PHighter Friday: Keti

Adventures with a Loser Disease

Today is a most excellent day ! And as time goes by, the good days have been out numbering the bad days.

It was late 2006 when midday at my workplace someone said to me that my ankles were very swollen, and I was shocked at the size of my feet as a whole.  Of course all kinds of advice were thrown at me as to what to avoid eating, but I knew that it was something more than water retention…. I started paying close attention as to when the swelling would start in my day to day, and noticed that all remedies for water were useless. In fact prescribed water pills caused ER visits due to dehydration and potassium issues… Diagnosed with Congestive heart failure… while dehydrated?? Hardly ! 

I had two separate  A-fib episodes that year at 3 a.m. , and was assigned to a cardiologist who in a course of 2 years fed me all the tests and stories of a heart condition that I didn’t have but “ you need to take heart meds just in case”.  I did not follow his advice, I was a very healthy and gym fit woman now terrified as to what could be wrong.  After 2 years of being misled I went to a different  and younger cardiologist who found that my right ventricle was stressed at  76 , and sent me to a PAH specialist who did the Cath and determined what was ailing me for more than 5 years actually, he said.

He also sent me to the sleep center where they found that I stopped breathing 82 times in one hour. I was given CPAP which gave me energy and filled me with air all night long.

We all know the course of things, the fears, the loneliness when no matter who loves you and who is around you… you are walking this road alone moment by moment unable to think of anything else. Oh, they all “understand”…  but no one can possibly know unless they face death eye to eye.  It became the demon for me,  to fight and flight better and faster than “IT”.  I started researching on lungs, and their maintenance… and yes I was and still am a smoker… and I didn’t talk about PAH to anyone.

I have been a healthy eater all of my life and yet the stress of this demon filled me with cortisol and I started to balloon. I was told to give up my bicycle and “ just sit in a chair a wither away” more or less. And so I signed up at the gym …
Most days feeling like I will pass out but at least I was doing something I liked. The 14 months that they gave me in life meant nothing to me. I was going to go down fighting, learning my lungs,  being pro active, advising others not to listen to anyone but their gut. My kids gave up on me, blamed my smoking…  couldn’t deal with my “dying”… Bless them for deserting me for I found unimaginable strength on my own, because of it.

14 months came and went, and my every 6 months six minute tests went from 175 meters to 280, in 2 years my number had gone from 76 to 54 and the doctors now wanted to know what I was I doing. Tracleer, the wonder drug, stops the PAH from worsening, but we all know that no drug cures us !!

I told them that I went to the gym, took vitamin B3 for my lungs and blood flow, Mega dose B 12 daily B Complex and was convincing myself that I HAVE NO DISEASE! I stopped making the demon my priority until it became a fact of my life like brushing my teeth. I listened to no one and even today I could care less what advice I am given… Be me, live me, and then come tell me how to fight.

By April 2013 the Cath showed that my PAH was now a 32.  The six minute walk I did was 260 meters. 
By January 2014 the Cath showed a normal number of 25-26 and I walked 400 meters in 6 minutes.
But I knew that even before the tests, I could walk upstairs 2 flights no stopping, just normal tired when I reached the top.

I truly believe that the CPAP, along with forced lung expansions,  deep breathing wherever I was and whatever I was doing, my vitamin intake, my stubbornness in winning and belief in God, won my fight.

I am not out of the woods they say, but that’s because no one knows everything about the disease…which I prefer calling a CONDITION…  a diabetic has more to worry about than I do. I now take only one Tracleer per day so I don’t damage my heart since PAH is barely traced inside me.  Oh, I am supposed to take two per day alright, but I listen to my body more than I listen to  my doctor* …. That’s how I am. On guard are my doctors LOL not meeeeee.   The 14 months became 5 plus years to date, to infinity and my morning pill is just that… a morning pill. 

PAH? Who, Me ?? nope.. and there will never be a round two either! Demon is scared of me now!

*Editors note: The PHight or Flight Project and it's contributors do not endorse or suggest changing prescribed medication in anyway. The PHight or Flight Project always recommends talking to your PH specialists about any questions you have about medications.

