PHighter Friday: Kris Ross

The road to diagnosis was incredibly long and frustrating. I was mostly symptom-free as a child. I would get blue lips when I was cold which was attributed to being so skinny. In my later teenage years, I started having breathing difficulties when I would do physical activity and when it was cold out. I was told I had a reactive airway and an intolerance to cold so I should use an inhaler before going to the gym and before going out in colder weather. My blood work indicated high hemoglobin so I was sent to a specialist who did some nuclear medicine tests. He diagnosed me with “couch potato polycythemia” --- basically, my hemoglobin was high because I was lazy and didn’t do very much physical activity.

Over the next decade, I signed up for gym memberships I couldn’t use, despite my best efforts, and tried different inhalers. I had high hemoglobin results on my blood work during this time period but I was assured I was fine and my doctor told me he wished all of his patients could be so healthy. Finally, after a couple of my colleagues told me I didn’t sound like I had asthma or a reactive airway during a basketball game, I started questioning things and decided to go to a walk-in clinic to explore things further.

In July 2002, I went to see a doctor at the clinic. We had never met before but he listened to my concerns and sent me for a chest x-ray and scheduled some cardio-pulmonary tests and an arterial blood gas. The x-ray was deemed normal but my lips and fingers turned a bluish-purple shade during the cardio-pulmonary tests and I had pain between my shoulder blades. My arterial blood gas was 44. I was sent back to the clinic immediately and, once I convinced him I had never been a smoker, the new doctor told me the only things that would give me these types of results were a major heart or a lung problem. I was scheduled to see a respirologist the next week. He determined my x-ray was not normal because my pulmonary artery was enlarged. He felt the most likely reason was an undiagnosed hole in my heart.

Within two weeks, I met with a cardiologist and had my first echocardiogram. It revealed a large ventricular septal defect (hole in the wall between the two pumping chambers of my heart). Over the following three weeks, I had a number of tests, culminating with a right-heart catheterization. It was determined I had pulmonary hypertension caused by the hole in my heart. It was left open for so long that my lungs were irreparably damaged. To say this was devastating isn’t enough to really capture how I felt. The future I had imagined for myself was gone and, in its place, I was left with fear, anger and uncertainty. The road to a diagnosis took a decade to travel and there was some thought that had I been diagnosed when I first began exhibiting symptoms the hole could have been repaired and the lung damage minimal. My body was my enemy.

There were very few specialists at the time so I was sent to Toronto for assessment. I was told, untreated or unresponsive to treatment, I might have a couple of years before I would need a heart-lung transplant, if donor organs were available. Thankfully, the medication worked well, stabilizing things, and I was able to return, mostly, to the life I’d known before. There were things I would never be able to do and there were things I had always wanted for myself that would never happen. I mourned them and had to move on, living life as fully as possible. My family and friends were great supporting me, adapting things so I could be included in their activities and events. Eventually, they opened a Pulmonary Hypertension Clinic in Ottawa at the Heart Institute, closer to my home, and I starting being treated by the fabulous team there.

I was stable and leading a relatively normal life for years until many bumpy months last year after a nearly fatal case of pneumonia. I had excellent care at the Heart Institute and from family and friends. With some medication changes, months off of work and a staggered return, I was able to get back to my pre-pneumonia state --- even better in some ways.

It’s 2016 and I continue to be relatively healthy with the support of family, friends and the team in Ottawa.

PHighter Friday: Colleen S

In the PH community, I often hear people being asked what their life was like before diagnosis. I've been asked this myself many times over the years. The thing is, my life before I was diagnosed with PH was the same as my life is now. I don't know what it's like to breath normally. I don't have a clue what going from an active lifestyle to a PH lifestyle is all about. That is because PH has been my entire life. It's all I've known. I actually thought, as a kid, that it was normal to take hours to recover from running around with your siblings and friends. I thought everyone took naps when they were exhausted! And although everyone around me, family included, always told me I looked purple, to me that was just my thing!

I was diagnosed when I was 9 months old, but technically, I should have been diagnosed months earlier. My mom brought me to a free clinic to get my first set of immunization shots, and a doctor there thought something was up with my heart. My mom took me to the pediatrician, who didn't think anything was wrong. So at the same free clinic for my 2nd set of shots, the same doctor who saw me the first time told my mom that something was seriously wrong with me, because I looked blue. My parents ended up taking me to a children's hospital, and after many tests, they discovered I had a pretty bad congenital heart defect and pulmonary arterial hypertention. At that time (1975), the doctors couldn't do anything. It was too late for surgery to correct the holes in my heart, and there wasn't anything to treat PAH. My parents were told I might not make it to my first birthday, or I may possibly live til I'm 50. They weren't given much hope! But here I am, 39 years later, and I'm still living to share my story!

