“Fasten your seatbelts – IPF is a long and bumpy road.” Barbara Barr was diagnosed with Idiopathic Pulmonary Fibrosis (IPF) in 2010. She helps others through the disease by sharing her story and advocating for IPF patients. (Photo: UHN News)
"Easy or not, we have to accept that in being born we are going to die.
Our clocks may be ticking louder and faster now than we expected, and our disease is most likely going to be the cause of our death, but just like life pre-diagnosis, we just don't know.
Our doctors weren't given a crystal ball and I haven't bothered wasting time finding one." –Barbara Barr
Barbara Barr has Idiopathic Pulmonary Fibrosis (IPF) – it's a disease that an estimated 5,000 to 10,000 Canadians suffer from, says Dr. Shane Shapera, respirologist at TGH. IPF is a disease of scarring in the lungs, which gets worse over time. There is no known cause. The most effective 'cure' currently -- is a lung transplant.
Barr has been a partner in her care at TGH – and takes pride in knowing her disease inside and out. She spends her time helping others in similar situations. This week is IPF World Week and we asked Barr about her experience with IPF.
How were you diagnosed with IPF?
My mother died of lung cancer and my older brother of emphysema.
Being on high alert for lung disease, when I experienced my first "chest" cold in 2005 with a persistent cough that lingered for several months accompanied by shortness of breath (SOB) I insisted and my primary care physician (PCP) agreed that we needed to investigate.
I underwent a complete and thorough cardiovascular workup and also had my pulmonary function tests (PFTs) done.
The respirologist continued to suspect seasonal asthma.
Another chest cold in 2008 led to another visit to the respirologist and a repeat of my PFTs.
While the pulmonary function had declined the respirologist's only answer was, "You have sensitive lungs."
Then late 2010 the rug was finally pulled out from under my feet.
Another chest cold, I was almost unable to walk the 10 feet to the bathroom where I proceeded to attempt to cough my lungs out, told me that I was in serious trouble that this was not just "sensitive lungs."
My initial diagnosis was Interstitial Lung Disease (ILD) which indicated serious lung damage in the peripheral area of my lower lobes showing scarring.
When discussing next steps the doctor recommended I go back to see the respirologist, I nixed that idea immediately.
I knew that UHN, just 30 minutes away from me, had one of the premier lung transplant centres in the world at the Toronto General Hospital (TGH) facility
AND that there was something called an ILD clinic at TGH.
I got myself referred there immediately!
What resources helped you understand IPF?
The internet has been my greatest source.
Please note the information out there is not perfect and finding valid
and current information has been a struggle.
But, the last couple of years, and in particular the last year, have seen an explosion of the good information that not only patients and caregivers need but from which the medical and health care community can benefit.
We are bringing two of the largest stakeholders closer together by opening up the communication channels.
I am working closely with the Canadian Pulmonary Fibrosis Foundation (CPFF) to not only provide the information that is initially needed but to also point visitors to other important information elsewhere.
In your experience, why has it been important for you to be a partner in your care?
IPF like other forms of pulmonary fibrosis is a disease of symptoms that need to be managed if we are to maintain our quality of life.
Understanding that there is no "silver bullet" out there, that there is no single prescription, and that it is a combination of things/actions that can be brought to bear in disease management is an important message to get out there.
We need to be our own medical advocates. We need to accept responsibility for our own medical team.
Doctors have long recognized the positive impact of patient-driven disease management but I think if other patients are spreading the same message, we may find more fertile ground.
Why did you become an advocate for IPF awareness?
Knowledge is power.
Understanding my disease changes how I approach it and manage it.
Not everyone is able to or willing to become as obsessed with their disease and take an informal doctorate degree in IPF.
I want to help others to find the information they need and also to show that obsession can be good – I choose to use it in a positive direction.
Helping others in a positive way also helps me manage my disease significantly adding to my personal feeling of control over my disease.
What advice do you give to others who are diagnosed with IPF?
I usually tell people that it is likely to be a long and bumpy road so fasten your seatbelts.
The diagnosis is fluid and dynamic and often just as frustrating to our ILD/PF specialists but as time goes by, more information becomes available.
My biggest single advice is when you hear the word "pulmonary fibrosis," get to an ILD/PF specialist or clinic.
A respirologist is a lung doctor but you want to see the lung doctors who are also experts in ILD/PF.
TGH's ILD clinic is staffed by experts in ILD and that is especially true for IPF – only one of about 200+ different types of ILD can cause scarring in the lungs.
Disease and treatment news often takes time to trickle down and be adapted and ILF/PF specialists are closest to what is happening.
In addition, these specialists are more experienced at taking out the noise of the early symptoms that are common to many diseases and conditions and to see or find the information that leads to the correct diagnosis.
What do you want the public to know about IPF?
If you have a loved one or friend who has been diagnosed with IPF, I think one of the best things you can do for them is to become educated about the disease and the management of the disease.
With a rare disease such as IPF, we often don't have an opportunity to interact with other patients or caregivers.
Being able to talk with our family and loved ones with knowledge and understanding, I believe, can greatly help to alleviate the isolation and hopelessness that many suffer.
Help us grow our support group.
Most of all, we were first and foremost people and while we may need some accommodations throughout our disease, we still want to be treated with dignity and respect and feel that we still valuable members of society.
IPF is a horrible disease.
The disease affects patients, caregivers, and other loved ones and the medical/healthcare community.
But there is still HOPE.
They say it takes a village to raise a child.
Well it takes a community to deal with this disease!
The future of IPF
Sharing her story is just one way patients like Barr is helping others live with the disease.
Barr works with patient groups such as the Canadian Pulmonary Fibrosis Foundation to ensure that patients and caregivers have a voice and are able to provoke change and raise awareness.
"At some point, you have to accept that you're not going to move that huge rubber tree plant – so go find a smaller plant to push," she says. "The important thing is to keep pushing in a forward, positive direction because a healthy, positive attitude while fighting this disease has a huge impact."
To learn more about IPF please visit
www.cpff.ca.
