The long fight to bring a miracle drug to Canadians

Jordan If you’ve been fortunate enough, not to have cystic fibrosis touched your life, or the lives of your loved ones, then your main exposure to what it does to those who have it probably comes from a public service announcement.

Public service announcement clip Cystic Fibrosis fills your lungs with fluid makes every breath a struggle. It’s like drowning on the inside.

Jordan I have remembered the phrase drowning on the inside. Since I first heard it more than a decade ago. It is terrifyingly evocative. And if you speak to people who live with the disease, they say it’s accurate, that is, until quite recently, for some of the recent breakthroughs have led to a new drug that patients call a game-changer. This drug was approved in the United States more than two years ago. It was approved in Canada last June. And it’s taken longer than that to be approved by various provinces and territories. So what is this drug? How does it work? What does it feel like to cystic fibrosis patients who have literally been waiting their entire lives for a drug like this? And what does the long fight to bring it to Canada reveal about our health care system, and about how it might be changing?

Jordan I’m Jordan Heath Rawlings. This is the big story. Jeremie Saunders is an award-winning, actor, producer. He is the host of several popular Canadian media projects, including for the purposes of this discussion today. An exceptional podcast called Sick Boy. Hi, Jeremy.

Jeremie Saunders Hello, Jordan, how are you doing today?

Jordan I’m doing really well. Thanks so much for coming on the show.

Jeremie Saunders Thanks for having me. If you don’t mind, for people, first of all, who don’t have experience with it, And I’m sure you’ve explained this millions of times, what is Cystic Fibrosis? And what’s it like living with it in general? And I know that obviously, it can be different for different people.

Jeremie Saunders Yeah. So first of all, thanks for bringing that point up. Cystic fibrosis is it’s a complicated disease, to put it mildly. And it is very different for everyone who has it. So you know, my case of cystic fibrosis could look completely different from the next person’s case. Essentially, cystic fibrosis is the, it’s the most common fatal genetic disease affecting Canadians. And currently, there is no cure. And in layman’s terms, and let me just put this out there, I’m not a doctor nor scientist, or researcher. So I’m gonna, I’m gonna dum down this down as best as I can, but basically, see if it affects multiple organs in the body. And primarily, it’s affecting my digestive system and my lungs. And what that looks like is is a series of complications. So I have a difficulty in digesting fats and proteins. I deal with malnutrition and vitamin deficiencies because of this inability to absorb nutrients. And then when it comes to the lungs, there’s a progressive lung damage that happens from you know, chronic infections, constant inflammation within the lungs. And then some people with CF, not myself, but some people also deal with CF-related diabetes. And it’s also really common with people with cystic fibrosis to deal with a lot of sinus issues, sinus infections, sinus polyps, things like that.

Jeremie Saunders On average, a person living with cystic fibrosis spends the equivalent of about four months of full-time work doing life-sustaining treatments, every single year. And so yeah, it’s a , it’s a disease that I was born with. So you know, I was, I was born in 1988, I was diagnosed at 18 months old. And it’s something that I’ve lived with my entire life. It’s it’s all I’ve ever known. And it’s it’s definitely been a challenge, you know, over the 34 years that I’ve lived with it.

Jordan First of all, thank you for sharing all that. And I think it’s it’s really important that we understand, how this disease can affect people differently. But in general, when you mentioned it’s the most common genetic disease Canadians live with, how many Canadians are we talking about here? And I guess, one of the things that I want to kind of set the table with before we talk about the new treatment, which is why we’re here today is you know, what kind of support Have they gotten from health care systems across the country? And, and you know, as we look at the numbers today, how has that changed during the pandemic? Because I imagine it can’t be good.

Jeremie Saunders Yeah. So cystic fibrosis is considered a rare disease. I believe the stat in Canada is that there’s currently just over 4300 Canadians living with cystic fibrosis. And when it comes to support, I think the best way to put that as it sort of depends on where you live in the country, right. So I live in Halifax, Nova Scotia, I’ve pretty much lived here the majority of my life. And Nova Scotia has actually done a really fantastic job in supporting those with living with cystic fibrosis when it comes to the medications that I require. Day in day out. Nova Scotia pretty much covers every single medication that I need. And you know, I take upwards of 40 pills a day. So there’s quite a vast array of treatments that are necessary for me to get through the day. You know, when I mentioned that those life-sustaining treatments, those include daily nebulizers, so taking liquid medicine, things like Ventolin, and, and hypertonic saline and putting those in a little machine that turns it into essentially vapor and I breathe it into my lungs, the pills that I take, for example, I take oral enzymes every single time I eat because of that malnutrition, because I can’t break down the foods properly.

