PHighter Friday: Brooke

Ever since I can remember, I loved being busy. So much so, that at 4 years old my parents enrolled me in almost every sport imaginable – dancing, figure skating, gymnastics, swimming, the list goes on and on.

Figure skating seemed to stick and over the next 17 years, the sport would take me on some pretty spectacular adventures. I spent my entire childhood on the ice – 4, 6, 8 hours a day and by the age of 14, I was a national level athlete in both singles and pairs skating.  I represented Canada at several international skating events and spent hours in the gym when I wasn’t on the ice.  My aerobic capacity was second to none.

Which is why I found it alarming, in the spring of 2013 (only a few years after retiring from figure skating) that I was unable to keep pace with my Mother during a charity bike ride.
At the time, I brushed off my inability to breathe as simply being out of shape and found comfort in the idea that more training would help.

Unfortunately, it never did.

Fast forward to February 2014 when my world was forever changed.
It was the morning of the 2014 Olympic Gold Medal Hockey Game, Canada vs the US, and my sister and I were walking downtown Toronto to meet some friends for the early morning game. Out of nowhere, I had to stop. It’s hard to describe what I was feeling in that moment because I can’t really recall. I was fully aware of what was happening around me but I was struggling to even breathe. Needless to say, I visited my local ER shortly after – thinking I would be told I had asthma. Long story short, after almost every test in the book, I was discharged ten days later, with a mystery diagnosis and referrals to both a cardiologist and a respirologist.

It was on March 19th 2014, during my appointment with Dr. Granton at the Pulmonary Hypertension Clinic at Toronto General Hospital that I first heard of Pulmonary Hypertension.

With my PH diagnosis, my world was turned upside down.
Not only was I diagnosed with PH but I also learned that I have two congenital heart defects, both of which made it a miracle that I was able to compete at such a high competitive level when I was younger.

So then came the next dilemma – do ‘we’ operate on the two congenital heart defects (Atrial Septal Defect and Superior Vena Cava Displacement) in the hopes that by correcting these two defects we would stabilize the pulmonary hypertension? Or do we treat the pulmonary hypertension and the congenital heart defects as two separate entities?
Again, after more tests than I’d ever care for, it was decided that there was too much risk involved for the potential reward with regards to correcting my congenital heart defects. They would stay!

To limit the progression of my pulmonary hypertension, I am currently on Adcirca, Tracleer, and Xarelto (even though my PH is not related to blood clots, there are studies that show better prognoses while taking blood thinners). These medications are literally saving my life!
My doctors tried me on Bosentan prior to Tracleer but it was causing some less than ideal side effects.

Growing up, I was an independent, young woman who was comfortable and confidant. I don’t mean that in a cocky, over-pretentious way but in the sense that I knew who I was and was comfortable with who that was. After being diagnosed with PH, I felt as though that had been taken away from me. The body I had known and relied on for the past 24 years had failed me.

I found it hard to let my friends into my ‘new normal’ and instead of relying on my friendships to move forward, I found myself pushing them away.
I hate to burden anyone, and with the state of mind I was in post-diagnosis, I felt as though that is what I would be to my friends. Hindsight being 20/20, pushing them away was the last thing I should have done and the last thing they would have wanted.

Fast forward almost two years later and here I am, better than I was pre-diagnosis but still learning to adapt to my new normal. I still find it difficult to talk about my journey with PH and I have moments where thinking of what the future holds paralyzes me with fear, but I try to remind myself to live in the moment. After being diagnosed, I was so closed off from the real world and guarded with friends and family – I refuse to go back to that.

As backward as this may seem, coming from someone with a chronic illness, I am one of the blessed ones. I’m surrounded by some amazing friends and family who would reach the ends of the earth if it would help. I now realize that that’s pretty priceless!

As a patient, it seems that every time I think I have found my footing some test, complication or setback rears its ugly head.
I won’t sugar coat it, adapting has had numerous challenges. I think the hardest part of my diagnosis was realizing that there was no cure; no way to make the incurable go away. Being sick is being stuck in a limbo of sorts where feelings of anxiousness and depression can creep in at any given moment, with very little notice. The future becomes one big question mark (even more so than any other average 20-something).

And although it’s easy to get lost in the negative aspects of Pulmonary Hypertension, I try to remain positive - for as much as PH has taken from me, it has given surprising gifts as well.

Throughout this journey (that I’ve hopefully only just begun) I’ve met some amazing people. PH has intensified my love of life, taught me patience where I had little, and shown me how beautiful and fragile life can be.

