World Haemophilia Day 2014

Hello there and happy World Haemophilia Day!

Whether you're a bleeder or a clotter you may be interested to see this video which I made last week of me having one of my regular clotting factor injections.  As a severe bleeder with type 3 von Willebrand I learned to self-infuse factor 8 aged 14 (26 years ago now) and it revolutionised my life.  I am lucky to find injecting relatively straightforward, most of the time.

I was intending to re-record this today, wearing red, for World Haemophilia Day but instead I'm taking my dad out for a birthday lunch as he was 75 yesterday, so you're gonna see my trial run which I figure is good enough.  Please bear with my efforts as it's the first video of myself I've ever attempted!

I wanted to record and post this to raise awareness of the thousands of women who live with bleeding disorders and also to reach the millions of undiagnosed to encourage anyone who thinks they might have a clotting problem to consult their doctor with a view to getting tested.  

Not everyone has to inject like I am doing, those who are severely affected do and you don't have to do it yourself.  I chose to so I can take my treatment and travel / work anywhere.  I am lucky enough to be able to do this and I appreciate that massively.  Others in less affluent parts of the world do not have access to the most basic treatment.  To find out more about that and how you can help please follow this link:

Save One Life

I hope you enjoy (?) my video or at least find it interesting, and if you're a fellow bleeder or carer feel free to feedback on my technique or sanitary arrangements!  

Funny Blood Jab

I've tried to upload the video but blogger won't let me so I now have my first ever video on YouTube.  Apologies the sound is a bit rubbish - I'm a novice!  Thanks for watching ;)

xx

In the words of Gloria Gaynor ...

I am Rosamund Mary
I was born on the Isle of Man
I was adopted when I was 12 weeks old
I have a rare form of Von Willebrand's disease
I have less than 3% Von Willebrand's factor
I have less than 1% factor 8
I have injections to replace the clotting factor and stop bleeding
I learned to do them myself when I was 14
I started my periods when I was 13
I would bleed for 3 weeks out of 4 and then start all over again
I have taken hormone pills ever since then to keep the bleeding under control
I have always had acne on my chest from the hormone pills
I used to have horrendous period pain every month
I once had to stop my car and knock on a strangers door for help because I was blacking out from period pain
I now take hormones continuously because it's safer for me not to bleed
I was given Hepatitis C from contaminated NHS blood products
I was probably exposed with every injection I had in the 70's and early 80's
I had on average 1 injection a week then to control bleeds
I was not told by my specialist about my infection with Hepatitis C
I found out by accident at another hospital
I had a 12 month and then a 6 month course of treatment for the Hepatitis C
I had a biopsy before the 2nd course of treatment
I lost six pints of blood and nearly died after a bleed in my liver
I discovered just before I got married the Hepatitis C was no longer detectable in my blood
I have been ill with chronic fatigue ever since those treatments
I now have on average 2 injections a week of clotting factor to control bleeds
I have been exposed to vCJD
I have been unable to work since 2008
I have been unable to have children because of my ill health

I am what I am
I am just right
I am all this
I am me

Betrayal and Lies -- Please Share

This video has been put together by a fellow contaminated blood campaigner and friend of mine, Richard. It contains contributions from a number of friends, many of whom are tragically no longer with us.

It is disturbing but essential viewing if you are interested in the unnecessary infection of thousands of bleeders like me, through our NHS treatment.

Please take the time to watch and share it where possible.

Thank you x

Contaminated Blood House of Commons Debate - 14.10.10

Around 100 of us crowded into the public gallery in the House of Commons last Thursday to hear a historic back bench debate on contaminated blood products.  Haemophiliacs and von Willebrands, infected and affected, wives and husbands, families of those too sick to be there and widows and children of those who have died.

We had come to London with a sense of anticipation, a sense of hope that after over 20 years of campaigning and the loss of 1974 lives, we were finally being met by a government who stood for fairness.  A government who had indicated from the PM down that they wanted to help us to bring closure to the victims, of what has been described as the "worst treatment disaster in the history of the NHS" by Lord Robert Winston.

