New Beginnings

Happy 1st May / Beltane Blessings!

beltanealtar2017

I thought I’d better brush aside some of the tumbleweed blowing through this place, after only posting once in the entire month of April.  In my defence I’ve been very busy, but I look set to get even busier in the coming weeks so I thought I’d better at least say hello and give a brief update for anyone who’s interested, before disappearing again for a few weeks!

This time last year I was on the verge of some big changes, and had some major decisions to make, so on the 30th April, aka Beltane, I lit some candles, got myself a cold beer and sat cross legged on my bed and dug deep to figure out what it was I wanted and needed to do in the coming months.  Looking back now, I can see how important those decisions were, and appreciate how brutally honest I was with myself that evening.

So again last night I did the same thing, (except, switch beer for wine, and add some dubious-smelling incense into the mix), and this morning I’m feeling good about the challenges that lie ahead.

This summer I’ll be starting a new job (probably within the next couple of weeks), starting therapy (this Wednesday) and starting a brand-new writing project  The job is back at my old hospital, although in a very different role, the therapy is high-intensity CBT, and the writing project is a young-adult fairytale with a twist.  All of which I’m pretty excited about (well, I don’t know that I’d say ‘excited’ for the therapy actually, but…’positive’, maybe?!)

Between those three things I’m not sure how I’m going to have time for much else to be honest, but I will try to share as much as I can about my experiences, in case it helps anyone else.

I’m still posting my YA contemporary over on Wattpad, and I have a loyal fan base of about ten readers (most of whom are friends/family) who are consistently reading every chapter update and telling me they’re enjoying it, which I think is all most of us really want when we share our work, so I’m calling it a success so far.  I’ve also been pushing myself outside my comfort zone in other ways, like entering writing contests, submitting short stories for anthologies, and taking up burlesque dancing!

My CIDP continues to be well-managed and I’m hoping to reduce my dose of immunoglobulins over the summer months, and my PCOS is also behaving, so for once I don’t feel like I’m using up half my energy in a battle of wills with my own body.

Generally, aside from crippling anxiety, (which the therapy will hopefully help with), and a distinct lack of money (which the job should surely help with), life is good.  (Although obviously I hate typing that for fear of jinxing myself in some way, damn anxiety.)

 

 

 

 

 

 

 

on being (begrudgingly) realistic

I am not renowned for realism, it has to be said, and so in cases where it is required I am a late-adopter, holding out hope long after it ceases to be sensible to do so.  That’s pretty unusual for someone with such high levels of anxiety I think, and at odds with my general belief that EVERYTHING IS GOING TO GO WRONG…and yet I hope, and hope that it won’t.

I particularly hate the need to be realistic when it relates to my chronic condition.  Even when I was really quite acutely unwell and newly diagnosed I was all “I totally can shower myself!” When it was quite plain for all to see that I could barely lift a spoon to my mouth to feed myself, never mind transport myself to a bathroom and attend to my hygiene needs without falling down flat on my face.

I was like ‘the little engine that could’, and this was my face whenever it turned out that actually I couldn’t:

pissythomas

 

(Incidentally, if anyone ever requires an image of a pissed off looking Thomas the tank engine- there are loads to choose from.  The dude has issues it would seem.)

 

Four years later, honestly, not much has changed.  I still like to think I can do everrrrrything, and you don’t want to be around me when I find out that I can’t.  I am getting better at accepting my limitations (I think?…Ok, maybe not…) but still don’t often fully realise them as part of my self-image and awareness.  This leads me to do things like apply for jobs that are actually beyond my physical capabilities.  That’s not a random non-specific example, that’s an actual thing that I did last week- securing myself an interview for a job that in reality, after considering it at length, I probably can’t actually physically manage- at least not reliably anyway.

Honestly?  It totally sucks.  Every time I tell myself that I can DO WHATEVER I WANT, and then struggle to drain a pan of pasta, or open a can of beans, or fasten a set of buttons without looking like I have the DT’s, I am reminded that actually Positive Mental Attitude is only a tiny part of the battle when it comes to life with CIDP, and that at the end of the day- it’s my nervous system that’s in control, not me (terrifying for anyone, but particularly a control freak like myself.)

