Friday, 4 July 2014

PHighter Friday: Ashley

Professional PHighter

My day started like any other.  It was a Monday and I was outside my office building checking on some inventory levels for work.  It was a frigidly cold Ohio morning. The kind of morning that made your fingers numb, your nose red and made you wish Spring would be sprung sooner rather than later. 

I began walking back into the building and I felt myself getting short of breath.  This had been happening for a while now.  I’d get short of breath going up stairs, walking too quickly, playing with my children, doing laundry, carrying the groceries in the house. I had completely forgotten about push mowing the lawn. Previously, I had been able to mow the lawn while nine months pregnant.  The last time I had attempted it though, I almost passed out after only two laps around my yard.  I thought I was just overweight and that my lack of physical activity was catching up with me.  As a result, I began to lose weight simply by watching what I was eating.  I did lose weight, but my weight loss didn’t seem to matter though.  I kept PHighting for more oxygen. Clearly I was not capable of much exercise. 
As I continued what felt like a never ending trek into work, my heart started to race and I became hot and dizzy.  I knew I should sit down and take a break but it was so cold. My plan was to just make it inside and I would sit down.  My breathing became extremely labored.  I had to PHight and push myself to keep going.  I didn’t make it. I opened the door and stumbled in.  I could feel myself begin to lose consciousness.  As the darkness enveloped me, I went down, hitting my head first on a hand truck and then my body slamming onto the concert floor. 
I think many people have a life changing moment.  A moment in which you look back and realize that nothing will ever be the same.  Maybe it’s a sudden moment of clarity or an untimely tragedy.  This was my moment, the moment which shifted the direction of my life.  I was twenty nine, had a wonderful husband, two beautiful daughters and a blossoming career.  It was March 5, 2012 and this was the day I began my PHight.
I made an appointment with my doctor the next day. I explained all of my symptoms.  I didn’t leave a single detail out. He immediately asked me if I had ever heard of Pulmonary Hypertension. I hadn’t. He ordered an EKG, echo-cardiogram, chest x-ray, blood tests and a holter monitor test. I completed all of my tests that day except for the echo-cardiogram which was scheduled for two days from then. With my mind racing, I went home to research PH.
Incurable. Fatal. Lung transplant, Heart failure. 24/7 IV therapy. Five year life expectancy. These are all words and phrases that describe PH. I was not ready for a single one of them.  How could this be? My five year plan included raising children, spending time with my husband and working. A lung transplant was not part of the plan. While I’m on the topic, neither was dying. I felt such a sense of loss, as if my entire life had already been taken away. I could picture my girls crying at my funeral and my husband, the kind soul who promised to love me in sickness and in health, forging on as a widower. The sickness part of our vows, unfortunately, came far too soon.

Whenever something negative happens in my life, I have a system I use to cope with it. This situation was no exception. First, I wallowed for a couple of days. I cried, I screamed, I cursed my piece of crap lungs. In general; I felt sorry for myself. Once the pity party was over, I tried to figure out how to get control of the situation. I may have PH but it wasn’t going to have me. I decided that I couldn’t be married, raise kids, work, take care of a house and take care of myself with a lung disease. What was the first thing to change? I quit my job. For the first time in my life, I needed to make myself a priority.
In the following days I went in for my echo and got the results back a few days later. My pressures were elevated, confirming that I had PH. My doctor began talking about specialists and treatments. He said he would send me to any hospital or clinic I wanted, but if he had his choice, he would send me to Mayo Clinic in Rochester Minnesota. I asked him how soon they could see me. My husband and I began the first of many twenty hour round trip drives to Rochester the following week. I was finally going to PHight this head on.

When we finally arrived in Rochester, I had a slew of tests including more blood work, another chest x-ray, EKG, echocardiogram, six minute walk test, pulmonary function test, sleep study and the test that would technically prove what we already knew, a heart catheterization. We received the results the next day. I had PH (which was no surprise,) but they determined I was a functional class 4. As if being diagnosed with PH wasn’t bad enough, I was in the worst possible class.  There are few things more depressing than knowing that aside from having a terminal lung disease, my heart had begun to fail as well. 

My doctor then moved on with treatment options. He explained the three different classes of PH drugs. They all fight PH but they do it in different ways. He used the following analogy. When you go to war, you don’t just fight from the ground, you fight from the ground, air and sea. He was going to attack my PH the same way, hit me with all three classes of drugs at once. After my PH was under control, we could look at backing off from some medications.  I credit his plan with saving my life. I went home with a prescription for Revatio, a plan to start Letairis in three months and in two weeks I was scheduled to come back to Mayo Clinic to have my Hickman Line surgically implanted in my chest to begin my 24/7 IV Veletri therapy.  

