Joy and Pain

Two days ago the world lost a wonderful person.
That person just so happened to be Chris’s Mum, Joy.
I have, in the past, been known to refer to her as “my mother-in-law”, usually for simplicity’s sake. But she wasn’t my mother in law. Not least because Chris and I aren’t married, so the law had nothing to do with it.  More than that though. She was nothing like how a Mother in law is supposed to be.
I have listened, over the years, to people talk of these creatures, sneering over spectacles at the women who dare to fall in love with their sons, full of unwanted advice and hidden criticism. Treating grown men like the five year olds they once were, and their wives and girlfriends like you might vermin during the plague.
Joy was nothing like that. Not even remotely. It’s like she read a book on “How to behave like the archetypal Mother-in-law” and disregarded every single word.
She made me feel a part of Christian’s family from the moment I first met her. Contrary to everything ever written about mother-in-laws, one of the first and most frequent things she told me was how good I was for her son, how happy I made him (and her), and how thankful she was for that.
When I had Toby, my first child and her first Grandson, she made it her business to pass absolutely no comment on anything I chose to do as new Mum.  When I struggled she empathised, without burdening me with unwanted or unachievable advice. When we announced pregnancy number 2, and pregnancy number 2 take 2, and pregnancy number 3, she worried about how we would cope, reflecting on her own experiences of life with little ones, and yet was unwavering with her support and enthusiastic congratulations.
When she told me 6 weeks ago that she had been told by her doctors that nothing more could be done, it was without a hint of self pity, her only concern was how and when she would relay that information to her children.  And when I saw her 2 days ago, the only word she said to me was “thank you”, opening her eyes just long enough to look at me as she said it, before drifting back to sleep.
Having only known her for 9 years, I might not be best qualified to talk about who she was, and what she liked. But I can tell you definitively the kind of person she was. She was clever and she was kind. She was unwavering in her beliefs but tolerant of others and theirs. She was a woman who wished she had travelled, and written a book, and dyed her hair blue, but was without regret or bitterness about the life she had lived instead.
We, all of us, live hoping to change the world and assume that can be done only in the grandest of ways. We will build the tallest tower, amass the greatest fortune, write the wittiest play or cure the deadliest disease. Perhaps some of us will be successful in our endeavours, but most of us will face hurdles, failure and disappointment along the way.
I believe Joy did change the world. I believe her personality and temperament are a lesson to us all. I believe she produced three beautiful and talented children, one of whom is the man I love, and through her, exist two, soon to be three grandchildren. And I believe the memory of her will live on, in our memories and their hearts.
The pain of her passing is ours to bear, but the joy of her existence is also ours to celebrate.
Rest in peace Joy.
And thank you.
(1983)
(2010)

 

Happy Monday!

Some days I feel like I’m in my own twisted version of The Truman Show. You know, where everything you touch turns to shit and you can’t help but wonder if there’s some underlying conspiracy to drive you to the brink of insanity?
Today is not one of those days. Today is the opposite of one of those days.
First up, Chris went for his first ever driving lesson. That probably sounds like no big deal. Except that it’s a huge super massive deal.
When we discovered I was pregnant with Toby (on the 13th March 2009) I told him “You need to learn to drive before this baby is born!” and again when I found out I was pregnant with Rudy on the 12th February 2011, I said the same thing. Those embryos are now 4 and 2 years old respectively and until this morning Chris had yet to actually get behind the wheel!
After his lesson (which was apparently a great success, and certainly the Audi appeared intact on his return) I had to get bloods done as part of my molar pregnancy follow up. I figured it was going to be a huge pain in the arse as the specialist centre I’m under send all the specimen bottles and request forms etc direct to me and if there’s one thing us HCP’s hate, it’s patients’ who have more of a clue what’s going on than we do 😉 But in fact I got called through right on time, presented my blood bottle and paperwork and it was done and dusted within a couple of minutes. In fact, because I am driving again these days, the whole experience from leaving my house to walking back through the front door took exactly 20 minutes. There was even a parking space in the GP surgery car park! There is never a parking space in the GP surgery car park! (That was my first clue toward the reverse Truman show effect).
Then our lovely friends Emma and Ben came to collect the boys to take them on an exciting trip to the Sealife Centre and for a sleepover! Faced with the prospect of 24 hours child free in which to do whatever we pleased (so…that’ll be 24 hours of uninterrupted sleep then 😉 ) whilst our boys had a whale of a time (excuse the pun!) I really didn’t see how the day could get much better.
Then a letter from my consultant dropped through the letterbox.
It begins:
Well that got my attention. This guy could definitely write novels, he’s certainly got that “hook them from the first line then reel them in” quality.
He goes on to say that given how negatively I reacted to my recent trial of steroids he believes he is justified in allowing me 5-6 months of IVIG treatment before considering other options.
That means not starting prednisolone this week and waiting 2 months to see how I react to it. It means that for the first half of 2014 my CIDP will be managed and I will be able to lead some semblance of a ‘normal’ life and take a break from hospital admissions and trialling medications and their side effects. It is, in short, very good news. Which, as a family, we haven’t had much of recently.
So since today appears to be going so well, and since our children are safe and happy at Mermaid Cottage I think the only right and proper thing to do is to go out and celebrate…just as soon as I finish this blog post…and take a long hot bubble bath…and have a disco nap…

Swimming

Today I went swimming for the first time in 5 months.
The last time I went swimming was the 21st August. I was already having bizarre neurological symptoms at that point but had been told 2 days previously, by a locum GP that I simply had “sciatica” and to do more exercise. Thus, the swimming. Although truth be told, I knew deep down it wasn’t sciatica at all.
I managed 17 lengths in 30 minutes that day, when usually I’d do 20 lengths in about 20 minutes. I kept having the sensation that my limbs were simply not obeying my commands and that I might drown at any minute. Of course with hindsight I now know that’s true. Afterwards I struggled to get dressed and in the end had to walk home bra-less as I couldn’t manage to fasten it without help at that point.
Two days later I was admitted to hospital for the first time and the following week I was diagnosed with GBS, which we all know later turned out to be CIDP, the chronic version.
There aren’t many things I love, that I know I am crap at, but swimming is one of them. Swimming and singing, and maybe other things beginning with “S” too…like err….sailing perhaps?! I wouldn’t know as I’ve never tried.
I usually need to at least hold a half-hearted belief that I’m moderately good at something, to get any enjoyment out of it. Which may explain why I detest cooking and cleaning so much. For reasons unknown that is not the case with swimming though.  I can only really do breaststroke, and even then, my technique is abysmal and I’m very slow but I love it. I like that it’s just me, and the water, and that it’s a life skill that makes sense, in case you ever fall into a vast expanse of water and wish to stay alive. I have a hard time understanding sports that make no sense from an evolutionary point of view. Like golf for example.
Swimming raises my heart rate and works my muscles and all the while I feel calm and happy, although I confess other feelings surfaced when someone’s icky disembodied blue plaster floated toward my face this afternoon.
I managed 30 lengths, although admittedly it took me 45 minutes. I still don’t think that’s bad for someone who was staggering around on crutches a couple of weeks ago though. Just being able to go there and get in the pool and not drown is a massive achievement as far as I’m concerned.
Somewhere around lap 22 I started thinking about Dory from finding Nemo and it occured to me that her little song may be the simplest yet most inspirational mantra I could adopt to see me through these tricky times…
JUST KEEP SWIMMING
I’m half tempted to get it tattooed somewhere really prominent so that when the prednisolone makes me feel like shit, or fails to make any difference to my symptoms, or when I am next in hospital attached to a drip and missing my boys, it will serve as a reminder not to give up.

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.