PHighter Friday: Elsa

My name is Elsa. I am a South African citizen, currently residing in Australia. I was born with congenital heart disease and a VSD was closed at age 10. During the procedure it was discovered that I had PH and I was left with a ASD that could not be closed due to the pulmonary pressures. I was not put on any medication at the time and was just sent home.
I suffered from frequent chest infections and in 1986 I ended up in hospital for 6 weeks with pneumonia and water in my heart sack. I nearly died but with the grace of God and my mother's persistence to insist a different antibiotic I pulled through. I started drinking Garlic and Parsley, and cod liver tables every day and until today I never had a chest infection again. Please note that this is my personal experience and in no ways intended as a medical opinion.*
I met my husband and got married in 1993. We were married for 5 years before we decided to start a family, but knowing the risks we consulted a intern specialist before and we were advised not to have a baby and rather seek alternative options. My husband's sister offered to be a surrogate mother and our baby boy was born healthy and strong without any health issues.

In about 2004 I was getting out of breath more easily and was sent to the city to see a specialist. He was brilliant and started me on Adalat, Spiractin and Aspirin. I was advised to start using nocturnal Oxygen too. This was the first treatment I got since 1983 when PH was diagnosed. It made a huge difference in how I felt and the oxygen at night would charge my batteries for the next day. I have always known my limits and would live accordingly but the new medication and oxygen just made my life easier.

I believe in living life as normal as possible and I have been working as a qualified hairdresser. When we moved to a rural location I opened my own hair salon from home, and worked full-time from 9-5 as a stylist. I had a full time maid that would help with chores in the house. This way I was free to work in my salon.

In 2011 PH south Africa was established and I was asked to be on the committee to raise awareness. We had a very active group, but in 2013 my husband was offered a position in Saudi Arabia and we moved to Jeddah. At first I thought the heat would affect me, but it did not, and living at sea level made a huge difference in how I felt. I had more energy.

I have joined the Saudi Association for Pulmonary hypertension as a honorary member assigned to Patient support. As a foreigner to a closed Muslim community that was a very big honour. I have learned a lot about the disease when I was invited to the International seminars we had in Oman, Abu Dhabi and Jordan.

In 2015 my pressures went up to 120 and I was put on Sildenafil as a treatment. I am using 75 mg of Viagra, and my pressures have since dropped to a stable 75.

Earlier this year we moved to Australia and I decided to have a career change. I have taken up a course in Pastoral Care and Dementia, and I am volunteering at the local nursing home 3 times a week.

I have always been a positive person, and I would not let my condition define me. I live life to the fullest, and believe that a positive attitude to live is beating the disease with at least 50%. If you look around you will see people that is worse off than yourself, and that keeps me going and motivates me. I live in a 3 level house and the stairs is a challenge, but I do one level at a time. I go for lengthy walks on a level surface and that is the best exercise for me. It is important to listen to your body and rest when you need it.

I am 43 years old now, and I am still going strong. Don't give up hope!

A doctor once told my mother I would not live to the age of 10....and gosh did I outlive that prediction. Live life to the fullest of your ability, and stay positive and strong. Thank God for every

day as it is a precious gift.

Take care and feel free to join my Facebook page SAUDI ASSOCIATION FOR PULMONARY HYPERTENSION - SAPH.

*The PHight or Flight Project does not endorse or recommend taking any medications, including natural supplements without discussing them with a medical professional. Please be sure to discuss your treatment options with a professional, such as a specialist. The PHight or Flight Project does not offer medical advice, and the opinions are based solely upon the writer.

PHighter Friday Follow-Up: Colleen

On January 2, 2016 I hit my eight-year anniversary since diagnosis. This makes me a long-term survivor, I have officially defied the odds.

It’s a curious milestone to celebrate. Of course, I am deeply grateful to be here. There is so much living left to do. The flip side of the statistic, however, is that half the people diagnosed the same time I was are no longer here. It’s a fact I am acutely aware of far too often… watching others with this disease suffer and pass on is  something that has never ceased to cut through me.

Although incredibly hard some days, I can’t stay in that dark space – I think that knowledge is perhaps the most valuable lesson (and skill) I have learned these last eight years. It’s a balancing act between weathering the really hard parts and then getting back up and focusing on the good. In fact, this lesson has been so deeply ingrained in me, I wrote a book on it.

