Ok, this blog is long overdue... now though I don't know where to start or how truly shit its going to be to write. If I'm going to do this whole 'book thing' though I need to address my early years and some tougher topics about who I am and why things may have gone the way they did. I offer no apologies to how self involved this will be again but its the opposite to my last blog, in my last blog I was positive and happy this is going to be negative, dark and quite upsetting for me and my close friends who will read this I'm sure. I have also come to the realisation that I have rarely stopped and thought about my disability and even less often read up on it or even simply 'googled' it until today!
Ok so I guess we shall start at the key part of who I am, even though I have always argued it doesn't 'make' me its just a 'part' of me but its a massive, daily affecting part of me and I never have a day where 'it' doesn't cross my mind or affect my day.
'It' of course being my heart condition;
Marfans Syndrome, genetic disease, "marzipan syndrome" as my closest boy calls it... whatever I have called it in the past, that I was born with and will continue to 'suffer' from for the rest of my life. I am trying to think of the easiest way to describe what is it and the most simple way I can put it is:
An inherited connective tissue disorder with characteristic skeletal, dermatological, cardiac and ocular malformations and defects. Estimates indicate that approximately 1 in 3,000 to 5,000 individuals have it.
There are more than 30 clinical features that are variably associated with the syndrome, some features or characteristics I possess I will describe now:
Cardiac CharacteristicsI have an abnormally large heart and very defected aortic route (the main blood vessel to and from my heart) its weak and stretched quite thinly, and it beats twice as quickly as the average heart to get blood round all of my body. The Separation of the layers of the aorta can cause it to tear (aortic dissection) this terrifies me, as well as a possible future heart transplant.
My heart is a huge burden on my life, I take medicine (beta blockers) every day and have since I was about 2 years old and that's not really an issue, its the constant fear of a heart attack when I have regular palpitations (my heart beats very quickly for a short or long period of time)or my chest feels tight. I don't know what a heart attack feels like and I don't want to but even a little normal 'rush' scares me into what it might turn into.
Recently on my way to work I had a 'palp' and it terrified me, it also hurt so much I had to sit down, ring mum and go home to rest. I couldnt work and I cried and it was horrible. They dont happen too reguarly though *touch wood*
I'm also not meant to do too much physical activity and in school I was excused from P.E and I wanted to do it! In actually fact I CAN do exercise and I have done a lot to get down to my current weight but I have to pay attention to my hear and my pulse a lot. Sometimes I have to have a break and its just about managing it but again that's a shit limitation I have to put up with. Walking is even a bit difficult sometimes, I don't walk particularly quickly, much quicker then I used to be though, but yeah I'm slow. Sometimes walking can hurt my back and my balance is shit and I stumble quite a lot (also due to Marfans.) This particularly stresses me out at work because although my boss knows about my heart condition he regularly tells me to speed up and I cant be in a job that daily reminds me of my limits.
Ocular / vision CharacteristicsI have very bad eyesight. I can see very little without my extremely high prescription glasses on but this isn't the worst. Its shit, don't get me wrong, that I struggle to read text on my TV or I have to hold books crazy close to my nose, but the worst is the Marfan related issue. Because of my Marfans both of the lenses in my eyes are shifting. They are dislocated and moving to the right, so at some point my vision will be much worse and its like I'm waiting for that day, twiddling my thumbs. I have two options when that day comes, I can either sit it out until it moves completely out of my line of vision and get corrective glasses or have an operation to remove both my lenses while its in the midst of moving and either have fake lenses inserted or get corrective glasses.
Atm my main issue is the amount of floaters I have in my eyes. Floaters are little black dots or squiggley lines that race across my line of vision constantly and people with Marfans experience a large number of floaters and there is nothing you can do about them. They piss me off, I often mistake them for wasps and wasps scare the shit out of me so I scream and turn around for no reason and look like an utter crazy. I also experience a fair few 'black outs' I call them, where I stand up and blood doesn't make it to my head quite quick enough and I can see for a few seconds and feel slightly faint.
The other day I had my 1st driving lesson and although my Doctor told me I am fine to drive with my vision atm I struggled to read the 20ft away license plate I needed to. It was horrible I felt like I had a disability that day and I don't often feel like that. Hopefully when I get slightly stronger glasses it will be fine. It would be shit if I couldn't drive, I have my heart set on it...
Aesthetic Characteristics:People with Marfan's are typically tall and thin, with long limbs, and long thin fingers. If anyone knows me this is me to a tea. Both my arms and legs and really long, my fingers and toes regularly get some sort of comment and I'm tall. I get commented on my height often and its usually friendly banter but if I wasnt tall it wouldn't happen now would it? lol. I'm a bit apprehensive of loosing too much more weight for the fear of looking 'lanky' or like a 'daddy long legs' (kids can be really mean) it does play on my mind a fair amount as I get slimmer I seem to be taller too...
