PHighter Friday: Brit

The first thing I ever learned about pulmonary hypertension was that Stage IV patients like myself only lived 6 months post diagnosis.  I read this on the first hit off of Google because my diagnosing doctor only handed me a prescription and sent me on my way.  Upon seeing a specialist, I was told I probably had a good two years in me if I was lucky but I’d never work again.  I was 23 years old.

At the time it seemed like my entire life was telling me what I couldn’t do; my body wouldn’t let me complete the simplest tasks, I couldn’t keep a steady job due to my health, my medical bills were through the roof.  I couldn’t live on my own because I could hardly take care of myself with the frequent hospital stays.  I could barely even manage the relationships in my life.  The disease made me sick and the treatments made me sicker; the medications I took for the treatments made me even worse.  It was like a cruel joke  

At about two and a half years post diagnosis I hit a plateau - no longer was I so sick that everyone thought I was going to pass out and die every time I attempted a flight of stairs!  I was just sort of there.  I put in my 8 hours at a desk (long gone were my days as a pastry chef) then I went home.  Most of my friends had stopped calling on me and my biggest ambition was to make it a few months in a row without a site infection.  Not exactly what I expected from life.

Finally one night I relented and went out to see a band play in a small seated venue downtown with a friend.  I survived the minor anxiety attack of climbing a flight of stairs in front of others.  We ended up going out for drinks afterwards with the band and a bunch of people neither of us knew and didn’t make it home until 3am.  To my surprise I still woke up the next morning!  And even better, I had such a great time with all these strangers.  I had forgotten what it was like not to be treated like the sick girl.  That’s when it occurred to me that I had been spending so much time focusing on staying alive that I had been doing a really poor job of living my life.

That one change in perspective changed the entire direction of my life.  Instead of watching bad TV every night at my mom’s house, I moved into an apartment with a friend.  I started spending more time with people who only knew the post-diagnosis me so there were no expectations of who I was or what I was capable of.  I went back to enjoying the things I loved - food, live music and traveling.  And even though I was being harder on my body I was feeling better than I had in years.  Medicine had taken me far, but this new attitude made a world of difference in my capabilities.

Today I am nearing my 32nd birthday and 9th anniversary with pulmonary hypertension.  I do pretty much anything I want (as long as it doesn’t include stairs or anything athletic).  One of the most important aspects of my life is my volunteer work with the PHA.  What started as being active in the chats has grown into fundraising, advocacy, creating programming for young adults, speaking at conferences and most recently creating notepads for doctors so patients find out about PHA early and don’t have the diagnosis experience that I had.  The ability to feel like I’m fighting back empowers me whenever I feel helpless against this disease.

Here’s some wisdom I’ve gained through the trials and errors of almost a decade of life with pulmonary hypertension:

Don’t put a timeline on yourself.  Keep having dreams and goals and go for them!  When I was first diagnosed I stopped going to the dentist because if I was going to die in 6 months I didn’t really need to go to that teeth cleaning, right!?  After about two years I realized that was probably a bad idea.  Now I keep a google doc of places to see and things to do and eventually I will cross every one of them off.  

Don’t be scared to have people love you.  It’s easy to shut people out using the “it’s all going to end badly” excuse or the “they won’t get it” excuse.  But guess what, there’s someone out there willing to love you regardless of what tubes are coming out of your body and regardless of what the statistics say.  Don’t waste valuable time because you’re scared.  Treatments are getting great and we’re going to be around for awhile.

Ignore the people who tell you no.

Let me put a caveat on this -  we do have some limitations as PH patients and you should listen to your doctor/body.  But don’t listen to the blanket statements that say things like you’ll never work again, never live a normal life again, you’re going to die in xx years.  You can do whatever you want - it just takes willpower, patience, and the right combination of drugs. I have a PH friend who ran one of those Warrior Dashes and I could’ve never done that at my healthiest!  It’s about setting a goal and going for it!  I own a house, work full time, and travel whenever the bug hits.  That’s a far cry from the girl who couldn’t get enough breath to complete a sentence.

Having PH is hard - it’s a complete life adjustment.  But it’s up to you to decide whether you use it as an opportunity to slow down and really appreciate every moment and do those things you want to do but maybe wouldn’t normally do or see it as a hinderance that drags you down.  It took me 2.5 years to make that decision so it’s not something that comes without a little effort.  I’ll close with one of my favorite quotes:

“Always when you’re crying you could be laughing, you have the choice”  -- Andy Warhol

The PHight or Flight Project would like to thank Brit for sharing her incredible PH journey for PHighter Friday!

