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: 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: 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!

PHighter Friday Follow Up: Reinee

Since my last post, I've just been trying to take in all of life's blessings. I recently participated in the Race Against PH held by Stanford Hospital's Vera M. Wall Center for Pulmonary Hypertension. I had about 20 of my family and friends participate with me and it was fun! The outcome of these annual races are pretty overwhelming with the amount of supporters and sponsors. It's amazing to see everyone come together to raise awareness for Pulmonary Hypertension. I didn't actually run the race, but I power-walked the entire way and saved my energy to jog across the finish line. My family and friends who ran ahead waited for me a little before the finish line so we could all finish the race together. That moment was amazing and it felt great. I've only participated in Stanford's Race Against PH one other time before, in 2011, and compared to that, the event has really grown. If you haven't participated in a marathon for PH, I urge you to. It's such a wonderful experience.

Another major thing that has been going on in my life is the process to have kids. I say "process" because it is in fact a process, a very long one. My husband and I are currently going through the process of gestational surrogacy. That is when they take our embryo and implant it into our gestational carrier, or surrogate. When it came to our talk about having kids, which was well before we got married, we knew that it was not in the cards for me. I cried. I was depressed, but I had work with the cards I was dealt. My husband and I knew we wanted to try to have our own kids and obviously, this was the only way. We both had long talks with my doctors, nurses, and social worker about the risks, but I was willing to take them... and so was my husband. I honestly feel like I'm at my healthiest I've ever been so now would be the perfect time. There was a catch to going through with surrogacy though, and that was to get off of Tracleer. For those of you who don't know, one of Tracleer's main side effects is birth defects. My doctor decided that I needed a 3-month wash out period so that Tracleer would no longer be in my system at the time of egg stimulation. There haven't been any studies on the effects of Tracleer on eggs, but there have been studies of the effects of Tracleer on pregnant rats. So my doctor wanted to be safe and wanted me to produce the healthiest eggs needed for fertilization. I wasn't worried at all, but my doctors were a bit concerned. Of course they had to be concerned, they're my doctors. Their exact words were "we are not encouraging this, but if this is what you want to do, we fully support you and will take whatever steps necessary to make sure this happens for you." And honestly, that was exactly what I wanted to hear. Support from my team of doctors is all I need to know that everything was going to be OK.

My husband and I are now at the point of looking for legal representation and we're also waiting for our gestational carrier to be cleared of all initial testing with our fertility doctor. Genetics testing has been done, bloodwork, psychological and personality tests have been performed, as well as physicals. I have been off of Tracleer for about 5 weeks now and I feel fine. The first week was hard. I thought I wasn't going to be able to take being off of Tracleer, but I think my body just needed time to adjust; it was definitely going though withdrawals, though. I experienced a tight, heavy chest and minor palpitations, but I'm not a stranger to these symptoms so I just took it easy and continued to work. I'm completely fine now, and I'm still on Revatio and Tyvaso. 

Aside from the PH race and surrogacy, I have also recently challenged myself to snowboard with my husband in Lake Tahoe. I was scared at first because the last time I visited Lake Tahoe I didn't do so well. The elevation got to me, BUT at that time I was not on all the therapies I am now. Honestly, this time I was real nervous and still skeptical. I even had second thoughts about actually trying to snowboard so I had my husband hold off on buying me a lift ticket. I sat and waited in the cafeteria until it was time to do my next session of Tyvaso. When my husband came to check up on me after he did a few runs, I just said "alright, let's go!" I figured that if I came here with the intent to TRY, I might as well TRY. I felt fine, nothing was really holding me back except fear. We ended up snowboarding for about 5 hours. I had more than a few good spills, but I got the hang of it and I think I have a new found love of snowboarding! 

Sometimes I feel guilty about sharing my experiences, only because I know there are newly diagnosed patients out there who are feeling horrible. I remember being newly diagnosed and attending a support group with a couple of patients who were doing so well. For some reason, it made me feel worse because there they were living life and here I was thinking mine was ending. However, I want all of you to know that it took me almost 12 years to feel this way. It wasn't an easy battle, either. Please know that I'm active because my body is at the point where it's allowing me to be active. I don't feel crappy and try to push myself to do these things. I also want all of you to know that challenging yourself, even just a little, will give you hope. Don't ever be scared to get off of oxygen, or go for a walk, or get out of bed, even. Give yourself a new challenge everyday, it doesn't even have to be physical, but if your body doesn't allow it then at least you know your limits. Don't ever give up on yourself, keep PHighting! And always remember, you don't live with PH... PH lives with YOU.

