Preparing for the Holidays

Spencer telling me Star Wars spoilers

Hello everyone! Sorry I haven’t written any posts or had any PHight Fridays in a while. I hope to have more PHighter Friday stories in the New Year to share with all of you. If you are interested in writing for a PHighter Friday please send me a shout at phightagaisntph@gmail.com. From there I would be happy to send some examples, along with various questions to help with the writing process.

As the holidays approach I thought it might be nice to do a little post about how to prepare for the holidays. I know that although the holidays are supposed to be a happy to time, sometimes the holidays can be difficult for a handful of reasons. I was diagnosed with Pulmonary Hypertension a few days before Christmas, and I had my RHT on Christmas Eve. Needles to say I feel my emotional roller coaster go up and down a lot during the holidays. The holidays can also be physically demanding (even for people without a chronic or life-threatening illness!)


Here are four suggestions to help prepare you for the holidays;

1. Get a Good Night’s Sleep

Many people with PH (and other conditions) can feel worn out and tired more easily than the average bear. The holidays can be an exciting but also a very busy time. It is important to try and reserve your energy wisely.

The first step in keeping a good amount of ‘gas in your tank’ would be to get a good night’s sleep before an event, like New Years or any other holiday gatherings.

Here are a few quick tips to getting better sleep;

-To ensure you have a goodnight’s I would recommend turning off electronics (like your phone) 20 minutes to an hour before you want to sleep. The light omitted from many electronics can trick your brain into thinking it is daytime, thus making it difficult to fall asleep.
- Try to do something relaxing 20 minutes before bed. This will help clear your mind of unnecessary clutter and thoughts. What do you find relaxing? Some people prefer doing a bedtime/relaxing yoga routine before bed (you can find some for free on Youtube.com and Pinterest.) There are also various meditations and soothing noises for sleep that can help you drift off. Here is a list of some of the best-rated apps to help with sleep.
- Scents can be very soothing, which is perfect for helping you feel relaxed enough to get a good night’s sleep. Lavender is one of the mostly largely accepted scents associated with relaxing and a good night’s sleep. If aromatherapy and diffusers aren’t your thing I would highly recommend Badger Balm’s Sleep Balm. It is made out of essential oils, but comes in a wax rub form instead of a liquid. It isn’t messy, it is very easy to use, and it only involves one step. I rub a little bit on my temples when I hop into bed. I absolutely love the smell of lavender and bergamot in the Sleep Balm. (I am getting sleepy just thinking of it.)
- Read a few pages or a small chapter of a good book before bed.
- Don’t be afraid to nap for 20-40 minutes the day of an important outing!

2. Take Care of Yourself

Preparing for Christmas Eve by slothing around

Remember to take care of yourself. If you are not feeling up to something, ask if you can reschedule. Chances are everyone would feel happier knowing that you felt well enough to enjoy something more than you would have if you were tired, in pain, not feeling well etc. I know that many of us struggle with the associated risk of ‘cancelling’ plans but sometimes things are beyond our control. This is why it is so important to put yourself first. Good friends and family will understand. They would want you to tell them how you are really feel and what would be best for your situation.

Don’t hesitate to ask for help, or to let others know how to make a situation easier for you. Don’t be afraid to need to relax before, during or after an event. Don’t be afraid to take a day to recoup after a gathering. Take a sloth day. Stay in comfy and cozy clothes, cuddle up with a cup of tea and your favorite human, or animal (real or stuffed.) Have a bath, marathon a TV show, read a good book, draw, surf the web, meditate. Just relax if you need to (and don’t feel guilty about it!) PH or not, everyone is entitled to a good sloth day after dealing with all the business that the holidays can bring.

3. Eat Right

One year in high school my friend Aileen gave up sweets for lent. I vividly remember saying “what good is life without dessert?” A little dramatic, I know. I certainly eat better than I did in high school
(I am on a gluten-free diet, and try to eat mostly healthy goodies,) but I maintain the idea for myself that everyone needs a good treat everyone once in a while. My only advice is to pick and choose your dessert wisely. Make sure to indulge in something that you will really enjoy. At the end of the day, I don’t think I will regret any of the cupcakes that I have eaten. I thought about it more, I won't.

My favorite special treat are the cupcakes from Kelly’s Bake Shoppe in Burlington, Ontario. My poor boyfriend has to drive me all the way to Kelly’s on the regular. The cupcakes at Kelly’s (which I swear by) are not only gluten-free, but also vegan and made in a nut free facility. Kelly’s also uses whole ingredients along with organic and fair-trade ingredients. Even better than that, their cupcakes (along with donuts, cookies, muffins...) taste amazing. My boyfriend is a little on the picky side when it comes to my venture into healthier food, and he loves them! They are unlike any vegan/gluten-free cupcake you have probably ever tried. They are not good for ‘gluten free’ or ‘vegan’ cupcakes (which can be dry, or have a funny texture.)  They are just good. Okay- I rambled too long on this one. I really like Kelly’s. I think I should go before New Years to get some cupcakes for my celebrations.

