Marfan For Life

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The search for clothing and shoes when you are very tall

This was highlighted as a problem by one of my friends earlier today and what came from it was a productive discussion which has led me to write this piece in the hopes that it will make your lives a little easier.

I have added links to the blog already for both (that are not showing up for me annoyingly) http://www.tallgirl.com and also for http://www.casualmale.com  I have no experience of shopping with either of these or with another I found from a web search called Tall Clothing Mall whose site address is http://www.tallclothingmall.com

Also check out http://www.walktall.co.uk according to their website they have a SALE on (accurate at time of publication).

*NEW EDIT* For specialist footwear for those with foot issues try these three, provided by Sonja from the Marfan Support Group on Facebook)

http://www.equiptomove.co.uk/epages/es134890.sf/en_GB/?ObjectPath=%2FShops%2Fes134890%2FCategories%2FShoe_Gallery

http://www.abilitymattersdirect.com/fitzkidz/toddlers-to-juniors.html

http://www.reedmedical.co.uk/products/children-products.html?productPage=-1

This is also a good source of information too.  The National Marfan Foundations teen section.  Enjoy.

http://marfan.org/teenspace/2558/Fashion-Marfan-Style#Printable

*EDIT ENDS*

I personally don’t have a problem being only 5’10” but my daughter is just over 6′ and takes a larger shoe also so I know she has had problems and I wanted to try to make your lives a little easier if I could.  We all know life is hard enough as it is without extra unneeded hassles that we can avoid if we put our heads together on resources that we use in our individual lives.  I will of course continue to add to this section names and if required, once people have used a site, I am willing to collect and publish reviews of products and services to better identify the best outlets, all you need do is tell me about your shopping experience with a specific site.

I am also told that Nike Factory Outlets do shoe sizes up to size 20 (yes that is TWENTY) although I understand they are priced above ‘normal’ but if you can’t get footwear then it could be worth paying the prices they are asking.

During this quest I also found details of an author called Arianne Cohen who is 6’4″ and has written a book about living as a tall woman.  The site made it look very interesting and I thought I would mention it in passing in case any of you wanted to consider it for purchase.  The web addy is http://www.ariannecohen.com/tall

I am going to leave this one here for the moment but I will happily add other links both to my blog and into the body of the text of this post.

Government benefit changes in UK and ATOS.

Many of you will already know that the British government have effectively declared war on the sick and disabled over here through a private company called ATOS Healthcare.  They are under instructions to call in all people who are on Long Term Incapacity Benefit or other such state benefits to ‘decide’ whether they should be getting them or not.  I have been waiting for it for some time now but at 7pm Monday 16th July 2012 the phone call came telling me I was to report to the Medical Center in Castleford for medical assessment on 3rd August at 11am.

Despite my having been on sickness now for SIXTEEN years because of a degenerative genetic condition somehow they seem to think I might be getting better.  I WISH!  The medical assessment is actually misnamed because they ask a series of YES/NO questions and then pass your answers to someone else to make the decision (allegedly) and every person I know who has been for this assessment has said the same, the ATOS organisation is totally not interested in anything to do with pain levels.  We are not allowed to be in agonising pain, it should not be so high that you are not fit to work.  Again to this I say I WISH!

As I told you earlier I think, I am on Oxycodone (Oxycontin) twice daily plus several other different pain relief meds as my pain varies in intensity day-to-day.  But there is one thing about it and that is that it NEVER goes away, not even for just a short time.  They have totally disregarded the fact that Remploy, a disabled employment charity told me that they could not help me get work six years ago so what miracle the government are hoping for I don’t know.

What I do know is that they are using the system against us at every opportunity and what is worse they are targeting us and all the ordinary citizenry too through cuts and savings instead of targeting the mega-corporations that are doing all they can to avoid paying taxes.  Our national debt has increased, miraculously by around the same amount as our tax revenues over the last 30 years have fallen .. hmm .. coincidence?  Not likely.  Our government is supposed to be representing US, the people of the UK and it is not doing so.  IT is representing the shareholders and board members in big business instead to line their own pockets.  Most of our key politicians now have private monies into the millions and despite this they tell us constantly that “We are all in this together”.  That would be us using the Royal ‘We’ would it?  Meaning us not the politicians as well, heavens, imagine that, them being treated as we are .. gasp, it would never happen!

