PHighter Friday: Adam Kingz

Adam with Mary McCarthy,
Clinical Nurse Specialist

Hello, my name is Adam Kingz. Here's a little back story on my life so far. I was diagnosed with Primary Pulmonary Hypertension when I was about 13 years old. The doctors said they think I had it for about 2 years before finding out. The testing was long and doctors had no idea what it could have been. One of the main reasons I started going to the doctors was because I was passing out in gym class all the time.

The day I was diagnosed I was sent to sick kids hospital in Toronto. I pretty much lived in the hospital after that. It was hard being 12 and having PH. As my friends played I sat. They continued in school and even that was to much for me. I was in a wheel chair and on oxygen. The oxygen was a pain to have with me, plus being stuck to the wall sucked. Then they started me on flowlan. I was the first child in Ontario on it. So it was a little nerve raking. The fact that it had to stay cold and be changed daily was quite taxing on my family. I also developed an allergy to all tapes so they could only use gauze on me.

My health continued to decline and I was able to do less and less. I got the call for my transplant August 23. I was now 13. It was 3:00 in the morning and I was whisked away to sick kids. (One of the few times I was home. Of course). After my surgery things became amazing! I could breath, run, play! It was truly amazing! My life was no longer on pause. It aloud me to grow up! I got to meet my beautiful wife and become a father to her daughter. I was only taking anti rejection medication and that wasn't to bad. Besides the fat face from prednisone.

After 13 amazing years I developed a cough. After a few tests the doctors found out that I had chronic rejection. This was heart breaking and terrifying! I was so happy and it was all going to disappear right through my fingers. But then the doctor gave me a glimmer of hope. I was told they do re-transplants. It was scary but it was something. I could keep my family, my life wasn't over. Shortly after my now wife and I decided to try and have a child. I was already a father to a little girl that may have not been mine from blood but she was and is a huge light in my life. So why stop living? We were blessed with a son!!! All though out my wife's pregnancy I got worse. Everyday it was getting harder and harder to breath. When I saw him I forgot about it all. He was perfect. And just one more reason I had to survive!

My wife and I also got married in this time. To see how beautiful she was walking down the isle was incredible! She truly is my other half. My health was getting incredibly bad. Well you wait for your transplant you have to do phsyo. It's to keep you as strong as possible. That way you recover fast after transplant. It's just some light weights, stretching and walking on a treadmill. Well by the end I was barely doing 5 minutes on the treadmill. I could barley walk up 2 or 3 steps. I used a walker and refused a wheelchair. I never wanted to go back in one again. My breathing was so bad I was burning so many calories I was down to 105lbs. That's coming from about 210lbs. Every breath I took reminded me of having PH again. It hurt so bad. Every minute of every day it hurt. After 1 false call the day finally came. I got my lungs! 12 days in the hospital and I got to go home. I pushed hard and recovered fast! Now as I sit here healthy and being able to breath once again I will never forget how lucky I am. I love my life! I am extremely thankful to both my donors. Organ donation is incredible! I get to be a dad, a husband, son and friend.

PHighter Friday: Charity

With the Terezin quartet at The New School in New York City

I'm a doer. It's the way my little soul entered my frequently-challenged body. When I couldn't keep up with my peers, I tried harder. I went to the gym more. I dieted. But no matter how hard I tried to keep up, lose weight and exercise, physical activity just appeared to be much easier for other people. In certain respects, it was a blessing. Turning my attentions to music and academics has been a much more sustainable road to happiness, a career and a life for me. 

I was diagnosed with a stage IV case of IPAH when I was 20. I was transplanted the first time at 26 and then again two years later. Over those years, it became clear that "my" mindset and can do attitude wasn't mine alone. It belonged to many of the people I met who had Pulmonary Hypertension. But once a diagnosis comes, how do we cope? How do we move forward with a life despite the seeming impossibility of our situation? How did I move forward?

