My story..

Well this is officially my very first blog post. I decided to do a blog as I have been inspired by other bloggers like Hannah Maggs and Zoella etc, and a fellow friend of mine called Stacie. I've linked up their blogs so you can take a look.

My disease is called Idiopathic Pulmonary Arterial Hypertension which is a very serious but rare medical condition and I seem to have been one of the lucky ones who was picked out to have it.

Idiopathic Pulmonary Arterial Hypertension (PH for short) is where the pulmonary arteries are much thicker than a healthy persons arteries therefore my heart has to work much harder to pump blood around my body and to my lungs; especially during periods of exercise or physical activity. When exercising, the heart beats more quickly to get more oxygen to the muscles and at the same time the pulmonary arteries have to expand to allow more blood through, but because my pulmonary arteries are thicker, they are less able to stretch. This means that my body and lungs are not receiving enough blood and oxygen because the arteries are much thicker than normal arteries and are unable to stretch to let more oxygen and blood through.

Because of this my heart works less effectively than your own or a healthy persons. The symptoms i suffer from are: breathlessness (mainly), dizziness, feeling faint, chest pain (particularly during exercise), migraines and tiredness.

So there's the information about my disease, now here's my story: before i got diagnosed in September 2011 at the age of 13 I was constantly going to the doctors as I was finding it very hard to breath when walking up hills or stairs and when doing exercise. I could never keep up with my friends when doing PE and it was rather quite embarrassing as I was the girl who always had to stop, take breaks, catch her breath and I always felt quite lightheaded and faint. My friends were angels though, always staying behind with me, getting me water, making sure I was ok. I guess it was an excuse for them to not do PE or to walk with me just in case something happened but I didn't mind. Every doctors appointment I had it would be the same diagnosis (asthma), the same treatment but I knew it wasn't asthma which everyone told me it was. Anyway, I took the inhalers and all that malarkey but nothing changed and no one listened.

A month or so later, on my birthday, a few days after we had left school, I was on my way to my friend's house to go out for my birthday meal. We all dressed up nicely and I was really excited to go. My friend Paige, lives 4 streets away from me so definitely not far at all, just a 5 minute walk and I'd be there. I left to go, and while I was on my way there I started to feel sick, dizzy and lightheaded however I continued to walk as I thought it would just blow over; plus I'd be there soon. I text my friend Megan to come and meet me which was literally only 10 steps away from Paige's front door and just as she came out I passed out. Everything stopped working, and because it had never happened before they thought I was messing around. I came around, got up and walked inside and there I passed out again. Yep, again. Not even a minute had gone by since I had last passed out. Paige rang my Mum and my Mum begged me to not go out however I did, only because it was my birthday and I felt a little better as well so in my head it was ok. My Mum continuously told me I looked like crap and needed to go to the hospital but I did not listen, I never do really.

Anyway the next day I was fine, it was like nothing had happened. Mum had no idea what to do, she thought it may have been the heat but took me to the doctors anyway. They told me that they couldn't detect any changes except a little murmur in my heart but apparently that's quite common. The doctor, a man I recall, told me to book an ECG and so we did but they had no space for me for at least another month.

I returned to school in the September of 2011 and on the 3rd day, the Thursday, I decided to go to town after school with a couple of my friends. When I came home I was absolutely knackered and had no energy at all to move, so I took myself to bed. Well it wasn't really bed because I was so tired I could not climb the stairs as I had a bunk bed so I slept on the floor. Lol. My Dad was on shift that night, he's an Emergency Care Assistant for the ambulance service, as he was finishing his shift my Mum asked if himself and his crew mate Lisa could pop round and take a look at me. They did so and my heart rate and blood pressure was completely off the scale, I also had blue mottled legs so there was also another cause for concern. I went to A&E at The Great Western Hospital, in my Mums car as I refused to go in the ambulance, A) it's not my style and B) it's embarrassing. 

I waited hours to be seen in A&E and they kept me and my Dad up till stupid o'clock and nothing happened. I spent the night there and the next morning, the Thursday, I was transferred to the children's ward, they took my blood, hooked me up to a monitor, took an ECG and did an echo gram which is pretty much a baby scan but on my heart. They had no idea what my insides were doing, especially my heart but they kept me in anyway. I went for a chest x-ray and still no conclusion. In the end I stayed in and got transferred to Bristol Children's Hospital on the Friday. I continued to have more and more tests, pretty much the same ones and they still had no conclusion as to what was wrong with me. They kept me in till Sunday night and that evening I got transferred by ambulance to Great Ormond Street. Again, that night, I was kept up till stupid o'clock in the morning having my bloods taken, god know's why it took so long but it did.

For a week and a half I was constantly having tests to try and find out what was wrong. When they finally found out what the problem was I felt so happy, no more hospital trips, no more tests, just a few tablets and an inhaler and then I'd be fine again. But that unfortunately was not the case, having my disease meant coming to regular check ups, taking constant medication, having oxygen when needed and setting up my medication and taking it all by myself. 

