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: Jamie

Post-transplant

I always find it incredibly difficult to explain my PH journey. Even within the small tightknit community of PH, most of our journeys are vastly different. My story starts as a disaster but ends five years later and includes a family, a job, school, and enough energy to walk five kilometers a day.

Let me start from the beginning. Firstly, I was diagnosed late… actually 23 years too late. I was born with a hole in my heart and after countless doctors’ appointments where I was either given antibiotics, or accused of lying to get out of work, I spun my car out on the busiest highway in Toronto, which finally led to my diagnosis.

After going to the local walk-in clinic, and having an oximeter reading done, I was diagnosed within the month with stage IV pulmonary hypertension.

My health for the next four years really only went downwards, oral medication did not work, subcutaneous medication did not work, and when I was finally put on IV Remodulin, while it was true that I was feeling much better, a month in a clot broke off the line and caused a minor heart attack.

The final breaking point before my life changed forever was here in Ottawa. I had chest pain so I texted my nurse coordinator who told me to go to emergency immediately. I was pretty busy, so first my wife and I signed my school loan documents before heading over. This was a VERY bad idea, my saturation levels were sitting at under 50 percent, and I was heading into full organ failure.

The decision my PH team came up with was to fly me to Toronto General for a transplant workup. It turned out that I was so far gone, that they put me at the top of the national registry immediately, and I had a double-lung transplant/heart repair within 48 hours.

As I said this was the shift in my life to make everything better. At the end of three months in Toronto, I was jogging, walking up seven flights of stairs, and doing things that I was never able to do.

 I’m not suggesting transplant is for everyone, certainly it has its up’s and down’s, but for me, I’m the healthiest – and happiest – I have ever been.

PHighter Friday: Becca

 At one month old, when my parents adopted they were told, "I has a 13% chance of survival to the age of 5."When she was 3 months old they were told by the doctors there was nothing more they could do... they felt that I had 6 months or less to live. Hospice started coming to our house to help my family through the darkness. When I was a year old we were told that "things" had changed with her heart. There was a surgical procedure that could be performed that would enable me to grow up!

Miracle #1.On November 19th 1993 when I was 13 months old I had my second open heart surgery. The operation went well but I would not come off of the heart and lung machine. I was put on ECMO, a portable heart lung machine considered a "heroic measure" and taken to the Intensive Care Unit. Four days later she was taken off of ECMO and a month later she left the hospital.

Miracle #2.When I was 26 months old we traveled to UCLA for my 3rd surgery. My family had been told it was very high risk and I would have a very "rocky" recovery period. I surprised everyone, did wonderfully and was home in 10 days.

Miracle # 3.When I was 5years old she had her 7th heart catherization. In this cath massive ballooning and the placement of 2 stents were performed. I began bleeding from her lungs... they felt it would be very difficult to stop the bleeding, and if the bleeding did not stop she would die.

Miracle # 4.I had surgery at Lucile Packard Children's Hospital (LPCH), part of Stanford, when she was 12 years old. She did well but 2 days after we returned home she was back in a local hospital’s Intensive Care Unit fighting desperately for her life. During surgery she had contracted a deadly infection Methicillin Resistant Stafyloccus Arus (MRSA) that raged through my body. After 5 weeks in intensive care I went home very weak, in a wheel chair, with a PIC line and 6 more weeks of IV medicine to finish.

Miracle # 5.In my 8th grade year I started having arrhythmias. After trying for 4 months to control my irregular heart beats my condition turned life threatening and I was life-flighted to LPCH. I spent 2 weeks in the intensive care unit as they increased a strong anti-arrhythmia medicine and placed into me an experimental pacemaker/defibrillator.

Miracle #6 Just this past summer I had to go all the way to Pittsburgh for a heart-lung transplant evaluation. After being turned down by 3 other centers, Pittsburgh was our last hope. Thankfully the surgeon has decided that he thinks he can successfully do the transplant, but that at this time I am too healthy.

Looking back at my life, there were more than a few times when I wasn’t expecting to make it. Now I just celebrated my 22nd birthday with family and friends. Going to college was something my family didn’t know I’d be able to do, but I’m in my 3rd year at a community college and I love it.

I was lucky in the sense that I was born with my PH (secondary to my heart defect) because I have had my whole life to learn how to cope with this and I don’t have an old life to look back on and miss. But there have been times when I think about having a normal life or the things I wish I was able to do (go on roller coasters, fly without oxygen and go into the mountains) and miss the thought of those experiences.

In all honesty though, I’m not sure I’d change the fact that I’m sick if I was given a chance to. If I hadn’t been born sick I wouldn’t have been adopted by the amazing family I have now, wouldn’t have gone to the wonderful heart camp where I made so many friends, might not have picked up my two favorite hobbies; writing and performing ASL. Having PH has impacted my life negatively and I know I will more than likely die from it, but I’ve learned to look at what it has given me as well.

