Normal or Difficult Problem? All depends which specialist I see……..the joy of chronic illness!

I had a Personal Independence Payment medical review this week (more of that another time!). My GP had requested a home visit, mainly as my dysautonomia/fainting is so much worse, but I was merely given another appointment a little nearer to home.  As it seemed near impossible to tie up the DWP who would have received the letter, with the review service (formerly known as ATOS) we decided between us that Duncan would try to get me there, but the GP was adamant not without all the letters that she had been sent recently.  Half a tree later we left her surgery – and this was just the correspondence since the beginning of the year.

IMG_20170715_125451671

PAIN PALS BLOG

The day after the assessment, I received a copy of the letter dictated by my new rheumatologist to my GP.  Lovely man, but the person who he describes in this letter doesn’t appear to be me.  In fact she is completely at odds to the lady described a month earlier by the orthopaedic consultant, after a dislocation that required a trip to A&E!  The history of my shoulder pain was wrong, and apparently I have “a reasonable range of movement” …”with some discomfort” – ahem, I am hypermobile with very stretched ligaments & tendons so everything moves in every direction and I was actually referred for PAIN management.  Apparently my “other joints are normal” and I “really know how to manage my hypermobility syndrome”.

6e49d4ef1804a67cbe00f04a08dbad28

Image saved from Pinterest

The ortho consultant said “she has been having pain in that shoulder for some time and was seen by my colleague 2 years ago…” and “on examining her shoulder it moves through a reduced range and her x rays show progressive glenohumeral joint arthritis, with a marked difference from 2 years ago”.  He goes on to say “Unfortunately Claire has a very difficult problem to solve. She has EDS….& has developed arthropathy within the glenohumeral joint”.  I was told 2 years ago that if I were 30 years older I would be whisked in for a shoulder replacement……this time the consultant could see a huge deterioration on x ray, but again explained that a shoulder replacement with my EDS is just not really an option as my ligaments & cartilages are so stretched.  A shoulder replacement would not stand up to recurrent dislocations!

So, the thing that the two specialists agree is that I have a really good knowledge of EDS and both do recommend conservative treatment with physiotherapy – again!  The physio with expertise in hypermobility syndromes who the rheumatologist recommends is out of area; my old physio who works with the ortho consultant is no longer able to see me on the NHS as he is a Consultant physio……..

This is the reality of a condition that affects multiple body systems with no one person leading the care!  So now the search for a physio, who understands that I can’t be “fixed” but need ongoing rehab,  is ON!!!

Monday Magic – Inspiring Blogs for You!

Good Morning!

I hope that you are all ready for another week – tennis at Wimbledon, more sun, heat and BBQs, and for many the end of the school term and for some the beginning of the long summer holidays.  So I really shouldn’t moan about this heat wave that we have in the south of the UK, but it is really sending my POTS/dysautonomia off the scale.  Please send me all your top tips and I will put a post together – funny tips too please!

Anyway I bought a big straw hat – not easy when you have inherited the family huge head! – and have been away for a couple of days with my parents to visit my brother at his new house.  We are talking brand new – living on a building site would aptly describe the estate at the moment – and my sister-in-law still has a lot of boxes to unpack.  Think I would be correct in saying mainly make up and bling…..she won’t be offended!  We were taken to the school play and end of year prize giving on Thursday, which was an incredibly hot day.  The children, aged 5 – 11, did a fantastic job of an interesting amalgamation of Romeo & Juliet, Peter Pan and the Cow who wanted to grow Sunflowers – beautiful costumes!  The whole event took place in a marquee – small Oxfordshire private school – and the heat during the afternoon was horrendous.  I think maybe the head should have adjusted her speech….shortened it!!

IMG_20170706_125802603_HDR

Well earned picnic at nephew’s school – featuring Grandad!

Anyway,  despite being armed with said hat, water and the salt grinder from my brother’s kitchen, Auntie Claire had already fainted on leaving the portaloo.  But I completely stole the show at the end of the day with a fantastic backwards faint when standing up from my wheelchair to get into the car.  I came round on the gravel carpark floor surrounded by faces….not just those of my family!  There was a parent who is a doctor, the school nurse, a teacher……and my mum trying to explain POTS, my spinal cord stimulator etc etc…..and please don’t call an ambulance!!  The school nurse was quite excited, having come to these events for years and having nothing to do.  As a fellow nurse I loved this

!