But I decided that the right thing to do in this case, even though it felt pretty miserable, was to step back, and decline the post.  It’s one thing for me to be affected by CIDP, when I’m having a bad day or staring down a potential relapse, but in a job role where someone else would be physically dependent on me, it *wouldn’t* just be me that was affected.  So there it is.  Sometimes you want to do a thing, and think you can do the thing, but you actually can’t do the thing, and it’s better to realise it before you’re committed to the thing.

So that’s where I’m at.  Back in Job Search Hell.  I’m trying not to panic or feel too sorry for myself, because really what will that achieve?  But on the other hand I’m definitely feeling a bit sobered by the slowly dawning realisation that shit, I really am stuck in this malfunctioning body hey?

A Bad Attitude is Really Really Not the Only Disability in Life.

Can we talk about this?

theonlydisabilityisabadattitude

Like, no.  Just, no. No no no.

If you’re short on time today then feel free to stop reading now and get on with your afternoon, because to be honest- that’s a pretty good summary of what I’m about to say.

I thought I’d blogged about this issue before, but a quick trawl through old posts didn’t turn anything up, so possibly I *thought* about blogging about this before, but then bit my tongue.  Hard.  The way I frequently do.

But I’m feeling a bit, umm…sensitive at the minute.  After a week in which the kids went back to school and I went back to my fitness regime (that had given way during December to evenings under duvets and increasing volumes of Baileys), my body is protesting, I guess you’d say.  Not quite dramatic enough for me to bust out the word ‘relapse’ but enough to mean that I am a quivering wreck- literally.  My hands have been shaking near constantly for about three days, which leads to anxiety, which leads to panic attacks, which leads to adrenaline- which REALLY helps*, obviously.

(*sarcasm klaxon)

And the thing is, throughout all this, I can’t get this fucking meme out of my mind.

It’s not the image.  Let me repeat, for the cheap seats in the back: IT’S NOT THE IMAGE! I don’t know why I’m even bothering to emphasize that, knowing full well there’ll still be someone out there who thinks I am bitterly opposed to such a powerful image of a disabled person displaying power and strength and all the things we’re told by society, a disabled person can’t have/be. It’s an incredible image.  My problem is not with the image.  My problem is with the slogan someone has helpfully superimposed onto it.

 

‘ The only disability is a bad attitude’

Really?

Really?

*raises eyebrow until it lifts off my forehead and floats off into fucking space*

Anybody who thinks that the only thing preventing people from accessing public spaces, education, work opportunities, social events and from taking care of their activities of daily living is their attitude, should refer to the diagram below:

wheretoputyouropinion

Most people reading this will know why this kind of able-ist bullshit bugs the crap out of me, but in case you’re one of those who doesn’t- here’s the deal:

Three years ago I went from being a busy young mum of two small boys, working part-time nights as a nurse on a neonatal unit, running (ok, jogging) 10ks and generally ‘leading a normal life’ to lying in a hospital bed, largely unable to move.

But the only disability in life is a bad attitude, right?! So I got my shit together and got me the fuck outta that bed and GOT ON WITH THINGS.  Because really, it was only my bad attitude holding me back, amirite?!

Umm…no.  You see what was actually holding me back, was my body.  Specifically my immune system, which had decided my nervous system was a foreign invader and begun stripping all my peripheral nerves of their myelin sheaths.  Don’t know what a myelin sheath is? Well, let me tell you- they’re important af.  Without them, your nerves can’t transmit signals .  So, to be clear- my brain was like: LET’S STAND UP

And my legs were like: …………..

*neurological tumbleweed*

 

This kind of message, this idea that anyone can overcome ANYTHING as long as they’re DETERMINED, sounds very aspirational and wonderful, but there’s one teeny tiny problem- it’s not true.  And it’s damaging.  It’s damaging because it makes people- vulnerable, scared people who’s lives might be falling apart, who might be in pain and terrified feel RESPONSIBLE FOR THEIR PROBLEMS.

I know, because I literally sat on my toilet, stared at my legs and thought “Maybe I just didn’t try hard enough last time…come on legs, we can fucking do this.”

And guess what happened?

Nothing.

Of course.  Because pure longing doesn’t actually repair biological damage.

I also know because I told myself, in the brief time I spent at home deteriorating rapidly, that I CAN DO THIS.  I SHOULD BE ABLE TO DO THIS.

