PHighter Friday: Michelle

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

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

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

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

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

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

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

PHighter Friday: Maddy

My Happily Ever After

My husband Andy and me at a Military Gala. 2014

I was 25, an Air Force wife, married to the love of my life, and about to finish my degree in education. I was healthy and happy. My biggest problem back then was when my dear Oklahoma State Cowboys lost to OU. I had this weird “thing” with my index finger though. I affectionately called it “my dead white finger disease” and saw it as nothing more than an annoyance. I eventually decided to see a doctor about it and was soon diagnosed with CREST Syndrome, a form of Scleroderma. Scleroderma is an autoimmune disease which includes Raynaud’s, the cause of my dead white finger. There is no cure and the progression and course of the disease was pretty much a crap shoot.

I vividly remember the rheumatologist handing me a pamphlet about Scleroderma. Almost as an aside he said “CREST is a much more mild form of Scleroderma...you only need to worry if you ever develop Pulmonary Hypertension. It’s very rare so you don’t need to worry about it.” I find it interesting that I don’t remember much else about that conversation, but I do remember the comment about PH. I also remember thinking it would suck if my annoying dead white finger led to something as horrible as Pulmonary Hypertension. I was glad to know it was very rare.

Thankfully, life went on. We moved every couple of years, I completed my degree. I taught school. I had a son in 2001, and another in 2006. The Raynaud’s was the symptom that plagued me the most, but I dealt with it. I was living my happily ever after.

About four months after my 2nd son was born, I began to get short of breath when I walked up stairs or hills. I figured I was just out of shape and needed to push through it. I began to walk and jog for exercise. I was determined to lose a few pounds and feel better.

We moved to a new state and at around that same time, I began to lose my voice. We all, including the doctors, assumed I was just allergic to the pine trees in my new location. Many months went by and we eventually discovered my left vocal fold was paralyzed. My voice was nothing but a whisper. For many months, I would whisper-sing nursery rhymes to my youngest, and he would whisper-sing them back to me. Giving my older son a stern talking to wasn’t nearly as effective when whispering. It was difficult to communicate by phone with family who all lived thousands of miles away. Even calling the dogs in from the backyard was a challenge, and more tragically, I could no longer order my beloved Dr. Pepper at drive-thru windows. Losing my voice was very isolating and it was a difficult time.

My symptoms worsened. Walking across the room caused me to be short of breath. I slept upwards of 20 hours a day and my youngest had to attend daycare. I was a stay at home mom and unable to care for my child. I was miserable and scared. My doctor placed a silicone implant in my vocal fold which gave me a pretty decent voice again. I couldn’t sing opera, but I never could before. I was just thrilled I could order that Dr. Pepper again. We thought the implant would help with the “air wasting” but things only got worse.

Eventually, at the age of 36, this all led to a diagnosis of Pulmonary Hypertension in October 2007. The cardiologist who did my RHC came into the room and told me I had PH. My heart was moderately to severely enlarged, and it was surrounded by fluid. He told me I might see 38, but I’d never see 39. Like a snapshot in my mind, I remember that I went home that day and as I lay on my couch and watched my 18 month old son play with my 6 year old son, I felt angry and heartbroken. My husband and I were reeling. This couldn’t really be happening right? My happily ever after suddenly seemed to have a different ending.

My pulmonary doctor put me on Tracleer and Revatio along with a slew of other not fun drugs. It seemed I took diuretics by the handful, potassium pills as big as a house, and others I don’t even remember. I soon found myself with a pill box labeled Sunday through Saturday. I suddenly had lots in common with the retirees at the pharmacy on base. Women with names like Ethel and Hazel were giving me recommendations on pill splitters and telling me which lab techs were best. Thankfully, over several weeks, I began to notice a difference in how I felt. I still had to sit down at the pharmacy while waiting for meds to be filled, but at least I could drive to the pharmacy myself. It was a step in the right direction.

