PHighter Friday: Tegan

First international trip to Japan 2010

My PH story starts when I was six, sometime in 1995. I suddenly started having fainting episodes and was quickly diagnosed with Epilepsy, having uncontrolled Tonic-Clonic seizures. 

Within a year I developed a chest infection/cough, that wasn't going away with antibiotics. The local doctor sent me and mum down the road to have a chest x-ray. I don't remember specifics but I know that it landed me pretty quickly in the Royal Children's Hospital Melbourne on the Cardiac ward. Throwing my family in to chaos with my two younger sister being looked after by my grand-parent, while mum looked after me.

Being diagnosed with "Primary Pulmonary Hypertension" Functional class 4 (now known as Idiopathic Pulmonary Arterial Hypertension) was life changing to say the least. This diagnosis didn't just effect me emotionally and physically, but my entire family, my circle of friends, my school life, after school activities and general living. I was initally started on "Diltizem", and given a 5 year life expectancy. As a 6 year old I was blissfully unaware of this.

I managed to go to school full time, partake in sedate after school activities and play with my 2 sisters. If I forgot to take my medication, or refused (as i did in the beginning), I could feel the difference, and would often have a seizure as a result.

Around the 5 year mark I got really sick. I was unable to start high school, I was in and out of hospital trying to manage the symptoms. The addition of a few medications and eventually oxygen.

Hosptial stay 2001/02, recently started
 Epoprostenol and looking much better already

I tried "Beraprost" for a short time, but this made me feel sick three times a day for about thirty-sixty minutes after each dose. It made me feel isolated from my friends as I had to go to the school sick bay to have it during class, and rest until the side effects wore off. It also didn't make me feel much better. 

This period is very vague to me and everything sort of blends together. I know I had multiple seizures at school, ambulances were called on multiple occasion, late night trips into the city to get admitted quickly and always extra drugs and bloods and tests. Mum and I spent countless hours stuck in traffic on long stretches of road on the way to the hospital, we played many cars games, but often I was vomiting or simply too lethargic to play. 

Around this time I was placed on the transplant list waiting for a heart and lungs. I was too ill to go to school at this time, nothing was working. I made it through the year using a wheelchair, oxygen and many many medications. I was in heart failure and the rest of my organs were soon to follow, fluid retention, vomiting and weight loss among the symptoms. I was palliated, something i wasn't really aware of. New teams of medical professionals were seeing me all the time to help with one symptom or another, there was a constant stream of consults, reviews and medical rounds. I had privileges from hospital, and small windows at home.

Early 2001 I was given the green light to start this magical, mystical drug from the US (Epoprostenol) as well as oral "Bosentan" (plus my cocktail of "normal" meds). Conditions applied, a fair few. Mum had to be trained in making the medications, and had to commit to making it up from powder to liquid every single day. We had to be self caring with a PICC line (a semi-permeant line directly into the heart.) I had to do any test they requested (6 minute walks, ECHO's, ECH/EKG, bloods and Cardiac catheters). I was so unwell I was not yet able to have the permeant line, so I had a temporary one inserted with light sedation. 

Kakadu National Park 2014

The temporary line proved to last quite a while. By the time the 2nd one had packed it in, I was well enough to have a bit more sedation and get my permanent line. This was a pretty good indicator that this drug was working. I made leaps and bounds from here. I came off the transplant list, I was no long made palliative and I slowly started going to school. I had missed a few months of school and had to be slowly reintroduced. Luckily with a supportive school, and some great friends I got back into it.

The combination of drugs has changed a bit over the years,. I'm no longer in any organ failure, and I take a portion of the drugs I was taking. I take 2 medications for PH, Epoprostenol and Sildenafil. However, I do take a few a preventions of to make life a little bit easier with symptoms.

It took me a very long time to come to terms with my illness and accept it as part of my identity, but not something that will define me. I can thank the use of this drug for the life I lead now.

I work full time as a paediatric cancer nurse, I ride my bike 7kms each day to work and I have the travel bug. I have it in a big way. 

