PHighter Friday: Steve Van Wormer on becoming #phaware

My son Lucas was born a happy and healthy child but life as we knew it changed when he turned four years old. Lucas was misdiagnosed with asthma, so for months doctors put him on nebulizers, but nothing seemed to help and he was on a downward spiral. Nine long months later, after an unrelated routine chest X-ray, his pediatrician recognized that he had a slightly enlarged heart. We were referred to a pediatric cardiologist, who gave my wife, Marina, and I the devastating news – our only child was diagnosed with ideopathic pulmonary hypertension.

About a week later, as we waited to meet with a PH specialist at UCLA, around 4am, Lucas stumbled into our bedroom, struggling and moaning. He had chest pain, he couldn’t breathe and his heart was pounding. He was rushed to UCLA in an ambulance to be stabilized.

Thinking back to that time (nearly ten years ago), I realize how lucky Lucas was. Only weeks earlier, combination therapy had come into play to treat pulmonary hypertension. That coupled with his relatively speaking early diagnosis, has contributed to Lucas being a robust responder to low dose oral therapy. Tragically, not every PH patient responds to treatment as successfully as he did (and continues to do).

As I write this blog entry, I’m thrilled to report his 14th birthday was yesterday and he’s never felt better with regards to his PH!

Lucas’ greatest wish is to find a cure for all the types of PH so no one young or old will have to go through life breathless. This is the reason why our family has worked so hard to raise global PH awareness - in honor of patients like Lucas and in memory of all the patients who have lost their battle with this deadly disease.

GIVING A VOICE TO PH

From day one of Lucas’ diagnosis we knew that if there is to be cure for this rare, life- threatening lung disease it needed to start with awareness.  Awareness for what PH is… Who it impacts… Where people can find information… and most importantly, how can people help? That process started with collaborating with talented graphic artists, editors, music designers at FOX to craft a series of awareness videos and PSAs that, over the past few years, have aired across multiple national networks, played literally a million times in taxi cab TVs, have been translated to over 50 languages and distributed to every global PH organization on the planet. The Lucas voiced pediatric PH research PSA even went from “rare to Time Square” when it graced the NASDAQ tower (the largest free standing screen in the world)!

FROM AN APP TO AN .ORG

With each of these awareness successes, with every email received from countries all around the world from patients responding to a #phaware tweet or reaching out because they saw an ad on TV, we realized the community was looking for new ways to connect.

And so phaware global association was born out of a simple hypothesis: “If we capture a single patient… if we save one life… what will it be worth?” That hypothesis has been proven repeatedly as phaware™ continues to discover diagnosed and undiagnosed patients, connects medical professionals, educates the general population, enlightens new investors and supporters, informs congressional leaders, and activates government and industry decision makers.

One of the prime objectives of our new 501(c)3 organization, which was founded by a group of pulmonary hypertension awareness activists, is to leverage innovative technology and use it to capture, engage and enable diagnosed and undiagnosed PH patients, caregivers, and medical professionals by providing them with state of the art tools and creative content focused on education, resources and knowledge.

GLOBAL DISEASE. GLOBAL AWARENESS.

From Burbank to Buenos Aires... Barcelona to Beijing. At phaware our focus is to inform and educate the masses, using impactful and innovative methods. phaware aims to make the reality, a day when no PH patient goes undiagnosed or misdiagnosed. Our goal is to reach and discover patients and supporters across multiple touchpoints. Our initiatives and awareness activations engage audiences through a variety of screens, mediums and platforms.

This exposure allows us to tell patient stories in ways never before imagined. This opportunity will be the catalyst to create greater impact on the discourse of healthcare and disease prevention not only in this country, but across the globe and bring activated individuals into the fold to help forge a new course to a cure.

BECOME #phaware365: A DAILY DOSE OF PH AWARENESS

Of course, we celebrate and honor the global PH community on Rare Disease Day (February 29th), World PH Day (May 5th), and during PH Awareness Month (November), but at phaware, we’ve created a new way for you to raise global PH awareness 365 days a year…It’s a snap with our phaware365™ app.

Show the world you are #phaware of this rare, life-threatening lung disease.