“Defining the New Normal: A Guide to Becoming More Than Your Diagnosis” is the book I published in November 2014. By 2015 it was on the Amazon bestseller list, a feat that not only shocked (okay, and thrilled) me, but one which left me really humbled. I knew when I set out to write this book, that everyone knew somebody who would need it (it’s for all people rocked by a major diagnosis, not just PH). I just didn’t know how many people would resonate the way they have. It’s just… well, it’s just the best thing knowing that this really crappy PH thing can be turned around for good. I am nothing but grateful for how it has been received, and grateful to the Caring Voice Coalition and PHA for supporting my efforts.  And I absolutely love traveling to speak to other patients about how they too can define a life they love, no matter the diagnosis.

In addition, I’ve been given the opportunity to speak to the FDA, Women in Government and PH related industry several times. I really believe it is the patients who have to take our stories right to the change makers, and the policy makers. They need to hear how their every decision impacts our wellbeing and survival.

A book is fun, but the most significant thing in my world as of late has been the adoption of our daughter this past July. We started with fostering the tiniest sweetest little four-pound bundle in November of 2014 (oh yes, she was placed with us the same week my book launched – no pressure there!). Eight months later, in a little courtroom, a judge ruled her ours. Forever.

That moment was literally a dream come true. I had wanted to adopt ever since I was in college. For a long time it looked like PH was going to derail that dream. Questions about how sick I might get, and how bad the outcome might be, kept my husband and I from moving forward.

Finally though, it seemed I was going to be pretty stable for a good long time. I have a kick-ass team of doctors and each one of them was 100% behind the idea of adoption – which was incredibly reassuring.

She and my nine-year-old son keep me very busy. They get most of my energy these days. Adopting was the best decision, but caring for an active toddler and a boy on the move is no joke! I’m writing a lot, and coaching clients all over the country to better health on their terms. I don’t think this journey is ever going to be an easy one. But in choosing whether I PHight of take Flight… I choose to stand and PHight… or sit down on the couch and PHight from there if I have to – because let’s be honest, PH fatigue is no joke either. Either way, I’m defining this life on my terms and it’s a life I love.

*This entry is a follow up to Colleen's original #PHighterFriday, which can be viewed here.

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: Alex

From left to right: Alex with her sister Debbie

I had already spent many years sick and in bed by the time I was diagnosed way back in 1998. I had delivered my third and final daughter, Luzy, in 1996. I was always exhausted- sleeping, anywhere from 18 to 22 hours a day. I would get up in the morning, make my kids breakfast, turn on the television, and fall asleep on the couch. I'd get up to make a quick lunch, send the kids outside to play with the neighbors, and fall asleep on the couch. I'd then get up to make dinner, wait for my daughter's father to get home from work, hand him the girls and say "I'm exhausted, I need to sleep," and I would sleep for the rest of the night. This went on for many months until I went to a psychiatrist's office, and told him what was going on. I had no idea where else to go, or what was going on. I explained that I had delivered my gorgeous baby. The first thing out of the doctor's mouth was postpartum depression (of course.) I was pumped full of antidepressants and started doing better, bit by bit, day by day. 

After my ex husband got out of the military we moved to Texas, where I began passing out. I was just getting out of the shower the first time (that I know of) when I passed out due to Pulmonary Hypertension. I stepped out into the hallway, walked towards my bedroom. The next thing I remember was being woken up by paramedics. Apparently when I fell I hit my head against a wine fridge that was stored just outside the bedroom. We were living in my former husband's mother's home at the time. When I was found I was the paramedics were called.  They checked my blood pressure and pulse, and said that everything was fine but suggested I go to the emergency room just in case. I did not have insurance and declined because I was afraid of the cost of going to the E.R, and riding in an ambulance.

I had all but forgotten the fainting spell I had until a few weeks later I passed out again. This time my late sister, Susan, was visiting us from Southern California. We were playing chase around the house with my youngest daughter, Luzy. She was just under two years old, and was having such fun playing and running around the huge yard. I sat on the steps in front of the home and said "I'm tired..." The next sound my family heard from me was head hitting the cement steps, and sliding down several steps from the top. Emergency was called again, and I declined help.

I passed out in total at least 30 times in a three month period. I couldn't bend down to; clean up, tie my shoes, pick up one of my daughters, without getting short of breathe and passing out. I could no longer walk from my from front door to the car, without having to stop at the top of the steps to catch my breathe. Walking to the bathroom or the kitchen from the less than one thousand square foot home, would wear me out nearly completely. My oldest daughter had to take care of her sisters because I certainly couldn't. My daughter's father was working far away, and would come home on the weekends. Even seeing how sick I was, he refused to try to transfer to a prison less than 15 minutes from home. His mother refused to help as well saying it was all in my head, and I that better snap out of it. She had been told about being me diagnosed with postpartum depression and decided that I was just depressed. My family was in Southern California, where I was born and raised.