There were 2 other treatment options as well as tablets to take everyday. The first option was the Hickman Line, which gives the drug Epoprostenol (Epo for short). This drug would help make my arteries thinner so more blood can get to my lungs, however I said no straight away because it was all so new and it seemed like a little too much for me; plus it creeped me out big time as it is an intravenous line, and I would be connected to a pump 24/7 and I would not be able to do certain things like swim and dance. It just seemed all to much to me at my age and at such an early stage of treatment. The doctors however never stopped going on about it, trying to push me and push me to have it but I just wouldn't so in the end we settled for the Nebuliser, treatment option 2. The Iloprost which would be inhaled through the nebuliser has the same effect as the Epo, making my arteries thinner so more blood can pass through; however this process was less reliable and not guaranteed to work as the drug would not stay in the body for longer than 70 minutes and I was inhaling the drug every 2 hours.

They sent me home with a Nebuliser which was quite small but heavy, I would have to inhale the drug Iloprost every 2 hours daily from 9am till 10pm. It took a while to get used to and to get into the routine but I did and I was allowed home. As well as the nebuliser I was taking 2 different types of tablet a day; Sildenafil and Bosentan. I would take two Sildenafil tablets four times a day and I would take one Bosentan tablet twice a day. Every month I was to go to the hospital to have my bloods taken just in case anything had changed they could see the difference. I had 2 weeks off school after my hospital tour from Great Western to Bristol Children's Hospital to Great Ormond Street. I had met some lovely nurses and doctors that I will  never forget. After being in hospital I had check ups every 2 weeks for a few months and then they finally moved to every 3 months.

I managed on Iloprost through the Nebuliser for a year and 4 months. In 2012 I had an appointment for the 20th of December. Ever since summer 2012 I had been feeling more tired and was experiencing more breathlessness, my parent's highlighted this to the doctors and they kept an eye on me I suppose. I was dreading that appointment because I knew what was coming, I don't know how but I just knew. I had all my tests and went to my consultation. I was 14 years old. They basically told me I was getting sicker and that the Iloprost was not doing it's job for me anymore, I was deteriorating and there was nothing they could do except put me on Epo through the Hickman Line. I agreed to it and set a date for the 8 January 2013 to have the operation. I agreed to it because if I didn't have it would be the end and I wasn't ready for the end, it was something I had to do, for myself and for my family or I would've died and I certainly did not want that. 

When they explained what would happen when I was on Epo I didn't listen to all of it, only some; I would feel rubbish after the anesthetic and I would feel drowsy, I would have stitches that need to be taken out 3 months after, I could possible suffer from headaches and sickness for a few months until my body got used to the drugs, I could not get the site wet so no swimming or showers or baths (I had to wrap my upper body in cling film to stop the water from getting to the site when I washed), medication to be changed every 24 hours which only my Mum and Dad can administer because you have to be trained as there are lots of measurements to do etc, I would need to be able to work the pump in case anything went wrong and know what to do in an emergency for example if my line got pulled out. I understood and was preparing my self for the operation and change in my life so you could say I had a pretty crap Christmas.

On the 8th January 2013 I was admitted to Great Ormond Street and went down for surgery. My Hickman Line was put inside me and I was all stitched up. It was about a 4-6 hour operation. When I came around from the anesthetic I was very drowsy and had no clue what was going on. The next day I was stiff and could barely move. I needed assistance to get to the toilet and back and I was walking with a hunch back because if I straightened my spine the line would pull which was painful and lots and lots of blood would pour out of me where they inserted the line. I had one bath in the 9 days I stayed there and my parents went under and intense week of training for my medication. Once my line was inserted and I was on the Epo I suffered from migraines and sickness which I was expecting but I did not expect my site to bleed near enough every day. My family came to visit me and keep me company and on a few days I managed to go to the canteen. Everything was going really well and I was allowed home.

After about a month or so of being on the Epo I started to develop this very harsh red and blotchy rash. Mum and me thought I was having an allergic reaction and went to A&E; they gave me an antihistamine but it did nothing so they let me home, what else could they do? This rash continuously came up every single day and I looked like a fried tomato with extra tomato sauce on top lol. It turns out it was an Epo rash so the cause was my drugs, and from this day forward I continue to be a tomato. The rash never goes and it's the one thing that get's me down because it's so disgusting.

Since then I've been doing quite well, I had a transplant assessment last June and this October I've asked to see them again however I'm not quite sure if I want a lung transplant yet. Maybe in the future but not right now. Ever since i've been diagnosed i've had to attend check ups that have consisted of MRI scan, sometimes a CT scan, Echo, ECG, 6 minute walk test, sometimes the exercise test and a consultation. There was no sign of improvement until March 2014 and it turns out my heart is pumping a bit more blood out than it was before and I've stabilized, I don't feel much better but as long as my insides are getting better I suppose. I'm still on Epo through the Hickman Line and I still take two Sildenafil four times a day and one Bosentan twice a day. I've recently been put on diuretics to get rid of any remaining fluid in my body and they seem to be working. I still get quite tired and sometimes I need to use the oxygen because I find it hard to breath but apart from that it's still quite hard for me to get out. On wards and upwards though.

Thank you for taking the time to read my story and I'm sorry it's so long.

Erika X 

P.s I'm sorry if there are any mistakes, I've read it twice now and I can't see anything so fingers crossed!!