Like I said earlier, I love being busy – that hasn’t changed. I currently work a full-time job (that I love), a part-time job (at a local hospital) and go to school part-time (Go Marauders!).
PH definitely makes this tricky and I find that I tire more easily than before but I’m a firm believer in mind over matter, and until I’m no longer physically able to do the things that bring me comfort (like staying busy), I plan on keeping on!

I don’t know why it takes hardship to gain insight, but it does. And as much as I wouldn’t wish chronic illness on anyone, I’m grateful for the insight it has given me.

Being sick doesn’t only make you weak, it makes you strong. It gives you knowledge – you learn to stop and smell the roses. You know it’s not important to sweat the small stuff because you’ve had bigger things to worry about.

 My life may be far from perfect but I am blessed.

PHighter Friday: Basil

I am a chronic optimist. I look for the positive in everything. It takes a lot to get me mad or upset and even more to shock me. When ever something negative occurs in my life, I try to look at it as “Is there a lesson to learn from this?” I am not a religious man. I do not conform to any religion. I am however, a very spiritual man, and it is these traits of positivity and faith that have mainly taken me from a man told eight years ago that he would need a heart transplant in five years, to the man I am today, with my original heart, still beating in my chest. And for all intents and purposes, it is now once again a normal functioning heart. And from the man in 2009 that was told he had pulmonary hypertension, and that the combination of conditions I had, probably only gave me a less than 50% chance of living to 2014, to the man I am today, now living without pulmonary hypertension and without a short term prognosis.

My journey with my conditions was, and is not an easy thing, but I never got negative. I never once asked “Why me?” I was given this journey, and I did not question why. If I needed to know, the reason would eventually show itself. What lesson did I have to learn? I never asked, because I also learned that sometimes our journeys are not a lesson for us, but a lesson for others.

I am tirelessly asked how did I get over having heart failure and pulmonary hypertension. What is the cure? What did I take? What is the secret? I didn’t go looking for a cure, or a magic potion. I thought about this hard and long and it was one simple change in attitude and thought that got me to where I am today.

When I started on this journey, I researched and researched and researched. I learned everything I could about all of my conditions and I could talk with my doctors in their language. They respected this, and it made it much easier to decide on courses of treatments to follow. And I stuck to the treatments. I became a dedicated pill man. And I would get better, and would have instances where I would fall back. It was all part of the whole getting better thing.

I did meditation. I exercised regularly. Took my medications. Researched what supplements I could take to support my journey. I was doing it all. I was fighting. But there is something I learned a long time ago. Sometimes, the more you push against something, the harder it will push back. But why was I pushing so hard? Was it for me? Was it for my family? What was it for? The bottom line was, I was not ready to die as yet. My wife needed me. I was fighting for my life.

Then, one day, I was going into have a medical test, and I saved a man from being hit by a bus on a New York street, and in the process, his phone was destroyed by the bus. The man was startled, but then he got angry. Really angry because his precious iPhone was destroyed. He started shouting at me. And as I looked into his angry eyes, I realized that it could have been me. I could have been the one hit by the speeding bus and not the one that pulled the man to safety. For all the fighting that I was doing, the research to get over my disease, I could be killed right now by a freaking bus. What was the point? All we have is today. To live in the moment. The here and now. 

That's when I stopped researching and obsessing over my conditions. I let go. I was no longer afraid to die. Yes I still wanted to live. I didn't give up. I just let go. I started to live for the moment, to appreciate everything in my life. I just needed to mind my business, try to do good in the world, and live. Just live.

And that’s where everything changed. I followed the same medical regimen, but I improved. I continued to improve until just this summer I was told I no longer had pulmonary hypertension and that my heart was no longer in failure. I was able to come off some of the drugs I was taking and now I just take four medications. At one point I was taking thirteen different prescription medications.

I told someone about living in the moment, and they asked me what exactly does that mean? Everybody says it. Living in the moment, to me, means to be content. Be content and appreciate all you have right now. We live in a world where we always want bigger, better, more, and that is all fine to want those things. But we need to be content and happy with what we have now. Try to get out of the “grass is greener on the other side” and “everyone else’s sugar is sweeter” syndromes. If you are not happy with what you have right now, when you get the better thing, you will be happy for a while, but it will not be enough. You will want something even bigger and better. If you don’t learn to be content in the now and stop looking for what tomorrow can bring, you will never be content, because when tomorrow becomes today, you will look again to tomorrow for happiness. As far as chronic illnesses go, always remember that God, the Universe, whom ever, never gives us more than we can handle. Just take deep breaths and picture yourself getting better. But be happy with who you are now. Don’t wait to get better to be happy. 

