PHighter Friday: ​​Starése

#MiraclesHappen

Hello fellow PHighters! 
What an amazing privilege to share my story with you all today.
My name is Starése Coote and I am a 30 year old wife and mother, living in Somerset West, South Africa.

When I was diagnosed at the age of 27, my husband and I had only been married a year and were planning our futures without a care in the world. As you can well imagine, our rose-tinted honeymoon phase came to an abrupt halt with my diagnosis, but even though the last three years have brought many unforeseen challenges, it has definitely created an even stronger bond between us. I am so grateful for his unwavering love, support, sacrifice and strength.


My PH journey began like many others' with breathlessness that slowly increased over time and strange bouts of gasping for air even when doing mundane things like making the bed - clearly not normal, but when you consider yourself to be a healthy young adult with your whole life ahead of you, you don't pay too much attention to the initial symptoms.


At first I was misdiagnosed with exercise-induced asthma, but before I could even start the inhalers, my grandparents-in-law made an appointment with a Pulmonologist to get a second opinion. I am so grateful that they convinced me to go see a specialist because I was quite happy to just accept the asthma diagnosis and carry on. But thanks to their generous offer to pay for the appointment, I went and received a very different diagnos is: Primary Pulmonary Arterial Hypertension. As I am writing this I can still vividly remember how I felt when the doctor explained what that meant, leaving me in a pool of tears with a very devastating prognosis. Most of you reading this will know what it is like to be told you have a very short life expectancy and won't be able to have children etc etc, but I look back at the way I received my diagnosis and realize that the hopeless picture that was painted was not true at all! Three years on and I am living my life filled with abundant blessings - not least of which is my precious baby girl that I gave birth to a year ago! That's right - PH couldn't stop me from fulfilling my dream to have a baby!


At the time of my diagnosis my PH was very advanced and I had reached the dreaded syncope stage. I even lost consciousness once while lying in bed fluffing my pillow! That mini sit-up proved to be a bit too much for my struggling heart, so needless to say I was not in a good place.

We had to leave our hometown in Johannesburg because of the high altitude (Johannesburg is 1700m above sea level) and after a brief stint in the UK, we moved back to South Africa to our current home in Somerset West which is a beautiful coastal town in the Western Cape province.

While in the UK I started taking a very high dose of calcium channel blockers which proved to be just what my narrowing pulmonary arteries needed. My mean pulmonary pressures went from 62 to 27 in a matter of weeks - praise God! And just a quick note on the topic of faith - I seriously cannot imagine being on this journey without my belief in an Almighty God carrying me through the rocky terrain of this mysterious condition.

God has been so faithful along the way.


But let me get back to having a baby after being diagnosed with PH. I just want to say that I in no way advocate going against doctors' orders when dealing with an unpredictable disease and a decision of this magnitude, but this is my story of doing just that.


After my diagnosis, my desire to be a mom was crippling. I found myself deeply depressed and I hate to say this but I even felt a bit of resentment towards those around me having babies. It was a very dark time emotionally even though my body had physically made a remarkable recovery. I felt normal again and had no obvious trace of this disease ruling my life except for the medication I took each night. In a way it was harder to accept that I wasn't 'allowed' to have a baby because my body felt strong again. I honestly felt deep down that I can do this!

Initially my husband was not on board with the idea which is totally understandable - he had nearly lost me once and was not about to take any chances. If I'm honest, his resistance to the idea and my overwhelming belief that I would be fine put a lot of strain on our marriage, but after about 10 months of visits to my cardiologist that showed that my PH was stable and my heart back to normal, we finally and very prayerfully decided to fall pregnant.


Besides the usual pregnancy discomforts and 17 weeks of terrible morning (afternoon and evening) sickness, I had a very normal pregnancy. I was monitored very closely of course, but all the doctors were amazed how well my body coped!

I was admitted to hospital early because it was suspected that I might have developed a blood clot, but my time in hospital ended up being a really chilled experience of just relaxing before the birth.
It was decided that I would deliver at 37 weeks via c-section  which was scheduled for the 12th of August 2015. All I can say is that seeing our baby girl for the first time was one of the most exhilarating moments of our lives!

I went  straight into ICU for 24  which was just a precaution in case my heart struggled to get rid of excess fluid post delivery. Thankfully I cruised through ICU without any issues and was reunited with our perfect princess, Micaela Grace,  the very next day.

Micaela has just celebrated her first birthday and she is absolutely perfect! We are eternally grateful for the privilege of being her parents and for the miracle her life represents.

Over the past year my pressures have slowly increased again, so I will be going on new medication soon to bring it back down again, but I am trying not to fixate on the numbers. I have learned that the human body has an amazing ability to fight, adapt and recover. Don't lose hope even if you are physically in a bad place right now because things can turn around very quickly!

I love making connections, so don't hesitate to add me on Facebook or connect with me on my blog www.goingwithgrace.co.za

I have loved sharing my story and if you have taken the time to read through it then I am truly humbled. Thank you!

Keep on PHighting brave ones!

A little bit of faith and hope can go a long way.

Love,

Starése

PHighter Friday: Elsa

My name is Elsa. I am a South African citizen, currently residing in Australia. I was born with congenital heart disease and a VSD was closed at age 10. During the procedure it was discovered that I had PH and I was left with a ASD that could not be closed due to the pulmonary pressures. I was not put on any medication at the time and was just sent home.
I suffered from frequent chest infections and in 1986 I ended up in hospital for 6 weeks with pneumonia and water in my heart sack. I nearly died but with the grace of God and my mother's persistence to insist a different antibiotic I pulled through. I started drinking Garlic and Parsley, and cod liver tables every day and until today I never had a chest infection again. Please note that this is my personal experience and in no ways intended as a medical opinion.*
I met my husband and got married in 1993. We were married for 5 years before we decided to start a family, but knowing the risks we consulted a intern specialist before and we were advised not to have a baby and rather seek alternative options. My husband's sister offered to be a surrogate mother and our baby boy was born healthy and strong without any health issues.

In about 2004 I was getting out of breath more easily and was sent to the city to see a specialist. He was brilliant and started me on Adalat, Spiractin and Aspirin. I was advised to start using nocturnal Oxygen too. This was the first treatment I got since 1983 when PH was diagnosed. It made a huge difference in how I felt and the oxygen at night would charge my batteries for the next day. I have always known my limits and would live accordingly but the new medication and oxygen just made my life easier.

