I’m Aware That I’m Rare: Jason Weatherald, MD (467)

phaware global association®
7 min readMay 6, 2024


the phaware® interview

Dr. Jason Weatherald, a pulmonologist at the University of Alberta, discusses a study on the socioeconomic burden of pulmonary arterial hypertension (PAH) in Canada. The study, conducted through PHA Canada, surveyed PAH patients and their caregivers to understand how the disease affects their ability to work and perform daily activities. The results showed that a significant number of PAH patients were unable to work or had limited career options due to the disease. The study also highlighted the impact of PAH on caregivers, with many having to assist patients with daily activities. The findings emphasize the need for healthcare professionals to discuss the implications of PAH on patients’ ability to work and access support.

Read the report here: https://phacanada.ca/burden

My name is Jason Weatherald. I’m a pulmonologist at the University of Alberta in Edmonton, Canada. I’m really excited today to talk about a new project called the Socioeconomic Burden of Pulmonary Arterial Hypertension in Canada, which was a study that we did in the fall of 2023 that was a national survey through PHA Canada, looking at how pulmonary arterial hypertension can affect patient’s ability to work and to execute daily activities. We also asked some questions about how the disease affects their caregiver’s ability to work.

We know from previous burden of illness surveys that there’s a huge burden of illness and that a lot of patients are unable to work or to work full-time if they are able to work at all. We wanted to dive down and uncover a bit more about how people are affected in terms of their ability to work. We used a validated measurement tool that’s called the Work Productivity and Activity Impairment Questionnaire, which contains a whole bunch of questions about employment and activity levels.

Through PHA Canada, we distributed this to their members and asked participants to self-identify about what type of pulmonary hypertension they had, provide some information about what medications they were on, some demographic related factors. We then asked them to fill out some quality of life questionnaires. This specific questionnaire, which abbreviated is called the WPAI, asking questions about productivity and impairment.

So really excitingly, we’ve submitted some of these results to be presented at the World Symposium on Pulmonary Hypertension, which is coming up in June in Barcelona. As people on the podcast probably know, it’s one of the biggest events in pulmonary hypertension that only happens every five or six years. We’ll be presenting some of the more detailed results there. Also the summary of the results have been posted by PHA Canada in timing with World PH Day.

Just to summarize the results of this study, we administered this survey again, sent out by email in both English and in French in Canada. The study was coordinated through an academic research organization based at my university called the Canadian VIGOUR Centre, who provided a lot of the logistical support to this. We had quite a few responses. We had 217 patients with Group 1 PAH who answered the survey. Based on the responses, it was fairly representative of what we think about Group 1 PAH affecting… The average age was around 57, and the majority of people were women that answered the survey. We had a reasonably good representation across the country and a pretty representative breakdown in terms of how people describe their functional class.

When we asked questions about how PH affects their ability to keep and find a job, there were some really interesting results. 87% of respondents said that PAH limited their career options. Furthermore, 63% said that the PH prevented them from finding a job altogether. So I think that’s really huge seeing that 2/3 of patients were really not able to work at all. Digging down a little bit deeper into that, even of those people who were employed at the time of the survey, which was only about 28% of people, a lot of them were actually not able to fully participate in their workplace or they reported diminished workplace productivity. For instance, 54% responded that they felt that their activity or productivity at work was impaired because of their PAH. Furthermore, 12% of these people missed work due to PAH-related symptoms or testing.

I think this was really quite profound, not only in current workplace, but a lot of other people said that their PAH interfered with their ability to move forward in their work such as getting promotion or even seeking promotion. I think that’s another important factor even among the people who are able to hold down a job. Interestingly too, because there were some older people or people further along in their career that answered the survey and about half of those people said that they would have to retire because of PAH or they were planning to retire early because of being diagnosed with PAH. Almost 1/3 of the people reported that they were effectively disabled by PAH. This questionnaire allows us to drill down a little bit in terms of more specific impairments related to the disease and how it affects people’s ability to work beyond just saying, “I can’t work.” There’s a lot of factors going on.

I think there were some other really interesting insights here in terms of how their caregivers are affected. We did look at who people had as a caregiver, whether they required a full-time caregiver and whether that was a family member. Interestingly, 77% of the people that answered this survey said that their partner or spouse had to assist them with just daily activities in their home. Another 29% of people said that their children had to help them with daily activities. This was not surprisingly also related to people’s functional class. People with more severe symptoms were less able to do daily activities around the home. So I think this does have a lot of impact, not just on patients, but on their caregivers.

To give a specific example, when we looked at the people who reported being in functional class IV of the most severe symptoms, they reported that their caregiver spent on average about 43 hours per week related to their personal or medical care, which is essentially a full-time job. So we’re able to really quantify the impacts of this disease on patient’s caregivers, which I think is unique.

I think there are really broad insights and implications of this survey that, as a clinician, tell me a lot about what patients are going through. But also, it started to make me think a little bit more about what type of supports we need to help patients access. For instance, when we’re talking to newly diagnosed patients about how the disease is going to affect them, we typically, in clinic, spend a lot of time talking about tests and prognosis and risk and treatment and treatment side effects, but we very rarely have time or even habits of discussing how this will influence people’s ability to make a living or advance in their career.

We often don’t spend enough time talking about the impacts of this disease on caregivers either with the patients or with their caregivers directly. I think this survey really helps us understand the magnitude of this, and it highlights the importance of talking about the implications of the disease on patient’s ability to work and also their caregiver’s ability to work. This can have major repercussions in countries, for instance, where health insurance is tied to employment. That could be a crucial thing for patients to maintain in order to have access to medications. Fortunately, in Canada, the vast majority of the pulmonary hypertension drugs are covered and fully reimbursed through our universal healthcare system. Not all of them are covered, but access to medications isn’t as big as of an issue in Canada as it may be in other areas. But certainly income being able to support daily needs is of course strongly tied to this disease and to the ability to survive.

I think as clinicians, we need to make sure the patients understand this and then we need to start thinking about ways to support patients in doing this. If there’s any one benefit of the recent COVID-19 pandemic, it’s realizing how many jobs or responsibilities can be done virtually or from home. For those patients who are able to do that in work, it may actually help them maintain their employment. If they’re able to pivot to doing things more virtually or remotely, it certainly has the potential of being much less exhausting for them and perhaps allowing them to still participate in their job in different ways.

So what are the next steps here? I think one thing we want to watch out for and see whether new treatments that are coming down the pipeline, in addition to improving things like symptoms and quality of life, it may be very interesting to see how emerging therapies actually improve patient’s ability to do these other things in daily life. I think this gives us a baseline. As everyone knows, sotatercept was recently approved in the United States and hopefully will be approved in other countries soon. There’s a lot of other really exciting new therapies being explored and studied in pulmonary arterial hypertension. So now we have a bit of a baseline measurement. My goal and my hope is that in a couple of years we can repeat this and look at whether new novel therapies are actually improving, at least at the population level, some of these reported inabilities with respect to being able to work and do daily activities.

The other thing I think it highlights is the utility in using these types of measures, for instance, in clinical studies or in clinical trials. It goes beyond just quality of life. It’s about ability to support daily life and ability to do those things that make life meaningful, like find employment and for caregivers to be able to continue their employment. I hope that this study will highlight the utility and measuring those things when we’re looking at effectiveness of treatments or programs in pulmonary hypertension.

I’m Dr. Jason Weatherald, and I’m aware that my patients are rare.

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