So, there you have it. There is no secret potion to my getting over pulmonary hypertension. And heart failure. Each of us have a separate journey to follow. Some of you will never get better. That's your journey. But never give up. Be positive. Be happy. Let go and live. Just appreciate the life you were given and just say to the universe, to God, to whom ever – “ Thank you. Thank you for this life that I was given. It may be painful at times, but it is better than the alternative.”

By Basil Rene

Basil Rene maintains a blog at http://lifeasananomaly.com, where he has positive posts about his life in general as well as his journey with pulmonary and cardiac sarcoidosis, heart failure and pulmonary hypertension, an implantable defibrillator, as well as a hole in the heart that took over six years to stop leaking after it was fixed. Basil also sends out positive tweets and can be found under the handle @BasilRene.

PHighter Friday: Kayla

Hi my name is Kayla and I am 22 years old.  I am not your average young adult, I have overcome a lot of health issues during my life.  I was born with a lot of health problems including being born with a Giant Omphalocele, (all your organs outside of your body in a jelly sack) hole in my heart, (fixed when I was seven), club foot, scoliosis, and I was tube fed until 5 years old.  I also didn't get to go home from the hospital until I was about 3 months old.  During my childhood I was in and out of the hospital.  After middle school, I had a seven year gap of non-serious health issues.  Until the summer of 2014, when my world was turned upside down. 

I remember it was a hot summer day and I was walking in the mall with my mom and I was walking very slowly and trying to catch my breath.  I thought I was tired from the heat and maybe hungry.  So we made it to the food court, sat down, and got something to eat.  After that I felt better and could walk back to the car.  The next morning I was gasping for air while I was asleep.  I have never experienced that before.  My mom immediately called the doctor and got an appointment that morning to check out what was wrong with me.  When I got to the doctors I was still having a hard time breathing, but I was still talking and alert.  They decided to check my o2 sats and I was in the 60s.  They immediately called the ambulance and rushed me to the ER.  The ER thought I had an asthma attack, but I never had one of those before.  I do have asthma but it's only reactive when I have a bad cold.  So they gave me tons of breathing treatments and it helped a little, but not enough.  The doctor decided to admit me to the hospital.  He told me he wouldn't let me go until he found the answer.  It took a week to do all the tests to determine what was going on.  The last test to confirm their diagnosis was the right heart cath.  That was very scary being awake on the operating table.  I held it together barely.  When the heart cath was done the doctors told me I had Pulmonary Hypertension.  I was confused, scared, and speechless.  I had no clue what PH was.  The doctor explained in detail and still I didn't understand it.  All I remember was it is not curable and I would have to live with it for the rest of my life.  In the past I was use to having health issues and having the doctors solve them the best they could.  But I never thought I would have to deal with a rare and non-curable disease for life.  On top of that I had to be on oxygen 24/7 because of my low oxygen stats and I'm still on oxygen today.  

All of this was new territory for me.  I was in denial for awhile and kept asking my parents why is this happening to me.  They kept explaining it to me and they were really supportive.  Eventually maybe about a couple of months later I adapted with my new way of life.  I have always been a positive person and I knew I could get through this tough time because I have done it my whole life.  The o2 and the meds were what was saving my life.  I was in a new semester in college when I was diagnosed and had to get back to it.  I went back a week after coming home from the hospital.  I had to drop 2 out of 3 classes because I would have been too overwhelmed.  My parents helped out and bought me a portable oxygen concentrator, so it would be easier to go to school and get around.  I even bedazzled my concentrator, to make it more fashionable!  I got through that semester and during that time I knew I wanted to spread awareness about PH.

I never heard of this disease before until I got diagnosed with it.  So I knew I needed to do my part and raise money to help find a cure.  That's when I decided to start my own fundraising jewelry line called PHantastic Jewelry.  I always have been into fashion and love being crafty.  I created my own website (phantasticjewelry.com) to share my story about being diagnosed with PH and to sell my handmade jewelry for awareness.  The net proceeds go to the PHA to help find a cure.  I knew for myself to cope with this "new normal", I had to give back somehow and turn it into a positive.  I enjoy making the jewelry and spreading awareness about PH at local craft fairs and open markets.  Also at these events I get to meet a lot of great people and hear their stories of their own health battles. 

