PHighter Friday: Kayla

Hi my name is Kayla and I am 22 years old.  I am not your average young adult, I have overcome a lot of health issues during my life.  I was born with a lot of health problems including being born with a Giant Omphalocele, (all your organs outside of your body in a jelly sack) hole in my heart, (fixed when I was seven), club foot, scoliosis, and I was tube fed until 5 years old.  I also didn't get to go home from the hospital until I was about 3 months old.  During my childhood I was in and out of the hospital.  After middle school, I had a seven year gap of non-serious health issues.  Until the summer of 2014, when my world was turned upside down. 

I remember it was a hot summer day and I was walking in the mall with my mom and I was walking very slowly and trying to catch my breath.  I thought I was tired from the heat and maybe hungry.  So we made it to the food court, sat down, and got something to eat.  After that I felt better and could walk back to the car.  The next morning I was gasping for air while I was asleep.  I have never experienced that before.  My mom immediately called the doctor and got an appointment that morning to check out what was wrong with me.  When I got to the doctors I was still having a hard time breathing, but I was still talking and alert.  They decided to check my o2 sats and I was in the 60s.  They immediately called the ambulance and rushed me to the ER.  The ER thought I had an asthma attack, but I never had one of those before.  I do have asthma but it's only reactive when I have a bad cold.  So they gave me tons of breathing treatments and it helped a little, but not enough.  The doctor decided to admit me to the hospital.  He told me he wouldn't let me go until he found the answer.  It took a week to do all the tests to determine what was going on.  The last test to confirm their diagnosis was the right heart cath.  That was very scary being awake on the operating table.  I held it together barely.  When the heart cath was done the doctors told me I had Pulmonary Hypertension.  I was confused, scared, and speechless.  I had no clue what PH was.  The doctor explained in detail and still I didn't understand it.  All I remember was it is not curable and I would have to live with it for the rest of my life.  In the past I was use to having health issues and having the doctors solve them the best they could.  But I never thought I would have to deal with a rare and non-curable disease for life.  On top of that I had to be on oxygen 24/7 because of my low oxygen stats and I'm still on oxygen today.  

All of this was new territory for me.  I was in denial for awhile and kept asking my parents why is this happening to me.  They kept explaining it to me and they were really supportive.  Eventually maybe about a couple of months later I adapted with my new way of life.  I have always been a positive person and I knew I could get through this tough time because I have done it my whole life.  The o2 and the meds were what was saving my life.  I was in a new semester in college when I was diagnosed and had to get back to it.  I went back a week after coming home from the hospital.  I had to drop 2 out of 3 classes because I would have been too overwhelmed.  My parents helped out and bought me a portable oxygen concentrator, so it would be easier to go to school and get around.  I even bedazzled my concentrator, to make it more fashionable!  I got through that semester and during that time I knew I wanted to spread awareness about PH.

I never heard of this disease before until I got diagnosed with it.  So I knew I needed to do my part and raise money to help find a cure.  That's when I decided to start my own fundraising jewelry line called PHantastic Jewelry.  I always have been into fashion and love being crafty.  I created my own website (phantasticjewelry.com) to share my story about being diagnosed with PH and to sell my handmade jewelry for awareness.  The net proceeds go to the PHA to help find a cure.  I knew for myself to cope with this "new normal", I had to give back somehow and turn it into a positive.  I enjoy making the jewelry and spreading awareness about PH at local craft fairs and open markets.  Also at these events I get to meet a lot of great people and hear their stories of their own health battles. 

I feel good that when I share my story people are comfortable to share their's.  Some people may wonder how can someone on o2 be doing so much.  Well, since I was born my family, friends, and God have been my support system and my parents taught me an important lesson early on to never give up.  There is always a "silver lining" in hard times.  I strive to do my best and achieve the goals I want in life.  This past year in a half has been intense, but I learned so much.  With being diagnosed I actually have had more energy and developed an exercise routine with the help of Pulmonary Rehab.  I just finished another semester in college and I took 3 classes.  First time having that many classes since being diagnosed.  I feel so good that I am back at where I wanted to be.  I am so appreciative because I am stable with PH at this time and I can focus on school, enjoying being with my family and friends, and working on my fundraising jewelry business.

For the first time in awhile I feel like I can slow down and "stop and smell the roses" instead of being in survival mode.  I hope sharing my story will help others that are having a tough time that it is important to remember take it day by day.  And your life will change a lot being diagnosed with PH, but really try to have a positive outlook.  It truly helps in this situation!  Also allow yourself time to adapt to your new way of life.