●      Snap a selfie.

●      Decorate your photos.

●      Customize them with PH facts.

●      Add awareness stickers.

●      Participate in global phaware activations.

●      Share across social media and on our phaware365 Global Selfie Stream.

●      Available for FREE on the App Store or on Google Play or at www.phaware.global/apps

Each time you share your phaware365 selfie on your social media, you raise awareness to your inner circle. Each time you share it to the phaware365 app, you raise awareness globally. With each post, you continue to bring PH from rare to everywhere!

THE BIG GAME. BIG 50 CONTEST

For Rare Disease Day 2016, we are kicking off our 2016 global awareness efforts during the biggest event on the planet - Super Bowl 50!

Launching January 25th through February 29th, (Rare Disease Day), phaware will be featured in a full-page ad in the Official Super Bowl 50th Anniversary Program. phaware will also be featured in the 2016 NFL Pro Bowl & NHL All Star Game programs, as well.

Go to www.phaware365.org to learn how you can help make PH history.

The Goal:

50K FANS  - Follow @phaware on twitter, facebook & instagram

50K SHARES - Visit www.phaware365.org to share their new digital awareness video

50K DOWNLOADS - Get the phaware365™ & phaware™ mobile apps

50K DONATIONS - Donate $25 to receive your FREE "I'm aware that I'm RARE" T-shirt for

Rare Disease Day 2016

These limited edition "I'm aware that I'm RARE" T-shirts are also available at www.represent.com/phaware

PHighter Friday: Amanda

On October 1, 2012 my life changed forever. This should have been the happiest time of my life. I had just celebrated my 3 year old’s birthday and had a 6 month old. My life should have been at its peak, but that all changed on that day.

For several weeks prior to that fateful day, I had been having what I thought was “panic attacks”. I found myself completely out of breath. I thought that maybe I was suffering some sort of social anxiety because these attacks coming on mostly out in public. Slowly but surely they started happening at home. I was slowly unable to even walk 10 feet without feeling air hunger. I couldn’t get enough air in my lungs and was starting to get scared.

On the morning of October 1st my oldest son had a speech appointment just like he had every single week. I got up to go to the bathroom that morning and was so out of breath and was having horrible palpitations. This was starting to get very scary and annoying all at the same time. I made it back to my bed and called my Dad. I was in tears when I was on the phone and told him, “I just can’t do this anymore. I need to seek help for this anxiety.” I then called my son’s speech teacher and told her there was no way we were going to make it because I wasn’t feeling well.

After calling my kids were hungry and I hadn’t been to the store due to my “social anxiety”. So, I loaded up my almost 6 month old and 3 year old into the car. I started walking to my front door to lock it and BOOM. My sister found me laying on my front steps turning blue and lifeless. My husband began to do CPR and they called 911.

The next thing I know I woke up in the back of an ambulance and thinking, “this HAS to be a dream. Why am I here?” I woke up and thought that I had a stroke because I had post partum REGULAR hypertension. I tried moving my limbs and figured that I in fact didn’t have a stroke. I was admitted overnight to the hospital and they ran every.single.test known to man. I was released the very next day with a diagnosis of “anxiety”. I was told that I needed to seek some counseling and get on some Xanex or antidepressant ASAP.

My cardiologist wanted me to follow up with him the following day. I went in for a stress test and ECHO. The ECHO showed right ventricle enlargement. I continued to the stress test which I only made it through the first minute and a half before feeling like I was going to pass out and throw up. The cardiologist rushed in and sent me to get a CT of my chest ASAP. She thought that I was having a Pulmonary Embolism.

The CT came back that I did in fact have right ventricle enlargement that was consistant with Pulmonary Hypertension. My doctor told me NOT to go home and look up Pulmonary Hypertension. He said he would do a heart catherization to find out for sure what my pressures were. At this moment I didn’t think anything of it. I heard the word “hypertension” and thought, “oh, great, more medication so I don’t have a stroke.”

Of course the first thing I did when I went home was got on my computer and Googled,“Pulmonary Hypertension”. The first thing that popped up was the mortality rates. I worked myself into a real panic and thought there is NO WAY I have this disease. I am a healthy 27 year old who just had a baby. I have 2 kids that I have to care for and this isn’t happening to me, but it was.