* If you have an inspirational PH journey that you would like to share to help provide hope to those who are newly diagnosed, please contact me at phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.

Life Leading to Diagnosis

I was diagnosed with Idiopathic Pulmonary Arterial Hypertension in December 2013. Before diagnosis I had just started my first real grown up job. I was managing a busy office at the University I graduated from. In June of 2013 I was flown out to Montreal to have a week of training for my new job. My flight to Montreal was my first flight, and I was terrified. I watched Lost too much when it came out. (To the left is a picture I took on the flight home.) I would spend hours each day after my training wandering around the city. I would then go back to the hotel and work out. I never felt short of breath, and I was certainly during active walking around the city. It was an amazing experience. Never did I think I would graduate from University with a degree in Fine Arts and find such a fulfilling business oriented career. When I came home from my trip I felt the most sick I had ever been in my entire life, which says a lot because I was often ill as a child and have had my share of hospital stays. I didn’t want to miss a day of work so I went to work despite having flu like symptoms for a few weeks. I recovered and felt better, life resumed and I felt fine.

A very flattering picture of me eating a hamburger in Montreal

At the end of August of 2013 I was in a bus accident on my way uptown after work. When I left work at 5:00 pm it was bright and sunny out. The forecast called for rain, but it was so nice out that I decided to leave my umbrella at the office. By 5:12 pm a tornado warning from Environment Canada went out on to almost everyone’s phone on the fully packed bus. About a minute later the sun was gone. The sky was completely grey and it was raining down sheets of water that blurred the windows on the bus. Moments later I heard screaming from other passengers on the bus. A bolt of lightning hit an electrical box in front of the bus causing a small explosion. The bus had stopped moving. Then an electrical pole had fallen just missing the end of the bus where I was sitting. A live electrical wire dragged down the window in front of me and hugged the bus' window. Next; two larger trees fell just in front and just behind of the bus.

A picture of the bus after the accident

There I was, stuck on a bus in the middle of an almost-tornado near a construction site. Debris continued to be picked up into the sky and thrashed to the ground. I thought to myself, this is it, I am signing off. Everyone on the bus was in a panic and of course I was stuck near engineering students who were discussing how screwed we were with that wire snuggling the bus. A tweenager decided this was a good time for an Instagram session. I can’t decide who wanted to break her phone more; her father or me. I decided to call my parents, just in case things got any worse. It was a very scary phone call- part of me thought there was a good chance I would not make it off of the bus. I asked them to stay home because the weather was so bad, there was nothing anyone could do. Emergency workers didn’t come until after the storm cleared up. It felt like an eternity trapped on that bus. My anxiety was through the rough. Once the storm passed a bunch of university students came out of their houses to take pictures on their smart phones of us trapped on the bus. Great. There we were, trapped on a bus, surrounded by dozens of people taking pictures of us. Eventually emergency responders came. The roads were blocked so I had to walk 30 minutes to meet my dad at the nearest open road. It was difficult walking by all the wreckage, but I never felt short of breath. In fact, prior to this I was working out for nearly 2 hours each day while working full-time!

By the middle of October I started to notice that I was short of breath, but I have always had asthma that has flared up during autumn so I didn’t think too much of it. It really didn’t feel that out of the ordinary. After all, I was followed by a man after getting off of a bus after work in October. I was able to haul some ass to my destination (the hair salon) where I was able to tell the front counter that I was being followed. Sure enough, the strange man followed me into the hair salon. Luckily the ladies there whisked me away to a hidden area and confronted the man and he ran away. I bought bear spray and a rape whistle- I thought I was protected from nearly anything now!

By November I noticed that something was wrong, this was no longer asthma. I noticed that sometimes I would be completely breathless going up the stairs. On a trip to Toronto I had to stop on the way back to the hotel from a bakery because I was completely out of breath. This was really unusual because I love walking; I used to be able to walk for hours and hours on trips. I never got tired or short of a breath from walking, nor did I ever have asthma symptoms from it. I realized something was “wrong.” My feeling lead me to the ER where I told the doctor that this was not asthma. There was no way, I haven't had an asthma attack since I was 14. She did not listen and just gave me steroids. What I find the most unusual is that this ER doctor did not even TOUCH me. She didn't ask me to walk and measure my 02 saturation, nothing that would have indicated more asthma.

