Friday, 27 February 2015

PHighter Friday: Sean

The year was 2002, I was still 18 and my mom wanted to go to Colorado because a pseudo-sister was having her first child and wanted my mom there. Not being the one to let my mom travel cross country in the car by herself, I opted to go with. It was going to be great, I was going to be able to go back to where I grew up, see friends/family and most importantly enjoy the scenery I have come to miss more and more each day. The road trip started out like any typical road trip loading up the car, getting fuel and hitting the road. We stopped in Hesperia to get food at In-N-Out and then continued on. It was night time (because no one wants to drive across the Mojave Desert during the day) and I was slated to drive at some point. My mom wanted to drive first, until she got tired, and then she was going to let me take over—or at least that’s what we thought.
After eating and the getting on the road, I fell asleep to make sure I was well rested to take on the second leg of the journey. In Flagstaff, Arizona we stopped for fuel and I proceeded to get sick. Maybe it was bad food… no one really knows—actually, they do. I was going into heart failure but didn’t know it. We continued on the road trip, but I still was sleeping a lot. Unfortunately, my mother had to drive the whole trip by herself because I was just that tired. Something clearly wasn’t right, but maybe it was the food. Ok, not really but we didn’t know better at the time.

Upon arrival to Colorado Springs, I had another episode where I made my mother pull over so I could proceed to get sick outside of the car. Walking around at altitude was difficult, I couldn’t breathe well and it felt like I had an elephant tap-dancing on my chest in stilettos. Walking into a restaurant was quite interesting. Well, I didn’t actually make it into the restaurant, 10 feet from the car the elephant started his Irish jig, and 30 seconds later I was blue and on the ground. I woke up shortly afterward without much recollection of what had happened. However, I had previously had an episode like this four months prior while dropping off a portly cat at my former bosses house before I moved to California. Finally, my mother could see what the black-out episodes were really like. Still, we thought nothing of it. Well, my mom thought something of it, I was oblivious to it probably because of the invincibility complex that most 18 year old males have at that point in their life. After eating a late dinner, I felt a little better, perhaps it was the company, regardless sinister things were still taking place much to our unsuspecting wonder.
Eventually we made it back to the hotel, and walking from the parking lot to the room was a struggle. Horton the Elephant seemed to want to crush my heart with his size huge stilettos and bad dancing. Though out the night I was running to the bathroom, throwing up over and over. Another case of food poisoning? No. A stomach flu? No. The next morning I took a bath to clean up from the night of sweating and puking my guts out. After getting out of the tub I sat on the toilette. Next thing I knew I woke up under the vanity, my head hurting and my chest pounding. I proceeded to get dressed and sat at the foot of my mom’s bed. Not 5 minutes later she walked in with Michelle, my long time babysitter though elementary and middle school. “Mom, it happened again,” I said. I think all the color went out of her face at that point, and Michelle stepped in and said, “You’re going to the hospital whether I have to drag you kicking and screaming or not.” While still reluctant, I went with it. We stopped by Shrap Metal to get retainers for my piercings at the time (I mean who want’s lightning rods in their body if you code?). Off to the hospital we went.

Upon arrival at the hospital, I was taken into triage. The nurse put on a pulseox and took my blood pressure. While my blood pressure was normal, my oxygen saturations were low, but more importantly my pulse was through the roof. She thought the machine was broken… she tried the other hand… same result… she took it by hand… same result. Next thing I know I was being whisked to the cardiac area of the emergency room. There was a slew of doctors and nurses around me. All I can recall is an echo and chest x-ray at the time. I still didn’t want to stay, and I told the doctor the only way he was keeping me was if I had a hot nurse. Of course, the doctor delivered and my ICU nurse was gorgeous.

