1 day ✦

Everyone loves a picnic in the summer ☀.

Myself, Dan, my Dad and our 4 dogs went to Shaw forest for a picnic. The weather was absolutely gorgeous and the boys loved having a run about. We had lots of goodies to tuck into and the boys had a bowl of water too keep them going. It was quite quiet at Shaw so we were able to relax and just enjoy the weather. Boo decided to roll in fox poo which wasn't amusing at all and he absolutely stunk but apart from that and the boys being covered in mud, we had a fabulous afternoon.

Some of you that have me on Facebook would have seen my recent status about 'Health Care at Home' and my medication delivery. Needless to say, they are the worst company ever and have no idea how much I need my medication.

Last Thursday I was due a delivery off all my medication which comes in 3 massive boxes, between the 3 boxes I should have: white needles, green needles, sterile gloves, antiseptic wipes, epo, 50ml syringes, 20ml syringes, 10ml syringes, cartridges, smart sites, extensison lines, batteries, dressing packs, saline, alcohol solution, dressings, chlorapreps and sometimes some other bits and bobs when I need them. I should have a months supply of meds within the 3 boxes and nearer the end of the month my Mum does a stock check, where she counts up how many of each thing she has left and gives the figures to 'Health Care at Home'.

On Thursday, myself and Dan stayed in all day to wait for my delivery that never turnt up. Now that may not seem a big deal to much of you but it is to me, I could have spent that day enjoying myself and getting out there but I did not as I was expecting a delivery. Of course I don't mind staying in but when it doesn't turn up I do mind and it's a different story. Anyway, my Mum contacted them saying that my delivery of medication had not arrived in which they told us that it had not been put on the van. Oh how wonderful of them, not like I'm terminally ill and need my meds. My Mum reminded them of that too.
The manager of the company rang and said that she would get my meds to me the next day (Friday) before 12, this was decided because my Mum had appointments in the afternoon which she did not want to cancel unless she really needed to. She also said that when the delivery was just about to leave she would ring to let my Mum no so that she had an approximate time of delivery, that's what they called it anyway. My Mum didn't get that call till 6pm at night, my delivery turnt up at 8:30pm.
My Mum went through the box and not everything was there, well only the Epo and a couple of other bits and bobs. Mum emailed them saying that everything was not there and that it had only just turnt up, they said they would send another delivery on Monday after 6pm, again this time was decided so that someone was definitely in and that my Mum had done my meds by then so they wouldn't clash.
On Monday I was supposed to go to the dentist, leaving my home at 5 to 9 but they cancelled due to the Dentist being unwell. at 9:00am approximately a delivery man knocked the door with my meds and I signed for them, not knowing that they weren't supposed to be coming till 6. Mum came home and was fuming at the fact that they had delivered it at 9, luckily for them I was in, but if I was at the dentist what would have happened? My Mum said this to 'Health Care at Home' as well as the fact that everything requested and that wasn't in the previous delivery wasn't there.
My medication has to be made up each night by my Mum/Dad and it HAS to be in a sterile environment; all surfaces wiped down and scrubbed, sterile gloves and needles etc. However, 'Health Care at Home' do not seem to understand that. Mum had requested a box of small gloves, the usual ones which we have had for the past year and a half which are sterile, and they chucked un-sterile medium gloves into the box. UN-STERILE. ARE THESE PEOPLE OK.
What makes this situation worse is that she spoke to a lady at 'Health Care at Home' who said she would personally pack the delivery box so how could anything be wrong or missing? She explained this to them in which they replied saying "Delivery this morning was a back order, meaning one of previous deliveries had no stock for one of the items, so it automatically goes through our system to be delivered as soon as we got stock." and another email saying "**** raised order for you as you spoke to her. I've just checked the order and everything was as requested. About them purple gloves must have been picking error from warehouse.", but we were told **** was going to pack the delivery herself?
No one even informed myself or my parent's of the extra delivery at 9am. Anyway, we were expecting my usual delivery that was supposed to of arrived last Thursday to come yesterday evening at 6pm yet it did not turn up. Mum asked them why that was and they said that no one was in when it was delivered. Seems somebody's telling porkies as I was in all day, and all the family was in in the evening. The delivery was definitely not delivered, no one knocked the door and we were all in. Mum told them this and suddenly there story changed to "2 major accidents on M1 therefore driver lost 3 hours. Driver called our depot and advised about it and ETA given 1.30am, our depot advised to return as it being out of social hours." Now, I don't mind if there was an accident because that can't be helped BUT if they had just been honest and rang us to let us know it wouldn't be arriving then there would have been no more problems; however they didn't therefore there is more problems. After a while we were told the delivery that was supposed to come yesterday evening should be coming tonight after 6pm so we shall look forward to seeing what arrives, if anything. They are such a wonderful company.