I tried going to the family doctor where he agreed it wasn’t asthma, but he didn’t know what it was. He tested me for various poisons but everything came back clear. As the month went on I felt worse and worse.  By December things had become “scary.” I remember crying and telling my dad that I knew that something was wrong with me.  I woke up in the middle of night gasping for air two or three times. At work I could barely leave my desk to go to the bathroom. I could hardly walk up the 5 stairs to get to the office. I could no longer walk to the bus stop because it felt like I was going to black out trying to make it up the hill. I went to the ER again. Luckily this doctor took my symptoms more seriously. I was hooked up to a machine where they noticed I had something called “flipped Ts.” They were concerned about a blood clot in my lungs and sent me right away for x-rays and a CT scan. It came back clear but she noticed inflammation in my lungs. That was on a Thursday. On Monday I went to the hospital to visit the airway clinic, where they were going to discuss asthma treatment with me. (The previous ER doctor from November had made this appointment.) I told them that this wasn’t asthma and someone needed to help me. I did a lung function test and it turned out I was at 43% lung capacity. A medical student then came in and asked me a bunch of questions. A lung specialist then came in and touched a vein in my neck and let out this heavy "you're totally screwed" sigh. He said I might have Pulmonary Hypertension but that they needed to run a few tests to confirm.  
My dad and I waited to be admitted to the hospital so they could run more tests on me first thing in the morning. I looked up Pulmonary Hypertension on my phone while in the waiting room and made my dad promise he wouldn’t look it up. All the information I found said I had maybe 3 years to live, and that I would need a lung transplant. I stayed overnight in the hospital on a floor where it was mostly older people who were very very ill. My roommate was someone with lung cancer who just got out of ICU. It was terrible to be around so many gravely ill people. Nurses took me during breakfast to run more tests. No one would tell me what was going on, and my doctor was MIA. It was very frustrating being wheeled from test to test with no information. Around 6:00 pm the doctor came sand said it appears that I have Pulmonary Hypertension and that they would be sending me to a specialists. The news hit me hard. How could this be happening to me? I had a lung/heart disease but I had done everything right to take care of myself. I didn’t smoke or drink. I ate healthy and worked out. It didn’t seem fair. The doctor asked if we had any questions and my dad said yes but we don’t want the answers to any of them right now.

The days, weeks and months following diagnosis are still too scary to relive and share- so I will leave that part blank for now. I hope to be able to look back someday and think "Wow, that was scary but I am so much better now!"

During my “early retirement” or “vacation from hell” I have started The PHight or Flight Project. The months following diagnosis involved me doing a lot of PH research.  All I could find was information about how screwed I was. Everything online that I found concluded that I maybe 2-3 years left, and I refused to believe that. Eventually I had found a few stories about people who have had PH for 20 or more years. One story that stood out was one I had found in a chatroom about a man who won a million dollars through the lottery, and then was diagnosed with PH. His doctors told him he had maybe a year left so he spent all of his winnings. He was talking about how he was upset with his doctors for telling him he had a year left over 20 years ago, because that lead him to go through his winnings within several months after diagnosis. I know this sounds like an episode of Lost, but I swear I read it.

What I try to tell myself each day is that my future is unwritten. If you wouldn't trust a psychic with your future, don't put your future completely in the hands of statistics. Anything is possible, I mean look at the odds of having IPAH. (The odds of having IPAH is one in a million.) So who is to say things won't go my way, even if there is only a one in a million chance? I have already proved that I am one in a million!

PHighter Friday: Lindsay

Hello!  I'm Lindsay Thurman.  I was diagnosed with severe pulmonary hypertension officially in November of 2008.  I was Class 3 at the time and I had trouble walking up a few steps and had passed out several times doing things that most people would not even think twice about doing.  When I was first diagnosed I was told that the condition could not get better but they could stop it from getting worse.  I was also told that I would probably only live for 8 years at the most (this was 6 years ago).  My doctor at the time didn't say much about what I could or couldn't do.  He pretty much told me to avoid stairs and to for sure not have children.  That particular doctor wasn't very helpful and he made things sound pretty bleak.

Now I am on two medications (adcirca and opsumit) and last summer my doctor told me that I was now Class 1.  I usually walk 2.5-3.5 miles a day with my dog at a brisk pace (about 3.5 mph).  My life is very different now than it was at diagnosis.  After being diagnosed I finished my bachelor's degree and I currently teach high school math at a Title 1 school which requires a lot of energy!  This summer I'm getting ready to start on my master's degree.  I'm more active and feel better than I have in my entire life.

