Keeping Vocal - Speech and vocal problems in EDS and how to manage them

Speech and vocal problems are very common in EDS. In fact it is one of the earliest signs that a child may have EDS that is now becoming more known. Speech delay, selective mutism, extremely low-volume speech, and other speech problems are very common in early years of EDS children. Speech problems, swallowing problems, frequent coughing and choking are common problems that continue through adulthood.

Speech delay was probably one of the first signs there was something wrong with me as a small child. I didn't really speak until I was about four years old. Even then it was only when absolutely necessary that I actually spoke. I think I was even sent to a speech therapist to find out why wasn't talking. I was very quiet, and when I did eventually speak it was with a very soft, low-volume voice.

Thankfully as I got older I got more confident with speaking and eventually it was hard to shut me up LOL.
However, now with EDS affecting me more than ever, speech and vocal problems are common parts of my day.

The main problems now are that my voice, or rather my vocal chords and throat muscles, get tired very easily. The effect of this is pain, throat spasms, and fatigue. This results in my voice getting quite croaky, low and manly sounding, and either quiet or strangely louder.
I get sore throats a lot and I can also get quite breathless whilst speaking, however this breathlessness is more to do with POTS I believe.

As I've got older I found I struggle more pronouncing words that I used to have no problem with. Words that start with a W, WH, and WR and when a R word comes before or follows a W word e.g. ‘white rabbit’, or ‘real writing’, take a lot of concentration, and sound just wrong. It frustrates me because up until a couple of years ago I didn't have these problems pronouncing words.

Looking after the EDS voice

Thankfully as a trained singer I am very aware of how the voice works, problems that can develop, how to protect the voice, and treat problems. The voice is basically a group of muscles, and like any other group of muscles that are worked regularly and harshly, they need good maintenance and care.

If you went running every day you would warm up your muscles beforehand, know when to stop pushing yourself too hard, and how to care for sore muscles.
The voice needs looking after, especially in EDS.

Here are some things you can do to look after your EDS voice, as well as deal with some of the common problems you may have:

1. warm up your voice if you're going to be talking for a long time e.g. given a presentation, speech talking on the telephone. Warm-ups include humming, making exaggerated chewing movements, moving the jaw around in circles in both directions, gently moving the neck around, rubbing and patting the neck and face gentle to encourage blood flow and pursing lips e.g. kisses.

2. Take regular sips of water, at room temperature, when talking.

3. Avoid relying on throat medicine e.g. lozenges, medicine etc too much because these numb and coat the vocal cords and throat muscles which means you can damage them more without realising. Instead soothe sore throat muscles with warm (not hot) drinks and rest.

4. Pace yourself. Just as we EDSers need to pace our energy usage, those of others with voice problems should also pace ourselves vocally. If you know you are going to need to talk a lot later rest yourself and avoid talking too much beforehand. After talking for a long time, or if you start to get sore and tired vocally, rest your voice afterwards.

5. Avoid straining your voice such as screaming, shouting, talking loudly, talking for a long time etc. Also be aware of certain environments and situations that can cause your voice problems such as dry atmospheres, smoky atmospheres, breathing in lots of cold or hot air, smoking, drinking strong spirits, eating spicy foods, eating foods that you know cause your throat to get scratchy or swell, and catching colds and flu.

6. Although typical throat lozenges aren't great to have all time, there are some products which are good to use and will help you vocally. Vocalzones are lozenges that I use regularly when I'm going to be singing. Instead of soothing the throat, they open your airways and throat which improves vocal quality and helps the voice. You can buy Vocalzones and similar lozenges over-the-counter. Ask your pharmacist for lozenges for speakers/singers not for sore throat. http://www.vocalzone.com/

There will be times when no matter what you try you still struggle vocally. There are times when I can't speak, speaking is really difficult, usually because of brain fog, although at times because my throat vocal chords are just too tired and sore.

Because of this I have some communication apps on my iPhone. ‘Buzzcards’ and ‘Quiettalk’ my favourite and can be found in the App Store. These apps enable me to communicate with people when I can't speak by either typing what I want to say or selecting a pre-typed card.
For more information and to see how I use these you can watch my video about from my youtube channel at the end of this post.

I hope these tips and information is helpful. If you would like more information or advice on specific problems contact me. I'm not a doctor or in any way authorised to give medical advice however I can recommend ways to protect and look after your voice as a vocal teacher.