Finally, my sister Debbie Drell came to visit. She saw me pass out, and have a grand mal seizure (I had several before the time before I was diagnosed.)  I had told her of all the times I had gone in to see the doctor after I had passed out. This doctor always refused to do any testing or blood work. He just kept upping my dose of Prozac. It wasn't until Debbie saw me pass out that she made my former husband go with my to speak to the doctors. Luckily, the medical professional I regularly saw wasn't in that day. We saw a different doctor, who, once listening to my symptoms, listening to my heart and lungs, said to me, "This is not in your head. This is in your heart!" I was immediately sent for an echo-cardiogram, a type of ultrasound test to the heart. The next day I went in to work and called as soon as the office opened. The conversation that took place I remember as if it happened yesterday, but in fact, it took place 18 years ago!

"Mrs Flipse, you need to come in right away" said the nurse. "But I'm at work. Can I come in tomorrow?" I asked. "No, the doctor needs to see you today." Knowing the test was of my heart, I had to ask the nurse on the line "Am I ok? What did the test say?" to which her response was: "You're standing aren't you?" When I hung up, I went to my boss and I said that something was wrong with my heart. I had to go see my doctor right away. My boss didn't want to let me go until my lunch hour, but I knew I had to leave right away. I never returned back to work at that place. 

I asked my ex mother-in-law to come with me to the appointment at the cardiologist. She came certain that she could chastise me after, expecting that there was nothing wrong with me. We didn't wait long at all for the cardiologist, they were expecting me. He told me that I had a terminal disease called Primary Pulmonary Hypertension. My ex mother-in-law asked him how long I had to live. The doctor stated that I had less than two years. He said I was pretty severe and my heart was very enlarged.  After that, I had no idea what else was said.  Right before leaving the room, the doctor kneeled beside me, putting his hand on my knee saying, "if I do one thing, I had to remember the word prostacyclin."  He said it would save my life. I left the hospital dumbfounded. Sobbing in the car ride back all I could think was what about my girls? What about my husband? His mother said to me "Buck up. You can't cry. Grow up."  I sat straight up and did not say another word the rest of the day. 

I was referred to see the Pulmonary Hypertension specialist in Houston Texas, which was a four hour drive each way. I woke up attached to Flolan after my very first Right Heart Catheterization. I stayed on Flolan for four years,  and  transitioned to Tracleer as soon as it became available on the market. I was transitioned off of Tracleer and onto Sildenafil after my latest Right Heart Cath, which showed that I also have Pulmonary Venous Hypertension.  

Over the years I have learned several things, and I would like to share a few of them with you, Please. keep in mind, that even though I have learned these things, it is very very hard to do them!! 

1. Advocate for yourself! I can advocate for anyone else on this planet, and do it running circles around everyone. However I can still not advocate for myself. Since I know that I cannot, I always have a member of my family with me when I go to see the doctors, or have an emergency room visit. If I feel like I won't need to speak up for myself, I go alone and take notes, or record audio of the visit. 

2. Take notes at your appointments, and always go in with questions or comments about your condition, or things you don't want to forget. This is so important for me because I forget so quickly! While I'm taking notes, I miss out on something important, so after having asked permission, I record the entire appointment with the doctor. 

4. Keep a list of all your doctors and their phone numbers in a wallet or purse. I also keep the list in my phone. I keep this information in my  name in my contact list.

5. Keep a list of diagnoses, who treats that condition, and the medications, with dosage, in the wallet or purse. This is also on my phone. I even have my over the counter meds listed as well (like vitamins, tylenol, etc.)

6. Keep yourself busy. I don't ever just sit and stare into space, or sleep the day away, unless my body needs it. If I do, I'm in depression, and know that I need to do whatever I can to get myself out of depression. I was diagnosed with clinical depression when I was 18, but have had it since at least five years of age. I keep busy by doing things I enjoy- like crafting, cooking, playing with my granddaughter, watching movies on Netflix and listening to music, Facebooking and of course raising awareness! 

and lastly but not least in the bunch:

7. Exercise. When I was diagnosed back in 1998 I was told not to do any exercising at all. Cardio was completely out of the question because I didn't want to overwork my heart. After many years of studies researchers and PH specialists are finding that we do need to exercise! Exercising is something that I rarely do, but I know that I should exercise. Unfortunately, getting out of the apartment for more than a few minutes is very taxing on me. However, when I do manage to get out and exercise I really enjoy myself!  

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: 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.

PHighter Friday: Donna

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

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

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

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

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

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

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

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

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

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

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

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

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






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