PHighter Friday: Stacie

It was 2002, so nearly 13 years ago now, I was 11 years old just going up to secondary school. I was born with 3 holes in my heart and was regularly being seen at Bristols Children's Hospital at this point in time so I had always been ill and knew how to not push myself in regards to exercise. I had recently had a operation because my condition was declining more rapidly than we were used to I was due for a follow-up appointment at Bristol the first week of secondary school. During my first week of school I collapsed several times because unlike my previous school this school had a lot of stairs. When I was seen by my consultant at Bristol he told me he had no way of treating me any more and I either needed a heart transplant or it could be Pulmonary Hypertension in which case I had to decide whether to go for a heart transplant or try the trial that Great Ormand street were carrying out on children. A heart transplant was obviously extremely daunting for 11 year old me like it would be for anybody, no matter their age, so I decided to try the trial with Great Ormand street and if that didn’t work we could then go for the heart transplant. I was later diagnosed in November of 2002 with Secondary Pulmonary Hypertension.

I was extremely lucky, they inserted a small 7mm (millimetre) tube into my pulmonary artery which helped keep it open during exercise, I was placed on Bosentan and warfarin and these worked great for quite some time I managed to make it through secondary school as a normal teenager or at least I appeared normal to everyone around me, I was also having oxygen whilst I slept, it was when I was 18 and in college that things started to decline again we put me on Sildenfil and then had to up it again because it made no difference, once again it made no difference at this point it I am 20 years old and it is 2011 the time had come for transplant. In the UK you cannot be put on 3 different types of medicines or “therapies” as they call them due to financial restraints within our NHS unless you are on the transplant list so for me to get more meds this was the way forward.

In May 2011 I had to meet with a transplant consultant from one of our transplant centres Harefield this was to see if I was even a candidate for transplant, she decided that I was and and this then proceeded to a 3 day assessment at Harefield hospital just outside of London. For any of you reading this who have Pulmonary hypertension to explain the tests you have to do for a transplant assessment just think of all the tests or procedures you’ve ever had to do for your PH and that’s probably in the region of how many tests you have to do, just to give you an idea. I had to wait 6 weeks following this to find out that Harefield would not transplant me because at the time they could only do double lung transplants and my heart was just way too damaged for that procedure this was when I was passed over to Papworth hospital near Cambridge 1 of only 2 hospitals at the time that could perform Heart and double lung transplants in the UK. I had to do everything all over again the initial meeting the 3 day assessment until finally after 11 months I was placed on the transplant list on April 20th 2012.

Now that I was on the list I could be given extra medication we first tried the Iiloprost nebuliser which I persevered with for 10 months but it did in fact do nothing and my condition declined in that time and then finally we gave me a Groshong Line and placed me on Flolan/Epoprostenal which although has made no significant improvement to my condition has been keeping me stable for the past two years while I wait for my transplant.

On April 20th 2015 I will have been waiting 3 years for my transplant. I can’t say being on the list is easy because it is extremely far from it I’ve had some very scary times whilst on the list but it’s like anything I would imagine you have your good and your bad days but you really just have to make the most of the good days. If you happen to end up in the same position that I am in I think you just have to try and stay positive, I’ve had 3 false alarms for organs in those 3 years and although at the time they were devastating to me because they didn’t end up going ahead they were each learning curves. My first taught me what types of organs I was willing to except, the second showed me just how scared I really was and the 3rd taught me that I really truly am ready for this.

Although I am on the list I still manage a few things I’m very close to getting my history degree after 3 years of studying which I wasn’t sure was going to happen when I was told at 18 I wasn’t allowed to go to university by my doctors. I still manage to be a awesome sister, daughter, aunty and friend. I also actively try to promote and raise awareness for Organ donation and Pulmonary Hypertension. As much as it sucks needing a transplant and having Pulmonary Hypertension I have never let it define who I am or stop me from being who I’ve always been and it has in fact let me help other people. It is a hard road to travel but it’s what you do whilst you are on that road that matters.

Blog:- stacie-lifeisworththefight.blogspot.co.uk
Twitter:- @staciep90
Facebook:-  www.facebook.com/stacielifeisworththefight

PHighter Friday: Colleen S

In the PH community, I often hear people being asked what their life was like before diagnosis. I've been asked this myself many times over the years. The thing is, my life before I was diagnosed with PH was the same as my life is now. I don't know what it's like to breath normally. I don't have a clue what going from an active lifestyle to a PH lifestyle is all about. That is because PH has been my entire life. It's all I've known. I actually thought, as a kid, that it was normal to take hours to recover from running around with your siblings and friends. I thought everyone took naps when they were exhausted! And although everyone around me, family included, always told me I looked purple, to me that was just my thing!