New found finger freedom! The joys of voice recognition software

Hello folks, this blog is coming to you via the amazing Dragon naturally speaking voice recognition software.

I've been struggling with typing for long periods for a while now because my hands are getting much weaker and painful. When I'm not using my hands they curl right up into loose fists and I have pain in my fingers, knuckles and wrists most of the time now. I also have shooting pains through my hands and up into my arms a lot. This has been making it increasingly harder to use my hands for certain tasks. Writing with a pen has been hard work for at least a year now, typing for a few months.

Annoyingly I can touch type with great precision and speed having learned when I was younger and working as a legal secretary. So imagine my frustration when I realised recently that I was no longer be able to rely on typing as much as I have. Add to that my increasing brain fog and you can see why I was getting worried and nervous about starting to study towards my second degree in June!

I'd heard about voice recognition software including Dragon naturally speaking, I noticed that a few of my disabled friends were using it for studying. I decided I definitely needed to have a try because it was probably something I was going to need in order to get through my degree. Luckily I have a friend who already had Dragon and offered to lend it to me for me to try it out.

I've installed dragon onto my laptop about an hour and half ago. After spending about 10 min going through voice training, which gets the program to recognise your voice, vocabulary and accent etc, I then had a quick play about dictating a few Facebook posts as well as learning the basic program commands.

It seems quite surreal but after just an hour or so of playing with it, I am already able to dictate this entire blog post having never used voice recognition software in the past! The best part, I don't have to worry about my awful spelling! LOL
What I find most amazing about this is not the way the program works, or how precise it is at recognising what I'm saying, or even the speed at which I'm able to write this blog. The thing that excites me most is how much I'm going to benefit from this program.

There are many days now where I'm unable to answer e-mails, chat on Facebook as much as I would like, and there is some work that I've needed to do for the past three weeks for my Bible study class that have been unable to do. And as for my blog, it's something that I rarely get to do now because my hands and brain just aren't up to it.

With this program I will be able to keep up-to-date with my work, friends, and studying. Not only that but I will also be able to do things and try things that I've been wanting to have a go at but just not been up to it. I hope that with voice recognition software I will be able to get blogging regularly. One thing that I'm looking most forward to is been able to do work, type letters and chat with my friends online on my bad days where I am stuck in bed with very little energy and too much pain.

I realise now just how invaluable software like this is to people who are far more disabled than I am such as people who have quadriplegia, spinal cord damage and other paralysis.

So there we have it, thanks to modern technology, you have been able to enjoy some late-night ramblings from me thanks to Dragon naturally speaking voice recognition software and my very supportive friend Angie!

I'm off to catch up with my snail mail. I have at least 10 letters from my chronically ill penpals that need answering. And guess what… I think I'm going to get through them all :-)

Moving on

It’s been nearly a year since I last blogged. You are probably wondering where I’ve been and what I’ve been up to.

The last year has been a tough one but it has also been filled with positive experiences, new friendships and a lot of personal reflection.

The main focus of the past year has been on my gastro-intestinal problems which have deteriated further. My large intestine/bowel has shut down completely now. There is no motility anymore which caused increasing blockages and pain. I am now very blessed to be under the care of a great GI dr who not only has a particular interest in functional GI problems but also treats a number of patients with EDS/POTS and has developed good understanding of how these conditions affect the GI system.
We have tried a number of different medications and intestinal flush systems but none of them helped. We did consider ACE surgery but have since discovered that my small intestine/small bowel and stomach are now also affected. These are causing me to be sick a lot and have problems eating. This makes surgery too risky and we need to keep my small bowel and stomach functioning as much as possible for as long as possible.

My GI dr wants me to take part in a clinical trial using Sacral Nerve Stimulation to give some motility back to my large bowel along with a new medication waiting for UK licence that is supposed to be very good for the type of abdominal pain I have.
We are running out of options so we are both hoping that the Sacral Nerve Stimulation and new pain medication will help.

2012 is becoming quite a year of change for me. I am in the process of applying to do voluntary work at my old College and I am tightening my purse strings so I can save up to study with the Open University. My aim is to study a few undergraduate modules to get me back into studying mode and then go on to study for a Masters in Art History.
This is a huge challenge but I am determined to do it. I have always wanted to go on to do a Masters degree and when I decide I want to do something I get it done. Even if that means a lot of hard work, life changes and suffering more health problems as a result of pushing myself harder.

