Don Downey - phaware® interview 431

Sep 12, 2023

Canadian pulmonary hypertension care partner, Don Downey 

My name's Don Downey. I'm from Calgary, Alberta. My wife, Kathy, was diagnosed with idiopathic pulmonary hypertension in March of 2020. It actually goes back way before that. She started having problems in December of 2015. We'd gone many times to her general practitioner. He sent her to specialists. She went to an internist, who basically told her she had asthma. That didn't really work. She tried puffers and all that type of stuff. Nothing of that worked. She was then sent to a respirologist, one of the top ones in the city. It basically boiled down to she was told to lose some weight, and then, hopefully, that would make things better.

Over the next couple of years, she'd do an annual visit with him. It wasn't getting better. He just kept saying during the one year she had lost like 60 pounds over the course of the year, it was still "Just lose some more weight" and everything. In February of 2020, she went to the emergency room because she was having numbness in her arms. She was talking to the ER doc. He was going through her case file and all the tests that had been done over the years and everything else. There was one that he was looking at. She asked him if she could have a copy of it, and she sent it to me. I was down in San Diego visiting a friend who's a forensic pathologist, so a doctor. She sent it to me and said, "Well, can you ask him if there's any questions I should ask this ER doc?" I asked him, and he looked at it, and I think he spent about 30 seconds, and he said, "Yeah, can you ask him why you have pulmonary hypertension?" Actually, on the report from this test that they had done two and a half years prior, September 2017, it said "pulmonary hypertension?"

Nothing had happened in between. Nobody had noted that and thought to test her for it. She asked the ER doc, and he got a whole bank of tests done right away. From there, it ended up she was sent back and ended up going back to the same internist who told her again, "I think it's just asthma," even though we already had a good idea it was PH. The following week her regular checkup with the respirologist. She had to do a stress test before it. She got about halfway through the stress test, and she just couldn't go any further. 

Then we went upstairs. Normally, we'd be sitting in the waiting area for a while, and then they put us into one of the exam rooms, which was across from his office. We would be sitting in the exam room. We could see across. He was working on his computer, finishing off whatever the previous patient was. On this day, he met us at the door. We didn't even do the waiting room. As soon as we got up there, it was right into his office. So we knew there was something weird going on. Very quickly, he had us booked in with the PH clinic in Calgary. She did her right heart catheterization and everything else.

She had her right heart catheter on Thursday, March 12th, 2020, and then we went back for the results on the 13th. So, Friday the 13th of March, which was the day that the world shut down, was the day that she was diagnosed. We had a lot of uncertainty and a lot of things where what's going to go on, because even they were trying to figure out. All of a sudden, you've got COVID. Everything's locked down. How do you go about doing all the visits and everything else that normally would happen? We were lucky enough to have a doctor that, on the one day, it took a little bit longer because he was doing stuff. So it took a few extra days before he was really able to do stuff where we would've normally done trips to the clinic. But there was one day where he spent an hour and a half on a Zoom call with us just to make sure that we were ready for it and all. 

We, at the time, didn't look back. We looked forward. But as we've gone along, we've looked back at things that might have been missed. There was this disconnect because the clinic was still trying to figure out what they were going to do. The day that we got diagnosed, that was the last time we were in that clinic for a couple of months. Normally, you'd do half a dozen visits to the clinic. It took a little while for stuff and just getting the information. And, of course, the first thing he said as soon as he said, "You have PH," is, "Don't Google it." Of course, when you're not getting information, and they could only get the information out to you so quick because they're trying to do the transition -- so we Googled it.

You're looking at now we're in this situation where we've Googled it. It says that this is the average life expectancy. We know that we had two and a half years from when it should have, in our opinion, at the time because now we know that this was questioned at that time. It's like, "Okay, so we take two and a half off. Well, that's not a lot of time." So, that was the thing. It took a while because we had to wait for the clinic to be able to give us the actual information and give us stuff that worked for her situation. They tried to keep us out for as long as they could, so it was probably, I think it was about six weeks before we did one. But even that, it was a matter of you had to go through all the protocols to get in. You had to do everything that you were safe and all that because she had severe PH right from the start.

She was on high-flow oxygen within days of her diagnosis. So it was something where they wanted to put her in the hospital to be able to try to do stuff, and they said, "Well, it's not safe." So she didn't actually get to some of the treatment and some of the stuff that they wanted to do until the hospitals were opening up a little bit more, and they could do stuff.

One of the nice things is that we actually had two adult children that were living at home at the time. So we had them. Also, we've got a house, a two-story house with a walkout basement, and there's a basement suite. Her parents lived down there. The girl that my son was going out with at the time was also with us there. So there were seven of us in the house. It was a busy place. We figured out how to deal with things. I had to figure out how to deal with the oxygen and figuring out how to change cylinders and do that quickly so that she wasn't without oxygen for any periods of time. We did a lot of grocery delivery and that type of thing, so that I wasn't going out just because of the risk.

She's had a lot of ups and downs. She's been through different therapies that try something. Some of them have worked. She's on triple therapy. So far this year, since the beginning of January, she's had three different stints in the hospital. So she's probably spent about seven weeks total. She had a gastrointestinal bleed that nobody knows what it was, but the way that her numbers were for her hemoglobin back in January when this happened, they couldn't even do a scope to check. She can't be sedated or any of that. So she ended up one of her other times that she went in, they were able to do the scope. But they weren't able to figure it out, which is causing some issues with her potential for transplant.

Kathy's the type of person that she was actually not overly outspoken. She was the type that didn't want to have anybody mad at what she said and those types of things. Once she was diagnosed, and she started looking into things, and she was pointed out by the clinic to PHA Canada. Then, she got onto the Canadian friends' Facebook page and got talking to people there, it just exploded for her where she started wanting to know more, wanting to learn, wanting to help other people learn and that type of thing. She was a research librarian in the past, so of course, she's the type that, "I'm going to look into this. I want to do that."

We could see there were things, like the amount of time it took for her diagnosis is one of the big things for me for becoming an ambassador to try to advocate for better educational situations for doctors, for general practitioners, to know enough about PH to think of it a lot sooner. That was the big thing. It just morphed out of what she was doing. I'm along for the ride, but I'm loving it. The big thing is you literally go on a day-to-day basis, because the ups and downs can happen real quick. You hope that she can keep going for as long as possible, that she can get all the stuff figured out, get her weight down that little bit, and figure out this GI bleed, and be able to look at the transplant, which would be a major thing for her because her PH was already advanced enough by the time she was diagnosed to be problematic.

It almost puts it where that is the next outcome. She also has a hole in the heart. It's one that you have when you're born that normally closes up. Hers didn't. So there are certain therapies she can't take because of it. We know that, basically, the next thing is a transplant. So it's figuring out all of the things and hoping that that's going to happen. We both want to make as big of a mark on things to try to help so that people don't have to go through what we've gone through and so that we can get it towards finding a cure or finding better therapies that'll make things better.

I'm Don Downey, and I'm aware that my wife is rare.

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