Alex Cowie - phaware® interview 308

Alex Cowie lives in the South West Coast of Scotland, UK with his partner Jo. Alex was diagnosed with Hereditary Pulmonary Arterial Hypertension in August 2016. He has participated in 4 clinical trials to date. Alex's daughter, Erika, lost her battle with PH in 2016 following her 18th birthday.

My name's Alex Cowie and I'm in Girvan, a little town on the west coast of Scotland. We moved here a year and a half ago just due to life circumstance, we needed a fresh start and a new beginning. Primary one of the reasons was because my introduction to pulmonary arterial hypertension or PH, was when my daughter Erica was diagnosed when she was 11 years old.

So, PH was an unknown disease condition I'd never heard of. So, obviously Erica was young, she was diagnosed when she was 11, but there was a lot of toing and froing to doctors saying "Oh, it's just asthma, teenager", that kind of thing. It didn't really go any further until she actually collapsed on her eleventh birthday going round to her friends. She wasn't very well but she carried on. She wanted to go out with her friends because they were going for a nice meal that early evening. So, it's all snowballed from there.

She spent a time in hospital. Eventually it got to a point that they thought it was a hole in her heart, but she actually ended up staying in a week in a hospital where I was living, which was in south of England at the time. She was then sent to another hospital in Bristol. Bristol Children's Hospital. They did further tests and stuff. Then they sent her to Great Ormond Street in London to have further tests to see what was exactly going on. That's where they gave her a right heart catheterization. That's when she was diagnosed as severe pulmonary arterial hypertension. Then, she was classed as idiopathic, because she did do genetic tests and everything else. That was my first introduction of what PH was. I never had even heard of it before. It was an unknown disease to me. You hear a lot of things about cancer, cancer this, cancer that. To be told about pulmonary arterial hypertension and how severe my daughter was, it was quite scary because they gave her two years to live.

They sent her home with loads of drugs, tablets and she used a, iloprost inhaler four, five times a day. She did that for a year and then she had to go onto a Hickman line, a CADD pump. That was a major change for a young teenage girl going into womanhood, changes to body and stuff like that. It was scary for her and it was scary for all of us, because we were like "What the hell", this disease, this condition, how severe it is and how critical it is. Then, she just battled on with the PH doing different genetic tests and trips to hospitals. Then from leaving Great Ormond Street to actually going to adult services, she was still on the Hickman line with intravenous [medication].

She had lots of severe symptoms that you get with being on the Hickman line. One of the things that she had was a rash. As a young woman, it changed her life. She was very confident. She got a job and she was working and she did a lot at school and stuff like that. So she was a very, very inspirational young lady and then she saw her 18th birthday and then, unfortunately, she passed away in the 26th of September in 2016. Just six weeks after celebrating her 18th birthday. So that was my introduction to PH.

But prior to Erica getting really sick and losing her battle with PH and pneumonia, I was sort of having some dizzy spells. I'd probably had a chest infection the back end of 2015, after that Christmas period in 2016. I sort of started getting dizzy spells, postural hypertension. When I stand up I'd get a little bit dizzy or if I bend down I get a little bit dizzy.

So I went to my doctor and said, "Oh, you know, this is what's occurring, I am getting these dizzy spells". [He] did the normal stuff, blood pressure and all that and said, "No, can't find anything wrong, it's just postural hypertension." Bearing in mind, they knew the history of my daughter having pulmonary arterial hypertension. So this carried on for a couple of months. I used to walk up three flights of stairs and run up them, go mountain biking and I was physically active. All of a sudden, I started to get short of breath when I've climbed three flights of stairs or when I was out mountain biking. I'd hit a hill and I'd be puffing and blowing. I was thinking, "Oh here's me. Really unfit now." Because I'd always been fit and active all my life because I was in the Army, I enjoyed running a marathon, always active, playing football or as you call it, soccer and that kind of stuff.

So I was always active and all of a sudden I was blowing like I'd run three marathons. I was really struggling. Then the dizzy spells continued to happen and I went back to the doctor's suddenly, these dizzy spells are becoming more frequent and that it's not like a passing thing. I'm standing and I've got to stand still or sit down for a good 20 minutes just for them to pass and I get me bearings again, because you know what I didn't want to do is end up collapsing because I'm six foot one and I'm a big chap. So anyway I went to the doctors and that again and he said we'll do an ECG and done that. Nothing came back on the ECG and he said what we'll do, we'll send you to the hospital, get an echocardiogram, see what's going on.

I was all familiar with all this kind of stuff from Erica and her having all these tests and continued to have all these tests when she'd go to her PH clinic appointments. So, I was like "Oh, bit scary and that" and wondered what was going on. Maybe it was just an age thing or had something a bit more sinister going on. So we celebrated Erica 18th birthday in end of July. Then, a couple of weeks after that I was going to go on annual leave from work. A couple of weeks off, go away, catch up with friends and family and stuff like that. Then one evening, oh you went upstairs to pick up the cat litter trays, just so I could empty them and clean them out, so they had fresh cat litter. As I was walking down the stairs, I sort of got a bit dizzy.

What I did was stand there holding the two cat litter trays and holding them and normally waiting for the dizzy spell to pass. Unfortunately, the dizzy spell didn't pass and I woke up in a heap in the bottom of the stairs, covered in cat litter thinking what the hell happened and realized that I passed out. Luckily, I hadn't broken anything, maybe a few little bumps and bruises. What I did is I cleaned up the cat litter and then I text my other half, said "Look, I'm not feeling very well, I've just passed out. I'm going to go to bed and sleep it off and arrange to see the doctor in the morning." She texted me back saying, "No, you're bloody not. You're going to go to A&E and get checked out." So, I went to A&E, got checked out and they just did some normal tests, blood pressure, stuff like that.