The next week was pretty intense, I think I had every test known to man run on me including the infamous right heart catheterization. The pulmonologist and cardiologist taking care of my case came in and said, “We are pretty sure you have pulmonary hypertension, but we’re not 100% sure. We need to send you up to Denver where they have pulmonary hypertension specialists to evaluate you further.” On my birthday, October 18th, 2002 I was transferred to St. Joseph’s Hospital in Denver, Colorado. Dr.’s Lang and Kenard wasted no time and completed the work up for pulmonary hypertension. After reviewing everything Dr. Lang came in and said, “Sean you have Pulmonary Hypertension… right now there are two medications, Flolan and Tracleer. Tracleer is new and with as advanced as your case is you probably won’t get any benefit out of it before the inevitable. Flolan is the Gold standard and would probably be your best bet. If you don’t do any treatment, you’re looking at maybe 2 years.”

Yeah, let’s just say my 19th birthday present from a doctor was what seemed like a death sentence. Fortunately, St. Joe’s was a teaching hospital. Every floor had tons of laptops. I was scared, but very compliant so the nurse brought me in a laptop. I spent most of the night researching PH on my own. Mind you, in 2002 there weren’t tons of resources that were easily accessible. After spending the rest of the night soul searching and trying to decide what I wanted to do I fell asleep. My mom arrived back at the hospital early that morning, and the doctor stopped in. I told Dr. Lang know that I’d go with the Flolan, after all, who really wants to die at 21—and that’s what he pretty much gave me. I was slated to have a central line put in and the rest is history.

After getting back to California about two weeks after this whole ordeal took place I met with my current pulmonologist. He told me at that point I wasn’t likely ever going to work or go to school. The same sentiment was resonated by the PH specialist I saw in Los Angeles. However, as I began increasing on the Flolan I began feeling better. My current pulmonologist never mentioned the work or school thing again. I told him I was going to go back to school and he supported me. Now I am a second year Medical Student at Western University of Health Sciences College of Osteopathic Medicine of the Pacific in Pomona, California. I’ve worked, I’ve loved, and I’ve lost but I couldn’t be more blessed.

The Pulmonary Hypertension Association contacted me in early 2009 about things they could do to make PHA better for young adults. In December of 2009, I was asked to join the Young Adult Advisory Board in which Generation Hope arose with special thanks to Colleen B., Britt R., Lindsay N. and myself. Shortly after that, I was asked to become a PHA PH Patient Mentor. In 2012, while getting my Masters of Science in Medical Sciences, I got a wild idea to come up with a cartoon series for children recently diagnosed with PH and so Lips Painted Blue ( was born. Needless to say, PH has shown me everything that I can do.

Mind you, I still have bad days occasionally, especially when modifying medications, life couldn’t be better. I will take the first part of my boards this summer and then finally head out to rotations to do good with actual patients. I will be able to take my experiences as a patient and relate to other patients to ensure they get compassionate and comprehensive care. My journey with PH may have begun back in 2002, but I look at each day as a new beginning. Every day gives me the opportunity to learn something new about myself. I couldn’t love life any more than I do, and continue to preserver. The good will always outweigh the bad, and the resources that are available for individuals are limitless. Through opportunities like the Tom Lantos Innovations in Community Service Award from PHA, if a resource doesn’t exist you can let your creative juices flow and create it.

My final remarks are something that I still believe to this day. Even though you have PH, it doesn’t have you. You should never give up, never surrender and most definitely live your life to the fullest. If you have any questions you can feel free to contact me at

Wednesday, 25 February 2015

Partners with PH: Mike and Reinee

When you first found out about Reinee's diagnosis how did you feel? Has that feeling, and how you view PH changed throughout the years?  Is there anything that scared you about the diagnosis at first that has changed?

Mike:  Reinee first told me about her condition only after a month or so of dating.  It was a very intimate setting – lights off and blanket over our heads – in the living room of my apartment, just us two hanging out.  She softly spoke and told me about her condition.  At first, I didn’t comprehend the severity of PH, that there was no cure and that it would progressively get worse.  But, honestly I didn’t care how this would impact my life because I already saw her being a part of mine.  I fell in love with this girl.  That night, I made it a point that I would support Reinee but not treat her any differently than I did prior to knowing.  Since then I never looked back and never strayed away from the promises I’ve made to her that night.  Not once have I ever felt obligated to stay with her or take care of her during the down times because I was willingly glad to and I just wanted to be with her… All of her.
I guess the only thing that scared me about her condition was knowing that it could get worse to the point where I’d lose her.  I will admit that there were some close calls but ever since we got married about a year ago, our bond has never been stronger and both of us are the healthiest we’ve ever been.