I believe in living life as normal as possible and I have been working as a qualified hairdresser. When we moved to a rural location I opened my own hair salon from home, and worked full-time from 9-5 as a stylist. I had a full time maid that would help with chores in the house. This way I was free to work in my salon.

In 2011 PH south Africa was established and I was asked to be on the committee to raise awareness. We had a very active group, but in 2013 my husband was offered a position in Saudi Arabia and we moved to Jeddah. At first I thought the heat would affect me, but it did not, and living at sea level made a huge difference in how I felt. I had more energy.

I have joined the Saudi Association for Pulmonary hypertension as a honorary member assigned to Patient support. As a foreigner to a closed Muslim community that was a very big honour. I have learned a lot about the disease when I was invited to the International seminars we had in Oman, Abu Dhabi and Jordan.

In 2015 my pressures went up to 120 and I was put on Sildenafil as a treatment. I am using 75 mg of Viagra, and my pressures have since dropped to a stable 75.

Earlier this year we moved to Australia and I decided to have a career change. I have taken up a course in Pastoral Care and Dementia, and I am volunteering at the local nursing home 3 times a week.

I have always been a positive person, and I would not let my condition define me. I live life to the fullest, and believe that a positive attitude to live is beating the disease with at least 50%. If you look around you will see people that is worse off than yourself, and that keeps me going and motivates me. I live in a 3 level house and the stairs is a challenge, but I do one level at a time. I go for lengthy walks on a level surface and that is the best exercise for me. It is important to listen to your body and rest when you need it.

I am 43 years old now, and I am still going strong. Don't give up hope!

A doctor once told my mother I would not live to the age of 10....and gosh did I outlive that prediction. Live life to the fullest of your ability, and stay positive and strong. Thank God for every

day as it is a precious gift.

Take care and feel free to join my Facebook page SAUDI ASSOCIATION FOR PULMONARY HYPERTENSION - SAPH.

*The PHight or Flight Project does not endorse or recommend taking any medications, including natural supplements without discussing them with a medical professional. Please be sure to discuss your treatment options with a professional, such as a specialist. The PHight or Flight Project does not offer medical advice, and the opinions are based solely upon the writer.

PHighter Friday: Tina P.

 Pre-Transplant,
during my stable years.

I met Joel in high school when I was 16 years old. We were young, selfish, foolish, naïve and completely unaware of what life had in store for us. I tell you this as he is an important piece of the puzzle that is my life. He is the most important piece. Back then, things were great! I was actively playing sports, attending school dances, was greatly involved in school committees, managed a part-time job and all while still living that crazy free-spirited teenager lifestyle. My only complaint health wise was that my left knee and surrounding area was always hurting. But, after a visit with a doctor it was confirmed that it was nothing more than a common sports injury called Patella Femoral Syndrome that would eventually go away. Or so we thought…

After high school, Joel and I left our hometown in Sudbury for the big bright lights of Toronto where we were to further our studies in College. As starving students, walking was our ultimate mode of transportation. But when my walk to school and back started to get more difficult, I started to question whether there was something wrong with me. Joel, however, thought that I might just be out of shape and suggested that I go for a jog with him. And so I tried, and failed royally. It was then that I knew I had to see a doctor. Shortly after, I was diagnosed with a mild case of asthma and was sent on my way with puffers and such to treat it. Me not being a doctor, did not think anything of it. Many weeks had gone by and I still had yet to see any improvement in my breathing, even while on medication. One hot summer day, I had what I thought was an asthma attack while working outside at work. I immediately rushed over to my bag where my Ventolin had been hiding and sucked in a few puffs. But it was not giving me the relief that this medicine was designed to do. In fact, it was making my breathing worse! It was then that I knew there had to be more to this. So I phoned up my family physician and explained how the medicine was not helping any of my symptoms and he suggested I stop taking them. Unfortunately, he never pursued or investigated any further. This seemed odd to me and was quite frustrating, but as a young adult still finding her way into the world, I didn’t want to question my physician. After all, he went to medical school, not me.

Not too long after being removed from the meds, I woke up one night with excruciating sharp pains in the chest which only got worse anytime I tried to lie down. At this point, Joel was getting very concerned and took me to emergency. After several tests were done, the doctors at the hospital discovered that I had what they called an over-inflated lung. Basically, my lungs were being aggravated by something, but they just could not tell what. And so they decided to refer me to a respirologist. 

I saw Dr. Born for the very first time in September of 2003, exactly one year after I started experiencing symptoms. He immediately sent me for a few tests including a heart echo, where they finally discovered that I had Pulmonary Hypertension. But Dr. Born was not convinced that this was my final diagnosis. He truly believed that my PH was secondary to something else, and did further investigations. His suspicions were leaning towards me possibly having a cancer called Mesothelioma or what most people know it as “the asbestos cancer." Now this truly did not make any sense to me as I had never in my life been exposed to asbestos, but weirder things have happened.

I was referred to Dr. Hutcheon, a surgeon who would perform a biopsy on me to either prove or disprove this theory. Dr. Hutcheon however, had some reservations about this possible diagnosis and wanted to explore a notion of his own. What if all this was due to blood clots in the lungs? So before putting me through an invasive surgery, he opted to look into his idea first by sending me for a chest CT scan with dye. Low and behold, he was right! The test showed multiple micro blood clots just chillaxing in my lungs having a merry old day. Thus, I got a final diagnosis of Chronic Thromboembolic Pulmonary Hypertension or CTEPH.

I was immediately sent to the Toronto General Hospital Thrombosis Unit, where they had already begun the steps necessary to start me on a much needed blood thinner treatment. But one mystery still remained…where were these clots coming from? Many tests and examinations following that proved to have no impact at all on unlocking this mystery. I had no family history of clotting, no weird abnormal gene in my blood and my recent activities showed it to be impossible for it to be a lifestyle thing. We were all clueless. Meanwhile, as the focus was primarily on the source of my blood clots, my PH was being completely put on the back burner and was not being treated. After hounding my doctors on that very fact, they finally referred me to a respirologist named Dr. Granton who they had mentioned to me was, and I quote “a doctor that had an interest in PH."