So, what I aim to do throughout the ATOS process is to keep you FULLY informed on my progress and their procedures as I think that the world deserves to see just what our so-called democratic country is actually all about.  The most vulnerable people in our society are being targeted it is utterly unjust and light MUST be shone on what they are doing.  The increased stresses for people with Aneurysms such as myself could be fatal, and likely for some it already has.  We are branded in the media as scroungers and lay abouts but I tell you this now, I would gladly swap my pain and suffering for doing a job any day of the week, wonder if Mr Iain Duncan-Smith would be willing to do me a swap …  More on this topic will follow as it progresses.

You learn something every day; Aneurysms.

Well now, you would think that after 45 years with this condition that I would have it all right wouldn’t you.  But I just discovered that my knowledge was actually flawed to what you must think a MAJOR degree and so, in light of this and to correct the thoughts of others who like me were thinking on the wrong lines I write this piece and can only apologise for being so remiss.

An aneurysm (AN-u-rism) is a balloon-like bulge in an artery. Arteries are blood vessels that carry oxygen-rich blood to your body.

Arteries have thick walls to withstand normal blood pressure. However, certain medical problems, genetic conditions, and trauma can damage or injure artery walls. The force of blood pushing against the weakened or injured walls can cause an aneurysm.

An aneurysm can grow large and rupture (burst) or dissect. A rupture causes dangerous bleeding inside the body. A dissection is a split in one or more layers of the artery wall. The split causes bleeding into and along the layers of the artery wall.

Both rupture and dissection often are fatal.”

(source the NHLBI)

 

Now I always thought that the enlargement was referred to as simply that, an enlargement (ie enlarged aorta) and that it meant that it had ruptured when people said that a person had died of an Abdominal Aortic Aneurysm (for example) as this is stated as the cause of death on people’s death certificates.  But now I understand that this is not the case and felt obliged to help spread this knowledge.  So there, knowledge shared, and thanks to Rachel Martin for correcting this misunderstanding for me.  You would think that by now I would have got it right wouldn’t you, just shows how good my cardio is that he watered his jargon down to simple terms for his patients to understand.  Oh the shame! LOL.

This blog and its readability

Something I forgot about Marfan, despite having glasses like Jam jar bottoms is that some of us have difficulties with reading text on the screen if it is not very clear.  So, as my followers will have noticed I have just edited each blog post and turned all text to bold in the hope that this helps those with this issue.  Thank you to my good friend Darren for highlighting this issue to me.  I hope that this has now remedied this issue for him and others too.  Here endeth my amendment.

Marfan and the healing problem.

All through my life this has been apparent although it is only since formal diagnosis in 1991 that I realised this was the reason I had so many different healing issues.  The bigger ones were obviously the healing of surgical scars, my appendix, having caused a serious issue with being bent inwards went on post op to get a really nasty infection which resulted in three weeks of antibiotics and this in addition to the two-week recovery time they gave us back then (1980).  I was a mess, without upsetting your delicate tummies I will say that it refused to even consider healing until fully 5 weeks had passed.  The same applied to my motorcycle accident in 1988, I tore through my knees to the bone and again it took weeks of antibiotics to help it even start healing.  All my scars are much worse than they should have been as a result of this infected period.

Each and EVERY time I go to the dentists for either a filling or an extraction I get an abscess which requires antibiotics to move it.  Sadly here in the UK our government has directed Dentists to only provide pretreatment antibiotics to patients who get full-blown endocarditis so despite the risks this would bring should I get it they make me leave it to chance.  This is something that causes me grave concerns knowing how my body heals and the state it is already in.

Even simple things, like the seven point jab you have as a child (for something like TB inoculation I think), this injection site got infected, despite all the clean materials being used and has left quite a nasty little scar as a reminder of just how well my body reacts.

