PHighter Friday: Tegan

First international trip to Japan 2010

My PH story starts when I was six, sometime in 1995. I suddenly started having fainting episodes and was quickly diagnosed with Epilepsy, having uncontrolled Tonic-Clonic seizures. 

Within a year I developed a chest infection/cough, that wasn't going away with antibiotics. The local doctor sent me and mum down the road to have a chest x-ray. I don't remember specifics but I know that it landed me pretty quickly in the Royal Children's Hospital Melbourne on the Cardiac ward. Throwing my family in to chaos with my two younger sister being looked after by my grand-parent, while mum looked after me.

Being diagnosed with "Primary Pulmonary Hypertension" Functional class 4 (now known as Idiopathic Pulmonary Arterial Hypertension) was life changing to say the least. This diagnosis didn't just effect me emotionally and physically, but my entire family, my circle of friends, my school life, after school activities and general living. I was initally started on "Diltizem", and given a 5 year life expectancy. As a 6 year old I was blissfully unaware of this.

I managed to go to school full time, partake in sedate after school activities and play with my 2 sisters. If I forgot to take my medication, or refused (as i did in the beginning), I could feel the difference, and would often have a seizure as a result.

Around the 5 year mark I got really sick. I was unable to start high school, I was in and out of hospital trying to manage the symptoms. The addition of a few medications and eventually oxygen.

Hosptial stay 2001/02, recently started
 Epoprostenol and looking much better already

I tried "Beraprost" for a short time, but this made me feel sick three times a day for about thirty-sixty minutes after each dose. It made me feel isolated from my friends as I had to go to the school sick bay to have it during class, and rest until the side effects wore off. It also didn't make me feel much better. 

This period is very vague to me and everything sort of blends together. I know I had multiple seizures at school, ambulances were called on multiple occasion, late night trips into the city to get admitted quickly and always extra drugs and bloods and tests. Mum and I spent countless hours stuck in traffic on long stretches of road on the way to the hospital, we played many cars games, but often I was vomiting or simply too lethargic to play. 

Around this time I was placed on the transplant list waiting for a heart and lungs. I was too ill to go to school at this time, nothing was working. I made it through the year using a wheelchair, oxygen and many many medications. I was in heart failure and the rest of my organs were soon to follow, fluid retention, vomiting and weight loss among the symptoms. I was palliated, something i wasn't really aware of. New teams of medical professionals were seeing me all the time to help with one symptom or another, there was a constant stream of consults, reviews and medical rounds. I had privileges from hospital, and small windows at home.

Early 2001 I was given the green light to start this magical, mystical drug from the US (Epoprostenol) as well as oral "Bosentan" (plus my cocktail of "normal" meds). Conditions applied, a fair few. Mum had to be trained in making the medications, and had to commit to making it up from powder to liquid every single day. We had to be self caring with a PICC line (a semi-permeant line directly into the heart.) I had to do any test they requested (6 minute walks, ECHO's, ECH/EKG, bloods and Cardiac catheters). I was so unwell I was not yet able to have the permeant line, so I had a temporary one inserted with light sedation. 

Kakadu National Park 2014

The temporary line proved to last quite a while. By the time the 2nd one had packed it in, I was well enough to have a bit more sedation and get my permanent line. This was a pretty good indicator that this drug was working. I made leaps and bounds from here. I came off the transplant list, I was no long made palliative and I slowly started going to school. I had missed a few months of school and had to be slowly reintroduced. Luckily with a supportive school, and some great friends I got back into it.

The combination of drugs has changed a bit over the years,. I'm no longer in any organ failure, and I take a portion of the drugs I was taking. I take 2 medications for PH, Epoprostenol and Sildenafil. However, I do take a few a preventions of to make life a little bit easier with symptoms.

It took me a very long time to come to terms with my illness and accept it as part of my identity, but not something that will define me. I can thank the use of this drug for the life I lead now.

I work full time as a paediatric cancer nurse, I ride my bike 7kms each day to work and I have the travel bug. I have it in a big way. 

I still have to undertake the same tests, just much less frequently. I can't swim in the ocean/pool, I can never be too far from a hospital and I have to carry around a pump everywhere I go 24 hours, 7 days a week. BUT I will always try to do this with a smile on my face and a positive attitude!