Fundraising and the ALS Bucket Challenge

Several months ago I contacted a local Menchie's to see if they would be interested in helping me raise funds for PHA Canada. They generously agreed to the fundraiser, and on August 7th Menchie's helped raise $2000 for PHA Canada. Between all of my fundraising efforts I have raised a couple thousand dollars for PHA (US) and PHA Canada. I am so touched by the friends, family and strangers who have helped me raise money for Pulmonary Hypertension.

By now you probably have heard, and maybe even participated, in the ALS Bucket Challenge. According to the Huffington Post, the ALC Bucket Challenge has raised over $88.5 million dollars in a matter of weeks.This is truly amazing, especially because a large amount of funds have been raised in a short amount of time. I sincerely hope that the funds raised will help find a cure and/or better treatment as soon as possible. If you know about the ALS Bucket Challenge, you have also probably watched the heart wrenching video of a newly diagnosed 20-something year old male who was diagnosed with ALS this year. In the video he breaks down and describes the fear he has about the disease eventually taking over- unfortunately this is a fear that many of us with PH can relate to.

Another point mentioned in the video is that ALS is not a profitable disease. This is also another thing that those with Pulmonary Hypertension can relate to. PH was considered an orphan disease, but is now classed as a rare disease. Idiopathic Pulmonary Hypertension affects about 1-2 out of a million. Needless to say it is not a profitable disease, and therefore does not get nearly as much research funding as other diseases. From a sales point, there aren't enough sick people who will need to purchase anything made to treat or cure the disease. The cost of PH medications are also INSANE. I am so grateful to live in Canada where expensive medication can be eligable for coverage under the province. I have read stories by PHers who live in America and can no longer afford medication and are forced to stop taking it. While these medications cannot cure PH, they can slow down the progression. So how much is PH medication? Well, one of the "life prolonging" pills I am on is 40 mg of Cialis- yes that is a really high dose of boner pills. It costs about $20,000 a year Canadian. I am on a new medication that costs over $50,000 a year. How would anyone could pay for that without assistance? Especially if your disease has made it so you are no longer able to work.

Pulmonary Hypertension is a progressive disease and it eventually leads to heart and/or respiratory failure. There is also no blood test that can be done to diagnosis the disease. As the disease progresses, it becomes more difficult to breath and shortness of breath increases. Simple tasks like getting dressed, going up stairs, showering, walking to the kitchen become more difficult to perform.
Specialists diagnosis PH by process of elimination and then by a right heart catheter. I was kept overnight at a hospital so they could perform all the necessary tests. I can't tell you how hard I prayed that they would find a blood clot in my lungs (something I never thought I would wish for.) I had so many x-rays and CT scans done within 48 hours that the technicians did not want to perform anymore as it could be dangerous. They also performed dozens of blood tests on me, requiring over 30 viles of blood to rule out other diseases. No one told me that they hadn't found a blood clot on my last CT scan. Instead I was rolled (in my wheelchair) to another room where I had to perform a pulmonary function test. That is how I knew they hadn't found a blood clot and I started sobbing uncontrollably. This was inconvenient for the staff and the patients. They rolled me into the hall way facing a wall to cry by myself. All morning they just rolled me from room to room to do more tests. When everything came back negative it was nearly certain that I had Pulmonary Hypertension, the cause? Unknown.

Because of the word "hypertension" some people assume that I have a disease that I brought on by a poor diet, and that I could get better by not eating as many ribs covered in barbecue sauce. Pulmonary Hypertension is very different from regular hypertension, and unfortunately, it is often fatal. The average life span was 3 years. Now the average life span is somewhere between 3-7 years. Scary, right? I turned 26 in February. My parents decorated the house and did everything they could to give me a nice birthday. I tried very hard to enjoy it for them- but it was hard. I couldn't help but wonder how many more candles will I get to burn out?

On online support groups other PHers have been brain-storming ways to raise funds for PH that could be as successful as the ALS Bucket Challenge has been. One idea is that someone who is challenged must wear a clothes pin on their nose, and walk up the stairs while breathing through a straw in their mouth. I was fine with this idea until the stair factor was put in. Not having enough oxygen delivered to your organs is dangerous, which is why Pulmonary Hypertension is often a fatal disease. What other ways can we create a fundraising campaign that will go viral? Another issue is that Pulmonary Hypertension Month is the month of November. It is very difficult to think of something that could compete with "Movember" (people grow mustaches for prostate cancer,) especially before Christmas.