First Airplane Trip

I grew up with a lot of "cannots." I cannot participate in sports. I cannnot take gym class. I cannot go on rides that'll get your heart rate up too much. I cannot get pregnant. And so I lived my life following the "cannots." I often wonder if that really was a good thing or a bad thing, but I guess since I followed those rules pretty closely, and I'm still here, it was a good thing? I followed my grade school dream of becoming a teacher, getting my Bachelor's and eventually ending up in a preschool classroom. I loved it! But after only a few years, those preschooler germs started loving me. Too much. I was on my third major respiratory illness during my third year of teaching when my primary doctor came into the exam room with tears in her eyes and told me I had to quit my job. She wrote me a note that I took to my director right after the appointment. I was 24 at the time, and never even thought about quitting a career at that age. Who would??

A few years later after my PAH symptoms continued to get worse, I ended up going to the Cleveland Clinic in Ohio. I was referred by my doctor to go for a transplant evaluation. At the time, the shortness of breath started happening more frequently (just getting dressed in the morning was becoming a 30 minute event), and an elephant had permanently moved in on top of my chest. That was one symptom I didn't have growing up, and it was not a symptom I particularly enjoyed! Being sent to Cleveland was very scary. Transplant was even scarier!! But my first visit, I was sent back home after so many tests with instructions to start taking Coumadin, a blood thinner, and to wear oxygen all the time. After several more visits to Cleveland in the following months, I was put on Tracleer. I had no idea what it was, or exactly what it would do. All I knew was that I surely hoped it would help my elephant to move on, and that I would start feeling better!!

My first shipment of Tracleer brought me not only the first medicine I'd ever try for PAH, but it also brought me into the world of the pulmonary hypertension community. I grew up knowing I had PH, but it was something that was never focused on. My heart condition was the central star all those years. Once I found the Pulmonary Hypertension Association website, my knowledge of PH started to expand. But what really hit me?? THERE WERE OTHERS LIKE ME!!!! I seriously spent a week reading the message boards and crying, because there were people I could relate to and totally understand!! It was like finding a miracle! 

Tracleer got that stupid elephant to move out several months after being on it, and I also didn't feel as short of breath doing certain things like I was always used to feeling. It was a pretty awesome feeling for a few years, until I felt like I needed to add another medication to the mix. Revatio was added, and the combination of the two is what I am still on today. Pulmonary rehabilitation was something I found out about from phriends (friends who have PH), and after inquiring about it, I started going twice a week at one of my local hospitals. It's been a little over six years since I've started pulmonary rehab, and I truly believe it's a part of what's been keeping my PAH stable in the last several years. Kinda hard to believe I went from no gym or sports as a kid/teen to working out a couple times a week at least! No, I am not running marathons or doing heavy weightlifting, but that is ok! Exercising at any capacity is beneficial, even if I can only do it a little at a time!

So after my PAH seemed to be stable for awhile, the next part of the equation was....what am I going to do with my life?? I couldn't teach. I knew I'd never go back to that. But I didn't know what else to do. Some days I had more energy than others. I'd have one day where I wish I could be at a job, to several days when I was thanking my lucky stars that I didn't have one. Then one day I got a call that started the ball rolling for me towards a direction I never thought about. I was asked if I'd consider starting a support group for PH patients in my area. At first, I was pretty terrified. I could handle being around kids, but to lead a group for adults??? I guess you could say that my shy background made the whole prospect a bit daunting for me! I really had to think about it. So, that's what I did. I spent a couple weeks thinking and praying and talking to my loved ones about the idea. I finally decided to go ahead! Yes, I was incredibly nervous about it, but I felt it was something I just HAD to do. How many years did I live with this disease by myself?? I just knew I had to at least give others the chance to not feel so alone after their diagnosis! 

In the 10 years since starting a support group in the Niagara Falls/Buffalo, NY area, I have really become involved with helping PH patients. I've had so many meetings, a large variety of topics and speakers, and phriends I care about very much. I have also become a big part of the online community for PHers, by being a PHA mentor, a chat leader, a blogger, and just a presence on Facebook. I had always wanted to teach in a classroom, but it took me awhile to realize that a "classroom" didn't have to be a place with 4 walls. It could be anywhere! I have even educated complete strangers during shopping trips about PH! And, in the 10 years since starting my support group, I have also learned to not be so shy with people. Sure, I can keep quiet sometimes, but more often than not, I put myself out there and let others know about this awful illness. Awareness is the key to so many answers, and I never know when one day I will talk to someone and they tell me they have PH, too!