It started with a worrying turn of events.  The government tried to amend the original debate motion.  Not to change a couple of words within it, but to re-write it, bar the first three words "That this house...".  The government wanted to take out the acknowledgement of past failures and limited response so far to the tragedy, wanted to take out the proposal to implement Lord Archer's inquiry recommendations; even wanted to remove the apology to the survivors, their families and the bereaved.  In its place their amendment merely recognised the [inadequate] recompense that had been provided by previous administrations, estimated the cost of implementing the Archer recommendations as being £3 billion, pledged to review some of those recommendations and deeply regretted that many people were infected by contaminated blood products.

We were shocked and dismayed by this attempt to wreck our motion.  However the speaker ruled that as this was a back bench debate the original motion should not be amended by front benchers and that our debate would go on as it was.

Hooray we thought.  There followed three hours of passionate, personal and moving 5 minute statements from 24 MPs, all of whom had put in to speak at the debate.  The support and understanding we had from MPs of all parties was amazing. There was no arguing over the basic facts about what happened, no dispute that here we had a terrible medical scandal that resulted in a human catastrophe and great need. There was no argument about the fact that a proper financial settlement and hopefully an apology was required urgently. I say urgently because, as was pointed out by one MP people continue to die at the rate of one per week. We don't have time to hang around whilst politicians play games.

MPs paid tribute to members of our community who have died and to those who are still waiting and dying.  Whilst tears were shed in the gallery I was horrified to see two front bench MPs talking and laughing as Owen Smith spoke of Leigh Sugar, a haemophiliac from Wales who died from Hepatitis C earlier this year.  Where was the respect there?

It was debated as to why we had referred to the scheme in the Republic of Ireland, as this was felt to be too generous for the government to commit to.  It was said more than once during the debate that this was put into the motion because it was what the campaigners had wanted.  To an extent this is true, but that is because it was the bottom line for compensation that was cited by Lord Archer in his private inquiry report - a report largely ignored by government and one that the judicial review earlier this year compelled them to revisit.

What the victims of this tragedy want is a settlement that is fair and adequate - a settlement that treats this group with decency and respect and ensures that what remains of our ruined lives is spent at least financially secure.  Ireland's settlement which was implemented in 1996, and is still being honoured despite their financial difficulties, is one that is considered appropriate for the nature of the difficulties victims face.

As one of our campaigners recently said:

'You can't give us back our health, but you can give us back our dignity'.

The greatest despair though, came with the government's apparent inability to work out simple maths.  The question was how did the government arrive at the figure of £3 billion that was in their amendment, and that was their estimated cost of putting in place a similar compensation scheme.  The calculations were apparently accurate but were not able to be produced during the debate.  We are still waiting to see them.  We believe the costs to be far lower. 

We are well aware as a community that the timing on this is far from ideal however it is important to note that the reason we were infected by these devastating viruses was not least because the government diverted money away from developing self-sufficiency within the UK blood products production.  Money was not made available then, over thirty years ago and on many occasions since then when finances were there, administration after administration has ducked responsibility.  We haven't just turned up wanting a hand out.  For years successive governments have ignored the issue. It's not about party politics, it's about doing the right thing and it's about time that the victims of this disaster are given what they deserve - justice.

This was all acknowledged at the debate and the need to act now, in the name of justice and moral responsibility, was stressed time and again. 

However the vote was lost.  Why was this?  Because the government had produced a three line whip.  This compelled over 200 MPs who had not necessarily even attended the debate to come and vote against it when the bell rang.  It also meant that my own MP spoke passionately for us, but voted against us.

We appreciate that times are tight and that a cost in excess of £3 billion at this time of comprehensive spending reviews and cuts may be seen as irresponsible.  However what about getting your sums right before trying to write us off?

We are not a greedy, compensation grabbing bunch of individuals - we are genuinely sick, dying, unable to contribute to society the way we could and to look after our families the way we want, because we received contaminated blood products from the NHS.  All we want is to not have to fight for justice anymore and to have our losses recognised.  It wasn't right then, it isn't right now and we won't be going anywhere.

What we came away with is the promise to review the situation by Christmas.  The present government have the opportunity to put this right and they need to know that we will never give up.

Fairness, Mr Cameron?  Mr Clegg?  Let's see some at last.

Bleed Hazards

As a type 3 von Willebrand, life is like a box of chocolates. One which has been sabotaged by an ex lover and is filled with poisoned soft centres and pebble filled truffles which will break your teeth.