At present, thanks in no small part to my kitten ‘Boots’ (so named for his  white forepaws and white back legs up to his knees) despite all the proper flea treatments he brought enough of the darn things home with him to cause me to get a pest control chap in from my landlord.  These nasty biting insects have me covered in bites and each and every one has gone funny causing me great concern.  Overall I now have about 50 infected bites and regular use of antiseptic cream and plasters on legs and arms has failed to shift the worst of these.  So tomorrow, no wait, today even I will be getting an appointment with the Doctor to make sure that these are in fact flea bites and not something more sinister.  I expect the fleas will have it because I always have problems with midge and mosquito bites, as well as Bee and Wasp stings.  But if it is something else I am sure it will be a good while longer before I am rid of these infections.  I am already into my third week with them and despite having eliminated 99.5% of the fleas he brought me I have acquired another 10 or so spots that are driving me insane with the itching in the last 24 hours alone.  I need to get myself some scratch mitts.  This is worse than my bout of childhood chicken pox and that was bad enough!

Because of this failure to heal properly I imagine this has been added into the mix for deciding to put my corrective surgery off to the last possible moment.  With the amount of work that they will have to do to get at my heart through a pigeon chest that stands out about 4 inches (9cm) from my chest I am guaranteed to get a major infection post op, something to look forward to for sure.

I so envy ordinary folk in this regard.  To hurt yourself and be well again in a short time period must be so great.  People take it for granted, not giving it a second thought but when you think that the body replaces EVERY cell in it within 10 years, EVERY cell.  so not one of us is technically more than 10 years of age its astounding.  What I want to know, drifting off a second or so from the topic, WHY do these new replacement cells have to be old-looking?  That is so unreasonable, we could have stayed young-looking forever but no, we are not made that way. Humph!  It would be like taking your rusty car in for a respray and the rust removing only to find that the garage had applied fresh rust along with the new coat of paint!  You just wouldnt pay them would you, but of course our body was free and you do get what you pay for … anyway …

Back to topic, the future in regards to the healing process scares me somewhat.  I can see my skin becoming weaker as the years pass by.  I know that for ordinary people this results in problems aplenty.  So when you think that we are at such a major disadvantage to start off with I dread seeing how things will go as the years pass.  I look at my mothers issues with her skin on her legs and it makes me shudder.  You see she has several ulcers that formed on her leg over 12 months ago now and despite them sending her nurses twice a day to change dressings and apply special creams and ointment she is simply not healing.  Things go so far down the road and then they adjust the extreme treatment regime to account for them looking better and within 48 hours she is back where she started (AGAIN!).  As I see the same problems in my legs now, but in a milder form than she has I cannot help but know that I am going to go the same way.  She is now 64, and is doing okay in terms of her heart and the like but her healing is truly diabolical.

How many of us Marfs have this problem?  I am not sure for certain, but what I do know is that I sympathise totally with each and every one of you who does.  There are so many things in our lives that could bring us down even when fully healthy and having a body that refuses to heal itself as it should is just an extra burden to carry that we could really do without.

Oh, and should any of you who are reading these blogs wish it I would gladly welcome the chance to get to know you all.  I can be found on facebook at https://www.facebook.com/simon.lowe.98 just please drop me a message explaining who you are, and feel free to tell me your views on the blog at the same time and I will look to add you as a friend.  Be advised though I do not tolerate fools or those who abuse either my friends or myself and if you are posting inappropriate material to any of us I WILL erase you without trial!  But if you want a good, loyal and honest friend then I am your man.

Anyway, for now I shall say adieu, but rest assured that I shall keep you posted on how I get on with the quack in the near future.  God bless and stay healthy, if you can …

Marfan impact on your daily life. Draining …

For some it is not easy to understand just how this condition affects us suffers in our daily lives.  Why is it when we look okay that we are off work?  Well here is my example, just of the last three days.

I was up at 7.30am BST and got myself ready to drive my daughter Dannii and her boyfriend Dan down to Leicester Forest services on the M1 motorway where I was to hand them over to Dans Mum for them to continue their journey down to Dans place.  It is a journey from my place of a little over 100 miles, only an hour and a half at legal speeds traffic permitting.  To most people this would be a doddle.