The value of worthy distractions

A performance at a TED conderence at the
Hotel Del Coronado on Coronado Island, CA

The grief associated with diagnosis can be overwhelming for an individual and for a family. It's almost like having your funeral while you're alive. But in the midst of the emotional avalanche, I looked towards my most cherished distractions. After diagnosis, my family and I felt lost in the hyper-realism of our word. Everything was so clear; so poignant; so beautiful and painful, it seemed there was little to nothing we could do.  While my specialist in Colorado (Chris Lang at Kaiser if anyone is wondering) was amazing, another specialist told me singing would kill me. Because of the severity of the diagnosis, we didn't know if I had weeks, months or years left but the prospects didn't look good. Almost as soon as we found out, I focused on a project. If I was going to die, I wanted my family to have a record of my voice. I put together the music and found a pianist. A friend who managed a recording studio donated the time for me to record. It was a gift for which I will always be deeply grateful. In those first weeks, it gave me a place to escape the seeming bleakness of my reality. Meanwhile, I began to subconsciously process everything that was happening to me. By the time the recording was done, I was much better prepared to emotionally cope and chart a path forward. 

With my family at the Cleveland Clinic

Focus on the CAN

My physical, spiritual and emotional well-being were largely grounded in a determination to focus on the can instead of the can't. The body is amazing. It learns to cope -- to pace itself -- but I think the only way that happens is if we embrace the possibilities we have as opposed to lamenting over those we don't. Despite the clumsiness of my pump (in my day, it was really big), the shortness of breath, synchope (sp??) and complicated medications, I learned how to really live with PH.  I couldn't run a marathon, but I could sing. I wasn't going to be a great mountain climber, but I could paint. I couldn't eat salt, but good fruit is the best thing in the world anyway and it doesn't need salt. I couldn't climb to the top of the Washington Monument, but I could get another graduate degree. I couldn't play intermural sports but I could run an after school music program for kids in Baltimore. I learned my limits and how to live within them. Eventually, I lived in Italy, Hungary, Israel and in China all while I had PH. I performed in major theaters around the world. I met my future husband and fell in love. By focusing on what was possible, my emotional and spiritual health was excellent which in turn, helped me professionally and my overall physical well-being. 

I Never Wanted A Transplant

A performance in the US Capitol Rotunda

With my lift team at the Cleveland Clinic

Transplant is undeniably amazing. It's also a challenge. First, let's talk about amazing. I never could run. Seriously. From the time I was a little girl, it just wasn't something I was able to do. But the other night, I ran all of the way from our flat to the Lincoln Memorial in Washington DC with my husband. Its something that until I did it, was unimaginable. I never feel like I'm going to faint anymore. This is also a huge deal since much of my life was spent trying to figure out whether an activity would land me unconscious. I can eat salt and people finally really enjoy my cooking -- something that was a challenge when I couldn't add salt to anything. I don't have the pump anymore. I'm married (though I suspect my husband and I would have married regardless of whether I received a transplant since we began dating when I had PH). We just took an amazing trip to Patagonia where I climbed some of the most beautiful mountains in the world. Life is good. The challenge is, this is my last shot. When I had PH, if things went south there was always another medication they could add to my regimen or if all else failed, transplant. There is no safety net for transplant. I was crazy fortunate and my insurers and hospital let me go on the list for another transplant and I got it, but that doesn't always happen. A first transplant is hard enough to get. A second transplant is nearly impossible. The word rejection is super apt

Relearning how to climb stairs after the transplant

because when it happens, it feels like the organs are rejecting you, personally. Its like the old, more vengeful version of God right inside of you all of the time. It knows whether I took my meds on time, whether I ate shellfish, whether I crossed my legs, whether I'm exercising, whether I am doing everything I'm supposed to do. When I rejected, I experienced guilt in a way that I never had before. It reminded me of when I didn't get into the college I wanted to go to, steroids. It was devastating and I felt like I was making my donor die all over again. The process of watching my body break down so quickly didn't give me time to grow used to my condition. It was hard and fast and