Being a severe bleeder, activities which are undertaken without a second thought by a non bleeder, to me can be high risk activities. A high risk, that is, of causing bleeding or bruising.

This is my top 10 11 list of everyday, bleed inducing things:

Ferocious Fodder:

1.  Fruit - apple skin is one of the most efficient ways of getting a gum bleed. The safest way for me to eat an apple is to cut bite size chunks off with a very sharp knife... Don't worry, I wear Teflon gloves. Bananas can have the same effect if they are a little unripe, which is, of course how I like them.

2.  Puff pastry - the equivalent of eating razor blades, very dangerous to gums, tongue and inside cheeks. Ruins the enjoyment of a good pastry.

3.  Muffins - surprisingly risky - had 18 hours of gum action following a particularly chippy bit of chocolate in a muffin the other day.

4.  Sandwiches - a crusty roll has obvious dangers. Crusty bread equally so. However I have bled from an encounter with the softest fluffiest baps filled with only egg mayonnaise, perhaps it was the cucumber...

Personal Mean Hygiene:

5.  Tooth brushing - most common cause for me of gum and tongue bleeding. I've been told many times by many dentists that brushing more frequently will reduce the overall bleeding tendency - I can tell you I've put this thoroughly to the test and it's nonsense: more brushing = more bleeding - fact. I am still to try the Sonicare toothbrushes that have been recommended by a fellow bleeder - I will let you know how that goes...

6.  Flossing - not something I relish as this is akin to running wire thro mozzarella.

7.  Hair removal - waxing, plucking, epilating, even hair removal creams can cause anyone to bleed or bruise - with a type 3 von Willebrand this can be to very dramatic effect. Legs which turn purple upon waxing may be a little alarming for your beautician. My current preferred method of defuzzment - epilation - even post clotting factor treatment can leave my legs covered in petechiae. My first ever attempt to shave my legs as a naive teenager resulted in a blood soaked kitchen (I know, weird choice) and a cover story about dropping a knife and it impaling my shin - not sure that was ever actually believed!

Household Sore Chores:

8.  Painting - an activity I used to enjoy is now more risky due to a spot of ceiling painting which caused a shoulder bleed. This has weakened my shoulder and it has a tendency to bleed if used to decorate. No really, it does.

9.  Correspondance - writing letters, whilst now an old fashioned activity is also a hazardous one for me. Paper cuts from the writing paper is common and if you've had letters from me they may well have contained at least one of my bloody prints - I'm never gonna get away with murder, I'm far too forensically generous. Licking the envelope slices my tongue into strips. Stamps used to stick to my lips and rip them into bleeding. Owwee. Thank goodness for self adhesiving ones I say.

Medical Maladies:

10.  Plaster removal - luckily I'm not allergic to latex having such an aggressive plaster habit. However removing a plaster is in itself a dangerous job and whilst ripping it off in one smooth, swift motion is ideal from a pain point of view, it is a perfect way to cause post plaster bruising. Nice.

11.  Medic alert - it is ironic that a medic alert bracelet, worn continuously in case of emergency because it just might save my life, causes bruises and burns. If I lean on my hand and the bracelet is in the wrong place it bruises me, this most commonly occurs when I'm injecting clotting factor into that arm. When stirring a hot pan of food the bracelet heats up so quickly I don't notice until it's branding me with "Von Willebrands Disease (Type 3)". I suppose that's one way to avoid having to wear it in future...

For ME

Happy International ME/CFS and Fibromyalgia Awareness Day everyone!!

Today, 12 May, is a day for raising awareness of ME / CFS and Fibromyalgia internationally. (Ok - so it's clearly not the 12th any more but that is when I started writing this piece.)

In honour of this I would like to make the subject of my post today me. Or rather ME.


This blog was created to chronicle my bleeding condition - von Willebrand's disorder. This is a rare disorder which means my blood doesn't clot very well. It is a little like haemophilia and has caused me to have an interesting a blood soaked life, since diagnosis at 6 months old.

I'm now 36 and whilst I am a happy little bleeder generally - who finds handling a long term chronic disorder, within my capabilities - the last few years have been a bit tougher. (If you would like to find out more about the von Willies please do peruse my previous posts - preferably with a cup of tea and slice of summat to keep your energy up.)

Something I have blogged less about is my experience of having CFS or ME.

Why is that?