I said it was an ongoing thing, because it was postural hypertension. They said, "Okay, well what we'll do is we'll just do a quick ECG to make sure there's nothing sinister going on and then we'll let you go home". So they did the ECG and it came back a little bit abnormal. They said, "Oh, unfortunately going to have to keep you in. Not sure why, but there's something going on. So, we'd like to do some further tests." I went, “okay.” So I told my partner Jo and let my daughter know that I was being admitted to the hospital and they all come and saw me the next day. I was in there for about three or four days waiting for further tests. I had an echocardiogram, lots of ECGs, lots of blood [tests], and then they said, "Oh, well what we're going to do is we're going to refer you to some further tests, because of the history of Erica having idiopathic pulmonary arterial hypertension." They just wanted to do run a few more tests.

They came back and they said, "Ah, actually you've got pulmonary arterial hypertension at the age of 46.” They classed mine as hereditary pulmonary arterial hypertension, because my daughter had it. Prior to that there was no symptoms or link between us having pulmonary arterial hypertension. That was in August the 18th, 2016 I was diagnosed. Funny how you remember dates. Then six weeks after me being diagnosed, Erica lost her battle with pulmonary hypertension.

Looking back now, it's like not really having that revelation of this could be PH. I think it was because you know, I've always been fit and active. I was in the Army for eight years and there was no sign of PH for 46 years in my body. How crazy is that? I've got this rare disease, but it didn't manifest itself for 46 odd years.

That was the head thing I think that I struggled with a little bit to begin with. But because it was so rare, it was like, oh, the doctors, which weren't sort of saying, "Oh, I've got it". They weren't sure. There's obviously thinking maybe I might be PH, but because what is the statistics?  1 in a million people get PH? So my daughter had PH, so she was that one in that million. I was the second 1 in that million to get PH. So, that was a bit of a strange thing to happen that all of a sudden this disease manifested itself within me. But when I was diagnosed, it was like, okay, I've got PH and Erica was with me with Jo, my partner, but before prior to him actually telling me that we had it, and they'd come into the room to say, Erica turned around and said to me "Dad, wouldn't it be funny if he got diagnosed with PH?" We both laughed because it's such a rare disease.

We both had a good laugh about that. Then 10, 15 minutes later the doctor come in, Dr. Jay and he said, "Mr. Cowie, I'm sorry to you've say you've got pulmonary arterial hypertension". The initial reaction was me and my daughter just laughed and went, "Okay, it's not the end of the world. We'll just fight it together." We've got up this united front, me and her to fight this disease and do as much as we can to have a normal life, if that makes sense.

Now, I think I've got this disease, but the disease doesn't define me as a person. It's just I've got this disease and it's challenging. It does make your life limited, but you can work around it and that's what I found over these last few years. It doesn't define me as a person. I know I've got it, so I have to do things differently and do things that I can do without falling back into the trap saying "I used to do this, I used to do that" but that's all in the past. I can't change the past now, I can only do what's in front of me. That's I think a good thing in a way for me. Focusing on the now and not looking back.

I think when I first was diagnosed it's like ah, can't do this, can't do that anymore because I've been knackered and plus it could kill me. I can't go mountain biking up in mountains and hills like I used to. I've ridden my mountain bike on the flat, but it's not the same. You don't get that sort of buzz, but also it was a lot harder, right? You know, mountain biking on the flat with PH than not having PH, if that makes sense.

The reason I got involved in clinical trials, I think it's because my daughter. There isn't a cure as such for PH, it's only drugs that can stabilize it or slow it down. My daughter never had that opportunity to survive PH or have drugs that enabled her condition to improve or for her to improve. I think for me, because it's such a rare disease and there's not many people with PH, is that the more clinical trials that I can be involved in, the more data that may help somebody else's daughter or somebody else's son or just somebody further along the line when they get diagnosed with PH. Also, if I do a trial it might also benefit me. Which is a win/win. A win for me and a win for the study into PH in new medication. I think that's what's important to me is to give people the opportunity to use me as a guinea pig, to see what works for PH but also what would work for somebody else.

I think that sort of strive because of my daughter, because she didn't have the opportunity to try any clinical trials and it's a horrible disease, especially somebody young like my daughter. I've seen some children younger than when Erica was diagnosed and it is a hard life for them because they seem to be different but they're not different. They're just people like everybody else.

I think the only thing I can think of really as parting wisdom is you may have this disease, the person you're caring for may have this disease, but it's not the end of the world. It shouldn't define you and life is to be lived whether you've got PH or you haven't got PH. I guess you've got to live your life and I think family's important. Spend time with those that you love and care about and want to be with, friends, family. That's important.

I think we get caught up in the world too much of looking for money or trying to have that normal life. When actually, the closest things that are more important to you are you, your family and making those memories with those people I think. I think the biggest thing you can do is go and enjoy your life and live it. Live it to the full. Live it to the max, whether you have PH or not. I think it's just about enjoying it and making those memories with people. So when you're not here, they have those memories of you just like, I have these memories of my daughter, if that makes sense, you know.

My name is Alex Cowie and I know, I'm aware that I'm rare. Also, I'm a PH fighter, so there you go.

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