Wednesday, 15 January 2014

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.


8 comments:

  1. Hi Rebecca,
    Just read your blog. I've just been diagnosed with CIDP and am currently sat in bed in Salford Royal having started IVIG yesterday so hoping to see benefits of it soon. I have been in here 10 days now and desperate to escape but scared of how will cope at home.
    How long did it take for you to start seeing benefit when you had your first dose?

    ReplyDelete
    Replies
    1. Hi Lisa, I'm so sorry I've only just seen your comment as this is my old blog so I don't check on it very often! How did you get on with your IVIG? I really hope that you saw some improvements after it. For me, it worked really quickly. The first time I had it I went from needing help with EVERYTHING, to being able to stand unaided within two days, it was very quick. Although from what I understand that is quite rare and just because you don't get an instant boost doesn't necessarily mean it isn't working for you (if that makes sense?) Anyway I'm really hoping that you're doing well. I'm still under Salford Rpyal but only there every 3 months now as I'm doing subcutaneous immunoglobulin infusions myself at home. I've just been writing about it on my new blog today which is why I've ended up checking over here and seeing your comment! The new blog address is www.rlholland.wordpress.com and I'm a lot better at replying over there honestly! Apologies again about the massive delay.

      Delete
  2. Greetings. I included your blog and or video in my website. It's a list of first-person accounts of people who are battling GPS and its variants.

    http://whatyourdonotknowbecauseyouarenotme.blogspot.com/2015/12/what-you-do-not-know-if-you-do-not-have.html

    ReplyDelete
    Replies
    1. Hi Michael, thanks for including me on your list of GBS/CIDP related blogs :) This is an old blog, which I don't post on anymore (although obviously the archives are here for anyone to read who is interested) but I blog regularly over at www.rlholland.wordpress.com and can respond a lot quicker to comments over there too as I am notified about them straight away, so if you'd like to include that on your list then you're very welcome to.
      Thanks again for stopping by :)

      Delete
  3. Hi, i feel your pain. I am day 4 post 2nd dose of pulsing dexamethasone, you describe the side effects perfectly! I was diagnosed with CIDP early December and unfortunately due to antibodies in my blood, was not suitable for Ivig. Plan is for me to have 6 months of dexamthasone (not sure if I can cope with these symptoms for 2 weeks of the month) I so far haven't had any real improvement, but with no alternative I have to continue. At 44 years old this was not on my agenda.......oh and work are asking me when I'll be back!

    ReplyDelete
    Replies
    1. Hi Wendy, so sorry to hear you're struggling with the pulsed dexamethasone too. I believe that for those who it works for, the overall result- i.e. going into remission, must be so worth it in the end. But for those of us who just get every negative side effect and none of the positives it's a nightmare! :( I hope that you start to see some improvement in the coming months. It's such a shame that you aren't able to have IVIg, does that also rule out SCIg for you too (subcutaneous immunoglobulins) do you know? I only ask because that's the treatment I'm on now and it's brilliant. I've just been blogging about it today actually as I'm coming up to having been on it for a year now.
      This is my old blog (as you can probably tell as it hasn't been updated in a while!) but my new one is over at www.rlholland.wordpress.com and I do blog about CIDP-related stuff still fairly regularly.
      I hope work aren't putting too much pressure on you- I think a lot of people don't understand how unpredictable this condition can be and how long it can take to find something that helps.

      Delete
    2. Hello Wendy, I have had CIDP for 17 years. I did IVIG for most of those years. But I read your Reply. I saw that you can't have IVIG. Have they said anything to you about Plasma Pheresis? It is the best way to deal with CIDP. It circulates your blood, while doing that it takes your albumin and replaces it with donated good albumin. So with IVIG they are giving you immunoglobulin to suppress your immune system. With the Plasma Pheresis they are taking the bad stuff out and replacing it with healthy albumin. Only problem with PP is that you need to have a way for them to do the procedure. I have had numerous subclavian catheters.They are okay but are risky because of the possibility of infections that are so easy to get and the catheters go straight to the heart. I had an AV Fistula made this past December and January with a few complications but it hasn't been used yet because my Neurologist thinks I am in remission. Yippee!! But ask your Neurologist about Plasma Pheresis. I think that would be a great option for you!

      Delete
    3. Hello Wendy, I have had CIDP for 17 years. I did IVIG for most of those years. But I read your Reply. I saw that you can't have IVIG. Have they said anything to you about Plasma Pheresis? It is the best way to deal with CIDP. It circulates your blood, while doing that it takes your albumin and replaces it with donated good albumin. So with IVIG they are giving you immunoglobulin to suppress your immune system. With the Plasma Pheresis they are taking the bad stuff out and replacing it with healthy albumin. Only problem with PP is that you need to have a way for them to do the procedure. I have had numerous subclavian catheters.They are okay but are risky because of the possibility of infections that are so easy to get and the catheters go straight to the heart. I had an AV Fistula made this past December and January with a few complications but it hasn't been used yet because my Neurologist thinks I am in remission. Yippee!! But ask your Neurologist about Plasma Pheresis. I think that would be a great option for you!

      Delete