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: Karen S

I was diagnosed with IPAH 8 years ago at the age of 52, after years of palpitations and breathlessness on excretion. Prior to diagnosis I presented to my GP on various occasions with my concerns about these symptoms. Each time I was told that it was anxiety/depression and finally prescribed anti depressants. Despite the advice from my GP my concerns and symptoms remained.

I would become breathless during my household chores, especially vacuuming and climbing the stairs. My palpitations would start, or as I referred to them, my flutterings. I even called it my 'jelly heart'. I would joke to my family ''you will remember this when I've gone''. I never dreamed it would soon be so serious.
The breathlessness and palpitations continued and were now accompanied by dizziness. I would become dizzy just walking from work to my car. I knew it was time to return to my GP.

At that visit my usual GP was unavailable and I was seen by a locum doctor. I was not expecting much but to my surprise I truly believe that this doctor saved my life! He listened to me, examined me, and decided to send me for an ECG. There then followed a battery of tests at my local hospital which lead to a diagnosis of IPAH and a referral to Papworth Hospital. I had no idea what IPAH was. When I inevitably consulted Dr Google I felt like I had been hit with a sledgehammer. I was in turmoil, I panicked I didn't know what to do with myself. I wanted answers, why me? I was a healthy woman, I had never smoked or abused my body how could I have something wrong with my heart and lungs? The words 'incurable' and 'fatal' kept going round in my head.

After attending Papworth for a 5 day stay, I felt more hopeful as I was now on a drug - bosentan and had now been given a prognosis of about five years.

I was doing well on Bosentan but it suddenly began affecting my liver and so I had to stop taking it. I was then put onto sildenafil. A few months later I agreed to take part in a trial for a pill form of treprostinil.
I was feeling so much better, I could climb stairs, I had more energy, I would go on walks and bicycle rides. Life was good again. I continued like this for about five years 'the deadline'. Then very, very worryingly, I started to feel ill again. The breathlessness and palpitations started again. I was in panic mode again and denial, I even lied to the team at Papworth telling them I felt ok, but they knew differently. I was then prescribed ambrisentan along with the other drugs that I was taking I was elated, another ray of hope. I just got better and better. My 6mw improved and my lung function tests were better. I was back to my normal life.

Then a few months ago I was asked to take part in another trial. This involved having iron infusions. I felt fantastic! My family noticed a difference in me and my daughter claimed she had her 'mom back'. My exercise tolerance and general wellbeing were like they were before I ever had PH.

A diagnosis of PH is frightening and devastating, but I am testament that with a positive mental attitude, support from a wonderful family and friends, a wonderful team at Papworth ( and mustn't forget the drugs ) life can go on and there is so much help available. I feel very hopeful for the future.

PHighter Friday: Shannon

Confessions of a Young Adult PH Patient

Hi everyone, my name is Shannon O’Donnell and I am twenty years old, and I have primary pulmonary hypertension. I am on IV Remodulin, Revatio, blood thinners, high blood pressure medication, and oxygen at night.  I was diagnosed when I was six years old. I was told that I wouldn’t live pass a year without a double lung transplant and now it’s been fourteen years since then. And now, I’m doing great, I’m off the transplant list, and talking to my doctor about getting off IV Remodulin and going to the oral form of it.

My story begins on May 31^st, 2001, I was six years old and  had no idea what was going on due to being a young child not really understanding why I’m different from my friends. My doctor wanted to admit me right away but my parents took me home in order to graduate from kindergarten with my friends, my parents wanted good things to remember of me before we started treatment. A few days later, I graduated kindergarten and had huge party with my family. The next day, we went back to Boston children’s started this rollercoaster called fighting PH. I started out on nitrous oxide then that stopped working and I had no choice but to go on Flolan, which was only approved for adults since there were no medicines approved for children at the time. That’s what I remember of the start of this uphill battle.

In second grade, I went on a Make-A-Wish to Orlando; my wish was to go to SeaWorld and swim with the dolphins and feed Shamu (a killer whale or an orca whichever species name you prefer.) I got to Disney too, and see my family mom’s side as well. Then six years later I was put on IV Remodulin due my school system threatening to take away my rights to education due having to be on ice packs 24/7 unless I switched to medicine that did not involve being on ice. Then being on the cad pump got in the way of being a teenager and I switched to the crono-5 pump. About three days after going on the pump, it decided to go off in the middle of class. I raised my hand and calmly told my teacher I had to go the nurses office and my headmaster (principal) saw me asked me to hand over my phone (he thought the pump was a phone.) I flipped over my pump and he escorted me to the nurse when saw he look of fear on my face.  Then a year later came prom, and I had no idea what to do with my pump, so my sewing teacher transformed a spandex skourt into a spandex skourt with a pump pocket. I still use it till use it today whenever I wear a skirt or a dress. Then a year later came senior year. Senior year ah the worries of college acceptance letters, SATs, and graduating. Not for me. I was in and out of the hospital and was barely passing all my classes. Well, I graduated and moved on to a community college where teachers don’t understand zero below temperatures aren't good for PH patients to be waiting for a crowded train.

I was told I couldn’t go to school full time or do sports. Now I go to college part time at a local community college, and take karate at a local dojo. I volunteer at The Hole In The Wall Gang Camp founded by Paul Newman (I was a camper there as well.) I also teach karate to mentally challenged children, and teach second grade religious education at my church.  Life at diagnosis was hard because you can’t tell a six year old not to run around. I don’t really remember much expect that. Back then, I coped with everything by playing my dolls, coloring, reading, listening to music, and playing video games. Today, I still cope by drawing, reading, listening to music, playing video games, and watching TV and watching movies. If I have learned anything from watching many Disney movies is that even if you’re different you will always have good friends by your side no matter where you go.

The advice I would have given myself when I was younger is that you got to hold your head up high and smile because you are the greatest star. My advice to anyone that reads this is that "Your imperfections make you beautiful; they make you who you are.” –Demi Lovato.