In May 2008 we added Ventavis to my regimen. And courtesy of the Air Force, we also moved again. Within a week, I got my life back. Ventavis was the missing link! One day I was thrilled that I was able to go into Target. A week later I was shopping and lunching like a professional. I was meeting new friends, carpooling the boys, walking the dogs, and just being NORMAL. I began to attend all of the social functions at my husband’s work. I met people who had no idea I was fighting PH. I was a regular wife and mom again. My happily ever after seemed possible again.

I switched to Tyvaso in 2009 (from Ventavis). My heart was no longer enlarged, and the fluid was gone. I do not, and have yet to need oxygen. I do fight fatigue, and the shortness of breath still gets me when I walk up hills. But put me on a flat surface, like...say a mall, or the sideline of a soccer field, and I can walk all. day. long.

My three loves...Matt, Katelyn, and Nathan 2014

I’ve since completed a 5k Color Run. I walked, but I did it. I’ve gone tubing on the lake, traveled internationally, swam in the ocean, survived Walt Disney World, moved a few more times for good measure, and I do all the normal mom/wife things that I want to do. I’m certain my parents never thought I would view housework as something I “get” to do, but after being so ill, you tend to look at things a little differently. I sometimes think of sending that cardiologist a picture of myself on the lake, or in the Color Run, or turning 39 (and 40, 41, 42, and 43), but I can’t bring myself to write very kind words to him quite yet.

I have often said that God got me through the trial of diagnosis. He literally held me upright at times, and there is no way to describe how thankful I am. Being honest, I did spend some time in a pity party, and being angry. But He has showered me with blessings beyond my wildest dreams. And I’ve grown closer to Him than ever before.

God gave me a husband and a love that are unmatched. I would not be doing this well if not for Andy and his strong, unwavering support and love. We’ve had some scary moments, but he has never blinked. Andy can always make me laugh, especially at inappropriate times. He has been there for all of the difficult moments, and the fun ones too. We have been married 20 years, and it just keeps getting better and better.

God led my husband and me on an adoption journey that brought us a happiness that defies words. We were blessed with our sweet daughter just over a year ago. We traveled to Taiwan to bring her home and I could never, ever have imagined that joy back when I was absorbing the news of my PH diagnosis. There is no one on this planet who could have convinced me back then that we would be adding to our family through adoption.

Katelyn is 8 years old and joins her brothers Nathan also 8, and Matt, who is 13. I homeschool all three of them and I love it. Of course, some days I dream about dropping them all off at public school, but other days I look around in awe at this life I get to live. It is so different from what I imagined when I was diagnosed.

I was so sad and hopeless at that time. But those feelings have only served to make the joy sweeter. The predictions the cardiologist made were wrong. They were wrong 100 times over. Don’t misunderstand, PH is still hard. Some days are better than others, but isn’t that how it is for everyone? Some days I’d like to throw all the meds in the trash, and go to the movie without trying to manage the timing of my diuretics. I’d love to just have a break from PH. But I refuse to let PH define me. I’m a Christian, wife, mom, homeschooler, friend, daughter, OSU Cowboys fan, and Dr. Pepper drinker. PH is but a small part of that.

I hope anyone who is reading this and is newly diagnosed will realize that PH is not an ending. It’s just the beginning of a new chapter. My best advice would be to surround yourself with people who love you, and are willing to know and understand this disease. And then choose to make the most of every day. Some days it’s easy to do that, others it might take some work.

I don’t know if it is in spite of PH, or because of PH, but I know that I’m still living my happily ever after. And it is sweeter and fuller than I could have ever imagined.

For I know the plans I have for you, “declares the Lord,” plans to prosper you and not to harm you, plans to give you hope and a future. Jeremiah 29:11

To follow Maddy and find out more about her adoption journey please visit her blog.

My sister and me after the Color Run in 2012

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

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

PHighter Friday: Nicole

I was very very healthy when I was younger. I didn’t really have any issues growing up except a few migraines here and there.

When I was 18 (in 2006) I went to the gym and passed out. I didn't really think much of it. A few months later I went to school and passed out walking up a flight of stairs (talk about embarrassing!!!!!) My parents decided that I should go to a cardiologist. I went and they ran every test imaginable and guess what?!!! They couldn't find anything wrong. They said it was all in my head!!! You know because I enjoyed making a fool of myself and passing out in front of everyone lol! (Def not funny at the time!!)

