Pulmonary hypertension a rare disease that causes high blood pressure in the lungs
BY MARILYN LINTON | JANUARY 02, 2012
“I don’t look sick, but you don’t know what goes on inside my body,” says Carol Doyle. The 42 year-old Georgetown, Ont., mom knew something was not right when, in 2004, just after her son was born, she retained fluid and occasionally blacked out.
“I could just pass out,” she says, recalling how doctors found nothing significant – even after another pregnancy during which she had similar symptoms, including serious fluid retention.
“After my daughter was born, they again said it would go away. But it didn’t and I could barely walk. If you pressed your finger into my leg it was like a kind of memory foam,” Doyle explains. Fortunately, her family doctor, realizing her symptoms had nothing to do with pregnancy, sent her to an internist.
Results of further tests confirmed pulmonary hypertension or PH, a condition rare enough to be termed an “orphan” disease. “Like many uncommon diseases, we struggle with awareness,” says Dr. John Granton, director of the Pulmonary Hypertension Program at the University Health Network’s Toronto General Hospital. “We find that nobody has heard of PH and the people who have it often look very well.”
Say the word “hypertension” and people think of high blood pressure, but with PH your blood pressure can be normal. “It’s an internal pressure problem that lies within the circulation of the lung,” says Granton, explaining how blood is pumped through the heart’s chambers and vessels. “In PH the tiny blood vessels in the lung become progressively blocked or constricted, so less blood goes through and the flow becomes blocked.”
Think of a garden hose that is kinked: The pressure in the hose goes up and the hose becomes dilated and distended, and the pipes back up and leak into your basement – the flow through those pipes being reduced because of the kinking. To diagnose the condition, doctors rely on an ultrasound of the heart to see the size of the chambers; they also employ heart catheterization to determine how well the heart is pumping blood to the body.
Estimates are that 10,000 Canadians live with PH – many of them undertreated or misdiagnosed. It’s not unusual for people with PH to be mislabelled as asthmatic, says Dr. Granton. In a busy medical practice, it’s appropriate to think of common things like asthma, first. But if the condition doesn’t respond to, say, puffers, and the patient isn’t getting better, a good clinician needs to ask questions and do further tests.
“PH looks like a lot of things,” he says, adding that a racing heart and fainting can occur with other conditions. “A lot of things also make you feel tired and out of breath. But if your legs are starting to swell, if you’ve a history of blood clots, if you have other immune conditions, these are all risk factors.” Breathlessness is one symptom, but the doctor cautions that people with PH find themselves breathless just walking on level ground – never mind running up the subway stairs.
Years ago, people with PH were told they would die within a few years without treatment. While a lung transplant is still the gold standard, many patients with PH do well even a decade after diagnosis thanks to advances in medication which aim to relax the circulation and improve blood flow through the lungs.
Carol Doyle risked serious heart failure before she was able to get her PH under control. Now that she has stabilized she wants people to know that as rare as the disorder is, it’s important to recognize its signs. “I had never heard of PH, and I now know it affects everyone differently,” says Doyle, now a spokesperson for the Pulmonary Hypertension Association of Canada. “When I had all those symptoms and no diagnosis, I thought people would be thinking I was crazy. But I knew something was wrong with me.”
It’s a rare but potentially fatal lung disease in which the arteries of the lungs become closed off or scarred, resulting in high blood pressure in the lungs. This is different from normal hypertension or high blood pressure. Up to 5,000 Canadians have been diagnosed with PH, and as many more are undiagnosed. For more, see www.phatoronto.ca.
More than breathless
Symptoms of PH include breathlessness or shortness of breath, especially with activity; feeling tired all the time; feeling lightheaded, especially when climbing stairs or standing up; swollen ankles, legs or abdomen; fainting; chest pain
What causes PH?
In PH, the right side of the heart works harder than normal. It may be caused by autoimmune diseases, congestive heart failure, heart valve disease, chronic low oxygen levels in the blood, diet drugs, sleep apnea or lung disease such as COPD.