Canadian resident, Stephen Gariepy, was diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) in 2018. After initially being active in senior hockey, his condition worsened, leading to difficulty breathing and performing daily tasks. Gariepy continues to live with this rare and terminal disease, adapting to the limitations it imposes on his daily life.

My name’s Stephen Gariepy. I’m from Edmonton Alberta in Canada. I was first diagnosed with CTEPH in 2018. What had happened was I had just recently retired and was beginning to enjoy retired life. I had joined a senior hockey league and was very involved in that playing hockey three times a week. Of course, everything came crashing down with this diagnosis. 
 
Every September I’d go out and do some recreational skating and preparation for the season openers, in October. I had started my skating program. There was a large clock on one of the ends of the rink, and I always use that to guide how my times were doing. I was noting there was a little improvement. I’m going, “Wow, I must have really got out of shape this summer.” So I continued my skating, was getting nowhere with it, so I figured, “Oh, well, I’ll be like the other guys and I’ll just play into shape.” Fortunately, I was able to do a couple of games, but my breathing was very compromised. I didn’t know what was going on. Then, I got a shoulder injury and that pulled me out of hockey and then I could concentrate on why I wasn’t breathing. This progressively really got worse to the point… I live in a two-story house and I was barely making it up the stairs. It was just ridiculous. I didn’t know what was going on. 
 
Fortunately, my GP I’ve had for 30 years, so the familiarity with me and him was very fortunate, because I went in to see him explaining what was going on. He could visibly see I couldn’t even breathe. So we set up a bunch of tests. He was able to pick off right from the original chest X-ray that it appeared that I might have pulmonary hypertension. What he did with that was completed the rest of the tests, sent in the referral to the Pulmonary Hypertension Clinic in Edmonton. I was able to get in there within the usual four to six weeks. I got my appointment there. I met with the specialist. They did a CT scan during the appointment and an ECG. I got lost in emergency somewhere, because they couldn’t fit me in anywhere. I had to go through the emergency system. 
 
During my appointment, finally, around 9:00, I was able to get it done. Fortunately for me, it proved the dedication of the staff at the Pulmonary Hypertension Clinic. It was a Friday night and the doctor waited until it came through. He was going to Disney World the next morning. I don’t know how he did it, but he stayed. Then, he came and got my wife and I from the emergency back to his office, back to the clinic. And he quickly looked at the CT scan and he advised me that yes, I had chronic thromboembolic pulmonary hypertension.

At that point, he told me the only option was if I had an operation, which would have to be done in Toronto, because they had lost their search and had Edmonton. They went at it right away. There was another test that was required for the surgeons in Toronto, which was the heart catheterization and some other test. I’m not sure what they all were. I was getting tested so much I couldn’t keep track anymore. Then, they had to set up a video interview with the surgeon in Toronto. That was Marc de Perrot. Great man, very impressive person. He determined that I was a candidate for a pulmonary thromboendarterectomy. 
 
They also noted that after the heart test and all that, they should probably do a bypass while I was opened up and that would allegedly make my heart stronger to survive the endarterectomy. So from October when I was diagnosed, I was able to get down to Toronto for the surgery. Of course, that came as quite an expense. The government did provide airfare and, of course, I had to travel with somebody, so I brought my wife. Then I had the surgery. It worked out to be, I think it was a little over 13 hours, because they had to do the bypass initially, and then they went in and did it. I was septic. I developed pneumonia twice. 
 
The real kicker here was that I turned out to have allergic reaction to the Heparin and then I developed a blood clot in my leg. The Heparin reaction like that is unbelievably rare. The only way they confirm it is to send your DNA to a laboratory in Hamilton, where then, of course, it was confirmed. To make it a long story short, ultimately it ended up the thromboendarterectomy being futile because I instantly re-clotted because of the Heparin allergy. With all the complications and everything, I wound up in Toronto in the hospital there for 45 days. I came out no better than when it went in, actually. Possibly a little worse. 
 
As I did not get better, I continued on with the Pulmonary Hypertension Clinic and they re-referred me back to Dr. Perrot in Toronto to look at other options. Ultimately, what was concluded was that I was too high of a risk to endure another pulmonary thromboendarterectomy, because of existing scar tissues and things of that nature just made the procedure too risky for me to have.
 
But they did conclude that they could refer me to a vascular surgeon who could look at doing what’s called a balloon pulmonary angioplasty. That was arranged for me to go back to Toronto. That doctor said until he got in there, he couldn’t tell. Whether or not he could proceed with it, because if it was 100% blocked, he had nothing to work with, he couldn’t get a catheter around it. But he said he wouldn’t know that until he went in. But he saw some kind of backflow with the blood and figured he could attempt it. So I was scheduled to have it done again the next day for both lungs. He was able to get through, inserted a balloon in one lung. That worked in opening up one of the arteries. Then, he went in to the other lung and successfully opened that one as well, which was great. Then, we flew home. I felt much better. I was still on oxygen, but that didn’t last very long. 
 
At some point in the future, if I meet the requirements, I will be subjected to a lung transplant, but that’s down the road. I just take it every day by day, wake up in the morning and appreciate that I’m awake. On a humorous note here, what pulled me through the last time through the first endarterectomy was I said, “Please let me live so I can watch…” One of my daughters was just finishing off a degree, and I just wanted to be around for her to graduate. I take this as an omen. She was accepted for a post-graduate degree, but she started in September. So, I figured that’s got to give me two years because I have to see her graduate again! 
 
My wife just recently retired in June, which is fantastic because being alone with this thing, you’re just sitting at home dwelling on your illness, so it’s great. I have my partner back. We’re guardedly proceeding with our retirement and seeing what limitations I have. There’s all the flying restrictions. I have to go through all that kind of crap, but that’s just part of the disease and learning to live with it. 
 
I finally after years and years through the Pulmonary Hypertension Association of Canada, I was finally able to get hooked up with a PH buddy. I talked to him about twice, because he had had endarterectomy 10 years previous. After the 10 years he was deemed PH-free, so he kind of disappeared. I got to start working on that again. 
 
When I was diagnosed with CTEPH, the first emotion is, “God hates me. What did I do?” You get a diagnosis like this. First thing you do, like everybody does, you look it up on the internet. It says one to three years, you’re dead. God. You go through the five stages, right? Anger, denial, until ultimately you’re at peace with it. I know what I’m up against. I’ve been through it all and living day by day. I wake up, I’m happy I woke up. I do everything I possibly can. Of course, there’s good days and bad days. There’s days I can barely get out of bed, and there’s days I feel okay. Of course, if I go to cut the lawn, I’ll throw the oxygen tank on and get out there. I can’t do anything physical just the way it is, at least without oxygen. 
 
The surgery is a major surgery. It is huge. You’re made hypothermic. They stop your heart. They stop your brain. You’re clinically dead for 20 minutes for three periods, so you’re dead for an hour. The reality is that I’m living with a terminal disease. I realize that. I’ve gone through all the stages of grief and everything, and I just carry on. I know there is possibly a chance of getting a transplant down the road. By the time that happens, I wouldn’t even be eligible till I’m 70, and then the whole process will have to be reevaluated again. Until such time, I’m just going day by day, living with it, going through every emotion known to mankind. I got nothing left. I’m just living. It’s such a rare disease, you almost get tired of trying to explain it, so you say, “I’m sick.” There’s no denying it’s hard, what I’ve been through. I don’t think anybody’s been through, but I’m still here talking to you, so I’m winning. You just adapt and do what you can do. 
 
My name is Stephen Gariepy, and I’m aware that I’m rare.