I know my von Willies well. I was born with it. It is who I am. I am a bleeder. I am able to live in that knowledge and with the challenges that presents in a fairly confident, capable and good humoured way.

The von Willebrand's is treated with clotting factors. Which are made from blood products. From these blood products I contracted Hepatitis C way back when they were shooting clotting factor into my little girl's veins.

The Hepatitis C stuck with me, infecting my blood and messing with my liver, until I had the second of two lots of treatment, which managed to eradicate the virus from my blood.

Good news right?

But wait...

I really struggled with the treatment - Interferon and then Pegalated Interferon and Ribavirin.
And I mean really struggled.
It wiped me out.
Beat me up.
Wrang me out.
Shook me up.
Drained me out.
Chewed me up.
And spat me out.
It felt like being in a washing machine for 12 months.

Ever since the second lot of treatment in 2003 I have had recurring CFS or ME.

I still felt I was on the treatment six months later, 12 months later, 2 years later and still now 7 years later. Unable to work. Unable to live the way I used to. Existing. Waiting.

Don't get me wrong I have had periods of being well - being able to work, to live a full life, to fit everything in I wanted to and more....

However the reality now is not so good. My natural outlook is a sunny one. I see my CFS as a grey cloud that contains me and is not always easy to shine through.

The tricky thing is that to the untrained eye I look well. You look at me and don't see the cloud. Or the fog or the mist that clogs up my mind and body.

I used to find it hard to acknowledge the CFS. My mum once gave me a leaflet on ME and said - this sounds much like what you've got. Oh no, said I, that's not me - I'm just getting over the Hep C treatment. I'll be fine in a bit.

Even now if people ask me how I am - I'm fine! Or if not fine - I'm a bit tired.

A bit tired!! Understatement city :)

It is difficult to write how hard things can be. Equally it is difficult to talk about how hard things can be.

That is a problem.

How can I expect people who do not have, have never had ME or CFS to understand what it is like if I don't let it out.

You may be looking at the picture above and saying to yourself - she looks right enough. That is understandable.

If you see me looking well - which I do when I'm in public, most of the time;
or see me getting to the supermarket - which I do some of the time;
or if you have a conversation with me and I'm upbeat - which I try damn hard to be all of the time;
you cannot be expected to understand the reality.

I have tried to be a bit more explicit, but when I mention how exhausted I am - you might say, oh yes I'm tired too.
When I say it's hard to get things done - you might think, oh but you're lucky you don't work, you have plenty of time.
When I explain how hard it is to sleep - you might suggest, perhaps if you did more in the day you'd be more tired and sleep better?
When I say how much my body aches - you might sympathise, maybe if you got out more you would be fitter and be less stiff?

Often I don't have the energy to respond.

To argue that my exhaustion is so deep it is like a coat of lead that I wear 24 hours a day - that prevents me getting out of bed, that means I hurt to wash my hair and hang out my washing because my arms have to be held up, that means I pant after going up the stairs and need a rest after washing, then after dressing, then after eating my breakfast...

To explain that it is hard to get things done because my brain doesn't work properly any more. You know how you feel when you're hung over? Like your head is full of wool and your body is poisoned? That is my normal state. I forget things that used to be second nature. I struggle to make a cup of tea if you're talking to me, to hold a conversation without losing my thread, to write a blog without wanting to smash the computer because I keep getting words wrong, to run a bath without putting the wrong tap on and flooding the house, to put toothpaste on the brush and not Veet. I can't expect you to understand that some days I struggle to fit in getting up and dressed and doing some tidying and making the tea. Let alone cleaning and ironing and paying the bills and having people drop in to see me. The washing machine beep sends me demented. Running out of cat food is a constant worry because it means having to go out to get some.

To elaborate that I can't sleep because I'm so tired it hurts. That my joints ache and my muscles twitch and I can't be still for longer than a couple of minutes without twisting and turning and writhing trying to find a comfortable spot. That I feel things crawling on me - on my arm, then on my foot, then on my back, then my leg, then my face. There's nothing there, it's just a symptom of the CFS, Crawling Effing Sods. That if I do get to sleep I often wake in the small hours and then it starts all over again - the pain and the aching and the crawling and then the mind chimes in telling me that I need to sleep, I need to sleep, I need to sleeeeeeeeeep!!