Not only did I have this horrible and deadly progressive disease but now I was being told by every doctor that my dreams of having another baby were dead in the water. I would never be able to carry another baby due to the high mortality rates. This just added to my depression. I watched as my friends added to their families. I just began to sink deeper into depression.

In mid November I finally had my right heart catherization that showed my mean pressures were 65. I was diagnosed with severe pulmonary arterial hypertension. I spent weeks searching the internet for anything that I could find about this disease. I joined multiple support groups and researched and researched. I researched myself into a depression. I had this impending feel of doom. I was just waiting to have another attack and be laying lifeless on my front porch, except this time no one would find me.

In January of 2013 I was put on all oral medications. The handful of meds I was prescribed was; Letairis, Adcirca, Warfarin, and Lasix. I hated to take all these meds since I had never had to before but I know that they were the key to saving my life. So, I took them and hated every second of it. About 3 months later I had an ECHO follow up just to estimated my pulmonary pressures. At that time my pressures had gone from 65 to 35. My doctors were ecstatic to see such a difference. I think one of my cardiologist even cried out, “it’s a miracle!”

After having that ECHO my hope escalated! I became empowered by my sickness instead of feeling depressed about it all the time. I got a new lease on life. I decided that I will not let this disease define me. In fact, I will let it encourage me. In April 2013, I opened my own business with my Mom. It was something that I had always wanted to due but had pushed it to the back burner.

I continued my medications and kept on with my “New Normal”. No, I was never going to be the old Amanda I was going to be the PHighter Amanda now. It was battle time and I was ready to kick some butt. No more feeling sorry for myself and being down in the dumps. I may have PH but that is not who I am it is what I have.

In May 2014 I had another routine ECHO to check on my pressures. My pressures showed that my right ventricle was no longer enlarged and my pressures were at 29! I knew that I was kicking this diseases butt. I did what I wanted to do when I wanted to do it and didn’t let anything stop me. If I wanted to go run around and chase my kids at the park I could. I continued on with my business and made a cross country move with my family.

Finally, in December 2014 I had another routine ECHO since I had switched doctors. She also exclaimed, “oh this is amazing, this is soooo amazing!” My pressures were at 13 and my heart was doing great! She was amazed at the progress I had made in just 2 short years.

Currently, I continue to operate my own business, play with my kids when I want to, walk my dogs, ride bikes, clean my house for hours, and most importantly my husband and I will be starting IVF soon to have our 3rd baby via surrogacy. I am so excited about my life. PAH has become much less of a thought these days. The medications remind me that I do have a disease but if I didn’t have those I wouldn’t even think about my PAH.

A positive outlook on this disease has helped me heal tremendously. Many of the support groups I have joined have also helped me stay positive. Surround yourself with positive thoughts. I don’t want to get preachy but my motto this entire time has been, “God gives his toughest battles to His strongest soldiers.”

PHighter Friday: Kellie

Life beholds many unforgettable experiences.  One of those cherished moments occurred for me on February 1, 2007 when Paul completely surprised me with his marriage proposal.  I was ecstatic to say the least, and we quickly set to work on planning our August wedding.  In mid-February the wedding planning came to a brief halt as I dealt with a short bout of the flu.  Once I was feeling better I dove back into wedding planning mode! However, something was not right as I became more active.  I was unusually short of breath during my workouts.  After a few weeks of trying to "get back into shape" I decided it was time to schedule a doctor visit.  The results came back fine, so once again I decided to start the workouts and push even harder.  By May my breathing was continuing to get worse.  Paul convinced me to set up another doctor appointment.  By the time I set up this appointment I could barely walk from my car into work without having to stop and rest.  At this second visit my doctor decided it was time to look at my heart.  A few tests were done and an echocardiogram was scheduled for a couple days later. 