So why is the ALS Bucket Challenge so successful? I think the recognition for people who do donate is a large aspect to it. With social media, people who are challenged are placed under a microscope. Their "followers" will be waiting to see how they respond, and now they essentially have the world watching to see if they make a donation. It is a smart campaign. It would be hard for people in that situation to say no to making a donation and to taking on the challenge.

Some people have refused to donate to ALS because they test on animals. These people include Pamela Anderson who has cosmetic surgery, which was tested on animals and beloved Canadian singer-songwriter Grimes who is a vegan that was under criticism last year for eating Ben and Jerry's Icecream. Ben and Jerry's does not have vegan ice cream, but they do treat their cows ethically.

This is where I have an issue. An argument made by animal rights activists is "who is to say your life is worth more than animals?" I don't want to sound like an asshole by saying this, but I must admit that I think I am. I think most people who are going through a similar situation, or have a loved one going through the same kind of situation can understand that sometimes animal research does need to be done. I don't agree that animals should be tested on for cosmetic products- but if something could be life altering or life saving, I wouldn't be able to argue against. It is an unfortunate situation to be in and take a stance on, but I figured it would only be fair to give my view on it as someone who could benefit from animal testing. I know that animal testing isn't always successful, but who is to say it couldn't leave to a path of success?

Researchers have reversed Pulmonary Hypertension in rats a few times now using various techniques, one of them involves estrogen. I think researchers are so close to finding a cure! Research funds are desperately needed.

If you would like to quietly and graciously donate money to Pulmonary Hypertension Research please donate here. I would suggest donating directly to PH research, because when money is donated to an assoication it is often divided among other areas (paying for staff, awareness materials and so on.)

PHighter Friday: Tia

Myself a few days ago at a place near where I live
called Deception Pass

How Long Have You Had PH? What medications are you on?

I was diagnosed with Severe Pulmonary Hypertension when I was 9 years old, although doctors believe that I have had it since birth. When I was diagnosed at age 9, a lot happened. I was told I wasn’t going to live because my heart was so enlarged. A PICC Line was put in and I was put on the medication Flolan. I was only on it for less than 3 months when my PICC Line fell out in the middle of the night. Doctors made a big ordeal that if I was off the medication for more than 24 hours I would die. It was well over 24 hours without it and I didn’t die. Doctors then realized I didn’t need it. At age 12 I was put on Tracleer (Bosentan) where I did amazing on it! That was almost 15 years ago. I am now on Tracleer (Bosentan), Revatio (Sildenafil), Prednisone, Pulmicort Inhaler, Foradil Inhaler, and Spiriva Inhaler.

What are you able to do that doctors said you couldn’t?

When I was born October 22, 1990 my parents were told I wouldn’t live and if I did I would be mentally challenged due to being born at 28 weeks. Well, guess what? That didn’t happen. I was raised by incredible parents who told me I am no different than anyone else and I can do anything I set my mind to. I was always determined to get good grades in school; I was never in any special education classes because of my “disability”. I did have an I.E.P. (Independent Education Program) that was only because of having my oxygen and if I were to get sick and be away from school they had a plan in action to where I wasn’t to get behind in school. I graduated on time, with my class in 2009. I was Senior Class President and graduated on the honor roll.

Last Hospitalization:

I have not been able to move forward with my education because I had a health set back the summer after I graduated in 2009 where I was in the hospital for a month and a half, due to some not so great decisions on my part. Not due to being immature but being forgetful and caught up in being a High School senior. I forgot to take my medications that are so very important! I was in the hospital where I had a PICC line put in once again but this time I was put on Remodulin. Doctors were talking Lung Transplant, my family and I then until May 2010 went through everything to prepare for transplant. Testing, doctor visits with the surgeon, transplant classes, etc. I then decided when they were prepared to list me I told them no I wasn’t ready. In August 2010 I received a new doctor. I asked to see if I could have the PICC Line taken out. I was tested to see what was more effective, the PICC Line medication or Oral PH treatments and in conclusion I was removed from infused medication in September 2010 and my PICC Line removed that November. I would love to go back when the time is willing, I’d like to become and Echocardiographer. By this time in my life I could probably do them on myself in my sleep! Haha

What does your day consist of with PH?