To tell you that the aches and pains are such that I can't sit still for more than a few minutes without stretching and flexing my legs, arms, fingers. Next time you have the flu and you feel so sore and drained you can't move out of bed or off the sofa - that it how it feels on a bad day with CFS. It feels like my bones are rotting and my muscles are aching and twitching in an endeavour to get out of this body.


Is it better for me to acknowledge the CFS? Does it help to let it out? Or does that just remind me how much fun it isn't??

I know fighting the condition doesn't work - that the energy used constantly battling to overcome the lack of energy and limitations of your body aggravates the condition exponentially.

So do I need to accept ME in order to get better? Embrace my little grey cloud and let it hug me back. Take care of myself rather than push myself to the limit? Listen to my body rather than shout at it? Allow my body to recuperate rather than expect it to continue at an unsustainable pace? Give my mind space to rest and restore rather than shower it with anxiety and pressure and stress?

Love and cherish not berate and frustrate.

Worth a try, right?

Night Mare

This was the evening after the morning before.

Another weeny gummy bleed. Where this one came from I'm not sure - think it started around mid afternoon - aaaah mebbe it was that piece of Soreen I had with my cuppa?? It is very sticky and probly sucked a bit of my gum off. Nice. Anyway, it was still going as I headed off to bed.

You shot up? says Ade.
No, says I.
You won't sleep, say he.
Oh but I will, say I.
I got my night nurse you see and with that I could sleep through a boobquake.

Where was I?? Oh yes, the clotting factor conundrum...

I was talking to a fellow bleeder at the hospital Tuesday lunchtime and we both admitted that where possible - we put off having an injection until it is absolutely desperately necessary. Now it's not like either of us have a problem injecting - we don't. Though I know bleeders who hate it with a passion and I really wouldn't want to be in their sensible shoes.

Injecting is:

• quicker than it's ever been (with these drugs companies reducing what used to be a squishy bag of plasma down to a couple of glass bottles)
  
• pretty effective (though not always so, being a Type III von Willie, that would be far too simple)
• almost painless (if I do it in my left elbow and through the scar tissue - not if I have to use my right elbow vein, but even that ain't too bad; hands - now they would kill me but I've yet to have to resort to that entry point)

So why put it off??

It's funny - when I have to do prophylactic treatment, i.e. twice daily injections to cover a period or a procedure of some sort, I set my alarm at 9am and 9pm and do my jabs no problem. Ok - so mebbe there is a little cursing and harrumphing but I do em - night after morning after night after morning.

So why is it different when I need to treat on demand??

I'm not sure.

It seems much more of an effort. Much more of a chore. A hassle. It bugs me frankly that I need to have my treatment. I mean why for God's sake?? Why do I have this damn disorder that means I need to stick a needle in myself to stop my gum from oooooooooozing like an insidious leaking leech.

Is it because it reminds me of my imperfection? But why would that be, as this process fixes me?

Do I hate injecting that much that I avoid it at all costs? Nope, once I get on with it, it's easy and generally restores me to a non bleedy state.

What then?

Am I just a lazy arse? Partly I think. My friend and I both agreed that when it comes to it we often just simply cannot be bothered.

Is it the cost?? Is it that I feel it needs to be really bad to warrant injecting? I have access to this treatment that not everyone with funny blood has. Sometimes yes, I think I postpone longer and longer. I wait - wait for the bleed to continue past 5-10 minutes normal bleeding time, past 1-2 hours slightly annoying bleeding time, onwards through 3-4 hours really annoying bleeding time, we reach 8 hours and I realise that I'm still oozing. Then do I inject??

With my gums - I sometimes wait til 12 hours before I jab, 24 hours. Why do this?

Any longer than 10 minutes is abnormal, should I not just inject once that has been breached?? Why wait so damn long...

You know, sometimes I think if I just ignore it, it will stop. If I don't think about the bleeding it'll just clot up and a few hours later I'll go - "Ooo, a blood free gob! How lovely." Very occasionally I find that happens, and a bleed I was previously aware of has miraculously gummed up of its own accord. Another reason to put off the prick.

Of course, should I be bleeding at a more profuse rate - period pace for example - I will get on with it. I don't sit around gushing. (Except over a really luscious piece of cake - and even then I'd stop to shoot up if I were bleeding over it.)