IMG_20170707_120939418

The stunning Hornbeam Walk at the Aston Pottery

Like many of you, I have spent the weekend feeling constantly giddy and last night managed to pop a hip and dislocate the knee and ankle on the same leg – the joys of EDS.  So whilst I should be at a governors’ visit day at our local primary school – Duncan would not let me go unless I could weight bear – I am instead sitting with leg up and finding some great posts for you.  The final You tube video comes from a young vlogger who appeared on the BBC yesterday morning to discuss organ donation in the UK – he has cystic fibrosis and is awaiting a lung transplant.  This post is about living with a chronic illness as a teen.

Time for your cuppa and relaxation time with some inspiring posts! Enjoy!Monday Magic

http://alifewellred.com/embrace-the-years-with-dignity-and-beauty/

http://xofaith.com/boost-your-confidence-beyonce-edition/

http://www.fromthispointforward.com/2017/07/facing-forward-jayne.html?m=1

http://www.thepaincompanion.com/blog/dancing-through-pain-to-freedom

http://bladder-help.com/role-hormones-bladder-health/

https://itrippedoverastone.com/2017/07/07/what-my-husband-said-to-me/

https://neurodivergentrebel.com/2017/07/07/lets-talk-take-a-break/

https://picnicwithants.com/2017/07/05/floaters-and-flashers/

https://theedschronicles.com/2017/07/06/model-with-eds-uses-her-condition-to-stand-out/

 

Please remember to like posts and follow these great bloggers!

Claire x

Friday Feelings with Pain Pals Blog

I am really pleased to have been featured on The Zebra Mom regular Friday Feelings feature. Please check it out – and the rest of her great blog! Claire x

The Zebra Mom

Hey there, hi there, ho there!

As it is Ehlers Danlos Syndrome Awareness Month, during the course of May, we will be reading the diary entries of EDS sufferers. Each person experiences their illness differently and I think it will be interesting to see these differences throughout the month.

This week I spoke to Claire from Pain Pals Blog. The mum of two previously worked in health care but medically retired nine years ago. She now works in the education system and enjoys Spoonie friendly hobbies.

Claire was diagnosed with hypermobile EDS at 42. She also suffers from migraines; dysautonomia/POTS, chronic nerve pain, gut problems, Raynauds, neurogenic
bladder and reactive depression. You can find Claire on Twitter, Pinterest and Instagram. 
EDS - Claire pic

“Hi, I’m Claire. I am a married mum of 2 boys aged 21 and 18, and a girl aged 14 living on borders of South London & Surrey, UK. My career was…

View original post 1,195 more words

For EDS Awareness month from Sunshine & Spoons “What It’s Like To Be A Kid With Ehlers Danlos Syndrome” – including mine!

Another great post for Ehlers Danlos Awareness month, this time from Hannah at Sunshine and Spoons blog.  As a child growing up with aches and pains, dislocations, sprains, dizzy spells, clumsiness, migraines, circulation problems….it really was just how life was as no one even thought to join the dots, let alone consider a syndrome that might be responsible.  I never even heard the name “Ehlers Danlos” until my late 30s – although Marfans was mentioned to me aged 20.

I do know about EDS now and have made it my business to as so many with rare illnesses need to.  The result has been that I recognise my kids are growing up symptomatic – although getting a diagnosis is proving difficult as referrals are pushed from the desk of one consultant to another – and understanding that not everyone has pain daily, struggles to keep up with hand writing in class, is constantly twisting an ankle or popping a joint.  One son is living with severe migraines for which we seem to be in a constant loop of changing medication to bring some control as he is about to sit his A level exams.  His long neck gives him daily pain and undoubtedly contributes to his migraines.  My daughter, aged 14, has dislocations, daily joint pain, dizzy spells, writing problems…..the eldest, the student engineer aged 21, is hypermobile, has a sternal “deformity”, clumsiness, dizzy spells – sound familiar?? All three, whilst very bright, suffer massive anxiety to such an extent that over recent years we have visited CAMHS, Heads Together, use of anti depressants and mindfulness techniques.  I feel a whole post coming on…..being the age they are, mum has not been able to pin them down for a quote as I write this!!