On Friday, May 25, 2007 I arrived for my echo a bit apprehensive, but hoping I would receive a simple explanation for my shortness of breath.  It wasn't long before I sensed something "wasn't right" during the echo, but the technician wasn't about to reveal what it was to me.  I will never forget the look on my doctor's face when he came in to break the news to me that my pulmonary pressures were extremely high, there was major concern about my heart, they were going to do a few more tests, and then I would probably have to be admitted to the hospital that day (which I was). 

After being admitted to the hospital the panic really set in.  As I was being wheeled away for a right-heart catheterization, I remember looking into Paul's eyes and both of our eyes immediately pooled with tears...tears of fear and the unknown. After the catheterization I was sick, chilled and scared.  Whatever I was given at that point knocked me out for the night.  The next eight days I slowly learned more about my disease and the aggressive treatment regime I would be starting to try halt this progressive illness.  It was decided that triple-therapy was in my best interest, so I began sub-q Remodulin, Tracleer and Revatio.

I spent the next three months focusing on healing and learning how to manage my medications so I would be ready to walk down the aisle.  It was a summer filled with much pain and suffering, but I was determined to move on and live life to the fullest.  By the grace of God, I had the most fabulous wedding day.  On August 11, 2007 I was able to marry my best friend and companion for life.  The challenges of the previous three months had only brought us closer together and made me all the more sure that God was taking care of me in a special way through the gift of my husband. 

One of the most difficult realities of my illness was the loss of being able to bear children. Although my heart ached, I was blessed with an adorable nephew and a beautiful niece, as well as a classroom full of first grade students to enrich my life. I was thankful to be alive and thankful to be back working full-time with children. I was gifted a notepad with the saying, “keep a dream in your pocket and faith in your heart.” This immediately became my motto, as it deeply resonated with the way I wanted to live my life. So I kept on dreaming and had faith in God’s plans for my life. I trusted that my life would continue to be filled with blessings, many greater than I could imagine.

In 2011, my husband and I felt fortunate with my improved and stable health condition and felt it was time to pursue adoption. Just as we finished the paperwork to be listed as a “waiting family,” PH was brought to the forefront in our lives when my mother was unexpectedly diagnosed with PH. That’s a whole other story in itself, but it was a magnificent reminder that anything is possible with God. We witnessed a true miracle in my mother’s recovery and are blessed to have her here with us today.

In 2012 the greatest blessing of our lives came into the world. My heart sings that we were chosen to be the parents of a beautiful baby girl. I will never forget the day she was born and the love that swelled inside of me as I took my first look at her. God had a plan, and it was even better than I could have imagined. My heart that once ached with loss is now bursting with love and I feel at peace. As I watch her play, learn and grow, I am reminded everyday how beautiful and precious life truly is. I am blessed to be a mom.

Since my diagnosis, I was determined to thrive, not just survive. I have taken an active role in doing my best to lead a healthy lifestyle that has allowed me to thrive. I immediately began a low-sodium diet. It was a difficult change in the beginning, but I could sure tell a difference. Prior to my diagnosis I was active in sports that my body no longer tolerates. I have since found new activities that I enjoy and are a benefit to my health. I enjoy yoga and mediation. I love going for walks and recently got a Fitbit so I can track my daily steps and activity. I have also delved into the world of Young Living Essential Oils and am so excited to have something that helps me as I manage the side effects of all my medications and has given me a boost in my overall feeling of wellness. 

I am thrilled to be involved in the study with the implantable pump for Remodulin. It is such a delight to swim and shower without worrying about an external line and pump. It’s amazing to think of all the advancements in therapy that have come about since my diagnosis 7 years ago and I trust that even better things are coming.

I am thankful for the continued support of wonderful family, friends and medical professionals. I am grateful for the Pulmonary Hypertension Association and all that has been accomplished thus far. I will continue to PHight and give my support until a cure is found!

Although the path can be bumpy at times, I am confident that God is with me every step of the way.  I am keeping a dream in my pocket and faith in my heart because anything is possible with God!

http://phight4wellness.wordpress.com/

PHighter Friday: Donna

 I had my son in April 1988. He was 4 weeks premature because I had pre eclampsia. He was perfect though. I was very lucky. When he was 3 months old I traveled with my parents to Hawaii to visit my brother and his baby girl. She was born 2 weeks exactly after my son. We were there for 2 weeks.  One day while we were there, my brother, his wife, and I decided to climb Diamond Head.  We were climbing and my sister in law was just bouncing up the volcano.  I was having a rough time and said, well, I can't blame it on just having a baby because she just had one too. Then we finally got to the top and there were about 30 stairs straight up and I looked up and said. What's wrong. I can't do this. But my brother was very patient and I finally got to the top and the view was GREAT.

