MRKH Typology

It wasn’t until a hospital support group meeting about 4 years ago that I even knew there was more than one ‘type’ of MRKH let alone which I had.  Whilst I knew there were other effects associated with MRKH I didn’t realise that like with other conditions there was more than one manifestation of it.

Even though I had been going to the hospital for some 12 years by this point this was never mentioned until a seemingly flippant comment by one of the specialists during a discussion.  At the time I thought perhaps I had missed something, switched off during an appointment whilst looking at the ceiling and waiting for it to be over, yet it seemed like I wasn’t the only one and others hadn’t realised this either.  Someone asked ‘how do you know what type you have?’ to which the response was that it was normally confirmed during the MRI at diagnosis (I was diagnosed by laparoscopy) or as a separate kidney scan (never had one of those).

The outcome of the syndrome is the same whichever type you have it is still not possible to carry your own child, that doesn’t change.  This is because in both types there is no full development of the uterus, some have nothing like me whilst some may have uterine remnants, they serve no specific function they are just partially developed and sometimes have to be removed.

Whilst everything looks normal externally the underdevelopment of the uterus leads to the underdevelopment of the internal vaginal tract itself.  This can be stretched through dilation (natural or with dilators) or a full length vagina can be fabricated through surgery to allow for a comfortable sex life.  Ovaries are usually present in both cases and provide the hormones that ensure our usual female development and is therefore often why MRKH is only detected in the teenage years when periods don’t start as otherwise everything else develops and appears as expected.

The difference between the two types is the additional physiological effects or manifestations caused by the additional genetic changes the syndrome causes.  Most commonly this is related to kidney development issues but can also manifest itself in a small number of cases as hearing difficulties and skeletal problems such as scoliosis.  A type 2 sufferer may have some or in rare cases all of these problems.

 Type 1 is the more common type.  Type 1s do not experience any specific additional issues such as those related to kidneys, hearing or skeletal. Not that that is often any consolation for anyone that has this type of MRKH of course.

 Type 2 is far rarer and includes the additional effects such as kidney, hearing and skeletal problems.  ~40% of women with MRKH have a chance of kidney problems with ~15% only having one kidney, ~10% have hearing difficulties and ~10% with skeletal problems.  By its nature, type 2 is often (not always) diagnosed earlier in life because through examination of these issues, especially those related to kidneys, which is the most common indicator for type 2 MRKH, it means that the absence of a uterus is often identified at the same time.  This can of course mean that diagnosis can happen even earlier in life where it could be even harder to comprehend the future implications of not being able to have a child.

The typing of MRKH is often now achieved at formal diagnosis which is now typically by an MRI scan.  The benefit of the MRI over the more traditional laparoscopy approach is that within one scan an MRI can identify not just bones and joints but also internal organs making it useful to confirm (or deny) the presence of ovaries, kidneys and womb etc more easily.

Back when I was diagnosed I had a laparoscopy which meant a day in hospital and a general anesthetic.  To get the best view with the camera sent down through your belly button and the other sent upwards from just above your pubic bone they pumped your tummy full of gas which makes you look pregnant. Oh the irony.   I’d had ultrasounds as part of the process to try and work out why I hadn’t started my periods but they never focused on my kidneys and whilst I had never had any major issues with them you never know what’s going on so I got these checked a few years ago to confirm my Type 1 MRKH diagnosis.

Aside from the physiological effects related to type 2 for me the biggest impact of MRKH is psychological.  That is not to belittle the condition at all the differences of the two types but the impact it has on your mental well being is really not to be underestimated particularly for a condition which for the most part doesn’t make you physically look any different.

Whatever your type the way you deal with it will differ from person to person.  There is no way to predict how you will feel or what the best way will be for you to start to come to terms with it.   Coming to terms with it may seem like it’s an easy thing to do, but it’s not.  It’s taken me many years to get to where I am now.  I’m never going to get ‘over it’ as such but you find a way to put it into some perspective and get on with your life.  To be blunt you kind of have to find that way, however long that takes.  It’s one of those things that you can’t change but you unfortunately have to learn to live with in some way and if I can help by channeling my experiences to help raise awareness then even better.

Support is therefore vital, getting that at the right time and through the right channels can make so much difference.  How people prefer to access that support will also differ but the main thing is knowing that you aren’t alone and that there is always someone there to talk to if you need it.  We all need that sometimes, however strong we are.