6 months post transplant:Went up and down 
what felt like a million stairs at this arena.
Did it without feeling short of breath at all

To be honest with you, before I met Dr. Granton, my knowledge on the severity of PH was pretty slim. I didn’t even know what PH was until I was diagnosed! It never occurred to me once that this was the very thing that would one day drive my life towards the path of a double-lung transplant. I mean truly everyone was so focused on the blood clots and their creator that I guess in some ways it told me that PH was nothing to be concerned about. Boy was that an understatement on my part!
In the midst of this mess, Joel and I decided to move back home to Sudbury to be closer to family. We were young, afraid and unsure of what to do next. 

The years following were a non-stop roller coaster ride of ups and downs. I had a wacky amount of tests done, saw more than my share of doctors and had way too many hospital stays. And still, no explanation was found on how I got those blood clots in the first place. Many different treatments were tried and played around with to treat both my CTEPH and my embolisms. Dr. Granton even tried his luck on giving me Viagra, but it actually unfortunately made me feel worse and so the medicine was stopped. Throughout those fun filled years, we also discovered yet another mystery; there were random holes slowly forming in my lungs and not one doctor had a clue what was causing it. After several tests were done, one open-lung biopsy, one needle biopsy and what felt like a million sputum samples, they discovered that it was a strange bug that was causing mayhem in my lungs and was the source of all these holes. It is called Mycobacterium Xenopi and it was making itself way too comfortable for my liking. Apparently lung tissue was its food of choice. A hard core antibiotic treatment was in order and it took a whole two years to finally kiss it goodbye! To this day, the doctors still have no idea where I picked up that sucker.

On top of all that, my knee problems had re-emerged with a killing and it was not letting up. In fact, the pain was starting to spread to my calf, my thigh and basically my entire left leg. This problem was supposed to dissipate in my twenties, but it was only getting more aggressive. This was seriously starting to concern me and so after a visit to my family physician, he decided to send me to physiotherapy in hopes that it would help. It was there that I learned that my knee pain/swelling was not acting at all like one affected by Patella Femoral Syndrome and that none of the physio therapist had ever seen the likes of it. Meaning, they had no idea what I had, but they were certain it was not Patella Femoral Syndrome. What was I supposed to do with this information?  

By November of 2006, Joel and I had had enough with our lives completely evolving around my

4 1/2 months Post-Transplant, bought this cup
specifically because of the saying. So appropriate.

health. We desperately needed a change and we wanted to finally start living! So we picked up our bags and made the grand move to Ottawa. Why Ottawa you ask? The better question is “Why not?”.
After finally getting settled in our new home, my thoughts started to drift towards what the physio therapists had said about my knee not being my original diagnosis and so I decided to finally get a second opinion from another orthopedic surgeon, Dr. Habib. Upon meeting me, he immediately decided to send me for the one test that I had yet to go for, an MRI of the legs. I was truly relying on this test to give us some kind of answers as I was frankly getting tired of my body producing so many unsolved mysteries. My body unfortunately did not want to wait the few weeks till my appointment as the pain was progressively getting worse and was actually starting to affect my ability to walk.

Joel acted out of concern once again, and took me to emergency. Over the years, I have dealt with many difficult situations involving doctors who are unaware on how to approach my rare diseases and this emergency visit was no different. After explaining to the doctors not only the pain I was feeling in my left leg, but my entire health situation, they unbeknownst to me, sent me for what is called a D-Dimer blood test. Without giving me any explanation at all, in a very panicked manner, they arranged for me to see the Ottawa Thrombosis Clinic the next day. In fact, they said if I didn’t go first thing in the morning, they would admit me to hospital. Like many, I am not a very big fan of the accommodations a hospital can offer and so I gave them my word and went on my merry way. The problem was that the much anticipated MRI was scheduled for that very morning! So I decided to go to the MRI first. Now this is probably not one of my proudest moments, but in all honesty I truly believed at the time that the ER doctors were overreacting slightly. I mean, it would not have been the first time and in my defense, they really didn’t explain to me why they needed me to go and what the test results showed. So going by that, I really felt that the MRI was more important. Of course when I didn’t show up at the thrombosis clinic first thing in the morning, they immediately called me to express how important it was that they see me. And so Joel and I made our way there.

Once we arrived, it was clear to me that the ER doctors failed to mention some important information to me as I was instantly lectured by both the admin staff and the doctor on how irresponsible it was to disobey the doctor’s orders. Apparently, my D-Dimer test results were astronomically high which indicated that I was perhaps clotting. And after a leg doppler was performed, it was indeed confirmed that I had a DVT in my left leg, which would absolutely explain the unbearable pain. But I was on a fairly high dose of blood thinners, so how was this possible? Well apparently that dose was just not high enough. From that moment on, Dr. Forgie became my regular hematologist and would for years to come be involved in my care. After a few adjustments to my meds, Joel and I were finally sent home.

In early February of 2007, not even a month after my first encounter with Dr. Forgie, I landed yet again in emergency with severe chest pains and a serious lack of oxygen.  After a chest CT with dye was done, the doctors were able to indisputably tell me that I had new blood clots to the lungs. So even with the new dose of blood thinners, I was still clotting. At this point, Dr. Forgie was extremely concerned and was determined to find the source of the clots as she felt it was the only way to solve this matter. By this time, the results to that very important MRI I had gone for was finally in and it just so happens that Dr. Forgie had access to it because it was done at the hospital. A lucky find indeed, as it showed that I had a very large Arterial Venous Malformation(AVM) in my entire left leg. What is this, you ask? It’s a knotted mess of extra veins and arteries all balled up together. It’s very rare and is mostly found in the brain, and so to find one in my entire left leg was definitely an interesting find. Apparently, I was born with this lovely thing and it has been shooting baby micro clots up to my lungs since birth. But as the clots were very tiny, it took a whole 19 years before my body seriously started reacting to it. The only true indication that I had, but would have never known was the pain I felt in my leg back in high school. Who would have thought?  So, an IVC filter was inserted to protect my lungs from any new clots and another adjustment was made to my blood thinners. 

The months following felt like a constant vicious battle of me vs. the blood clots. New clots were still forming and so my medicine required even more changes. My IVC filter had magically shifted which meant that it needed replacing and because of the extremely high dose of blood thinners I was on, bleeding was starting to become a serious problem. Life was definitely challenging us in ways we had not even imagined.

