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: Kris Ross

The road to diagnosis was incredibly long and frustrating. I was mostly symptom-free as a child. I would get blue lips when I was cold which was attributed to being so skinny. In my later teenage years, I started having breathing difficulties when I would do physical activity and when it was cold out. I was told I had a reactive airway and an intolerance to cold so I should use an inhaler before going to the gym and before going out in colder weather. My blood work indicated high hemoglobin so I was sent to a specialist who did some nuclear medicine tests. He diagnosed me with “couch potato polycythemia” --- basically, my hemoglobin was high because I was lazy and didn’t do very much physical activity.

Over the next decade, I signed up for gym memberships I couldn’t use, despite my best efforts, and tried different inhalers. I had high hemoglobin results on my blood work during this time period but I was assured I was fine and my doctor told me he wished all of his patients could be so healthy. Finally, after a couple of my colleagues told me I didn’t sound like I had asthma or a reactive airway during a basketball game, I started questioning things and decided to go to a walk-in clinic to explore things further.

In July 2002, I went to see a doctor at the clinic. We had never met before but he listened to my concerns and sent me for a chest x-ray and scheduled some cardio-pulmonary tests and an arterial blood gas. The x-ray was deemed normal but my lips and fingers turned a bluish-purple shade during the cardio-pulmonary tests and I had pain between my shoulder blades. My arterial blood gas was 44. I was sent back to the clinic immediately and, once I convinced him I had never been a smoker, the new doctor told me the only things that would give me these types of results were a major heart or a lung problem. I was scheduled to see a respirologist the next week. He determined my x-ray was not normal because my pulmonary artery was enlarged. He felt the most likely reason was an undiagnosed hole in my heart.

Within two weeks, I met with a cardiologist and had my first echocardiogram. It revealed a large ventricular septal defect (hole in the wall between the two pumping chambers of my heart). Over the following three weeks, I had a number of tests, culminating with a right-heart catheterization. It was determined I had pulmonary hypertension caused by the hole in my heart. It was left open for so long that my lungs were irreparably damaged. To say this was devastating isn’t enough to really capture how I felt. The future I had imagined for myself was gone and, in its place, I was left with fear, anger and uncertainty. The road to a diagnosis took a decade to travel and there was some thought that had I been diagnosed when I first began exhibiting symptoms the hole could have been repaired and the lung damage minimal. My body was my enemy.

There were very few specialists at the time so I was sent to Toronto for assessment. I was told, untreated or unresponsive to treatment, I might have a couple of years before I would need a heart-lung transplant, if donor organs were available. Thankfully, the medication worked well, stabilizing things, and I was able to return, mostly, to the life I’d known before. There were things I would never be able to do and there were things I had always wanted for myself that would never happen. I mourned them and had to move on, living life as fully as possible. My family and friends were great supporting me, adapting things so I could be included in their activities and events. Eventually, they opened a Pulmonary Hypertension Clinic in Ottawa at the Heart Institute, closer to my home, and I starting being treated by the fabulous team there.

I was stable and leading a relatively normal life for years until many bumpy months last year after a nearly fatal case of pneumonia. I had excellent care at the Heart Institute and from family and friends. With some medication changes, months off of work and a staggered return, I was able to get back to my pre-pneumonia state --- even better in some ways.

It’s 2016 and I continue to be relatively healthy with the support of family, friends and the team in Ottawa.

PHighter Friday: Brooke

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

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

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

Unfortunately, it never did.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

PHighter Friday: Anastasia

Slowly but Surely

I wanted to travel the world. I wanted to be a powerful business woman (like Samantha Jones in “Sex and the City”) and have a family. Also, I love sport such as swimming, bicycling, playing basketball, playing badminton and playing tennis.  Most of my childhood was filled with fun activities, travelling, hanging out with friends and breaking the rules. But those dreams were crushed when my doctor told me that I will not be able to do sports that I love, I will not be able to travel, and having children is out of the question….

I was diagnosed with Primary Pulmonary Hypertension when I fifteen years old when I moved to the U.S. Back in Indonesia, I was always chubby and sluggish back in middle school and part of high school. My parents thought it was because lack of exercise and my general doctors in Indonesia did not detect any health issues. When I was about to start high school in U.S., through regular check-up, the family doctor detected irregular heartbeat and I started to notice apparent health issues such as difficulty breathing, chest pain and blue lips. So, I was urged to see a cardiologist and pulmonologist. Sure enough, I had VSD (Ventricular Septal Defect) and PPH (Primary Pulmonary Hypertension).

I was devastated…

For a while, I could not understand why this defect happened to me. Although, overtime I realized that wallowing is not getting me anywhere.

After the diagnosis, I had my open heart surgery on my 16^th Birthday to close the hole in my heart. I am taking medications to control my PPH. It is not easy to integrate this disorder into my life at first.

I realize that I am rare. So, as long as that is the case, why not go all the way.  So the key is to accept that I do have this unique condition and I am going to have to live with it for the rest of my life.

So I learn to get to know myself.

I am thirty years old now. I am working full time as a supervisor, I volunteered at convalescent homes, I go to the gym at least three times a week, I went hiking (5 miles up the hills recently) and I am finishing my bachelors in Management and International Business. I do what I can now and everything else will fall into place. I may not go with the phase of normal people, but I will finish what I start slowly but surely.

I changed pulmonologist who is better and gave me support. She advises that I should exercise and keep doing what I do at my own phase.

I believe that our journey gives us strength; the destination is just the icing in the cake. And getting to know myself is the fundamental part of starting the journey and setting up goals. Also, it is important to surround myself with people who are supportive of me and my goals.

Life may not always go as planned, but it does not mean that I cannot make the best out of the one I have now. Like Mother Teresa said ““Yesterday is gone. Tomorrow has not yet come. We have only today. Let us begin.”