Anyway, onto a better note, it's my birthday tomorrow!!!!!!!!!! Ahhhhhhhhhhhhhhh I'm so so so excited, I can't wait. I'm off our for lunch with my Grandparent's tomorrow and in the morning I THINK my Dad is bring me breakfast. Wahey. My birthday party is on Saturday too and I can't wait to see everyone's fancy dress. It's going to be fabulous yay.

1 FLIPPING DAYYYY.

Well 7 hours to be exact.Erika X

hm

I haven't posted in a while which is really bad but I've been so busy this week which is a very rare occasion for me. I've enjoyed this week so much even though it's meant I've slept quite a majority of the time that I've had to spend with Dan or my family.

I'm not really sure how to write this so it's going to be a bit like a list, I don't know if it's going to look right but I might as well try. On Monday Sophie came over to do my nails because they were atrocious, she did shellac on them for me and I'm in love with them. Tuesday I had my 6th form induction day, to be honest it was pretty boring but it's made me really excited for the future, especially September. In the afternoon Mia came over with Baby G, he's so adorable and cute. He sleeps like a turtle on your legs and he always seems to be asleep when I see him which I don't mind, but i'd actually like to see his eyes open sometime, but it's better than crying I suppose. Myself and Dan went to the stables in the evening with Dan's Mum Sharon and my Mum. Wednesday I met up with Mia, Paige and Hannah; we sat in Costa and had a lovely time with Baby G, until his nappy needed changing and we all crammed into a baby changing toilet for god know's what reason. On Thursday I finally got to spend time with Dan, we had tea with his Mum and I have no idea how she managed it but there was at least a 1/4 of tomato sauce all over her. In the evening we watched a film, and I fell asleep at about 9, Dan woke me up at 11, and then I went upstairs and went to bed at 11:30. Dan's used to me bailing out on him with films and TV series we watch, sometimes I manage to stay awake but it was just not possible. On Friday we had a nice chilled, relaxing day and I went to Hannah's in the evening with Mia and Paige for Pizza and board games. I cheated in nearly every game in cluedo and we stole answers from Mia in which she will only just find out by reading this, so sorry babe, it was kind of obvious though, we laughed for ages and couldn't stop but you still managed to not find out. Bless ya. I've had such a fabulous week and for once I've sort of managed to get through it. All though now I want to hibernate because of it and all the stress I'm under. 

On the upside it's my 16th birthday in 11 days, and i'm so excited, as well as for my birthday party too. They can't come quick enough. Ahhhhhhhhhhhhhhhhhhhhhhhh I'm so excited.

Erika X

Stacie xoxo

I saw my very good friend Stacie on Sunday, she was mentioned in my very first blog for being one of my inspirations. Like myself she has PH but unfortunately as well as that she has 3 holes in her heart. One of the reasons me and Stacie get on so well is that we can relate with so many things other people can't and she lives 5 minutes away which is a bonus! Especially when we both have such a rare disease. Like myself, Stacie suffers all the symptoms I do and on Sunday we found an effect that made our rashes' look absolutely horrendous, way more than usual, and we sat and laughed at it because it amused us so much that it could look a lot worse.

When we see one another we tend to sit and rant about every single little thing in life and very rarely do we actually have a normal conversation (I think it's because we don't see each other much and when we do we have so much to rant about that other people wouldn't understand). There are many reasons I love seeing Stacie and one is that I actually feel normal around her and that everyone else is not normal. Weird but it works for me. 

This blog post is very short and sweet compared to my others.

Enjoy.

Erika X

☀Wow..

I am absolutely shocked at the response I've had. I do not know what to say except thank you for taking the time to read it and to some of you for sending me messages.  

When I decided to write a blog I was absolutely terrified of not getting any views and no one wanting to know but that's not been the case and I'm so confident with it now so thank you.

Since my first blog post I've had a few people ask me questions and it occurred to me some of you may be wondering too so I thought I'd mention it.

• Is it a life long thing?
  Yes it is a life long disease and it is terminal. Terminal means that it is predicted to lead to death slowly and it is incurable.
• Is it scary?
  Yes the whole situation can be very scary but there is nothing I can do about it so I just have to keep going. 
• What's scary about it?
  The unknown side of it, you never know what is going to happen like with most things in life but when it is something to do with your health and life it seems to me that the unknown is 10x scarier. One day I could be fine, able to get up the stairs and go out and enjoy myself and the next day I might not be able to get out of bed, or my legs are painful and I can't breath very well. Plus, the not knowing when the end is, is the one thing that gets to me badly, I'm not exactly doing everything I want to right now as I'm not able to and I do not want to die anytime soon but you never know and I try so hard to say to myself "Every one dies at some point" or "At least you've made it to 15" but I know that even thought I'm still lucky to be alive I will never get to experience life like everyone else but 'you never know' and that gets on my nerves because I want to know.

I hope I've answered your questions and if you have any please feel free to ask, I'd rather you ask than assume and read a bunch of crap on the internet. 

 Erika X

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!!