To me however it is a draining journey and thankfully we were there well too early for our planned 1pm handover.  I was in agony, although I tried to keep this fact from my daughter as I did not want her worrying about me on the return journey as she has worries enough of her own.

Had I tried to do the return journey straight away I would not have made it.  I would have been so drained that I would have been falling asleep behind the wheel, the pain and discomfort that it causes, even with extra pain killers is most unpleasant and that pain is truly draining.

When I made it home I messaged my daughter to let her know I got home safely, it should have been the other way round with ME doing the worrying for her!  I had a cuppa and fussed and fed the cat, and then, at 4.30pm BST I went to bed.  BED!  I am 45 and I look fine until I start to move when I do so like a snail (speed wise not slithering along I hasten to add).  I did not wake until several hours later and when I did wake I had seized up.  All the muscles in my legs were screaming at me.  My spine, hips, knees, ankles, shoulders and wrists all causing me much distress.  So I am into the pills again, all my regulars plus extra pain and inflammation tabs too.  I rattled when I had done and after my cuppa, since it was now officially time to be in bed I went back again.

At 13.30 BST I awoke on Friday, joints and muscles still screaming at me.  Half of the day gone already and with no energy.  I spent the afternoon putting off going to the local shop, not visiting my mother (which I promised her I would do on Wednesday!) and building my determination to go to the Working Mens Club Friday night to see a band I had been looking forward to seeing since getting my ticket aver a month before (called Complete Madness – top tribute act, worth every penny).  There was no way I was not going, even if I was going to have to crawl I was going to be there.

Well, I made it. I even lasted to the end of their performance at just before midnight.  I felt utterly frustrated however at the fact that I had to sit there watching other people dancing and having so much fun doing so when I could not.  I loved my dancing, I always had (even if not very good) until the early 1990’s.  I would have spent almost the entire time on the dance floor if I had been fit.

Then after my walk home (all 40 meters or so) I spent the entire night unable to get comfortable.  I have had about an hour or so asleep, and have to admit that this was as a direct result of my enjoying myself.  You see when I drink it reduces the effectiveness of the pain relief booster that is Amitryptaline, of which I take 100mg every night, in addition to the Oxycontin and Paracetamol.  When they are neutralised by the alcohol I can NEVER sleep, but sometimes it is a price you have to pay if you want to have any sort of social life (that involves alcohol of course) and so here I am blogging at 8 something on a Saturday morning.  My muscles and joints STILL screaming from Thursday and me wondering whether I will get out and to the shops today.  I have already put my Mum off until tomorrow morning when I hope to be okay to pick her up and take her with me to All Saints Parish Church.  I so need my church, its like being put on charge.  When I get there I immediately feel my energy levels rising and by the end of the service I feel so light and free it is amazing.  I would not be able to cope with this condition these days if I did not have my faith to keep me going.  With the prayers of my friends and the love of my church family I think myself BLESSED, even with this condition and all its negative energies and emotions.  Marfan made me who I am today and I am, ironically, glad to have it .. now how weird is that? LOL.

So, why blog this?  There are many hundreds of conditions from which sufferers appear healthy on the outside to look at, Marfan Syndrome being just one among these and I want you to realise, if you did not already that just because people look healthy does not make them healthy.  What we need is for the global society to SEE this fact and to make them more able to be considerate towards everyone just in case they’re keeping their health suffering inside, trying not to share the fact that they are in a living hell and want to cry sometimes but don’t because they know how others will react and they don’t want pity.  Remember – consideration of others costs you NOTHING but to those you are respecting and giving that consideration it is PRICELESS.  Thank you for reading my friends.

Marf and Parenthood. Baby. Yes or No?

Inspired by someone on one of the fb groups asking this question earlier this evening.  What you will get her is MY take on it, as a Marfan patient, a Marfan parent and a Marfan child all at once.  Hopefully these perspectives will allow you to see how this is viewed, from a male perspective at least.  But please remember that these are MY feelings and opinions on this subject and others may tell you different.