Jeremie Saunders There’s a whole list of other medications that I take on a daily basis just to kind of like, get myself through. And luckily living in Nova Scotia, all of those medications are covered. And that’s a huge, that’s been a huge support. Nova Scotia has done a really great job at doing that. Some provinces don’t cover all those meds. An example would be I moved to British Columbia for about a year, maybe five, six years ago, when I got there. Unbeknownst to me, not all my meds were going to be covered. And so I went from paying $0 a year to looking at paying upwards of you know, 10 grand a year to get some of the meds that I need.

Jeremie Saunders When it comes to the pandemic. I mean, although my, my immune system is fine, I’m not technically immunocompromised, living with a chronic lung disease does present me with a little bit of a higher risk factor when it comes to COVID Of course, I mean, I’ve just had to be extraordinarily cautious over the last oh my gosh, two years. Are we two years already? So yeah, it’s one of those things where I just had to be really extra cautious and, and kind of live the same way that a lot of us are living, which is just day to day doing what I can to ensure that I’m not putting myself at risk. And you know, fingers crossed. So far so good. I’ve dodged COVID All those memes coming out of what it looks like when we’re the people who haven’t caught COVID Yet, that is me

Jordan Right,the Super Mario level with all the fire sticks?

Jeremie Saunders Yeah, that’s me. I’m, I’m Mario just jumping through all those firesticks. And hopefully, hopefully, I don’t get burnt anytime soon.

Jordan In terms of treatments, you’ve described some of them already. But I guess what I’m trying to get a sense of is, what has been traditionally available in Canada? And how much do those treatments impact quality of life for people who live with CF in the past, before we get to the new stuff?

Jeremie Saunders Yes. So it’s really interesting. Since I was born, up until today, I’ve been essentially doing the exact same treatments, as long as I can remember. So those nebulizers twice a day. I’ve been doing that since I was a child. The medicines that I take through the nebulizer have shifted over the years a little bit. The medications that I take, so the oral enzymes, the vitamins, the you know, medications to reduce inflammation in the sinuses, those are also medications that I’ve been taking pretty much my entire life. And not much has changed in terms of the types of medications that I’ve been receiving, or that we as you know, folks living with cystic fibrosis have been receiving.

Jeremie Saunders Recently there has been a sort of an advancement in the world of treatment, when it comes to cystic fibrosis with these genetic modulating medications. And the one that we’re talking about today is Trikafta. This has been kind of like a total game-changer, like a miracle drug that has that has changed the lives of a lot of people living with CF today. And this drug Trikafta and a couple of others that are similar to try CAFTA but only treating a smaller percentage of people with cystic fibrosis ones like ORKAMBI. Those came out a few years back. But this one Trikafta is actually targeting the vast majority of the people who live with cystic fibrosis who live with this specific gene mutation, the delta f 508 mutation, which is the more common mutation amongst people living with cystic fibrosis and happens to be the one that I live with as well.

Jordan I’m going to ask you a question appreciating, as you said at the beginning of this interview, that you’re going to explain complicated things in layman’s terms. So I’m not expecting, I’m not expecting a scientific answer, but just given the kind of discussion that happens in the medical community. Now, I shouldn’t say in the medical community, but happens around medical advancements, particularly with COVID vaccines to hear you use the term genetic modulators? My honest question is, what does that mean? What do they do?

Jeremie Saunders I wish I could explain, I wish I could explain it to you. You know, I think, I think I can, I think I can explain it to the best of my ability. So like I said earlier, cystic fibrosis is a genetic disease, right. So what’s happening in my body is that I have a gene mutation. And because I was born with this gene mutation, that mutation is affecting one particular protein in my body. And this protein is known as the CFTR protein. So that protein has a very important job. Its job is to move chloride or salt through the cell lining of my body. So because that protein is all messed up, because of the mutation, chloride, so it’s essentially salt becomes trapped within the cells of my body. And so without the proper movement of that salt, water can’t hydrate the cell surface in my body. This is the reason that my body is full of thick, sticky mucus, because my cells can’t be hydrated. And that thick mucus is the cause of all of my problems. It’s the reason why my lungs are this breeding ground for bacteria, it’s the reason why my body can’t produce the enzymes needed to break down food. So that’s a huge issue, this, this filling of mucus within my body.