Are there any activities that you and Reinee do to together? For example, vacation once a year, walking the dog for 30 minutes after dinner, cooking, watching movies etc

Mike:  I’m very proud of Reinee and all the things that we’ve been able to accomplish just this year alone.  Ever since the installment of her new medication (Tyvaso), it has given her the ability to become more active and endure more strenuous activities.  I’ve literally witnessed her from being out of breath after walking up a flight of stairs to her first time snowboarding with me just this past weekend!  Now snowboarding can be very tiring even for me at times but Reinee, although cautious, arose to the occasion and attempted to ride down bunny slopes like she had no PH at all.  At the end of the day, she was S-curving the slopes and was hardly falling down.
It also brings me joy knowing that I’ve been able to experience all these “firsts” with her.  Earlier this year, we were vacationing in Oahu and I finally persuaded her to hike up Diamond Head with me.  We hiked up that ~1 mile crater and were able to share the miraculous views from the top.  At that moment, I really felt that this was a milestone for Reinee and it made her realize that if she was able to do this, that there’s no telling what else she has the potential of accomplishing.  Later on that trip we went paddle boarding and further into the year we also tried indoor rock climbing for the first time too.  Again, another feeling of self-fulfillment as I saw Reinee ringing the bell atop of each climb she made.
Is there anything that you do to help Reinee with her condition? For example, carrying laundry up the stairs.

Mike:  Simply put, I’ll do anything and everything in my power to help her with her condition.  I’ll do anything from helping her set up her medication to carrying all the groceries/laundry from point A to B.  Although Reinee does a great job of holding her own which I greatly appreciate, I will never hesitate to help her with whatever she needs.

What impresses you the most about Reinee? What do you admire about her the most? Is there anything about her personality that you feel has helped her thrive?

Mike: I love her fight and unwillingness to give up on herself and our future.  We take it a day at a time but definitely have our sights on raising a family of our own.  This is our goal in life and although she cannot bear a child for herself we’re looking into all options and it has led to the path of surrogacy.  Wish us good luck!
I admire the fact that Reinee continues to live her life regularly, meaning she carries herself as if she has no condition at all.  At the same time, Reinee knows her limits and knows when to slow to down if her PH gets the best of her, but this is always short lived.  Reinee can bounce back and continue to act as if nothing is wrong.  Her resilience is impeccable and is something that motivates me to be a better person for myself and for each other.
Reinee’s personality is fun-loving and upbeat.  She’s a pleasure to be with and it’s always a laugh fest when we’re with each other.  We’re very random too about the things we talk about or the things we do like singing/rapping out loud while driving in the car or spontaneous tickle fights in bed before falling asleep.  Reinee knows I’m very ticklish and takes advantage of it all the time when she wants something.  She has this power over me and without fail can always make me feel better when I’m down and keeps me in check when I need to be! Lol 
Is there any advice you would give to other people who are in a long-term relationship with someone with PH, or anyone who is considering marriage with someone with PH?

Mike: Like any relationship everyone has their own “deal” that they bring to the table, but you as the significant other must be able to love that person for both the good and the bad.  When Reinee and I were taking marriage prep classes, the deacon told us that love is unconditional and you must accept each other as whole, especially for their faults.  You’re not just marrying the person you fell in love with, but also the person that gets on your nerves and irritates the heck out of you sometimes.  This message solidified how I felt for Reinee and that I would love her despite any deal she had.
But maybe it was easier for me to be with Reinee because she appeared to be a healthy person when I first met her.  And maybe it was also for the fact of dating an older woman that attracted me to her more, but whatever it was I knew she was someone that I could relate to and most importantly have fun with.  Reinee was honest with me about her PH and upfront about the possibilities that could happen throughout our relationship.  But until now, we’ve weathered the storms, overcome every obstacle, and continue to stride forward with gusto because nothing is going to hold us back from living our lives to the fullest and enjoying our family to we’re old and grey.