Enjoying a cupcake at Kelly's

*Fun tip: their cupcakes freeze very well. Freeze some and keep them on stand by for a rainy day. I always treat myself to one after a PH appointment.

A very serious issue in terms of PH and dietary needs is maintaining a low sodium diet. People with PH are advised to have a low sodium diet because salt can cause water retention. This is a problem for people with PH because the volume of blood your heart has to pump increases when too much water is stored in your tissue. Hearts with PH are generally working overtime, which is why a low sodium diet is so important.

If you are going over for dinner at someone's house, make sure to let them know about your low sodium diet. I know that this is a pain the butt, so I will often ask if I should bring a meal for myself, or come over for dessert. You can always eat a meal before going over and have some salad with low/no sodium dressing while at your family or friend’s house. This way you’re not missing out on any bonding over dinner. Don't be afraid to bring your own condiments or whatever you need to ensure you have a meal that is safe for you.

If you are going out to a restaurant for dinner, be sure to let your server know that you cannot have salt on your meal. When I go out to eat I explain that I cannot have salt due to a very serious heart issue. I have to say this because I look healthy, and because of this, servers and chefs have not always taken my request seriously. This has resulted in a few episodes where it feels as if my heart is trying to self destruct. Not fun. Be sure to ask what the chef would recommend for your request. Some places will be happy to make something off menu for you. Your best bet is to get a protein that isn’t seasoned a head of time, along with veggies. Ask for pre-made sauces with salt to be served on the side. This way you will have better portion control for how much salt you do have.

Salt is hidden in tons of things from butter to broth. If you are making your own meal, look for low to no sodium products. Stay clear of recipes that ask you to use a lot pre-packaged items. An example of this would be a curry recipe that calls for you to use a pre-made jar of curry sauce for butter chicken. Look for a recipe that will actually ask you and instruct you on how to make the curry sauce. You can alter any recipe as needed. Omit the salt completely and add low to no sodium products that the recipe calls for.

If you find that your meal is a little bland Mrs. Dash offers salt free seasonings and marinades. (Southwest is my favorite seasoning. I put it on corn or potatoes with a bit of organic butter.) Another company called Mr. Spice creates organic, no sodium marinade and sauce. Their sauces are so flavorful you probably wouldn’t guess that they don't have sodium! I was very happy to find their products; they have been a total life saver for me. (Some of my favorites from Mr. Spice are the Honey BQQ sauce, and the Hot Wing sauce.)

4. Have Fun

Sammy posing in his Christmas
sweater.

This is one of the most important steps- have fun this holiday season! As I have mentioned before, I know how hard the holidays can be when you have something as serious as PH. It affects everyone so differently. We all have different abilities, and perhaps issues. This is my third Christmas since being diagnosed, and I am looking forward to (hopefully) having my best Christmas and New Years since diagnosis. I know that I am very fortunate to improve the amount that I have, and to have the energy level that I have. Things aren’t perfect, and things can be really hard- but I want to take advantage of good things when and while I can.

I have learned through this experience how important it is to utilize my good days. I’m going to say yes to opportunities that I feel well enough for, and want to do. I will also say no thanks to anything that sounds like it might be too physically or emotionally demanding (or that I just don’t want to.) Remember, it is okay to take care of yourself. Sometimes that means saying no to some invites. Your true friends will understand if you explain the situation to them.

If the moment approaches and you are feeling up for it, celebrate! Have fun, laugh, take pictures and make good memories. If you can’t stay up till mid-night on New Years have your own count down. Celebrate in your comfy clothes, or dress up. Stay in or go out. Do whatever feels best for you, and will bring you the most joy. Kiss your dog, partner, teddy bear or even a consenting stranger at a party for the countdown.

Wishing you all a very safe and happy 2016.

Serena xo

See some of my holiday fun on Instagram

Cross Over Post: "I Want Kids, But My Progressive, Fatal Disease Means I Can Never Get Pregnant"

The website xojane asked me to do a re-write of an article that I previously wrote here (previous article can be found here 'Why I Shouldn't Get Pregnant.') xojane posts a lot of real stories from other young women facing a variety of different issues. Some are fairly light hearted, others are more serious. If you haven't been there before, check out their website. I have even found a few other articles by other young women with Pulmonary Hypertension.

If you are interested in reading the re-write of the article I wrote for xojane please follow the link: http://www.xojane.com/healthy/pulmonary-hypertension-kids

I would like to add that xojane changed my title to be a real bummer. They also took the liberty of adding some pretty lame tags to my entry like 'death.'

Here is a preview of the article:

"December 21, 2013 is the day that forever changed the course of my life.On this day, I was told that I had Pulmonary Hypertension. I had never heard of this disease before, and chances are you haven’t heard of it either because it is fairly rare.