I was diagnosed when I was 9 months old, but technically, I should have been diagnosed months earlier. My mom brought me to a free clinic to get my first set of immunization shots, and a doctor there thought something was up with my heart. My mom took me to the pediatrician, who didn't think anything was wrong. So at the same free clinic for my 2nd set of shots, the same doctor who saw me the first time told my mom that something was seriously wrong with me, because I looked blue. My parents ended up taking me to a children's hospital, and after many tests, they discovered I had a pretty bad congenital heart defect and pulmonary arterial hypertention. At that time (1975), the doctors couldn't do anything. It was too late for surgery to correct the holes in my heart, and there wasn't anything to treat PAH. My parents were told I might not make it to my first birthday, or I may possibly live til I'm 50. They weren't given much hope! But here I am, 39 years later, and I'm still living to share my story!

First Airplane Trip

I grew up with a lot of "cannots." I cannot participate in sports. I cannnot take gym class. I cannot go on rides that'll get your heart rate up too much. I cannot get pregnant. And so I lived my life following the "cannots." I often wonder if that really was a good thing or a bad thing, but I guess since I followed those rules pretty closely, and I'm still here, it was a good thing? I followed my grade school dream of becoming a teacher, getting my Bachelor's and eventually ending up in a preschool classroom. I loved it! But after only a few years, those preschooler germs started loving me. Too much. I was on my third major respiratory illness during my third year of teaching when my primary doctor came into the exam room with tears in her eyes and told me I had to quit my job. She wrote me a note that I took to my director right after the appointment. I was 24 at the time, and never even thought about quitting a career at that age. Who would??

A few years later after my PAH symptoms continued to get worse, I ended up going to the Cleveland Clinic in Ohio. I was referred by my doctor to go for a transplant evaluation. At the time, the shortness of breath started happening more frequently (just getting dressed in the morning was becoming a 30 minute event), and an elephant had permanently moved in on top of my chest. That was one symptom I didn't have growing up, and it was not a symptom I particularly enjoyed! Being sent to Cleveland was very scary. Transplant was even scarier!! But my first visit, I was sent back home after so many tests with instructions to start taking Coumadin, a blood thinner, and to wear oxygen all the time. After several more visits to Cleveland in the following months, I was put on Tracleer. I had no idea what it was, or exactly what it would do. All I knew was that I surely hoped it would help my elephant to move on, and that I would start feeling better!!

My first shipment of Tracleer brought me not only the first medicine I'd ever try for PAH, but it also brought me into the world of the pulmonary hypertension community. I grew up knowing I had PH, but it was something that was never focused on. My heart condition was the central star all those years. Once I found the Pulmonary Hypertension Association website, my knowledge of PH started to expand. But what really hit me?? THERE WERE OTHERS LIKE ME!!!! I seriously spent a week reading the message boards and crying, because there were people I could relate to and totally understand!! It was like finding a miracle! 

Tracleer got that stupid elephant to move out several months after being on it, and I also didn't feel as short of breath doing certain things like I was always used to feeling. It was a pretty awesome feeling for a few years, until I felt like I needed to add another medication to the mix. Revatio was added, and the combination of the two is what I am still on today. Pulmonary rehabilitation was something I found out about from phriends (friends who have PH), and after inquiring about it, I started going twice a week at one of my local hospitals. It's been a little over six years since I've started pulmonary rehab, and I truly believe it's a part of what's been keeping my PAH stable in the last several years. Kinda hard to believe I went from no gym or sports as a kid/teen to working out a couple times a week at least! No, I am not running marathons or doing heavy weightlifting, but that is ok! Exercising at any capacity is beneficial, even if I can only do it a little at a time!

So after my PAH seemed to be stable for awhile, the next part of the equation was....what am I going to do with my life?? I couldn't teach. I knew I'd never go back to that. But I didn't know what else to do. Some days I had more energy than others. I'd have one day where I wish I could be at a job, to several days when I was thanking my lucky stars that I didn't have one. Then one day I got a call that started the ball rolling for me towards a direction I never thought about. I was asked if I'd consider starting a support group for PH patients in my area. At first, I was pretty terrified. I could handle being around kids, but to lead a group for adults??? I guess you could say that my shy background made the whole prospect a bit daunting for me! I really had to think about it. So, that's what I did. I spent a couple weeks thinking and praying and talking to my loved ones about the idea. I finally decided to go ahead! Yes, I was incredibly nervous about it, but I felt it was something I just HAD to do. How many years did I live with this disease by myself?? I just knew I had to at least give others the chance to not feel so alone after their diagnosis! 