Friday, 20 February 2015

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.”

Friday, 13 February 2015

Phighter Friday: Michelle R

Before I got PH I had a very active lifestyle, running, sailing, caving, climbing, hiking up mountains, and my passion, horse-riding. All my life I owned, rode or worked with horses. I would think nothing of biking 8 miles to work (running a Riding For the Disabled Yard) and back, and then going for a 5 mile run in the evenings. I guess i was a bit of an adrenaline junkie, competing cross country and show-jumping with the horses…..and often going to theme parks to ride all the scary roller coasters.

During the winter of 2009 we had a lot of snow, and one evening my car got snowed in at the stables.  I ran the four miles home in the snow, getting pretty cold and wet. A few days later I got pleurisy. I never really recovered from that. When all the snow had cleared I tried biking to work again and found it incredibly hard. I tried to go out for a run, but found I was out of breath after a few metres. I knew something was very wrong, but the doctor assured me it was down to the pleurisy and I had to get fit slowly again.
I spent the whole of 2010 trying to do just that. I walked everywhere, but still couldn’t run. Going up hills I used to run up I had to stop to catch my breath. I found working the horses really hard work, and I kept getting a  weird tingling feeling down my arms and into my wrists. My hands and feet were numb even in the summer!

Early in 2011 I seemed to be getting worse and I went back to the doctor, who thought I might have asthma and gave me an inhaler.  I pushed myself so hard to get fit I don’t know how I didn't kill myself instead. A few months into 2011 I was at the doctors for a general check up and she asked how my breathing was. As it was no better she sent me for a x-ray.

A few days later I had a phone call from an alarmed doctor, who told me I had an enlarged heart on the right hand side. I was told to be very careful and was referred to hospital. After a series of tests one thing after another was ruled out until finally 2 days before my 45th birthday the consultant said he thought I had PH and was referring me to a specialist hospital, the famous Papworth. In the mean time I googled PH as we all do and was thrown into a panic after reading I only had two years left to live!

I lost my job as I was deemed too unfit, and I put my own pony out on loan. At that point I couldn't even climb the stairs without stopping.  Papworth initially classified me as a WHO class 2/3 and started me on Sildenafil and Warfarin.  They helped a bit but I sunk into depression.

Without the horses I felt like my right arm had been cut off. I found it hard work just walking the dog round to the shop, a mere 1/4 mile away. I was even jealous of runners. I mourned the loss of my lifestyle. I applied for several jobs without success, and was in a really black place.

Finally in April 2012 I got a job at Tesco on the checkouts.  Papworth also started me on Bosentan , and those two things were the beginning of the turnaround. Slowly I started to find I could do more, walk further and felt stronger. I discovered the Facebook PH group and was amazed to find people that have lived with PH for 20 years. I began to feel ‘normal.’ again.
In April 2013 I got a job in my home town in the ice cream shop, which is physically harder than Tesco, but again the more i did the better I felt. I also got a second job in the evenings, waitressing in a bar, and walked everywhere. In my 6 minute walk tests I could do nearly 600m. I almost forgot I had anything wrong.
This year I have had a couple of set backs as I was diagnosed with Breast Cancer and the local hospital refused to operate due to the risks of the anesthetic. Lucky for me really as I was referred to the brilliant training hospital Addenbrookes only 10 miles from Papworth . I spent a couple of days at Papworth having all the tests again, and was delighted to discover the pressures in my lungs have lowered and I am now a Who class 1. I sailed through local anaesthetic lumpectomy and removal of 3 lymph nodes, making history into the bargain, and the following 6 weeks of radiotherapy. Unfortunately that caused pneumonia in October and I ended up in intensive care, which has reminded me, that I do actually have a lung condition and to slow down a little bit!!
Back in the summer I met someone local who keeps horses and started helping her out. To my delight I found myself back in the saddle, and not out of breath! I am also trying to start a dog walking business,  although I only have one customer at the moment, I find it no problem striding around the countryside, doing 3 miles or so. I firmly believe the exercise helps the condition. So although at the beginning it felt as though PH had stolen my life,  little by little I have stolen it back again!