When I first got ill 5 years ago I had no idea it would take as long as it has for me to get to the point where I could get control of my life again. The first two years were spent dealing with the grief that everyone goes through when diagnosed with an incurable condition like EDS. Once I had dealt with that and accepted my new future I then had to learn as much as I could about EDS and POTS. The knowledge I gained has played a massive role in the treatments I’ve chosen (and those I turned down) as well as helping me adjust and adapt to my new challenges. Last year focussed mainly on figuring out what I was going to do. Would I fall into a pit of despair and let my health dominate my life and dictate everything I did and didn’t do? Or would I fight and take back what I could control and find ways to deal with those I couldn’t control? I chose the latter.

I am ready to get my life back on track. To find a place in society where I can contribute and make a difference. I will no longer let my life be controlled by a condition that I have little control over.
I had to find myself again. To figure out who I was, who I had become and where I fit in.
I have started the process of fitting back into the world. I have been leading teaching sessions at bible study and currently preparing my second course for the group. I have volunteered to help with my Church’s Sunday school and youth group. My voluntary work at the college should start soon and I am hoping that it may lead to a small part time job to get be back into employment.
All I need to do now is get my brain-fogged brain working again so that it will be capable of studying the courses I am aiming to do.

At the beginning of this whole journey when I was first diagnosed I had no idea how much my life would change. There was no warning given that my life would be put on hold for so long or that it would be as hard as it was to get out of that hold.
But I am ready and I can’t wait to start this new stage of my life.

With the love and support from friends and family, and the grace of God I am ready.

The Drama of EDS - The TV Freakshow

EDS is becoming the subject of TV dramas as the condition is becoming more well known. You would expect that this would be applauded by sufferers and their families as a much needed awareness achievement. However the storylines used by show writers using the condition to boost their ratings are failing to raise any awareness of what living with the condition is really like. Instead EDS is frustratingly becoming the freak show storyline causing more confusion and increasing ignorance surrounding the condition.

Last night's episode of House sparked a flurry of complaints and discussion on social network sites just weeks before May becomes EDS Awareness month in many American States.
While support organisations work on the finishing touches of awareness strategies and campaigns for more funding for essential research into what can be a fatal condition, they are sidelined into dealing with the aftermath of another poorly researched storyline causing upset and anger amongst sufferers.
The episode focussed on a woman with a severe anxiety disorder that culminated into an extreme state of hoarding. The character's house was filled to the ceilings with years of personal belongings, rotten food and even a dead cat in the freezer.
The hoarding caused the woman and her husband to become ill from a disease caused by raccoon droppings. The husband quickly recovered while the wife was suddenly struck down with a heart attack. A few scenes later and House's medical team discover the woman had suffered three miscarriages which sparked the anxiety and hoarding.
'You have Ehlers Danlos Syndrome which explains the symptoms and probably caused the miscarriages. We can treat the symptoms as they occur'.

Most people with EDS watching the show did a double take as her diagnosis was so quickly achieved wondering if they had missed a few scenes while making a coffee.
'Did I miss something?' One EDS sufferer asked her husband...'No you heard it right' was his reply.

I watched the show knowing the expected diagnosis so looked for small clues but other than the heart involvement there were no clues that an EDS diagnosis was to come. Even then heart attacks are rare in people with EDS and are not a known symptom or complication of the condition.
In the Vascular type of EDS patients are at risk of aneurysm in the brain and heart and it is this rupturing of the heart vessels that leads to death, not heart attacks.
The only symptom that connects to EDS is anxiety. Anxiety disorders are very common in EDS but feature more in the Hypermobile type, not the Vascular type. As the woman had no joint or skin involvement the only possible way that EDS was believable is that some patients with Vascular EDS can be affected with no joint or skin involvement and the condition is only discovered following death from an aneurysm. It seems totally implausible that a patient could present with no joint or skin symptoms, no signs of aneurysm but then quickly and easily reach a diagnosis of EDS because of repeated miscarriages and anxiety.

The main concern of EDS sufferers watching the show is that this story line has only fuelled the already present attitude amongst some doctors that EDS patients have a tendency to have mental health problems.

