Cheetahs In My Shoes

living with the imaginary menagerie and all that it entails

What’s in a name? – and why I’m not really looking forward to Christmas

26 Comments

One of the reasons I started to blog was because I wanted to make people aware of what living with Ehlers-Danlos Syndrome, Platelet function defects and everything that comes with them is like.  I’ve had an idea for this post brewing for almost as long as I’ve been blogging, so as my penultimate NaBloPoMo post, here goes…

Only after the birth of the Cheetah Keeper and a very bizarre (but fortunate) set of consultations did I get an explanation for why my body has been going ‘wrong’ for almost as long as I can remember.  Ehlers-Danlos Syndrome needs to be diagnosed by looking at the person as a whole and there were few in the medical profession at the time who did that – you see an orthopaedic consultant, they look at bones, you see a neurologist, they look at your brain, you see your GP, they think you’re mad/attention seeking/weird.  Over time, medical practises have changed and now, very slowly, some health professionals are beginning to look a the whole body – what affects what, how physical health can affect mental health and vice versa.

With my EDS having a name for the collection of bizarre bodily goings on was a huge step forward.  I could suddenly explain why my jaw partially dislocates when I yawn or why my hideously swollen ankles from pregnancy never really got better.  It explains why I found it so difficult to breastfeed, why my symphysis pubis dysfunction was so extreme (to the extent my pelvis was rotating up to 3.5″ at a time – and yes, that really hurts) and still, 4 and half years after my 2nd delivery, still comes back.  It explains my over-sensitivity to some hormones and why pain killers don’t work properly.  It explains why I had such a hideous time delivering the Cheetah Keeper’s sister (this is as close to a birth story as you’re going to get – the PTSD was too much to revisit) and other things like why my feet break when I wear high heels.

This is just a snapshot of what EDS does to me – and mine isn’t nearly as dramatic as others.  The diagnosis threw up a lot of ‘what if’s’ though – I was displaying signs from the age of about 3 but the paediatricians put it down to my mother being overly concerned.  If I hadn’t swum to the degree that I did (I used to be able to swim 2000m in an hour at the age of 11) maybe the damage I did to my knees wouldn’t have occurred.  If we had known what the underlying cause was, I may not have 18 lots of surgery under my belt.  Maybe I wouldn’t have been signed off with depression, agoraphobic and stoned to the eyeballs on painkillers – that didn’t really do much, making it a constant struggle to keep working?   All those questions make me even more determined that my children will not go through the same thing.

However, getting a diagnosis for your children is hard – when the main specialist unit is at Great Ormond Street.  GOSH is a tertiary care provider – ie where you go when your local hospital can’t provide what you need – thus making sure it remains the centre of excellence that it is.  You have to be in genuine need to go there and it takes time – mainly because they are looking after all the other children who need their time, skills and expertise.  In our case EDS is not a life threatening condition – life changing maybe but not life threatening – but we were seen by the most amazing multi-disciplinary team and the Cheetah Keeper’s sister has received the most amazing treatment and follow up care.

The main problem though, is that it doesn’t show and it doesn’t have a box associated with it to be ticked or crossed.  EDS is hard to explain – it is often grouped into the lazy/uncoordinated/disruptive child box.   In our case, her speech was slurred because the muscles in her face were too lax and her tongue was so weak it couldn’t get around the sounds to make words.  Her fine motor skills are poor because the messages sent from her brain to her fingers go ‘fuzzy’ before they get to where they need to be and the muscles are too weak to cope.

EDS doesn’t have a box in the Speech & Language Therapy list of things they can treat list though.  EDS is something new in school – it doesn’t come under their list of things that they have dealt with before or have been trained in.  It’s different – even from one sibling to the next it changes.  It presents differently in boys and girls and from family to family so there’s no cast iron diagnosis technique (it’s evidence based) and no clear guidelines to follow.

When you have a child, for example, with asthma, excema or a food allergy, you have, at your disposal, a host of medication, specialist medical professionals and information.  There are protocols that are followed and as much as any episode with a sick child is horrible for a parent, there is a ‘plan’ that will, to all intents and purposes, get you out of that situation.

For children with EDS and a whole host of other conditions, that ‘plan’ is not there.  It’s created by a team, the centre of which is not the consultant but, as far as I can work it out, the parent – me.  It is me that transfers the information from one health professional to another, me that researches late into the night to be able to present information to the health professionals in a way they understand, me that is trying to think of fun ways to incorporate the physiotherapy into everyday life and the occupational therapy requirements into a format that school can use without making them feel ‘different’.  That’s without managing my own condition too.

With the Cheetah Keeper, I knew, in a way, what I was looking for and when he walked late I suspected (dreaded) that there was some link to the EDS.  It also explains his reflux and why, when his system is under stress the contents of his stomach return at speed (resulting in a night in hospital with ‘just’ chicken pox and other such joys).  It doesn’t however, explain his blood problem.  I’ve produced a nigh on statistically impossible child who needs special medication and generally keeping an eye on.