Some days consist of being with my 1 1/2 year old and 8 year old cousins. Being silly and having fun with them. A lot of the time I like to relax at home, being with family every chance I get and spending all the time I can with my boyfriend Mac when he isn't busy working. I am sort of a homebody. I like movies. whether sitting at home watching them or going and seeing one. i love to ea and having being trying to get more involved and learning to cook more meals. I like going on little adventures here and there. Too much activity and I get worn out easily.

Hardest part of life with PH:

The one thing that has gotten to me though having PH the last few years is the fact I don’t have a good friend support system. To be honest I don’t have any at all. I mean best friend or good girlfriends that I can pick up the phone and cry to, talk to and know they’ll be there for me. On my part it’s hard for me to be that sort of friend but it would be nice if I were to be able have that. It is hard, and has always been hard. There is a difference between having friends and the people you went to school with. I was only invited to a few birthday parties by girls I went to school with here and there. No, one besides a few people actually reached out and tried to be my friend, not just in school but outside of school as well. My mom tried to push me to make more friends but it was hard when not everything girls want to do I could just go and do with them like walking the mall, or going and watching the high school football games, where I would get cold and turn blue just sitting there for less than an hour. I am girl, I like to get pretty, do my hair, and makeup. I like to shop but only when I know I need something because I know the mall is not friendly to me with all that walking. I’m more laid back, I love food and to go out and eat. I love going and seeing movies. More so yes I am a homebody because I can just run all day. I get tired, my oxygen only has a certain time frame of hours it lasts, and I get headaches when I do too much. I sound lame that way but I would give to have friends who understand and tell me its okay.

What is your dating life like with PH/Oxygen?

Everyone on the PH site asks about dating, having kids, etc. Honestly I was like any other girl I had crushes, oh many crushes growing up. I never dated though; I had my one tiny middle school relationship. A slow dance at an after school dance turned into a little fling for a few months. Didn’t involve holding hands or kissing but I did get flowers on Valentine’s Day in 6^th Grade from him. Then you move on. I have only had 2 serious relationships in my life; the first was the summer before my junior year of high school. A guy friend from school added me on MySpace and that’s how my life went in a direction I thought it never would. I got caught up in the whole my first love. I don’t regret that time but I was young, naïve, immature and definitely had low self esteem. He was a nice guy, but it only lasted so long. At that age it’s hard to get someone to comprehend this is what your life will entail being with someone that has PH and oxygen. He didn’t get the magnitude of it and I wouldn’t want someone to at age 18 fresh out of high school. I had a small relationship the summer I got sick after I graduated; he was a nice guy but not the guy for me. I then had the next 4 years. I took those to myself. I learned to love myself and build my self esteem up more than it ever has been. I had a long time to learn what I wanted and didn’t want in a guy and out of a relationship. That was the best thing I could have ever done for myself. In February 2013 I met my boyfriend on an online dating website. We have been together for a year and a half. He is the sweetest, funniest, cutest tattooed guy to come into my life. Not only does he understand my life, he has done things and wants to continue to do things that change his life to have me in it. I will admit he knew a good amount of time before me that I was the one before I knew he was the one. Only in part I was scared, I hadn’t dated someone since after High School. He has never thought differently of me with having oxygen. He told me the first time he seen my picture he loved my smile. He has told me early on that he wants to take care of me, no matter what happens. He is the one I am going to marry someday, and have children with. He knows I can’t have children and he would never let me risk my health to be selfish because he wants kids. We both do want children one day but doing it the right way. I have always said I wanted to do Gestational Surrogacy. When the time comes we will but adoption is also an option as well for me.