The news I received was bad, very bad. In addition to being rare, PH is also progressive, incurable and fatal. The doctor came into the room and announced that I have 5, maybe 10, years to live. I was only 25.

I looked out the window of the doctor’s office and saw many of my plans for the future floating away. I was immediately advised against pregnancy because of the high mortality rate associated with it for women with PH. I hadn’t even had time to process all my other losses.

In simple medical terms, Pulmonary Hypertension is considered a contraindication to pregnancy. Pregnancy for women with PH increases the risk of death for both the mother and child."

Please read the rest here

PHighter Friday: Adam Kingz

Adam with Mary McCarthy,
Clinical Nurse Specialist

Hello, my name is Adam Kingz. Here's a little back story on my life so far. I was diagnosed with Primary Pulmonary Hypertension when I was about 13 years old. The doctors said they think I had it for about 2 years before finding out. The testing was long and doctors had no idea what it could have been. One of the main reasons I started going to the doctors was because I was passing out in gym class all the time.

The day I was diagnosed I was sent to sick kids hospital in Toronto. I pretty much lived in the hospital after that. It was hard being 12 and having PH. As my friends played I sat. They continued in school and even that was to much for me. I was in a wheel chair and on oxygen. The oxygen was a pain to have with me, plus being stuck to the wall sucked. Then they started me on flowlan. I was the first child in Ontario on it. So it was a little nerve raking. The fact that it had to stay cold and be changed daily was quite taxing on my family. I also developed an allergy to all tapes so they could only use gauze on me.

My health continued to decline and I was able to do less and less. I got the call for my transplant August 23. I was now 13. It was 3:00 in the morning and I was whisked away to sick kids. (One of the few times I was home. Of course). After my surgery things became amazing! I could breath, run, play! It was truly amazing! My life was no longer on pause. It aloud me to grow up! I got to meet my beautiful wife and become a father to her daughter. I was only taking anti rejection medication and that wasn't to bad. Besides the fat face from prednisone.

After 13 amazing years I developed a cough. After a few tests the doctors found out that I had chronic rejection. This was heart breaking and terrifying! I was so happy and it was all going to disappear right through my fingers. But then the doctor gave me a glimmer of hope. I was told they do re-transplants. It was scary but it was something. I could keep my family, my life wasn't over. Shortly after my now wife and I decided to try and have a child. I was already a father to a little girl that may have not been mine from blood but she was and is a huge light in my life. So why stop living? We were blessed with a son!!! All though out my wife's pregnancy I got worse. Everyday it was getting harder and harder to breath. When I saw him I forgot about it all. He was perfect. And just one more reason I had to survive!

My wife and I also got married in this time. To see how beautiful she was walking down the isle was incredible! She truly is my other half. My health was getting incredibly bad. Well you wait for your transplant you have to do phsyo. It's to keep you as strong as possible. That way you recover fast after transplant. It's just some light weights, stretching and walking on a treadmill. Well by the end I was barely doing 5 minutes on the treadmill. I could barley walk up 2 or 3 steps. I used a walker and refused a wheelchair. I never wanted to go back in one again. My breathing was so bad I was burning so many calories I was down to 105lbs. That's coming from about 210lbs. Every breath I took reminded me of having PH again. It hurt so bad. Every minute of every day it hurt. After 1 false call the day finally came. I got my lungs! 12 days in the hospital and I got to go home. I pushed hard and recovered fast! Now as I sit here healthy and being able to breath once again I will never forget how lucky I am. I love my life! I am extremely thankful to both my donors. Organ donation is incredible! I get to be a dad, a husband, son and friend.

The 'S' Word

What comes to mind when you think of the 'S' word? Is it a four letter word, or is it an eight letter word? The word I am referring to isn't a bad word (although it felt like one for a while.) It felt like word I wasn't allowed to use. I thought this word was off limits for me because I haven't "beaten" Pulmonary Hypertension, or because I haven't lived with the disease for over seven years.

The word I am talking about is survivor.

People who have lived with Pulmonary Hypertension for over seven years are considered a 'long-term survivor.' For those of you not living with PH, I can't even begin to explain how daunting it is to know that if I make it to my 33rd birthday I will be considered a 'long-term survivor.' For those of you who have PH, you already know how this pit in your stomach feels. I have only ever seen the word 'survivor' attached to the phrase 'long-term' when referring to PH. As I have mentioned before, there are not any treatments available that provide a chance of remission or a cure for PH.

When I was diagnosed the specialists came into the room and slapped an expiration date on me. He said I have " about 5 to 10 years." I asked "5 to 10 years for what?" Dying clearly wasn't on my mind. Sure, I Googled the disease before I went to my appointment, but me? Fatal disease? Part of me thought that couldn't possibly happen to me. But I guess it did happen to me, and it is happening to me. Most of us do not get the chance to beat the disease, and are therefore not often considered a 'survivor' unless we beat that crummy expiration date that doctors try to pin on us.