I still have to undertake the same tests, just much less frequently. I can't swim in the ocean/pool, I can never be too far from a hospital and I have to carry around a pump everywhere I go 24 hours, 7 days a week. BUT I will always try to do this with a smile on my face and a positive attitude!

I've been to over 20 different countries and can never wait to plan my next one. Of course I have limitations, but I'll take what I can get.

Repackaging 3 months worth of medication
for my upcoming holiday

My next trip is the biggest and the scariest and the absolute most exciting thing that I have been able to achieve since diagnosis. I have spent the last 6 months planning everything I might need for a 3 month holiday in Sweden and Europe. There is about 40 kg of supplies and 4 kgs of clothes.

This is a recent development as I switched to a more stable version of my drug. It means more freedom and less volume of medications (Thanks Veletri!). 

It has been a lot of preparation, research and challenges at every corner. I started to document my issues with travelling with a serious chronic illness and intend to continue while I travel.

I know I've had a hard life but I know its also been full of interesting moments, filled to the brim with love and most of all its been surprising. When you exceed the doctors expectations by 15+ years you learn to live life to the full.

There are so many people who help me with my challenges. I feel this is the key to my success.

Stonehenge England 2013

My blog can be found at https://www.tumblr.com/blog/tegamegadrive and my instagram at https://www.instagram.com/tegamegadrive/

PHighter Friday: Brooke

Ever since I can remember, I loved being busy. So much so, that at 4 years old my parents enrolled me in almost every sport imaginable – dancing, figure skating, gymnastics, swimming, the list goes on and on.

Figure skating seemed to stick and over the next 17 years, the sport would take me on some pretty spectacular adventures. I spent my entire childhood on the ice – 4, 6, 8 hours a day and by the age of 14, I was a national level athlete in both singles and pairs skating.  I represented Canada at several international skating events and spent hours in the gym when I wasn’t on the ice.  My aerobic capacity was second to none.

Which is why I found it alarming, in the spring of 2013 (only a few years after retiring from figure skating) that I was unable to keep pace with my Mother during a charity bike ride.
At the time, I brushed off my inability to breathe as simply being out of shape and found comfort in the idea that more training would help.

Unfortunately, it never did.

Fast forward to February 2014 when my world was forever changed.
It was the morning of the 2014 Olympic Gold Medal Hockey Game, Canada vs the US, and my sister and I were walking downtown Toronto to meet some friends for the early morning game. Out of nowhere, I had to stop. It’s hard to describe what I was feeling in that moment because I can’t really recall. I was fully aware of what was happening around me but I was struggling to even breathe. Needless to say, I visited my local ER shortly after – thinking I would be told I had asthma. Long story short, after almost every test in the book, I was discharged ten days later, with a mystery diagnosis and referrals to both a cardiologist and a respirologist.

It was on March 19th 2014, during my appointment with Dr. Granton at the Pulmonary Hypertension Clinic at Toronto General Hospital that I first heard of Pulmonary Hypertension.

With my PH diagnosis, my world was turned upside down.
Not only was I diagnosed with PH but I also learned that I have two congenital heart defects, both of which made it a miracle that I was able to compete at such a high competitive level when I was younger.

So then came the next dilemma – do ‘we’ operate on the two congenital heart defects (Atrial Septal Defect and Superior Vena Cava Displacement) in the hopes that by correcting these two defects we would stabilize the pulmonary hypertension? Or do we treat the pulmonary hypertension and the congenital heart defects as two separate entities?
Again, after more tests than I’d ever care for, it was decided that there was too much risk involved for the potential reward with regards to correcting my congenital heart defects. They would stay!

To limit the progression of my pulmonary hypertension, I am currently on Adcirca, Tracleer, and Xarelto (even though my PH is not related to blood clots, there are studies that show better prognoses while taking blood thinners). These medications are literally saving my life!
My doctors tried me on Bosentan prior to Tracleer but it was causing some less than ideal side effects.