Again though, a ‘platelet function defect’ explains what’s wrong but it doesn’t have a proper ‘name’.  If I wrote ‘haemophilia’ here you could ping over to Wikipedia and it would explain exactly what it is.  The Cheetah Keeper doesn’t have that – and platelet function defects are not that high on the list of things that your terribly busy GP covers on a daily basis.  So it’s a case of educating the health professionals as you go along – and staying up way too late finding out whether the oh so elusive medicine is on the Autumn 2011 formulary for pharmacies (it’s not).

Thankfully we’re under the care of a fantastic team at GOSH.  When he goes in for his next surgery after Christmas there will be a ‘plan’ created by his Clinical Nurse Specialist from the Haemophilia Unit and the ENT consultant who specialises in children who bleed.  They’ll have platelets ready for him (and possibly they’ll transfuse them first) and the pain control team will have been briefed as he won’t be able to have anti-inflammatory drugs post-op (or ever for that matter).   That’s the NHS at their best – and for that I am so grateful.  The play specialists will be there too, and those from Radio Lollipop, and those from the GOSH Charity – all trying to make the experience as positive as possible.

GOSH is a strange place.  It is not a place that you would ever wish to attend but it is a place that you’re very glad you go to.  It is a place of amazing positivity but one of acute despair.  It messes with your emotions – you’re so relieved when you come to leave but feel incredibly guilty leaving others behind; you marvel at the other children there and feel terribly inadequate as you sob in a corner, knowing that others are going through things that are far worse than you.  It’s your child that you cradle in your arms (and I’ve been there with both of mine) and that is the most heart-wrenching, stomach churning thing ever – and then you realise that the child in the next bay has no one coming to cuddle them.

So, please forgive me when I say that I’m not overly looking forward to Christmas.  As much as the time together, decorations, baking and gifting will be anticipated and shared with joy, it means that our next visit to the hospital will be rapidly approaching.  The Cheetah Keeper had surgery (at a different hospital) in the first week of January this year and Christmas was a countdown to that.  Not that I really told anyone else, but it was.  It’ll be the same this year.

I know that the operation should mean an improvement for everybody but it’s still not a nice prospect to face.  His EDS meant that his last “just an overnight stay” op resulted in a week long stay – this one, in theory is “just a day case”.

It’s a tricky old beast is EDS – just because it’s a name you haven’t heard of and doesn’t have a box to tick doesn’t mean that it’s insignificant, imaginary or something to be dismissed.  Those with it don’t have a choice – we can’t make it go away.  It is in our DNA and it will do to us what it will.  We can do our best to manage it but, at times it will affect both our mental and physical health.

In the meantime, I’ll be doing my utmost to make sure that the Cheetah Keeper and his sister have the best Christmas ever and that hopefully, one day, they won’t feel the need to write posts like this.

 

To read a bit more about living with a condition without a name have a look at the lovely Just Bring the Chocolate writing about The UnDiagnosis

If you would like to give someone the most amazing Christmas gift ever and you’re able to, please Give Blood  – there are thousands of recipients and their families as grateful as we are x

 

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Author: Jenny

Life, photos and recipes mixed with family life, additional medical needs and a whole load of imaginary friends

26 thoughts on “What’s in a name? – and why I’m not really looking forward to Christmas

  1. Blimey Jenny – I’d never heard of it. It is so important that the NHS can support families like yours…. I know it’s far from perfect ….. and it’ll always be difficult for GPs to diagnose diseases they might see once in a lifetime of practice….. but we are so lucky to have the service at all. Try to enjoy Christmas the best you can. It’s a difficult time for so many people, not least because the media portrays it as a time when the whole world is wallowing in happiness and good cheer…. it’s not like that for so many people (for all sorts of reasons). But with kids there is always some fun to be had and joy to be taken from their faces when Santa has been 😉

    • The stats are about 1:150,000 for EDS that manifests like we have it – although I think they will change as more research is done. Thankfully the adult services are based at UCH in Euston and Northwick Park Hospitals – both under an hour’s journey for us. For those that live at the other end of the country it is a mighty journey… hopefully more studies and research will make it a more widely known condition with proper support 🙂

  2. am informed and incredibly impressed at your positivity in the face of all this. Good luck and best wishes with all that happens over the next few week and I quite understand why you wouldn’t look forward to Xmas.
    I used to give blood, I stopped because I’d travelled abroad and had a tattoo. It’s on my Day Zero Project. I have the leaflet – I WILL DO IT. thank you for the impetus.
    Big hugs to you. XXX

  3. I think this post demonstrates what a fantastic woman and mother you are – brave for having to live with EDS (which I’d never heard of until now – thank you for educating me) and determined for fighting for what’s best for your children. I hope you can enjoy Christmas (just think of the look in your children’s eyes when they see that Santa has visited) and know that, even though your son will have to have an operation, he will be under the best care possible. And by that I don’t just mean the healthcare professionals, it’s you too x

    • I don’t think of myself as ‘brave’ – like so many others, I just don’t have a choice – but thank you so much for reading and commenting, it was a hard post to write and I wasn’t sure whether to post it x

  4. I’d never heard of EDS either although I have some experience of living with an illness with no name (although not on the same scale as you).