My question is why not? Why are we  not considered survivors the day we are diagnosed?

How do I go from a doctor's appointment where they discuss putting me on the transplant list as 'insurance,' and try to maintain a relatively normal social life? What do I have in common with my peers? Suddenly we are forced to find the fine line between living and dying, and balance it in a modern world. Pay your bills. Take your pills. Don't have an emotional breakdown in public. (Although, it is totally okay to cry in public. I have seen my fair share of people cry at night clubs before I got sick, and it was mostly over guys and french fries.)

How do you love something when you feel like it betrayed you? For me, it became incredibly hard to love myself after diagnosis. After diagnosis we have to try and learn to live with ourselves (and our diagnosis) the best we can. Suddenly our bodies are different and we rely on medication. The most effective medications have horrible side effects, are quite visible, and require that the medication is administrated 24 hours a day, everyday. You also never get a break from the equipment, or a vacation from being sick. Some of us also require supplementary oxygen, which comes with its own separate handful of problems. (Like "do I have enough oxygen to go shopping with my friends? What happens if the power goes out?") Suddenly our bodies are different, along with our lives and what we thought would be our future. I remember feeling as if I could physically feel my body betraying me when I first diagnosed. Worse than that, I felt like everyone could see my body crumbling away. It was hard to love myself, or believe that anyone else could love me. I felt invaluable because my body, for whatever reason, decided to self destruct. After diagnosis, I feel like many of us question our self worth and face self-esteem issues.

We have to try learn to accept, and hopefully love our bodies, despite all the things we might require to stay alive. I learned that I could not love others if I did not love myself. (I know how cliche that sounds, but there is a reason so many people offer this advice.) While I still struggle with trying to accept all the changes in my life, I am also less hard on myself. Superficial things don't hold much merit anymore. Sometimes you just have to accept things for the way they are. That way you can move on and really enjoy what is happening in front of you in this very moment. My boyfriend told me a few months ago that I wasn't affectionate anymore. Truth is, I hadn't been affectionate since after my diagnosis. I was scared he could feel my body betraying me when we hugged. Could he see the expiration date stamped on my body? I also had a hard time really connecting to him. I was tuned out a lot. I was in my own little world full of fear and depression. I still have those days, but I try to focus on living in the moment. I was so closed off, my boyfriend wasn't sure I loved him anymore. I realized that I had shut him out because I was so scared of him leaving me because of my diagnosis. I felt so unlovable, and it was easier to shut him out so I couldn't get hurt in case he decided he couldn't be with me anymore. I learned that it wasn't my diagnosis that was pushing him away, it was me.

Not only have I battled physical obstacles since diagnosis, but emotional. I know that I am not the same person anymore, and I doubt anyone who goes through this experience could be. In fact, I think the core people in my life have changed as a result of my diagnosis as well. They say death changes you. Well, what do you think happens when you get an invitation to the Grim Reaper's party instead of the standard surprise party? Obviously you have time to think about a party that you weren't really planning to attend yet (or even prepared to go to.) This is what I mean about the fine line between living and dying. We are constantly trying to balance both, and still be functional. "Did I leave the stove on? I need to pick up dog food. Should I make a living will?" The last thought shouldn't be something I have to worry about, but it is.

When I was first diagnosed I noticed how easy it was to stay in bed, not change out of my pajamas, and make excuses not to do things with the people that I love. It was easier to stay in bed, be alone and wallow with my thoughts. Once I started to stabilize I was still avoiding doing things that I would genuinely
enjoy. I had this idea in my head that I had to wait for things better. By better, I didn't just mean my health stabilizing. I meant I was waiting for some kind of miracle to make the PH go away. I felt like I couldn't enjoy life unless I was perfectly healthy. I have started to accept my health for what it is (although it is challenging.) When I was diagnosed I was very sick. I was diagnosed somewhere between a stage 3 and stage 4. There is no stage 5. As you can imagine I was in rough shape. I went to bed each night terrified I would die in my sleep because of how sick I was. My heart was severely enlarged. Even with oxygen walking a foot to the bathroom was a major challenge. Because I was so sick, I am starting to really appreciate how far I have come. It isn't perfect, and of course I wish I could be better, but where I am now is so much better than where I was when I was first diagnosed and I am fortunate it for that. I've slowly started to realize that I am doing okay, and that there is no better time than now to start living. I am not sure what my health will be like in the future, so I can't waste anymore time waiting for things to get better before I start living again.

