Last updated on April 23, 2014 at 11:04 EDT

Canadians with rare, fatal lung disease dissatisfied with poor standard of care: new survey

September 12, 2012

Increased awareness and education will help give IPF patients a fighting

TORONTO, Sept. 12, 2012 /CNW/ – According to a new patient survey, what
you don’t know can hurt you when it comes to living with a rare, fatal lung disease. The
first survey of idiopathic pulmonary fibrosis (IPF) patients in Canada,
An Investigation into Patient Experiences with IPF reveals that a lack of knowledge among patients, their caregivers and
primary care providers hinders the diagnosis of the disease, access to
specialized care and the availability of safe and effective treatment

“Given that IPF is rapidly fatal, even one day waiting to see a doctor
in order to be diagnosed and referred to the proper specialist, or to
get access to effective treatment, is one day too many,” said Robert
Davidson, President of the Canadian Pulmonary Fibrosis Foundation
(CPFF). “The Canadian IPF community has spoken through this survey – we
don’t accept the current paradigm, and we know what needs to be done to
give us a fighting chance at an improved quality of life and life

The release of the survey results this September coincides with the
first IPF Awareness Month in Canada. The month-long commemoration was
established to increase awareness and understanding of this
progressive, debilitating and life-limiting disease that has no known
cause. IPF is characterized mainly by honeycomb-patterned scarring, or
fibrosis, in the lungs. This scarring hinders the exchange of oxygen
and carbon dioxide, making it increasingly difficult for patients to

Patients frustrated by delayed diagnosis and access to specialized care

According to An Investigation into Patient Experiences with IPF, greater awareness among patients, caregivers and the primary care
providers of IPF and its symptoms is needed to hasten diagnosis of this
disease. On average, patients wait almost 20 months from when they
first begin to experience symptoms to receive a confirmed diagnosis of
IPF, and unfortunately only 32 per cent of patients receive IPF as
their first diagnosis.

As indicated in the survey, patients without access to IPF experts in
specialized care clinics across the country are dissatisfied with the
current level of care they receive. One third of patients (33 per cent)
who are not managed by experts in IPF clinics report being dissatisfied
with their level of care, compared with 67 per cent of patients who are
extremely satisfied under the care of these experts.

“I am all too aware of the horrible prognosis associated with IPF and
being diagnosed with it was terrifying. My father succumbed to the
disease in March 1990 and it took his life in just 11 months,” said Rob
Alexander-Carew, 39, a double lung transplant recipient who lives in
Oakville, ON. “I am very thankful to be one of the few lucky ones. My
specialists are very knowledgeable about IPF and I am fortunate to be
receiving the best available care, which means I have more precious
time with my wife and my two children.”

Few, ineffective treatment options top patient needs

New, safe and effective treatment options for IPF are needed to respond
to low patient satisfaction levels with currently available therapies.
According to the survey, IPF patients are dissatisfied with the
efficacy of available IPF treatments, underscoring the need for new,
more effective treatment options. Thirty six per cent of IPF patients
discontinued treatment because their doctor said it was no longer
effective and 27 per cent of patients report they have never received
treatment for their IPF.

“There is a significant unmet medical need for people living with
idiopathic pulmonary fibrosis,” said Dr. Charles Chan, IPF researcher,
consultant respirologist at the University Health Network and professor
and vice-chair of Medicine at the University of Toronto. “IPF is
extremely life-limiting with lower life expectancy than many forms of
cancer, yet sadly, there are no therapies to treat IPF currently
approved in Canada.  Clearly there is a need for new therapies in this
disease area.”

An Investigation into Patient Experiences with IPF was conducted by Doxapharma and surveyed 63 Canadians with IPF about
their care and quality of life between March and July 2012. Please
visit www.cpff.ca for more information, or to locate an IPF disease clinic near you.

About IPF in Canada

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with
no known cause. Studies suggest that 5,000 to 9,000 Canadians suffer
from IPF, with an estimated 3,000 deaths each year. IPF is more common
in men than women and is usually diagnosed between the ages of 40 and
80 years. In patients with IPF, the lung tissue becomes scarred and
over time, as the scarring becomes thicker and more widespread, the
lungs lose their ability to transfer oxygen into the bloodstream.  As a
result, patients become short of breath and the brain and vital organs
are deprived of the oxygen necessary for survival. Unfortunately, there
is currently no cure and very limited treatment options for people with

About the CPFF

The Canadian Pulmonary Fibrosis Foundation (CPFF) is a registered
foundation and not-for-profit charitable organization established to
provide hope and support for people affected by pulmonary fibrosis. The
CPFF aims to raise funds to finance research to better understand,
develop treatment and find a cure for pulmonary fibrosis; raise public
awareness about the fatal lung disease; and offer support to those
affected by it. For more information about the CPFF, please visit www.cpff.ca.

About InterMune

InterMune is a biotechnology company focused on the research,
development and commercialization of innovative therapies in
respirology and fibrotic diseases.    InterMune’s research programs are
focused on the discovery of targeted, small-molecule therapeutics to
treat and monitor serious pulmonary and fibrotic diseases, such as
IPF.  For additional information, please visit www.intermune.com.

SOURCE Canadian Pulmonary Fibrosis Foundation

Source: PR Newswire