My life with PAH is something I cannot change, and honestly, I think it has helped shape me to be the person I am today. Do I have days when I am angry about having it? Of course! I allow myself to have those angry days, or days of self-pity, but not for long. What good does that do me? I have learned to deal with this disease, and to cope the best I know how, and I have learned to help others going through the same thing. I have also not let PAH BE my entire life. I enjoy things like reading, painting glass, making jewelry, spending time with family and friends. In the last few years I have allowed myself to take on challenges I never thought I could do, because I always had that "cannnot" attitude with me. So I've gone on hikes, I've been on a jet ski, I took my first ever trip on an airplane! There are so many more things I've let myself try, even if I may not be able to do them again. I try my best not to let PAH take over my life, because if I did, I can't honestly say that I'd be living to the best of my ability!

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.

PHighter Friday: Evelyn

On December 21, 1988 I was born a preemie. A few days later I was diagnosed with a small heart murmur, Dextrocardia. Dextrocardia - is a condition in which the heart is pointed toward the right side of the chest instead of normally pointing to the left. I was also diagnosed with Mitral Valve Prolapse (MVP). MVP occurs when the valve between your heart's left upper chamber (left atrium) and the left lower chamber (left ventricle) doesn't close properly. 

Despite all of this, my parents were told not to worry and that I would grow up to be a healthy young lady. Every year I would just come in to get a check up. As a child I was very active, always riding my bike, running around with the neighborhood kids and swimming. I don’t remember ever getting out of breath.

It wasn’t until my preteens that I noticed my breathing had started to decline, and that I couldn’t keep up with my friends anymore. Even if we were just taking a walk I often had to stop to catch my breath or I was given piggy-back rides. Luckily for them, I was a tiny little thing. But that didn’t stop me from doing everything they did even if it took me a little longer or it meant we had to walk slower because of me. At the age of 15 I was in the10th grade and I had two classes I had to walk outside to get to. It didn’t seem to be a problem until the cold weather started to come in. Once again, my breathing was acting up and I often had to take a break causing me to be late for class. I ended up going to the doctors and they did a few tests to find out I had asthma. They gave me an inhaler and sent me home. A month passed and I didn’t see a difference in my breathing even though I was doing my inhaler as instructed. A few times walking to class my lips would be tinted blue but I wasn’t alarmed because it was the middle of December and it was freezing out.

One morning walking to class I felt myself starting to get a panic attack. The school nurse sent me home. I couldn’t seem to calm myself down so I laid down on my couch to see if a nap would help. I hoped I would wake up feeling better but instead I woke up about ten minutes later drenched in my own sweat. I was coughing uncontrollably. I could feel myself passing out, everything seemed to get blurry around me. I woke up a few seconds later to my mother hovering over me, crying hysterically. I was in heart failure and if my mother wasn’t there to give me CPR I don’t know if I would be here right now telling you my story.

I was rushed to one hospital and then another, finally landing at Children’s Hospital of Philadelphia. I was so weak that I couldn’t talk but I was very aware something was wrong. I pushed myself to not fall asleep again for fear of not waking up. I was 15 years old and I was not ready to die. Children’s Hospital Of Philadelphia would soon become my second home and family. I was tested right away for pretty much everything you can think of. One of my earliest memories was of a Doctor coming to see me in the Cardiac ICU. He told me he thought he knew what was wrong but that I’d have to have a right heart catheterization to officially diagnose it.

A few hours later I woke up with a port in my chest and medicine flowing through it, into my body. As sick as I was I truly did not care what the doctors needed to do. I just wanted to get better and get out the hospital. At this point I didn’t realize how serious the situation was. I was finally diagnosed with Pulmonary Arterial Hypertension by my Pulmonary Doctor, who is now like a second father to me. I will never forget when he told me “you’re crying, that means you understand.” He promised me I would walk out of Children’s Hospital but I didn’t believe him. All I saw was a dark cloud over me for the longest time. He was true to his word, though, and I can never repay him for being the amazing person he truly is to me, and many others.

