By Navneet | 18 April 2012
On a snowy February morning, Helen Britt swings open the festively painted door of her suburban bungalow home calling on her guest to park closer and step carefully.
Her gentle smile and cheery blue green eyes at first mask the clear plastic tubing lining her nose and upper lip. Under a soft light gently lining the furrows of her aged skin, Helen tells of the day those long omnipresent tubes became a permanent part of her life “The doctor looked at me and my lips were so blue, he said you have to go to emergency right away […] and from then on I have been on oxygen”
This harrowing day came more than a year after Helen first noticed she was losing energy and experiencing increased shortness of breath. Helen was diagnosed with asthma, when her symptoms worsened Helen returned to her respirologist and was immediately written off “She said “You’re just not fit!””
Helen had always worked out, exercising on a treadmill, golfing and curling; nevertheless she took the respirologist’s advice and intensified her workouts.
At the emergency room, Helen was diagnosed with mild pneumonia and sent home with an oxygen tank, when her oxygen levels failed to return to normal after a month, her respirologist ordered a battery of tests which finally lead to the diagnosis of pulmonary hypertension.
Pulmonary hypertension occurs when tiny blood vessels in the lungs constrict, causing blood pressure in the pulmonary artery to rise; the right side of the heart in forced to work harder to oxygenate blood.
According to the US National Library of Health, pulmonary hypertension especially in its early stages can take months to diagnose because the symptoms mimic many common disorders such as asthma. Early symptoms of PH include:
shortness of breath upon exertion
Ruth Dolan co-director of the Toronto chapter of The Canadian Pulmonary Hypertension Association recalls her young daughter’s ordeal “She started getting short of breath when she was 9 years old whenever she would run and play with her friends, or even when we went for walks in the evening. She would lag behind trying to catch her breath”
Ruth Dolan’s daughter would be properly diagnosed more than a decade later, by then her condition deteriorated significantly.
The only means to definitively diagnose pulmonary hypertension is an invasive test, which is typically done under conscious sedation called cardiac catherization; this involves stringing a long lube through an artery in the arm, neck, or groin to the heart to measure pressure in the pulmonary artery.
The rarity of pulmonary hypertension means patients not only faced delayed diagnosis because of lack of awareness regarding their condition, but bureaucratic denial of effective treatment. Treatment of pulmonary hypertension often requires more than one drug to dilate blood vessels in the lungs (flolan, revatio).
In 2009 the Ontario government stopped funding combination therapy, restricting PH patients to only one vasodilator; the justification given for this decision was inadequate sample size in clinical trials showing combination therapy to be more effective than use of a single drug. Most PH families could not afford the vasodilators alone; the cost of intravenous flolan for one patient is about $100,000 annually and the cost of revatio is nearly $20,000.
Dolan says that after the Canadian Pulmonary Hypertension Association engaged in a lengthy arduous battle with Queen’s Park the Ontario Government revised the decision and now funds combination therapy under its Exceptional Access Program.