A recent episode of Casualty, a medical drama by the BBC (UK) included a very small scene of a patient with EDS appearing in the A&E department with a dislocated shoulder. The Dr quickly pushed the joint back into place WITHOUT pain relief or any warning and then told another DR that the patient dislocates his joints ON PURPOSE in order to obtain morphine.
This storyline also fuelled outrage and upset amongst the online EDS community with the BBC receiving many complaints.
A lot of people with EDS refuse to go to hospital following a dislocation having received terrible treatment and attitude from Drs who do not understand the condition in the past. Some patients have been accused of dislocating their joints on purpose for attention as drs refuse to believe that joints can dislocate so easily without force repeatedly. Others have been refused pain medication for a dislocation as drs believe that the patient does not need it if they can relocate their joints easily.

I myself have avoided attending hospital for dislocations because of previous treatment. Having been sent to A&E directly from a hydrotherapy session that left me with drop foot and numbness in my left leg the consultant dr on duty asked me 'What leg do you THINK is numb'. Set back for a few seconds by his intended meaning I explained that I was sent by the physio and that I had not come of my own accord. But with a past history of depression the Consultant jumped to the conclusion that I was either attention seeking or that it was in my head.
My physio discovered a few days later that I had suffered from a build up of trapped nerves in my spine that had bulged during the hydro session but later released themselves of their own accord, not uncommon in EDS patients.
I have only attended A&E once since then following a fall and was lucky to be seen by a different Dr who was over cautious and kept me in for observation. Luckily for me this turned out to be a blessing as monitoring showed erratic blood pressure and heart rate which led to the first suspicion of POTS.

Another show which instigated anger was another BBC show, shown just weeks before the episode of 'Casualty' which also received complaints. Some of these complaints were addressed and received promises to look into any misrepresentation of the condition in order to improve further mention of EDS on the BBC.
The show 'Freak like me' followed a young girl who 'loved clicking her body'. The girl, undiagnosed, would click all her joints, including her hips and shoulders, many times a day. The show addressed this as a 'freaky habit' with no mention of the medical danger of this behaviour. Many EDS sufferers who saw the show wrote to the BBC asking them to inform the girl that she should look into EDS due to the obvious extreme hypermobility of her joints and other signs that she may have the condition. What upset EDSers is that the actions she took to 'click' her joints were similar to that they needed to do to relocate dislocated and subluxated joints. They were not only worried that the girl could be causing long term damage to her body but that the BBC had not looked into the medical risks of such behaviour which could have made them aware of EDS in order to give a medical warning to viewers.

It seems that EDS is becoming the condition to use if show writers want an interesting storyline with a freakish twist but they are failing to show even the basic symptoms of recurring dislocations, chronic pain and mobility problems. Instead they are using the condition to highlight drug misuse, mental illness and freakish habits.

The only hope is that writers of Coronation Street, a UK soap opera, uses the character of Izzy, a wheelchair user with EDS, to highlight some of the effects of this chronic and disabling condition. Izzy is played by actress Cherylee Housten who also suffers from EDS who, when joining the show, sparked interest and hope from the EDS community that her character would have the condition involved in some of her storylines. However, so far, there has been no mention of 'Ehlers Danlos Syndrome', only 'a degenerative disease' and a few small mentions of being able to stand and walk 'which makes people think I'm faking being in a wheelchair'.

We know that EDS is rare and there are many more interesting conditions out there to boost ratings but all the EDS community ask is that if writers insist on using the condition in their storyline that they stop using it as a basis for freaky and offensive storylines that are only adding to the misunderstanding and potentially damaging attitude amongst Drs. Instead show EDS sufferers the respect and admiration they deserve living with a painful, life changing and sometimes fatal condition.

April Showers and Springtime Sun

We are a quarter of the way through 2011 and for people with EDS a lot can happen in 3 months.

Last time I posted things weren’t great and I was in abit of limbo-land waiting to see new drs and move on with new areas of my condition. Since then there have been some new issues to deal with as well as increased hope in other areas.