In the midst of all that drama, my PH care was transferred over to the clinic in Ottawa where I met Dr. Mielniczuk and Dr. Chandy  for the very first time. There, they decided to try treating my PH with Bosantan or Tracleer.  Unfortunately, after only being on this medicine for a few months the doctors decided to stop it as it was not producing the results we expected and was actually causing severe headaches. So once again, my PH was being untreated, but it was manageable.

From 2008 on, years would pass with my health being pretty stable. I did incredible things and am so grateful for those years! I worked a full time job, traveled to some amazing places, experienced exciting new things like skiing, and did my best to try everything and anything at least once. I enjoyed and cherished every waking moment with friends and family, and had quite a few good laughs as well as some meaningful conversations. I watched as my wacky childhood friends grew up, got married and became extraordinary mothers. My baby brother also made me an aunt of three and I am still very impatiently waiting for more nieces and nephews that I can spoil. But probably the most important celebration that occurred in those years would have to be when I finally got married to the man of my dreams, my high school sweetheart; Joel. We also bought our first home, which was oddly enough down the street from my brother. Life was good and we were so proud of ourselves for learning the tricks of living with CTEPH. In fact, we thought we were pros at it! We had it completely under control. But how can you possibly control something like that?

3 months Post-Transplant, Bought myself a fancy mask to help
with germ preventions. As my immune system is suppressed
now, this is an unfortunate reality of being post-transplant.

By the end of June of 2014, I was finally taking a well needed vacation from work. Joel and I had no special plans, but to relax and enjoy the beautiful June weather here in Ottawa. In essence, we were doing a stay-cation. It was something I was so looking forward to as the past few months had been extremely busy in both life and work, and I was getting tired. My breathing even seemed to be slightly affected, but I pond it off as me needing this wonderful time of doing absolutely nothing. Unfortunately, by the end of that week I didn’t feel any better. In fact, I felt way worse!  In the past, it had always been my clotting issues that had given me a run for my money and so I assumed that whatever was making me feel this terrible must have something to do with that. So I phoned up Dr. Forgie and was seen that day. As per usual, she sent me for a chest CT scan with dye to see if any new clots had emerged, however, the results showed something entirely different. My heart seemed larger and was showing signs of my PH having worsened.  My PH doc Dr. Chandy was immediately notified and an appointment to see him along with a few other tests were scheduled.  He also had mentioned starting me on this new drug that was specifically catered to CTEPH patients and was supposed to make me feel fantastic. It was called Adempas. Sadly, I never made it to those appointments as after another visit to the emergency department I was admitted to hospital. I spent several weeks in and out of hospital that summer. I was however still put on the Adempas treatment, but after a few months of it and the results not being as expected, Dr. Chandy decided it was time to look into other avenues.

So an appointment was scheduled to see Dr. Rubens so that I may be evaluated for the infamous Pulmonary Thromboendarterectomy (PTE) surgery, where they would remove those nasty clots permanently. I had been evaluated for this before but was told that my blood clots were too small and too far to reach and so I did not qualify. At this point, there was no harm in trying again, but regrettably the results were the same. This is finally where the subject of a double-lung transplant came into the picture. By this time I was 31 years old.

Being told that I needed a double-lung transplant in order to continue living was unlike anything I have ever experienced before. 

I felt helpless, scared and yet totally ready for a fight. After all, I had been fighting this battle for a very long time and I was not about to give up now. So in April of 2015, Joel and I picked up our bags once again and made the grand move back to where it all began, Toronto. Here, I would be put on the transplant list and would wait for that amazing life-saving gift. Why was the move necessary you ask? Well, there are only 6 hospitals in Canada that perform this miraculous surgery and Toronto General Hospital, being the closest one to me, requires all patients to live within a 2 hour proximity to them. Finally, after a few complications and bumps in the road, I was officially listed on June 22 2015.

Although I expected the wait to be long and agonizing, it surprisingly went by pretty fast. The hospital actually keeps you quite busy with appointments and physio that it truly helps fill up those days for you and keeps your mind at ease. After a few months of being listed, my health had deteriorated even further and I was admitted to hospital. At this point, the doctors decided that my heart was under way too much stress and needed a little help until a matching set of lungs came in for me. They decided to put me on ECMO, a device used to help deliver oxygen to the body. This would give my heart the much needed break it was longing for. Dr. Granton was back into the picture now and was the lucky one to shockingly tell us that the machine would be hooked up directly to my heart and not through the neck or groin as Joel and I had researched. This meant open heart surgery.  But at this point, what did I have to lose? So I put my big girl panties on and was rolled into to surgery. I was on this machine for 6 days, when the doctors came into my hospital room with best news in the world; they had finally found a set of lungs for me.

I am now 6 ½ months post-transplant and I feel amazing! Words cannot express enough how grateful I am to my donor and their family for this selfless gift they have given me. They have given me time to share more incredible adventures with my family and friends, especially my truly outstanding husband Joel. Remember when I told you that he was the most important piece of this puzzle? It’s because without him I would not have survived this so effortlessly. Without his words of encouragement, his love, patience and understanding, this journey would have been a lot more difficult. I say this because so many people focus on the patient; on their strength and their will and their ability to get through any difficult matter. Yet a lot of these people would not get through these ugly times without the love and support of another. I was lucky enough to have more than one person by my side, including my super amazing parents who sacrificed so much and went above and beyond just to be there for me. But as my husband, Joel was at the center of it all. He was there for me when I could not walk anymore and needed to be pushed in a wheelchair. He was there for me when bending down was virtually impossible without passing out and I needed someone to tie my shoes for me. And he was there for me when life took a sudden turn and we needed to put everything on pause and head to Toronto for transplant. And so this is my homage to all support people out there, THANK YOU FOR EVERYTHING YOU DO!

Joel and I are now back in Ottawa and I feel like I can achieve anything. I no longer fear stairs, or hills or parking lots or walking in general. I get excited over things like cleaning the house or being able to NOT park in handicap parking for once.  I can play with my nieces and nephews without running out of breath and I can sing and dance again!

4 months Post-Transplant,
Went to gala for organ donation awareness.
Danced the night away here!

If I could give anyone who is currently going through the transplant process right now any words of wisdom it would be this… I know it’s scary and you may feel at times like you can’t do it, but the truth is YOU CAN! Trust me when I say this, it is totally worth it! Just stay positive and remember what’s waiting for you on the other side, being able to breathe again, being able to live again! You are strong enough and you are worthy.