From the perspective of being a Marfan parent I have to say that the biggest thing I have in life is not worrying about how my daughter will see me, will she hate me?  The answer to this is the same for any parent whether Marfans is involved or not, one day Yes, the next No.  It’s what kids are about.  The issue you have to deal with is the worst of all, GUILT.  How will you feel about giving a child, YOUR child, the genetic condition you have got when you know how badly it can affect people.  Can you live with that guilt?  Me personally, in all honesty, some days I think the answer is No.  When I am feeling especially down, having a bad day or I hear that my daughter is having a bad day I have been known to cry.  There is no easy way to say it, it IS MY FAULT.  Now I have her though I would not swap her for anything (sorry Dannii if this embarrasses you).  I know however how it will affect her in later life, because it has already done it to me, to my Mum, my sister, my Uncle and My Maternal Grandfather.  It is an almighty burden to carry and how I will cope with this should she go down the same path as me I do not yet know.  She is only 17 (almost 18) and it will be years yet before (and IF) she develops the problems that I have today.  It could be that she will be fine, she could be the same or, she could be worse.  That is the problem with Marfan, it varies so much from person to person BUT familial similarities mean that it is more likely that she will be like the rest of us.  Thankfully she is armed with the knowledge that she MUST adapt her life to manage the condition as best as is possible where me and my generation were not so lucky and did the harm before we knew what it would mean to us in later life.  I will always carry that guilt, no matter what others say, no matter how things turn out.  It IS my fault.  I cope, but only just.  Will you?

From the perspective of being a Marfan child. Do I hate my family, especially my Marfan Mother for me being like this?  Some days, yes.  I am being honest here so that you get the real picture as to do anything else would be pointless and I don’t do the lying thing.   I spent a good few years hating not only my Mother but my God with such a passion that I was ashamed of myself, something that only compounded the problem really.  The worst 5 years, helped by other circumstances too I might add, had me being very nasty towards her.  This was not me at all, but I can’t make excuses because I did do it and I poured my venom out at her one day and am surprised that it did not destroy her completely because I was pure evil at that time.  Even knowing from my own situation as a parent that she would be carrying her share of guilt already I still laid into her verbally and actually got a kick out of hurting her, and other family members too.  At this time I was definitely not someone you would have considered to be a friend via facebook or any other way either.  I hated everyone for how I was, blamed everyone and sought revenge on them too where possible.  Thankfully this eased off when I refound my faith in God and realised that yes, I have got Marfan, but it is Marfan that has made me the person I am today and I know that from my own life experiences I can perhaps help others come to terms with what is after all a really life changing condition.  I hope that by sharing my experiences and thoughts that you are getting something from this, my legacy if you will to future generations of Marfs.  Now my relationship with my blood relatives have been restored to a better place than they have been in over two decades and we are now more able to talk about our Marfan issues than we where before.  I guess that the chaos and heartache highlighted to us all just how it was affecting us, not just me personally but each of us, including those in the family who don’t have it too, although where exactly they are at with their Marfan journey I can’t begin to imagine and until just now I had not given it much thought.  I know they have to be relieved but do they carry guilt for NOT having it?  I shall have to ask that question next time I see my cousin, the only non-marf in my generation of the family.  It will be interesting to hear her thoughts on it and perhaps I can add those thoughts to this post at some point in the future.

These are some very negative perspectives I know but there IS a positive twist to it too.  As any of you with Marfan will testify, when you have grown up with this condition and have survived all the rubbish that it throws at you during that life you become a very positive person, at least outwardly, you laugh, you smile, you makes friends with relative ease.  Your also smarter than the average member of the race, your bodies physical weakness has caused you to be mentally stronger (and emotionally too I think).  Your children may suffer as they grow up, they may be limited in adult life or at worst be disabled by the condition BUT they will be among some of the most loving and genuine people on this Earth because they value every minute and know how it feels to be different and downtrodden.  They WILL be the carers and lovers of the next generation, Marfan will make them so.  They may even be among the leaders of future generations because of their empathy for others too.  Abraham Lincoln is believed to have been a Marfan, and many other influential people too, yours could be among these greats one day.