Of course you cannot discuss PH treatments without discussing a lung transplant. I was told I might need new lungs in a year or maybe twenty. We really would have no way of knowing until I started my treatment. Either way, a year or twenty years, I didn’t care for my odds. He wanted to start the work up for the transplant in a few months from then, in case I needed it sooner rather than later.

Getting used to taking my new pills around the clock wasn’t too much of a daunting task. The IV is what I despised with every fiber of my being.  It wasn’t just being permanently attached to a lifeline; it was everything else that came with it. I had to become my own nurse, mixing my medication, programming the IV pump, making sure there were no air bubbles in the line and having to change my protective dressing. 

I hated how my life now revolved around this IV. I had to eat somewhat regularly otherwise I’d get sick. Unfortunately, I also got blinding jaw pain when I ate (that’s not much of an exaggeration) so that was always something to look forward to. If those side effects weren’t enough to deal with, there was also diarrhea, headaches and flushing to the point that I looked like a Buckeyes fan without even having to paint my face red.   I couldn’t go swimming with my kids. I hated having to be tethered to my IV by four feet of tubing while my pump hung on the outside of the shower as I was inside trying to keep my dressing dry with aquaguards and cling wrap. I despised the horrible fanny pack I had to wear to keep the pump on me. I wanted to scream at the staring strangers, “This is a medicinal fanny pack! I’m not making a somewhat dubious fashion statement from the early ‘90s!”

I put up with all of this pain and hassle because I wanted to live.  I never got used to all of it but I adjusted to my new norm.  That’s what this PHight is all about in my opinion. We have to play by the PH rules. It wants to take us down and we have to PHight with everything we have in us and just when we think we don’t have any more PHight left, we dig deeper. I could have declined the IV treatment and not taken my medication when I was supposed to. That would have been a lot easier but it wouldn’t have done any good. We only have so many weapons, only so many things we can do to Phight this disease. I was going to try to do every one of them.

The first few weeks of treatment were rough but the weeks became months and suddenly it was time to go back to Mayo Clinic. I was hoping for good news and I was fairly confident I would receive it. I had been taking my medication everyday on the dot, getting enough rest, got myself down to my target weight and had been working out three or four times a week. My breathing was the best it had been in a long time. 

I went through all of my testing again and watched while my doctor stared at a computer screen, reading silently the results of my tests while I waited for my fate to be delivered to me. I felt like my whole life was riding on this and in a way, it was. He finally told me what I had been waiting to hear. Every single test result had improved since my last visit. I felt overjoyed! I couldn’t believe what I was hearing. My doctor said he couldn’t be happier. The lung transplant work up was put on hold since it looked like I wouldn’t need it any time soon. My pressure had come down and my heart had even started to heal and was no longer showing signs of failure.

My wonderful doctor then mentioned possibly removing my IV. I would have to decrease my IV Veletri as I was slowly starting on inhaled Tyvaso. The Veletri to Tyvaso transition lasted about six weeks and then I had to leave my IV line in for two months while we monitored how I did on the inhaler. All of my oral medications stayed the same.  I went back to Mayo Clinic to go through all of my testing. My pressure during my heart catheterization was still great and I even improved my six minute walk test.
As of May 2013, I have been IV free! I’m now a functional class 1 and my best six minute walk test has been 747 meters. It’s a wonderful feeling but also one of fear. I fear the day I have to go back on the IV. I fear the day that my disease progresses to the point of no return and a transplant is my only option.  Most of all, I fear that my children will inherit this disease from me and that the brilliant minds working on finding a cure will come up slightly short and far too many of us will perish while waiting. 

I will not take this lying down. I will take my medication daily. I will take all of my herbal supplements and vitamins my husband found for me.  I will hop on that elliptical and do my three miles whether I want to or not as long as I reasonably can. I will continue to eat healthier and keep my weight down.  These are the only weapons at my disposal and I will use all of them.  My job, amongst other things, is now my health. I am a mother, a wife and a professional PHighter. I’m not going anywhere.

If you would like to follow Ashley on her PH journey you can find her on Twitter @phwarriorAshley
The PHight or Flight Project would like to thank Ashley for sharing her incredible PH journey for PHighter Friday!

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

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