THIS being: EVERYTHING.  Driving (yes, I cringe now but I drove around Manchester unable to feel the soles of my feet, unable to change gear one-handed.  I could have fucking killed someone but hey, got to admire my can-do attitude right?  NO.)  Looking after my kids- even though I couldn’t lift my one year old. Dressing myself- even though my hands shook so badly I couldn’t fasten my bra. Walking down the stairs- even though I fell, multiple times.  Yes one fall down the stairs was not apparently enough to convince me that I could not actually ‘do this’.  And what’s even more terrifying is that I was pregnant.  Pregnant falling down the stairs.  We all know how this ends of course, i.e. not well.  It turned out, to my relief that those falls- particularly the one bad fall I had, hadn’t actually caused my miscarriage, and that the pregnancy was doomed from the start.  But I didn’t find that out until a month later.  A month is a long time to carry that burden of guilt.

Able-bodied people (because to be honest, I’m pretty sure that’s who images like this are for- to make able-bodied people feel GOOD and INSPIRED and MOTIVATED etc) suggesting that anything can be overcome with the right mentality are not only grossly mistaken but also contributing to the societal idea that disabled people come in two categories:

1.Wonderful celebrated specimens of humanity- patient, humble, kind, achieving above and beyond what seems possible even for able-bodied people.

2. Bitter, twisted people who ‘let their disability rule their lives’.

We all love “Doctors told me I would never walk again but I did!” stories.  Not so much “Doctors told me I would never walk again and actually they were right and I still can’t walk but hey guess what I’m still a valid fucking human being and actually not your motivational piece” stories.

Disabled people, sick people, chronically-ill people are told “you don’t look ill/disabled” and on the face of it- yes it’s a compliment.  Who the fuck wants to be told they look like they’re about to dodder off this mortal coil?! But on the other hand, it’s a bit of a backhander isn’t it? “You don’t look disabled” i.e. “Well done hiding your disability.  God it would be awful if us ‘regular’ folk were confronted by the notion that we’re all just one biological failure away from disability.  Thanks for keeping it tucked away there, sport.”

I love an inspirational picture/article as much as the next person.  I love stories where people overcome barriers- regardless of what those barriers may be- to achieve things that are important to them.  I don’t want people to stop pushing themselves, to stop shouting from the rooftops when they achieve things that they, or other people thought impossible- whether that’s pull-ups in a wheelchair or just wiping your own ass when you’ve previously had to rely on others to do it.

But let’s not kid ourselves that pushing ourselves- that Positive Mental Attitude is the only thing that’s required.  For disabled and chronically ill people to achieve their full potential- their personal, individual full potential- not the dreams and goals YOU set for them, but their own, a can-do attitude is only a very small part of what’s needed.  Societal acceptance, and wide-scale change is also pretty essential.  Psychological input, a strong support network, and acceptance that some things might not be possible.  Reassurance that even if it turns out you’re NOT capable of pulling yourself up in your wheelchair, or indeed wiping your own ass. that you’re still- shockingly- a valuable person, who deserves to live and is worthy of time, and space and respect.

Of course, my story falls into the “happy ending” category- so far at least because I did walk out of the hospital.  It took time, but I hobbled out on crutches and now my remaining crutch waits in the hall, for a day in the future when I might need it again.  Because the reality is- it isn’t a “happy ending” until The End.  And I’ll be living with CIDP for the rest of my days, and I’m really hoping I have a lot of those left.  There’s no guarantee I won’t deteriorate, that I will remain ‘inspirational.’ Will I be less worthy as a human if I can’t ‘perform’ physically, if I can’t contribute to society in the only way it knows how to measure- labour and profit?

So when I see able-bodied people sharing the above meme, complete with caption (note, not the stand alone image) you should know that I don’t think “how wonderful that you’re celebrating this man’s achievement” I think “Do you even know what the fuck you’re talking about?!”

