Just a quick hello

Morning all, (afternoon and good evening to those of you in different time zones).

Unfortunately I have woken up again with ptosis and right sided facial weakness. My stomach is complaining due to taking 30mg x 3 of mestinon yesterday. It only worked for around 2 hours at a time and was taking a good ninety minutes to be absorbed into my blood stream. So either my stomach was on a go slow as it usually works within 15-20 minutes or I needed the full whack of mestinon in my system for it to make a difference.

I feel like I am stuck between the devil and the deep blue sea today. I feel dreadful and I know I will feel better taking the mestinon…….but do I want the stomach cramps that go with it? 30mg is obviously not enough so do I take 60mg and hope for the best. This is getting like the bad old days of 2007 where for nine months I had ptosis continually. It took around 9-12 weeks for my vision to adjust to only having usable sight in one eye. The brain is an amazing thing as long as I was in a place I knew my brain would step in and create the rest of the picture for me. You know the stuff I would have seen with my right eye had I been able to open it. That’s pretty cool until you realise that you can’t actually see on the right hand side because you have just fallen over an obstacle that your brain wasn’t aware of. I still think how the brain compensates though for loss of vision on one side is incredible.

Although I only have vision in one eye today, due to being unable to open my right eye I am feeling more confident with it today. Yesterday I felt so scared and vulnerable with my poor vision, today I am ok with it. I hate the fact I get so scared when I can’t see, it really terrifies me. I lose my hearing before I faint and that doesn’t bother me, but losing my vision terrifies me. I wonder why that is?

The only time I have not had problems with ptosis and double / blurred vision was when I was on 20mg of prednisolone daily. I may have had to toss in the odd mestinon but on the whole it was an awful lot better. When will they (medical profession – mainly neurologists) realise that this is either ocular, bulbar or generalised Myasthenia Gravis? What else could it be?

Ok my eyes are getting sore now as I am not blinking enough and they are drying out. So I will sign off for now. Thank you for reading.

Before I forget I believe in the USA (it may also be the case in Britain) that this is Myasthenia Gravis Awareness month. So lets raise the profile of this awful disease.

Apologies for my absence

This is just going to be a quick update to apologise for not updating my blog, there are several genuine reasons (feel like I am at school explaining why I haven’t handed in my homework! lol)

1. Had no Internet connection from late friday night until Sunday 11am BST

2. Due to humidity / heat I have had an increase in my MG like symptoms. I have had pretty much constant ptosis, double vision and blurred vision, which is not conducive to using a computer screen. Mestinon will open my eye but it doesn’t get rid of the blurry vision or the double vision. Also in these symptoms very weak shaky legs. Its very much like when I first got ill in 2007.

3. Pain not being very well controlled.

Ok so those are the reasons. If there are any massively glaringly obvious mistakes that I have missed in this post its due to my vision being extremely wonky. So apologies ……again.

I have a physio appointment next week on Wednesday……or should I say torture session because its going to hurt. I am so deconditioned anything above and beyond my normal level is going to hurt. Luckily the place I am going to is the pain management clinic and physio department.So might kill two birds with one stone.

As I haven’t been on any Internet sites properly for a bit I have a bit of blog reading to do so apologies for not swinging by your posts. Its highly unusual for me to be absent this long. I hope you understand.

Special message for Rachel who writes this crafters Yarn.

You may have noticed an increase on your visitors to your page. This is because Midmomga on Twitter has advertised you as a good MG blog to read. She has signposted your readers to come via me due to my blog being linked to yours……So exciting. So if anyone searches twitter for MG your blog will come up! The message reads as follows..(exactly how it was on twitter)


A difference of opinion

I read a blog earlier in the week that really annoyed and upset me.Which I actually think was the intention of the blogger, it wasn’t aimed directly at me (I hope) but the wider POTS and EDS community. The blogger had chosen to post her opinion on what people with EDS and POTS should do to get well. What her post failed to understand was that  illness is not black and white, its not one size fits all and what works well for some people may not work so well for others.