This might sound a little strange, but I don't identify with the colour periwinkle or zebras (things affiliated with PH awareness.) I consider myself more of a unicorn, and I like the colour seafoam. I also don't identify with the word 'patient.' I have had to correct people from referring to me as one because my name is Serena and I am so much more than my diagnosis. (Unless I am in the hospital, I am not a patient.) It took me a while to come to terms with the word 'survivor,' and perhaps you don't feel comfortable referring to yourself as one. Through this experience I have come to realize that we are constantly learning in life, regardless of conditions. After my diagnosis I had to do a lot of re-learning. I am re-learning to live again. I am learning how to live a different life. The lessons that I have learned are things that can not be taught, but only gained through experiences (which are sometimes very painful.) What I have learned is that if you can continue to live, learn and love despite your diagnosis, which could very easily make a person hard, you are a survivor. Even if the word survivor is not a word that you are comfortable with, please do not deny yourself the love and recognition that you deserve. If you continue to live, love and even laugh despite everything; you are at the very least a bad ass.

PHighter Friday: Carmel

Just an average pre-dawn trip to the ER

When it comes to Pulmonary Hypertension, I am the boogie man.  I am literally living the life that everyone is afraid of.  Think PH diagnosis prior to the new medications that came out in the 1990’s, the medications “that work,” as they are so often called.  To put it bluntly, when all of us were told at diagnosis not to google PH because of all of the horror stories you would find on the internet, they were essentially telling you to avoid people like me. 

However, I believe that my story is worth telling.  I have developed a way of living with my disease (and that’s with my disease, not in spite of my disease) that allows me to have a completely satisfying, present-moment oriented life without regret, remorse, or wishing things were different.

I was surprised diagnosed with PH January 8^th 2014.  After about six months of increasing difficulty breathing, I finally went in to see a pulmonologist when I could no longer walk the six blocks to my regular bus stop without having to stop multiple times to steady my breathing.  Because my symptoms were so severe at my first appointment, I was quickly scheduled for a series of escalating tests culminating in a lung biopsy.  The lung biopsy confirmed the PH, as well as a secondary lung disease thought to be completely independent from my PH.  I woke up from the surgery on oxygen and have been on oxygen 24/7 ever since then.  My first right heart cath confirmed what my echo had estimated, that my pressures were in the 100’s and that my heart was significantly enlarged from all of the extra effort it has to exert.  I was promptly started on Sildenafil and daily diuretics, and when that didn’t work they increased my diuretics and added Letaris, and when that didn’t work they increased my diuretics and added inhaled Tyvaso, and when that didn’t work they increased my diuretics and switched me from Tyvaso to Sub Q Remodulin, and when that landed me in a three week ICU stay in which I was given a 0% chance of surviving, they increased my diuretics and switched me from Sub Q Remodulin to IV Veletri (epoprostinil).  Since being switched to IV Veletri this past January, I have survived one serious line infection, a blood clot, multiple septic emboli in my lungs, and a six hour hemorrhaging episode after my new line was placed.  I have spent a total of 35 days in the ICU, my walking radius has decreased to within the confines of my studio apartment, and I am currently on 10-15 liters per minute of supplemental oxygen.

I am also having the highest quality of life that I have ever experienced.

  

Many years before my diagnosis I began learning about and practicing Buddhist mindfulness.  Since I had been raised Jewish this was not much of a stretch.  There are countless numbers of us “Jew-Bu’s” walking around!  In a journal entry from 2009 I wrote to myself “When I look in the mirror I can either believe ‘my hair is brown,’ or I can believe ‘I’m having a thought that my hair is brown’.”  Both thoughts are true, but how will thinking one versus the other impact me in my daily life?  Let’s say that my mind automatically goes to the thought “my hair is brown.” What if I don’t want to have brown hair?  What if I prefer red?  In fact, my grandmother had red hair.  Why should I have been born with brown hair?  It’s not fair!  I should be able to change my hair color, maybe I should get some dye.  Of course, my hair being so dark I would have to bleach it first.  Again, not fair!  Other people don’t have to do that so why should I?

As you can see, suddenly from the thought “I have brown hair,” I have created a story about the value of having brown hair, and then I began emotionally reacting to my story .  The negative feelings I experienced were not actually caused by having brown hair, but because of the meaning I had invented and then assigned to it.  Suddenly, I am lost in suffering.  However, when instead I have the thought “I’m having a thought that I have brown hair,” I can instead notice the natural human instinct to want to categorize and label, and I can lovingly allow those thoughts to disperse naturally, like leaves being carried away in a river current.  In fact, because I am not caught in a self-perpetuated story that things should be different, I am free to enjoy what actually is. For example, the naturally occurring red highlights that do appear in my hair when the sunlight hits it just right.

My wife and I at our wedding reception

To me being terminally ill is like this.  I get to have a very different quality of life when I separate out the story which I attach to a thought from the actual thought itself.  It’s not fair, this shouldn’t be happening to me, and nobody understands are all examples of thought patterns that create their own suffering in my life.  If instead I’m able to think “I’ m having a thought that this isn’t fair,” I am more likely to be able to approach myself with loving kindness.  I can use that thought as a reminder to take extra good care of myself in that moment through reaching out to a friend, taking a bubble bath, or putting on my favorite fleece pajama pants.  And because I am staying fully in the present moment and not spiraling out in self created story, I am fully present to notice, receive, and be soothed by the self-nurturing that I am providing to myself. 