I've been to over 20 different countries and can never wait to plan my next one. Of course I have limitations, but I'll take what I can get.

Repackaging 3 months worth of medication
for my upcoming holiday

My next trip is the biggest and the scariest and the absolute most exciting thing that I have been able to achieve since diagnosis. I have spent the last 6 months planning everything I might need for a 3 month holiday in Sweden and Europe. There is about 40 kg of supplies and 4 kgs of clothes.

This is a recent development as I switched to a more stable version of my drug. It means more freedom and less volume of medications (Thanks Veletri!). 

It has been a lot of preparation, research and challenges at every corner. I started to document my issues with travelling with a serious chronic illness and intend to continue while I travel.

I know I've had a hard life but I know its also been full of interesting moments, filled to the brim with love and most of all its been surprising. When you exceed the doctors expectations by 15+ years you learn to live life to the full.

There are so many people who help me with my challenges. I feel this is the key to my success.

Stonehenge England 2013

My blog can be found at https://www.tumblr.com/blog/tegamegadrive and my instagram at https://www.instagram.com/tegamegadrive/

PHighter Friday: Alex

From left to right: Alex with her sister Debbie

I had already spent many years sick and in bed by the time I was diagnosed way back in 1998. I had delivered my third and final daughter, Luzy, in 1996. I was always exhausted- sleeping, anywhere from 18 to 22 hours a day. I would get up in the morning, make my kids breakfast, turn on the television, and fall asleep on the couch. I'd get up to make a quick lunch, send the kids outside to play with the neighbors, and fall asleep on the couch. I'd then get up to make dinner, wait for my daughter's father to get home from work, hand him the girls and say "I'm exhausted, I need to sleep," and I would sleep for the rest of the night. This went on for many months until I went to a psychiatrist's office, and told him what was going on. I had no idea where else to go, or what was going on. I explained that I had delivered my gorgeous baby. The first thing out of the doctor's mouth was postpartum depression (of course.) I was pumped full of antidepressants and started doing better, bit by bit, day by day. 

After my ex husband got out of the military we moved to Texas, where I began passing out. I was just getting out of the shower the first time (that I know of) when I passed out due to Pulmonary Hypertension. I stepped out into the hallway, walked towards my bedroom. The next thing I remember was being woken up by paramedics. Apparently when I fell I hit my head against a wine fridge that was stored just outside the bedroom. We were living in my former husband's mother's home at the time. When I was found I was the paramedics were called.  They checked my blood pressure and pulse, and said that everything was fine but suggested I go to the emergency room just in case. I did not have insurance and declined because I was afraid of the cost of going to the E.R, and riding in an ambulance.

I had all but forgotten the fainting spell I had until a few weeks later I passed out again. This time my late sister, Susan, was visiting us from Southern California. We were playing chase around the house with my youngest daughter, Luzy. She was just under two years old, and was having such fun playing and running around the huge yard. I sat on the steps in front of the home and said "I'm tired..." The next sound my family heard from me was head hitting the cement steps, and sliding down several steps from the top. Emergency was called again, and I declined help.

I passed out in total at least 30 times in a three month period. I couldn't bend down to; clean up, tie my shoes, pick up one of my daughters, without getting short of breathe and passing out. I could no longer walk from my from front door to the car, without having to stop at the top of the steps to catch my breathe. Walking to the bathroom or the kitchen from the less than one thousand square foot home, would wear me out nearly completely. My oldest daughter had to take care of her sisters because I certainly couldn't. My daughter's father was working far away, and would come home on the weekends. Even seeing how sick I was, he refused to try to transfer to a prison less than 15 minutes from home. His mother refused to help as well saying it was all in my head, and I that better snap out of it. She had been told about being me diagnosed with postpartum depression and decided that I was just depressed. My family was in Southern California, where I was born and raised.

Finally, my sister Debbie Drell came to visit. She saw me pass out, and have a grand mal seizure (I had several before the time before I was diagnosed.)  I had told her of all the times I had gone in to see the doctor after I had passed out. This doctor always refused to do any testing or blood work. He just kept upping my dose of Prozac. It wasn't until Debbie saw me pass out that she made my former husband go with my to speak to the doctors. Luckily, the medical professional I regularly saw wasn't in that day. We saw a different doctor, who, once listening to my symptoms, listening to my heart and lungs, said to me, "This is not in your head. This is in your heart!" I was immediately sent for an echo-cardiogram, a type of ultrasound test to the heart. The next day I went in to work and called as soon as the office opened. The conversation that took place I remember as if it happened yesterday, but in fact, it took place 18 years ago!

