Iain Hess - phaware® interview 300

In our 300th episode, Iain Hess discusses his lifesaving double-lung transplant he received two years ago on January 17, 2017. Iain is a former pulmonary hypertension patient from Colorado who was diagnosed at age 5. 

My name is Ian Hess, and I'm here to share my story.

One year ago, I was standing on top of one of Colorado's 14,000-foot peaks. It was a difficult climb to the top, but overall it was one of the most rewarding experiences of my life. It was something that I'd wanted to do for a long time, but never had the opportunity to, because for 12 years I had lived with pulmonary hypertension [PH]. Doing something like this was just simply impossible. But thanks to my physicians, nurses, parents, people at the pharmaceutical companies, friends, everyone, I was able to do such a thing.

Going back to 2004, two weeks before a planned family trip to India, I was diagnosed with pulmonary hypertension. At the time, I couldn't really comprehend what my diagnosis meant, but the doctors essentially told my parents that if I were to go on a flight pressurized to 8,000 feet, it would be a big mistake. So, 10 days later I had a right heart catheterization, which confirmed that I have super systemic levels and I immediately was diagnosed and had a central line put in and was put on a CADD pump which administered Flolan.

For years after diagnosis, the progression of the disease was constant, but it was not frightening. In a way, it was like boiling the proverbial frog in that I couldn't feel the difference day by day, but overall and after time, my body was taking the damage. There was a lot I could no longer do.

Since I was two years old, my family had started me skiing, but as soon as I was diagnosed I could no longer go to high elevations or do anything of that sort. So initially ,that didn't really affect me too much. But as time went on and my siblings started skiing and my friends started skiing, I definitely felt like I was missing out.

Being connected to a central line and pump obviously had many drawbacks. Number one, it would fail if it got wet. Number two, I had to carry a backpack with the CADD pump in it at all times. This proved difficult when it came to do activities, mainly swimming. I would have to wear a dry suit. For me, that was just one of the biggest pains because swimming was always something that I had wanted to do and always had kind of a passion for. But as soon as I had to put on that dry suit, it just took the fun out of swimming for me.

That dry suit did its job perfectly, but on the few occasions I may have ripped it or something, but water did get in on a couple times, which caused the pump to fail or my Broviac to get wet. Which in many cases, which happened many times, I either had my pump fail, which meant me not getting my medicine, which resulted in the paramedics having to come and do whatever they do.

The worst situation with my central line, overall, was probably when my parents were having a dinner party and the kids were running up and down the house, just having a good time. All of a sudden, my dog took one misstep, stepped on my central line and ripped it right out of my chest, basically causing blood and Flolan to spray everywhere. So, my dad called the paramedics and fortunately they came in time and took me down to the hospital. My dog didn't even bark when about 10 first responders came in just of the shock. In thinking back, my parents had to always be at the ready, so to speak, in case something like this happened because even the smallest situation could turn into a big deal.

Despite many drawbacks, pulmonary hypertension came in handy once or twice during my life with it. Again, once or twice, not often. I would use it to skip a class or turn in an assignment late once in a while. But the biggest positive came from the Make-a-Wish Foundation.

My dream car has always been the Bugatti Veyron. And for those of you that don't know, it was at the time, the fastest car in the world at 267 miles per hour and had a price tag of over $1 million. So, I just loved everything about it. What Make-a-Wish did for me is they sent me to France, to the Bugatti facility in the Alsace region of France in a town called Molsheim. The special thing about this trip, is that it had never been done before, first of all. Bugatti generally only lets their customers on the grounds. So, it was a very one-off experience that I got to have. Yeah, I got to ride in it, which was fun.

Pulmonary hypertension is disease that progressively worsens over time. Like I was saying before, at the beginning of freshman year of high school, I started noticing that walking up a flight of stairs would leave me breathless, gasping for air. You can see in this picture, that my face is very red, number one, due to the constantly increasing doses of vasodilators that I was on. I was also turning a kind of blue color because of the lack of oxygen that I was getting. Basically, at the time I was the color of PH. This is the color of PH.

I dealt with the worsening disease for about two years before my doctors in Denver decided that something had to be done. But what to do was unclear. One option was to pick a transplant facility and go through the application process and then decide based upon that. The other option was to go to St. Louis, Missouri, where they had recently adopted a fairly new procedure in the U.S. called the Potts Shunt.

Essentially what this procedure is just a shunt that basically directs blue blood around your heart protecting it. After visiting various transplant teams, my family and I decided that the Potts Shunt surgery was the best option for me. I can say now that picking St. Louis was the best decision of my life.

During the summer of 2016, my parents took all three kids, both my siblings on a European trip for six weeks. We went to the U.K. and saw family. We relaxed on the Coat du Soir in the south of France and went to various art exhibitions in Italy. Little did I know at the time, though, that my parents had planned this trip out as it may have been our last together. This was the calm before the storm.

I went back to Colorado and then immediately my mom, my dad and I drove to St. Louis. Initially it went pretty smoothly. I had a quick but extremely painful recovery. The incision that my doctor, Dr. Eghtesady had made was called a thoracotomy. It was about a six-inch incision vertically up the left side of my back. In doing the incision there, he had to cut through tons of deep muscle, which just turned out to be extremely painful. So, I was on Oxycodone for months and months afterwards.