I have long subscribed to the alterna-healing world, and I have continued to use many complimentary forms of healing alongside my traditional allopathic PH care.  Among my regular tool kit I have used the anti-inflammation diet, a brief foray into juicing medical grade marijuana (legal in my state), hand making medicinal bone broths and eating traditional organ meats, learning to both give and receive Reiki, five element acupuncture, chiropractic care, yoga nidra, prayer, meditation, guided imagery, and specialized breathing exercises.   I believe in the alterna-care world very much, but here is another place where I have to be very careful not to create story (and therefore suffering).  I receive acupuncture, for example, because I like the relationship with my body that it helps to foster and I have also noticed that many times it can alter my experience of my symptoms.  However, if I start to become attached to the thought that acupuncture should cure me, I block myself off from the healing that is available to me, and is happening on a daily basis.

I mentioned earlier that I am currently having the highest quality of life I have ever experienced.  And I mean it, literally.  This last January, as I lay in my ICU bed in heart failure and unable to move, talk coherently, or care for any of my basic needs independently, the people that I had known and loved throughout my life dropped everything to take up residency in my hospital room.  Friends and family, and sometimes even near strangers, literally put their lives on hold to make sure that I was never alone.    My best-friend-since-the-sixth-grade even private chartered a plane with her husband to come out to visit me.  For three weeks, people sang me songs, massaged my aching body, slept in the hospital bed with me, and told me over and over again what it had meant to them to have me in their lives.  We said our goodbye’s, but with so little time left and the perception of nothing to lose, we metaphorically stripped down bare and truly got to show our souls to each other.  The joy of life was palpable to all during this time, and pure unencumbered love was the only language that anyone spoke.  Suddenly, it became clear to everyone present what it truly means to be alive (hint: it has nothing to do with if you can work, how much money you have, or what your body looks like).  My sisters decorated the ICU walls with colorful and beautiful artwork for me to look at.  We kept a mini fridge in my room filled with delectable taste sensations for when I could eat.  A good friend rented a button making machine and hand-made buttons for visitors and medical staff alike celebrating my life.  I hosted and judged a dance competition between my sisters, a close friend, and medical staff.  Although I was dying I was also, for the first time in my life, fully alive.  Far from feeling burdened, the close friends and family who walked this path with me were also woken up to the beauty and splendor of life.  Many of them feel permanently altered for the better from that experience.

Wedding day!

Truth be told, I started dating the woman who would become my wife during that ICU stay, proving to myself once and for all that my self created story (no one will ever love me, I am a burden on others, who will want me now), is nothing more than self created story which only serves to create needless suffering.  The night we confessed our love to each other we both thought was going to be my last night on earth.   Turns out we were wrong.  In what could only be described as a Disney princess fairy-tale story come to real life, I was literally saved by true love’s kiss.  Which is to say, the very morning after we confessed our love, with no medical explanation at all, I suddenly reversed course, came out of heart failure and could sit up and walk again.  My wife and I are madly in love with each other, and I have been sick with PH the entire time that she has known me.  Having lost her own father to lung disease nine years ago, she knew fully well what she was signing up for.  She does not feel that she is missing out on anything because I am sick, and I know with absolute confidence that no one else on this earth could do a better job of loving her than I do.  Our joy at being together fills every room we enter.  In the hospital the nursing staff has nicknamed us “the love birds” because every time that I am hospitalized, and I am hospitalized quite a bit, she spends each night sleeping in the narrow hospital bed cuddled alongside me.  She loves me enough to tell me that if it ever becomes too hard to keep on going, she will understand if I need to leave my physical body.  And she loves me enough to support me through the grotesquely rigorous and often unpleasant lung transplant process I am currently engaged in.

Two books have helped me considerably on my journey towards peace and freedom with my illness, and I would recommend them without reservation to anyone who is either sick or knows someone who is sick.  The first is called How To Be Sick: A Buddhist-inspired guide for the chronically ill and their caregivers, written by Toni Bernhard.  And the second book is simply titled Fear, written by Zen Buddhist monk Thich Nhat Hanh.  Both books have helped me shift my thoughts so that I when things are hard, as they often are, I experience the necessary and expected pain of being a human being who is mortal, but not the suffering caused by own self created story.

I hope that my orientation towards my disease can be helpful to all of us in the PH family, whether you have six months or twenty years to live.  Whether the new drugs “work” for you, or they do not.  Whether you are working a full time job, or just working on being able to get out of bed in the morning.  Regardless of your circumstance, there is an equal amount of peace and freedom to be found for each of us.

Carmel is currently raising money for her lung transplant. Please visit her fundraising page here to learn more on how to help, donate or share her fundraising page.