I feel good that when I share my story people are comfortable to share their's.  Some people may wonder how can someone on o2 be doing so much.  Well, since I was born my family, friends, and God have been my support system and my parents taught me an important lesson early on to never give up.  There is always a "silver lining" in hard times.  I strive to do my best and achieve the goals I want in life.  This past year in a half has been intense, but I learned so much.  With being diagnosed I actually have had more energy and developed an exercise routine with the help of Pulmonary Rehab.  I just finished another semester in college and I took 3 classes.  First time having that many classes since being diagnosed.  I feel so good that I am back at where I wanted to be.  I am so appreciative because I am stable with PH at this time and I can focus on school, enjoying being with my family and friends, and working on my fundraising jewelry business.

For the first time in awhile I feel like I can slow down and "stop and smell the roses" instead of being in survival mode.  I hope sharing my story will help others that are having a tough time that it is important to remember take it day by day.  And your life will change a lot being diagnosed with PH, but really try to have a positive outlook.  It truly helps in this situation!  Also allow yourself time to adapt to your new way of life.

PHighter Friday: Janeris

 Pulmonary Hypertension is a disease I had never heard of until it almost killed me. I've suffered with Lupus since 1997. I was 16, a senior in high school, and began having severe joint pains and fevers. I've dealt with the countless effects of that deadly disease for so long, I didn't look too much into it when I started having shortness of breath in 2006. I figured the fatigue, shortness of breath, and rapid heartbeat were just part of it. I went to a couple of doctors who just confirmed what I thought. It was the Lupus. They'd tell me to take more Prednisone for the inflammation and I did. The shortness of breath kept getting worse. I couldn't bend over without getting dizzy. The doctors I saw would say my heart was working pretty hard but for me this was all normal. I went on living; suffering.

I met my now husband in 2008 and our relationship quickly moved toward marriage. I warned him that I had a lot of issues and might not be able to have a baby. He said that he didn't care. He said God would make everything ok.  He was very supportive through it all.  I still worked out every week and was very active in church. My lack of health couldn't stop me.  We got married less than a year after meeting. It was just in time for my biggest supporter to get the test of his life. We chose to get married in Mexico. It was 4 wonderful days spent with family and friends. We stayed 2 more weeks for our honeymoon. For someone with breathing problems, Mexico is not the place to be. It seemed much harder to breathe in Mexico than in Miami. We still did everything we set out to do. We went zip lining, swimming with dolphins, walking in the city and more. Most of it, however was done with my new husband carrying me. Everyone thought it was so cute because we were honeymooners but I was in severe chest pain and honestly just couldn't walk. The last 2 days of our honeymoon were the worst days of the trip. I began hallucinating and having severe chest pain. I thought of calling 911 but was afraid I'd be stuck in Mexico longer than I wanted. I sucked it up and relaxed until we went home. We requested a wheelchair at the airport but still didn't think about asking for oxygen. I didn't know what was wrong. The air in the plane was much thinner and I thought I was going to die.

We went from the airport in Miami straight to the hospital where I stayed for 3 weeks. I just wanted to go home to be with my husband for the first time. My oxygen levels were at 50%. I was immediately put on oxygen. This is where I started to hear that I had problems with my lungs and might need a lung transplant. Once I left the hospital I sought out a pulmonary specialist. I found a great one in Cleveland Clinic who knew exactly what to do. I was quickly taken in for a right heart cath among other important tests. I was finally diagnosed with Pulmonary Hypertension and put on the right medication. I survived on Revatio for a while. But having to take it 3 times a day proved too much for me and within time I had worsened my condition by not taking the medicine properly. In 2011 I started passing out often. I once passed out at a doctor's appointment and was taken to the emergency room. That was the beginning of my new life. There I was put on Veletri, an intravenous drug. It was a tremendous loss of independence and control. It was a severe shock for me.

I was so sad and had a hard time picking myself up. Every night was hard for me. Tears would soak my pillow.  I didn't understand my new body changes. There was constant urinating, swollen ankles, hot spells, weakness, jaw pain, headaches, you name it, I had it. How would I ever get through this? I felt my life was over.  My church family came through to make sure I did get through it. They knew I couldn't cook, clean, or barely move. So for weeks they came and took me to doctor's appointments, cooked, and were right there for me. What a team God placed in my life. I am loved! Another thing that got me through it was something that my Veletri nurse told me. She said I was allowed 5 minutes daily to feel sorry for myself. After that I was to enjoy the rest of my day. Eventually I didn't need those 5 minutes. That was an important part of the early stages of my recovery.