File_000 (20)

My younger 2 zebras and me!

So without further ado let me introduce some great children who have spoken to Hannah:

This post may be a little hard for you to read. I know I had tears in my eyes more than once while typing this up.

If you’ve been hanging around Sunshine and Spoons for very long, you know that at least 3 of my 4 kids have Ehlers Danlos Syndrome, which they inherited from me. Davy (3) and Nano (7) don’t really understand it yet, but Katie (9) does. She goes back and forth between being proud of being a zebra to asking why God would make her have EDS and all of the pain that goes along with it.

Kids are supposed to be able to run and play. They shouldn’t have to deal with chronic pain and fatigue. They shouldn’t have to spend their childhoods at doctors’ offices, wearing braces and explaining random bruises.

But, that’s not how things always work. I interviewed 25 kids who have EDS to see what it’s really like to be a child or young person with the disorder. “

What it's like to be a kids with Ehlers Danlos Syndrome

 

For the remainder of the post and the children’s quotes please click on the following link

What It’s Like To be a Kid with Ehlers Danlos Syndrome

Living with Ehlers Danlos Syndrome – film for #EDSAwarenessMonth

I am really pleased to have contributed, in a small and rather quiet way, to this video that Jenni has put together for EDS Awareness month.  Jenni is a vlogger/blogger and goes by the name 1nvisibl3Girl – please have a look at her channel & blog and the social media sites of the other great (very young!!) EDSers on this short film!!

“This video is all about living with Ehlers Danlos syndrome (EDS) as the zebras I have been lucky enough to get to know, and I, share our own experience of this chronic, invisible illness. We talk about what EDS is to us, how we manage our symptoms, how EDS has changed our lives, why we started our own EDS based blog or vlog and our hopes and dreams for the future. We hope this is shared as much as possible this May as it is #EDSawarenessmonth so people can learn what it is really like to live with EDS but also to support those also living with the disease. I know it is long but please watch it all if you can. There are some amazing people describing some very difficult things in their own words. This is a project I am very proud of.” Jenni Pettican

Managing dislocations – “I’m just Popping out for a While” – article by Jason Parry, Specialist physiotherapist

This article appears on the Ehlers Danlos Support UK Facebook page during EDS Awareness month.  It offers great tips for managing the dislocations that are a part of every day life for us zebras.

‘I’M JUST POPPING OUT FOR A WHILE!’

Article by Jason Parry, Highly Specialist Physiotherapist

18157207_1436435379741855_1444168691171431439_n

One of the most common questions I get asked is how to manage dislocations and subluxations, which is quite strange if I happen to be popping to the shops for some milk at the time. However, in all seriousness, this really is one of the most frequent questions I face in clinic when treating many of my hypermobile patients and let’s face it, it really is no laughing matter.

So where do I begin? Well, a good place is to firstly understand the difference between a dislocation and a subluxation.

A dislocation is defined as “displacement of a bone from its natural position in the joint”. This is where the two bones that form a joint fully separate from each other. If we consider a shoulder, for example, which is a ball and socket joint, a dislocation occurs when the ball (which forms the top of the arm bone) slips entirely out of the socket it should sit in within the shoulder. This can happen in any direction, but the main point is that the two bones completely separate. Ouch!

A subluxation is basically defined as “a partial dislocation”. It can be no less painful than a full dislocation, but the two bones that form the joint are still partially in contact with each other. So once again, if we consider the shoulder joint as an example, the ball which completely came out of the socket in the dislocation example above would still be partially sitting in the socket in a subluxation.

These events can happen in almost any joint, but some are more commonly involved than others, with shoulders, knees, thumbs and ankles seemingly some of the most prevalent.

