In Sickness & In Health

A guest blog post from my husband Jamie (aka Jay).

Jay has been saying that he would write a guest piece for around a year. Well I have finally pinned him down. The first time I read it was when he handed it to me ready to be typed up onto my blog. I won’t lie some of it moved me to tears. He is a typical bloke who quite often doesn’t say what he’s thinking or what’s worrying him. I know he loves me that much is clear but I am so proud of him for writing this piece and introducing himself to my readers. So here it is…..

Hi,

I’m Jamie. I am 43 years old and I am married to Rachel. You may be a frequent visitor to her blog and while you are all probably well aware of our dogs (as understandably so) they get more blog time than me, you may not know much about me.

I met Rachel in 1997, whilst we were both attending a management training course. I knew from the minute I met her she was going to play a massive and pivotal role in my life. We were engaged around one month after we met and I moved in with her the following year.

We were married in 2000 in Sri Lanka. An incredible setting for such an important moment in our lives. We eloped and our trip to Sri Lanka was not only our Wedding destination but the first time we had been abroad on holiday together.

Three years later we purchased our now home and lived happily ever after…….

The End.

Well not quite. You see regardless of what happened then to her health, nothing changed for me. She is still the same incredible person.

A lot of people told me to “walk away” or told me “this isn’t what you signed up for”. However what I signed up for, was to spend the rest of my life with my lover and best friend. And that is what I am doing.

Others have said to me “oh being a carer and working full-time must be so exhausting”. My answer is “No it’s not. She is there when I wake up and there when I return home from work.” During all the visits to the doctors / consultants, guess what? We are together and we talk for hours. In fact it doesn’t matter how bad things get, she’s always talking! We rarely even listen to music in the car because every journey is filled by the sound of us laughing and chatting. So don’t feel bad for me. I have the best life there is, spending so much time with my amazing wife.

Yes there are up’s and downs. Occasionally I get very stressed out because she is so unwell. Sometimes I need to take emergency time off work to look after Rachel and I have to say work have been fantastic in supporting me and looking out for my mental health.

Rachel has supported me selflessly over the years. She has supported me as I have grown as a person and as a husband. She has always been there for me, as I have been there for her.

Yes I make mistakes, I leave the toilet seat up, smear shaving phone on the mirror, leave blue hair gel in the bathroom sink and I undercooked a pizza once. I also promised to write this blog post last week when I was on holiday from work but instead found myself overtaken by the other love of my life my PS4.

Through all the bumps on the road of life, we know and understand each other better everyday. In February we celebrated 20 years together as a couple, this September we will have been married 17 years.

So that’s about it, short and sweet I know but not a downbeat, oh woe is me post. I couldn’t be happier. I do hope you’ve enjoyed my blog post and that you will continue to support my wife’s blog.

As I said in 2000, In sickness & in Health.

Hello Darkness My Old Friend

Last week was a bit of a nightmare. I was so fatigued it was ridiculous. I was sleeping hours but still felt like a zombie when I awoke. My legs felt like they were made of lead. My brain was sending the messages to them to move but they would refuse, causing me to fall over. When I could move them they felt as if they were made of lead. Climbing the stairs (which I limit anyway) felt like I was scaling mount Everest. Then on Tuesday afternoon, I recognised a feeling that I haven’t had in a long time. My face, eyelid and eyebrow were beginning to drop. I ended up going to bed for a lie down and slept for three hours. When I awoke the feeling was gone and I was relieved to have dodged a bullet. But I hadn’t, when I woke up on Wednesday morning this had happened.

Now I have a level of Ptosis everyday, normally unless you were specifically looking for it you wouldn’t know it was there. My eyelid droops to where the pupil starts to become obscured. That level of Ptosis I can cope with, it doesn’t affect my vision and it doesn’t tend to make me feel unwell.

Wednesday morning I woke up feeling horrendous, arms and legs both felt like they were made of lead. I was dizzy and due to my eye being nearly completely closed I couldn’t see very well. My forehead felt as if it was being ripped in two, with my left eyebrow arching in an attempt to keep the right eye open, only it was failing miserably. After a couple of hours I decided this really wasn’t worth the hassle of trying to stay out of bed. At 8am (I had risen at 6am) I gave up, took some HuzerpineA (like a herbal Mestinon as I can’t tolerate the real stuff) and set my alarm for 9am hoping when I woke up the Ptosis would be gone.

