Pulmonary Arterial Hypertension: The Rare Progressive Disease That’s Often Misdiagnosed

For many Canadians, it’s difficult to identify PH and begin treatment before it’s too late.

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Allison Wells, a 32-year-old substitute teacher from Newfoundland first realized something was wrong when she was decorating her Christmas tree in 2016. Every time she stood up after fluffing the branches, she felt lightheaded and dizzy. Though she thought it was strange, it was also winter and she hadn’t been getting much exercise. “I thought I was out of shape,” she remembers.

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But after months of getting winded after climbing a single flight of stairs and feeling her pulse throughout her whole body when she’d lie down in bed, she finally decided to book an appointment with her doctor in the spring of 2017.

“I had shortness of breath, an elevated heart rate and I had a cough,” she says. “First, my family doctor tested me for allergies. Then he thought maybe it was my kidney, so I had to carry around a huge bottle to pee in for a week. Nothing showed up.”

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Allison began doing her own research online and speaking with family and friends. “My mom thought I needed a chest X-ray,” she says, “because I could only talk to her on the phone for a couple minutes and I would be out of breath with a dry cough.” Though her doctor was reluctant to provide the requisition because he thought it was unnecessary, he eventually did. But before Allison could book an appointment, she passed out at work in front of a classroom of grade nine students.

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Allison went to emergency and ended up staying in the hospital for an entire month, where several tests including a chest X-ray and echocardiogram revealed that her heart was enlarged to twice the normal size due to extreme pressure. She was diagnosed with a rare, progressive disease called pulmonary arterial hypertension (PAH), a form of pulmonary hypertension (PH).

What is pulmonary hypertension (PH)?

There are five types of pulmonary hypertension (including PAH) that are named for their causes. Each one creates high blood pressure in the arteries of the lungs and can weaken and enlarge the heart. Though people living with the disease often don’t “look” ill, they may have symptoms such as shortness of breath, fatigue, dizziness, fainting, chest pain, swollen legs and ankles as well as bluish lips, hands and feet. Since PH is a progressive disease, symptoms get worse over time. Without treatment, the average life expectancy is less than three years. However, the average life expectancy of PAH patients exceeding seven years has been associated with improvements in treatments and care in recent years.

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How many people does PH affect?

Around 5,000 Canadians have been diagnosed with PH, but as many as 10,000 may be affected. That means that half of Canadians living with the condition are undiagnosed or misdiagnosed.

Why is PH so hard to identify?

While there are centres and experts across Canada that specialize in PH, most family doctors aren’t very familiar with the disease and its symptoms and will often diagnosis more common conditions like Chronic Obstructive Pulmonary Disease (COPD), asthma or bronchitis. Many patients will spend two- or three-years seeking answers. Once diagnosed however, many patients are living longer, healthier lives now thanks to available medications.

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How do you treat PH?

For Allison, current treatment involves three oral medications as well as additional medications to mitigate side effects like heartburn and low potassium. While there is no cure, Allison can live a more “normal” life and her symptoms aren’t as severe as they were before her diagnosis.

What if you think you may have PH?

“Listen to your body,” says Allison, “even if a doctor or doctors say there’s nothing wrong. Find another doctor or ask to see a specialist.” She also recommends educating yourself through online research and speaking to others who have the same condition. That way you can push for testing and know what questions to ask your doctor.

If you are diagnosed with PH (whether it’s PAH like Allison, or another type), she recommends finding a support group. “Mine’s online, so I’m connected to people across the country who have this condition in various forms,” she says. “I’ve gained a lot of knowledge from hearing all these people’s stories. If you have a condition, find others you can relate to and learn from and vent to.” It’s also an informative -way to learn about the latest research about this rare disease.

For more information on pulmonary hypertension, visit phacanada.ca.

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