"Mrs Flipse, you need to come in right away" said the nurse. "But I'm at work. Can I come in tomorrow?" I asked. "No, the doctor needs to see you today." Knowing the test was of my heart, I had to ask the nurse on the line "Am I ok? What did the test say?" to which her response was: "You're standing aren't you?" When I hung up, I went to my boss and I said that something was wrong with my heart. I had to go see my doctor right away. My boss didn't want to let me go until my lunch hour, but I knew I had to leave right away. I never returned back to work at that place. 

I asked my ex mother-in-law to come with me to the appointment at the cardiologist. She came certain that she could chastise me after, expecting that there was nothing wrong with me. We didn't wait long at all for the cardiologist, they were expecting me. He told me that I had a terminal disease called Primary Pulmonary Hypertension. My ex mother-in-law asked him how long I had to live. The doctor stated that I had less than two years. He said I was pretty severe and my heart was very enlarged.  After that, I had no idea what else was said.  Right before leaving the room, the doctor kneeled beside me, putting his hand on my knee saying, "if I do one thing, I had to remember the word prostacyclin."  He said it would save my life. I left the hospital dumbfounded. Sobbing in the car ride back all I could think was what about my girls? What about my husband? His mother said to me "Buck up. You can't cry. Grow up."  I sat straight up and did not say another word the rest of the day. 

I was referred to see the Pulmonary Hypertension specialist in Houston Texas, which was a four hour drive each way. I woke up attached to Flolan after my very first Right Heart Catheterization. I stayed on Flolan for four years,  and  transitioned to Tracleer as soon as it became available on the market. I was transitioned off of Tracleer and onto Sildenafil after my latest Right Heart Cath, which showed that I also have Pulmonary Venous Hypertension.  

Over the years I have learned several things, and I would like to share a few of them with you, Please. keep in mind, that even though I have learned these things, it is very very hard to do them!! 

1. Advocate for yourself! I can advocate for anyone else on this planet, and do it running circles around everyone. However I can still not advocate for myself. Since I know that I cannot, I always have a member of my family with me when I go to see the doctors, or have an emergency room visit. If I feel like I won't need to speak up for myself, I go alone and take notes, or record audio of the visit. 

2. Take notes at your appointments, and always go in with questions or comments about your condition, or things you don't want to forget. This is so important for me because I forget so quickly! While I'm taking notes, I miss out on something important, so after having asked permission, I record the entire appointment with the doctor. 

4. Keep a list of all your doctors and their phone numbers in a wallet or purse. I also keep the list in my phone. I keep this information in my  name in my contact list.

5. Keep a list of diagnoses, who treats that condition, and the medications, with dosage, in the wallet or purse. This is also on my phone. I even have my over the counter meds listed as well (like vitamins, tylenol, etc.)

6. Keep yourself busy. I don't ever just sit and stare into space, or sleep the day away, unless my body needs it. If I do, I'm in depression, and know that I need to do whatever I can to get myself out of depression. I was diagnosed with clinical depression when I was 18, but have had it since at least five years of age. I keep busy by doing things I enjoy- like crafting, cooking, playing with my granddaughter, watching movies on Netflix and listening to music, Facebooking and of course raising awareness! 

and lastly but not least in the bunch:

7. Exercise. When I was diagnosed back in 1998 I was told not to do any exercising at all. Cardio was completely out of the question because I didn't want to overwork my heart. After many years of studies researchers and PH specialists are finding that we do need to exercise! Exercising is something that I rarely do, but I know that I should exercise. Unfortunately, getting out of the apartment for more than a few minutes is very taxing on me. However, when I do manage to get out and exercise I really enjoy myself!  

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.