I began to see that light at the end of the tunnel again. I knew there was a purpose, I just had to find it. Time passed and I was tired of being tired. I had gained 30 lbs. from the prednisone and lack of activity and was still having a hard time breathing. But after 3 months it was time to get back to work as a full-time photojournalist at a local newspaper. Slowly, I regained my strength and started looking for alternatives. I found a functional medicine doctor in my area and she taught me the importance of the right diet for my body. My joy came from documenting my journey on a blog  called My Clean Cutting Board. It dealt with showing people what I was going through and how I was improving my health through whole foods. What a difference eating right made. I took out gluten, dairy, potatoes, eggs, coffee, alcohol, and a few other foods that were causing inflammation and a negative immune system response. I started to get better faster than I thought I ever would. I started the gym again full force. I started working at my photo studio, which I run on the side of my full-time job at the paper. I started singing in church again. And less than  1 year later my husband and I began talking about adoption. He saw my progress and together we decided that we were ready to start a family.

Soon the good food changed my body completely. I was taken off the prednisone and the doses of some of the other drugs I was on were lowered. I was so happy. Just 2 years after I was put on Veletri, we became foster parents. Doctors said I was in good health and gave me clearance for this huge task. We got a 2 year old boy in May of 2013. I realized then that all I had gone through was for this moment. You see, he is autistic and was a handful. He cried when he heard loud noises. Not out of fear but out of pain. I couldn't control him or anything he went through. He was a little Tasmanian Devil.  Through my diet change I had learned about the benefits of a gluten and dairy-free diet on children with autism. I cried tears of joy when I realized all my struggles meant something. I changed his diet and he got better. As I was taking care of him God was taking care of me. I continued to get better and they called me to take in a little baby girl. She was 3 months old. I didn't know how I would do this. Here I am with two babies within a year,  health issues, and 2 full-time jobs? Was I nuts? I knew God was in control. He had a plan for me and He wouldn't let me down.

My husband and I have been taking care of our babies for almost 2 years now. We are close to adopting one of them. I have since lost 40 lbs. (They really get you moving don't they?) My husband has been the best thing that could have happened to me. He came at the perfect time. God has a plan for me and I didn't let PH keep me from fulfilling that plan. I could have been in bed feeling sorry for myself. That would have been easy. But I said NO! I got up and chose to change my life. I chose to seek alternatives because I refused to believe that I would need a lung transplant, that I was sick, that I would not survive. I will not be held down. God needed me for something amazing and I didn't want to let Him down. I am beating PH!



My Clean Cutting Board

PHighter Friday: Carson

My journey with Pulmonary Hypertension began before I even realized it.  I thought I was out of shape and needed to train harder. Looking back I now know what I didn’t know then; it was PH and not my lack of training that was keeping me from being able to run as far and fast and long as I wanted.  In middle school and the early part of high school I identified myself as an athlete.  I played soccer and softball, cycled, and ran cross country and track. My stress relief was running. I also danced tap, jazz, ballet, and pointe. 

Because I was so used to exercising all the time I eventually gave-in to the fact that something was wrong, it wasn’t just being out of shape.  I asked my pediatrician for an inhaler and when I said it wasn’t working she sent me to a pulmonologist.  That started me down the 2 year long road that many PH’ers experience when they are originally diagnosed.  The reasons I was given for my trouble breathing ranged from hyperventilation to a vocal cord disorder to psychological pressure I was putting on myself to be perfect.  I was eventually referred to St Louis Children’s Hospital where they did a right heart cath and found that I had PH.  My pressures weren’t very elevated and the docs thought for sure I had secondary PH, so I endured months of testing only to end back up on the operating table for another cath.  This time though they went in through my neck so I could sit up and ride the exercise bike with the catheter in.  My pressure spiked with exercise. I was told I had exercise induced primary pulmonary hypertension (what is now called idiopathic).  At this point they started me on Tracleer, which was at the time a newly approved PH medication, and Procardia XL. 
My doctor told my parents if I was his daughter he would get a second opinion, so we did.  I started being seen at Columbia Presbyterian Hospital in New York City.  This was a long way from my home in western KY, but seeing one of the best doctors was worth it.  It was at Columbia Presbyterian that they discovered interstitial markings on my chest CT and recommended I get an open lung biopsy to diagnose what they felt sure was Pulmonary Veno Occulsive Disease (PVOD).  The best place to have this done, they said, was St Louis Children’s Hospital. So there I was, instead of attending my first day of my junior year of high school I was being wheeled into surgery for a lung biopsy.  The results showed PVOD, and a week later I was on the lung transplant list.  At that point lungs were still allocated by time on the list instead of severity of illness, and my doctor told my mom his biggest fear was that I wouldn’t make it to transplant. I spent a couple weeks completely grief-stricken.  I had just been told I had a disease that 150-200 people worldwide were diagnosed with (most people were diagnosed with the disease in autopsy) and that if I hadn’t gotten a transplant by the time I graduated from high school I likely wouldn’t be alive any more.  And as they say, the rest is history…