One thing I know that is important for any Marfan woman to consider is the risk to their lives to go through pregnancy.  The strain that being pregnant places on the heart is often too much for a Marfan heart to cope with BUT, and this is key, if you are under a good doctor they will be with you every step of the way should you decide to go for it all the same.  I know that those that do are rewarded with a beautiful, if slightly abnormal (but not always I hasten to add) baby.  That baby will change your life regardless the problems that you or it have.  That is guaranteed.  You might not even pass on the defect, its supposed to be 50/50, but in a strong gene pool it could be more, in a weak pool less.  My family it’s about 75-80% I reckon looking at rate of us being affected by it.  Only you, with advice from your GP/Cardiologist can make the decision and only you will know in the months and years ahead if it was the right call.  But know this.  IF you do have a Marfan baby that we, your fellow Marfan patients are like a family here on facebook.  There is NO question you can ask that someone on these pages can answer for you.  We are all here for you.  REMEMBER THIS.  You are NOT ALONE.  God bless you in your decision-making.

ADDITION TO INITIAL POSTING:  I told you that my knowledge was limited 😛  Regarding the female position on having babies with Marfan syndrome in the mix, my male opinion and understanding was wrong, and I thank my Marfan female friends for the correction.  Apparently the risks are much varied dependent on the specific situation of each Marfan patient.  My own sister is, when all said and done a parent of three fine daughters (all Marfan of course) but she survived it with only concern from her family.  I guess I should have talked to others first.  Anyway, I am a man and as such am allowed to make mistakes so women can correct them 😛  Its my job.  I am after all an old school Gentleman 🙂  Please ladies don’t hit me when we gather in Hull it was a JOKE! lol

Pre 45 things I missed that I should share ..

It is always the same when you start to write stuff down, you forget important stuff.  Why is that?  Anyway, there are two things from my younger days that Marfan played a major part in and I felt they must be included so;

At 13 years of age I decided that when I hit 16 I was going to sign up for the Army, I felt a genuine buzz when I came to this decision and knew it was what I wanted to do, no question.  So after I had quit school I went along to sign up. I passed the medical (I KNOW!) and also the tests, but this was in Thatcher’s time and unemployment was at 3 million.  As a result the army had 10 applicants for every vacancy and so I did not get in and was told to try again at 18.  Which I did.  I passed the test with flying colours and had decided on either the Signals or RCT as my chosen areas, the recruiting staff were delighted at this as they were from the two aforementioned sections.  I went up for the medical and FAILED inside the first 30 seconds of taking my shirt off.  The doctor took one look at the pigeon chest and asked what was wrong with me.  I said the two words I already hated even before the 1991 diagnosis, Marfan Syndrome.  The doctors face dropped and he picked up his regulations book, found the appropriate page and read these words to me “There is a very slight possibility that persons with Marfan Syndrome may, through continued physical exertion, develop a minor heart condition and as a result the Army is not willing for you to take that risk”.  This he told me had been added to the regulations just one month before.  My dream came crashing around my ears and what was left of my faith died with it.  I blamed God for my situation.  I could not see that although the health was going to be in trouble that my not getting in was a good thing.  All I saw was the negative and this stayed with me for 25 years.  The fact that if I had got in it would likely have killed me slipped by unnoticed for the longest time.