And if that makes me over-sensitive, well it wouldn’t be the first time I’ve been accused of it.  And perhaps you’ll be better able to understand my ‘sensitivity’ when I tell you that I had a panic attack in my bathroom this morning because as I brushed my teeth I caught sight in the mirror of how badly my hand was shaking, and from there it was only a brief leap for my brain to the memory of when I first bought an electric toothbrush (summer 2013) because I no longer had the strength required to move a regular toothbrush around my mouth.  Yes, I was really that bad.  And it only got worse- the electric toothbrush was a temporary fix, but as my condition deteriorated it became too heavy- I had to use two hands to hold it, and then eventually the concept of brushing my teeth unaided became a pipe dream.  But throughout it all, of course, I maintained my positive mental attitude- which only served to make me an utter bitch to be honest, because I felt I should be able to do all the things I couldn’t and if I couldn’t….well I just wasn’t trying hard enough. That kind of pressure is destructive.  Just ask my boyfriend who bore the brunt of most of my outbursts

“I should be able to do this!”

“Why can’t I do this?!”

“I don’t want to be like this”

“This can’t be my life”

It’s hard enough to accept you’re not in control of your own body, life, future, without being expected to OVERCOME YOUR BARRIERS and FIGHT YOUR DEMONS and ALSO BE INSPIRATIONAL FOR ABLE BODIED FOLK TOO PLEASE.

So please, stop with the able-ist propaganda.  Yes some disabled and chronically ill people might put your complacent asses to shame, but others might be struggling to exist without help, and perpetuating the myth that ANYONE can overcome ANYTHING if they only want it badly enough, is not just insulting, it’s damaging.

 

 

 

 

One Year On SCIg

As my one year anniversary on SCIg approaches, I thought it would be appropriate for me to do a post about what life has been like these past 12 months, and how doing my own subcutaneous immunoglobulin infusions (which is what SCIg stands for) at home has impacted my life.

scig

You may remember that prior to SCIg I was on regular IVIg, which I had in hospital once every four weeks, and was truly amazing and improved my life in so many ways.  But you may also remember some of the pitfalls- having to spend one day every month in hospital, having nurses and doctors digging around for a decent vein, bruised arms, headaches afterwards unless I’d managed to drink the required 2 litres of water during the infusion, and of course the inevitable dip in my mobility and increase in symptoms in the last few days of each month before my next infusion was due.

Well…with SCIg, there is none of that!  It’s been fantastic.  I used to have 80g of Privigen every four weeks, so initially I was started on 80g of Hizentra (the subcut version of Privigen) across the month which I have been doing as two infusions a week (i.e. 10g twice a week over a month= 80g).  However in December I had a massive increase in symptoms, and although it could have been coincidence (fostering-related stress, being run down and having a cold virus etc) I was really worried that I was relapsing.  So my lovely neuro specialist nurse increased my dose to 12g twice a week, which I know doesn’t sound like much of an increase but that equals 96g of immunoglobulins across the month, which is an extra 16g on top of what I was getting before, and already just two months in to the new prescription I am seeing results from that- with an increase in energy and a reduction in nerve pain, tremors and jerks (all good things!)

There are downsides of course, like there are with everything.  Instead of someone else jabbing a needle in my arm or hand once a month, I am stabbing  four tiny needles into my belly with every infusion- so stabbing myself 32 times a month.  But there are people on insulin and other medications jabbing needles into themselves every day, so I try to keep it in perspective.  Also, being the one in control of when the needle hits, and having an abundance of flesh to choose from (haha…boohoo) is preferable to someone else rooting around in the crook of my elbow for a viable vein.

We’ve had to find ways to store my medication and all the associated STUFF that comes with it (and there’s quite a lot).

stockingup

I get a delivery once every 12 weeks and it takes me maybe an hour to unpack and check that and put it all away, making sure to rotate the remaining stock so that I don’t end up with out of date human immunoglobulins stuffed at the back of a drawer.  I had some issues in the summer with not being able to keep the room cool enough (Hizentra needs to be stored at room temperature) but a fridge thermometer and a fan have helped, along with moving the drawers that my medication is house in, into a shadier area (as in out of the sun, not further along the A6).