Mebbe it's just I judge the oozers as not bad enough to treat initially. They are just something I should accept and not treat unless they hit 12 hours? 24 hours? It's a valuable treatment - should it be saved for more extreme circumstances. Praps I'm not truly suffering unless I'm swallowing blood for a significant number of hours?

But why should I accept any excess bleeding even if it is an infinite dribble?

Just get on with it - shoot up and shut up.

Mucky Mush

This was the state of me when I woke up this morning.

Gorgeous right??

My tongue was more gory but it's rude to poke that out without a warning. This is the morning after a night on the towels. Actually it wasn't that bad. No towels required for drooling blood - just a cast iron stomach from gulping goo.

I have been doing prophylactic injections for my latest period but stopped those yesterday morning when things eased off down below.

It amazes me however how quickly things get back to normal, or in my case - way below normal. Last night my gums bled from when I brushed my teeth at around 10pm, until after I got up at 9am this morning. My lips weren't too bad really. They have been known to completely stick together, so much so that I would win any sponsored silence by a very clear length. This morning they were, as you can see, just a little bloody. The way yours might look after a few too many red wines. Not how I would like to answer the door admittedly but far from scary to passing passersby peeping through my curtains.

Luckily, as is sometimes the case, things seemed to clot off once I'd had my breakkie, mebbe it's the ice cold milk on my crispies?? I didn't need to shoot up this time.

And therein lies a common conundrum - to shoot up or not to shoot up - that is my question...

My Psycho Moment...

Got out the shower yesterday to find this little bleeder:


Not quite sure how that started - think it could've been Norman stabbing me in the shoulder with a pin, ee ee ee...

I kept mopping and it kept not stopping:


Managed to get me towel on as you can see - not that you'd have wanted to see otherwise!

My hair towel came in very handy:


Clothing meself proved a little tricky but got so far. Managed to locate and apply a number of plasters - three to be precise - but within a minute there was a leak:


Took myself off to have an injection. Sitting in the dining room in my brassiere hoping that the neighbour wouldn't choose that moment to gain access via our passage.

Blood drains from under me plaster
tissues blot it
don't bleed on floor
attempt to mix bottles of treatment
don't drip on table
mop it with tissue
fill my syringe
get blood on my hands
all over my arm
hope window cleaner isn't due
put on tourniquet
put tourniquet on other arm cos need to mop up bleeding arm
hold tissue on shoulder with chin
stick needle in
inject stuff
stop half way through
wipe blood up
start again
finish
take needle out
don't care if blood spills on table
or floor
wait
dab
still going
wait
dab
wait
dab
still going
take off blood soaked plasters
still going
put on clean plasters
clean arm with steret wipe
another steret wipe
another
wonder why didn't just have another shower
watch plaster
watch plaster
nothing.
No blood.

Hooorah.


Gum still going tho.

xx

On the first of December . . .

. . . my von Willies gave to me
a bleedi-ing belly button.



Hmmm.

Yes, I know - that isn't right. I'll have a jab and if that doesn't knock it on it's little button head I'll get it checked out.

Yes, I know - that is the Love Bugs nightie - sorry Louise :( but I'm sure it'll come out in the wash.

Half and half sleep / no sleep tonight - mebbe I'm excited bout Christmas - well it is December after all . . .

X

Clots of Love. . .

I'm back home and pleased to report that this was the only time I've had an op and not had any bloody surprises! No post op bleeding at all, as far as I can tell. By that I mean the pain has been containable, not unbearable, and there has been no major blood leakages from any orofi or entry points.

Woo hoo for "The Plan"! It worked.

Thank you to Dr's L and W for keeping a close eye on me and my levels and making sure I was topped up with lots of lovely platelets and juicy clotting factor.

The op went well and discovered that I had some endometriosis which they heat treated and an inflammatory cyst which they lasered. My tubes were clear tho which is good, always nice to have clear tubes.

Doc A who put the scopes in said he couldn't really see if I had a septum in my womb cos there was too much blood squirtage any time he touched anything, so I could've probly done with higher levels prior to the op but at one point post op my factor viii was 271% and my Ricoh activity 194 - that's damn good for a Type III.

Now just resting up with a very bruised belly, chomping pain killers, injecting for a few more days, under exerting and waiting for my next apt in six weeks time to find out a bit more about what they saw.