Growing up, I was an independent, young woman who was comfortable and confidant. I don’t mean that in a cocky, over-pretentious way but in the sense that I knew who I was and was comfortable with who that was. After being diagnosed with PH, I felt as though that had been taken away from me. The body I had known and relied on for the past 24 years had failed me.

I found it hard to let my friends into my ‘new normal’ and instead of relying on my friendships to move forward, I found myself pushing them away.
I hate to burden anyone, and with the state of mind I was in post-diagnosis, I felt as though that is what I would be to my friends. Hindsight being 20/20, pushing them away was the last thing I should have done and the last thing they would have wanted.

Fast forward almost two years later and here I am, better than I was pre-diagnosis but still learning to adapt to my new normal. I still find it difficult to talk about my journey with PH and I have moments where thinking of what the future holds paralyzes me with fear, but I try to remind myself to live in the moment. After being diagnosed, I was so closed off from the real world and guarded with friends and family – I refuse to go back to that.

As backward as this may seem, coming from someone with a chronic illness, I am one of the blessed ones. I’m surrounded by some amazing friends and family who would reach the ends of the earth if it would help. I now realize that that’s pretty priceless!

As a patient, it seems that every time I think I have found my footing some test, complication or setback rears its ugly head.
I won’t sugar coat it, adapting has had numerous challenges. I think the hardest part of my diagnosis was realizing that there was no cure; no way to make the incurable go away. Being sick is being stuck in a limbo of sorts where feelings of anxiousness and depression can creep in at any given moment, with very little notice. The future becomes one big question mark (even more so than any other average 20-something).

And although it’s easy to get lost in the negative aspects of Pulmonary Hypertension, I try to remain positive - for as much as PH has taken from me, it has given surprising gifts as well.

Throughout this journey (that I’ve hopefully only just begun) I’ve met some amazing people. PH has intensified my love of life, taught me patience where I had little, and shown me how beautiful and fragile life can be.

Like I said earlier, I love being busy – that hasn’t changed. I currently work a full-time job (that I love), a part-time job (at a local hospital) and go to school part-time (Go Marauders!).
PH definitely makes this tricky and I find that I tire more easily than before but I’m a firm believer in mind over matter, and until I’m no longer physically able to do the things that bring me comfort (like staying busy), I plan on keeping on!

I don’t know why it takes hardship to gain insight, but it does. And as much as I wouldn’t wish chronic illness on anyone, I’m grateful for the insight it has given me.

Being sick doesn’t only make you weak, it makes you strong. It gives you knowledge – you learn to stop and smell the roses. You know it’s not important to sweat the small stuff because you’ve had bigger things to worry about.

 My life may be far from perfect but I am blessed.

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: 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!

PHighter Friday: Elise

When were you diagnosed with PH?

I was diagnosed with PH at the age of 19. Thirteen years ago.  At, the time I was living in Long Island, and was being my usually active self, when I realize the tasks of everyday living including walking were becoming extremely difficult.  I remember being at the gym and doing a leg workout, and having to sit with my head between my legs from being so dizzy.  That same summer, my mom came down to visit me from CT and as we were walking on Jones Beach, I couldn’t take more then 5 steps without having dizziness, ringing in my ears, and shortness of breath.

After finally passing out a few times, it was tine to see a cardiologist.  And after several tests including an EKG, Echo Cardiogram and a right heart Catherization it was determined that I had primary pulmonary hypertension and was in stage 3.  Being so young I didn’t quite fully understand how life threatening this disease was. Growing up as competitive dancer and being a personal trainer and weight lifting, I just wasn’t going to let this get in my way.

Medication:

After responding to the first oral medication Traceller and being on Procardia XL I was back and able to perform my daily activities, and even went back to the gym to train and workout.    With motivation behind me I decided to compete in a fitness competition, and I successfully did 3 years after being diagnosed.  Along with competing in the Miss CT/Miss USA pageant with the platform of raising awareness for PH.