    There are some posts that we fret over, the ‘darker side’ posts as Maxabella called them this week.

    They can leave you feeling exposed and vulnerable.

    But it can also be very freeing to share.

    You have brought an awareness of EDS to all of us despite your reservation and you should be proud of that.

    I’m glad you posted.

    • I like the name ‘darker side’ posts x exposed and vulnerable is so very true and some of the process involved typing stuff and then deleting it which I guess is healthy – thank you so much for reading it xx

  5. In the face of everything you and your children have faced and are facing, may I just say how fortunate you are to live where health care is something your family doesn’t have to struggle to find or obtain. So many of us here in the United States don’t have the option (no health insurance) of finding the right doctor to diagnose a difficult and rare condition. Even if we have health insurance, it’s more like “wealth” insurance – a way to stave off losing your home in the face of catastrophic illness or accident. I’m wishing you well, in all senses of the word, and thank you for writing.

  6. Eek I know this will sounds weird but I’m so excited to find someone else who’d understand how I feel, I have a version of EDS too and never found anyone else with it! You’ve written about it really well x

  7. Wow i so enjoyed reading that post. I had no idea such an illness/disease existed and ot must be hard to deal with and cope with. I have learnt so much in one post and i hope your Christmas is a happy one and the new year brings you luck and less hospital visits. Thanks for sharing xx

  8. Jenny,

    I read your post this afternoon, and have been mulling it over since, wondering what to say. I’m completely torn between the ‘all experiences are subjective’ side of my thinking, the one that thinks I should be supportive in the strokey, strokey kind of way, and the other part that thinks that I should be a friend, and be honest.

    So I’m taking a risk on the fact that I’ve known you long enough to say what my first thoughts were when I got to the end of the post. I think that you need to keep things in perspective. I know you’re worried about G, it’s natural, but it’s a simple procedure. Have faith in the fact that there is no way it would be a day case unless they were pretty sure that you’ll be going home pretty much straight afterwards. At that time of year there are no beds at all in GOSH, so they wouldn’t book anyone in for a routine procedure if there was any risk of them not leaving. They are obviously taking precautions, which is great and the upside to having a team that knows him. The reason I’m saying to keep things in perspective is not to be flippant about your worries, but because this isn’t going to be the last time this happens, and the thought of you worrying about January, to the point of it ruining the next month for you seems unfair on them and you. And as you know after this there will be something else to worry about and something else after that until the only thing left that you’re thinking about is what is wrong with them and how bloody irritating the NHS is. You’ll fall apart if you do. Luckily, at the moment, their issues are a tiny part of who they are, they live a relatively normal life and achieve just as much as their peers. To worry so much about a small part that is not like children of their age, and to let it over shadow the huge part of their lives that are going so bloody fantastically just seems like such a shame.

    Their issues are likely to be long term, you just can’t put yourself through this every time a little bump comes along. You’re also putting them through it, as however hard you try to hide your feelings, they always know, and it just makes it a horrible and stressful experience for everyone, which then continues to get worse with each and every time something like this comes up.

    Admittedly I haven’t read all your blog and so perhaps my perspective is skewed by those entries that I have read, but I would just like to hear my friend writing about her amazing children, not only about her amazing children’s various issues, because I don’t think they see themselves in that way. I know it can be hard to be positive sometimes, and I think the way you have written about it is very eloquent but very careful. It is the fact that it is so carefully put that makes me wonder, and I apologise if this is not the case, but I wonder if there is some grief that you’ve just not properly admitted to yourself, so rather than separating that part of your lives that makes you angry, or sad, or guilty and addressing that raw emotion, it bleeds into all of family life as a carefully managed sadness about it all. A sadness that perhaps seeps into parts of life that it has no business being in, making it all about the EDS, all about the struggle.

    I guess I want to hear your real voice. You say this was emotional to write, but, to me, the emotion was managed and kept in its little box. I’d like to hear how you really feel, not thinking about what your readers will think, but for you to face it all and just be honest. Equally, I’d love to hear the optimistic Jenny, who I know is in there, I’d love to hear you capture the joy in celebrating what your children can do, but I wonder if one can be done without the other.