What annoyed me more was the bragging on Twitter that she would get hate mail for posting this. I checked her blog this morning and it seems no one has bothered! Why would you try and reason with someone who is telling you in her post that you aren’t sick and you need to change your attitude?

Her reasoning is that it is that EDS is a genetic abnormality so that means we aren’t sick. Perhaps I should suggest that to parents with children with Fragile X syndrome that their kids aren’t sick? Or perhaps mention to my cousin who has cystic fibrosis another genetic condition that she isn’t sick either? The author of the post has very strange reasoning. Which is why her post has upset many people in both the EDS and POTS community.

If I didn’t feel like death warmed up, need morphine to cope with my hip and back pain, need a wheelchair to get around outside or use a walking frame around the house, I wouldn’t be stuck at home blogging I would still be working. Her post made it seem like it was a life style choice. I can assure you its not.

The basis of the post was that we should exercise to condition and strengthen our muscles. If we can’t get out of bed we should exercise in bed lifting cans of beans as weights. We should push through the pain and just get on with it. It seems to me that this person is seriously ill informed about this condition. Either that or she is at the very mildest end of the spectrum and hasn’t experinced dislocating her shoulder just by rolling over in bed.

Due to our crappy collagen we injure much more easily than “normal” people. Unsupervised exercise or exercise routines that have not been devised by a health professional such as a physio that has knowledge of connective tissue disorders should not be undertaken by people with EDS. Due to the fact that they can do much more damage than good.

Our village hospital physio department is not allowed to treat patients with connective tissue disorders for just this reason. All EDS patients or patients with other connective tissue disorders have to be treated at the City hospital. More damage was being done to these patients because the people treating them didn’t understand the condition.

When I was walking more than 20 miles a week and doing aerobic exercise videos I still slipped discs in my back and dislocated my knees more times than I would care to mention. Exercise can make the joints stronger but it can’t make up for the fact that the glue that holds my joints together doesn’t work.

 I am aware of the fact that my body has become deconditioned and I need to strengthen it. However I am also aware of the fact that just rolling over in bed at the moment can send me into tachycardia (a heart rate over 100 beats per minute). My POTS consultant has labled my condition as unstable as its not responding to increased fluid and salt or with the steroid florinef. He is very unhappy at the prospect of me commencing physio with it not being controlled.

I would love the author of the post  to try that exercise line on my internet chum Minxy who has EDS. Her EDS has given her such bad gastroparesis (a paralysed stomach) that she is again looking at being hospitalised to have a feeding tube placed into her stomach as she has lost so much weight. Apparently she’s not sick and just needs to lift a few cans of beans to get well. I hope you are reading this Minxy, so you know how to get better. I’d also like her to have a chat with my Dutch friend who can not transfer herself from her bed to a wheel chair without dislocating her wrists. Perhaps she should start lifting some cans of beans to improve her condition too?

I’m mad! Can you tell? Its just sheer ignorance about the condition that would allow someone to make such sweeping statements.

Why deliberately try to upset people? Why not be a little more understanding and appreciative of the differences between people? Why not try to understand that EDS is a wide ranging spectrum of a disorder?

I’m really pleased that this person has found a way to keep herself strong and healthy. I was glad to see that she was trying to publicise EDS and dysautonomia, what I didn’t appreciate was the attitude towards others who are more severly afflicted with the conditions than she is.

Some people with EDS have very few symptoms other people are very badly affected. The condition is a syndrome – meaning it has a wide ranging number of symptoms that not everyone will have. Also POTS is a syndrome meaning it has a wide ranging number of symptoms. How I may feel and how others may feel with it will differ considerably. What  is agreed upon by the medical professionals is that both conditions can leave patients feeling severly fatigued. Its suggested by some that a POTS patient uses as much as five times as much energy as a “normal” person standing up. EDS patients use 3 times as much energy as a “normal” individual just moving around and keeping their bodies together.

What disappointed me most about the post was that at no point did it suggest that someone who has POTS or EDS seek the advice of a trained professional before embarking on a programme of exercise. Be that professional a Dr or a physio.