When I was first diagnosed I think the hardest part for me was having to cope with my own mortality.  It's very scary at 23 to hear that you aren't supposed to live past 31.  I also had a hard time with the fact that I would never be able to safely have children of my own.  Coping wasn't easy and I found it hard to talk to people about it because I didn't want to worry friends and family.  I'm a Christian and I turned to God with all of my frustrations and fears.  Now that it's 5.5 years later my outlook has changed a lot.  I know that there is no way to know how long I'll live.  I went from Class 3 to Class 1 which I find amazing and I feel great so I don't expect to die anytime soon.  However I have made peace with the fact that there's a possibility I will die young.  I think that my disease has helped me to focus on what's important in life and let go of the little things.  Knowing I can never have children is still and will probably always be hard for me.  I might adopt someday but I might not.  I try not to think things have to be one way or another and just treat each day as it's own adventure.  I look forward to the future but I live in the present because while I don't expect to die as young as my first doctor said I would, I do respect the fact that I have a very serious disease.  Besides, even healthy people aren't promised tomorrow!

I think the best advice for someone who is newly diagnosed is to first off know that doctors don't have all the answers and in my experience the best doctors know they don't know everything and know that anything is possible.  I would encourage them to celebrate any victories and let go of disappointments and stresses.  Hope is everything.  I could have believed my first doctor that I wouldn't make it another decade on earth and just given up and my life would be very different now.  I would also encourage them to listen to their body.  Know and respect the limits of your body but also understand that those limits could change.  Do what you can to stay healthy by eating right and I really enjoy walking but since many people with PH have trouble there I would also highly recommend yoga! 

The PHight or Flight Project would like to thank Lindsay for sharing her incredible PH journey for  PHighter Friday!

* If you have an inspirational PH journey that you would like to share to help provide hope to those who are newly diagnosed, please contact me at phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.

Preparing for Visits with Your PH Specialist

Last week I had a visit with my PH specialist. I am very fortunate to have been sent to such a great specialist that is so close to where I live. Fortunately the PH clinic is located just an hour or two out side of the city where I live. Specialists visits can be difficult, especially at first when everything is still a new experience.

To make the most out of my specialist appointments I make sure to keep a list of all the questions that develop in the weeks before my visit. If anything unusual happens make sure to write it down. Write down the details of what you were doing when the event happened. If the event feels serious, or if you are worried about anything, don't be afraid to call your specialist right away. Don't feel like any questions or concerns must wait until your appointment to be addressed if anything has you worried. I have needed to call my specialists a few times with some concerns, but luckily he called me back very quickly and assured me that these new feelings I was experiencing were all part of my diagnosis.

I live at home with my wonderful parents, so I make sure to include advances or set backs they notice. Even though I am more in touch with my symptoms, my parents have been able to make connections that I wouldn't have. Some of my non-PH meds were making my PH symptoms worse and causing insomnia. I chalked this up to my PH, but by talking to a doctor and stopping/switching medications we were able to pin point why I was feeling worse. I have also found that keeping a journal where you document your symptoms, medication, and achievements for each day helps for documenting progress and set backs. You're specialist will want to know about your progress/achievements as well so make sure to remember these milestones to discuss with your specialists. I shared with my specialists what I have been able to do in terms of physical activity, my 02 levels without oxygen at rest, and my heart rate.

My father also comes with me for my visits. I would recommend bringing someone close to you for support. Because my parents are my caregivers, my father often has his own lists of questions to ask. If you feel comfortable with your caregivers/parents/partner/etc asking questions- go for it. As a caregiver my father has different insight into how I am doing and we make sure to discuss what questions he will be asking ahead of time. At my last visit I got both good and bad news. My dad is there to remind me of the good news and how we should focus on that. Realistically, the bad news is that I have severe Pulmonary Hypertension.  However- the good news is that I am responding "beautifully" to the medication. If you are newly diagnosed, remember to be patient with yourself. These things take time. I was in very bad shape when I was first diagnosed, and although I trust that I will continue to improve, I know that improvement will take time.

You will certainly want to remember what you discussed with your specialist. Don't be afraid to take notes during your visits. You may even want to ask if your specialists minds if you record your discussion for better documentation. (Most smart phones have voice recorders as a built in app.) I like to keep notes from my visit because I can track my own progress, and I can ensure I have all the answers I need from my questions in the chance I forget. It can also serve as a sense of comfort between visits too. It may be comforting to hear or read the positive notes from your visit. My visits keep getting spread further and further apart. One month, two months, three months, four months. I assume this is good news that they don't need to see me as often, but as the months drag on I find it difficult to not have any medical "feedback." The notes help remind me that I am doing well despite my diagnosis and some expected downfalls. For example, my oxygen level dropped (even with my supplementary oxygen) while doing my 6 minute walk test- but I improved my walk test since my last visit. I was very proud of how far I walked. My specialists said it was good progress and he even set a new goal for me for my next walk test! Remember to balance the good with the bad.