If you are interested then take a look at some Useful links on my website, look into the facebook groups or feel free to email me if you have any questions.

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Age isn’t just a number

We often say age is just a number.  It’s a way to perhaps make ourselves or others feel better about advancing years and those ‘big birthdays’ whether that’s reaching 30, 40, 50 etc .  But it’s not just a number.  Age can often relate to our life experience, like a good wine or a whisky, as we age we experience more both personally and around us.  But of course some people even from a very young age, may end up experiencing so very much that their lives already show so much experience regardless.

Last week my Granny turned 100.  I mean that’s just bonkers. IMG-20160813-WA0009

Some people don’t like to celebrate their birthdays (weirdos) and let them sneak past unawares but you can’t let 100 go past unnoticed.

She was born during the First World War, her mother died when she was 20 her dad when she was 42, she married my Granddad during the Second World War in 1941 then my Uncle was born in 1946 and my Dad 3 years later.  They lived in the same part of Kent for their whole lives meeting as teenagers they were besotted, never hiding their love and affection for each other.  My granddad; the once very sporty, funny, cheeky, larger than life character and my always more meek, patient and passive seeming introverted Granny.  Sadly my Granddad passed away just a few days before their 65th wedding anniversary but what a life they were able to share together.  Whilst not unexpected given his age (he was 90!) and general health it hit my Granny and the rest of us hard as of course it would have done.  He was an absolute legend of a man.

You so often see it that in these situations the remaining spouse or partner sadly often passes away soon after.  In some ways I don’t think we would have been surprised if it were to have happened like that.  But it didn’t.   Even at 95 she was still completely mobile and living in her 2 bedroom house (with stairs!) and was able to get around reasonably easily.  One year later, just before Christmas she unfortunately had a massive stroke in her home and was found the following day by her next door neighbour and long term friend.  It was touch and go if she would survive.

But somehow she did, she bounced back with the vigor, strength and determination that I assume has helped keep her going as long as she has.  Despite having to move out of her house (at her own request) into a nursing home she recovered almost fully from the stroke.   She still has almost full mobility, can still get around, albeit with a zimmer (which she is an absolute demon with) and aside from some dodgy hearing, failing eyesight and arthritis she has no real ailments – not bad for Centenarian.

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She has seen life change so significantly for the good and bad around her over the years.  She was nearly blown up during the blitz by anti-aircraft guns (English ones!) and had she made it to her usual hiding place that day with their little dog then I wouldn’t be here and she wouldn’t have just turned 100.

 

My Dad made a book for her birthday which captures some of those key moments in her life as well as including various historical (political, social and cultural) events that have happened during her lifetime.  If I am quite honest I never really saw my Granny and Granddad as really on top of ‘popular culture’, particularly my Granny.  She had other hobbies like painting, marquetry and sewing that always kept her busy and which she was extremely good at.  I suspect there are a number of facts in this book that my Granny probably won’t understand but it doesn’t make it any less interesting to document it.

Particular facts in the book that I liked but I fear will have so easily slipped past my Granny are:

  • Microsoft was founded
  • Vodafone launched the UKs first mobile network
  • The first Harry Potter book was released
  • Hubble telescope was launched
  • Midsomer Murders was first broadcast
  • The first Kindle was produced
  • Top of the pops was shown on TV for the last time
  • The new Wembley stadium was opened
  • The UK’s last circus elephant retired

 

But hey, she is a 100 and despite all that has happened in her life and in the world around her she is still here and shows little sign of that changing.  I often panic about age, not because I am 32 specifically but because time does go by so fast and I don’t want to miss out on anything.  I never expected to be at this stage of my life now.  We all have these big plans and thoughts and ideals and living in a flat by myself at this age wasn’t one of them.  But that’s life and there is no point in getting all ‘woe is me’ about it.  I like my life, my space and spending time with my friends and family and the people I love, it’s just different to how I thought it would unfold.  Times change and whilst we still often seek to compare our lives to others the general date, get married, have children route just doesn’t work in that same way anymore if it is followed at all.   We often forget that.

My Granny can be quite demanding with her requests to my Dad from birthday cards which need to be plain but go from being allowed to have landscape scenes, flowers and dogs to only allowing landscape scenes and flowers and if the landscape scenes include dogs then she doesn’t want it (because some people don’t like dogs).   My Dad now knows how hard it is to find a good landscape scene suitable for a birthday without a dog in it!  To requesting new clothes in a size much much too big than she actually is but which she is convinced provides the perfect fit.