A Year in Review

Me vs Pulmonary Hypertension

It has been one year since diagnosis. I really expected things to be different.

When I started this blog another person with Pulmonary Hypertension told me to keep it light- no one wants to hear you complain or cry. While this advice is sound- it is difficult to explain how this process has been without sounding like I am complaining. Truthfully- the past year has sucked. There is no way around it. There is not a nice way to word it. It sucked- and that is okay. Things are allowed to suck. You are allowed to have a bad year- and it is okay to own it. So, if you don't want to hear about my year (which most people would categorize as the physical shits) I would skip this post and continue with your day.

Being diagnosed with Pulmonary Hypertension, especially so suddenly and at such a severe stage proves complex and difficult. It has complicated all of my relationships, and sometimes I carry a lot of guilt. I know this disease isn't just hard on me. It is hard on my parents, my family, my boyfriend, and the few remaining friends I have left.

Someone who cares deeply about me deeply told me the other day that sometimes he thinks that I am faking. I was initially hurt by his words, but then I thought about it. Other than the oxygen, I look completely the same. He has no idea how I feel, and even if I were to describe how I physically feel, he will not truly understand how it feels because he is not in my position. He will not understand that even though I look fine, I am exhausted from working harder than him just to breathe all day. Something like a wheelchair is a visual cue to others that someone is not able to use their legs. When you have a heart/lung disease there is no visual cue.

In the movie Maleficent, the main character has wings because she is a fairy. One day someone she trusted cuts off her wings in her sleep. When she wakes up and discovers her wings are gone she lets out a giant scream and sobs while holding her back where her wings once were. It sounds silly, but I really related to this scene. It felts as if someone, somewhere, decided someday to take away my ability to use my heart and lungs properly. When you are unable to breathe properly- it is hard to do many physical things that you one enjoyed. I can't explain to you how much I miss my independence and exploring the world around me.What hurts more is not being able to do a basic task, like dress myself without supplementary oxygen. Doing so without oxygen leaves me breathless and my lips blue. It is a shitty feeling for anyone. It is something you never expect to happen to you, especially at such a young age. My sense of normalcy has been taken from me, and I don't know why. Sometimes I wonder if I did something wrong to do deserve this. Was it something I ate? Was it karma? Was my name drawn from a hat?

I am in a long-term relationship with a wonderful young man who is in his 20s. He has been as supportive as he knows how, and I am thankful for that. I know it takes a lot of love and faith to stay with someone with a condition with mine- and I know that not even marriage makes people stay in situations like this. I obviously have some guilt about someone so young choosing to stay with me. I don't know what my health will be like in the future. I cannot give him biological children. I really don't know what could happen. I don't know what kind of future I could give to him, or promise him- or anyone.

Not many people have the life experience of dating someone with a serious chronic life-threatening disease. Oddly enough, this is something that I do have experience with. I was in a relationship with someone for nearly 6 years who had childhood cancer. There were a few scares that his cancer returned, this seemed to happen around Christmas (just like my diagnosis.) One year, the year I was supposed to apply to university, the scare seemed more serious than the others. They had to run more tests- and we were obviously very scared and worried. This is a story I have never told anyone else... I shared with a woman at work about the potential cancer scare with my boyfriend at the time, and she shared with me that she too had dated someone with cancer. She said that he was the love of her life- but she couldn't stay with him. She felt it was too much of a gamble to love someone who was more likely to get sick- and perhaps, die. She advised me to do the same and said I should break up with him before things get worse. In a weird way, her advice helped me because I disagreed with it so strongly. Her advice taught me to love everyone as much as I can in all the time that we have together- which is a beautiful lesson that not everyone is brave enough to follow. This woman chose not to spend the rest of her life, with the love her life out of fear. After that I chose to do whatever I could to help. I decided not to apply to university just in case the next round of results were not what we were hoping for. I was prepared to put my life on hold and take care of him, because everything else would be waiting at the end if he did need treatment. Luckily, his cancer did not return. I went to university a year later...I had an early acceptance and was offered multiple scholarships. Everything was waiting for me a year later.

