diagnosed him with IPF. McFadyen was stunned. He had no history of the disease in his family and had worked in an office all his life. "I had heard of miners or farmers getting these serious lung diseases. I was totally shocked when I found out I had IPF." McFadyen enrolled in a clinical drug trial soon after his diagnosis, which he says was helpful in getting information on the disease and follow up care.
"Idiopathic" means the cause is unknown. IPF varies from person to person. In some people, the disease progresses slowly over many years. In other cases it may progress rapidly. Symptoms range from moderate to severe. In McFadyen’s case, his condition was fairly stable for several years and began getting worse more recently.
McFadyen says he remains hopeful, yet realistic about the road ahead. McFadyen has been using oxygen full-time since January 2012. He has been on the lung transplant list since 2010 and belongs to a lung transplant support group which he attends once a month. The members of the support group include those who are on the waiting list and those who have had successful lung transplants.
"The transplant process is a long haul. First you have to find a suitable set of lungs and then there’s the whole procedure itself. There are a lot of steps and a limited supply of donor lungs," explains McFadyen. "There’s always a glimmer of hope, but at this stage in my life I am a realist."
McFadyen says increasing awareness of IPF is critically important. He is hopeful that sharing his story will help spur more research into the causes and treatments of IPF – and eventually a cure.
Sadly, Ralph MacFayden passed away on May 16, 2012 of IPF. His wife Leyah hopes that by sharing his story, it will help to raise the profile of IPF and inspire further research.
Robert Alexander Carew's story
It has been almost a year since Robert Alexander Carew, 39, first learned he has idiopathic pulmonary fibrosis – a progressive disease that causes scarring of the lungs and makes it difficult to breathe. Carew first suspected something was wrong when he felt breathless after climbing stairs. "I was very fit – I used to take the stairs two at a time – but I noticed that I was out of breath after just a few stairs."
Carew also felt tired and found his usual exercise regime of spinning classes and workouts at the gym increasingly difficult. He made an appointment with his doctor who ordered a chest x-ray. The results were shocking – the x-ray showed signs of pulmonary fibrosis. "I just slumped down into the chair," recalls Carew. Further tests confirmed the doctor’s suspicions. Carew’s father had been diagnosed with idiopathic pulmonary fibrosis some 20 years before so he had firsthand knowledge of what he was facing.
Despite his sobering diagnosis and the inevitable challenges that lie ahead, Carew maintains a positive outlook. Following his diagnosis, Carew immediately began doing research to better understand the disease, its causes and his prognosis. Arming himself with information and talking to others with the disease helped him cope. "I’m a proactive person by nature," he explains. Carew is also an active member of an online IPF community where he shares information and offers support to others with chronic lung disease.
"As it turns out I’m not your typical IPF patient. I don’t fit the profile – I’m young, otherwise healthy, don’t have any occupational risk factors – it was my genetic link that somehow predisposed me to this disease."
Carew is optimistic about his prognosis. He has explored the possibility of a lung transplant – an option that will become more likely as his lung capacity decreases. So far he is tolerating his medications well with minimal side effects and doesn’t have any other health conditions. "Age is definitely on my side," he says.
Carew uses oxygen to complete strenuous activities like chores or exercise. He says he will likely need to use oxygen full-time in a few months as his breathing becomes more difficult. Still, Carew is continuing to lead a full and active life that is typical of many men his age. He is busy building his career at a computer software company, while juggling the demands of raising two young daughters with his wife. IPF has taught him to live in the moment. "Life goes on," he says.
"IPF is definitely not good news," he says. "But as time goes on you realize it’s not as bad as you think it is."
Val Friesen's story, patient - Winnipeg, Manitoba
As I think back, my first encounter with a breathing problem occurred in 2003 while doing yard work. I had a strange feeling in my chest as I emptied the wheelbarrow full of soil and felt too tired to continue. In 2005 my left lung collapsed and a 10th of it had to be removed. I was told I had IPF, originally diagnosed in 2003, but not told to me.
I’m now in my 8th year, going on 9th, since my diagnosis. I have done better than most with this disease. I was put on continuous oxygen 7 months ago.
We purchased a candy apple red scooter to match our candy apple red retirement CUV as I am not able to walk long distances. We also installed an electric chair lift in our 2 storey home.
My husband, Bob, has been wonderful and has added the word chef to his resume. A very good one, I may add.
IPF has restricted our travel but we have gone, by car, to visit our son’s family complete with 3 beautiful grandchildren, in Ontario.
As a caregiver, I pretty much need to be around most of the time. I attend with Val, at her appointments with the transplant assessment team and support groups. It’s important to understand what’s going on. At the initial diagnosis, there was considerable apprehension and negative feelings. But once fully informed, and past the disbelief phase, knowledge becomes very helpful. The IPF support group has been wonderful.
Care giving means to be there to provide aid in whatever Val cannot readily do alone. For example, I manage her oxygen tanks, making sure they are full, ready, and stored properly in the car when we travel. We took a car trip last Autumn through Northern Ontario to Stratford. There were a few worrisome areas without cell phone coverage, but we made it okay. We have a GPS in the car and that’s very helpful in knowing where to next, and how far to go, at all times, in case of emergency.
Share your story with us!
Tell us how IPF has touched your life – whether you are a patient, caregiver, family member or health-care professional. Send us your story and photo to email@example.com.
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This initiative was made possible by an educational grant from InterMune.