Jeremie Saunders So what Trikafta does, is it doesn’t actually fix the gene mutation. Okay, so that mutation is going to stick with me, right until there’s a cure. But what it does do Trikafta goes in and it, it treats that protein, that’s all messed up. So, Trikafta goes in bonds to that protein and folds it in a way where it can actually do its job, which is to flush out that chloride. Flush out those, those cells, allowing the mucus to freely travel through my body the way that it should. And so in turn, it allows my, cell lining to be hydrated, and then I can get that mucus out. And it’s no longer causing the problems that it would cause. So it’s not a cure, but it’s, it’s kind of the next best thing. The way that it was put to me that made a lot of sense was that Trikafta isn’t treating the symptoms of the disease, but it’s treating at the source. Whereas the nebulizers, the enzymes, the, you know, the 40 pills a day that isn’t doing anything at the source, but it’s, it’s treating or managing the symptoms as best as they can.Whereas Trikafta is going in, and it’s going alright, we’re gonna, we’re gonna treat just the, the base of where all these symptoms are coming from, in order to, you know, produce benefits.

Jordan That was a great explanation. And I’ve been talking to scientists about diseases and what they do for way too long now, over the past couple of years, so I can appreciate it.

Jeremie Saunders Oh, I’ll take that as a compliment. Thank you,

Jordan In terms of it being a game-changer, and transformation. I mean, you’ve done a great job explaining what it’s doing. What kind of results does it get? And I guess maybe first in, in the general spectrum, and you know, what the research says, but also, you’re taking it right.

Jeremie Saunders Yeah.

Jordan Tell me about what it’s done to your quality of life.

Jeremie Saunders Oh, it? Honestly, it’s, it’s, it’s one of those things that’s hard to explain. Because it’s so profound, that you have to experience it to really know. So a quick little anecdote. A friend of mine, Stephen, he had access to Trikafta a little bit before I did, and I remember asking him hey, how does it feel? You know, how are you feeling these days? And he responded by saying, I don’t think I could explain to you exactly how it feels because it’s, it’s beyond anything I’ve ever or known. And so it wasn’t until I took it that I kind of understood what he was talking about. Maybe the previous six months of, before I got my hands on Trikafta, about 4,3,4, maybe even five days a week, I would cough so much to the point of vomiting. There’s so much mucus in my body. It’s so thick, it’s so heavy, that breathing at night would be troublesome. coughing so much that I’m throwing up, I would get daily, daily sinus headaches. That would, if I didn’t like take an Advil you know, before 12 o’clock every single day, it would lead to like migraine status pain in the sinuses.

Jeremie Saunders The first two days of taking Trikafta the coughing was next to nothing. I was no longer coughing. Since I’ve been taking Trikafta and it’s only been a month and a half now, I haven’t had a single sinus headache. And the thing that really blew my mind was, I would get winded just from taking my dog for a walk. It didn’t matter if I was walking flat ground. I mean if I was walking any kind of incline and I mean any like even the slightest incline, I would get fully winded. And I remember it was, it was probably the third or fourth day I was on Trikafta. And I took my dog for a walk and I decided to walk up Citadel Hill and here in Halifax Citadel hill’s, you know, it’s smack dab in the middle of the city. It’s quite a steep incline. And I got to the top of the hill. And I paused for a moment I went whoa, I’m not out of breath at all. And that was kind of like the lightbulb moment of like, oh, wow, this is really, this is really cool.

Jeremie Saunders But the thing that I think has transformed the most for me in taking the drug is that I’m no longer thinking about cystic fibrosis on a daily basis.

Jordan Right.

Jeremie Saunders It’s almost like I’ve forgotten that I have it. Whereas, for the last 33 and a half years or 33 years,I’ve, it’s always on the back of my mind, even when I’m not thinking about it. It’s there, subconsciously it’s there. And to say it’s been life-changing. It doesn’t do it justice.

Jordan Wow. Well, first of all, I mean, I’m so happy for you that sounds fantastic.

Jeremie Saunders Thank you. Yeah, thanks.

Jordan And then second of all, you know, this is why we’re talking today. This sounds like a wonder drug. I believe it is now being approved by Health Canada that came a little while ago. But is it still available to everybody who needs it in Canada? And why not? And why did it take so long? For instance, for you to get it?

Jeremie Saunders Yeah, so Trikafta was approved in the United States. In October of 2019. It was also approved around that time in the UK and a couple of other countries. It wasn’t approved in Canada until June of 2021. And then even after it was approved, it had to go through several other hoops in order to get to the provinces that need it. And so it was approved here in Nova Scotia in October of 2021. And then I got my hands on it. Maybe about a month after that. There was quite a fight to get the drug here in Canada to get the drug approved.

Jordan Tell me about that fight. First of all, why was it quite a fight when it had clearly been proven by pretty competent medical associations and other countries?