Came back home, very difficult marriage.  Very difficult. I got sick and couldn't shake the cough so I was sent to a pulmonologist, he gave me inhalers to treat me for asthma.  That didn't work, 2 weeks later I went back and he added more inhalers and did a chest X-ray. He remarked about this big thing on top of my heart but he didn't know what it was. Took the X-ray to the radiologist at the local hospital. That dr said ah that's normal.

Later that year, I was going through a divorce and the doctors I worked for ( I was a nurse), thought my racing heart was from stress so they gave me something for stress. That didn't help my heart slow down so they thought I had Mitral valve prolapse. They gave me a Beta blocker. This was in November 1991. I started the Beta blockers, now no one checked me for anything. I passed out one day at work so they sent me for an echocardiogram.  That didn't show mitral valve prolapse so they sent me the next morning to a cardiologist. I was put on a treadmill and within 3 minutes my heart was racing to 180. They pulled me off immediately. The sweet doctor said "I can't be positive but I think I know what is wrong with you, but you need a right heart cath." Okay this is getting scary but I was young, healthy, free of my horrible ex husband, there couldn't be anything bad.  A couple days later I had my heart catherization, my mom and pastor was there. Well, the weirdest thing happened- my mom acted as if she had been crying and said she was going home to take care of my 3 year old, my dad had him.  Weird, because my mom had never left me in the hospital alone. That evening my best friend came and brought me a beautiful long, lacey, cotton night gown.  So sweet- but unnecessary, I was fine. Then my brother dropped by with a hairbrush, magazines, and chocolate.  That should have sent off warnings but it didn't. Ignorance is bliss at times

The next morning the nurses came and took me for a pulmonary function test. When I got back to my room, my mom was there and my doctor. Mom had tears in her eyes and Dr. Fonenot said down on my beside and said " you have primary pulmonary hypertension ". I said "okay, how do we fix it?"  He said "we don't." I said "okay." He said "it's not okay, you will die in the next 2 years without a heart lung transplant." I laughed and pulled the covers over my head. I looked out from the covers at my mom and she was crying, my doctor was crying.  Okay- this is real.  All I wanted to do was go home and hold my son tight.

The doctor called the next day and said he had found a center that would do my heart/lung transplant in Birmingham Alabama. Wow. That close? About a week after my diagnosis it hit me, I was dying and soon. I cried a couple hours, got down on my knees and asked God to take care of my baby boy.  We scheduled the appointment with UAB in Birmingham for December and  my mom, dad, baby boy and I went to meet Dr. Bourge, the transplant doctor.  He agreed with my local cardiologist and scheduled me for a transplant evaluation for the first full week of January 1992

January came and all arrangements were made for my hospital stay that would take a week. The transplant team had made mom reservations at a hotel close to the hospital and even made arrangements for the hospital day care to keep my son during the day so mom could be with me.  We arrived on Sunday and found the daycare so mom wouldn't have any trouble finding it the next morning.  We found it easily enough but pulled up to the door and they had a sign on the door that they had chicken pox.  We had no choice, we had to leave him there and get exposed.  I was admitted and went through so many test, was told I needed to loose about 10 pounds because I needed to be the size of a 12 year old boy. The tests were never hard but always invasive. They drew around 20 tubes of blood a day. More radioactive tests than I can remember. My veins were so sore and I was so tired and got sick from all the dyes they used. Then on Thursday, the chaplain came in to see me, I had already seen psychiatrists, but they brought in the chaplain, he was one of my old pastors.  I felt so blessed and that I was doing the right thing. On Friday morning I had another right heart catheterization  and the most wonderful thing happened. My pressures were half of what they had been in November.  The Procardia was working. Yay!  The next day we came home with a plan. Low fat, low sodium diet and walking daily. I stopped dying and started living. I dropped the 10 pounds then 10 more. I looked and felt great.