Thank you for all the texts, emails, blog dedications, letters and phone calls of support - very kind and very much appreciated :)

Love and clots.

X

Is Nail Polish a No No??

Tomorrow I'm off into hospital. I'm going for some internal investigations - a laparoscopy and hysteroscopy to be precise.

My gynaecologist has told me this is to look at the womb, tubes and ovaries, to deal with any ovarian cysts and to see if I have a septum in the womb. A septum is ably demonstrated in the sketch he drew for me below...

I think the little thing coming in at the top right corner is his hysteroscope, taking a look at the womb from above. Quite artistic don't you think?? I hope his handiwork inside me is a little more precise...

Not wishing to worry you but I had a d & c and laparoscopy when I was a teenager and it's not a particularly splendid memory with post-op bleeding into my womb and pelvis and weeks in the Duchess of York hospital in Withington having it all drained out time and time again.

But I am putting my faith in the haemotologists at QE and at the Women's Hospital in Brum, they are working together and have come up with (in a deep and dramatic voice) "A Plan".

This is good. "A Plan" is exactly what they need, what I need, to avoid any of the previously experienced blips - such as hitting a hepatic vein whilst doing a blind liver biopsy. Where they scan your side, mark the spot with an X and then stick a big old needle in to draw out a sample of your liver without actually being aware of what bleeding vessels are loitering under the surface ready to be ruptured.

That was a horrible time, more so for my husband and family than for me - I was just in pain and then in recovery in intensive care. The lesson was hopefully learned by my doctors that von Willies do not always do what you expect - in a bleeding sense of course - and from now on they do all biopsies on all bleeders via the vein so I'm told. In from the neck and down through the vein so there's no chance of any accidental damage.

The poor registrar who performed the biopsy was ever so apologetic and guilt ridden and really rather attractive and I'm sure he would have taken me out to dinner to apologise had Ade not been by my side during most of my stay.

So, my "Plan" (same voice) consists of taking my clotting factor levels after admission tomorrow - the day prior to the op. Then giving me enough factor to get me to levels of at least 100%. Then maintaining those levels by checking and, if necessary, topping up every six hours up to and beyond the op. Also they are going to give me a platelet transfusion before the op and tranexamic acid pills throughout - which should prevent any clots from breaking down, which as von Willie clots they are wont to do. I'll be kept in for at least 24 hours afterwards to monitor for any post-op haemorrhages.

Sound good huh?

Sounds like they are thinking about my treatment, anticipating problems and preparing for them, which to me is as good as it gets. I'm happy. I think Ade is, although he is bound to be worried because his only experience of me being hospitalised was the cock up occasion. Hopefully a straightforward time in hospital will restore some of his faith and ease his concerns slightly?

I guess I'm used to being in hospital, used to handing over control of my welfare to healthcare teams and not worrying about it. After so many years. I think if I was watching someone else having an op then I'd be far more worried about them than about my own - funny that.

When they ultrasounded me earlier this year to see if there was anything adding to my heavy bleeding the scan lady (radiologist?) thought I might have endometriosis. The gynae thought that it looked more like a septum in the womb - see artwork above. This is where the top wall of the womb is either extending down into the womb or is fallen down into the womb. Looking at it from below (laparoscopy) will show if there is endometriosis or a septum. Looking from above (hysteroscopy) will tell him if the top wall of the womb has grown down or fallen down. Then he can deal with both options. Does any of this make sense?? I'm hoping it will when he comes and sees me tomorrow!

I'm supposed to be in for about three days and they're doing it all under general anaesthetic which I much prefer, could do with a good sleep!

My main concern right now is - should I touch up my toenail polish for the procedure or do I have to remove all nail polish for the op - help, I can't remember??!!

I'll let you know how it goes...

xxx

Prophylaxis

Well, after some time and experimentation I thought we might finally be getting somewhere with my bleeding control.

I've been taking prophylactic injections to manage my periods ever since coming off the pill last year. My specialist initially advised me to try 1000 units of Haemate P every day while I was bleeding. Haemate P is heat treated factor VIII concentrate and one bottle contains 500 IU of factor VIII and 1200 IU of von Willebrand factor:RCoF (ristocetin cofactor activity - don't ask me to explain this, I'll look it up! This link has some info on the pathology of vWd: http://www.massgeneral.org/pathology/coagbook/CO006200.htm).