The first time I really gave these words even a moments thought regarding them being right was in 1991.  I was out on the town with a group of friends from bedsit land where I was living at the time, and as we wandered our drunken way home I start to get a REALLY bad pain in the left side of my chest.  I was thoroughly unhappy.  As you can imagine all sorts of things start going through your mind, especially when two years earlier you are told about how your aorta could dissect or burst .. so I started to get stressed.  So too did my mates and within ten minutes of us being in my bedsit we were getting in a taxi to take us up the road.  And all credit to those friends they stayed with me for the several hours that I was in the A&E being tested and monitored.  I have never been so scared as I was that night I can tell you.  I was convinced and so were my friends, that I was in real trouble.  Thankfully we were all wrong and I learned a very valuable anatomy lesson too.  You see the very weak muscles that link to spine and shoulder blades wrap around your chest cavity and connect to your Sternum (the breast bone) and what I was experiencing was a sever spasm in these offending muscles.  It is a spasm I have come to know instinctively now as that was just the first of many.  I was somewhat embarrassed at the time for wasting their time but I was assured that under the circumstances I had done the right thing and should not hesitate to do the same if I was in any doubt in the future.  With this knowledge my drained friends and I left the A&E in party mood, which was a bummer since it was only about 6am and nowhere was open to celebrate in!  So we elected for sleep and made up for it by partying that night instead.  So my advice on this for any of you new Marfs, if in doubt get it checked out.  If you are having a real dissection or worse and aneurysm then you will pay for it (or just simply die) if you do nothing and your hospitals staff would rather you be sure your okay.  You will know in time to recognise the signs of muscle spasm just as I have.  But until then .. GO TO A&E.

There, omissions corrected.

30 to OLD .. no, not old .. mature .. no, stuff it 45.

What has happened to me medically in the last 15 years is, I must hasten to add, pretty minor compared to many with my condition, but then at least they are being patched up where I am not .. yet.

My mood with Marfan is still little better, I take so much medication now that I am sure I rattle when I move.  I am still feeling guilty as sin when I think of my now 18-year-old daughter having the same condition as I, especially as she is having issues with her joints now too and our present government are doing all they can to destroy the lives of many of my friends through their ‘Limited Capability for Work’ assessment which takes absolutely no regard for the levels of pain that people suffer AT ALL.  So these people, all crippled by pain 24/7 are being told by ATOS that they are fit to work.  It is a disgrace, and sadly it is a process that I too am now undergoing although I have only just taken the first step on this unpleasant road.  I expect them to say I am fit for work too, despite the fact that in 2003 REMPLOY told me they could not help me get work .. they being the charity for employing disabled people.  But hey, sure Cameron and co can find me something to do, perhaps acting as an unpaid stamp licker or something.

As to the body.  Are you ready for this.  I have ALL the following issues; enlarged aorta (now 5.3cm as at 2010 MRI) but still not on surgery list; diaphragmatic hiatus hernia (major, reducing lung function to 65%); osteoarthritis (in spine, hips, knees and starting in hands, wrists and elbows); spine is also twisted AND curved; pigeon chest (still); constipation (result of hernia); chronic prostatitis (painful AND embarrassing); currently low blood pressure (last 4 months are causing GP and nurse concerns resulting in blood tests and monthly BP checks) and yes, I am also officially depressed.  I take ten different medications now including Oxycontin (Oxycodone) for the pain which is a Morphine painkiller stronger than Tramadol.  I have also discovered that I have an allergy to nickel and that I actually corrode it when it is in contact with the skin for any length of time so have to choose glasses carefully.  My teeth are officially stuffed.  I have lost half the top set and almost half the bottom set with another one to go shortly (as soon as I make the appointment that is – hate needles so putting it off).

Basically the bottom line is I am falling apart.  Why have they not done anything to fix me up as they have with some other Marfs?  Overall my chances of surviving an operation are only 75% and as a result they will not do any procedure that involves major anaesthesia unless it is a matter of life and death.  The risk of death is to them unacceptable.  Me, I would welcome the operations if it meant I could get some sort of life back but they just wont do it and so I go on in agony every day wishing my life away.

My marriage is now history and in no small part Marfan played a major part in this, sure other factors were involved, some pretty serious ones as it happens but my general health and overall mood had to contribute to this too, to say different would be fooling myself.

The only positives in recent days for which I am grateful are my bungalow, no more stairlifts! YAY! and my return to my Christian faith.  this is a very major thing for me as I lost my way when about 17 years old and only got it back again in the latter half of 2011.  The only thing really keeping me going mentally now is this faith in Jesus, without it I would have plunged myself into darkness by now.  I have no doubt of this at all.  My faith took me back from the brink of drink related problems last year after the marriage failed and I know that had it not I would be dead already.  My GP at that time had me under an alcohol specialist nurse for monitoring and I went from 7 days a week drinking to none (bar an odd pint socially) so that is AWESOME!

