diagnosed with pulmonary hypertension: guide to getting care in canada
according to the pulmonary hypertension association of canada, 5,000 canadians have been diagnosed with pulmonary hypertension but as many as 10,000 people have it and don’t know it.
7 minute read
without treatment, the average life expectancy of someone living with pulmonary hypertension is less than three years, depending on the severity.
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there are a lot of misconceptions when people hear the diagnosis pulmonary hypertension, says andrea gardner, nurse and research coordinator at the pulmonary hypertension program st. joseph’s hospital in hamilton, ontario.
“they hear ‘hypertension’ and they recognize that word because almost everybody has hypertension or high blood pressure. so, we spend a lot of time differentiating that this is not a benign high blood pressure where we have dozens of pills that we can try to treat and manage it and you go on your way. this is a very different disease that will progress over time.”
pulmonary hypertension is a lung disease that happens when blood vessels in the lungs become narrowed, thickened, blocked or destroyed, making it harder for the right side of the heart to pump blood and blood to flow through the lungs. as a result, blood pressure increases in the lungs. while systemic hypertension can damage blood vessels and organs over time, pulmonary hypertension specifically refers to high blood pressure between the heart and lungs. the condition can weaken your heart, leading to symptoms like shortness of breath, feeling lightheaded or swelling in the legs or abdomen. the trouble is these symptoms can be mistaken for other conditions. and without treatment, the progressive condition can cause heart failure, dangerous blood clots, and shortened life expectancy.
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here are steps to take to get the best care and management of pulmonary hypertension:
learn more about types of pulmonary hypertension
according to the pulmonary hypertension association of canada, 5,000 canadians have been diagnosed with pulmonary hypertension but as many as 10,000 people have it and don’t know it. many people spend two to three years trying to get an accurate diagnosis. without treatment, the average life expectancy of someone living with pulmonary hypertension is less than three years, depending on the severity. another complication is that pulmonary hypertension can be invisible. people who have it can look as though they are completely well and healthy. the disease can strike anyone regardless of age, sex or ethnic background. it’s more common among women, non-hispanic black people, and people aged 75 or older.
pulmonary hypertension is categorized into five groups, which is important for treatment and management:
- group i pulmonary arterial hypertension that has a variety of causes, like autoimmune diseases, congenital heart disease, sickle cell anemia, inherited or no known cause (idiopathic pulmonary arterial hypertension
- group ii pulmonary hypertension because of left heart disease or long-term systemic high blood pressur
- group iii pulmonary hypertension related to lung and breathing conditions like chronic obstructive pulmonary disease, sleep apnea, pulmonary fibrosis that can cause lung tissue scarring, or hypoxia (low levels of oxygen in your tissues)
- group iv chronic thromboembolic hypertension from blood clots in the lungs or other clotting disorders
- group v pulmonary hypertension linked to blood disorders, metabolic disorders like thyroid disease, systemic disorders like vasculitis, or other conditions like kidney disease. group v can have multiple causes or an unknown cause.
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groups ii and iii are the most common types, according to the european heart journal.
since 1997, there have been 11 pulmonary hypertension-specific medical treatments approved in canada for the treatment of pulmonary arterial hypertension. one medication is also approved to treat chronic thromboembolic pulmonary hypertension. these therapies can help relieve the symptoms so that patients’ wellbeing, activity and quality of life is improved. medical treatment can also slow the progression of the disease and decrease risk of heart failure. these treatments, however, are not a cure.
“a textbook example is a very young woman in her prime, late teens or early 20s, where this is their only problem,” gardner explains of treating people with pulmonary arterial hypertension. “patients have shortness of breath and dismissed it for years, thinking they were out of shape. and then they come in for this rare diagnosis. so the challenge we have with some of these patients is in the conversations around the specialized therapies and how aggressive we want to be with treating it.”
she’s worked with patients who look like healthy, young women who may have young kids, telling them about a condition that’s going to get worse and recommendations for “the more medication the better right at the start,” she adds. “it’s a very hard conversation to have with someone. we’re helping them fill out disability paperwork and giving them handicapped parking passes. and we have this message of ‘we want to get ahead of this and prevent it.’”
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connect with your medical care team
dr. nathan hambly, a respirologist with the pulmonary hypertension program and associate professor at mcmaster university in hamilton, says that connecting with the right specialist is the key to appropriate care.
