By Lisa Tallyn, Independent & Free Press | Feb 10, 2011
Carol Doyle was like the majority of Canadians who had never heard of pulmonary hypertension (PH) until she was diagnosed two years ago with the rare incurable lung disease that results in high blood pressure in the lungs.
Unknown to her at the time, Doyle, 41, of Georgetown had been suffering symptoms of the disease for years before she was finally diagnosed.
“Over a period of four years, I went to my doctor and specialist countless times, but no one could figure out what was wrong,” said Doyle. “When I was finally diagnosed my PH had significantly progressed. Had I received a prompt diagnosis, I could have been on the treatments I needed right away and managed my disease better.”
The first symptoms she had, not knowing they were related to PH, started a few years back when she began experiencing blackouts.
“I would run upstairs and just black out,” said Doyle. “I just fell wherever I was.”
She was diagnosed with postural syncope, which occurs when a person’s blood pressure drops due to a quick change in position.
A couple of years later she began suffering periods of severe bloating, and was prescribed diuretics.
In the sixth month of her pregnancy with her daughter a couple of years ago she started retaining fluid again, her feet and ankles became very swollen, and she was diagnosed with toxemia.
The fluid buildup continued after her daughter was born, and it was so bad that she was having difficulty walking.
She said the fluid continued to build up in her legs. She was in so much pain she went to Emergency, where she said the doctor told her, “it was part of motherhood and I should just learn how to deal with it.”
The pain and swelling in her legs got progressively worse and due to exhaustion she was only able to climb the stairs in their home once a day.
Finally she was referred to Dr. Craig Browning in Georgetown, who immediately referred her to a cardiologist because he said there was a pulmonary issue.
“He was awesome,” said Doyle.
The cardiologist diagnosed pulmonary hypertension. Doyle was told she needed a lung transplant, and if she didn’t get one she wouldn’t live past two years. She and her husband were devastated by the news.
Doyle was referred to a specialist at Toronto General Hospital who put her on heavy diuretics and did more testing.
“I lost 36 pounds the first week— seven pounds the first night,” said Doyle. She felt significantly better with that fluid off.
“At least I could walk up and down the stairs,” said Doyle.
At Toronto General she learned that lung transplantation is often the last treatment option, and was started on medications that increased blood flow and diuretics.
Since 1997 Health Canada has approved several new effective medications to treat PH patients.
With treatment, Doyle was able to go back to work, but she could only manage it for about a year, and has been off since then.
“It’s a big adjustment. I love spending the time with the kids,” said Doyle, who has to visit the clinic at Toronto General regularly for follow-up.
She has limitations now. She’s only allowed 1,500 ml of fluid a day and has to closely watch her sodium intake.
Symptoms of the disease are difficulty breathing, fatigue, chest pain and faintness.
Doyle says “it’s been a rough road.”
“It gets difficult. My husband has been awesome,” she said.
She added she also has an “awesome network of neighbours” who help her when needed.
“It’s progressive, there is no cure, it will eventually get worse. But you have to live in the here and now, and you really appreciate life.”
She credits her daughter with saving her life, because the diagnosis came following her pregnancy. She’s also very grateful to Dr. Browning.
The disease affects up to 210,000 Canadians and according to a recent survey only one in five Canadians associated swollen ankles, legs or abdomen with symptoms of pulmonary hypertension. It has a very low profile, even among medical professionals, and as a result is often misdiagnosed or confused with a host of other illnesses.
Early symptoms are subtle and include unexplained shortness of breath, fatigue, swelling of the feet and ankles and fainting.
“Early detection and prompt treatment can significantly improve patients’ symptoms, quality of life and long-term outcomes,” said Dr. Sanjay Mehta, professor of medicine and Director for the Southwest Ontario PH Clinic in London, Ontario and Medical Director of PHA Canada.
Mehta said they are trying to increase awareness and education “for this invisible, incurable, but treatable disease so that we can offer patients the best outcomes possible as we continue to work towards a cure.”
The several treatment options available means pulmonary hypertension patients are living longer and healthier lives.
One such advancement is the Ontario Ministry of Health and Long Term Care’s recent decision to revise existing Exceptional Access Program (EAP) criteria to include funding of combination therapy for people living with pulmonary arterial hypertension.
“This decision is an important step for PH patients in this province. PHA Canada looks forward to continuing to work with governments across Canada to ensure that all PH patients have access to the best quality of care,” said Darren Bell, president, PHA Canada.
Innovative new therapies that are under investigation in clinical trials and may soon be available to patients. They include a new generation of oral and inhaled drugs that are easier to take and more convenient for patients.
For more information on pulmonary hypertension go to www.phacanada.ca or www.livingwithph.ca.