I gave myself an hour as like Mestinon this stuff takes about 20-30 minutes to get working. Plus I was also having a friend over, so needed to be washed and dressed before she arrived. I naively thought an hour in bed would be the magic cure. What an idiot! When it’s bad like this even proper Mestinon can fail to work. I should have realised when my alarm went off, that this was not a good day. I never fall straight back to sleep after being up for a few hours. I messaged the friend to advise them I would have to cancel, something I hate doing as it means I have knackered their plans for the day. She was absolutely fine about it but it doesn’t stop the guilt over cancelling. I text my husband to let him know I would be staying in bed for the day and that I might need him home if things got worse. I then went straight back to sleep.

Most of Wednesday was spent asleep if I am honest, I have a few brief periods awake mainly to get something to eat and have a cigarette. The fact that I was so utterly incapacitated scared me. This reminded me of the bad old days when I first got sick in 2007. How I had gone from working full-time to a year later being ill-health retired. I hadn’t been this sick what I call MG sick in years. Probably close to nine years, so you can imagine my shock, when this came totally out of the blue.

Thursday I woke up feeling an awful lot better but then by the afternoon was back in bed on home oxygen. I couldn’t take a deep breath in, as my chest just felt like it couldn’t expand. This really scared me as I have been blue lighted to hospital barely conscious due to breathing difficulties. This was exactly how it had started in 2008, I tried to walk across the lounge floor but stumbled as my legs wouldn’t work properly. The following day whilst hubby was out getting some shopping (helpfully forgetting to take his mobile phone) I had collapsed unable to take a proper breath in. I’d had to call 999 myself, trying to stay calm whilst the supply of oxygen in the house felt like it was running out.

There have only been a few times in my life I have been utterly terrified by my symptoms and that was one of them. I ended up being wheeled straight into resus, placed on oxygen running at 15 litres a minute and I still had very poor oxygen saturation. They  discussed with me about putting me into a medically induced coma and putting me on a ventilator. They wanted Jay to be at the hospital, I now realise it may have been to say goodbye, at the time I was oblivious and said he had to look after the dogs. I look back now and it moves me to tears. I wasn’t frightened at the time I was just exhausted from not being able to breathe.

I had all this running through my head again on Thursday, thankfully within two hours I was feeling much better but I had to spend the rest of the day in bed.

Gradually I have improved and back to my normal (but limited) levels of activity. However I am still having days where my arms and legs are refusing to work the way that they should. Today I was trying to do stuff around the house and I kept falling over my own feet as they felt as if they were stuck to the floor every time I went to move. I have started choking again (frequently) on fluids, something which hasn’t happened in a really long time.

It's frustrating that despite in many aspects of my health improving a great deal, no more headaches, much less joint pain since giving up dairy, that this has started up again after leaving me alone for such a long time. Thankfully I have a hospital consultants appointment coming up at the end of next month and I will be able to discuss it with him then. I think it maybe time to bring up the possibility of Myasthenia Gravis again as seronegative MG seems to be much more accepted than it was ten years ago.

This song was going through my mind the whole of Wednesday and Thursday.

EDS how it affects me three years on

Last week as part of Ehlers Danlos Syndrome Awareness Month, I recycled a post I wrote in 2014. As with any illness / condition / disease when you look back you can see how things have changed. Some things may have improved and new symptoms may have reared their ugly heads. Ehlers Danlos Syndrome is no different, I sometimes think it’s like playing a slot machine each morning never knowing what the combination of joints giving me pain (and problems) will be that day.

I am still as bendy as I was at this point three years ago, although getting my thumb to touch my wrist isn’t as easy as it was before, my thumbs now seem to sublux (partially dislocate) or strain the ligaments on an almost daily basis. Who’d have thought something as simple as taking my trousers / underwear down would be enough to cause pain but it does. Looking back on the photo’s of my fingers it is clear that they are more flexible now than they were two years ago. Although some with EDS will stiffen up with age despite being 44 this year, I seem to be getting more lax. I wonder if by the time I am 80 I shall just be a floppy mess unable to move due to the collagen (the glue that holds us together and is what is faulty in EDS) just being stretched beyond belief.