Three and a half years went by and I continued passing out, each time was worse and worse. I finally got a second opinion and that doctor said I had something called POTs. He put me on vasoconstrictor and sent me on my way. Six months later (may 2006) I got married and my husband’s job takes us to Missouri. I felt horrible the next 6 months. Tired, passing out, and just feeling miserable. I decided to take myself off my medicine. I could tell I felt a little better when I didn't take it.

Oct 1, 2006 my husband, his aunt, and I decided to go to a football game. We got home and I had a horrible migraine so I went straight to bed. I got up in the middle of the night to use the bathroom and passed out. My husband tried to get me to come to and he couldn't. He called 911 and they talked him through giving me CPR. He did that for the next 20 min until the ambulance got there. (We lived out in the middle of nowhere) I finally came to and they rushed me to the nearest hospital. Stayed there for 30 minutes when they realized that something was really wrong and they rushed me to the nearest big hospital.

I was scared but I had this sense of peace that everything was going to be ok. The next few days they ran so many test. Couldn't figure out what was going on. I passed out one more time in the hospital and my heartbeat went down to 11 beats. Scariest thing ever. My doctor finally came in and said we think you have Pulmonary Hypertension we need to do a right heart cath to confirm. They did a heart cath and found my pressures were in the 100s. At the time I didn’t realize how bad that really was.

The doctor had never seen or even heard of PH. So the first thing they told me is that I was going to need a heart double lung transplant! A what?!!!! I didn't even know that was possible.

He decided to call around and see if there were any other options. We were desperate for something other than a transplant. I was 22 years old for Pete's sake. I had tons of dreams I looked forward to accomplishing and that was not on my to-do list. The doctor finally came back and said there was this drug that they called the miracle drug...Flolan. He told me about it and I was excited and devastated at the same time. I stayed in the hospital in the ICU until October 31. I felt better on Flolan than I did in the past 4 years. It really was a miracle drug although a pain in the butt too! But it saved my life and I will forever be grateful for that medicine. I have since switched from Flolan to Remodulin and now I'm on the Tyvaso and Tracleer.

 

I am capable of doing almost everything I could before diagnosis with the exception of running. The best way I coped with being diagnosed is with prayer and keeping a journal.  I love looking back in my journal to see how far I have come since 2006.  I have also tried to keep a positive attitude!  I could let this disease get the best of me or I can make the best of having this disease.   

Since being diagnosed my husband and I have adopted two beautiful babies and are enjoying every minute of it.  Please feel free to email me with any questions at mcclelland06@yahoo.com or check out my blog (that I haven’t updated in while..oops!!) www.adoptionmcclelland.blogspot.com

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

Motivational Pick-me Up

Today I had a pulmonary function test that did not go the way I wanted it to. I feel discouraged because I felt I was showing huge improvement. I know that I was. It is confusing to know how I feel and what I am capable of and have some test tell me differently. Yesterday it seemed like getting off of supplementary oxygen was in sight, and now because of that test I feel differently.

I have always struggled with this test, some of you might know it as the "blow test." Even as a child I was incapable of having good results- despite being pretty close to "normal" and "healthy."

I decided to have some retail therapy, and eat french macarons (don't tell my naturopath.) Those treats are nice, but they only help me feel good for so long. Only I can dig myself out of this pit. I will let myself feel sad, but I will pick myself back up. I will work out more tonight, continue with my breathing exercises and repeat positive mantras to myself. Tomorrow is another day to do better. I have a visit tomorrow with a local breathing specialist to go over the results. Hopefully he will be able to give me better insight and perhaps more information that I want to hear (and that makes sense with my progress.)

For anyone else who could use a little pick-me up; here are some inspiring and motivational pieces from a 25 Piece Collaboration by Noel Shiveley & Bethany Conner. All photography is by Bethany Conner and all lettering is by Noel Shiveley. More of the 25 Piece Series can be found here. One of my favorite pieces is the one that says "NEVER GIVE UP BECAUSE SOMEONE DOUBTS YOUR ABILITY." 

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 phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.