If you have to do a bit of travelling for your appointment, do what you can to get the most of your "trip." I'm on a special diet at the recommendation of my naturopath- so I make sure to bring food with me since I have a difficult time finding healthy food that I can eat. If you know that you will be having a walk test, pack accordingly. Bring any snacks you might need to eat before or after, and make sure you stay hydrated. At my last visit I raided the gift shop and found some cute slippers. I decided I should get something at the gift shop every time I visit to reward myself, along with allowing myself some French macarons (which is not included in my special diet.) I think it is important to take the time to remind yourself that you are special, and cared for, and take joy in simple pleasures like small sweets and slippers. My father and I also always make plans to go out for a nice dinner on our way back home. However, we have a rule that if we are too sad after the visit it is perfectly okay to go home right away and just eat there.

If you received some news during your visit that has set you back emotionally, give your time to feel that emotion. When you are ready (and sometimes you might have to push yourself,) pick yourself back up and move forward in whatever way you can. If you find that in a few days/weeks you have any questions or concerns from your last visit don't be afraid to call your specialist for clarification.

Some of us might feel uncomfortable being so assertive. I would encourage you to step out of your comfort zone because your health is worth it. It is important to be your best advocate in anyway possible in terms of your health. If you don't understand something, do not be afraid to ask your specialists to explain something until you feel you fully understand the information being given to you. This will empower you to take better care of yourself. Chances are your specialist would feel more comfortable knowing that you have a better understanding of your current situation and health as well.

In case you were wondering, my father and I went out for a nice dinner on our way back home from the specialists last week.

PHighter Friday: Christine

My life is a little bit different from someone who was just diagnosed with PH. It's different in the way that I was born already having PH because I have congenital birth defects such as: ASD & VSD, Scoliosis, & Restrictive Lung Disease to name a few. So, I was pretty much told from day one that I'll never be able to do this or I should do that. Basically my time clock started on day one and after spending my first month of living at a hospital, my mother was told to come get me and take me home because there wasn't anything left the doctors could do for me. That was in 1969. I will be turning 45 in September. Yippee!!!!

You're probably wondering how I do it. How do I beat the odds? Never, ever give up. No matter how much life sucks or how dismal the outlook seems, you keep marching forward. I am a very realistic person who doesn't want explanations from doctors sugar-coated. I want facts. Facts are something you can work with... Not the "what if" scenarios that can keep you in fear of a future event that may or may not even happen. 

In 2006, I started going to a Pulmonologist who specializes in Pulmonary Hypertension. I went through the whole process of getting correctly diagnosed with PH for insurance purposes because my breathing was getting worse. At this time, I was already using oxygen 24/7 and taking cardiac medication for Ventricular arrhythmias. Although, I was still going out dancing at a dance club... Just no longer drinking any alcoholic beverages which I only did socially anyway years ago. Besides, I don't need alcohol to have fun! *wink* 

Since 2006, I've had a slow decline in health (so, no more dancing...boo!). I'm getting shortness of breath a lot more and I've had to increase my oxygen flow now. Instead of using 5 liters per minute 24/7, it's now at 6 LPM. Each step of a decline in health hits me hard emotionally. There's really no way to prevent the emotion side of things but if you can accept the fact that the human body wasn't created to live forever in pristine condition, then it becomes easier to move past the emotional part. Once you are past the emotional part of accepting how you are physically today, you can actually learn new ways of getting things done in your daily life. There are going to be things you'll find that you can no longer do on your own or at all. It will make you mad. It makes ME mad that I have trouble doing things that I once was able to do easier than before. It will help if you have family and friends who are willing to understand your condition and are able to pitch in to help without making you feel as if you're an invalid which is so important! There is a fine line that helpers need to figure out with the person who has PH where the helper helps up to a certain point. I really believe if a helper does everything for the person with PH, the patient could end up giving up on life's little joys. Then, depression sets in...which is a huge problem when living with PH. If you notice that you are getting depressed, talk to your doctor. Get help.. Don't ignore it. 