Friday, 6 February 2015

PHighter Friday: Colleen S

In the PH community, I often hear people being asked what their life was like before diagnosis. I've been asked this myself many times over the years. The thing is, my life before I was diagnosed with PH was the same as my life is now. I don't know what it's like to breath normally. I don't have a clue what going from an active lifestyle to a PH lifestyle is all about. That is because PH has been my entire life. It's all I've known. I actually thought, as a kid, that it was normal to take hours to recover from running around with your siblings and friends. I thought everyone took naps when they were exhausted! And although everyone around me, family included, always told me I looked purple, to me that was just my thing!

I was diagnosed when I was 9 months old, but technically, I should have been diagnosed months earlier. My mom brought me to a free clinic to get my first set of immunization shots, and a doctor there thought something was up with my heart. My mom took me to the pediatrician, who didn't think anything was wrong. So at the same free clinic for my 2nd set of shots, the same doctor who saw me the first time told my mom that something was seriously wrong with me, because I looked blue. My parents ended up taking me to a children's hospital, and after many tests, they discovered I had a pretty bad congenital heart defect and pulmonary arterial hypertention. At that time (1975), the doctors couldn't do anything. It was too late for surgery to correct the holes in my heart, and there wasn't anything to treat PAH. My parents were told I might not make it to my first birthday, or I may possibly live til I'm 50. They weren't given much hope! But here I am, 39 years later, and I'm still living to share my story!

First Airplane Trip
I grew up with a lot of "cannots." I cannot participate in sports. I cannnot take gym class. I cannot go on rides that'll get your heart rate up too much. I cannot get pregnant. And so I lived my life following the "cannots." I often wonder if that really was a good thing or a bad thing, but I guess since I followed those rules pretty closely, and I'm still here, it was a good thing? I followed my grade school dream of becoming a teacher, getting my Bachelor's and eventually ending up in a preschool classroom. I loved it! But after only a few years, those preschooler germs started loving me. Too much. I was on my third major respiratory illness during my third year of teaching when my primary doctor came into the exam room with tears in her eyes and told me I had to quit my job. She wrote me a note that I took to my director right after the appointment. I was 24 at the time, and never even thought about quitting a career at that age. Who would??

A few years later after my PAH symptoms continued to get worse, I ended up going to the Cleveland Clinic in Ohio. I was referred by my doctor to go for a transplant evaluation. At the time, the shortness of breath started happening more frequently (just getting dressed in the morning was becoming a 30 minute event), and an elephant had permanently moved in on top of my chest. That was one symptom I didn't have growing up, and it was not a symptom I particularly enjoyed! Being sent to Cleveland was very scary. Transplant was even scarier!! But my first visit, I was sent back home after so many tests with instructions to start taking Coumadin, a blood thinner, and to wear oxygen all the time. After several more visits to Cleveland in the following months, I was put on Tracleer. I had no idea what it was, or exactly what it would do. All I knew was that I surely hoped it would help my elephant to move on, and that I would start feeling better!!

My first shipment of Tracleer brought me not only the first medicine I'd ever try for PAH, but it also brought me into the world of the pulmonary hypertension community. I grew up knowing I had PH, but it was something that was never focused on. My heart condition was the central star all those years. Once I found the Pulmonary Hypertension Association website, my knowledge of PH started to expand. But what really hit me?? THERE WERE OTHERS LIKE ME!!!! I seriously spent a week reading the message boards and crying, because there were people I could relate to and totally understand!! It was like finding a miracle! 

