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.