My advice for people living with PH or just diagnosed with PH is give yourself a break. You did not cause this to happen to yourself. It's not your fault. It is very ok to allow yourself a "ME" day every now and then to give your body and mind a whole day to just rest. Find something that you absolutely love to do and have fun with it. For me, that would be reading, container gardening, or crocheting. Or just flip through a good cookbook to find a new recipe to experiment with. You need things that make you smile on days where you feel bad because, yes, you will have those days. Just don't let yourself get lazy. Try to stay as active as you can but remember there will be days where you feel like you just can't do it. Give yourself a break. It's ok.

However you chose to battle PH, do not let it do more harm than good. Take your medicine as prescribed. If the medicine isn't making you feel better or is giving you a bad side effect, contact your doctor to see if you can try a different medicine. Don't just stop taking it and not tell your doctor. That's dangerous. Also, if you are suppose to use supplemental oxygen, then use it like you're suppose to. By not, using your oxygen properly, you are only harming your heart by making it work much harder than it needs to to pump more blood out to your body. You will end up starving your heart, brain and other muscles from that much needed oxygen that you refuse to use. So, give your heart and lungs a break and add possibly years to your life by using your oxygen and taking care of your body. Stay away from sick people. I do whatever I have to do to avoid getting sick. I get the flu shot every year. I haven't had the flu in over 10 years. I also wash my hands a lot with just soap and water. You can use antibacterial soap but regular soap works just as well.. In my opinion! During flu season, my best friend are Lysol wipes....especially if my husband is sick. I tackle doorknob, tv remotes and light switches multiple times daily. Like I said, I do whatever I can to stay healthy. 

As for getting use to wearing your oxygen in public, it can be a little scary at first. I write a blog all about Living On O2 for Life. People are going to stare because they are curious. Little kids will ask about my oxygen a lot to their parents first and then to me. I ALWAYS take time to talk to people who stop and ask me. There's usually a family member or a friend who they know who uses oxygen or about to start using oxygen. If I can help them make life just a little bit easier for them, it's worth a few minutes of talking with them. Don't you think? I didn't have anyone to ask back when I started using oxygen when I was 17 years old and still in high school. Of course, there was no internet either!

Life is worth living. So get out there and live it the way you can not by what you can no longer do.

Christine

livingono2forlife.wordpress.com

The PHight or Flight Project would like to thank Christine for sharing her incredible PH journey for  PHighter Friday!

* If you have an inspirational PH journey that you would like to share to help provide hope to those who are newly diagnosed, please contact me at phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.

PHighter Friday: Tarya

I had my first echo the day my dad was checked into palliative care. It was a Friday. I knew it had to be bad when I got a call two days later (on a Sunday, no less) from a cardiologist, asking me to come into the hospital the next morning. The day after my dad died, that same cardiologist told me I had a rare, progressive disease called pulmonary arterial hypertension.

I had recently completed a degree in public health, so I was no stranger to health research. The first thing I did was start googling around on this strange, unheard of disease. Hypertension? That doesn’t sound so bad. How bad could it be?

Bad.

I have since learned that google is not my friend when it comes to PH. The statistics floating around the internet, the infamous 2-5 year lifespan stat that crops up everywhere, are just plain outdated. The first treatment for PH only came out in the late 90s, and there are people still walking around who were in those original clinical trials. Yes, PH is serious. Yes, it still cuts too many lives far too short. But the drugs we have now and the research going into understanding and treating PH have made those statistics obsolete. 

When I was first diagnosed, I assumed the worst. I thought that I wouldn’t be able to take care of my son, that I would never work again, and that all I had to look forward to was increasing disability and death. I think the only thing that kept me from falling into a very dark place was my son, Alex, who was just a year old at the time. Regardless of what was going on in my life, he needed me to wake up with him, feed him, play with him, and do all of those regular, everyday things. He brought me a great deal of joy, and helped me to live in and enjoy each moment.

That was almost two years ago. Despite what looked like a grim future for me at diagnosis, I responded really well to the medication, and went back to work (part-time) less than a year after I started treatment. I went from 10 hours a week to begin with up to 20 hours a week within four months, and my nurse practitioner recently gave me the green light to go ahead and try working full time if I want to. I haven’t decided yet if I want to, but it’s nice to know it’s not off the table.

I think the biggest thing my journey with PH has taught me is this: The only difference between the living and the dying is that the living don’t realize they’re dying too. I take better care of myself now. It’s funny – I had to get sick to take care of myself the way everyone probably should. I make sure I get enough sleep, I eat well, I rest when I need to, but above all, I enjoy myself as much as I can. I still have days when I get angry, when I mourn the life I thought I was going to have, but then I do my best to focus on the good stuff.
Because life is short. For everyone.

