Jay is a young man that has already shown he's not going to let his diagnosis get the better of him, he'll grab it with both hands and tackle it head on. He's an inspiration to all.
My Journey, By Jason
When I think about PAH I think of it like my favorite past time fishing, like we have all heard if it was meant to be easy it would be called catching or collecting not fishing, living with PAH, up to date, has it good days and its really bad ones and like fishing you have the good and the bad you have no real control you just take it as it comes and get on with it, but anyways heres my story the short version and it goes like this...
It all started in october 2011, as a 26 year old second fix carpenter and Ambulance officer I was reasonably fit and healthy I could work from 7 til 7 run up and down stairs all day long and go out every weekend and it would not bother me at all until that day in October where I noticed that I was tired and felt like I was starting to burn out, after a week of feeling rather crappy I decided to see a GP about my symtoms, I wasn't too worried about it after all I'd always had a heart murmur, liver and anemia issues and I was always adjusting my diet to suit my lifestyle since the age of 16 but anyways after a ECG, bloods and a echo my doctor mentioned that I was anemic again and probably needed to look at my diet again, this was nothing new due to the fact summer was comming up and "thus is the season to be making money" so simply the harder you work your body suffers and you need to adjust your diet to suit, this made sense so I just ran with it.
After a month I had not gained any energy infact I felt like things were getting worse I started to notice that I was getting short of breath during some activities, climbing stairs was a 2 stage task, first climb to the top, second stop to catch my breath, maybe I was just unfit? Anyway I decided to go back to the doctor who took some more bloods and discovered my liver functions were all over the place, at that time I became an out patient to Flinders Medical Centre (FMC) gastro and heptology unit for further investigation but like most things with public hospitals the waiting list was extensive, it would be 4 months before I could see the specialist, my doctor was under instruction from FMC to keep an eye on me which meant fortnightly bloods etc but my liver functions never seem to improve.
Things remained steady until the end of January 2012, one day when I was at a concrete pour I started to notice that my breathing was getting worse during activity and at the same time I was getting what I thought was a head cold, I'd let it go for about a week but the head cold did not improve nor did the shortness of breath, once again it was back to the doctors, it turned out I had a nasty sinus infection most likely because of all the saw dust and normal dust I had inhaled during another job and the SOB was just a part of it so I was sent home with antibiotics and told to have 2 weeks off, after 2 weeks nothing had changed I still had the sinus infection and the SOB was still persistant but still not overly bad, so once again I went to the doctors who once again sent me home with anitibiotics, a futher 2 weeks moved on by, the sinus infection was still there but the SOB was getting really bad fast! and I noticed my belly was "poped" the SOB got so bad that travelling to the toilet, picking stuff off the floor even getting out of bed was a major task where I had to almost plan how I was going to do it with out running out of breath!! I remember it being Monday the 5th of March when once again back to the doctors and once again sent home with stronger antibiotics and steriods, I only lasted a day when my I noticed my lips started turning blue even at rest and I could feel my heartbeat in my head and I had confined myself to the couch till in the after noon when my sister came around told me I had to get back down to the doctors because I looked like death, so once again back to the doctors this time the doc didn't waste anytime, he took an ECG and had a hard listen to my chest he also noticed that my belly looked quite large for a slim fit guy, he then proceeded to say "you wont be going home or to the local hospital you need to go to FMC in Adelaide, there's something wrong here and there's nothing we can do for you here I'im sorry but you are deteriorating fast." Within 1 and a half hours I was at FMC in emergency, this is where my "hell" begins.
Getting through emergency was a nightmare my body decided to start going into venious shut down, for those who dont know this is where your body reserves blood for the most important stuff like the heart, the brain and other organs to essentially survive so veins in your arms and legs become flat and useless for IV access. This resulted in a large bore canular in the neck, first of many invasive and unpleasent experiences. After a lot of blood tests, blood gasses, CT scan and an echo I was told that I was in CCF, congested cardiac failure, also known as right side failure and that my right heart was so enlarged that it had filled up its entire thorasic capacity and the artery to your lungs looks like its struggling. Not to mention my observations looked aweful, sats 95% on 10lts per min, heart rate 130, bp 160 systolic etc. it was a real mess!! After a long time in emergency I was sent out to ICU where I spent the night hooked up to a 12 lead monitor and was observed evey 15mins with out fail, no rest for the wicked. The following morning I was moved out to the Resp ward with all the lung cancer patients, when I arrived I was greeted by a large group of resp and cardiac specialists who then said to me "we believe you may have a rare condition called PAH" straight away I knew what it was, due to the fact my mum also has PAH. Now the chances of 1 person in 1 family getting PAH are like a million to 3 with a 50% greater chance in females, but 2 people in the same family is just...well almost un-heard of also not to mention that I'm a male under the age of 40, some people call it special I just call it unlucky!!! During what I nicked named "hell week" I was put through all the tests under the sun, echo's, Right heart cath, 6MWT, many, many blood tests and blood gasses, etc., etc.