Some EDS patients who aren’t affected severely by the condition have very little understanding of what its like to live at the other end of the spectrum. They say we are all doom and gloom in our blogs and there are never any success stories out there.

 I beg to differ I am a success story, I was written off by numerous Dr’s as having a mental health issue. I have fought and fought hard for four years to get a name for my conditions and start getting treatment. This is just the beginning of my journey.

Its all in the genes….

I wanted to update my blog yesterday but I was so spaced out from starting the slow release morphine that I slept most of the day! I have a hazy recollection of speaking to my GP in the afternoon, he rang to check I was OK on the morphine. All I could do was giggle and yawn, so I guess I sounded OK. Unfortunately the morphine is making me constipated…… I know too much information! So he has prescribed me some laxatives. I can’t do prunes or prune juice before anybody suggests that my body will not tolerate them and I don’t leave the bathroom for about 4 hours!

The morphine has worked wonders for my pain its now a 2/10 rather than being 8/10. I can cope with a small amount of pain. Today I’ve had my friend Amanda around  and I was actually with it! I still look pretty ill, quite pale and pasty but no pain.

So why was I desperate to post yesterday?

 It looks like its all in the genes for my sister and I. My sister and I got diagnosed the same day in two separate cities last month with Ehler-Danlos Syndrome Type 3 – Hypermobility. Yesterday my sister had her tilt table test. I made sure I gave her as much information as possible so she knew what to expect. As I chatted to her on the phone yesterday morning before her test she was very blase about it all. In fact she was confident that she didn’t have POTS, it was all a bit of a fuss about nothing.

Later that afternoon I had a text message from her confirming that she did indeed have POTS. She had almost fainted after they gave her the GTN spray up her nose. The GTN spray is the same medication angina patients use to open up all their veins and arteries – its a vaso dilator. Luckily they tipped the table back down again as she slumped forward. She was tested by the same team that treats me so they knew she was my sister. They talked her through her results, her heart rate had started at 80 beats per minute supine (lying down) on the full head up tilt (simulating standing) her heart rate increased to 162 beats per minute and she began to hyperventilate.

 Its an automatic response for people with POTS to hyperventilate when standing as our body is desperately trying to get our blood to circulate around our systems. We hyperventilate to prevent fainting. I noticed it when I had my tilt table test and managed to concentrate on my breathing to slow it down. At the time I was terrified that they would accuse me of inducing a faint by hyperventilating such is my mistrust of the medical profession. However since then I have learnt its a natural POTS response.

The team testing her stepped in and got her to concentrate on her breathing and bring it back to as near normal as she could manage. They were very good with her as they had been with me, even though I tried to puke on them! They talked through her results in a lot more detail than I ever got – yes I am jealous, I’m still waiting for a copy of my report! All I know is that my heart rate went above 150 beats per minute on standing but I have no idea what my supine heart rate was.

So we have both now  been diagnosed with EDS 3 and POTS it looks like its in our genes. Its not unheard of for siblings to have both, indeed I know a local family where everyone in the family has it to varying degrees. Its just taken 37 years for my diagnosis and 35 for my sisters.

The question is now for my sister does she have ME as well? We have been told for over twenty years that she has ME. Is it really that she has had POTS and EDS all this time? I know a lot of autonomic nervous system issues also occur in ME, it is a bit of a grey area. Although how POTS and EDS patients are treated by the medical profession isn’t great, (due to ignorance about the conditions) its a damn site better than the way they treat patients with ME.

This latest development has sent my parents into a frenzy to try and find out as much medical information about their families as possible. It isn’t that they are looking for someone to blame or which side its come from they are purely are looking for the genetic connection.

How to explain pain part two….

I know, I know, I post only twice last week and you end up with two in one morning. I just wanted to update you on how the phone call went with my GP.

There was no begging or pleading I just said I can’t do this anymore and I need morphine. He just said “OK”. There was no argument or trying to persuade me out of it. He was just very matter of fact and said no problem. I have been prescribed slow release morphine to be taken every 12 hours. He is starting me on a low dose as it can produce nausea. Ive never been nauseous with morphine but I appreciate his caution. He said he didn’t want me being sick as a dog and then being put off by it and not taking it.