My GI issues have continued to be a focal point in my dr’s concerns but thankfully haven’t changed a huge amount since the beginning of the year.
I had tried a new drug that is being used in women with chronic dysmotility of the GI system but had to stop taking it after one month due to complications with it. It seems my body always finds a way to dash the hopes of me and my drs when we hear of a new wonder-drug that we think will be the answer.
My GI dr at my local hospital has had to admit defeat and can no longer treat me due to his lack of knowledge of EDS and the eventual failure of medication based treatment being an option.
I have now been referred to a specialist further afield who has a particular interest in severe dysmotility and unusual GI conditions. His treatment ideas are on the ‘radical’ side involving pacemakers for the stomach and other surgeries that wouldn’t normally be used for the problem he is treating but things have got to the point of having to try anything that might help.
My stomach is struggling to digest food properly and absorb nutrients so I am starting to experience symptoms of malnutrition mainly a lot of hair loss and sudden energy slumps. I am hoping that we can find a way to get things working again quickly before I have to start buying a lot of hats!

I am very lucky to have been referred to an orthopaedic surgeon who knows and understands EDS and what that means in terms of what we can realistically acheive. I was referred for my recurring hip subluxations/dislocations and increased pain in my hips and pelvis. At my first appointment he was very honest admitting that EDS can only be helped so much with surgery and that he would not be able to get me walking again. Finding out that eventually I will be unable to stand due to the effect the EDS is having on my knees and back was a shock but knowing this now means I can finally let go of the hope that I will one day wake up miraculously healed and be running and dancing down the high-street.

We are focussing on surgery that will make my hips much more stable in order to reduce the pain and make sitting in my wheelchair much more comfortable for long periods.

I am also halfway down the very long road of POTS investigations. POTS (postural orthostatic tachycardia syndrome) was first suspected in November following a fall I had. I am lucky that 3 out of the 5 drs I see regularly suspect I have POTS now as this has meant bypassing some of the unnecessary testing and loop-holes needed to get a referral to a cardiologist for the all-important Tilt Table Test which is the primary tool for diagnosis POTS. I still hope and pray that I don’t have POTS and the symptoms I have are down to a medication or drinking too much tea but it’s becoming harder for me to not prepare for a POTS diagnosis.

My tachycardia (fast heart rate) and low blood pressure is becoming more and more frequent and I now have to try to manage other possible POTS symptoms which only aggravate my EDS. Fatigue and ironically insomnia are worse than ever. I seem to only manage an hour or two out of bed at a time without ending up exhausted and feeling like I have ran the London Marathon. Nausea and dizziness are constant and never go away now and I have developed headaches that at times are very painful. Hopefully I will get a diagnosis (or more preferred a “You don’t have POTS”) soon and treatment that can get things back under control.

I am also now just waiting for the appointment letter for my powerchair (electric wheelchair) assessment which will bring a huge amount of freedom and opportunity to my life again.

Although I’m spending more time living life from my ever-loving bed, I am still hopeful as ever that if a cure isn’t found during my lifetime that at least my Drs can work together to find a combination of treatments for the different EDS problems that will give me back some of my life. While I wait I continue to be positive using my time to help raise awareness of EDS and support others who are just finding their feet in the overwhelming world that is Ehlers Danlos Syndrome.

How to have a good GP appointment

For most people a visit to the gp is a quick 5-10min job to have a sore throat or upset stomach checked out.
For people with Ehlers Danlos and other multi-system conditions it’s a mammoth task that needs preparation and the ability to keep it together under pressure better than a politician on a pre-election live TV debate!

Quite often you don’t just have one thing you need to see the gp about, there’s a list. You have a new pain or symptom that needs attention, you need a referral to a new specialist or refferred back to a previous one because an old problem has reamerged. You need one of your medications looked at because its interacting with another and causing problems. You need your ribs checking because you dislocated them badly sneezing, oh and that reminds you, can you please have the flu jab you made the appointment for in the first place!

Its no surprise that your gp is stressed and flustered by the end and you are almost in tears.
You are half way home and realise you forgot to ask for a months supply of pain medication and your gp has forgotten you wanted referring to another specialist so you have to chase him up about it in a few months time when you realise the referral hasn’t been done.