After those 2 weeks I decided I wasn’t going to let this disease define me.  I was going to be the anomaly. I started speaking to civic groups to raise awareness for organ donation and raising money for the Children’s Organ Transplant Association.   I wasn’t going to let PVOD and PH stand in my way.  I applied for colleges just like I would have without PVOD (although St Louis became a top city on my list of places to be since my doctors were there). I attended Washington University in St Louis and graduated with a BA in anthropology in four years.  My first year there was hard, I had to learn how to take care of myself, and figure out how to tell people about PVOD without them thinking I wasn’t able to do anything.  After I graduated I stayed in St Louis to work at a research center and started riding my bike, albeit slowly, dancing salsa 3 times a week and doing yoga at least twice a week.  These were all things I dabbled in during college but I was able to really pursue them once I was out of school. Another thing that came with being out of school was getting moved from pediatric care to adult care.  This meant I was re-evaluated for transplant and since the qualifications for lung allocation had changed from time on the list to illness severity I wasn’t eligible to be on the transplant list. 

After that year of working I went back to school for a Masters of Public Administration, but not before I did a cross-country road trip with a college friend of mine.  We camped in a tent every night, making our way from Kentucky to California via all the national parks we could (think up to Mt Rushmore and Glacier national park to the redwoods, the Grand Canyon and back to LA.  It was amazing.  I was able to camp and hike and almost forgot I had PVOD. 

When I went back to school I worked 20-30 hours a week at a law firm and took classes at night.  I learned a lot in those 2 years, but I think the most important thing I learned wasn’t in the classroom.  Right before winter break my first year of the MPA I thought I was getting my usual end-of-semester-cold and I put off going to the doctor.  By the time I got home for break I wasn’t eating and couldn’t walk from my bedroom to the living room without being exhausted.  Finally on Christmas Day night my sister said to me “If I felt as bad as you look I would go to the ER.”  Leave it to a younger sister to be completely blunt. So I agreed to go to the ER.  The next morning I had to be intubated and flown to St Louis because my lungs were no longer working. I was sedated on a ventilator for 6 days.  The doctors still aren’t quite sure exactly what happened but some combination of mono and another virus allowed me to have 5 liters of fluid in my lungs.  I was basically drowning.  The lesson I learned with that was to trust my instinct if something feels wrong and that I should always err on the side of caution.

This past year I completed my Master of Public Health at Washington University in St Louis and to my parent’s great relief have been gainfully employed for the past 6 months.  I am now working at a large non-profit research company, Battelle Memorial.  I am doing public health research and am currently working on a study to see trends in childhood obesity and characteristics of programs in communities that have lower rates of childhood obesity. 

My life definitely changed with my PH and PVOD diagnosis.  I tell people that it really has shaped me as a person.  I am a stronger person because of it.  I face adversity with much less fear than I think I would have otherwise. Despite the fact that most PVOD patients don’t respond well to Tracleer, and most have a negative reaction, I am lucky that Tracleer seems to be working for me.  I am able to do more now than I was when I was diagnosed, despite the fact that I was a training athlete at the time.  Today I’m an avid bike rider, I just started rock climbing, and I volunteer with several organizations; 12 years ago I was told that without a lung transplant I wouldn’t be here today.   Management of the disease is still a daily part of my life, but I don’t let it define me or dictate my life.  The advice I try to remember when I have bad days or when I am feeling down is to remain positive and to keep fighting. I’m so glad that I refused to let the diagnosis be the death sentence it seemed. Having a good outlook has been the best thing for me.

Guest Post: Living with CTEPH

Living with CTEPH By Guest Blogger Marilyn

In June 2011 I almost died from multiple small blood clots in my lungs. I spent a total of 22 days in my local hospital before my pulmonologists figured out why I was still clotting even though I was on both heparin and warfarin. In the afternoon of my last hospital day, one of the pulmonologists walked into my room smiling broadly.

“I’ve figured out what’s wrong!” he said. “You have factor V (5) Leiden!” 

Looking at him a little blankly I asked, “What’s that?” 

“It’s a clotting disorder,” he said.

My mind racing, I asked “Does that run in families?”

“Who in your family had a stroke?” was his answer.