Whilst I very much understand and appreciate my Dad’s frustration, My Dad, the most patient man in the world is often rattled (probably more frustrated to nearly ‘mega drive’  moments)  at times by how she can be and I get that but in the same breath at the age of 67 he never expected his mother to still be alive and talking to my Granny on her birthday, she never thought she would get to 100 (I mean really none of us did!).  She is now one of over 14,500 people in the UK who is 100 or over, a tiny but ever growing percentage as we live longer and longer.

That book my Dad made my Granny was entitled ‘The first 100 years’, there is a certain irony there of course as despite the best will in the world we know she won’t live until 200 (I mean she can’t right?!) but if she lives older than Bessie Camm (current UK Oldest person at 112) then who knows what else she will see.  She already has the delight of 3 Grandsons a Granddaughter and 2 Great-Grandchildren…but what next?

Life can be short, and it often is, which is why living it as much as possible is so important.  I don’t know whether my Granny considers she lived her life to the full or not but she certainly has some wonderful memories to look back on.   I hope if I ever reach to even close to that age that I can look back with not only memories but also with the thought that I did everything I wanted, because I certainly aim to.

I’m made of strong stuff

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Did you really wear Hi-tecs for the walk?

This was a question that Richard my walking partner / enthusiasm booster / mood lifter from 80-90km of this years challenge asked me as he was telling me he was signing up for next years London2Brighton. But yes I did wear Hi-tecs for the walk and they were excellent.  They weren’t all that expensive and I went into Millets and bought something that was comfy.  I remember trying them on at the time and thinking yeah I could probably walk 100km in them because I literally had nothing else to base my decision on at this stage, they seemed like they would be ok but do you ever know until you actually start walking in them for like a seemingly endless number of miles? No.  Except now I know they definitely can walk 100km.  I think he was more surprised that not only did I buy Hi-tecs (how 1990s of me) but I didn’t really struggle.

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Now I did point out here that I did struggle but apparently ‘struggling’ means crawling over the line in more of a state than I was – I’ll take that.   I got called a ‘machine’ for my walking prowess and seeming OK-ness after walking so far, my Mum even told me it looked like I had just gone for a Sunday stroll when I crossed the line.

I did say I definitely wouldn’t do the 100km London2Brighton challenge again as I wanted a new challenge. I crossed the finish line in tears completely overwhelmed and exhausted, we raised so much money for a truly deserving charity thanks to our so very generous friends and family and that still blows me away (currently over £4,100 and still people have been donating!!).  My feet are still recovering, not from blisters but from soaking my feet for so long in surgical spirit which was very useful so I didn’t get blisters (as it hardens the skin) but now the skin just peels …a lot, which is always great in the summer.

But then I got challenged, could I do it quicker?  The competitive part of me debated for a bit but ultimately couldn’t resist….so despite the peeling feet the physical exhaustion and the complete craziness of contemplating actually doing it again… I signed up!  This time my aim is to do it <24hours which will be a whole 5 hours and 16minutes off this years ti20160528_123607me and again I will be walking for MRKH Connect.

This year was very important for me and Jen to do something together, pushing ourselves to do something so entirely bonkers that was really hard (and still is) to comprehend and for a charity that was so very close to our hearts.  It wasn’t about the time it was about completing it, together and we did it and I couldn’t be prouder of us and of course Laura.  2017 will see  Jen and I taking on another challenge which is currently being decided (watch this space!) so this walk will be my personal challenge and a kind of warm up – albeit a crazy one.

 Being strong feels exactly the same as being weak
The difference is you don’t quit
Not until you reach the peak

 I am the fittest I have ever been and I like that, I want to continue that.  Plus the prospect of more walks around the delights of where I live, those luminous leggings, peanuts, cheese and marmite sandwiches and too much Justin Bieber just adds more fuel that this is an entirely sensible thing for me to contemplate doing again….right?

 It’s all a test to see if you’re made for it
So go for it, you can’t wait for it
Cause you’ve got a destiny with your name on it

 I am not very good at giving up on things (except the Bronze Duke of Edinburgh, the first time), and I have done it once…so how hard can it be?!