My parents and my son and I spent a week at the beach just tanning, playing, fishing. Enjoying each other and life.  I was working again as a nurse and doing great. The death sentence hung over us but I wouldn't let it dominate me. The next weekend after coming home from the beach, a really good friend asked if I wanted to go out dancing on Saturday night, sounded good.  Mom and dad were watching my son. Well, I was coming out of her hall and this man walked in the front door and we just stared at each other.  He was a cowboy, I was a tiny woman with a big smile. We both will tell you- it was honestly love at first sight.

We started dating immediately and he was wonderful with my son. We had been going out a couple of months and my mom told me that I needed to tell him about my PPH (pah), so one night we stayed up all night and I finally got up the nerve to tell him I was dying and he held me and said " I already know, Ruthie told me".  My friend. Haha well we dated a while, I had a right heart catherization every 3 months.  Since there were no medications for pulmonary hypertension we had to keep a close eye on my pressures. 3 days before Christmas, Milton, my cowboy, took my son to see a movie in Mobile.  That night after I got off work, Milton asked me to marry him. I asked if he was sure because at the time we thought I only had 1 year left to live.  He said he wanted to marry me even if he only had 1 week left with me. We were married in June 1993.

I continued to have heart catherizations every 3 months. In 1995 I had a punctured lung during one of my heart Caths and I spent a week in the hospital with a chest tube. Horrible pain. I was still holding okay on my PA pressures, they stayed in the upper 40's.

Life was good for a long time, except for all those heart Caths. Somewhere along the line I was put on Coumadin and then lasix. Decided I didn't want to find a different doctor. Dr. Bourge and I didn't really get along all that well. I am a people person, he wasn't. Great doctor just not for me. So I was sent to Nashville and saw a wonderful PH group at Vanderbilt.  I had a bubble study there and they were convinced I had a hole in my heart and they could fix my PH. Wow!  After all this time we had hope. They sent me downstairs to a pediatric cardiologist who showed us what he would use to repair the hole in my heart. So the next morning I had an 8 hour long heart cath looking for a hole in my heart. After all that we were back to my original diagnosis of idiopathic pulmonary hypertension.  I couldn't keep going to Nashville, it was just too far away. So I found a doctor in New Orleans. The closest one yet.  I liked him and I had started getting worse. Luckily Tracleer had just come out so he added that to my calcium channel blocker.  That started working and I was doing better. Then he got an offer he couldn't refuse from Houston so he moved and I was without a doctor. I asked his nurse about someone and she told me about a Dr. DeBoisblanc. Thank goodness, he goes by Dr. Ben. We met and it was a perfect doctor/patient match. He was all about my quality of life like I was.

I started getting sicker again and he had heard about trials of Viagra, so we talked my insurance company into getting me Viagra. They sent me 3 months worth of that golden medication. Haha joke. I started taking it and the headaches were horrible, they just got worse and I started vomiting daily. Talked to Dr Ben and he said to stop taking it.  He was trying to keep me off IV medicines. Then sub-q Remouldin came out and he said. You'll love this. No IV. So my local cardiologist worked with him to get me the medicine. I went in the hospital to get started and trained on it. Everything was good for 24 hours and I got to go home. At the 25 th hour the site pain started. Oh no this is awful. On the 3rd day it got better, then I had to move it and titrate up my dosage.  Here we we again. The worst pain ever.  Then it would get better, then I'd have to start over again. It got so bad I started vomiting all the time again. I only got out of bed to vomit. It was awful. So 4 months of that and Dr Ben looked at me and told me to stop that medicine. He said this is not quality of life. Well then, Ventavis came out. Big bulky pro-dose machine that had to be plugged it. Every 2 hours I had to inhale the Ventavis   No side effects and between Ventavis, Tracleer and Norvasc I was doing great. Then we took a direct hit from Hurricane Ivan and were without power for a week. We had to use an inverter in the car so I could do my Ventavis every 2 hours.  Finally the little portable inhaler for Ventavis came out and I was one of the first people to get it. That made life so much easier.  I stayed on these medications until 2007. I started getting worse again. So as soon as Letairis came out I switched from Tracleer to Letairis .  The Tyvaso came out and my local cardiologist told me it was only 4 times a day.  So I changed from Ventavis because I felt like it would be easier for me. I responded really well to Tyvaso and loved how easy it was to me. I have beed on Tyvaso, Letairis, and Norvasc since 2007.