The PHight or Flight Project would like to thank Tarya for sharing her incredible PH journey for  PHighter Friday!

* If you have an inspirational PH journey that you would like to share to help provide hope to those who are newly diagnosed, please contact me at phightagainstph@gmail.com. More information about sharing your PH journey for a #PHighterFriday can be found under the Share Your PH Journey/Contact tab.

Travelling with Oxygen, by Loretta

Travelling with Oxygen

Loretta, PH Patient, Co-leader of the Toronto Chapter, PHA Canada

I've been fortunate to be able to travel POST PH dx. I've been able to go to France, California, Seattle and Florida in the past five years! I was on 2L at rest, 3-4L walking, 2L sleeping. I've been 24/7 O2 since 2006. Sadly, my traveling days are over now as my health has deteriorated greatly! boo hoo. Here is what I've learn over the years:

BEFORE FLYING

Did you know there's a test you can take to determine how much extra oxygen you may need? It is called a High Altitude Simulation Test (HAST Test). I highly recommend you do this test. It is simple and will put your mind at ease when you fly. During the test, you basically do a ABG at room air, then breathe in "airplane air", do another ABG and compare results. The test is available at St Michaels Hospital and maybe TGH. Luckily, when I took the test my O2 did not destat and I require the same amount of oxygen as on land. 

FLYING WITH O2

You won't be allow to use liquid oxygen on the flight. You will need to ask your oxygen provider for a Portable Oxygen Concentrator. So as long as your near a power outlet you won't run out of oxygen. The battery life however for the POC sucks. Most would last about 2-3 hours each (depending on the flow) per battery. So you will need a few batteries for the flight. There are a few models out there such as the Eclipse and Evergo that may go up to 4L. Again, you will need to check the manufacture websites and ask if your oxygen provider has that model. I'm with Medigas and they provided me both units before. They are bulky and you'll need to push it on wheels. Also, note that some of the POC can only provide pulse flow, so if you require a higher flow you may need a POC that can provide continuous flow POC. Pulse flow meaning the unit only gives a burst of oxygen when you inhale.

You can also purchased Oxgyen provided by Air Canada. Although, it is not worth it (It was $150 per leg when I used it for Paris) and you will still need to bring a POC to get around the airport. 

AT THE AIRPORT

Always request wheelchair assistance. You will basically have a chauffeur take you from the check-in desk, by pass all the lines, and pick you up after you land too. Also, request a seat near the front rows so you do not have to walk to the back. You may also want to enter the plane last so you can keep your POC charging on the wall outlet. The outlets on the planes, do not work! I tried!

To bring your POC on the flight. Your doctor will need to complete a Medical Approval (Fitness for Air Travel form) within 48 hours of departure and fax to the airline to approve your oxygen. Other air liners will have a similar form to complete. Here's example from Air Canada: http://www.aircanada.com/en/travelinfo/before/specialneeds/oxygen.html

Print a copy of the form and your Oxygen prescription with you!

WHEN YOU LAND

When I went to Orlando I paid out of pocket for liquid oxygen for the weekend. I found a local provider and they quoted me US$290. Yes, it is quite expensive and insurance does not cover it. This is truly a luxury item. They delivered a liquid tank to my hotel room and I bought my own Helios units to refill them up. I prefer using the Helios as they are small and light and fit in my purse. I don't like pushing a cart (Evergo POC) around! Purchasing liquid oxygen is totally optional and not necessary if you feel comfortable with your POC.

You'll need to send the local oxygen provider your doctor prescription. They are really strict on the usage, like if you required liquid and the flow at rest, walking, sleeping all need to be indicated. There are some companies that will not provide Canadians oxygen such as the big US national companies (Apria). You need to look for a small company (do a quick google search). Also, note that recently liquid oxygen is being discontinue because of high costs so it may not be available everywhere. Getting a walk-in prescription as some RTs has suggested is not an option and there's no government coverage that I know of!

TRAVEL INSURANCE

I see a lot of questions about this and it's hard to answer. A lot of insurance will not provide you if you have any "pre-existing" conditions. I had travel insurance with my work place and they said as long as my condition was stable at the time of travel, they would cover me. But it's really hard to say because they could deny you try to claim it. So fingers cross you won't ever need it!

Most importantly when travelling is good planning and be prepare for any worst case scenario. Have fun!