“the issue is that when people are considering the diagnosis pulmonary hypertension, all that you read about online when you do your initial google search is always pulmonary arterial hypertension. but the vast majority of patients that we see have pulmonary hypertension due to other causes. i would say probably 90 per cent of the referrals that we get through our clinic have hypertension that’s not pulmonary arterial hypertension or group i disease,” he says.
for those patients, in groups ii and iii, the treatment is focused on tackling the underlying cause or disease that has caused the increased blood pressure in the lung. “where we see this most commonly is in more elderly patients who have multiple different medical issues including heart arrhythmias, valvular heart disease, prior history of heart attacks, diabetes or they could have lung problems including chronic obstructive pulmonary disease or pulmonary fibrosis.”
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hambly explains that the role of pulmonary hypertension clinics is classifying a patient, figuring out what’s driving the increased blood pressure in their lungs and making sure that they’re referred to the right doctor to treat that problem, like a heart function specialist if it’s heart-related. and every patient with pulmonary hypertension is examined to see whether or not they have blood clots that are causing their disease, which changes the diagnosis to chronic thromboembolic pulmonary hypertension. in this scenario, surgery may be an option to extract the clot. pulmonary hypertension clinics primarily treat group i, pulmonary arterial hypertension.
“being able to weave through this to be able to describe the patient’s phenotype is critically important,” hambly says. “so, once you work your way through all those patients and appropriately diagnose patients, there’s a much smaller group who have group 1, pulmonary arterial hypertension. and for those patients getting them on the appropriate therapies is critical.”
there are options for the younger patient cohort with pulmonary arterial hypertension, and discussions can involve pregnancy planning right out of the gate because of potential side effects from some drugs. “if you’re thinking about having children, that needs to be put on the backburner,” he says. “it’s a huge amount of information to take on, not only for patients but also their family members.”
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as a respirologist, he follows patients with advanced chronic obstructive pulmonary disease (copd), sleep apnea, pulmonary fibrosis or interstitial lung disease. patients with sleep apnea may need a breathing device; those with advanced copd need help with their inhalers or getting them on oxygen if needed. there is also a new medication approved in the u.s. for treating pulmonary fibrosis and pulmonary arterial hypertension which hambly is now studying in clinical trials.
find your support network for pulmonary hypertension
while every patient’s experience is unique, knowing there is someone to reach out to for help, understanding, information or a listening ear is important. gardner, who supports patients and also works in education and advocacy, refers patients to the pulmonary hypertension association of canada for resources and services like peer groups.
“there’s no wrong way to go about coping with this disease. some patients find a lot of hope and comfort in talking to other patients,” she says, adding the association also hosts national patient conferences and online forums to make these connections more accessible. “a rare disease can be isolating and finding someone with the same rare disease that maybe you have something else in common with socially can help.” she cautions that questions about their specific condition and therapy should be directed to their healthcare providers.
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adopt lifestyle changes for better health and managing pulmonary hypertension
the recommendations on lifestyle habits to promote better management of pulmonary hypertension has changed. as hambly points out, “if you look at guidelines from 10 to 20 years ago, it actually says that patients with pulmonary hypertension should not exercise. we have to encourage patients to remain active, keep your muscles fit and keep up that conditioning because that’s what’s going to keep you going moving forward. you’re not necessarily short of breath because of your heart, but because you’re weak and so you need to build that up.” aerobic activity like walking or walking on a treadmill is important, along with light strength training.
gardner talks to patients with heart-related pulmonary hypertension about sodium and fluid restrictions and weight management. while eating plans are tailored to individuals, the general advice is to read labels and understand the sodium content in prepared foods, for instance, to make healthier choices when you’re grocery shopping or eating out. as well, many patients are taking diuretics or water pills trying to get rid of fluid, so restricting the amount of fluid is another treatment component that pushes against society’s messaging on drinking more water for your health. lifestyle changes can help reduce the amount of medications patients need and improve the way they feel, she adds.
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most important to recognize is that “life is not over” with pulmonary hypertension, hambly stresses. “we have options, we have quality of life. we have many people who are going back to work, who are taking care of their kids, and doing other things. some of my more senior members in the program tell stories about people who they started on therapy when these therapies initially became available. they’re still seeing them in clinic and have these lifelong relationships with patients, whereas 20 years ago you didn’t have that because there were no therapies, right? so that’s quite remarkable.”
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