    I fully expect you to disagree with me, and I fully accept that I am perhaps only party to half the story, I just don’t like thinking of you so bothered by this all the time. I know how traumatic you found trying to get the medicines, but honey, I’m worried you’re not going to survive the choppy waters you might encounter if you can’t let the medical stuff be the small piece of the picture rather than an all encompassing one. It’s ok to not be reasonable or pc or careful, it’s scary to try to understand how you really feel about something, but it’s not ok to not be ok all the time, and I just can’t shake the feeling that you write what you think you should feel, rather than what you actually do.

    Said with love (and some hesitance)

    • First of all – I know it is said with love and I love you more so for having the honesty to write what you have. In some ways I think I wrote this as a vent – to say yah boo sucks to the Christmas lights and the enforced jollity that seems to have arrived so early this year. After an evening of coaching “awayyyyyyyyyyyyyy in a maiiiiiiiinger” I admit to feeling a little more seasonal (must work on those vowel sounds…). But for the last couple of months (it’s autumn/winter, you know what happens here) I’ve felt really unsupported, belittled, patronised, diminished and generally ground down and you know when that happens, I wobble. And wobble I have – to the point that I haven’t trusted my own thoughts or my own ability to do what I need to do – or think clearly enough to have any decent perspective on what’s going on around me.

      I did write very carefully, I wrote bits and then deleted them – I guess I lacked the guts to put it all in. I also, very nearly, left it in Drafts/Trash because maybe, like that letter you write and then burn, that’s what I should have done – to get it out my system and then make it go away. Instead I hit publish – opening up a guarded 25+ years of resentment/guilt and just plain madness onto the blogosphere. The optimistic Jenny is a bit fed up at the moment – she’d like some structure, a clear plan, a date and some order! She’ll be back (probably with some chocolate cake in hand) running around like a lunatic trying to do far too much. I did (truly) make a coleslaw sandwich for the Cheetahs’ lunch this morning and there are lots of posts about them.

      I think taking on the challenge of doing a post a day for November was foolish (but you know I like a challenge!) but it looked like a piece of structure to fit into the jigsaw. As the posts return to their more sporadic, as and when I feel like it/have something to say levels I suspect the more bonkers side of our family life will come back – and if it doesn’t, come round and give me a good kicking. xxxx

  9. Thank you for sharing. I imagine the thought of opening up to anyone that might come across your post was very scary but it’s incredibly informative, especially for those of us with less/no experience of navigating the healthcare system and all the different professionals in each field. I hope the support you receive here will help keep you buoyed throughout the festive period xxx

  10. Never mind a good kicking! I will come round and give you a medal!! You are such a deserving individual, deserving of so much more than a printer (but hey, never look a gift horse in the mouth!).
    My husband and I were talking the other day about how sometimes we can’t believe we’re proper grown-ups, that at some point we’ll be discovered – two ill-equipped, irresponsible individuals, masquerading as adults, in charge of 3 children and 2 mortgages! But for you, it’s no joke – there’s no let up to the background drone that is EDS in your lives. It must be incredibly hard for you to not let it get on top of you.
    You already know I struggle to comprehend all the stuff you manage to achieve on a daily basis. But Renata has a point – no-one would blame you for having a meltdown. You probably ask way too much of yourself – you said yourself you like a challenge!

    G will be in very good hands come January, and children are very accepting and resilient. Just don’t forget to be nice to yourself, for every post that you write pleading with your body to behave, write one thanking it for all it allows you to do. Your children will learn from you how to accept what can’t be changed and how to fight for what can. Make sure you teach them the difference.
    Love you lots
    xxx

  11. Wow – what a post – you have an enormous amount to contend with and I’m not surprised you have a wobble now and then. I too had never heard of EDS and I am very lucky that I have never had to go through anything like this so I cannot pretemd to know what you’re going through but just wanted to say we will be thinking of you. I hope you can put the op in Jan to the back of your mind for long enough to enjoy Christmas – I am sure that your children (and all the cheetahs!) will love Christmas (they are at a good age for it – same as mine!). Take care.x

    • Thank you x – we shall get there and with the excitement of the advent calendars opening tomorrow I am going to make sure I try my best to get into the spirit of things. The Cheetahs seem to be quite excited too – although the prospect of that many stockings is a little concerning… x

  12. I hope you realise how important your blog is … and how important your voice is in educating us all about the very challenging you and your children have to content with. Your one hell of a woman you know!

  13. :hugs: Thank-you for this post, I also have never heard of EDS and can’t imagine how worrying having a child waiting for surgery can be. I’ve only given blood once (earlier this year), and felt sick and weak for a couple of days afterwards. But that was all, and I wasn’t well prepared. Posts like this remind me how important it is that I give blood again, and again, and again… xx

  14. Pingback: Glittering Images « cheetahsinmyshoes

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