Some nights when I realise I need to do my infusion, which takes about two hours from start to finish, I just feel like I would rather do anything else on earth.  That proper petulant child part of myself that I rarely allow to the surface comes stomping out, and I just want to fling myself on the sofa and say I CAN’T BE BOTHERED and possibly even whine WHY ME?  But I always shake myself mentally and remind myself that back in the early days of my diagnosis, this is what I dreamed of.  Seriously, to be almost symptom-free most days and doing my own treatment in my own home at a time that suits me (usually in the evening, watching TV in bed) that seemed like a FANTASY.  Now it’s my reality, and it’s easy to slip into taking that for granted and seeing only the negatives (it stings, it’s time consuming, I would rather be doing x, y, z etc) rather than the positives (it works, it has almost no negative side effects and it’s goddam FREE on the NHS).

eveningplans

So overall- my first year on SCIg?  It has been life-changing, and I say that without hyperbole (for a change).  I feel so lucky.  And no, I don’t always manage to hold that feeling, when one of my infusion sites blows and I have what is essentially a mix of thousands of other people’s sticky immunoglobulins seeping out of my belly and all over my clothes and/or bed sheets then yes I think “FML” because I’m only human.  But the majority of the time, I feel incredibly blessed and grateful because I know how fortunate I am to have this treatment freely available and that it works so well.  So well that there will probably even be people reading this who had no idea I even need it 😉

So here’s to a year of FREEDOM, and if not good health then at least well-managed illness, and may there be many more!*

(*Providing the tories don’t dismantle the NHS that is…but that is a separate blog post ENTIRELY)

The Trial

Back in August when I wrote “One Year On”– a blog post marking the 12 months since my first hospital admission for (what we now know to be) CIDP, I mentioned a clinical trial I was possibly going on.

Back then, it was just a pie-in-the-sky “this maybe might happen at some point” kinda thing, but last week I finally got The Call from a research nurse, to let me know that they were now recruiting patients and to ask if I was still interested (the answer to that being a definite YES!)

See, the trial is to see if CIDP can be successfully treated with subcutaneous immunoglobulins (SCIG) at home, rather than intravenous immunoglobulins (IVIG) at hospital.  The idea of being able to administer my own medication in my own home sounds so good that ever since my consultant mentioned the possibility last summer I’ve been dreaming of the day when it could be a reality.  So when the research nurse asked if she could send me some more information about the trial itself, I was delighted.

Unfortunately, that was where my elation ended.  As when the information itself arrived, my happy-feelings quickly went away.  The trial itself is lengthly, in fact it’s really of an undetermined length as there are three phases and the first two are entirely dependant on you as a participant, or rather- your body/condition.  The final stage being a set 3 months.

There’s a lot to it (obviously, as it’s a clinical trial!) but I’ll try breaking it down very simply.  Basically first up, they need to be sure you are actually reliant on immunoglobulins as a form of treatment, so they take you off them (eeeeeeeeeeeeeeek!) and then when it turns out that you really do need it, they re-stabilise you, but rather than with your usual product/dose/infusion, it’s with the SCIG, and then, when you’re stabilised (however long that takes) they assign you to one of three groups, either low dose SCIG, high dose SCIG, or a placebo (eeeeeeeeeeeek!) and then after three months it’s game over and you’re back on your usual IVIG in the hospital.

Now some of that, I already knew.  My consultant had pre-warned me that they would need to be certain the participants actually NEEDED the immunoglobulins, so I figured there’d be a period where I’d be required to go without, and I also knew that I’d need to have some of my investigations repeated, like the electric shock torture, I mean, err…Nerve Conduction Testing…so some of it wasn’t a surprise, but a lot of it was, and not in a good way.  There was a lot to read through, and it laid it all out bare how many extra hospital visits it would require and how much upheaval and disruption it would potentially cause, and by the end of my first read through I was already thinking:

NOPE.

I feel so guilty in a way as I think it’s a fantastic study, and the results could have amazing implications for the future treatment of others with my condition, but I just can’t justify turning my life (and the lives of Chris, and the boys, and potentially a foster child!) upside down to take part in something with so much uncertainty when it’s going to have such a big impact.

So I went to see my neuro specialist nurse yesterday as I had a routine review appointment with her anyway, and the research nurse was with her, and I was just very honest with both of them about how despite my initial enthusiasm, I’d changed my mind, and they were both very understanding and generally lovely and made sure I knew that I was under absolutely no pressure whatsoever to take part.