Pulmonary Hypertension can be very hard to wrap your head around. There are many questions to ask and some will always be unanswered. It’s scary for the patient and also for your friends and family. I am truly blessed with my support system. As long as you have people around you that care about you, things will be okay. While I was in the hospital getting used to my new life with PH I met my first PH friend. She is no longer with us but she will forever be in my heart. I’d be lying if I didn’t think I was going to be next. Whether you have PH or not though, everyone’s body is different and that’s important to remember.

I was finally well enough to be sent home. Going back to school with PH was very very hard; being a teen itself can be hard. Your teenage years are when you start the journey to discover who you are and there I was, hit with a curve ball with my health. But in some weird way it has shaped me to be stronger than I ever imagined. I’ve met so many wonderful people due to PH. I introduced PH to every classmate and teacher I had over my high school years. I figured, if I’m going to have it, I might as well make it aware and known. My way of dealing with things was to talk about it and educate others. All in all it makes me value life so much more than I ever would have if I didn’t have PH. Because of it I know what it’s like to be at the bottom. Now I’m at the top.

I wish I could meet everyone who is newly diagnosed with PH and let them know they’re going to be okay and not to focus so much on the negatives, which I know is easier said than done. Always keep your head held high with pride and know who you are. PH does not define you or own your life. Everything will become second nature and fall into place and that port you’re attached to 24/7 or all the pills you have to take everyday will become completely normal and a part of your routine. There will be times when you will question “why me?” and like I said earlier, some questions might never be answered. Just know that even though PH might take you for a ride every once in awhile and you will have some bad days, just go with it and remember you’ve come a long way already.


I’m 25 years old today and just celebrated my ten year anniversary with PH. I’m excited to celebrate many more.

Make sure to follow Evelyn on Instagram to see what she is up to!

The PHight or Flight Project would like to thank Evelyn for sharing her incredible PH journey for PHighter Friday!

* If you have an inspirational PH journey that you would like to share to help provide hope to those who are newly diagnosed, please contact me at phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.

PHighter Friday: Michelle

I was never "normal". I remember as a child, I would lose my breath before the other children. As a teen, I would get tired and couldn't be athletic. At 20 I had emergency open heart surgery to repair a hole in my heart. The Dr told me I would feel so much better. I did, for a short time. It all started again.... short of breath, flushing and getting tired easily. I figured it was because I was over weight and I smoked. That's what everyone thought.

At age 27, came the diagnosis, Pulmonary Hypertension. I was told life expectancy was around 2 years. I was scared and confused. No one I talked to had ever heard of it. I knew immediately I would need a specialist. I did research on the internet, made an appointment, and quit smoking. I started oral therapy and I felt a bit better though I was very depressed. I'm the one in my family that thinks I have to take care of everyone else. I let the depression win and I stopped my treatments. I could not afford the copays for the medicines. I didn't tell anyone this or I would have quickly learned of the help that's out there. 

  
 I continued to get worse. I reached out to another specialist. I was told five years earlier that I wouldn't live more than two years. I had proved them wrong. How much better could I have been if I would have kept up my treatments? That's when I met Dr Frank. He did not sugar coat anything. I was quickly scheduled to have a permanent IV in my chest to receive meds 24/7. I wasn't really given any options. I didn't argue. A month later I walked into his office without the assistance of a wheelchair! He was very impressed and confessed to me that he had thought I had waited too long and wouldn't make it to my follow up. I proved him wrong too!

Maybe I can beat this! I have now been on treatment for almost five years and I am doing great! I went back to work part time. I recently started walking a mile a day. I replaced old hobbies with new ones that doesn't require much exertion. I like canning, knitting and crocheting. I have good days and bad days. But as long as I'm still having good days I will continue to PHight! I have a new attitude about this disease. There is no expiration date stamped on my body! I firmly believe your attitude can break or make you in this disease. I allow  myself down days. It's normal! I have days when I can't do the things I want to do. It's normal! All of this is MY NORMAL. We are all different but I try to stay as positive as I can. I absolutely love talking to other PH patients. I try to keep them positive and strong as well. I could have given in ten years ago. I think I was close to that. I'm glad I started phighting. I now have a 2 year old niece that is my heart! I thank god that my meds are helping. My pressures have lowered tremendously! I have joined many support groups and love being an inspiration to the newbies as a long time survivor! Ten years and going strong!!

Editors note: I saw that Michelle works as an optical technician, which sounds like a very busy job! I asked her a bit about her position and she says she works about 20 hours a week and that she loves what she does.

The PHight or Flight Project would like to thank Michelle for sharing her incredible PH journey for PHighter Friday!

* If you have an inspirational PH journey that you would like to share to help provide hope to those who are newly diagnosed, please contact me at phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.