As previously mentioned, I cannot carry children. Pregnancy increases the risk of mortality in women with PH. Earlier this year I was sent to a gynecologist office to discuss my birth control options. I was sent on a few months after diagnosis. The nurse there asked me how I was dealing with the diagnosis. I responded that it was a "total bummer." She gave me a look and asked "total bummer?" It is probably the worst thing that has ever happened to me, and that extends to those in my immediate circle who love me. Do I want to share this with a stranger? Not really- and I assume on some level a nurse understands the degree of suckage adjusting to that kind of diagnosis is. Being there was a horrible experience. The waiting room wall was plastered with faces of babies the office helped deliver. It dawned on me- this is a place people go for something joyous. I cried in the waiting room facing the pictures of children knowing I was there to prevent having my own because it is too dangerous. I cried speaking to the doctor about my options. It was too soon. (This was the second only time I cried at the doctors.) I imagine it is devastating to find out that pregnancy is too risky for most women of child baring age. I should mention that I have Teaching Preparation Specialization on my degree and worked at various child care centres- I was someone who obviously liked working with children and looked forward to being a parent. There has always been a desire to adopt. I am just worried I won't be healthy, stable or here long enough to be the mother I dreamed of being. However, there have been stories on this blog from other PHers who have adopted and they are 30-40+ diagnosis. How silly would it be if I let opportunities pass me by because I was afraid? What if I reach my goal of living to 87 years old and decided not to pursue anything because I was afraid I may not be here? That would be a life wasted. I am trying to learn that I cannot let fear decide my fate, nor can I let this disease decide my fate for me.

A video posted by serena (@worshipandtribute) on Dec 12, 2014 at 3:03pm PST

I like to end things on a positive note. Although this year has really sucked (like really sucked,)  I haven't accepted defeat. As I mentioned in an earlier post I started to do Wii Fit Yoga as soon as I was able to stand again after diagnosis. I would have to do this at 4 liters of oxygen per minute. Now I walk for 20-40 minutes on a the Wii Fit bored on 2-2.5 litres of oxygen per minute. I can also box using a punching bag and do exercises on a Total Body Gym on the same amount of supplementary oxygen. I never thought I would be on oxygen for an entire year (or more,) but I have clearly made great improvements. I can sit completely without oxygen for the entire day. I have returned to work part-time and only use my oxygen to exit and enter the building. I am starting to do small tasks without oxygen, and my doctor is confident I will get off of it completely once my heart heals as it is. I was in bad shape and I suppose it will take sometime for my body to adapt and heal. I am looking forward to 2015. I am okay with one year out of my life being the physical shits. I plan on having many more, so one year out of 87 isn't bad.

I have also learned that I really really really like having control. Feeling as if a disease has control over me is not an option for me. As such, I have found ways to give me power back against Pulmonary Hypertension. I have done research on the characteristics of Cancer survivors (because no research of this exists for PH.) Oddly enough, a lot of the characteristics of Cancer survivors overlap with the qualities I have found in the PHers who have contributed their story to this blog! I have been trying to embrace those characteristics and incorporating alternative and complementary health care into my life. I have seen a naturopath, Bowen therapist, a CHEK practitioner, hypnotherapist and a healer. It might sound a little crazy- but why not try anything and everything if it is not dangerous? I also regularly get back massages at the fanciest spa in town and I think the world would be a better if everyone could get one. I journal everyday. I do at least 20-40 minutes of cardio everyday. I have altered my diet to suit my new needs. I meditate, I do breathing exercises. I practice self love and do what makes me happy. I say no to things I don't want to do, and use a bathbomb from Lush every Sunday. I do research about Pulmonary Hypertension, medication, so on and so forth and I am my own advocate.

I am not sure what 2015 has in store for me, but I am hopeful that this will be the year I complete my "comeback."

PHighter Friday: Jamie

Post-transplant

I always find it incredibly difficult to explain my PH journey. Even within the small tightknit community of PH, most of our journeys are vastly different. My story starts as a disaster but ends five years later and includes a family, a job, school, and enough energy to walk five kilometers a day.

Let me start from the beginning. Firstly, I was diagnosed late… actually 23 years too late. I was born with a hole in my heart and after countless doctors’ appointments where I was either given antibiotics, or accused of lying to get out of work, I spun my car out on the busiest highway in Toronto, which finally led to my diagnosis.

After going to the local walk-in clinic, and having an oximeter reading done, I was diagnosed within the month with stage IV pulmonary hypertension.

My health for the next four years really only went downwards, oral medication did not work, subcutaneous medication did not work, and when I was finally put on IV Remodulin, while it was true that I was feeling much better, a month in a clot broke off the line and caused a minor heart attack.