cruel. With PH, right before my 1st transplant, I didn't want one. All I wanted was to move to Canada to be part of one of Duncan Stewart's stem cell studies. I could still walk around -- albeit slowly. I wanted a cure, but if one never came, I was happy to spend my life living with PH. I'd already been diagnosed with PH for over 5 years and I was very happy with my life. While I was on loads of meds -- revatio, aspirin, diuretics and flolan among others -- I was used to living with and coping with my disease. What I didn't totally accept until two friends with PH died was that there is a window of opportunity for transplant. Wait too long and it closes. When I crawled through my window, the glass was broken and it left some nasty scars, but it lead to somewhere amazing. There is a story of a young woman who's removed her name from the transplant list in England because the wait is too hard and she wants to travel the world. While I understand her sentiment -- I kind felt the same way for many years -- being on this side of the experience I am so grateful to have made it. My life is filled

Our wedding

With my husband in Palos Verdes, California

with people and things I love. While I can't go clubbing, I can do so many things that I love to do and I am discovering so many other things that were out of reach for many years of my life. Transplant made me grow up. When I had PH, I would imagine a cure and imagine all of the things that could happen. Transplant has taught me that I am the ultimate arbiter of possibility. Every single day depends on me taking my medications on time, exercising, recording my spirometry, washing my hands, avoiding crowded places, wearing my mask on the plane. It has forced me to discipline myself in ways I never thought was possible. It's taught me to be more humble -- to accept help and really lean on the understanding of others. It wasn't my first choice, but it is a huge blessing. 

The Take Away

I want a cure. I hope and dream and pray for one. But for now, I try and focus on what I can do with my doctors, with my community, with my family and my friends. In the last weeks of waiting for my first transplant, I painted a beautiful table. It sits in my front room as a reminder of the beauty that can come in our darkest hours. If we look towards action, it will wisk us away and carry us toward the wondrous.

The table I talk about at the end of the post

Happy breathing.

With my sisters after my wedding

Charity Tillemann-Dick is a best-selling classical artist and writer. Last summer her album American Grace charted 10 times on the Billboard charts including the number 1 slot as top classical album. Along with her popular lifestyle and cooking blog 5istersTD she is a contributor to CNN, Huffington Post religion and arts sections. Currently, she is writing a memoir which will be published with Simon & Schuster in the spring of 2016. You can follow her on twitter and instagram @charitytd. It might take a little while, but she promises to tweet back.

PHighter Friday: Jamie

Post-transplant

I always find it incredibly difficult to explain my PH journey. Even within the small tightknit community of PH, most of our journeys are vastly different. My story starts as a disaster but ends five years later and includes a family, a job, school, and enough energy to walk five kilometers a day.

Let me start from the beginning. Firstly, I was diagnosed late… actually 23 years too late. I was born with a hole in my heart and after countless doctors’ appointments where I was either given antibiotics, or accused of lying to get out of work, I spun my car out on the busiest highway in Toronto, which finally led to my diagnosis.

After going to the local walk-in clinic, and having an oximeter reading done, I was diagnosed within the month with stage IV pulmonary hypertension.

My health for the next four years really only went downwards, oral medication did not work, subcutaneous medication did not work, and when I was finally put on IV Remodulin, while it was true that I was feeling much better, a month in a clot broke off the line and caused a minor heart attack.

The final breaking point before my life changed forever was here in Ottawa. I had chest pain so I texted my nurse coordinator who told me to go to emergency immediately. I was pretty busy, so first my wife and I signed my school loan documents before heading over. This was a VERY bad idea, my saturation levels were sitting at under 50 percent, and I was heading into full organ failure.

The decision my PH team came up with was to fly me to Toronto General for a transplant workup. It turned out that I was so far gone, that they put me at the top of the national registry immediately, and I had a double-lung transplant/heart repair within 48 hours.

As I said this was the shift in my life to make everything better. At the end of three months in Toronto, I was jogging, walking up seven flights of stairs, and doing things that I was never able to do.

 I’m not suggesting transplant is for everyone, certainly it has its up’s and down’s, but for me, I’m the healthiest – and happiest – I have ever been.