GP appointments don’t have to be like this. Your gp is your front line guy, the one in charge of your medical care and the guy you HAVE to have on your side right from the start.
Your gp and you should be a team working together not at loggerheads fighting and dreading every meeting.
Quite often I hear about EDSers having gps who started out ok but then they start having problems and eventually they have to move on and find another:

“He used to be really patient with me and very understanding. He would really listen to my concerns and always refer me to the specialists I needed. Now he rolls his eyes when I walk in his office and I’m having a hard time getting him to re-evaluate my pain medication”

I myself am having similar problems. My gp was very patient with me and never questioned my requests for referrals or treatments. The past few appointments however have had me in tears and him not making any changes to my medications. Iv even been told I can’t be referred to an essential specialist for my GI issues and have to stay within my local PCT when he said previously he would refer me to the specialist once my rheumatologist gave him the nod.

I understand where I have gone wrong though and caused my own problems. I’ve learnt through my own mistakes what needs to be done to ensure the best chance of having a good gp appointment.
I have now found a great female dr in the same practice which works even better for me as I can talk about the more sensitive female issues of the condition and be physically examined easily rather than the previous dr going on what symptoms I am describing alone.

So how do you give yourself more chance of a good appointment?

Here are a few tips…

1) Prepare, Prepare, Prepare.!! It’s almost like what teachers would say to you getting ready for a big exam. ‘The more you prepare and revise the easy the exam is. And the same is true for dr appointments.
If you just turn up to the appointment wanting to try new pain medication but don’t have any ideas which ones your gp has to work out which ones might be suitable for your condition, and that’s when he’s figured out if its for muscular pain, neurological pain, joint pain etc. If you have gone to EDS forums or asked other EDSers what pain meds are popular for EDS, work well and have little side effects you can suggest these to your gp who then has just a couple to choose from rather than hundreds for you to try.
Another example…you are having a lot of stomach problems and you know that this is related to EDS and you need reffereing to a gastroentologist to get it checked out. You just turn up to the appointment and say ‘I need reffereing to a GI dr’. Your gp has no idea if any of the GI specialists in the local area have even heard of EDS never mind know how to treat the GI issues. And that’s if your gp even understands this side of EDS. If you look into this yourself before the appointment you can find out what GI issues can occur in EDS, why they do, what causes them and what type of GI dr you need(functional GI, Neuro GI, etc) what tests you require if you gp can refer you straight for these (colonoscopy, slow transit, bariem xray etc).

2) Book yourself a double appointment if you have more than one (or 2 simple) issues to discuss. A single appointment is usually between 5mins and at most 7mins. Giving you both at least 10mins gives you more time so you can discuss things in full and not be rushed.

3) Write a list of the things you want to discuss. You can either hand this to your gp at the appointment or refer to it yourself. You can write notes next to the items on the list if you need to such as side effects of the medication you want reviewin, hospital details if you are asking for a referral, brief history of what happened and symptoms if you are wanting a injury looked at.
At the end of the appointment you can go down the list quickly to make sure you have covered everything.

4) Take a notebook and pen to write things down. When you have a lot of things to ask your dr he will have a lot of things to say in return. Its most likely by the time you get home you may have forgotten the correct dosage for your new medication or the name of the condition he mentioned they wanted you tested for because of your new symptoms. You can also ask your gp to write down names of over the counter medicatoin they recommend or website links etc.

5)Always take some information leaflets on EDS/HMS You can hand them one and say ‘I just wanted to give you this in case you wanted to look into anything specific on EDS or want to pass some support info on to someone else’
It’s a sneaky way of making sure they have information on your condition in case they know less than they let on. Support organisations tend to provide much better information on EDS than medical journals in terms of living with the condition so it will also provide your gp with information should they be asked to provide evidence for your disability benefit assessments.

6)If you don’t already have it, get Dr Brad Tinkle’s book ‘Joint Hypermobility Handbook’ A guide for the issues & management of Ehlers Danlos Syndrome hyper mobility type and the hypermobilility syndrome’ It covers pretty much every part of the body affected by EDS and is laid out simply and straight to the point. I take this book to all my appointments so that if I need to I can give my dr the relevant page/paragraph to quickly skim over. It also provides web links for further reading.

7)Don’t be afraid to challenge your dr. but don’t over challenge! If they are dismissing your concerns or putting an issue down to something else eg loss of appetite or digestive system problems down to depression or weight concerns, don’t confirm their lack of understanding by submitting to them.
Stand your ground if you believe your EDS is causing issues and needs looking into.
Researching any new issues before your appointment and being prepared will really help you here, especially if you can provide some printouts from the internet or a relevant page from Tinkle’s book.

