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I'm Aware That I'm Rare: the phaware® podcast

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John Granton, MD - phaware® interview 213

Jan 18, 2019

Dr. John Granton the head of Respirology at University Health Network, Mount Sinai Hospital and Women’s College Hospital.  He is a consultant in respirology and critical care at the Toronto General Hospital and Professor of Medicine at the University of Toronto. He established and remains the director of the pulmonary hypertension (PH) program at the University Health Network. In this episode, Dr. Granton discusses the importance of early diagnosis.

My name is John Granton. I'm a lung doctor working at the Toronto General Hospital, which is part of the University Health Network at the University of Toronto, and I specialize in critical care in pulmonary hypertension.

I first got into pulmonary hypertension really just at a meeting were one of the transplant doctors spoke to me and said, "You know, we know we need somebody to start looking after pulmonary hypertension patients. We have this new drug, Flolan coming down nobody's available to start using it or understanding it, and we need somebody to start work working with us, looking after these patients.” So I thought it was a neat opportunity so I took it on.

It's a disease of the blood vessels of the lungs and my analogy is like taking a garden hose and kinking the garden hose and as you know, the flow in the hose decreases, so the flow through the lung circulation decreases. And similar to if you're watering your garden or trying to wash your car, you can't get enough stream up through the hole so you can't do the things that you want to do. And the same for the patient. They can exercise and do things. If you look at the hose itself, it actually becomes pressurized and that's the pressure we're measuring. But is not the pressure that's the problem it’s the kinking that's the problem. As the kinking gets worse and worse and worse, the flow continues to go down, putting more pressure on the hose and stressing the hose which is the right side of the heart and the right side of the heart starts to fail as the kinking gets worse.

And so what we try to do and treat patients is relieve the kink. As a result, the pressures go down, the flow goes up and you can start to do what you want to do. You can walk further, enjoy life better, spend more time doing the things you enjoy.

Most people just come in because they feel little short of breath. If you're a family doctor, you have a lot of people coming in to see you because they feel short of breath. And so, it's really difficult to tease out just by looking at somebody that they have pulmonary hypertension, which is an uncommon condition compared to more common conditions. Even deconditioning, just being out of shape, which is probably a much more common problem that most North Americans face these days. But I think progression, particularly in a young person, which is out of character for them, you have to pay attention to. And doing the right tests. We often see people being prescribed inhalers, or puffers, to relieve asthma without formally testing for the presence of asthma. We recognize the importance of making the right to diagnosis and using the right treatment.

So doing breathing tests, if you don't find asthma then look at something else. Then as you work your way through the possibilities, consider doing an echocardiogram, maybe not looking for pulmonary hypertension, but seeing if they have a heart related cause for their difficulty breathing. By doing that as well, you can investigate the possibility of them having pulmonary hypertension, potentially as the cause why they feel short of breath. So I think it's thinking about it during the right test, making sure you try to find the diagnosis and then move on if it's not what you think it is.

So, the big red flags for us are when people start to notice swelling of their legs, as the fluid starts to build up. People start to feel faint or actually faint when they start to exercise. That really implies that there something more sinister going on and should really prompt a much more immediate investigation, really directed at the heart and the circulation in the broadest sense. Again the echocardiogram is a really useful tool to tease that out.

We certainly recognize there are people out there with conditions that are at higher risk for developing pulmonary hypertension like scleroderma, for example. We recognize the importance of asking questions. “Are you feeling more short of breath,” doing breathing test to see if there's a problem with the circulation and the lung. And also doing ultrasounds or echocardiograms to see if there is stress on the heart, and finding the disease earlier, and treating earlier.

First of all, pulmonary hypertension as I said is uncommon unlike asthma and COPD and emphysema, which is much more common. It affects the blood vessels unlike other conditions which affect the spongy bits of the lung. But they share commonalities or similarities in that a lot of people just feel short of breath. It distinguishes itself also by the treatments which are available, which are very different from other causes of lung disease.

If you're living in a downtown environment where you have access to specialists, you're probably diagnosed a little faster but we realize that not everybody is living in a downtown center and has access to specialists. I think as you work through the possibilities, that takes time and you go from one test to another, to another. Then the patient tries to get an appointment with a specialist. They in turn raise the possibility, do the echocardiogram, and then refer them on to another specialist who then refers to a PH center.

I think recognizing that if you really think it's there, contacting a pulmonary hypertension center to say, “Is this a possibility, and should this patient be seeing you in advance,” would be helpful to speed that up. I know all the pulmonary hypertension centers make themselves available to speak to primary care physicians and as well as specialists, internists, cardiologists, respirologists, rheumatologists to have that conversation.

By the time you actually present with having difficulty breathing and feeling unwell, you've lost a lot of your circulation and so it's already fairly late on in the condition. There's been a lot of damage already. We believe that earlier diagnosis and earlier treatment as a result, stands a better chance of actually working and improving the pressures. There's a notion that if you can reduce the pressures you may prevent some progression of the condition over time. So I think earlier intervention in most things makes sense and pulmonary hypertension is likely also the same sort of story that the earlier you find the condition, the earlier we treat it aggressively, the more likely the person is to have a better outcome.

I would appeal to the family doctors that when you see somebody with shortness of breath, do the right tests. If you're worried about asthma or lung disease, prove it before he just give them a prescription for inhalers. Do the breathing test to see if they have asthma or not and use the appropriate drugs if they do. If they don't, then you have to take the next step and figure out why their short of breath and that would shorten a lot, the delay in getting the appropriate referral to a center if they have pulmonary hypertension.

My name is John Granton, and I'm aware that I'm rare.

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