Friday, November 09, 2012

Pulmonary fibrosis remains largely under the radar

This article has special meaning for me because I was diagnosed with Idiopathic Pulmonary Fibrosis 13 years ago and told I had about 2 years left to live and to get my affairs in order. After the initial blow of hearing this news I was very fortunate to get on a waiting list for a transplant and thanks to a generous donor and donor family I received a lung transplant 10 years ago. Treatment of IPF hasn't changed since that time.

MONTREAL - Retired Montreal police officer Jean-Yves Trudeau has already lost his sister to the disease that now threatens his own life.

Trudeau has idiopathic pulmonary fibrosis — a progressive and ultimately fatal lung disease with no known cause or cure.

“Will it be my turn next? I’m not going to cry in a corner over my lot. My philosophy is: ‘One day at a time,’ ” said Trudeau, 69. He speaks in measured tones to conserve his breath. Common daily tasks such as climbing a few steps or even walking to the mailbox make him stop to catch his breath.

Despite ambassadors such as Ottawa resident Hélène Campbell, a high-profile double-transplant recipient, the disease continues to fly largely under the radar. Campbell, 21, received a transplant in April after gaining huge public attention through a social-media campaign that picked up celebrity followers such as Justin Bieber and Ellen DeGeneres.

Trudeau consulted a lung specialist because his family doctor in Saint-Hyacinthe didn’t like the sound of his chronic coughing, recurring pneumonia and shortness of breath. His diagnosis, in the summer of 2010, came after months of testing that included lung imaging and a biopsy.

“It was like a blow the head,” Trudeau recalled this week at the lung testing lab at Notre-Dame Hospital of the Centre hospitalier de l’Université de Montréal, a tertiary referral centre. “My little sister (Carmen) was diagnosed the same week, but in a different hospital. In the end, she was on oxygen 24 hours a day” and she died in July, less than two years after diagnosis.

The condition affects adults mostly age 50 years older. It is a degenerative disease in which lung tissue becomes thick and scarred over time. The excessive scarring is called fibrosis and it worsens until most patients struggle to breathe; many need extra oxygen or lung transplants, and many die prematurely from the disease.

Some 30,000 Canadians suffer from some kind of pulmonary fibrosis, a family of diseases associated with about 140 known causes. Many of them are environmental hazards — for example, those who work in mines or with asbestos.

But the sub-group called idiopathic pulmonary fibrosis, which is what Trudeau suffers from, is considered the worst, said pulmonary specialist Hélène Manganas of the Centre hospitalier de l’Université de Montréal.
Studies suggest that 5,000 to 9,000 Canadians suffer from IPF, with an estimated 3,000 deaths each year.
It is a horrible disease whose causes continue to stump researchers, Manganas said.

“IPF is extremely limiting with lower life expectancy than many forms of cancer,” Manganas added. “It’s an aggressive illness. The median survival rate is three to five years from diagnosis.”

According to statistics, the disease seems to be increasing independently of the aging of the population, she said.

Many sufferers are provided with home oxygen but, eventually, they need lung transplants to survive, she said.

“Unfortunately, not every patient is a good candidate for a lung transplant. Some are too old or have other medical problems,” she said, and then there are wait-lists. “Sometimes the condition progresses so quickly that we don’t have time to do a transplant.”

Current treatment is limited to teaching patients to breathe more efficiently, she said, but the field is exploding with researchers looking for new therapies to slow the disease and reduce inflammation. In the meantime, more education is needed, especially of community health workers who accompany such patients at home, she added.

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