The following months after the surgery, unfortunately, proved to be as hard on my body as ever. On one day, October 27, 2016, my memory started fogging up. It was a strange experience, because I couldn't remember, for example, what had happened 20 seconds prior. It was all just a foggy day and it's hard to explain. After that day, I woke up and I felt horrible. I felt the sickest I had ever felt before. I called my dad home. He got home and we checked my oxygen stats and sure enough, they had dropped to an all-time low of 75%.

We quickly took the car down to Colorado Children's Hospital where my cardiologist, Dr. Ivy, unfortunately found out that the Potts Shunt had not worked properly. I was too late, basically. At that point, the only option that I had was to go back to St. Louis to have the double lung transplant.

Two months went by and it was departure day. I exchanged emotional goodbyes with my friends at school and then later with my brother, sister, and mom. Then, my dad and I got in the car and took off for St. Louis. I can remember sitting in the car, looking back at the sunset over the Colorado Rockies and thinking man, I really hope I see this place again.

For about the first month in St. Louis, I had just constant appointments. Once that was done and out of the way, we were just kind of sitting in my apartment for two months and I was bored out of my mind. I accepted it, as even the slightest activity would completely wipe me out. I ended up staying in my apartment, basically just watching Netflix or playing video games. Because at that point, I was living a miserable life, effectively in prison by a failing heart.

When your body is fighting to survive, then there's no energy for you to actually live life. Your mind loses hope and your survival instincts turn on. You lose your sense of empathy for others and focus your energy on staying alive. You start to eat less because your body is physically shutting down. You know that somebody has to die for you to have a chance to live, and frankly and morbidly you wished for that. It's a depressing thought, but it's frankly the truth.

After about two months, of waiting on January 17, 2017, I got the call, a new pair of lungs were on their way in. The day they called me into the hospital was a day I'll never forget, I was just sitting in my room in the apartment, my parents in the room across from me, and all of a sudden, I heard commotion in the other room. I thought, what's going on? It's like 12:00 at night. Then, all of a sudden, my parents came into my room with a video camera on saying that a pair of lungs had been found and that we had to be in the hospital by 2:00 AM.

I got super excited, obviously. I got dressed, I got my shoes on, I got ready to go. I went into my parents’ room and they were still in bed. I was like what are you doing? But they had thought, and rightfully so, that the next couple of days they would be getting no sleep, so they wanted to get a little bit more sleep before we finally went in.

We ended up making it out of the apartment by 2:30, and I thought we were beyond late, but it turns out we weren't really. When we entered the hospital, we found the cardiac floor and they told us to go up, where I was initially met by my first nurse, Alyssa, who put an IV in me and basically told us the same thing, just get some sleep because the next couple of days were going to be extremely long.

Then the morning of the 17th, doctors made their rounds. Basically preop was initiated from the moment I woke up. Various units of the hospital stopped by and then around 12:00 PM they wheeled me to the elevators where I handed off my glasses and gave my parents final hugs, not knowing if it would be the last. Then we went down. The anesthesiologist took me up to the sleepy drugs and that was it.

What happened during the next month and a half are virtually nonexistent. Well, they are nonexistent from my memory. This was due to obviously the many sedative medications that I was on, and I can only recall what my parents have recounted.

Although the lungs were perfect on arrival, I ended up being on the slab for about 17 hours, because the doctors couldn't stanch endless bleeding. During that time, two massive blood transfusion protocols had to be called in, which means it's 50% of your blood replaced over a period of four hours. Only when they could stop the bleeding, which turned out to be about five days later, could they close my chest up. That was really the first battle that I faced during that time.

Soon after this, I was put on ECMO and dialysis because my kidneys had failed. After that, I had pneumothoraxes, ICU delirium, strokes, a seizure. I had vagal nerve damage, which is just basically damage to [the] area around your throat. So, I was not able to talk for a while. I also had something called neuroleptic malignant syndrome, which is a reaction to a specific kind of antipsychotic drug, which basically shot my temperature up to 107 degrees. At 107 degrees, you're very close to melting your brain. And the doctor had actually comforted my mom by telling her that the brain only melts at 108. It was an intense 48 hours of ups and downs, constantly being refrigerated and unheated. Fortunately, I don't remember any of that.

Looking back, my life has been a roller coaster of ups and downs, never knowing when you're going to rise and never knowing when you're going to fall. When I was initially diagnosed, I didn't understand what my life was turning into. Over the years as I grew and gained maturity and awareness, I also lost a lot of hope for the future. I didn't know how long I would live, but that's just something that kind of grew a part of me and I still have that mindset to this day.

At end stage pulmonary hypertension, when my body was dying and my mind weakening, I never gave up. I just kind of accepted each day as it was. In the end, I made it through. The nurses provided miracles, the doctors provided miracles. My friends and family provided plentiful support. In the end, no one knows how long they will live for. So, my overall mindset changed through this process. I kind of asked myself why do I need to rush through life so quickly? I came to the answer -- you don't.