Loretta at the Louvre, Paris, France. October 2009.

A "Spirtitual Flat Tire"

I decided to start The PHight or Flight Project in hopes of finding my "purpose."

The writings of Dr. Bernie Siegel made me question why did this happen? I imagine most of us wonder "why did this happen to me?" upon finding out the diagnosis of Pulmonary Hypertension. However, Dr. Bernie Siegel wants you to question why this happened in a different way. He thinks of these often devastating, life-altering events as a "spiritual flat tire." According to Dr. Bernie, these "spiritual flat tires" are supposed to redirect us.

I was very upset that Pulmonary Hypertension happened to me, and I still am. I imagine all of us with this diagnosis have felt the same way at one time or another, especially when your chances of developing PH are 1-2 out of a million. I did not smoke or drink, I was vegetarian, I worked out everyday, I ate healthy - but the unexplainable (or as doctor's like to call it "idiopathic,") happened to me anyways. Sometimes I feel like all four of my tires are flat.

I have had a lot of time to reflect upon things. I decided I don't want the purpose of this so-called "flat tire" to be the end of me, so I needed to find a meaning in all this. Before all this happened I was very future oriented. I was always focused on working hard so I could be happy someday. Now I am forced to see how important it is to make time to enjoy things now, and to try my best to live in the present. Those of us facing this kind of illness need to make the present a priority. All of the long-term PHers I have spoken to have said that the best advice they could give me is to not worry about the future-  focus more on living in the moment. Perhaps this is advice all of us should try and follow, ill or not.

As a type A personality the thought of living with so much uncertainty is very daunting. I am still in the in-between phase of all of this...waiting for my heart to heal and waiting for the medication to help me stabilize. The feeling of losing control over my life and health has been very challenging as well. This is why I had to find my reason. In a way, finding my purpose and the reason for going through such a difficult diagnosis gave me some control of the control back.

I've decided that the meaning in all of this is to make something good out this experience. My destiny is not to die a premature death, but to live a full life in whatever time I have. For me, I want to share my experience because the waiting part of everything to kick in after starting medication and being diagnosed is petrifying. I haven't been able to come across many fellow PHers who share the same diagnosis story as me, and who have had to wait several months and counting to get off of supplemental oxygen. (This probably has the biggest physical and emotional burden on me.) I have a feeling that more people like me are out there, but that we just haven't "met" yet. My hope is that this blog makes this experience less scary and lonely for at least one person.

I mentioned in my first post that Google is not your friend when it comes to this. I found that I didn't have a lot of places to turn to find hope in all of this. I couldn't find the information I wanted or so desperately needed to see. The specialists told me that the average "life expectancy" with PH is 7 years, but that some people have PH for 20 years or more. They know of people or have patients who have had it for over 20 years are still going. They told me about a women in her 50's who has had PH since her earl 20's and manages to walk a mile each night after dinner! That is great, now where can I find out more about these people who do not fit into the statistics? How can I learn more about them? Now that is what I need to see.

The statistics with this diagnosis are poor- but they are also outdated. I decided that I was also being redirected to share these PH journeys of those who have clearly proved those statistics wrong. For a while I had this idea, but I was to scared to go through with it. I wasn't sure if I was the right person to take on such a task, but then I thought about how I wanted a place for people with PH to turn to for hope. I have found a purpose again, and it is very meaningful to me. I want to make this information, or at least these kinds of experiences with PH, more accessible to others because hope can be so powerful.

This "flat tire" has also taught me about myself, and in a way, about life. Spiritual "flat tires" teach us to laugh longer, love harder, and smile wider when we can. It has made me want to be better. For a while I wanted to give up, but the soul is a powerful thing.

I would recommend reading several of Dr. Bernie's books. As a retired surgeon, he has an interesting approach on the relationship between patients and their healing. He also shares stories of those with terminal diagnosis, who managed to beat the odds in one way or another. Amazingly, these stories share how these patients were able to defy these odds given to them. Although these survivors do not have PH, I think hope in hopeless situations can be shared. Dr. Bernie's believes that hope and positive thinking can effect your health, which I think is a great lesson to be learned as well. His books certainly gave me hope, different strategies on how to "heal" spiritually, and how to better cope with my diagnosis.

For anyone who is interested, below are the books by Dr. Bernie that I have read and recommend. Please click the title's to read the book's description and review on Amazon;

Love, Medicine & Miracles

A Book of Miracles

From Incurable to Incredible: Cancer Survivors Who Beat the Odds

Peace, Love & Healing