In the 10 years since starting a support group in the Niagara Falls/Buffalo, NY area, I have really become involved with helping PH patients. I've had so many meetings, a large variety of topics and speakers, and phriends I care about very much. I have also become a big part of the online community for PHers, by being a PHA mentor, a chat leader, a blogger, and just a presence on Facebook. I had always wanted to teach in a classroom, but it took me awhile to realize that a "classroom" didn't have to be a place with 4 walls. It could be anywhere! I have even educated complete strangers during shopping trips about PH! And, in the 10 years since starting my support group, I have also learned to not be so shy with people. Sure, I can keep quiet sometimes, but more often than not, I put myself out there and let others know about this awful illness. Awareness is the key to so many answers, and I never know when one day I will talk to someone and they tell me they have PH, too!

My life with PAH is something I cannot change, and honestly, I think it has helped shape me to be the person I am today. Do I have days when I am angry about having it? Of course! I allow myself to have those angry days, or days of self-pity, but not for long. What good does that do me? I have learned to deal with this disease, and to cope the best I know how, and I have learned to help others going through the same thing. I have also not let PAH BE my entire life. I enjoy things like reading, painting glass, making jewelry, spending time with family and friends. In the last few years I have allowed myself to take on challenges I never thought I could do, because I always had that "cannnot" attitude with me. So I've gone on hikes, I've been on a jet ski, I took my first ever trip on an airplane! There are so many more things I've let myself try, even if I may not be able to do them again. I try my best not to let PAH take over my life, because if I did, I can't honestly say that I'd be living to the best of my ability!

PHighter Friday: Evelyn

On December 21, 1988 I was born a preemie. A few days later I was diagnosed with a small heart murmur, Dextrocardia. Dextrocardia - is a condition in which the heart is pointed toward the right side of the chest instead of normally pointing to the left. I was also diagnosed with Mitral Valve Prolapse (MVP). MVP occurs when the valve between your heart's left upper chamber (left atrium) and the left lower chamber (left ventricle) doesn't close properly. 

Despite all of this, my parents were told not to worry and that I would grow up to be a healthy young lady. Every year I would just come in to get a check up. As a child I was very active, always riding my bike, running around with the neighborhood kids and swimming. I don’t remember ever getting out of breath.

It wasn’t until my preteens that I noticed my breathing had started to decline, and that I couldn’t keep up with my friends anymore. Even if we were just taking a walk I often had to stop to catch my breath or I was given piggy-back rides. Luckily for them, I was a tiny little thing. But that didn’t stop me from doing everything they did even if it took me a little longer or it meant we had to walk slower because of me. At the age of 15 I was in the10th grade and I had two classes I had to walk outside to get to. It didn’t seem to be a problem until the cold weather started to come in. Once again, my breathing was acting up and I often had to take a break causing me to be late for class. I ended up going to the doctors and they did a few tests to find out I had asthma. They gave me an inhaler and sent me home. A month passed and I didn’t see a difference in my breathing even though I was doing my inhaler as instructed. A few times walking to class my lips would be tinted blue but I wasn’t alarmed because it was the middle of December and it was freezing out.

One morning walking to class I felt myself starting to get a panic attack. The school nurse sent me home. I couldn’t seem to calm myself down so I laid down on my couch to see if a nap would help. I hoped I would wake up feeling better but instead I woke up about ten minutes later drenched in my own sweat. I was coughing uncontrollably. I could feel myself passing out, everything seemed to get blurry around me. I woke up a few seconds later to my mother hovering over me, crying hysterically. I was in heart failure and if my mother wasn’t there to give me CPR I don’t know if I would be here right now telling you my story.

I was rushed to one hospital and then another, finally landing at Children’s Hospital of Philadelphia. I was so weak that I couldn’t talk but I was very aware something was wrong. I pushed myself to not fall asleep again for fear of not waking up. I was 15 years old and I was not ready to die. Children’s Hospital Of Philadelphia would soon become my second home and family. I was tested right away for pretty much everything you can think of. One of my earliest memories was of a Doctor coming to see me in the Cardiac ICU. He told me he thought he knew what was wrong but that I’d have to have a right heart catheterization to officially diagnose it.

A few hours later I woke up with a port in my chest and medicine flowing through it, into my body. As sick as I was I truly did not care what the doctors needed to do. I just wanted to get better and get out the hospital. At this point I didn’t realize how serious the situation was. I was finally diagnosed with Pulmonary Arterial Hypertension by my Pulmonary Doctor, who is now like a second father to me. I will never forget when he told me “you’re crying, that means you understand.” He promised me I would walk out of Children’s Hospital but I didn’t believe him. All I saw was a dark cloud over me for the longest time. He was true to his word, though, and I can never repay him for being the amazing person he truly is to me, and many others.