The final breaking point before my life changed forever was here in Ottawa. I had chest pain so I texted my nurse coordinator who told me to go to emergency immediately. I was pretty busy, so first my wife and I signed my school loan documents before heading over. This was a VERY bad idea, my saturation levels were sitting at under 50 percent, and I was heading into full organ failure.

The decision my PH team came up with was to fly me to Toronto General for a transplant workup. It turned out that I was so far gone, that they put me at the top of the national registry immediately, and I had a double-lung transplant/heart repair within 48 hours.

As I said this was the shift in my life to make everything better. At the end of three months in Toronto, I was jogging, walking up seven flights of stairs, and doing things that I was never able to do.

 I’m not suggesting transplant is for everyone, certainly it has its up’s and down’s, but for me, I’m the healthiest – and happiest – I have ever been.

PHighter Friday: Carolyn

Dorset Tower

My PH symptoms began after the birth of my second child. My son was born February of 1996 my symptoms began in October of 1996 and on April 15th 1997 I was diagnosed with stage 4 PH.

Camel back in Morocco

My PH Journey is not like most, you know the usual, 6 months to 2 years. I was very blessed in receiving the news that I may not survive the night. With given only a few hours to live my family was sent for to say their good byes.

After surviving the night to everyone's surprise I stayed in ICU for a few days and then sent home to “die” in comfort and to make proper arrangements for my demise. After surviving a month I was given 6 months, that is when we decided to take our last family vacation and took our 4 year old daughter and 1 year old son to Disney World. After surviving 6 months I was given a year. Originally I was put on Norsvac until a heart and double lung transplant was necessary. To this day I am still at the same dosage of Norsvac. To my understanding the DR’s do not even suggest Norsvac to new patients as there are so many other meds out there that have show a quicker response to PH patients.

Great Wall of China

When I was 6 months in with PH I am thinking I may survive a little longer. I called the Lung Association to find out where the support group meetings are held. The Lung Association (at that time) had never heard of PH and was told to call The Heart and Stroke Association. They too had never heard of PH. Well then, I will start the first of its kind in Canada. So it began. We met at TGH once a month with a social worker present.

My sister had made pamphlets with pictures of the people that had attended group. Unfortunately, due to the privacy act no one new heard about our group. I just thought that nobody cared. I did get involved with the association in the US (PHA) and attended several of the conferences with my family in tow.  

I had PartyLite, Pampered Chef, Fifth Avenue Jewelry parties, Yuk Yuk fundraiser’s etc etc. With all proceeds going to then, Pulmonary Hypertension Society of Ontario, to support Dr Duncan Stewart efforts for a cure. That was way back in the 90’s.

Snorkeling in Bahamas

In the mean time my Pulmonary pressures are coming down from the original 104 at diagnoses. After a year I was given 2 years,you guessed it then 5 years. Now they do not give me a time of survival as it has been over 17 years. I have always been a traveller, but now I have a bee in my bonnet and want to enjoy as much of the world as I can. I have visited 52 countries to date. This past summer I spent 5 weeks overseas visiting my daughter in Swansea Wales. While there we travelled to Morocco, covered north to south,east to west of Wales. We journeyed to Puglia Italy and I drove through the south of England and Northern Ireland.

Carrick -a-Rede Rope Bridge Northern Ireland

Some of the many things I have accomplished since I have had PH.

While in Oahu Hawaii, I climbed the 175 steps to the top of Diamond Head.

While in Beijing China I climbed the Great Wall.

While in Athens Greece I climbed the Acropolis.

While in Pisa Italy I climbed the 296 steps to the top of the Leaning tower of Pisa.

While in Dorset I climbed the 128 steps up the Dorset Tower.

It is not easy but I am determined to do it.

Tubing in Jamaica

I love to Zip-Line and Snorkel. I walk 5km a day with my dog, Sheldon. In February I will be Para-sailing in the Caribbean. Some much to look forward too.  

I keep myself busy daily. I have not worked since my daughter was born, 21 ago. When my kids were in grade school I ran the milkshake and pizza days the school choir and the reading program. I was a Brownie, Girl Guide and Pathfinder leader for seven years. I run a ladies book group every Tuesday morning at my home. Every Thursday I go into Toronto to visit friends. I organize a yearly ladies cruise getaway in February. I host a monthly Quiz Night in Brooklin.