Living with a rare disease

Jeff Green | Mar 06, 2014

On February 28, the day of the Olympic semi-final game with the United States, many of us had one ear, or both eyes, on the fate of the Canadian men’s hockey team.

February 28 is also Rare Disease Day in Canada and to mark the day, Jacqui Bowick, who suffers from Pulmonary Fibrosis (known as IPF Idiopathic Pulmonary Fibrosis) marked the day by presenting a petition to MPP Randy Hillier in his Perth office.

The petition, which had 650 signatures gathered mostly in Smiths Falls where she lives, asks the provincial government to add a drug, pirfenidone, to the Ontario Drug Plan. Pirfenidone has been approved for use in Canada as a treatment for mild to moderate IPF, but at a cost of $42,000 per year it is unaffordable for many IPF patients.

Jacqui Bowick took a two-week course of pirfenidone, but she had an allergic reaction to it, so it is not an answer for her, but nonetheless she has continued to advocate for it to be covered by the Ontario Drug Plan.

“A lot of other people can be helped by it, but at that cost it is out of reach for so many sufferers,” she said, when interviewed over the phone after presenting the petition.

Jacqui Bowick, who is now 49, did not always suffer from IPF.

She worked as a nurse's assistant and in a pharmacy in the 1980s and 90s. Then she went back to school and eventually landed what she calls her “dream job”, working in the administration office of the Ottawa-Carleton School Board.

A few years ago she developed breathing problems, and in 2010 she was mis-diagnosed, twice. At first, doctors thought she had bronchitis and then they thought she had asthma but she did not respond to treatment.

“Because rare diseases are so uncommon the system is not set up to detect them. They naturally look for the common diseases first, and until I got a diagnosis I did not know what was wrong,” she said.

Since then she has been learning a lot about how to live with her condition, which is not easy to treat and does not have a good long-term prognosis.

The website Pulmonaryfibrosis.org describes it as a condition in “which the lung tissue becomes thickened, stiff, and scarred. The medical terminology used to describe this scar tissue is fibrosis. The alveoli (air sacs) and the blood vessels within the lungs are responsible for delivering oxygen to the body, including the brain, heart, and other organs. All of the body’s functions depend upon delivery of a steady supply of oxygen. As lung tissue becomes scarred and thicker, it is more difficult for the lungs to transfer oxygen into the bloodstream. As a result, the brain, heart, and other organs do not get the oxygen they need to function properly.”

Because there is no known cause for the condition doctors call it Idiopathic Pulmonary Fibrosis (IPF)

Although treatments range from using steroids and other drugs, including pirfenidone, lung transplants give the most success in prolonging the lives of those with the condition.

Jacqui Bowick has been able to use the swimming pool in Smiths Falls where she does a water aerobics class with the aid of an oxygen machine that they have allowed into the pool area for her, and she travels weekly to Ottawa to participate in a pulmonary rehab program that is designed for patients with Chronic Obstructive Pulmonary Disorder (COPD), which has been helpful to her.

In May she might be travelling to Toronto to undergo a battery of tests in order to try and get on the lung transplant list.

“The problem is that sometimes you are not sick enough to get on the list and then you can be too sick or can have to wait too long, so it is difficult,” she said.

Jacqui Bowick says that she used to have an "A" type personality, but IPF has taken that away from her. She has, however, become an “A” type active advocate for those with the condition. In addition to participating in pubic functions and delivering the petition to MPP Hillier, she also keeps a blog about the trials and successes of people facing the daunting prospect of fighting for every breath, which comes with IPF. The blog is at This email address is being protected from spambots. You need JavaScript enabled to view it..