‘Im sorry but I know this isn’t depression. EDS does cause problems with appetite and eating. Here in Dr Tinkle’s book its says about early satiety’.

If they still don’t take it seriously and still put it down to something other than EDS don’t get into an argument. You need a good relationship with your dr. Drs don’t often like it when the patient knows more than them so you have to handle it correctly. Something like..

‘OK,I know this isn’t depression. It is EDS causing this. But I don’t want to ruin our good relationship we have built up over it. Can we come to an agreement that if things haven’t improved in 2 weeks/a month we look at it again?’

You might face the issue of a stubborn dr like many of us have at times but if you are politely assertive rather than angry and argumentative you have much better chance of winning.
I’v had 2 appointments recently with different drs where I’v had to really stand my ground and fight. 1 eventually gave in after 20mins of telling me I was wrong, my body wasn’t doing what I was saying it was and that my medication hadn’t stopped working because of interaction from another. He was so assertive that he had ME convinced I was wrong! But I stood my ground calmly without it turning into a argument or battle of wills. I just kept saying ‘but how come I am experiencing this symptom if my body is supposedly not doing that?’, ‘can you explain again how my medication can still be working if I am experiencing this?’ etc.I asked them genuinely not ‘well explain this to me then clever clogs!’
Eventually he couldn’t explain it and had to quickly research my medication and discovered I was actually right (by this point I was convinced he was right and I was wrong!).

The other appointment I had where I had to challenge a dr was when my gp refused to believe my loss of appetite was related to my EDS. He believed it was to do with depression and being worried about weight. He just kept saying ‘Why are you telling me you are worried you can’t eat, just eat. Its not hard, put something in your mouth and swallow it’. If you are depressed you loose your appetite but you just have to force yourself, you are going to make yourself ill if you don’t eat’.
I didn’t win that one because I ended up in tears.
Keep your cool, keep collected but be prepared to loose some battles. You just have to go back and try again another time.

DO NOT PUT YOURSELF AT RISK HARM

If you believe something is seriously wrong and needs urgent attention and your gp is not taking it seriously see if you can get an appointment with another gp at the surgery. Otherwise you can go to a NHS walk-in clinic and ask to be seen by the on duty dr or go to A&E department.

8) Be honest with your gp. If you don’t want to be referred for physic say so. If you are unhappy with how your last appointment went, tell him. Your gp will appreciate your honesty.

9) Always be prepared for the appointment not to go how you expect. What will you say if your gp says he won’t refer you to the specialist you want to see? What are the other options of medication if he says you can’t have the one you wanted because it interacts with others you are on?

10)Drs are people too and have bad days like the rest of us. Sometimes that means you can be in the firing line and this can result in a disastrous appointment through no fault of your own.

For example my last appointment with my gp went something like this…

I enter the room, he’s sat at his desk not waiting at his door like usual. He’s red in the face and not smiling like normal. it’s a few days after I was admitted to hospital overnight after a faint and concerns from an on-duty Consultant of possible POTS(postural orthostatic tachycardia syndrome) resulting from my EDS.
I have my discharge letter which asks my gp to do regular monitoring of my heart rate and blood pressure and recommends a referral to a vascular specialist if I have another fall for Tilt Table testing (standard POTS test). It also says Iv been referred for an endoscopy due to concerns for my loss of appetite and swallowing difficulties. The on-duty dr tells me to see my gp as soon as I can to discuss his recommendations and ask again for a referral to the GI specialist who specialises in EDS.

My gp asks what I want. I tell him I was admitted to hospital after a fall and Iv come with the discharge note to discuss the recommendations. He has a quick read and says ‘I don’t understand, what do you want me to do?’

I tell him that the dr thinks I may have POTS and wants my blood pressure and heart rate monitored regularly and a referral to the vascular specialist If I faint again.

He replies ‘whats POTS, Iv never heard of that, is that just fainting or something? I’m not going to do your bp and heart rate, what do I need to do that for you fainted cos you’re not eating. Just stop being silly and eat. Of course you will pass out if you don’t eat.’
I am just staring at him in shock as this is totally out of character.

He continues ‘Are you worried about your weight is that why you’re not eating?

Im crying at this point so he shoves a box of tissues towards me and rolls his eyes.