This is the year 2014 and in November that will be 23 years since I was given 2 years to live. I still have my original organs and hope I always will. I have still avoided IV medications but now there are 3 different ones available if the need arises

I still live my life to the fullest. I get tired more but I am now 53. So is it my PH or is it my age?  Who cares. I am going to live to be an old lady. My son is 26, I've been married to my cowboy for 21 years. I have cows, horses, goats, chickens, a cat and a wonderful Boston Terrier that is my little boy. Haha






*Editor's note: you might recognize Donna's story from my previous post where I posted this video of Donna who shared her PH journey with a local news station (video below.)

PHighter Friday: Lizzy

My PH Story

Lizzy

 I still can't imagine what it must've been like for my husband to find his wife laying on our bedroom floor passed out. Lifeless. Two little children by the body looking helplessly, at their sick mom. Jacob, 8, has since vowed never to leave my side. What a huge responsibility for a little eight-year-old boy! 

Now let's go back to the first symptoms. Breathlessness in summer, 2008. I had just birthed my daughter that same year in January and thought I was out of shape. Boy, too many enchiladas, if you asked me. Wink, wink. I had also just accepted an Operating Room position in Irvine, California that March. Lots of newness for my husband and I. The prior year we were living in Houston, Texas where thunder storms were really that, a THUNDER storm. Not like here in L.A. I laughed every time Jackie Johnson, the weather girl, announced a "Storm Watch" on TV. Whatever. Hehe. We were so new to the community and didn't have family nearby. Here I was running around potty training a rambunctious toddler while carrying a newborn in my arms. Oh -and did I mention my husband and I had just opened a new business? Yup! A Collision Repair Shop. We like excitement.  

We enjoy Thrift Store Shopping!

Then August came and I started getting out of breath by just climbing the stairs at home. In a matter of two weeks, it got so bad that I literally had to stop after just climbing five steps! By the time I got up to the second floor, I felt like I had an elephant sitting on my chest, as if I'd just ran two miles. Wha? My husband couldn't believe it. I've always been very active. Of course, right? These things only happen to us girls that love life! I use to run on the beach in Santa Monica before work every day back when I worked for a plastic surgeon. Back when I was so caught up with myself, making money and being 'successful'. 

Things have changed.

I believe the Lord took this opportunity to show me some humility. But above all, He's shown me an unmeasurable amount of GRACE. I can actually say that this, has been the best thing that's ever happened to me. And I know that sounds corny and movie-like, but it's true. I am so grateful for the wonderful friends and family that have stepped up to support me in my most darkest place. I've gone through a world wind of ups and downs, emotionally, physically, spiritually, and everything else "-ally". 

I like to host fun girly Tea Parties with my cousins

So I was officially diagnosed on Halloween October 2008 I don't know what stage I was at then but now I am considered a Stage 2, we are calling it "Primary Pulmonary Hypertension" because we have no idea why I got it. My PH is idiopathic, I never consumed recreational drugs, no heart problems run in my family, I didn't have 'rough' labor with my children (my labors were actually fairly easy). I have no idea why I have Pulmonary Hypertension. My daughter was 10 months old when I was diagnosed with this horrible disease, and my son had just turned three. I was a lively thirty-year-old working part-time in the Operating Room and finishing up with online classes in order to get accepted into nursing school. I was so proud of my 4.0 gpa. I felt like I could do it all. I couldn't. I was the one who never asked for help. Now I braced myself as I uttered these words to my husband, "Can you. Would you. Um. Empty the dishwasher? I get out of breath from leaning over." Not that he wouldn't do it, I just always did everything. I liked my independence. Loved it, actually. 