My actual review went well- my scores are higher than they were in the summer, which is apparently unusual as generally us CIDP sufferers are worse in the winter (I do find I have more nerve pain and tremors, at the minute but I’m nowhere near as lethargic as I was in the hot weather, so…?) and then the topic turned to my treatment and what to do moving forward.  I still get a dip at the end of each month, it’s not as bad as it used to be, but there are still those last few days before my next infusion where I’m a bit shaky, a bit weak, generally lethargic, and well…a bit naff all round.  So the question was- can my dose be increased?  The answer being yeeees, but a tentative yes, because I’m already on 80g of privigen, so the next logical step would be 90g or even 100g, but those kinds of doses can’t be given over one day, so I’d be back to where I was at the start of last year when I had to spend two consecutive days each month at the hospital (not ideal, obviously).

In the end, my lovely specialist nurse called in my equally lovely consultant who’s answer was: “SCIG”

Which, I’ve got to say, confused us all, as the three of us all assumed he was talking about the trial.

“No, I mean, just SCIG”  he said.

Turns out, aside from the trial, a small number of patients are being started on SCIG at home already, as the small amount of research there is (mainly from other countries at this stage) is showing- it works, and is better for everyone involved.  So he wanted to know- did I still fancy it?  Without all the hoops of the trial to jump through, just- get trained up, and get going with it, doing it myself at home?  Errr….YES!!!!!!!!!!!!!!!

So that’s what’s going to happen.  I don’t know when yet, but I do know it will likely be quite soon, which is super exciting.

I know I’ve said this before, both here on the blog, and over on facebook, and probably on twitter, and definitely in real life too, but I’ve got to say it again: I really am SO lucky, to have been diagnosed so quickly, to have found a treatment that works so well for me, and to have such a fantastic team of professionals taking care of me.  It’s days like yesterday that really hammer that home for me- I go into an appointment feeling anxious, and come out feeling reassured and really positive about the future.

 

JanuaryIVIG

(That’s me having my infusion 2 weeks ago, it was about 3 hours late due to the ward being busy- something that won’t be a problem when I’m doing it myself- yay!!)

CIDP and me

It’s been a while since my last post, and an even bigger while since the one before that. I don’t really know what I’m doing with this blog anymore, it started out as an attempt to maintain a slippery grasp on my sanity as I embarked upon parenting two children under the age of two, and here I am, with my boys both over the age of two now, finding myself writing about my life as it spirals out of my control thanks to a crazy thing called CIDP.
I realised yesterday that I haven’t ever really explained to some people what this condition I’ve been diagnosed with, and is quickly taking over my life, actually IS so I thought I’d give it a shot.
CIDP stands for Chronic Inflammatory Demyelinating Polyneuropathy.  It’s an autoimmune condition, which means my body is doing this to itself. No one knows why but the most likely scenario is that at some point last year my body was exposed to a virus, and for reasons unknown, when my immune system came to attack that virus it became confused and started attacking itself instead. More specifically attacking the myelin sheath that covers and protects the nerves in my peripheral nervous system, which causes them to function incorrectly, or stop functioning altogether.
Now before I lose all but the medically trained of you, with all this talk of myelin sheaths and whatnot I’ll explain what that means in real terms.
It means that without any treatment I would likely be totally paralysed within the space of approximately 12 weeks. The closest I have come to that is when my symptoms first presented and were thought to be a mild version of the acute form (Guillan Barre Syndrome). Within 8 weeks of waking up with a dead leg, I could no longer get out of bed or go to the toilet independently, I couldn’t walk without the assistance of 2 people and I couldn’t lift my arms to a 90 degree angle, meaning that washing and dressing myself were pretty much out of the question also. Even very simple things like feeding myself or changing position in bed were difficult and exhausting.
There is no cure for CIDP, it won’t ever go away, but it can be ‘managed’, which is to say that there are treatment options- obviously, as here I sit, typing away with a cup of tea next to me that I walked to the kitchen and made all by myself. Unfortunately, none of the treatment options are straightforward, or guaranteed to work.
So far, there is one option that not only works for me but works fantastically well, enabling me to live an almost ‘normal’ life for 3-4 weeks at a time. That is Intravenous Human Immunoglobulin Infusion or IVIG.

So far I have had three courses of IVIG, in October, November and January and responded brilliantly each time. Yes it has meant being admitted to hospital for between 4 days to a week on each occasion and yes it is an invasive procedure that isn’t without risk but is it worth it to be able to walk out of the hospital unaided each time following it? Hell yes!