I wrote at the time that my elbows are normal and as usual when I write anything about my condition, it’s like an open invitation for things to change dramatically. Doctors don’t seem to be able to agree on whether my elbows are hypermobile or not, to me they don’t seem to be but then it’s difficult to gauge what a normal range of motion is when you have been surrounded by anything other than the norm. My normal is growing up within a family that is hypermobile, so I never considered my flexibility to be anything other than that. My elbows can be very sore first thing in the morning, as I am asleep when I am doing the damage I can only assume that I am over extending the joint. It makes my elbows really ache and feel clicky, I can feel tendons and ligaments rub over the bones.

My spine is still the area of my body that causes me the main problems. I am currently waiting for a spinal x-ray to try to find out what’s going on at L5-S1 level. This pain is very different from my “normal” EDS pain, it hurts when I lie down or sit down and eases off when I am moving. In the mornings I am very stiff and look like a hunched over old lady. After a few hours it eases up but the minute I go to bed the pain starts up again. I have been taking a NSAID and it's really helping which is the first time a nsaid has ever helped. I am hoping the x-ray shows something as it's hard going with it affecting my sleep.

My fingers are also causing a problem not with them being more lax than they have been previously but with them swelling, clicking and pain. Some mornings I can barely use my hands as they are so swollen. When they are bad the only thing I can do is just wait it out until the swelling starts to subside. My grip is also a lot weaker, so opening child-proof bottle tops is a nightmare, I am struggling with even normal bottle tops because I no longer have the strength of grip I had previously.

These are just some of the problems I face with EDS on a daily basis and I could never predict what was going to hurt not hurt the following day. It feels totally random and at times, the pay back for relatively undemanding activity totally disproportionate. I always laugh when medical professionals say we need to learn to pace ourselves as I never seem to have the same energy level on a consistent basis. I never know when I have done too much until hours later and the pain starts. If I paced myself I would never do anything for fear of causing pain or fatigue. Both of which are just a standard part of my day.

I was finally diagnosed with EDS when I was 37, my condition suddenly deteriorated in 2007, it took until 2011 to get some answers. Whilst it’s great to find out that no, I don’t have a low pain threshold, yes I can feel things after I have had a local anesthetic the problem now is that aches and pains get blamed on EDS rather than being investigated properly.

There also seems to be a blanket policy in many of the hospital trusts that unless a condition is life threatening, surgery (if needed to correct a problem) will not be performed. This is done under the guise of “first do no harm” and the worry of making things worse however unless you’ve lived with this condition you have no idea the levels of pain we can function at. I have bowel adhesions (fibrous bands of scar tissue that have wrapped themselves around my intestines), I had them surgically removed in 1998 (before the EDS diagnosis) because they were causing me to collapse due to the pain they caused on a regular basis. I still get awful periods of time with them, I have to be careful what I eat, so I can’t have raw vegetables as it triggers the pain, yet when I saw a surgeon a few years ago I was told unless my bowel becomes obstructed they won’t operate. I have heard similar stories from other people around the UK, that they have various problems that could be sorted out by surgery yet due to the EDS diagnosis they are told they can’t have the operation.

I was so pleased when I got the diagnosis as it finally explained all my weird symptoms but it’s a poison chalice. I have better pain medication but I am pretty much left on my own to deal with medical issues as they arise. Many in the medical profession believe that because there is no cure for EDS it means that there is no point trying to treat us. Now I have the diagnosis the level of help I get is severely limited.

There was a lot of drama recently within the EDS groups on-line as the diagnostic criteria had been changed. Many people diagnosed with Hypermobility EDS would now be classed as having joint hypermobility syndrome rather than Ehlers Danlos Syndrome. Having checked the new criteria I would still be classed as HEDS and to be honest even before checking the new guidelines, I wasn’t that bothered. Regardless of the label most people with EDS or JHS will receive shit care, have their problems minimised or ignored and surgery that would be offered in normal circumstances declined. It would have been far better if the bodies that spent all this time and money on designing the new criteria actually focused their efforts on getting patients better care.

Ehlers Danlos Syndrome and how it affects me

I first published this post in May 2014 for Ehlers Danlos awareness month, as its that time again I thought I'd share for those new to my blog.

As May is Ehlers Danlos Syndrome Awareness month I thought I would share with you how my bendy body affects me and take some photographs to show you how bendy I am. Now I am well aware that as an Edser I am not supposed to perform "Party Tricks" every doctor I see tells me that after they've asked me to show them! The moves I have photographed cause me no pain - I wouldn't be that stupid to suffer so much for my art!