Although my journey may seem like a long list of unfortunate events I can honestly say that I am in a way grateful for them. I am proud of my story and how it has shaped me as a person. I would not be who I am today without it and am so happy with the “me” that I have become. Every scar on my body has a tale to tell and will forever be a reminder to me on how precious life really is, and how courageous and strong I can truly be even the worst of times.

*Editor's note: Did you know it only takes 2 minutes to register to become an organ donor in Ontario? Check your status or sign up here http://beadonor.ca/

PHighter Friday: Kathleen

I got diagnosed In July of 2009; by accident. I was under a lot of stress at my job and had developed ulcers. I went in to be scoped at the hospital and the anesthesiologist just happened to be a doctor.

During the procedure my oxygen dropped significantly. The doctor came to the recovery room and expressed his concern about my oxygen saturation. He recommended going to see my PCP right set.  This set off a chain of doctors etc.. I was being treated with Adcirca and Tracleer. For the next year that is all I was on and I was not getting any better.

I did some research on PH doctors and found the Cleveland clinic, where I am from. It is noticed as being one of the best PH clinics in the country. I decided to move back to Cleveland and seek treatment there. All of my family were there too, so they could help me out as well.

I saw a specialist there and he wanted to do another ultrasound and right heart cath. I went in on a Thursday in July for my heart cath. During the procedure the doctor called my PH doctor to tell him my pressures. My PH doctor told them to immediately admit me. I was a little nervous at this, but figured they were just going to do some additional testing. As they were wheeling me to my room I looked up. The floor I was being admitted too was heart failure ICU. It was at that moment I knew this was serious and my life was about to change.

I ended up getting a Hickman line and Remodulin pump.  I was in ICU for a week titrating up. A nurse from the drug company came to the hospital to teach me how to use my pump. Unfortunately, I was too sick from the side effects to even comprehend a little of what she was saying. I was absolutely devastated that I was going to be hooked up to a pump for the rest of my life. All I could ever think about was all the things I could never do again.

For the next six months I was miserable. The pain from the Remodulin was intense, and no pain killer that was prescribed helped. I was terrified to drive places in case I was in an accident. It took me forever to change my bandage and mix my Remodulin. I figured I would be confined to my house forever.

After many tries of pain killers I was prescribed Tramadol and that took the pain away. This was the first sign of hope that my life was not over. After feeling sorry for myself for a few months I decided that I was not going to give up and that I needed to get my act in gear and stop acting like a victim. It was hard at first, but everyday I pushed myself to increase my activity level and get out and spend time with other people.

I decided that I was going to enjoy every moment of my life and not look at life as what things I cannot do, but what I can do. I did not want people to look at me and see a sick person, but someone who is a vibrant person. I used the motto " fake it, 'till you make it".

Today I know I am a miracle. When I was in ICU my PH doctor told me without the pump I would probably not live more than a few weeks. It has been almost six years now.When I see him he is continually amazed at how well I am doing. I know I have gotten better because of my attitude. I see the glass as full.

I am unable to work full time, but I went out and got a part time job. It gives me purpose and also gets me out of the house. I will never be able to run, but I can walk, so that's what I do everyday.

The side effects are still there, but they are not as bad as they used to be, so I am grateful. They are manageable.

Life on life's terms is an important mantra for me. I know I will have some days that I really do not feel good, but I refuse to be depressed about how my body is failing me. I look at it as this is my new "normal" and try to stay focused on the things I can do. Having PH has definitely changed how I look at life. I used to always look ahead or behind, never staying in the moment. One day at a time, one moment at a time. Life is as good and as amazing as I let it be.

Today I am actually in the best shape that I have ever been in. I really take care of my health, something I had always taken for granite. I surround myself will positive people, and people who do not see me as "the sick girl". They do not coddle me or tell me I am too sick to do something. They let me decide what I can and cannot do.PH has also taught me how to ask for help. In the beginning I needed help around the house, cleaning etc...today I do not need any help, but if I should need it, I can ask for it without making myself feel bad.

The list of all the positive things in my life could go on forever. The most important one for me though is my positive out look on life. Without that I am doomed. It is what gets me out of bed in the morning. I look forward to each day.

I have PH, but I do not let it define me as a person.

PHighter Friday: Jenny Janzer

My childhood was a dream, a dream that I woke up from on April 6^th 2001 as I held my parents hands, shaking in a doctors office with ringing in my ears- you know, the stuff of movies, just… not the kind you want to experience. I was only 11 years old when I was told I had Primary Pulmonary Hypertension (That’s what they called Idiopathic back then). I was stage 4 and pretty sick… the doctors told my parents there was a chance I could be dead within 6 months. This week I just surpassed my 15^th year of dominating PH. I never thought I’d make it this far, but I’ve got a fighting pulse within me that refuses to quit.  My name is Jennifer Janzer. The name Jennifer means, “white wave,” or “white shadow,” or sometimes, “the fair one.” There’s a reason I tell you this because for those first 11 magical dream years, I grew up on a lake and from the minute I could, I’d spend every day in the water. I embodied my name- white wave. My mom called my sister and I her little fish. We would snorkel, just lay in the water, rock hunt, swim out over the weeds to sandy spots or catch frogs and turtles while swimming amongst them. I played in the lake, I bathed in the mud, I was an earth-kid, claiming Mother Nature as one of my nurturing parents. April 6^th 2001 I was told I would not be able to swim ever again. It was probably the biggest blow they could have given me at that time. I also had to start on an IV pump right away, which would pump a drug called Flolan into my chest. I had no idea what this meant and went to sleep for the surgery thinking when I woke up, I’d be fixed, not attached to a machine that my life depended upon.