Tracleer got that stupid elephant to move out several months after being on it, and I also didn't feel as short of breath doing certain things like I was always used to feeling. It was a pretty awesome feeling for a few years, until I felt like I needed to add another medication to the mix. Revatio was added, and the combination of the two is what I am still on today. Pulmonary rehabilitation was something I found out about from phriends (friends who have PH), and after inquiring about it, I started going twice a week at one of my local hospitals. It's been a little over six years since I've started pulmonary rehab, and I truly believe it's a part of what's been keeping my PAH stable in the last several years. Kinda hard to believe I went from no gym or sports as a kid/teen to working out a couple times a week at least! No, I am not running marathons or doing heavy weightlifting, but that is ok! Exercising at any capacity is beneficial, even if I can only do it a little at a time!

So after my PAH seemed to be stable for awhile, the next part of the equation was....what am I going to do with my life?? I couldn't teach. I knew I'd never go back to that. But I didn't know what else to do. Some days I had more energy than others. I'd have one day where I wish I could be at a job, to several days when I was thanking my lucky stars that I didn't have one. Then one day I got a call that started the ball rolling for me towards a direction I never thought about. I was asked if I'd consider starting a support group for PH patients in my area. At first, I was pretty terrified. I could handle being around kids, but to lead a group for adults??? I guess you could say that my shy background made the whole prospect a bit daunting for me! I really had to think about it. So, that's what I did. I spent a couple weeks thinking and praying and talking to my loved ones about the idea. I finally decided to go ahead! Yes, I was incredibly nervous about it, but I felt it was something I just HAD to do. How many years did I live with this disease by myself?? I just knew I had to at least give others the chance to not feel so alone after their diagnosis! 

In the 10 years since starting a support group in the Niagara Falls/Buffalo, NY area, I have really become involved with helping PH patients. I've had so many meetings, a large variety of topics and speakers, and phriends I care about very much. I have also become a big part of the online community for PHers, by being a PHA mentor, a chat leader, a blogger, and just a presence on Facebook. I had always wanted to teach in a classroom, but it took me awhile to realize that a "classroom" didn't have to be a place with 4 walls. It could be anywhere! I have even educated complete strangers during shopping trips about PH! And, in the 10 years since starting my support group, I have also learned to not be so shy with people. Sure, I can keep quiet sometimes, but more often than not, I put myself out there and let others know about this awful illness. Awareness is the key to so many answers, and I never know when one day I will talk to someone and they tell me they have PH, too!

My life with PAH is something I cannot change, and honestly, I think it has helped shape me to be the person I am today. Do I have days when I am angry about having it? Of course! I allow myself to have those angry days, or days of self-pity, but not for long. What good does that do me? I have learned to deal with this disease, and to cope the best I know how, and I have learned to help others going through the same thing. I have also not let PAH BE my entire life. I enjoy things like reading, painting glass, making jewelry, spending time with family and friends. In the last few years I have allowed myself to take on challenges I never thought I could do, because I always had that "cannnot" attitude with me. So I've gone on hikes, I've been on a jet ski, I took my first ever trip on an airplane! There are so many more things I've let myself try, even if I may not be able to do them again. I try my best not to let PAH take over my life, because if I did, I can't honestly say that I'd be living to the best of my ability!

Monday, 2 February 2015

Partners with PH: Josh and Nicole

So often people spend years searching for that thing that inspires there life and gives them purpose. I am lucky because I found my inspiration early in life and get to reflect on it daily. It is an incredible feeling to have that kind of inspiration day in and day out. I am going to pause here and give you a little background before I go any further…