Both dislocations and subluxations can be painful, irritating, infuriating and occasionally debilitating problems. However, they don’t need to ruin your life and can, with patience, effort, trust and time, be managed. Not necessarily completely eliminated, but managed.

Let’s first look at the reasons why dislocation or subluxation happens with a quick anatomy lesson. The main reason is abnormal collagen composition. Collagen – primarily Type 1 collagen – is the main structural protein of the various connective tissues in the body. It is found in ligaments, tendons and joint capsules, and makes ligaments and tendons strong like little ‘guy ropes’. What do ligaments and tendons and joint capsules do? Ligaments connect bone to bone, tendons connect muscles to bone, and joint capsules are like envelopes of tissue that surround a moveable (synovial) joint. So we can see that these ligaments, tendons and joint capsules play an important role in giving a moveable joint its stability.

Let us now consider hEDS (hypermobile type EDS). This is a Heritable Disorder of Connective Tissue (HDCT) caused by a defect in the structure, production or processing of collagen, which makes the collagen in ligaments and tendons stretchier (more lax). This means that joints are potentially less stable – hence greater propensity for subluxations and dislocations.

There are other reasons for dislocations and subluxations:

• Altered muscle tone: This can often account for dislocations. Inappropriate muscle patterning, in which certain muscles around a joint ‘switch on’ when they shouldn’t and then inappropriately work way too hard, can often ‘pull’ a joint out of place. The joint then also becomes easier to slip out, of course, if it is more lax in the first place. Muscle fatigue, spasms and stress can all play a part in this too.

• Impaired proprioception: Proprioception is the body’s ability to sense position and movement within joints and enables us to know where our limbs are ‘in space’ without us looking. It relates to coordination. Impaired joint position sense can cause joints to slip out of place.

• Repeated overstretching: Otherwise known as too many ‘party tricks’. I know many of you guys have the capacity to ‘amaze’ people with your ability to wrap yourselves into weird and wonderful positions that the rest of us gawp at. You know, those tricks you did as kids, popping your shoulders in and out of joint, or folding your legs over your head? Well, stop – it’s no good for you! Repeated overstretching to that degree will only exacerbate the laxity and the chances of the joints slipping out of place. I’m certainly not saying that you should never do stretches, but I am saying give up the party tricks and don’t stretch your joints way beyond ‘normal’ range. Just because the joint ‘goes there’ doesn’t mean you should take it there. So please forget about that career as a contortionist with Cirque du Soleil.

• The shape of your joint surfaces: Some of you may be born with shallow-shaped joint sockets or other bony shaped ‘anomalies’ that predispose a joint to possibly slipping out of position more easily. Unfortunately, that just may happen to be the shape of your skeleton.

• Traumatic incident: One of the most common reasons for a joint to come out of place for those of us without EDS. Traumatic incidents can happen to anyone, but your extra joint laxity may actually work a little in your favour with this one; it may prevent you damaging some of your ligaments/tissues in the way that a non-hypermobile person who suffered a traumatic dislocation probably would.

How often can these subluxations/dislocations happen, I hear you ask? The answer to that is different for different people. Some people get them maybe just once or twice a year, others once a month. Some people get them once a week and others once a day. Some people get them repeatedly throughout the day and in some people they never seem to stop. Either way, we need to try to reduce the frequency if we can, and manage them when they do happen.

In some people, the joint just finds its own way back in to place, and phew, what a relief. But in others, once the joint slips out, it won’t go back in again. The pain kicks in (often big time) and the most common and perfectly understandable reaction is… PANIC! At this point, some people pick up the phone and call for an ambulance – well actually they don’t; they’re often writhing in agony or they can’t actually pick up the phone especially if its their shoulder or wrist out of place, but someone else does – and off to A&E they go.

BUT WHOA THERE! STOP FOR A SECOND, DON’T PANIC, BREATHE NORMALLY AND STAY CALM!!