This change skyrocketed me into maturity. I no longer understood what my peers did, boys didn’t matter, the right shoes or clothes didn’t matter, making sure I had medicine with me 24/7 mattered, making sure I changed the ice packs on my Flolan every 4 hours mattered and keeping me isolated is what mattered. My doctors upon diagnosis, whether they meant to or not, instilled a fear within my parents and I that crippled us while I gritted my teeth through middle school. I’ve blocked out a lot. By high school I realized how ridiculous some of these rules were and fought against them like a little rebel. I wanted to be normal. I was in severe denial. I sobbed while watching my friends swim every summer, sometimes sitting on the pier, watching, listlessly chatting. I lost friends at school; people would point and whisper, “there goes the diseased girl.” People feared me- people didn’t understand me.
I was pulled from gym, but actually quite ecstatic about that as I had never been good at it. I was always last for the mile run, passing out and vomiting while we ran laps. Flolan was my lifesaver, but it left me miserable with side effects. I began to call myself the “blood leopard” because of the bright hot painful red rash the Flolan left all over my body, including my face. It’s this hideous splotchy white and red rash. I still have it today. I spent so many mornings as a teen crying and frustratingly trying to cover it up with layers of caked on make up and tears. I’d skip some days of school just because I hated how much I looked and felt sicker than anything. The pain drove deep into my bones and I became severely underweight. The side effects of this medicine make me miserable. I went through a depression and my anxiety grew to the point I became agoraphobic and couldn’t leave the house without having debilitating panic attacks.

At 18, I finally sought out counseling. I overcame that anxiety and so much more. I finally then, reached out to other PH patients, for the first time, breaking my shell of denial. My life began to brighten. I saw so many other PH patients going to school or working jobs. I had finished high school and decided to try college. I’d see doctors and when they’d find out I had PH they would say, “wow well good for you for going to college,” as if they knew I could never finish, but still felt like telling me, “hey, nice try.” It may have taken me seven years but I graduated with a Bachelor in English/ Creative Writing with a concentration in poetry. Writing has been my cure in many ways. 

If I wasn’t sick I’d love to be a geologist however, out in the field, digging up rocks, feeling the earth change beneath me, measuring its growth in sandstone dunes and river-carved valleys. PH took a lot but it also gave me a lot. I learned responsibility, I learned to laugh at myself, and I learned to love every miniscule moment. I know it sounds silly but I never find myself bored anymore… the world is bursting with possibility and even on the days I am restricted to my bed I’ve learned to value it and find ways to occupy myself if even just in my mind. I’ve always had a wild imagination. Any time I’m feeling good or even “okay”, it’s a good day. I no longer carry the denial or angst or hatred I did as a young teen. I grew to accept my rash- and found the best make up to conceal it on my face- (Kat Von D’s Tattoo cover-up make up has been my life saver, I so wish I had it through high school.) As of right now I’m on or have tried pretty much every medication for this disease. My story is so long that if I keep going much longer I’ll type you a novel. I dabbled in SubQ Remodulin for 2 years, but got sicker. However, in those two years I got so much freedom from the mixing and 24 hour watch of my pump that it was worth it. Flolan saved my life again as I went back on it at age 20. I also am on Opsumit, Warfarin, Digoxin, Lasix, Oxygen and my other least favorite- Adcirca; again for the bummer side effects it gives.

I also suffer from Ulcerative Colitis, Psoriasis, Asthma, and had Graves’s disease until I got that raging hormonal thyroid scraped out.  Life hasn’t been easy but it’s taught me to appreciate everything. It also taught me the value of people, true people that care. It’s rare to find a gem amongst all these grains of sand and the select support system I have I couldn’t be more grateful for. They make life worth living. I’ve found other things I love- music, for example. I don’t even care about what I look like anymore or who thinks what of me, I’ll drag my o2 tank right into a punk mosh pit, I’ll haul it around while I dance until I can’t breathe at folk punk shows and rock n roll festivals. I may look ridiculous, sometimes I get weird looks or rude comments but I feel strong. The more I smile, the better I feel. I also found a love in unsuspected places- abandoned buildings. Maybe I feel a connection with them but there is something exhilarating yet calming and just “right” about visiting them and photographing them. I can take the grimiest looking building and turn it into art with the right lighting and angles, or take a decayed peeling rusted post and showcase it’s abstract beauty, forcing people to look at things they would probably never think twice about. Forever, though, writing will be my special outlet to the constant pain PH produces and the constant fear, guilt, and anger it brings into my life.


Speaking of fear, since I was 11 and learned exactly what a lung transplant was, and had to listen to exactly what the surgery entailed, it has been my biggest life fear… and now it is my reality. My family was not ready for this; they have been my biggest support system. If it weren’t for them I wouldn’t be where I am right now. My mom gardens and when I was younger we had to come up for a new name for transplanting flowers because I couldn’t even hear the word “transplant” without having a panic attack. I’ve come a long way since that, but I’m still terrified, and I now need new lungs or I will most likely be dead in 1 to 2 years- or so my doctor said. Yeah, I’ve heard that before, DUDE, but I’m a WARRIOR and I don’t plan on letting this disease defeat me anytime soon. Life has given me a lot of lemons. And I don’t sugar coat it, no, I’m not about to make some lemonade. I eat that lemon, rind and all and spit the seeds out to the sky, grinning.

So, as much as this next step scares me, as much as I shake at night, and quietly sob through every morning, I will get through this, because like everything else I have to and I do. Looking back at what my name means, I now more so embrace the idea of the white shadow and the fair one… fair however, as in I keep a balance in my life. And white shadow as in, I will bring light to even the darkest corners I find myself in. SO! Bring it on, body! You may be trying to kill me, but I am not letting that happen anytime soon!

You can find me/my art and writing on these social media sites:

gofundme for my double lung transplant: www.gofundme.com/2bp6gthb
Instagram: @mostpulp
Tumblr: mostpulp.tumblr.com
Etsy: mostpulp.etsy.com

PHighter Friday Follow Up: Janeris

*Janeris' original post can be found here.

I have been doing great. It's been almost a year since I changed from an IV therapy to pills only. I have remained healthy despite being on a really dose of the new medication. My recent echos and pulmonary tests show so low that they can't be detected. That means my pulmonary pressures are so low there is no concern of the PH being active. I am active. I love going to the gym. The new drug requires me to eat every 8 hours. That on top of my regular meals has caused me to gain about 10 lbs in this past year. I am finding myself struggling to stay focused on eating healthy. I know that eating right has kept me healthy. So I have to always focus on maintaining a healthy diet. Other than that, my life has been amazing. I'm in the middle of 2 adoptions and am very happy. Stress is minimal. I see myself improving every day. My greatest pleasure is being able to motivate people around me to find their healthy place. It has been 7 years since my diagnosis. I'm hoping to one day not need medication.

www.mycleancuttingboard.com
facebook.com/mycleancuttingboard
instagram @mycleancuttingboard

PHighter Friday: Meredith

In February 2015, I received a diagnosis that I long feared.   I was diagnosed with Pulmonary Arterial Hypertension.