Jeremie Saunders You know, that this is the this is the million-dollar question. I don’t know, I couldn’t tell you. Although it took what felt like forever. It was pushed through pretty quickly in terms of how fast drugs are typically approved. But even then, it just felt like forever. And there was a massive gathering of people that really came together to make their voices heard. CF get loud, is is an advocacy group that really were at the helm of that. And they did a wonderful job of hounding the system that we have to push this forward. But it really did take a lot of work. You know, it’s it’s interesting to me how this all happened around the same time of or within the pandemic, right. It’s not like this drug wasn’t necessary for people living with cystic fibrosis. I mean, I can’t even begin to tell you the amount of people that passed away in the time that it took for Trikafta to, to be approved. And, and, and given out to the patients across Canada that that needed it.

Jeremie Saunders You know,people were, that people are dying of cystic fibrosis. It’s a fatal genetic disease. And it’s, it’s also a drug that, you know, if you are in end-stage cystic fibrosis, you know, your lung function is at 20%. And you’re really not doing okay. If you get that drug at that point in your life, it could completely reverse that situation, you know. It could add so many more years to your life.

Jordan Wow.

Jeremie Saunders So it it’s most certainly an important drug. But still, it had to go through these hoops that for whatever reason, our, you know, medical system has put into place and I am thankful that we we did get it to be honest with you, I thought it would take longer. You know, I’m a bit of a pessimist when it comes to those sorts of things. So when it was approved in the US, I was looking at, you know how maybe we’ll get it in five years from now? Because things are just so slow to move and shift in the healthcare system, especially here in Canada.

Jordan Is it everywhere in Canada now? Are there still provinces that have yet to approve it?

Jeremie Saunders Yeah, so as of right now, Trikafta is is currently approved in all of the provinces and the territories as well, which is fantastic, right? Once, I believe Ontario, or Quebec were the first provinces to approve it and to get that ball rolling. And once that happened, it was like, every few days, bam, another province, another province, another province, I believe Nova Scotia was the last province to kind of step into that. Now again, depending on which province you’re in, it may be different in terms of what that coverage looks like. So in Nova Scotia, it is fully covered, which I’m so grateful for the price tag of this drug is, it’s comical, you know, the it’s $25,000 a month $300,000 a year for this drug. So it’s it’s, you know, the price tag is really, it seems like something of an onion article. Thankfully, Nova Scotia did the right thing. And they’ve decided to cover the drug for the people that need it.

Jordan The last thing I want to ask you is, you know, it’s interesting that you mentioned your initial thought was really cynical that it would take almost five years to get here. And I guess what I’m trying to understand is if the pandemic has kind of changed the paradigm for this in Canada, because certainly, this wouldn’t be the only drug that in the past would have been approved in other countries long before it got here. And, you know, do you think that this has set a precedent for other technologies? Other new drugs that hopefully get to the people like yourself who need them quicker?

Jeremie Saunders That’s somewhere where I feel a little less pessimistic, I do feel quite optimistic about how Trikafta could potentially set a precedent for granting access to drugs, especially in the realm of rare diseases. Where beforehand, maybe, maybe there would have been a little bit more red tape to go through. You know, one of the things to keep in mind here is that the access to Trikafta not only is it going to benefit someone like myself in terms of extending my life expectancy, but it’s it’s also going to benefit the healthcare system as a whole, because it is reducing the amount of time that I need to spend immersed in the healthcare system, receiving care, receiving treatment, which in turn reduces the cost associated with living with cystic fibrosis.

Jeremie Saunders So you know, it takes a bit of the burden away from what, how much I cost as a human, to be entrenched in the healthcare system. And so if, if that’s the thing that comes out of this, then my hope is that, you know, our government, the systems that play in our government, when it comes to our health care system are going to see the benefits that come with providing these life-altering life-changing drugs, to the people that need it, not only to help them, but also to just relieve some of the burden that we, unfortunately, bear on the healthcare system as a whole.

Jordan Jeremy, thank you so much for explaining this to us. And, and again, I’m so glad that you’re on this and that it’s making a real difference.

Jeremie Saunders Thank you so much, Jordan, this has been a real pleasure.

Jordan Jeremie Saunders, host of sick boy, a podcast, you really should check out. That was the big story. For more from us. As always, head to thebigstorypodcast.ca to find all of our episodes, find us on Twitter @TheBigStoryFPN. To complain about them, and write us an email at thebigstorypodcast@rci.rogers.com [click here!] This podcast is available on every podcast player, it is especially available in the podcast players that allow you to rate and share podcasts. That’s where I’d like you to listen to it because I’d like you to rate and share. Because I’m vain like that. Thanks for listening. I’m Jordan Heath Rawlings. We’ll talk tomorrow

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