For those of you unaware of what Ehlers Danlos syndrome is or that are new to it I am going to give you a brief and basic overview. EDS is an inherited connective tissue disorder, affecting the collagen protein in the body. Connective tissue is the "glue" that holds the body together, it connects tendons and muscles to the bones and holds organs in place. Collagen is what makes this glue and in EDS its a bit defective. Our connective tissue is super stretchy and lax so it means that our joints aren't held in place properly. Our joints have an unusual range of motion - so in normal people your joints will only move to a certain point. In EDS ours move to that point and beyond - the photo below is of my fingers which are hypermobile.

If you don't have EDS you will recognise that my fingers should not be able to move this far. I have seen a video of a lady with EDS pull her fingers so far back that they touch her wrist. Thankfully mine don't move that much.

It is important to remember that EDS is a syndrome ( syndrome means a collection of symptoms) so it affects people differently. You may know someone who is double jointed or extremely flexible ( and probably not diagnosed with EDS) they may when having a look at the beighton diagnostic criteria clearly have EDS but have no pain or any other of the associated symptoms. Where as I have constant pain and joints that like to sublux ( partially dislocate) or dislocate on a daily basis. Also some people find that they have joints that are very unstable (sublux or dislocate) and other joints that are fine and actually normal. My elbows joints are normal and not hypermobile and although I can do this (see photo below) with my thumbs the rest of my thumb joint is stable.

My doctors have classed my EDS as type 3 or Hypermobility, ( there are a few different types of EDS) I was diagnosed by a rheumatologist who I paid to see privately. Getting a diagnosis was one of the few things that my GP and I have ever disagreed on, he felt I didn't need it I said that I did. Since I have got the diagnosis it has changed the management of my pain, my dental treatment and provided some much needed answers as to why I have had pain from such a long age. I was still being told I had growing pains at 26 when I visited a gp for my leg pain! You maybe wondering why my dental treatment changed after my diagnosis well with EDS we are more prone to periodontal disease (gum disease) and also we have a problem with all local anaesthetics. For some of us local anaesthetics don't work at all for others, like me we burn through locals really quickly. My dentist also has to give me a local anaesthetic that doesn't contain adrenaline. Due to my autonomic dysfunction adrenaline causes me to have tachycardia and that in turn can cause me to faint when getting up from the dentists chair - which I have done.

The worst area of my body affected is my spine which is very hypermobile from the back of my skull to my coccyx (tailbone) those little vertebrae rarely like to stay in place. My neck is hypermobile and I have what's known as "owl neck" so I can move it not all the way around but too far on both sides. I also found out by chatting to someone on twitter that doing (see photo below) is not normal. You see thats the thing with EDS we have no idea what the normal range of motion is for a body part as we have never experienced normal.

I have four discs at the base of my back that I can't even touch with my fingers without me flying through the air. I never have a day without some sort of back issue - muscles going into spasm, discs being naughty by locking up so I can't stand up you name it my back can do it. You may have heard me mention before how I have to go to bed really early its because by around 5pm I am struggling to hold my head up and my back is starting to resemble the letter 'C'. My muscles work hard all day trying to support me and by 5pm they are worn out and throw in the towel.

Although it may seem like fun that I can do all these things and I have the bonus of looking young for my age (the only good thing EDS has ever given me) life with EDS is not fine and dandy. EDS produces fatigue as our muscles have to work around 5 times harder than a normal persons every time we move. EDS is almost certainly the cause for my autonomic dysfunction its estimated around 78% of people with Ehlers Danlos Syndrome have autonomic nervous system issues.

There are currently no genetic tests for EDS Hypermobility ( other forms of EDS have genetic tests) and no cure for any type Ehlers Danlos Syndrome only treatment which consists of pain relief, physical therapy and bracing joints.

Throughout May I will be blogging about EDS to explain what its like to live with this condition.


Useful links
NHS CHoices explaining EDS and the types of EDS
Ehlers Danlos Support UK UK Charity for EDS patients and produces an excellent magazine called Fragile Links
Hypermobility / EDS and the other issues it can cause in the body- HMSA Charity (HyperMobility Syndrome Association)

   While I was getting my husband to take the pictures the dogs tried to join in. This is Mollie trying to give me a kiss!

This is me giving up as all the dogs tried to join in!