At a Blue Lips Foundation's kickoff event

I was 12 years old when I lost my Mother, Bonita, to the same disease.  In 1992, little was known about PH.  The first FDA approved therapy was more than 2 years away.  It was just as common to be diagnosed with the disease upon autopsy as alive.  My Mom was admitted to the hospital on a Wednesday after fainting at a grocery store.  She died the following Monday.  An autopsy revealed she died from right heart failure associated with primary PH.  I was devastated. I no longer had my mother’s guiding hand and heart to lead me through my life’s journey. My mom was 31 years old when she passed away – far too young – to lose her.  To lose her to a disease I had never heard of, and didn’t understand, was unfair and cruel to a young girl.

As I grew older, I dug into PH.  I needed to know how someone so young and vibrant like my Mom could unexpectedly succumb to a disease so unknown and misunderstood.  I researched the disease religiously throughout my high school and college years.  I often wrote research papers on PH.  I allowed myself to wander whether one day I too would be struck by PH.  And then it happened.

In February 2015 I was 8 months post-delivery of my second child.  Following his birth, I was eager to return to the gym, my normal workout and diet routine, and get back to “me.”  The moment I was released by my doctors, I re-started my high intensity workout routine.  I suffered mightily during the cardio segments of my workouts. I experienced extreme fatigue, shortness of breath, dizziness, and nausea – all classic signs of PH.  For months I chalked it up to the “baby.”  By February 2015, however, I could no longer ignore what my body was telling me.  I knew from the years I spent researching PH that the disease may have a heredity component.  I also knew the symptoms I was experiencing were signs of PH progressing throughout my body. 

I will never forget the moment I could no longer ignore my reality.  I awoke in the middle of night to cries from our young son.  I went to his room to rock him.  When I returned to bed, my heart was pounding out of my chest.  I could feel the beats travel up through my neck and down my arm.  My chest was beating so hard and so fast that I felt as though my heart was going to explode out of my chest.  I knew I needed to see a doctor immediately – and I was terrified of what I would hear.
I was placed under a battery of tests to rule out a heart attack and heart disease.  The doctors were pleased when those tests returned negative.  Instead of joy, I was attempting to prepare to hear news I had long feared.

I told the physicians about my family history. Within three days of my initial ER visit, I underwent an echocardiogram.  The cardiologist called my husband and advised him of the results – confirming to him that my heart was grossly enlarged and that a consultation with a pulmonologist was needed immediately.  We were then told the news – “you suffer from pulmonary arterial hypertension.”
My world stopped right then and there.  23 years of fear, agony and prayer for a better result all crashed together from those six words.  I was devastated. I instantly thought of my Mom, my two young boys, my husband and all those people I care desperately about.  I remembered losing my Mom at such a young age, and I didn’t know how to cope with the prospects my boys not having me.  I was inconsolable. 

While my universe was spinning, my husband and doctor immediately began to discuss treatment options.  What I didn’t realize at the time was that I was diagnosed as a Class II patient. Although I was feeling the effects of the disease, I was diagnosed early enough that options were available to me that weren’t so invasive and life-altering.  My local doctor started me on a regimen of oral medications.  I received a second opinion from the Mayo Clinic and a third from Harbor-UCLA.  Fortunately, I have responded well (to present) to the oral regimen, and I remain on those therapies today. 

As time has moved forward, I began to realize that I can manage PH.  Yes, there are days when PH wears me down and beats me up.  I feel it everywhere in my body – aches, pains, chest pressure and fatigue.  I no longer dwell on those days.  I have all the motivation in the world to beat this disease when I look at my family.  They support me.  They love me.  They give me space when I need a break.  Most of all, they tell me every day I am beating PH.  I believe them.  My tests results are steady.  My markers tell my doctors I haven’t regressed.  I beat PH by believing I can, and knowing that the science to treat and manage the disease has advanced beyond measure since 1992. Progress is being made every day.  I believe the scientists who tell us they are finding new and improved ways to treat PH.  The proof lies in the FDA approval of 14 therapies since 1995.  There were zero before that time.

The Blue Lips Foundation was born out of this urgency to provide hope.  I first had to find my own.  I did that.  I then turned my attention back to my time as a child.  I lost my Mom because the disease was not diagnosed in time to allow her to receive a heart and lung transplant.  The doctors didn’t know what they were looking for.  Times have changed.  The medical community now has an understanding (albeit not widespread enough) of how devastating PH is.  Unfortunately, early diagnosis rates remain poor.  It simply infuriates me that with the all the scientific advancements of PH that early diagnosis rates have not improved in 20 years.  That is unacceptable.

PH had bit my family twice in my 34 years of life.  I knew I needed to do something to change the course of PH for other families.  Blue Lips was founded by my husband and me to do just that.  74% of PH patients are diagnosed in advanced stages (Class III, IV) of the disease.  Generally, PH patients will see 3 or more doctors before an accurate diagnosis is made.  The time span between symptom onset and diagnosis is typically 2.8 years.  These statistics are harrowing.  Along with our team, we work every day to change them.

Blue Lips is the only organization in the world dedicated solely to advancing PH early detection and diagnosis rates. We work tirelessly to educate the public and the “gatekeepers of medicine” as to the signs and symptoms of PH, to raise their awareness and understanding that otherwise common symptoms may just be the signs of this medical “silent killer.” Additionally, we fund researchers committed to developing novel diagnostic and screening tools that can be used by the everyday practitioner to “rule-in” or “rule-out” PH during routine medical examinations.  We believe arming family practice physicians, pediatricians and geriatric specialists with easy to use tools will forever alter the course of PH by allowing those who harbor this disease an “earlier” diagnosis. 

It is well understood that those who are diagnosed early in the process, and begin a monitored treatment regimen, stand a far greater chance to be a long term survivor of PH. Until a cure is found, PH patients require as early a diagnosis as possible to continue to live full and productive lives.  My dream is that we, through our work at Blue Lips, are able to give others the same hope I have developed – that even with PH, you can continue to live your life.  Early diagnosis is essential to give others this belief. 

Today, I play with my boys.  I respect and appreciate the precious time I have with them.  Although modified, I work out at the gym and with my trainer.  I travel with my husband. I enjoy “girls’ nights.” I manage our house and home.  I no longer fear that my fate is the same as my mom’s, or that my boys’ fate is the same as mine as a 12 year old girl.  Yes, I have PH, but I am me.  I intend to keep it that way.