PHighter Friday: Janeris

 Pulmonary Hypertension is a disease I had never heard of until it almost killed me. I've suffered with Lupus since 1997. I was 16, a senior in high school, and began having severe joint pains and fevers. I've dealt with the countless effects of that deadly disease for so long, I didn't look too much into it when I started having shortness of breath in 2006. I figured the fatigue, shortness of breath, and rapid heartbeat were just part of it. I went to a couple of doctors who just confirmed what I thought. It was the Lupus. They'd tell me to take more Prednisone for the inflammation and I did. The shortness of breath kept getting worse. I couldn't bend over without getting dizzy. The doctors I saw would say my heart was working pretty hard but for me this was all normal. I went on living; suffering.

I met my now husband in 2008 and our relationship quickly moved toward marriage. I warned him that I had a lot of issues and might not be able to have a baby. He said that he didn't care. He said God would make everything ok.  He was very supportive through it all.  I still worked out every week and was very active in church. My lack of health couldn't stop me.  We got married less than a year after meeting. It was just in time for my biggest supporter to get the test of his life. We chose to get married in Mexico. It was 4 wonderful days spent with family and friends. We stayed 2 more weeks for our honeymoon. For someone with breathing problems, Mexico is not the place to be. It seemed much harder to breathe in Mexico than in Miami. We still did everything we set out to do. We went zip lining, swimming with dolphins, walking in the city and more. Most of it, however was done with my new husband carrying me. Everyone thought it was so cute because we were honeymooners but I was in severe chest pain and honestly just couldn't walk. The last 2 days of our honeymoon were the worst days of the trip. I began hallucinating and having severe chest pain. I thought of calling 911 but was afraid I'd be stuck in Mexico longer than I wanted. I sucked it up and relaxed until we went home. We requested a wheelchair at the airport but still didn't think about asking for oxygen. I didn't know what was wrong. The air in the plane was much thinner and I thought I was going to die.

We went from the airport in Miami straight to the hospital where I stayed for 3 weeks. I just wanted to go home to be with my husband for the first time. My oxygen levels were at 50%. I was immediately put on oxygen. This is where I started to hear that I had problems with my lungs and might need a lung transplant. Once I left the hospital I sought out a pulmonary specialist. I found a great one in Cleveland Clinic who knew exactly what to do. I was quickly taken in for a right heart cath among other important tests. I was finally diagnosed with Pulmonary Hypertension and put on the right medication. I survived on Revatio for a while. But having to take it 3 times a day proved too much for me and within time I had worsened my condition by not taking the medicine properly. In 2011 I started passing out often. I once passed out at a doctor's appointment and was taken to the emergency room. That was the beginning of my new life. There I was put on Veletri, an intravenous drug. It was a tremendous loss of independence and control. It was a severe shock for me.

I was so sad and had a hard time picking myself up. Every night was hard for me. Tears would soak my pillow.  I didn't understand my new body changes. There was constant urinating, swollen ankles, hot spells, weakness, jaw pain, headaches, you name it, I had it. How would I ever get through this? I felt my life was over.  My church family came through to make sure I did get through it. They knew I couldn't cook, clean, or barely move. So for weeks they came and took me to doctor's appointments, cooked, and were right there for me. What a team God placed in my life. I am loved! Another thing that got me through it was something that my Veletri nurse told me. She said I was allowed 5 minutes daily to feel sorry for myself. After that I was to enjoy the rest of my day. Eventually I didn't need those 5 minutes. That was an important part of the early stages of my recovery.

I began to see that light at the end of the tunnel again. I knew there was a purpose, I just had to find it. Time passed and I was tired of being tired. I had gained 30 lbs. from the prednisone and lack of activity and was still having a hard time breathing. But after 3 months it was time to get back to work as a full-time photojournalist at a local newspaper. Slowly, I regained my strength and started looking for alternatives. I found a functional medicine doctor in my area and she taught me the importance of the right diet for my body. My joy came from documenting my journey on a blog  called My Clean Cutting Board. It dealt with showing people what I was going through and how I was improving my health through whole foods. What a difference eating right made. I took out gluten, dairy, potatoes, eggs, coffee, alcohol, and a few other foods that were causing inflammation and a negative immune system response. I started to get better faster than I thought I ever would. I started the gym again full force. I started working at my photo studio, which I run on the side of my full-time job at the paper. I started singing in church again. And less than  1 year later my husband and I began talking about adoption. He saw my progress and together we decided that we were ready to start a family.