They say "It takes a village to raise a child." For me the saying would need to be changed to "It has taken five hospitals (in 3 different states), 4 pediatric cardiothoracic surgeons, several pediatric cardiologists, intensivists, pulmonologists, pulmonary hypertension specialists, infectious specialists, nurse practioners, nurses, respiratory therapists, a wonderful pediatrician, a special child psychologist, a loving family, caring friends and understanding teachers and schools to raise me and allow me to thrive as much as I have.

http://lifeasachronicallyillteen.blogspot.ca/

PHighter Friday: Randy

My name's Randy Shifflett. I was born with a hole in my heart and had it repaired at age four. Which I found out is what caused me to have pulmonary hypertension later in life. When I was thirteen I ended up having pneumonia in both lungs and it just kept getting worse and worse. It got to the point where whenever I went to the local  hospital, they would immediately take me back because my oxygen level would drop to the seventies. They kept telling me it resolving pneumonia and keep taking the medicine and rest. So, my mom ended up taking me to the University of Virginia (UVA) hospital and it was there when I was diagnosed with pulmonary hypertension.

They admitted me and tried medication after medication but none seemed to work. My mom asked one of the nurses what was going on and he told her that he wasn't supposed to say anything but they honestly didn't know because they were giving me medicine that they used for full grown men. Finally the next day they found a medicine called Tracleer that brought my pulmonary levels down. The medicine worked up to ten years. Then I started going back downhill. The medicine just wasn't working anymore so they ended up changing my medication (I'm sorry I can't remember the name of it). I ended up having an aortic aneurism and had to have another open heart surgery in 2010. It was a long recovery but I finally got through it.

After all that, I told the doctor that I just didn't feel like the medicine was working. So, I ended up on a Veletri pump that I was on for three years. I had headaches, body aches, chest pain, losing my breath, etc. My oxygen level would drop to the eighties during the walk test at UVA. I went through that for three long years after telling the doctors that it made me miserable. Three long years of constant misery and aggravation dealing with having to clean the site, covering it when taking a shower, getting the line tangled up when I went to bed... and they finally switched me to Opsumit and had my central line removed. Now I'm doing good. My oxygen stays in the 90s and improved my distance in the walk test.

I know I was very long winded in this message but I did it for a reason; no matter how hard the struggle, you can make it. It is hard to keep your head up dealing with PH but just take it one day at a time. Don't let life overwhelm you, enjoy it.

PHighter Friday: Anastasia

Slowly but Surely

I wanted to travel the world. I wanted to be a powerful business woman (like Samantha Jones in “Sex and the City”) and have a family. Also, I love sport such as swimming, bicycling, playing basketball, playing badminton and playing tennis.  Most of my childhood was filled with fun activities, travelling, hanging out with friends and breaking the rules. But those dreams were crushed when my doctor told me that I will not be able to do sports that I love, I will not be able to travel, and having children is out of the question….

I was diagnosed with Primary Pulmonary Hypertension when I fifteen years old when I moved to the U.S. Back in Indonesia, I was always chubby and sluggish back in middle school and part of high school. My parents thought it was because lack of exercise and my general doctors in Indonesia did not detect any health issues. When I was about to start high school in U.S., through regular check-up, the family doctor detected irregular heartbeat and I started to notice apparent health issues such as difficulty breathing, chest pain and blue lips. So, I was urged to see a cardiologist and pulmonologist. Sure enough, I had VSD (Ventricular Septal Defect) and PPH (Primary Pulmonary Hypertension).

I was devastated…

For a while, I could not understand why this defect happened to me. Although, overtime I realized that wallowing is not getting me anywhere.

After the diagnosis, I had my open heart surgery on my 16^th Birthday to close the hole in my heart. I am taking medications to control my PPH. It is not easy to integrate this disorder into my life at first.

I realize that I am rare. So, as long as that is the case, why not go all the way.  So the key is to accept that I do have this unique condition and I am going to have to live with it for the rest of my life.

So I learn to get to know myself.

I am thirty years old now. I am working full time as a supervisor, I volunteered at convalescent homes, I go to the gym at least three times a week, I went hiking (5 miles up the hills recently) and I am finishing my bachelors in Management and International Business. I do what I can now and everything else will fall into place. I may not go with the phase of normal people, but I will finish what I start slowly but surely.

I changed pulmonologist who is better and gave me support. She advises that I should exercise and keep doing what I do at my own phase.

I believe that our journey gives us strength; the destination is just the icing in the cake. And getting to know myself is the fundamental part of starting the journey and setting up goals. Also, it is important to surround myself with people who are supportive of me and my goals.

Life may not always go as planned, but it does not mean that I cannot make the best out of the one I have now. Like Mother Teresa said ““Yesterday is gone. Tomorrow has not yet come. We have only today. Let us begin.”

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.