I can't have recombinant (synthetic) factor products because they don't contain any vW factor and wouldn't be of much use to me. What I need is the factor VIII, the vW factor and the RCof activity - all of which can be found in heat treated factor VIII concentrate. My specialist at the QE was confident that 1000 units per day would be enough to calm down the bleeding. I wasn't entirely sure. I've had problems in the past where - even with treatment - I continue bleeding; with gums and nosebleeds and when I've had surgery I have stopped bleeding, only to start again with a vengeance.

For the first eight months of 2006 I injected 1000 units, about 40ml once dilute, every day of my period and felt that whilst it might not have been quite as horrific as when I was a teenager, I didn't feel it was making a huge difference to the amount I was losing. It became hard to carry on at work because I was bleeding so often and so heavily. I kept having to run from my desk to get to the loo, excusing myself pretty sharpish whenever I felt anything flooding out. I carried a handbag everywhere that weighed a ton due to the amount of sanitary stuff in it.

At the Haemophilia Society AGM in September I spoke to a friend of mine who is a clinical nurse specialist, she said in her opinion I wasn't having nearly enough factor to control my bleeding, confirming what I thought. However when I spoke to my doctor he wasn't keen to do any testing of my levels - to really investigate how my factor levels were affected by the treatment. He did suggest that I try 1000 units for 7 days and then do 2000 per day after that until it stopped. His concern was that with increased factor my factor VIII levels would be going too high and that there could be other reasons why my periods were so heavy. Fair point I thought and tried the new regime.

The first month it seemed to really do the trick. 9 days of v heavy bleeding, clots and flooding as per usual but then after three days of 2000 units it slowly calmed down and then stopped. 12 days! Yippee, I thought, we've done it!

The next month it had exactly the same effect, 10 days bleeding in total - still heavy but sooo much better for my sanity and sanitary. Hehe!

The next month I hit 10 days with gusto, 12 days still bleeding, 13, 14 with no sign of stopping, until boxing day when I finally clammed up. What was the bloody problem now??

I was due to see my specialist again in January and I was intending to use my 'patient power' ....

Now I'm free ..... free flowing

Since I started having periods again last February I've bled for a total of 152 days out of 364. That's an average of 12.6 days per month, which doesn't sound too bad eh? That is 41.7% of the year that I've spent with a sack full of sanitary products on my person and preferably been within 15 minutes of a toilet.

I figure that the average woman without a bleeding condition could bleed for 7 days per month maybe, which is 84 over the course of a year.

It's ranged from 5 days in the first month to 21 days Jan 07, and so far 9 days this month. I haven't worked out my total spend on Bodyform, Always Ultra and Lillets but I'm sure it's enough to keep them in product development (other absorbant products are available - but are frankly less useful).

I'm not going to suggest that women bleeders should get sanitary prods on the NHS but boy would it be helpful! I'm not suggesting it because when I was in hospital with hideous periods as a teenager I was given unwieldy flannelette covered towels with strings to tie them into your pants, and I don't fancy going back to that thanks.

Anyway, I often find myself staring into a blood covered bowl that looks as if a small mammal has met with a nasty accident in there, thinking why am I doing this?

Anyone who has heavy periods will know that there are solutions out there:

• the hormone pill - which I used so successfully for sooo many years
• the mirena coil - many ladies I've met with von Williebrand's have told me how good this is, although with anything - it works well for some and not for others
• hormone injections - not sure if these are used for bleeders but have friends who would heartily recommend these to knock your periods on the head for three years

I have made a conscious decision not to use any of these things because my husband Adrian and I are trying to have children.

Together with my haemophilia specialist, I have been attempting to control my free flowing with prophylactic factor treatment. This consists of injections of factor VIII concentrate every day of my period. I am lucky because I am able to do these injections myself and can do them at home when convenient. I say trying to control the free flow because even with daily factor injections I was using Super Plus and Super Plus Extra (supersized tampons), with large ultra towels (with wings, obviously!!) for the majority of those 152 days last year.

I will do a running total of how many san prods I use every day if you like? Vote A for 'yes, we want a running total' and B for 'no, shuddup with the tampon talk'.

Gotta go to the loo now, I have a leak ........