He also decided to read out a letter he had received from my old neurologist Dr X.  Dr X had obviously received a bit of a shitty letter from the rheumatologist I saw privately in May 2011. In his letter Dr X acknowledges that I have been diagnosed with Postural Orthostatic Tachycardia Syndrome and Ehler-Danlos Syndrome type 3. That must have killed him after saying that there was nothing wrong with me 12 months ago! He then goes on to say that I have been extensively tested for Myasthenia Gravis and there is no clinical evidence to back this diagnosis. In this respect he feels there is no point me being referred to his clinic. In lay mans terms he is refusing to see me, despite the fact I have something neurological going on. At this point both my GP and I dissolved into a fit of the giggles!

I was very calm and said “It must be very embarrassing for him to admit there is something actually wrong with me as 12 months ago it was all in my head”. This was greeted with more giggles from my GP so I added “sorry that’s not very fair of me to say that to you as you can’t comment.” At this point my GP cleared his throat and said “Yes, moving swiftly on”.

Its nice to finally have a GP that believes me and is willing to learn about rare conditions. It didn’t happen at the last GP practice I was at. They still believed in if you hear hoof beats its a horse not a Zebra. My GP takes the stance yep it could be  a horse but it could also be a Zebra. He doesn’t hold with “That condition is too rare” as an excuse not to test for it. Just because its rare doesn’t mean that no one has it, its just means very few people have it.

I’m still pissed off with my GP due to him forcing me to have a private referral to the rhuematologist. His logic at the time made no sense to me and still doesn’t. I understood today that he is actually against private referrals due to his political beliefs. I explained to him that I too hold those beliefs however when it is your health you are fighting for sometimes you have to compromise your principles. I certainly would still be without a diagnosis of Ehler-Danlos Syndrome if I hadn’t been prepared to compromise.

So I just need to find my previous letters from various neurologists and copy them for my GP and then he will refer me to another neurologist at the local hospital.

Is it weird that Dr X’s refusal has made me laugh?

How do you explain Pain?

With my health in the way it is I am often asked to explain the level of pain I am in. Like most EDSer’s I’m not a screamer or shouter when it comes to pain, I go quiet, get quite grumpy and do lots of breathing exercises. To the outside observer I may look absolutely fine, on the inside every joint in my body hurts. I can honestly say today that the only place that doesn’t hurt is my feet. I look dreadful as I am doing a Casper the friendly ghost impersonation and have huge dark circles under my eyes.

Why do we EDSer’s suffer in silence? Why can’t we scream and shout about pain the way that other folks do? I believe its because we are so used to pain and not being believed when we tell people that we are in pain, we have lost the ability to express it. I mean whats the point in telling someone if they can’t do anything about it? Or they are going to tell you its growing pains, its all in your head or you are making it up for attention.

When I try and describe what my pain feels like words escape me. I can’t tell you, all I can say is I have used every drug in my arsenal and I am still hurting. I can only reduce that pain by lying very still on an electric blanket put on its highest setting. It doesn’t mean that the pain has gone, it just means it is at a level where I can distract myself from it.

The one to ten pain scale means nothing to me. Everyday starts off at least a five at the moment and depending on how my body wants to play it the pain either increases, stays the same or drops down to a 2 or 3. Ten is supposed to be the worst pain you have ever experienced, most Dr’s and nurses compare a 10 score with giving birth. This is silly as it automatically excludes 50% of the population who happened to be born male and will never experience this. It also excludes me and many other women who have chosen not to have children.

 I have had a ten moment in 1998 when I had bowel adhesion’s and the pain was so bad I was going in and out of consciousness, do women in labour do that? I don’t remember hearing of any women doing that, maybe they do. That’s my ten that I use to score my pain against, so far its never been a ten again.