Panic causes more stress and more muscle spasms. Stress and muscle spasms cause more pain, and then there is less chance of resolving the dislocation. Easy for me to say, I know, sitting here with my joints all lovely and located without the associated agony. But trust me, if you want to start managing this situation and taking control, then this is what you’re going to have to begin to practise. Because what happens at A&E? Well, if they aren’t already fed up with you turning up 100 times a month and starting to get all ratty towards you – not fair I know – they will often give you pain relief of some sort (perhaps Entonox) or they may go the whole hog and give you a general anaesthetic. Then they’ll yank your joint back into place. All good, right? Not so, because often, maybe within minutes, the joint will pull itself back out of place again because of the muscles still spasming around the joint, and you’re back to where you started.

So what else do our A&E docs then sometimes do? They stick you in a plaster cast to ‘hold’ the joint in place. Imagine, then, the battle going on underneath it – your joint trying to pull itself back out of position again while being forcibly held in place by the cast. Sounds painful to me, and often is – and when then do you take the cast off? This doesn’t sound like a viable management solution to me or a good way of life for you.

So what should you do if your joint comes out? Here are the 6 key principles that I suggest you need to start incorporating in order to begin to get a grip of managing this situation as opposed to this situation managing you. The main aims are to stay calm, keep on top of the pain and allow the muscles to relax. It takes lots of practise and patience, but it can be done.

1. Breathe : Use slow deep, relaxed breaths. Try using some relaxation techniques, there are lots of different ones out there. As painful as it is, and as difficult as it may sound, you need to start to try to take control of this situation. So start to learn how to breathe through it.

2. Use Painkillers: Oh yes! I’m not a monster you know; I know how painful this situation can be. I’m not for a minute suggesting that you should just sit there and ‘suck it up!’ For goodness sake, take some appropriate painkillers (analgesia) if you have some. However, note the word ‘appropriate’. You should only ever take analgesia according to the dosage indicated by your prescriber. Never take more than the suggested dose. You might feel like it may not be enough at the time, but if it can take some of the edge off, then that’s a great start. Please don’t ever overdose.

What about Entonox (commonly known as gas and air) as pain relief? I am aware that some people have access to Entonox at home, or use it at A&E. There can be a role for it, but this must be used with caution. Prolonged use can lead to vitamin B12 deficiency and can interfere with DNA synthesis, not to mention cultivating a dependency, which are all big issues.

3. Support the joint: You need to try to make yourself as comfortable as possible (I know it’s not easy). Use pillows or a sling of you have one. Find a comfortable resting position as much as possible. This allows the muscles to relax and stop spasming.

4. Try heat: Hot water bottles, wheat bags and a warm bath can all help to relax spasming, overactive muscles.

5. Distraction: Try to take your focus away from the pain and the situation. Listen to music, watch a film if you can, talk to friends/family, try a relaxation CD/MP3. This can be helpful as a short-term pain relieving strategy. Again, it can help muscles relax.

6. Gentle massage: Sometimes gentle massage around the joint can help relax the muscles enough to be able to gently re-locate the joint or for the joint to just slip back into place by itself.

What if it doesn’t go back, I hear you scream (and I do hear you scream)?!

Don’t expect the joint to go straight back in. It is often not unusual for joints to remain out of place for hours or even days. But once it’s out, it’s out. It’s not going out even more, so try not to panic.

Isn’t it dangerous though? What about damage? Am I damaging my joint if it comes out?

It is highly unlikely. Your joint laxity allows for your ligaments and capsules to stretch. It is mostly just distressing as opposed to damaging.

When should you go to hospital or get help?

• if the limb starts to change colour due to a lack of blood supply
• if your limb goes completely numb
• if you have tried strategies 1-6 above, have waited a reasonable amount of time, and are still desperately struggling.

But as mentioned earlier, it is not unusual for A&E to relocate your joint only for it to pop straight out again or when the anaesthetic wears off. Therefore you need to learn to stay calm and to start to self-manage.

Learn Lessons!

One of the most valuable things you can do after a subluxation/dislocation is to reflect on the event once you have had a chance to calm down. Were you moving in a way that normally causes the joint to dislocate? Did you move without thinking? What was your posture like? Were you tired or overdoing it? Were you stressed about something? It is so valuable to look for triggers as to why the event may have happened. It may have been none of these reasons, but if it was, then you can hopefully learn to avoid repeating them in the future.