By the end of the week they had their first true prelim diagnosis which was Idiopathic PAH class 4, which we all know means well you've got it but no bloody idea why but its bad. I'm sure lots of you have got this before, but my cardiologist is a fighter and decided to get evey type of specialist inside the hospital to come and test me for everything else that may cause PAH. This happened during week 3 during my stay in hospital, but after the first week and after the results from the RHC came to light. My pressures off echo when I was admitted was about 70mmhg and after the RHC they had climed to 110mmhg, I was immediately sent to the Cardiac Care Unit and started on Epoprostenol. At this stage my doctor told me in his words that I was "a spiralling vortex of death and as much of the flolan shit we can get into you the better." I spent the next 6 days with my head in a bucket with serious neausea, even ondansetron, a very stong antiemetic usally used for chemo patients stuggled to keep it under control as they slowly raised the drug dose from 1.6 ml per-hour to 5ml per-hour. During this time my doctors, which I had and have alot of them, believed that if the nausea didn't get me that the PAH would catch up to me fast, but I refused to let it, I refused to die. I by no means have a hero attitude but I was warned that I should let my family and friends know that I may not come out of this alive. This was a sobbering moment. I guess this is proof that you can fight this if you want to. It's sure as hell not an easy thing to do, trying to stay possitive after being diagnosed with a life threating condition, but also being told you may die is a real trying experience. All I can really say, you can't put a price on the support from family and friends, they wll always keep you going.
After all the "fun" of the first 2 weeks came all the other specialists, I won't list them, mainly because I can't remember their job titles exactly but the tests came on thick. After a heap of blood tests they decided to put all their focus on to 3 concerning issues. First being a disease called wegener's granulomatosis which is an auto immune connective tissue disease which usually affects the sinus, lungs, kidneys and the vascualar system causing inflamation. It's once again a rare condition and even more rare is that if its primary to the PAH. They discovered that I had a high count of the antibody in my blood and a hole in the septem in the sinus track. To test for this they needed a sinus biopsey and a PET scan with a Glucose radioactive dye, the biopsey came back as border line and I'll have to go through the very invasive procedure again in a few months time but the pet scan came back clear as day. They also found something called polymyositis, another connective tissue disorder which affects the muscles and causes them to waste...so i was told. Turns out this also was border line but will be watched over time to check if it progresses. The second focus was clots. When I first arrived at FMC I had a CT scan which showed web like structures on my lungs. They believe it was one of 2 things, either artifact or scaring from a PE, so it was off to nuclear med for a VQ scan. Turns out I've got a clean bill of health on my lungs even after years of smoking when I was much younger, it was like I never did anything bad at all. They also looked at my legs for DVT's via ultra sound that turned out clear too. The third thing was the congenital factor due to the fact my mum had the condition, is there a chance that there's a defect in the DNA strand, unfortunately there is no test nor is there a treatment if they found out it was. On the brightside they would be able to watch my progress and compare, even that comparing one PAH patient to another is dangerous game and no doctor should ever do it!!!
My 4th and 5th weeks and 2days I spent in hospital was mainly for observation. During the first couple of weeks I responded badly to every treatment, my body was in major shock, but as the weeks went on my body got used to medications and adjusted. I lost all the retained fluid, all 10kgs of it, my liver functions are returning to normal, right heart is no longer congested and has gone down in size by about 50%, I can now breath a lot easier and walk further, rougly 600m+ in under 6mins compared to 75m in 4.5mins when i had to sit down. I'm currently on 3 PAH drugs, Epoprostenol via the usual CVC (PICC line type), ambrisentan, sildenafil and warfarin as a preventer and every week so far my pressures are droping. My PAH clinical nurse has told me "you came in as a very serious class 4 patient and left a class 2 to 3 patient and you have been through hell and never gave up even when you were nearing "the otherside." I am first and fullmost very thankful and consider myself fortunant that I survived, as I was told on my discharge day (april the 11th my bday :D) "not many people who come in as serious as yourself usually survive, most patients surcumb very quickly." I understand that PAH is no walk in the park and its a long journey but I look at this as my second chance and that I should make the most of it and try to make a diffrence to others who are also going through this, even that I'm relatively new and help educate non PAH suffers about what's going on "even though i dont look sick." I just hope the bits 'n pieces I've told of my story encourages more people to come out and tell their story especially the fellas, nothing against the ladies, but as males we are a stubborn bunch and refuse to be "sick" but here we are.