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

Increased awareness and education will help give IPF patients a fighting chance

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 options.

"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 itself."

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 breathe.

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 IPF.

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