Many people with CIDP have regular IVIG on a long term basis. I say many, it’s so rare there really aren’t that many of us to begin with (with between 1 to 4 people in a million newly diagnosed each year) but of the few of us that exist, a large proportion rely on IVIG.
The bad news is that there is an international shortage of immunoglobulins, and not only that but it’s expensive. And I mean, really really expensive.
How much would you pay for your life? What’s it worth to you to be able to get yourself out of bed in the morning and put on some clothes? To what value do you hold being able to walk, drive, go to work? What price would you put on being able to pick up your own children for a cuddle if they wanted one?
What if the number was more than you could ever hope to afford? What if your only chance was if an organisation such as the NHS agreed your life was worth it?
So far I have had 370g of immunoglobulins, at a cost of £19,980.
That’s just the cost of the medication itself, not the cost of the IV giving sets, the pump, the trained nurses, an obs machine, an overnight stay in hospital, 3 meals a day etc…just the immunoglobulins. Just the thing I need to walk, to move, to carry on leading an arguably ‘normal’ everyday existence.
If I were to have a maintenance dose of IVIG once a month it would cost the NHS £4,320 a month.
How many of you think I’m worth it now?!
It’s one of the NHS’s biggest expenditures. So (understandably I think) there are strict criteria to fill if you’re going to be approved to have it as a long term therapy. The main criteria being obviously a diagnosis of CIDP (or one of the other approved conditions) but also that you have tried other (read: cheaper) treatment options first, namely corticosteroids.
There has been some recent evidence that suggests ‘pulsed’ (which is really just a fancy way of saying ‘intermittent’) steroids, if taken over 6-8 months, can cause a period of remission in patients with CIDP. For anyone who doesn’t really know what that means, it would mean that for a certain length of time (maybe months, or even years) I would neither have any symptoms of CIDP nor require any treatment for it. Which would, as I’m sure you can imagine, be fucking awesome.
So it was for that reason that I spent the last few days of December drugged up to my eyeballs on a hefty dose (40mg per day) of Dexamethasone, one such steroid. I had to take 20 tablets per day between Christmas day and the 28th December. Fortunately they didn’t kick in immediately, but when they did, boy did they!
I basically spent 5 days in a Dex-induced fog, in which I found it hard to concentrate on anything, kept dozing off during the day but couldn’t sleep at night, felt very ‘spaced’ and ‘out of it’, felt starving but also nauseous. I had awful acid reflux and indigestion (despite also being started on a PPI called Omeprazole, which is a medication designed to reduce the amount of acid in your stomach and thereby prevent or minimise acid reflux).  My fingers and toes swelled and felt like hot, numb sausages on the ends of my hands and feet, my face rounded and became flushed, I had headaches and pressure behind my eyes  and my vision was altered, my legs felt like they were made of wood, and every single motherfucking muscle in my entire body ached like I had been beaten within an inch of my life, especially my neck and shoulders. On the whole, it was one of the least pleasant experiences of my life and not one I care to repeat, certainly not at monthly intervals.
I had hoped, that having given the steroids a go, I would be considered for funding for long term IVIG, but on seeing my consultant the day before yesterday, I learnt that in order for that to happen, more hoops must be jumped through and the next hoop is Prednisolone. Another member of the delightful steroid family (I just can’t wait to find out how strong the family resemblance is- NOT!) I will be taking 40mg but this time every single day (rather than for 4 days out of the month then a break) for the next 8 weeks.
If I deteriorate twice within that time, needing further IVIG alongside the prednisolone then my consultant is satisfied that proves I am a steroid non-responder, meaning my chances of being granted approval for regular IVIG are good. If however the prednisolone works, and actually improves my CIDP and keeps me walking around like a regular person, well then I’ll be on it forever and ever, for the rest of my life.
Sure, over the years it will turn my skin to tissue paper that breaks and weeps when so much as a cashmere fucking jumper brushes against it, and sure my head will look like a beachball on top of my body which will look like a PLANET, and sure it will cause my bones to crumble and break when I so much as stand up but HEY, at least there’s a treatment…right?!…