PHighter Friday: Mariza

Hello there guys!  My name is Mariza and I am 34 years old. I was diagnosed with CTEPH (Chronic Thromboembolic Pulmonary Hypertension) about a year and a half ago. I cannot quite pinpoint when my symptoms really started because I had trouble with breathing since high school. I was told I had allergy induced asthma and had an inhaler for when the asthma acted up. I had bronchitis more times than I can remember but my symptoms seemed to worsen about 2.5 years ago. I remember I was sick with a cold and coughing a lot and noticed I was having a lot of trouble breathing when my husband and I went to a park and just walking was tough.  I went to the doctors and was told it was my asthma and I most likely had a sinus infection that had cleared up on its own. So I continued taking my inhaler and my symptoms seemed to get better. I started running and working out, lost about 40 pounds and felt great.  Fast forward to Christmas 2 years ago. I was in North Carolina in the mountains with my family and husband and we went for a hike and I was having a lot of trouble with the smallest hills. I chalked it up to the altitude and my asthma acting up again.  But my symptoms didn’t seem to get better this time.   I found myself trying to find the closest parking spots, knowing that just the walk from my car to the store would be difficult. I let it go because I really believed I would feel better eventually. I had a bad cough from January until May of 2014. You would think I would have gone to the doctor but was seriously convinced I had a really bad asthma flare up.

I am a nanny and my job requires a lot of energy all day every day.  I would be at work and it would be really hard to do anything.  I would walk up the stairs and need to sit down immediately so I wouldn’t pass out, I would basically spend the day sitting and making excuses to not chase the kids around. I honestly don’t even know how I got through it and I don’t know how I managed to not pass out. When I got home I would sit down immediately and remain in that position for the rest of the night. Needless to say, I gained that weight back pretty quickly. It all came to a head when a couple of other nannies and I decided to take the kids to the Philadelphia zoo for the day. We parked at the zoo lot which is about a 5 minute walk to the entrance of the zoo; no hills. It was absolutely horrible. I had to stop a couple of times on the way in to catch my breath and it hurt so bad to even take a breath. I was gasping for air with every step I took the entire day. It was so bad I told the other nannies that I thought something was wrong. One of them said you might   have walking pneumonia and she told me her father had just gotten over that and he had the same symptoms. I told my husband and he said I had to go to the doctor. So I made a decision the next day to go to the urgent care after work. 

When I got there they took my blood pressure, weight and placed the pulse ox on my finger. The tech who was doing all of this kind of gasped and asked me if I was wearing thick nail polish and I told him
was just wearing regular nail polish.  So he said let me do this again. And I looked at the monitor and my oxygen level read at 78%  I asked him if that was bad. He said well you should be at 98-100% and he walked me over to the room and called the doctor to come in immediately as they put oxygen to my face. They also took an xray of my chest. I sat there for a while until my oxygen went up to 90% and said I need to go to the ER right away and they would call an ambulance. I told them no that I would have my husband come get me. I was terrified. Once I got to the ER I was pumped full of oxygen but it was so unstable that the doctors were seriously concerned. They thought for sure I had a pulmonary embolism and did all sorts of imaging and blood work which confirmed there were clots in my pulmonary arteries and I had pneumonia.  So I was in the ICU for several days and then moved a step down to progressive care after a couple days but I was still attached to oxygen. I was sent home after about 10 days in the hospital but was still on oxygen and if I wasn’t moving and not on oxygen my O2 levels would pretty much be at 92%. My mom came up from Mississippi to help and be with me so my husband could return to work. On my ninth night home I was laying down and my dog was laying next to me very concerned and wouldn’t leave my side. I was still coughing but this time I coughed up a lot of blood and then I took my o2 levels and they were in the low 80’s while on oxygen. I told my mom and husband and I said I think I might be getting another PE. So they said lets go to the ER and as we were walking to the car I was so close to passing out they had to catch me. We go to the ER and had the same tests done and back to the ICU I went. After a few days in progressive care and no improvement the doctor came to tell me that I was being transferred to Lehigh Valley Hospital where I would most likely be getting a lung biopsy done because they couldn’t figure out what was wrong with me and they didn’t have the resources to do any further testing at my local hospital. It was at Lehigh where the wonderful ICU docs figured out what was wrong with me. They told me I had Chronic Thrombo Embolic Pulmonary Hypertension and that the clots that are in my lungs had been there for so long that they had calcified and my heart had been working so hard to get oxygen around that my right heart had enlarged tremendously.  They told me I was really lucky because there was a surgery that could cure this and I might be a candidate but that I would have to go to UCSD or  Temple University Hospital for it which is in Philadelphia. So the obvious choice was for me to go to Temple. Because I was so unstable I was sent there via ambulance and kept in their progressive care there for about a week where they put me through a variety of tests.  I had an amazing doctor, Dr. Paul Forfia, come and talk to me and he told me I was a great candidate for the surgery and I would have it the next day.  When it came time to sign all the necessary papers, the resident who came to tell me about the surgery came right out and said exactly what the procedure entailed and the risk factor.  I had no idea the surgery was an open heart surgery and all the craziness that went along with Pulmonary Thrombo Endarderectomy.  I freaked out and could not stop hyperventilating. I said I needed a minute to think about all of this. Dr. Forfia came in and agreed that I needed time to process everything and it would be good for me to go home and gain strength before the surgery. So that’s what I did and I am so glad I did.  I left the hospital at the end of June and scheduled my surgery for September 22^nd, 2014. I had three months to research my amazing surgeon Dr. Yoshiya Toyoda, spend time with my family and friends and mentally and physically prepare myself for the biggest day of my life. I had that surgery and despite one set back when fluid filled my lungs after leaving the ICU, I got through it. They removed the clots and cured me of my pulmonary hypertension. I was in the hospital for 9 days after surgery and left there with no oxygen and walking and able to breathe.  