I think the shitest thing about that is my choice not to wear heels, due to being abnormally tall already and my ankles are not strong enough to wear them. Yeah it is a choice, I could wear heels but I think they would look silly, they wouldn't look feminine like they should, whats the point in heels if they don't look girly? And most highstreet shops only stock shoes that go up to a size 8 and I'm a 9 / 10... it just doesn't seem fair.
I also have the Curvature of the spine (scoliosis) characteristic which personally is the worst I think, because it is the one that affect me ever single day. To stand up for a long time is truly uncomfortable its better then it ever was to be fair, in high school I used to be in such pain I would leave lessons just to go and lie down in the medical room. Lying horizontally was the only way the pain stopped, I remember taking paracetamol every 3 hours just for my back pain. It became so bad in my 9th year that I had to have a 'special' chair for some lessons and I HATED that so much, but it felt better so I did it. Nowadays its only really a problem after a long shift at work, too long shopping or on an uncomfy chair. I think the truly shit bit about my spine is it always being a worry, I look for possible seating wherever I am, in case it starts to hurt and I'm caught short without painkillers. I have two instances to tell you about that where particularly difficult and I could do nothing about them;
1, was when I went to Bham Academy in March to see my fave band with my boyfriend and I don't know if you are aware but there is very limited seating in smaller music venues and its such a long time between the supporting band and the main event. In this instance there were two support acts and it was also really crowded and warm so after fighting the pain for ages I had to sit on the floor. This was shit. Its not a big deal per say but it made me very aware of my limitations and I felt embarrassed and that perhaps my fella was embarrassed a lil bit too, cause it just isn't 'normal' to sit on the floor!
2, was when a friend of mine had a house party and there was not enough seating, like there usually isn't at house parties! Anyone else would maybe moan a bit but deal with it I guess but I cant. I asked my boyfriend if we could leave and he did the worst thing he possibly could of done but I don't blame him. He wanted to stay longer and said I could go on my own, making me feel like a bit of a freak and a burden on him, eventually he agreed to leave but I didn't want to then, I didn't want to pull him away from his mates and it was at a time when his mates were very busy far too often, so I had that on my shoulders as well. It may not sound like a problem really but I cant stress enough how much I HATE letting my body control my life and actions.
Another horrid part of Marfans that is noticeable is that the Chest will either sink in (pectus excavatum) or stick out (pectus carinatum) and mine is the second one, my ribs and chest stick out a fair bit. Its also referred to as "pigeon breast" which is the best way to describe how it looks actually, but I hate it, I HATE that my boobs stick out only slightly more than my ribs and one side of my ribs is much larger then the other!
Other things I have are my abnormal joints and the flexibility I possess (Im double jointed) my knees are constantly popping out of place and back in again which hurts like a bitch, I have awful balance and my ankles can give way sometimes and my overcrowded teeth. I really hate my teeth and what I hate more is there is nothing I can do about them. I wasn't allowed braces as a child because of the Marfans, I must be the only kid who WANTED braces! But they said no because of possible infection and that could get to my heart rapidly and be fatal. So instead I have way too many teeth, they are crooked and ugly.... thanks Marfans. lol
Possibly the most life affecting issue about Marfans is pregnancy or in my case the lack of it. During pregnancy women with Marfan syndrome are at significant risk of aortic dissection, which is often fatal even when rapidly treated and each parent with the condition has a 50% risk of passing the genetic defect on to any child. Boys get it much worse then girls too apparently so its a 50.50 chance anyway but if its a boy (also a 50.50 chance) then it far more likely and they would have severe Marfans. After attending recent genetic meetings however its is possible apparently to have a test to see if a child is 'infected' with the Marfans gene but then what if it is? Have an abortion just like that? Hmm? And yeah I could have a C Section and not do the whole pushing thing and then my heart wouldn't explode necessarily but it isn't that simple, just bearing a child puts a lot of strain on a heart and 9 months is a long time of strain.
Now this is life altering stuff but luckily in my case I found out when I was little it wouldn't be in my "best intentions to fall pregnant" I think were words passed around the Doctors room. So I have grown up with the intention to not fall pregnant, I do not want to die if I have the choice and giving birth / carrying a child may do that and I also don't want to bring another 'Marfansian' into the world if I had the choice, I just couldnt. And as of today that is still my intention and because Im used to that fact it isnt too shit. I mean, it is, it should be my choice but its not and thats that. So I fully intend on adopting and my boyfriend knows this fact and accepts that... That is however a very shit conversation to have. I feel bad for my mum too, to never have grand children I think that upsets her but she will never tell me so.