Here's my wonderful husband and daughter
at our Shop in South El Monte, California

I realize now, how God's hand was on my life from the beginning. Always there to cushion the blow. If we had remained in Texas, I probably wouldn't have been able to afford the expensive treatments. If I hadn't gotten the job I did just five months prior, I wouldn't have had the benefits that now pay for pretty much all my treatments. We had recently started attending a church in the area, and some of those girls and their husbands, are now some of our best of friends, which we consider family. They've been with us through thick and thin. They've provided meals for us whenever I have catheterizations. They've picked up my kids to give me rest. They've brought dinners and laughed with us and cried with us. They've shown me Jesus' hands and feet.

I was put on Tracleer, blood thinners, Viagra, Ventavis, and now recently, switched from Ventavis to Subcutaneous Remodulin. Lots of learning curves. But with each one, comes appreciation. Gratitude for another medication. Thankfulness for another year of homeschooling my children. And working part-time with the nurses at the Labor and Delivery floor. Life is good. But above all, God is good. All. The. Time. 

This is what a normal day of Home Schooling looks like

You can check out my blog LifeAsLizzy.com if you'd like to. I'm sorry it's been a while since I've written a post on it, life with meds is busy! ;-) You can also follow me on Twitter, @LifeAsLizzy. And if you'd like to follow some crazy pics of my everyday life, request me on Instragam @LivingLifeAsLizzy. 

Many blessings to you! Thank you to Serena for giving me the opportunity to share a little about my PH journey through her blog. What a great work she is doing here. 

~Lizzy

My daughter's kinder graduation this year

PHighter Friday: Michelle F.

I am so excited to share my PHight of flight story with you all.  I hope this touches someone somewhere.

I’ll start with sharing with you that God blessed me with a child at the age of 16.  I am lucky to have wonderful parents that made huge sacrifices to raise her.  When I was diagnosed, my doctors told me that I couldn’t have kids because the mortality rate of pregnant women with PH was 50%. Instead of being angry about not being able to have another child, this news made me realize how blessed I already was to have a beautiful little girl.

Team photo before the race

The diagnosis changed the way I valued my relationship with my daughter Christine.  I know now that time is of the essence. The diagnosis really helped me refocus my energy on strengthening my relationship with her, my family and friends. 

July 23, 2013, is the day I went to urgent care for being short of breath.  I was swollen from head to toe.  I couldn’t walk 10-15 feet without feeling short of breath and my heart was racing uncontrollably. After a week of tests in the hospital I got the news. I had class II IPAH. 

I was prescribed Letairis and Adcirca but ended up taking Adcirca only.  In a year’s time, I went from a class II to a class I.

Pre-diagnosis, I had been so used to living with the classic PH symptoms.  I was always tired. I would have intermittent leg swelling, shortness of breath, rapid heartbeats.  Those things just became my “normal”.  I went to so many doctors who thought I had the regular systemic high blood pressure and I was actually relieved that now we knew what was really wrong. I was excited to see what life would be like on medication!

Post diagnosis, I am able to do 30 minutes of moderate cardio without feeling short of breath.  I can walk up 2-3 flights of stairs non-stop.  I even participated in The World Famous Camp Pendleton 5k Mud Run!  I completed this event with a group of amazing friends who helped me make it to the finish line even though I was ready to quit after the first quarter of a mile!  I walked most of the time, but I still finished the race!

It has been one year and two weeks since I was diagnosed.  Physically, I feel 100% better than I did before.  Emotionally, I’ve matured 1000% (one thousand!).  Yes, I know it is horrible to read the life expectancy statistics.  Yes, it is sad knowing this disease is progressive and there is no cure.  Yes, it is frustrating to accept that child bearing is not an option.  But the truth is, like the title of this blog, we have two options.  We chose the PHight or Flight. I chose to PHight. 

My “one year survivor” anniversary just passed.  As I reflect on the years I lived before diagnosis, I realize that I spent most of my life just existing.  In the one year post diagnosis, I have truly been living.  I work full-time as a paralegal at an amazing law firm.  I spend more time being active and making genuine connections with people.  I appreciate every minute have and spend my time doing things that make me happy.

Paola and Me

I didn’t start to live my life until I thought I was about to lose it.