Pulmonary Hypertension can be very hard to wrap your head around. There are many questions to ask and some will always be unanswered. It’s scary for the patient and also for your friends and family. I am truly blessed with my support system. As long as you have people around you that care about you, things will be okay. While I was in the hospital getting used to my new life with PH I met my first PH friend. She is no longer with us but she will forever be in my heart. I’d be lying if I didn’t think I was going to be next. Whether you have PH or not though, everyone’s body is different and that’s important to remember.

I was finally well enough to be sent home. Going back to school with PH was very very hard; being a teen itself can be hard. Your teenage years are when you start the journey to discover who you are and there I was, hit with a curve ball with my health. But in some weird way it has shaped me to be stronger than I ever imagined. I’ve met so many wonderful people due to PH. I introduced PH to every classmate and teacher I had over my high school years. I figured, if I’m going to have it, I might as well make it aware and known. My way of dealing with things was to talk about it and educate others. All in all it makes me value life so much more than I ever would have if I didn’t have PH. Because of it I know what it’s like to be at the bottom. Now I’m at the top.

I wish I could meet everyone who is newly diagnosed with PH and let them know they’re going to be okay and not to focus so much on the negatives, which I know is easier said than done. Always keep your head held high with pride and know who you are. PH does not define you or own your life. Everything will become second nature and fall into place and that port you’re attached to 24/7 or all the pills you have to take everyday will become completely normal and a part of your routine. There will be times when you will question “why me?” and like I said earlier, some questions might never be answered. Just know that even though PH might take you for a ride every once in awhile and you will have some bad days, just go with it and remember you’ve come a long way already.


I’m 25 years old today and just celebrated my ten year anniversary with PH. I’m excited to celebrate many more.

Make sure to follow Evelyn on Instagram to see what she is up to!

The PHight or Flight Project would like to thank Evelyn 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: Michelle

I was never "normal". I remember as a child, I would lose my breath before the other children. As a teen, I would get tired and couldn't be athletic. At 20 I had emergency open heart surgery to repair a hole in my heart. The Dr told me I would feel so much better. I did, for a short time. It all started again.... short of breath, flushing and getting tired easily. I figured it was because I was over weight and I smoked. That's what everyone thought.

At age 27, came the diagnosis, Pulmonary Hypertension. I was told life expectancy was around 2 years. I was scared and confused. No one I talked to had ever heard of it. I knew immediately I would need a specialist. I did research on the internet, made an appointment, and quit smoking. I started oral therapy and I felt a bit better though I was very depressed. I'm the one in my family that thinks I have to take care of everyone else. I let the depression win and I stopped my treatments. I could not afford the copays for the medicines. I didn't tell anyone this or I would have quickly learned of the help that's out there. 

  
 I continued to get worse. I reached out to another specialist. I was told five years earlier that I wouldn't live more than two years. I had proved them wrong. How much better could I have been if I would have kept up my treatments? That's when I met Dr Frank. He did not sugar coat anything. I was quickly scheduled to have a permanent IV in my chest to receive meds 24/7. I wasn't really given any options. I didn't argue. A month later I walked into his office without the assistance of a wheelchair! He was very impressed and confessed to me that he had thought I had waited too long and wouldn't make it to my follow up. I proved him wrong too!

Maybe I can beat this! I have now been on treatment for almost five years and I am doing great! I went back to work part time. I recently started walking a mile a day. I replaced old hobbies with new ones that doesn't require much exertion. I like canning, knitting and crocheting. I have good days and bad days. But as long as I'm still having good days I will continue to PHight! I have a new attitude about this disease. There is no expiration date stamped on my body! I firmly believe your attitude can break or make you in this disease. I allow  myself down days. It's normal! I have days when I can't do the things I want to do. It's normal! All of this is MY NORMAL. We are all different but I try to stay as positive as I can. I absolutely love talking to other PH patients. I try to keep them positive and strong as well. I could have given in ten years ago. I think I was close to that. I'm glad I started phighting. I now have a 2 year old niece that is my heart! I thank god that my meds are helping. My pressures have lowered tremendously! I have joined many support groups and love being an inspiration to the newbies as a long time survivor! Ten years and going strong!!

Editors note: I saw that Michelle works as an optical technician, which sounds like a very busy job! I asked her a bit about her position and she says she works about 20 hours a week and that she loves what she does.

The PHight or Flight Project would like to thank Michelle 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.