Since I was a baby Marfans has kind of dominated my life. When I was born I was premature and so ill I had to be kept in a incubator type thing for 3 weeks. I had glasses at the age of 3, quite often embarrassing geeky ones my mum chose... I had 6 monthly heart and eye check ups at local hospitals for years and then they turned into yearly check ups and I still have my heart check ups now, I had one recently and everything was fine thank god. *touches wood* I dislike them though, I know I need them but I feel like an experiment every time, being hooked up to wires and its foul when they turn the ECG machine volume on and I can hear my heart beating and so abnormally. I also have to be topless and when its a male doctor it displeases me :(
The main disadvantages of Marfans are: Pain in the joints, bones and muscles. Undue fatigue, shortness of breath, heart palpitations, racing heartbeats, a horrid smile, my eye sight, constant fear of dying suddenly or young, never experiencing a child of my own and my regular pins and needles, cramps, cold hands and feet linked to Marfan's syndrome because of inadequate circulation.
Advantages of Marfans, erm silly things like I get DLA monthly £200 for the rest of my life is kind of a sweetener I guess and I will get a car with the motability scheme kind of for free except for petrol... I get free prescriptions but then I have to have heart pills every day forever or I will die so its needed. I also kind of like being tall, sometimes and it means I have a fast metabolism due to my heart beating faster... Honestly though, I would rather not have Marfans. Oh and apparently Abraham Lincoln had Marfans or something very similar... lol
Its difficult though, I don't feel like I have a disability, I do, but its not obvious like others and it doesn't seem that severe. I mean I live a considered normal life and just have a dodgy ticker, or that's how I like to think of it. I feel quite shit when I sit here and moan then see someone in a wheel chair or someone that's blind, I feel like a fake a bit and that's silly but I do. Though I don't really do the woe is me thing, I know when something is shit and I just deal with it tbh.
I also don't really have anything mental going on, I'm quite normal in comparison to others and I know how lucky I am in that way. My mum is pretty insane, not actually but she is very insecure and doesn't like herself at all unless she has a boyfriend and well, its complicated, lets just say she is mad as a box of frogs and I struggle to deal with her.
I think I hold it against her that I don't have a dad and I hate that, I hate that I don't have a dad but I also hate that I blame my mum for that, but I kind of do... I often think who I would be if I had both parents and a 'normal' upbringing.
She recently told me that she feels guilty for me and my Marfans as she was the one to give birth to me and she didn't deal with it very well... that was a shit conversation. I have had a particularly shit time of it recently, we don't need to go into details but its safe to say I think I grew up wanting to be the opposite of her... In fact I think that's happened, I like the way I look, regardless of the stuff above I think sometimes I look really great and other days I'm kind of average but not ugly or fat. I also decided I cant be that ugly as I'm was very rarely without a boyfriend growing up and have a bloke atm who thinks the world of me and I adore him.
Something I have come to realise though is I'm quite straight forward, things are either black or white, I don't like grey areas or confusion and I am a complete control freak. I'm stubborn, harsh, sarcastic, bossy, lazy and bitchy. I feel better now... lol
See now I have to link all of this shit to my weight loss as this is kind of for my weight loss book... I guess my point was to assess where I am against where I was and my upbringing.
When I was young I hated how I looked, I was the tallest in every class I was in and until college, in my year too! I didn't like being different, I was bullied for being so tall and thin, the glasses and general geeky appearance which I blame on the Marfans completely, apart from maybe my bad dress sense :p Between the ages of 12 and 15 I was stick thin, I looked ill I was so thin, I think that's part of the reason I put on so much weight when I got older and had money...
I was always the ugly one, I will be honest I think all of my friends are more attractive then me, even now, but that's because its drummed into me that glasses are geeky but then I contend with the teeth and bone structure too! Its also my fault for choosing such attractive friends lol... On the other side of this however, I gained something completely priceless, my humour and personality. Because I was less attractive I attempted to win boys with my tom boyish personality and my brilliant humour and it worked almost every time. Personality is crucial to a relationship, something based on looks is doomed and I still believe that (I'm very lucky I got both in Rob <3)
In terms of weight I was always very light and skinny and then suddenly I put on 5 stone and it was if I didn't notice or I felt normal, as they say the average is a size 16. Maybe I was trying to control my body and change it to what I thought I wanted. I can safely say now I hated being that big, although I think I was in denial and pretended not to notice. I wouldn't say I comfort ate, but perhaps just wanted to tell my Marfans who was boss maybe? Or I just wanted that much chocolate as mum never allowed it in the house... So I got big, obese even. I had limited wardrobe options and it was a bigger strain on my heart and lifestyle. I HATED that my best friend wore such little cute clothes and looked fab and I felt like shrek next to her. Thank god not anymore, I mean, she is still much more petite than I and always will be but I am a size 14 now and eventually will be a 12 and am happy. I look good now, in spite of my Marfans.
I am aware that one day I may again look like I have Marfans but only a select few will know and that's fine cause I look healthy now and its much better for my heart, a person with a heart condition shouldn't really be a fatty, that's just silly. Never again, I am slim now and forever and it feels like my choice not my syndromes.
I wouldn't say I have had shit life because of my heart condition but it has had it difficulties and issues but such as life I guess... I dont do the whole why me its shit thing... its tough. It has definitely made me partly who I am though and why I am the way I am, I don't like it but I guess I wouldn't change it otherwise I wouldn't be me and overall I like that person.