The pain at the moment is at the point where I really would rather not move. If I could get away with not leaving my bed to go to the bathroom it would be ideal. Any movement triggers spasms and waves of pain. My fingers currently feel like a metal bar is being drilled down inside the bone. My back feels like someone is stabbing me and punching me. Everything else hurts, throbs etc but is not in the forefront like my back and fingers.

Apparently last night I woke hubs up by moaning and groaning in my sleep. Please remember he sleeps in a separate room to me. I vaguely remember him coming in and asking me if I was OK. I can remember feeling pretty cross at being woken up and just saying pain. I dropped off back to sleep making hubs even more confused than ever. He didn’t know if he should wake me and dose me up with medication or let me sleep. He decided to let me sleep as I had been so grumpy when he had asked me if I was OK. I don’t blame him, I can be very grumpy and aggressive if I am woken up from sleep for no good reason. Unless the house is on fire please just let me sleep!

So today yet again I am waiting for my GP to ring. I am going to be brave and just ask for the Morphine instead of dancing around the issue. I don’t care if he thinks I am a drug seeker or am a baby for not being able to cope. I can’t wait for a physio appointment at the hospital or the appointment with the acupuncturist. I am in pain now and I don’t want it anymore. I fully intend to only use it when absolutely necessary and to stop using it when I start the acupuncture and the physio. I don’t want to be on it all the time.

So wish me luck and hope that I don’t chicken out!

My Week….

I feel quite naughty as I haven’t posted as much as I normally do during this last week. By now on average I’m up to three posts. With all my new followers and roughly 20-30 page views per day I feel a bit guilty for not updating you on my week.

Well this last week has been a bit of a blur, mainly due to taking Amitriptyline 10mg during the day. Boy does that stuff really knock me out. Today is the first day that I haven’t felt like I have consumed 3 or 4 pints of strong lager. The Amitriptyline has been prescribed for my nerve pain and it works quite well. Its not gone completely but its the only thing that’s ever taken the edge off.

 On Monday my GP was so pleased with the results I am getting with using this medication he suggested adding in 10mg in the morning. Ive been in a warm fuzzy / sleepy state all week and totally out of it. To be honest I have barely been able to function. I am a member of quite a few forums and I have stupidly posted on a few of these boards. I was barely coherent talking so goodness knows what my typing has been like!

I have deliberately not done anything official or that needed high levels of concentration this week due to not being entirely on the same planet as everyone else. Thankfully the dopey/ dazed effect seems to be working its way out of my system. I have now been taking it for five days and my GP did warn me I could be completely floored by taking it during the day. I have spent most of the week in bed listening to Radio 4 Extra or the BBC World Service.

The results at night have been very good. I take 20mg of Amitriptyline about an hour before I want to settle down. Since taking it I have been sleeping a lot better and the quality of my sleep has also improved. I am still waking up during the night due to being thirsty or needing the loo, due to my excessive fluid intake but I am dropping off to sleep much more quickly when I get back into bed.

I didn’t think the amitriptyline would work but I gave it a go anyway just so that I could say that I had tried it. I am really glad that I have. I think the dose may need to be gradually increased to get the full benefit. However for the first time in over ten years I have no burning sensation in my left calf which always seemed to start at night as soon as I got into bed. My left thigh also no longer continually aches. I still have a numb spot on my hip just where your knickers sit, but hey you can’t have everything sorted straight away. Its going to be trial and error until we find the right pain medication to get everything under control and more manageable.

Due to my two falls on Monday I have had quite a bit of pain this week. The Amitriptyline is great for the neuropathic pain but it doesn’t stop the joint and muscle pain. Presently I feel like I have been trampled on by a horse. I am keeping on top of my pain medication and on the whole its dropping it down to a more tolerable level. Some days its worse than others but at present I am coping.

Mood wise I have been a bit up and down. Its been a much better week than last week and the support I have been shown has truly touched me. On occasions its hard to concentrate on the good things in life and you find yourself focusing on the bad. I am trying to stop myself when I notice that happening. I distract myself with music, TV programmes or ringing / text messaging friends.

I don’t have anymore to share with you in this post having been barely conscious this week! Hopefully I will be back on planet earth properly soon! Until then take care everyone.