You may have guessed I’m unenthused by the outlook of the rest of my life on steroids. Over time I would be weaned down from 40mg, to a dose that still worked but minimised side effects (possibly as low as 5 or 10mg daily) but I still don’t relish the idea of being on something so potent for the rest of my life long days. I am intending (or at least fucking hoping!) to be around for quite some time yet, and I’m only 29 for Pete’s sake.
The fact is, all my despair at the idea of another 40 years on steroids may turn out to be for nought anyway, as if the prednisolone doesn’t work, well then I will be facing the rest of my life on IVIG instead. So far I’ve had no nasty side effects from IVIG, barring one hell of a headache when my infusion rate was increased (I’ve learned the trick to counter this is to drink your entire body weight in water, seriously, just keep asking nurses, support workers, students, or anyone who walks by your bed, to fill that water jug up!) That doesn’t mean I won’t ever get any nasty effects from IVIG though. Everyone responds differently, and although they try to keep you on the same product, from the same manufacturer (I always have Privigen), you can respond differently each time.
Some people are able to increase the length of time between treatments over the years, stretching themselves from a 5 weekly cycle, to perhaps a 6…7…or 8. Other people start out needing it every 5 weeks and then wake up one month and find they’ve deteriorated at 4 weeks since last infusion…or 3, and for some people it stops working altogether and they need to try something new (There are a couple of other treatment options that are less common that I haven’t mentioned here because, frankly, I don’t know much about them and am unlikely to need them anytime soon since I’m such a HUGE IVIG success story).
Right now, just 9 days since my last dose of IVIG, I am feeling good. I can walk around, get up and down stairs, shower and dress myself and all that jazz. I can be left alone to take care of my children, and we can get out, to the shops and the park. Hell I can even drive (although that is always the first thing to go, when I do decline, as the idea of being behind the wheel and losing control of my limbs, is, I’m sure you’ll agree, shit scary).
The hard bit is knowing it won’t last. That I have about 2 more weeks of this before I start to become fatigued. Then after a week of being functioning but exhausted begins the slow decline…the pins and needles return, usually in my hands, then the twitching begins, where my nerves are misfiring electrical signals causing muscles in my feet/legs/arms/torso/face to pulse and/or visibly twitch.
From a mobility point of view, first up the stairs will become an obstacle, getting up them will feel akin to scaling Everest every time I need a wee, then getting down them safely will become an issue. I will start to struggle when I try to get up from kneeling, or squatting, and need either a hefty piece of furniture, or a sturdy person to pull me up.
Within days I will be walking like a pirate with wooden legs, not bending them at the knees at all (or I’d fall!) and eventually Chris will persuade me I should be using my crutch, and out it will come from it’s hiding place behind the coat rack in the hall. Within a couple of days again I won’t be able to lift either of the children, or anything heavy or cumbersome at all. It only takes a few more days for me to become ‘unsafe’ to be left in charge of my own children, as I won’t be able to necessarily get from sit to stand so can end up literally stuck on the sofa. And since I can’t walk or drive at that point, if anything were to happen, I wouldn’t even be able to get them to a Dr, or hospital, or whatever they might need. So then Chris has to be off work to effectively ‘take care’ of all 3 of us.
So you see how, slowly but surely it manages to take over. And of course at some point along the way, someone, my consultant, or my GP, decides enough is enough and I am readmitted to hospital, and after just 60g of IVIG I am usually off my crutch and mobilising safely, and certainly by the time I’ve had the full 110g I am pretty much back to baseline and feel like I’m ready to take on the world again.
And then it’s just a case of rinse and repeat.
A lot of people have expressed horror at the idea of being reliant on immunoglobulins for the rest of my days, and to be honest- it aint my idea of sunshine and lollipops either.
If I were approved for regular IVIG long term though, those last couple of weeks, where my body stops working and life falls apart a little, would cease to exist. Instead, just prior to my decline I’d go to Salford where I’d receive 80g of Privigen as an outpatient (i.e. sit hooked up to an IV for a day, come home and go to bed then go back and do it again the next day) and that would be me done for the month. So no it isn’t ideal, but neither is the current situation of being admitted to hospital every 6 weeks or so, and neither are the steroids with all their nasty side effects, and neither is the entire situation or the diagnosis or any of it.
Ideal is not an option anymore. The only option is coping, surviving…carrying on. So that’s what I’m trying to do.