I then filled him in on my mother and my grandparents: My mother had Parkinson’s Disease and started having frequent TIA’s (mini-strokes) in the last year of her life. Her father had many TIA’s over about 18 months and died from a pulmonary embolus (PE). My mother’s mother died of a stroke. My father’s mother also had a stroke which contributed to her death.  As I finished my list the doctor told me that factor V Leiden (fVL) is a genetic clotting defect that is fairly common, but is frequently only found in cases like mine of unexplained clotting problems. I went home the next day intending to do some in depth research on the internet.  Somehow the fVL drove the fact that I have pulmonary hypertension out of my mind.

Factor V Leiden is a genetic clotting disorder that can be either homozygous (one defective gene from each parent) or heterozygous (only one defective gene). I am heterozygous. Many people have this defect but never know it because it seems to require a trigger or secondary condition to cause clotting. Even then the person may not re-clot after an acute episode. Somehow that piece of information didn’t stick in my brain – I just knew I had already had “too numerous to count” pulmonary emboli. I didn’t want it to happen again. I might not be as lucky next time and I still have a lot of living to do.

In November 2009 my husband and I retired, closed up our house, moved ourselves, our Golden Retriever, Bonnie, and our Ragdoll cat, Zoey onto our boat and set out to realize the dream we had nurtured for many years. Our boat is a 36 foot motor sailor, which means it has a full complement of sails, but also has a hefty 90 horsepower inboard diesel engine. She was built in Finland for sailing in Northern Europe.  She is solidly built to handle storms with a pilot house and a diesel heater.  We had moved the boat to Baltimore in late October, so in November we returned to Baltimore and pointed our bow south down the Atlantic Intracoastal Waterway. The day we left, the temperature was 27 degrees and the harbor had a skin of ice on it.  We were glad to be heading to warmer climes – we thought.  Unfortunately we picked one of the coldest winters on record to make our initial foray into the world of full-time cruising.  We had friends in St. Mary’s, Georgia who regaled us with stories of this quaint Southern Georgia town.  St. Mary’s was supposed to be our first stop, until it got warmer and we could resume our trek further south.  Once we were here, though, we fell in love. This was 2010, when the housing market had finally just about hit bottom, and there were some nice homes to be had in this area. My husband decided to buy one of them, thinking that it is much easier to sail out of southern Georgia than northern New Hampshire.

We took the boat back to Maine for the summer and prepared to move our “stuff” south. We spent the rest of that year traveling up and down the eastern seaboard by car, truck, U-Haul, and boat. In November we left the boat in Elizabeth City, NC and went to Vermont, to my brother’s inn, for Thanksgiving. He was battling colon cancer, but it never occurred to us that this might be his last holiday.  He passed away on December 23, 2010.

Because my brother and sister-in-law didn’t like funerals, it was his wish not to have one. Instead, as many of his friends and family as were able to attend assembled at the Mad River Glen ski area to remember him and celebrate his life.  My husband doesn’t deal well with this type of thing, so he stayed in St. Mary’s with the animals and I drove north alone. For two days I drove the familiar Interstates and thought of my brother and what he meant to me.  The trip was two days up, two days back and two days at the inn.

So, why have I told you all this?  Because it was all that traveling, especially the last trip, that likely caused the DVT that was found in my right calf.  Part of the reason I got so sick before seeking medical care, was that I had absolutely no symptoms from the clot in my leg. Normally a DVT causes redness, swelling, heat and pain in the affected area. I had none of those.  When I started having trouble with coughing and shortness of breath, I thought it was my asthma, which I’ve had for 25+ years.  Only when I finally couldn’t walk more than a few feet without having severe respiratory distress did I give in and go to the local ER. They found the clots, and my life changed forever.

Once I was home from the hospital I started searching the Internet for more information. In my research I found out that there are a number of genetic clotting disorders, the most common being fVL and protein G20210A deficiency. Rarer are antithrombin III deficiency, protein C and protein S deficiencies and MTHFR, among others. MTHFR stands for methylenetetrahydrofolate. Basically it alters the body’s ability to properly process folate, leading to abnormal clotting.  There are also acquired or secondary clotting abnormalities stemming from diseases like Systemic Lupus and Scleroderma. These clotting disorders involve resistance to parts of the normal clotting cascade and are considered auto-immune disorders.   Now that I have you totally cross-eyed and confused, what does all this mean?