So now we wait together, in pained anticipation for the day on which they are going to be forced to operate on me.  Although I am due (actually overdue I think) for another MRI and consultant appointment so will keep you appraised of the results of that as and when the time arrives.  In the meantime I shall post on occasions with any Marf related news that I come across or any changes in my general health or the health of my family members.  The headcount at present is my Mother, Sister, Brother, Cousin, Daughter, Three Nieces with Marf and One Cousin and two Nieces without.  That is 9 of 12 including myself, over three surviving generations of the four we have had with Marfan.  Will we be seeing an addition of a fifth generation?  At this point the older Nieces and my daughter say no, but that is what I said too.  But then the risks of death to pregnant Marfs are much greater as the strain placed on the heart and other major organs by pregnancy multiply the risks expotentially.  So glad I am a bloke!!  For now farewell, don’t go too far, I may call upon you to read more in the future. 😉

Adult life to 30.

Even armed with the new knowledge and the advice that I ought to change my lifestyle I found that it was easier to say than to do.  I loved dancing see, fancy solo RocknRoll showboating.  Yes, me, a show off.  It is hard to imagine innit 😛  But as the years slowly ticked on by I became less and less able to do this.  I found myself having sudden pain in my chest and in my back.  My knees would not always do as I wanted them top and my ankles too were giving me severe grief.  This was my ONLY really enthusiastic pastime though so when I danced my last dance in 1993 it was quite depressing to say the least.  I was now considered worthy of a card to say I was registered as disabled, woohoo .. hmm.  The government even paid training providers extra to find me work, but even that incentive could not get me into the work market.  The words heart condition did nothing to help this.

The tests had shown that my aorta in 1991 was at 4.3cm (normal is 3.5cm)and I was put on 100mg a day of Atenolol which slowed growth in the aorta to 0.05cm per year.  No employer then, and still to this day wanted to take that risk on.  So you didn’t tell them.  My new lady in 1993 announced she was expecting in August on 1994 and I got myself three jobs in very quick time, all part-time.  two telesales and one cleaning, despite this being an intensive job.  I traded those three in for a full-time job with a cleaning firm in Leeds which meant I was out from 6am to about 8pm every weekday.  I was also studying two A levels at that point and kept up with the study and passed them in the summer, Business Studies and Economics. The job went from Telesales to Telesales, Admin, Purchasing, Client Liaison, budgeting, Stock Control and Project Management (inc field work).  I loved it, and happily ignored all the warnings I had been given about lifting, carrying and manual work which told me they could prove fatal if I chose to do them.  Forgot to mention this to the employer like, as you do.

I moved from that firm to another, with less responsibility for more money, still in cleaning, still doing too much and then .. bang .. in 1996 as I got ready for work I went to put my jacket on and the muscles in my back went into violent spasm.  In the months that followed I was in constant pain.  My pain killers went from Paracetamol right up to Tramadol in the space of about 2 years.  My legs took the strain while walking with a very bad back and as a result they failed me too and throughout both spine and legs I was hit with osteoarthritis also, just for good measure.  Eventually my employers patience ran out and I was told they could no longer keep the post open for me.  This crushed me badly.  I had worked hard to build this new career path and my own stupidity had taken it from me.  IF I had followed the advice and avoided the manual side I would have been okay I guess but I need to do it if I was to get on in this field.

I felt utterly powerless, hopeless and worse still my daughter at this time was three, I knew she had got my condition too, she was on Atenolol too and had been from being only 6 weeks old when they confirmed her diagnosis and that her aorta was growing too quickly also.  I couldn’t play with her as I had, I couldn’t pick her up and carry her about.  Life was never going to be the same again.  I was home 24/7/365 too which, as I am sure you can imagine was not too good for my relationship either.  I was walking with a stick and the Occupational Therapy people put me in a stair lift to help me get around the house.

Things looked pretty bleak at this point and yet still I did not know it but this condition had not finished with me yet.