Finally, prevention is better than cure! It is obviously better if we can prevent these situations occurring in the first place as opposed to having to deal with them. To that end, the following can hopefully help to reduce the frequency of such occurrences:

• physiotherapy to learn to control the muscles around joints and to use the right ones
• rehab to improve proprioception
• the possible use of supports/braces if required
• trying to manage stress and anxieties.

But ultimately, stay calm! The more you stay calm when these events happen and manage it yourself, the easier it should get each time.”

#EDSawarenessmonth

Rheumatology, Cardiology, Falling Down and Midodrine

Finding the time and energy to write has been a challenge recently.  I’m not sure if you would describe my worsening ailments as a flare, but my poor, body has been struggling to hold things together.0ff7167e97f625a09a50879d90d5057c

Last week I saw my GP and my cardiologist and managed to get two new referrals in the process.  The first to a new rheumatologist, recommended by the London hospital who diagnosed me, and the second to an endocrinologist to check if the cardiologist is missing anything.  Don’t you just love these multisystem chronic illnesses?!

017096efedcc1c8ede7971c3ed094485

For the rheumy we have a family outing on the cards, as the A level student and lovely girl have also been referred.  The bookings department called last Friday and asked if we would like consecutive appointments – I bet the consultant will love it when she sees the same surname appearing three times in one morning.  “What another genetic problem?!”  I have put off having the kids diagnosed with hEDS even though I can see elements in all three of them, but it has only been recently that I learnt from the lovely online support that they might be eligible for extra help in exams.  This is down to the pain and difficulty with writing that we with bendy fingers and dodgy collagen experience.  My daughter has never been able to hold a pen properly and they both struggle to be able write as fast as their peers – their brother, the uni student, was exactly the same at school.  In fact his fingers are so flexible that you might think they were missing their bones altogether.

The schools have responded really well and thanks to some fantastic blog posts that I was able to direct them towards, have now heard of Ehlers Danlos Syndromes.  We missed the deadline with the exams boards for the A level student to receive extra time (he needs a formal consultant recommendation) but he is allowed to be in a smaller room and to have rest breaks.  Had his typing been quicker, he could have opted to use a laptop.  My daughter’s school have been fantastic!  They are keen to learn – obviously they have seen me deteriorate after repeated surgeries and progress into a wheelchair – and many teachers have been upset to learn that she has not spoken up about pain, dislocations and difficulties in games classes.  Of course she is worried that a fuss shouldn’t be made as there are other people more in need!  But in terms of her year 10 exams after Easter, she will use a laptop and take rest breaks.  When I was at school I had no idea that the pain I always had and my difficulty getting through written work was actually down to a genuine problem – in fact at the beginning of this academic year none of us dreamt that our pain would actually be considered a reason for the kids to need extra help!  Of course the new guidelines came out last week for diagnosis criteria, so I’m not sure what they might be deemed as – well I could say a few choice words that have nothing to do with EDS!

More to come about the criteria and of course how we all get on.

2017_classification-small

The EhlersDanlos Syndromes International Classification

I have been on the waiting list for a follow up cardiology appointment since the end of last year.  Immediately after my tilt table test, I started the synthetic steroid fludrocortisone to increase my circulating fluid and raise my blood pressure…if anything it made me worse as the faints, falls and injuries have come thick and fast.  My GP tried to have an appointment fast tracked, but when this couldn’t be arranged the cardiology department told me to double the fludro dose and continue with compression tights, fluids, salt.  I have felt so unwell and so tired!  Last week the consultant started me on midodrine, which in the UK can only be prescribed by a cardiologist for extreme cases of low BP that haven’t responded to conventional treatment.  It is early days and I am only on a low dose three times a day – but I cautiously think there is a small improvement.  The last dose of the day mustn’t be too close to lying down in bed as there is a risk of increased blood pressure when flat and strokes.  One side effect I do have is a strange creeping, tingling sensation of the skin particularly my scalp – about an hour after I take it when it reaches highest blood concentration level.  So if you see me out and I’m itchy, I really don’t have nits!!13413444_143336392741362_1604407187_n