If you have a clotting disorder, and throw pulmonary emboli which then do not resolve as is normal with PE’s, the clots block the free flow of blood to the lungs and cause increased pressures in the pulmonary arteries and back pressure into the right side of the heart. Over time, these higher pressures lead to right-sided heart failure.  This is called Chronic ThromboEmbolic Pulmonary Hypertension or CTEPH, now given its own designation by the World Health Organization.  I have CTEPH. I am now a “lifer” on Coumadin (or warfarin), whose level is checked by a blood test – INR.

As there are a significant number of patients with clotting disorders who have clotted even while taking an anticoagulant, I tended to be paranoid about my INR being less than 2.5 for the first two years after my diagnosis.  It is only in the last year, really, that I have been relatively happy as long as my INR is above 2. In the back of my mind always lurks the possibility of clotting again and the thought that I might not be so lucky again.  For many years, my main concern when looking at my family health history has been that a stroke which didn’t kill me might steal away my ability to think, reason, and direct my own care. This new threat of stroke has led my husband and I to put the boat up for sale and give up our dream. I am thankful for the one year we had on the boat and heartbroken that I must give it up. I simply cannot take the risk of being offshore and having a PE or a stroke.

I said above that I am on warfarin.  I follow a couple of PE pages on Facebook, and a question that frequently comes up for discussion is whether or not the newer anticoagulants are a good choice. This is something that the doctors and researchers are still struggling with.  Warfarin has a few drawbacks, as do the others. Warfarin acts by blocking Vitamin K, important in the clotting cascade. Therefore, intake of Vitamin K in foods has to be closely monitored. That means that most green vegetables have to be omitted or limited.  Also, warfarin levels are measured by the INR, which is a blood test.  Many people find that a major disadvantage.  The drug is also notoriously difficult to regulate in some people (like me).  Luckily, I don’t mind having blood taken so it doesn’t bother me.  One of the major pluses of warfarin is that it has an antidote. If someone on warfarin arrives at the ER or MD’s office with bleeding, the INR can be checked quickly and Vitamin K administered. This counteracts the warfarin, allowing normal clotting and diminished bleeding.

The three newer anticoagulants – Pradaxa, Eliquis, and Xarelto - work by a different mechanism, so there are none of the food restrictions seen with warfarin.  They do, however, have their own concerns. There is no definitive test for whether they are working or not. I find the automatic assumption that you are taking the pill so your level of anticoagulation must be adequate less than reassuring. There is also no antidote to these drugs, although several are currently in development. The average half-life of the drugs is 48 hours. That may not sound bad to most people, but imagine yourself with a hemorrhage. That means not just a little nosebleed or a cut that won’t clot.  Not having an antidote can be a life or death situation. The third disadvantage is cost. Any new drug is very expensive, and sometimes will not be covered by insurance.  Warfarin has been around for many, many years, is generic and cheap.  Having said all that, please be aware that this is my opinion only.  I feel much safer on warfarin than I would on a newer drug.

Until last October, CTEPH was treated with the same drugs as other types of PH. Last October, however, a new drug was approved by the FDA to specifically treat CTEPH. It is called Adempas.  I started on Adcirca (which I got free from the manufacturer) in 2012, then switched to Revatio when I went on Medicare, because it was cheaper.  I wasn’t as pleased with the Revatio though, so when my pulmonologist suggested I try Adempas, I said yes.  I, like many others I have talked to, have had great results with it. My level of endurance has increased and the pressures in my pulmonary arteries went down by 20 points in six months. I am now freed from the restriction of being on oxygen 15 hours a day to mostly using it when I exercise or when I go to bed. I was doing so well, I decided to attend the 2014 International PH Conference and Scientific Sessions in June in Indianapolis.  It was a totally incredible experience. I drove myself there and back, breaking each trip into two days. I returned home ecstatic but exhausted.  It took me three weeks to fully recover, but it was worth every minute.

2014 has been a banner year for me. Last December I started a web page/blog called Of Bad Lungs and Blood Clots (www.ofbadlungsandbloodclots.com). I had discovered the Pulmonary Hypertension Association (www.phassociation.org) website in November and requested permission to use their logo with a link to their site. That email led to a phone call from PHA’s Patient and Caregiver Services Manager and my beginning close involvement with PHA.  I attended Conference, met a lot of new PHriends, participated in the fashion show, and did some writing. I am a member of the CTEPH Advisory Board and recently worked on setting up the personal stories on the CTEPH web page.  In late August I decided to start a new support group here in southeast Georgia.  I am very busy and doing things I never would have believed I would do.  But the best thing is the writing I have been doing.  I feel as though my 40 years as an RN were only leading me to this point in my life, where I get to do what I love and help others at the same time.