Soon the good food changed my body completely. I was taken off the prednisone and the doses of some of the other drugs I was on were lowered. I was so happy. Just 2 years after I was put on Veletri, we became foster parents. Doctors said I was in good health and gave me clearance for this huge task. We got a 2 year old boy in May of 2013. I realized then that all I had gone through was for this moment. You see, he is autistic and was a handful. He cried when he heard loud noises. Not out of fear but out of pain. I couldn't control him or anything he went through. He was a little Tasmanian Devil.  Through my diet change I had learned about the benefits of a gluten and dairy-free diet on children with autism. I cried tears of joy when I realized all my struggles meant something. I changed his diet and he got better. As I was taking care of him God was taking care of me. I continued to get better and they called me to take in a little baby girl. She was 3 months old. I didn't know how I would do this. Here I am with two babies within a year,  health issues, and 2 full-time jobs? Was I nuts? I knew God was in control. He had a plan for me and He wouldn't let me down.

My husband and I have been taking care of our babies for almost 2 years now. We are close to adopting one of them. I have since lost 40 lbs. (They really get you moving don't they?) My husband has been the best thing that could have happened to me. He came at the perfect time. God has a plan for me and I didn't let PH keep me from fulfilling that plan. I could have been in bed feeling sorry for myself. That would have been easy. But I said NO! I got up and chose to change my life. I chose to seek alternatives because I refused to believe that I would need a lung transplant, that I was sick, that I would not survive. I will not be held down. God needed me for something amazing and I didn't want to let Him down. I am beating PH!



My Clean Cutting Board

PHighter Friday: Carole

Like most people with PH my journey began without my noticing it and it gradually built until my old life was no more, a new one was to begin.

After going for a walk with my husband I noticed that my feet did not want to climb the steps, they kept hitting the edges, it was just too hard to pull them up all the way to the top.  I began to find it difficult to walk up the incline to our house.  I felt dizzy and sick and my legs were heavy.

One day after walking through the woods it just got too hard walk the the incline back to my house.  Time for specialist advice.  I was fortunate enough to meet with a doctor who had heard of, though not seen, PH.  He advised my husband to take me home, back to the UK, we were living in Spain at that time. He said I had maybe six weeks to live!!!!

The despair hit me like a brick wall, and, of course, we Googled PH.  Not a good plan at all.  On the journey back home I cried because I could not dress myself!,  looking back I can't believe the despair and hopelessness that hit me.

I was fortunate enough to be met at the Royal Hallamshire  by my A Team, my angels.  I was told not to get out of bed, they feared a heart attack as the right side of my heart was five times the norm.  I was told I had many tests to get through but would be pushed to them all. I must not walk! I was immediately given a cannula in my hand and attached to a drip pumping  iloprost into my body at two minute intervals.  Then the tears began, too many to mention but I remember the professor sitting at the side of my bed and telling me I would need a Hickman line inserted into my body that would enable me to get out of bed and carry on with life as best I could.

Line inserted I went home after five weeks to begin a new phase of my life.  It was hard, the things I could do before I could not now do.  My husband  took over the roll of housekeeper,  he also took over the roll of nurse.  He changed my Hickman pump daily and made sure it was kept clean.  My fingers didn't seem to want to work any more and my brain had turned to mush! I remember one day thinking I would vac the house before my husband came back from the shops and the despair I felt when I realized I could not even push the vac around.  Then came a year of adjustment and yes, I will admit of self pity.  Why me, what life did I now have!  It was hard, so hard but after a year I was given more tests after one of my six weekly visits to my hospital and how can I ever forget the jubilation that I was to see that day.

My bed was crowded around with all my PH team. Beams were to be seen by all.  My specialist doctor was all but jumping with excitement when I was told that the iloprost had worked!  My heart was how the size of a normal persons and my right heart catheter showed it was now 38!  Now a new journey began, one without the Hickman line and one with oral medicines, ambrisenten and sildenifil.

It took three months for the change over to be completed and for my new life to begin.  Slowly my body adjusted to the new medication and slowly I began to climb out of the pit of despair I had been in.  Small steps, baby steps at first.  I was scared but began a regime of walking further each day, always with someone at my side as the fear of dizziness of  collapsing was with me but I persevered,  gradually I began to feel stronger, was able to do more, was able to help in the house, able to shop on my own, able to be my own person again.