My heart is back to normal size and really healthy. I have a new outlook in life and consider this as my second life. I just recently had my first birthday and I feel amazing. The asthma I had been told I had, never really existed and I can take a deep breath like never before. I exercise at least 5 times a week, like really really workout. I lost those 40 pounds (20 of it after the surgery from not eating and being bed ridden) but the other 20 just from exercising and eating right. I am still working on being at my healthiest. I owe my life to the doctors at Lehigh Valley Hospital and my amazing doctors Dr. Paul Forfia and his NP Fran Rogers and my surgeon Dr. Yoshiya Toyoda at Temple. Not only do they dedicate their life to caring for patients with pulmonary hypertension but they cure those who are able to get this incredible surgery. 

This past Saturday I had a major milestone in my post-surgery life. My husband and I did the O2 Breathe walk to find a cure for Pulmonary Hypertension…at the Philadelphia Zoo. I cried when I spent the entire day there walking and able to enjoy the experience, I cried because of the guilt I felt since I am cured, I cried for the people that have lost their lives to this disease and I cried tears of joy because I know that this community will one day find a cure as long as we keep coming together, spreading the word and sharing our stories so people can become more familiar with this “invisible disease”. 

PHighter Friday: Rebecca

My health journey started a long time ago. 25 years ago, to be exact. I was three years old, and diagnosed with Stage 4 Embryonal Rhabdomyosarcoma. To summarize: Tumor in my abdomen. 5% chance of survival. The treatments I endured have long ago been deemed "inhumane, and no longer practiced on humans." I received experimental amounts of radiation, and chemotherapy. The doctors told my parents to expect long term side effects of said treatments, but were unsure what they might be.

Some side effects were: intestinal issues, weak teeth, extreme fatigue, infertility, and joint problems. I've lived my life in and out of hospitals, and doctors offices. I've had to become very in-tune with how my body is functioning.

Fast forward to April, 2015. I developed a case of bronchitis that just wouldn't go away, no matter the amount of antibiotics I took, or rest I got. I noticed that my heart was beating hard all of the time, and I was breathless walking from my living room to the bathroom. I couldn't speak a full sentence without gasping for breath. After months of different doctors, and tests, including a right heart cath, a new diagnosis entered my life. June 5th, 2015: Idiopathic Pulmonary Hypertension. My doctors can only assume it's from the radiation I got as a child. At the time of diagnosis, I was told I was at Stage 3. I was put on Sildenafil, Opsumit, and blood thinners within two days of my diagnosis. I had to quit my job-I had been a nanny for 6 years-because I couldn't even walk up a flight of stairs, let alone chase after kids.

I felt my entire world crumble beneath me. The first few days after the diagnosis, however, I felt relief. I was almost blissful. After months of doctors telling me I was fine, there was nothing wrong, it was in my head-one even told me that I should see a psychologist-it started to mess with my mind. "Was I fine?" I thought? "Is this just how people feel all the time, and I'm being whiny?" So to receive a diagnosis: I was happy. I threw myself into learning about this disease, it's outcome, the medications, the stories. But eventually google stops giving you answers you didn't already know, and the dust settles. In the weeks since I've been diagnosed, I've experienced sadness, anger, guilt, frustration, and fear. Sometimes all on a daily basis.

These days, I try my best to not panic or worry about the future. I spend my days with my cat, and baking. I've had to learn to allow myself to not rush projects, or cupcakes. But to take my time, rest often, rely on the help that people want to give. I'm only a few months out of the diagnosis phase, so I wholeheartedly believe that my health can only go up from here, now that I have an amazing doctor, and medications. I use oxygen only at night now, instead of 24/7, like at first. 

I joke that I've been given a "lemon" of a body. I feel as though the insides of me are barely held together somedays. And there are days where I think, it's impossible. It's impossible for one person to fight so hard, to endure so much, to receive blow after blow. But usually after a good nap, and an even better cry, and a hug from the world's most amazing husband, I brush myself off, and tell myself that tomorrow is a new day.

Pulmonary Hypertension may have been a stumbling block for me this year, but it won't slow me down forever.

I can be followed on Instagram at rlidenberg, or on my blog: phancyfree.wordpress.com