Daily Activates:

I’ve been a very good responder to the medication and have recently gone off of the Traceleer and now am just taking Norvasc 5mg, (generic brand to Procardia). This year all my tests came out normal showing that my PH is very much under control; I’m able to walk, run, lift weights, and do it all.  I’ve found that keeping my body in shape helps.  The only thing I do realize is that I need a lot of sleep, because my body gets run down and I’m more sucseptable to getting sick when I don’t take care of myself.

The Hardest Part of PH is the medical insurance part. Because I don’t work for a corporation and I am self-employed getting insurance and good insurance to cover all the expenses is so difficult.  When I was younger I was on my parents insurance and I stay in college purposely as a full time student until 26 so that I was covered.  I was also on Cobra for a while and paying over $600 per month on insurances. The struggle has really been financial especially because I’m now on my own.

What does the future hold?

 I’ve been extremely fortunate that I am in the 10 percent who responds well to being on 1 medication, but since going off of the Tracleer most recently, I will have to go for tests (EKG, ECHO, PFT’s) every 3 months to make sure that nothing has changed.

I don’t know what the future holds I could get worse with age, or maybe I could stay the same? I’m not sure. All I know is that I never take life for granite and I appreciate and live life to it’s fullest. 

I’ve accepted the fact that it’s probably not in my best interest to birth my own children but I’m okay with this.  I was always a believer in giving a child a home who needed it.  I’ve come to grips with life, and decided that I am the only one who can really make my dreams happen.

Elise Firestone
www.elisefirestone.com
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PHighter Friday: Clair

My diagnosis came on much like it does for many other young women.  During high school, I began to notice an increasing amount of chest pain, a racing heartbeat, and shortness of breath.  Characteristic of a developing adolescent, I denied that these symptoms were abnormal.  I attributed my inability to breathe and instances of passing out as being out of shape.  However, after a year of tests, hospital visits, and appointments with doctors and specialists, I was diagnosed with Primary Pulmonary Hypertension. 

As a senior in high school preparing to leave for college, the diagnosis was devastating.  Even though my doctor gave me the green light to go away to college and a positive long-term prognosis, I began to find that the emotional toll of having a progressive disease was more difficult than its physical manifestations.  Even though I attempted to cope with the anxiety that stemmed from my diagnosis with PH, it began to extend to other aspects of my life.  I began to doubt what I could make of and do in my future, and this doubt precipitated new insecurities.

Although there wasn’t a dramatic turning point, the woman with PH I am now is a stark contrast from that newly diagnosed teenager.  I changed the way I took care of myself.  I began to eat healthier by eliminating junk and adding fresh produce to my daily diet.  I also started practicing yoga and working out.  Of course, my workouts are modified.  I’m not running miles upon miles or doing intense cardio, and probably never will. But I keep a consistent schedule of yoga and lead a rock-climbing and yoga club for my students at the school I teach.  I do what I can, and I’m certainly doing much more than I ever thought would be possible after receiving my initial diagnosis

This past summer I spent 6 weeks in Europe.  I climbed Mount Vesuvius, a large volcano in Italy, climbed over 600 stairs to the top of a church in Florence, hiked in the Alps, and visited 6 countries.  The people with me understood that although I said yes to every adventure, I was going to do it at my own pace.  When I climbed Mount Vesuvius, I stopped a little at first, but as the altitude increased, I had to stop every few steps.  In our group, I was the very last person to make it to the crater and I didn’t care. I was only focused on completing my journey to the top no matter how long it took me.

Now a decade into my journey with Pulmonary Hypertension, I’m currently in the process of planning a trip this winter to Zambia, Africa.  Disease or not, everyone has one life.  Dwelling on what will or will not happen will only cause unnecessary worries.  Had I never broken free from my initial doubts or my perceived, grim outlook on my future, I know that I never would have taken the chance to start yoga, or to attempt an ambitious climb to the top of a volcano.  However, I was allowed to do both of these things, and I will continue to experience life, because I am committed to doing the absolute most that I can with my disability.  I will not allow Pulmonary Hypertension – or the psychological toll that comes along with it - to take away my potential and the opportunities that come my way.