Recently I walked my personal best on my six minute walk test of  720 metres.  Some days I can walk eight miles, though in fairness five is the norm, some days I can't do much, hence the roller coaster ride we are on.  At last I had joined the people who were responding, who were living with ph and not letting it beat them.

I remember at the beginning of this PH journey looking out of the window and seeing people walking, just walking and giving it no thought.  How envious I was of them and thought it would never again be me, now though it is.  I am not saying I don't get bad days, we all do but the good far outweigh the bad.  My mobility scooter has not been used very much in the last two years, it sits in the garage and sometimes, though rarely now it gets used.  Most times my legs do the walking!

My advice would be to push yourself a little more each day.  Don't set out to do a marathon, take small steps and find what makes you comfortable.  Listen to your body, if it says slow down then do it, if it says " I can do a little more" then do a little more.

Remember PH medications are changing all the time, research is going strong.  There will be a cure  for this and I believe it will be in my lifetime.  I trial anything I am asked to trial.  I want to be part of the team that finds this cure and beats it!

Stay strong in heart and spirit.  When the bad days hit remember it's a roller coaster, the good days will follow.  Remember we are a PH family and we have friends here who can relate to everything we are going through,  my journey began in a wheelchair but I have fought to use my body again.  Fight, fight , fight and keep a good spirit within  you, believe in yourself.  Most of all remember you are not alone, there are people here who KNOW exactly how you are feeling. 

You walk with friends. Xxxxxx

PHighter Friday: Maho

I guess my story starts in 2013. About May, I start noticing that I struggle going up stairs and hills. This gets progressively worse as time goes on. That year, I was doing my Masters degree, and had a place to do a PhD in microbiology once I finished it.

It all goes well in terms of getting started with the PhD, although I do notice that I’m struggling to keep up with people going up. However, I just put this down to a lack of fitness, which in hindsight was rather stupid - especially when a friend had said something’s not right!

In Nov., I finally go to my GP, who arranges an echo - 6^th Dec. There, I am told that the pressure in my lungs are high. I’m asked “Are you feeling ok?”, to which I reply “Yes”. This is another stupid moments in hindsight, as I knew something wasn’t right but I was not worse that day than I was around that time.

10^th Dec., I’m due at my Masters graduation. However, that morning, while getting ready, I lose control of my breathing. Call and ambulance, get taken to A&E. Having the information from the echo, they quickly realise that I probably have PH, and call the specialist down - I am really fortunate that the Royal Free has a satellite clinic with specialist doctors, nurses and a psychologist at my local hospital. Diagnosis and admission into intensive care followed, and I was started on epoprostenol and sildenafil. Two weeks later (Christmas eve!), I am transferred to the Royal Free to have a central line fitted so that I can continue the IV therapy at home.

With the teams and my mother’s support, I managed to slowly return to my PhD; I am lucky to have a supervisor who is very supportive. By Apr., I managed to return full-time, and I do think that if my supervisor hadn’t been as supportive as she is, I may not have been able to continue. Due to the line, I had to learn to use computer programmes to continue my research from another angle. In Nov. We completed a risk assessment so I have managed to go back to doing lab work, which is what I love. I’ve continued to improve, from being told that I should start the process for transplant assessment to being able to do most things. My mother is a therapist working with movements etc., called "eurythmy", and I believe this has helped me recover enourmously!

I still do have days where I feel frustrated with the whole thing, particularly the IV! I have had moments where I feel so stupid for not seeking help sooner. But I realise that I’m responding well to the treatment, and that even if I had sought help I may still be in the same position today; I have had excellent support from the psychologist, who has helped me realise that, helped me to organise all my emotions and so much more. I also recently fainted due to pushing myself too much around that time, which was a huge knock to my confidence as I felt that I was back to normal. Reality is that I’m ill, even if I don’t feel it sometimes.

One thing that will stay with me forever; the day I was discharged from the Royal Free, the nurse looking after me said “Be bold; if you want something to happen, ask God, because he decides what happens, not us.”. This stills means a lot for me to this day, and gives me hope that one day I will get well enough to not need the IV anymore.

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.