 

That is some of my story; I still have a lot more of it to happen. I hope any young adult woman or man who reads this that has PH gets some glimmer of hope from it and it changes how they feel about their life. I have always told myself when people say “Your life must be hard?” I tell them “Someone out there has it much worse than I do.” It’s the truth. I have never been unhappy about my life. This is my life and I live it to the best of my ability.

If you would like to read more about Tia and her day to day life please make sure to visit her blog: In the Life of Tia.The PHight or Flight Project would like to thank Tia 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 T-shirts

To help raise awareness for pulmonary hypertension and to help raise funds for Pulmonary Hypertension Association of Canada I have created #PHighter t-shirts. The way that it works is that I have 21 days to sell 21 t-shirts. I decided not to start small because I am not sure how many people would be interested in purchasing a shirt. (Having PH or being a caregiver to someone with PH is fairly expensive after all!) However, it is a purchase that you can feel good about because you know it is going to help support PHA Canada.

The shirts read #PHighter, come in the colour purple, and can be purchased in a premium unisex size or a girl size. Below is an example of a premium sized shirt. Please note that the shade of purple between a girly fit and premium shirt (which is unisex) are slightly different from one another. The prices range from $17-$18 USD. If I don't reach the goal of selling 21 of these shirts in 21 days none of the t-shirt sales will go through, and no shirts will be sold (if you reserved a shirt you will not be charged.) If I reach the goal of selling 21 shirts in 21 days and you reserved a shirt, you will be charged for it after the deadline (September 8th 2014) to sell shirts has been reach. To reserve a shirt and to learn more about where I will be donating the profits to, please visit PHight Against PH.

PHighter Friday: Michelle F.

I am so excited to share my PHight of flight story with you all.  I hope this touches someone somewhere.

I’ll start with sharing with you that God blessed me with a child at the age of 16.  I am lucky to have wonderful parents that made huge sacrifices to raise her.  When I was diagnosed, my doctors told me that I couldn’t have kids because the mortality rate of pregnant women with PH was 50%. Instead of being angry about not being able to have another child, this news made me realize how blessed I already was to have a beautiful little girl.

Team photo before the race

The diagnosis changed the way I valued my relationship with my daughter Christine.  I know now that time is of the essence. The diagnosis really helped me refocus my energy on strengthening my relationship with her, my family and friends. 

July 23, 2013, is the day I went to urgent care for being short of breath.  I was swollen from head to toe.  I couldn’t walk 10-15 feet without feeling short of breath and my heart was racing uncontrollably. After a week of tests in the hospital I got the news. I had class II IPAH. 

I was prescribed Letairis and Adcirca but ended up taking Adcirca only.  In a year’s time, I went from a class II to a class I.

Pre-diagnosis, I had been so used to living with the classic PH symptoms.  I was always tired. I would have intermittent leg swelling, shortness of breath, rapid heartbeats.  Those things just became my “normal”.  I went to so many doctors who thought I had the regular systemic high blood pressure and I was actually relieved that now we knew what was really wrong. I was excited to see what life would be like on medication!

Post diagnosis, I am able to do 30 minutes of moderate cardio without feeling short of breath.  I can walk up 2-3 flights of stairs non-stop.  I even participated in The World Famous Camp Pendleton 5k Mud Run!  I completed this event with a group of amazing friends who helped me make it to the finish line even though I was ready to quit after the first quarter of a mile!  I walked most of the time, but I still finished the race!

It has been one year and two weeks since I was diagnosed.  Physically, I feel 100% better than I did before.  Emotionally, I’ve matured 1000% (one thousand!).  Yes, I know it is horrible to read the life expectancy statistics.  Yes, it is sad knowing this disease is progressive and there is no cure.  Yes, it is frustrating to accept that child bearing is not an option.  But the truth is, like the title of this blog, we have two options.  We chose the PHight or Flight. I chose to PHight. 

My “one year survivor” anniversary just passed.  As I reflect on the years I lived before diagnosis, I realize that I spent most of my life just existing.  In the one year post diagnosis, I have truly been living.  I work full-time as a paralegal at an amazing law firm.  I spend more time being active and making genuine connections with people.  I appreciate every minute have and spend my time doing things that make me happy.

Paola and Me

I didn’t start to live my life until I thought I was about to lose it.