You can learn more about the Blue Lips Foundation at www.bluelipsfoundatio.org. Follow us on facebook, twitter (@BlueLipsORG) and Instagram (Blue_Lips_Foundation).

PHighter Friday Follow Up: Haley

In almost just a year and a half since I last wrote for Miss Serena (Haley's previous post can be found here,) Pulmonary Hypertension is quickly becoming a closeted thing in my life. Like a favorite sweater or coat you buy, wear the hell out of it, and after so many years it spends more time in a closet because you're somewhat discovering other fun coats? That's currently my situation.

I don't know how I've had such a great recovery, but I really do believe that recovery dwells within the mind, and spirit; The body will eventually follow. I've gone to therapy, explored medications (whoops - not such a great idea), explored natural healing for my mind, and I really do believe that because I was so focused on how I felt mentally and spiritually that my lungs are now keeping up quite well. While Pulmonary Hypertension will always be an unfortunate huge puzzle piece to my life it doesn't necessarily take the spotlight anymore, and for me that is quite healthy.

Knowing this information, and craving change, I moved myself up into the mountains and into high altitude as well. I came armed with my medication, oxygen, and it's been lovely. While some might think it's negative to have to slow yourself down because of your disease, I think it's wonderful to force myself to slow down, and catch my breath. Life up here is breathtaking in so many ways - not just because of the low oxygen, but because of the beautiful scenery, and all of my lovely animal friends (deer, and more!) I've pushed myself into jobs that are extremely physical, forcing myself to really break out of boxes that my disease put me in. While I know my limits, I'm discovering silly ones that my mind set for itself; something I believe we all unintentionally do. I love visiting my doctors, and watching the surprised look unfold on their face when they see that my breathing is almost normal, and I'm "looking" better.

As a patient of a rare disease, it's hard to force yourself into change. You get used to your doctors who know you inside, and out (quite literally), supportive friends, stores that you can shop in, pharmacies that you may really enjoy the customer service there...it's just hard to pick up and move. Not only do you have to move your furniture, but you have to scope out a whole new medical area, and if they will even be prepared to deliver what you need. I moved to a state that is so behind on two major things; education and medicine. Being an educator and seeing some of the schools in my surrounding area is just cringe worthy. Education is not pushed because of poverty, and how families function daily. Also, the nearest hospital is a little over an hour away from me, and even then it's a tiny hospital that barely has the means to go. It's been quite challenging in both these areas of my life to adjust to these lower standards. However, I love a challenge.

I think my overall theme for these last months of my life has been "challenge". We box ourselves in without even knowing we are doing it, and create kind of false realities of what we can and cannot do because of our disease. Don't be that person. Instead, be a safe person who knows limits, and is careful. A person who also wants to try new things, and do exactly what people think you cannot do. They said I wouldn't live, and I did. They said I wouldn't be able to work, and I have so ridiculously much. They said I wouldn't be able to dance, and I danced my ass off in ceremonies. They said I could never live in altitude, and I'm living quite healthy, and happy. Who knows what is next...maybe I'll soon be on the mountain snowboarding!

We think we know our bodies, and even doctors think they do, but for some reason I'm still the "mystery" patient, as my doctor calls me. Try things, grow, be safe, and actually live. Surviving is no longer an option.

-haley.
phenomenalhaley.wordpress.com

PHighter Friday Follow-Up: Colleen

On January 2, 2016 I hit my eight-year anniversary since diagnosis. This makes me a long-term survivor, I have officially defied the odds.

It’s a curious milestone to celebrate. Of course, I am deeply grateful to be here. There is so much living left to do. The flip side of the statistic, however, is that half the people diagnosed the same time I was are no longer here. It’s a fact I am acutely aware of far too often… watching others with this disease suffer and pass on is  something that has never ceased to cut through me.

Although incredibly hard some days, I can’t stay in that dark space – I think that knowledge is perhaps the most valuable lesson (and skill) I have learned these last eight years. It’s a balancing act between weathering the really hard parts and then getting back up and focusing on the good. In fact, this lesson has been so deeply ingrained in me, I wrote a book on it.

“Defining the New Normal: A Guide to Becoming More Than Your Diagnosis” is the book I published in November 2014. By 2015 it was on the Amazon bestseller list, a feat that not only shocked (okay, and thrilled) me, but one which left me really humbled. I knew when I set out to write this book, that everyone knew somebody who would need it (it’s for all people rocked by a major diagnosis, not just PH). I just didn’t know how many people would resonate the way they have. It’s just… well, it’s just the best thing knowing that this really crappy PH thing can be turned around for good. I am nothing but grateful for how it has been received, and grateful to the Caring Voice Coalition and PHA for supporting my efforts.  And I absolutely love traveling to speak to other patients about how they too can define a life they love, no matter the diagnosis.

In addition, I’ve been given the opportunity to speak to the FDA, Women in Government and PH related industry several times. I really believe it is the patients who have to take our stories right to the change makers, and the policy makers. They need to hear how their every decision impacts our wellbeing and survival.

A book is fun, but the most significant thing in my world as of late has been the adoption of our daughter this past July. We started with fostering the tiniest sweetest little four-pound bundle in November of 2014 (oh yes, she was placed with us the same week my book launched – no pressure there!). Eight months later, in a little courtroom, a judge ruled her ours. Forever.

That moment was literally a dream come true. I had wanted to adopt ever since I was in college. For a long time it looked like PH was going to derail that dream. Questions about how sick I might get, and how bad the outcome might be, kept my husband and I from moving forward.

Finally though, it seemed I was going to be pretty stable for a good long time. I have a kick-ass team of doctors and each one of them was 100% behind the idea of adoption – which was incredibly reassuring.

She and my nine-year-old son keep me very busy. They get most of my energy these days. Adopting was the best decision, but caring for an active toddler and a boy on the move is no joke! I’m writing a lot, and coaching clients all over the country to better health on their terms. I don’t think this journey is ever going to be an easy one. But in choosing whether I PHight of take Flight… I choose to stand and PHight… or sit down on the couch and PHight from there if I have to – because let’s be honest, PH fatigue is no joke either. Either way, I’m defining this life on my terms and it’s a life I love.

*This entry is a follow up to Colleen's original #PHighterFriday, which can be viewed here.

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.