My name is Josh and my wife’s name is Nicole, and I was asked to share with you a little about our story. Nicole and I met in college and it didn’t take long for us to realize that we were in love and meant for each other. We got married May 13th, 2006. In a three week period of time I graduated college, got married to the woman of my dreams, and started a new job with a Fortune 500 company that had endless opportunity. Life couldn’t be better. I felt like nothing could slow us down.
About two months after we got married, Nicole started having recurring episodes where she would pass out if she pushed herself to hard physically.  At first, the episodes were not severe and according to her doctor at the time, were something Nicole would just have to sort of “manage” by adjusting our lifestyle and taking medications. 
Prior to us getting married Nicole had some very minor symptoms but nothing like this and doctors never seemed to be worried. One doctor told her the symptoms were all in her head and that nothing was wrong with her. It’s like I knew something was wrong but since the doctors didn’t seem too worried about it, I was hopeful it wouldn’t get to bad. I just knew that I would be there to help her and we would get through it, whatever it was.
In a short period of time the symptoms worsened and her passing out turned into violent convulsions. One night she had an episode that was so severe she stopped breathing.  I started to give here CPR and called 911.  I had no clue what I was doing but somehow she came to and started breathing again. I am not sure there is a way to explain the fear I felt at that moment. I thought she was going to die in my arms right there. Lucky for us God was on our side that night.
The paramedics arrived shortly after I got her to start breathing again. We ultimately ended up in a hospital in downtown Kansas City where she was admitted and moved to the critical care unit. Doctor’s ran several tests to try to figure out why she was having these episodes.  She continued to have them in the hospital and actually coded two more times and was revived by the medical staff. I felt like life was falling apart and I was losing the most important thing in the world. After a few days of being admitted she was diagnosed with Primary Pulmonary Hypertension (PH). I remember feeling confused and wondering what that really meant.  Is it treatable? Is she going to be ok? 
The doctor explained the disease was a chronic lung disease that causes the heart to fail and that that the average life expectancy (at the time) after being diagnosed was 3-5 years and with a heart and lung transplant it could extend from there. He also noted there were treatment options but very few patients actually get better after starting treatment. For most patients treatment only slowed the progression of the disease.
Looking back I remember thinking heart and lung transplant… really?  How is this happening?  She is so young and really took care of herself. Why Nicole, why us? Life is not fair. Anger, pain, denial are all feelings that came to me over the next few days. There is no way to describe the experience we went through. This was the bottom.
Well let me explain how things can change.
While life at that moment seemed lost, God had a plan for us. I have never seen anything like it in my life. Nicole was determined to get better so we could live a full life together and it was like God was with her every step of the way.  There is a power in positive thinking because Nicole always finds a way to bring out the positive side of every situation. Each day Nicole seemed to get better. Am I saying it was easy no, there were days of struggle, sickness, and symptoms but somehow we got past it.

Over the next few years, life slowly returned to “normal”.  By “normal” I mean exceptional. Nicole got better and better.  She went from being on constant IV infused medication to an inhaler (nebulizer).  Growing up in Pensacola, Nicole dreamed of the day she could swim in the water again and guess what… she does every summer now! Not only is she physically better, we have grown as a couple and as a family. We have two beautiful children now (both adopted since we couldn’t biologically have children). They are amazing and the light of our life. If you think that PH is the end and you can’t get better, stop and know that it’s not true.  Sure there are days she doesn’t feel the greatest and yes she is still limited by physical activities but in the grand scheme of things, she can do more than she can’t.
We are an example of how being positive will carry you through. Pretty sure it’s evident by now but going back to where I started, Nicole is what inspires my life and fills me with purpose. Sometimes people tell me I am a good person because I have taken care of her but what they don’t know is that it’s the exact opposite.  Without her I would be lost. She is an amazing mother and wife that is the driving force behind our family. 
So for those of you that have someone you love going through this be positive and uplifting.  Your spouse/significant other needs your support and deserves it.  It can be tough but I promise you it means so much to them just for you to be at their side smiling and telling them you love them. Live in the day and know that God’s plan will take you where you need to go. It is so important to enjoy every day of the journey, even the tough ones.  Let the little things life throws at you go as it’s not worth it to stress over. Who knows, you could end up like me with all your hopes and dreams coming true.

**Nicole's previous PHighter Friday story can be found here.