That was one of the worst appointments Iv had and it was with a gp who I’v had nearly 3 years of brilliant support from. It just goes to show that drs can have really awful days too and unfortunately we sometimes get caught up in the firing line.

You have to just accept it was a bad appointment, cut your losses and go home.
Be polite as you leave. Don’t get angry or react to his bad attitude. Thank him for his time, smile and hold it together until you are out of his door.
Try not to let it affect you and especially don’t give up any of the concerns or requests you had.

Simply make another appointment at a later date if you feel you just caught him on a bad day or think about trying a different gp at the same surgery if you can.
Sometimes we can only go so far with a dr before we have to move on.

GP appointments for people with EDS can be quite an ordeal, they can be like doing a round of ‘Weakest Link’ with Anne Robinson or they can be like popping round a friends house for a cuppa and catch-up.
Whatever type of appointments you have remember your gp is crucial to your medical care so preparing well for your appointment and working as a team, not against them is how to have a good appointment.

cx

GI Joe!...the unrelentless gastric EDS soldier

Gastroenterology is a word that many with EDS become fluent in rolling off their tongues. Secondary to joint instability, the digestive system can be the most problematic system in the EDS body for some.
It is now becoming more accepted amongst the sceptic medics that EDS, or Hypermobility Syndrome is more than just 'being bendy' because of this increased awareness of the severity of GI complications in EDS patients.

The GI system, or digestive tract, is one long tube made up of lots of rings of muscle. You put food in your mouth, chew then swallow. The food enters this tube of muscular rings where it is squeezed down the tube by the muscles contracting in turn. It is kind of like when you are trying to squeeze the last blob of toothpaste out of a tube.
In an EDS affected digestive tract the muscles loose their tightness as the collagen breaks down. When they do squeeze they don't do so enough to push the food along. The food then is then simple passed through the system too slowly. In extreme cases the system may not be able to move, or function enough to tolerate solid foods.

When food reaches the stomach it can take an EDS stomach longer to do its job or it can not work as well by thinking it is already full. This causes mixed signals so that the person thinks they are full or is unable to eat decent amounts of food. It can also take the stomach longer to empty. The top of the stomach might not close properly making the contents come back up into the oesophagus or the stomach (reflus) or in extreme cases, fails completely and is unable to digest solid food at all. In this case it is sometimes necessary for the person to have a PEG or GI tube fitted so they can be fed directly into their stomach.

When the food finally leaves the stomach it enters the small and large intestine (or bowel). Here it is passed along using the same muscular method. This time squeezing the food along while digesting absorbs nutrients and water and turns the food into stools (faeces) ready to be eliminated from the body.
In someone with EDS that affects this part of the system, the muscle weakness can cause slow transit, constipation, ibs, cramps, pain, and in severe cases the bowel can become unable to move the waste along at all. Finally the rectum and anal muscles can be affected which can cause incontinence or inability to expel stools.
In the severe end of the lower GI symptoms the results can lead to the person requiring irrigation treatment (inserting catheters into the rectum to fill the bowel with water to flush the lower GI system. When the top part of the lower GI system becomes affected the irrigation is sometimes done through a tube that is surgically inserted through the upper adomen wall). If irrigation fails then the bowel is removed and a stoma (colostomy/illiostomy) is placed. This is in rare circumstances when the bowel no longer functions at all.
Usually if a person is affected badly in their lower GI system they may require very large doses of regular laxatives.

Other GI issues not caused by muscle weakness in EDS can include fool allergies an intolerances, hiatus hernia (where the stomach comes up into the chest cavity), pelvic organ prolapse (where parts of the pelvic organs including the bowel collapse into the pelvic floor), rectum prolapse (part of the bowel falls out of the body through the anus), severe and chronic acid reflux, constant nausea and vomiting.

The majority of people with EDS who have GI problems will only have the milder end of symptoms that can be managed with diet or medication. However it is becoming more recognised now that the GI problems that were once thought to be unrelated are caused by EDS or the Autonomic Dysfunction that EDS patients also tend to have.

I have always have lower GI problems since birth. I suffered from slow transit and a number of impacted bowels as a child.
I have been using irrigation treatment for over a year now which is no longer really effective. My bowel has started to collapse in areas which causes immense pain and blockages that make the irrigation even less effective.

I was very lucky to meet a neurogastoentologist who has taken an interest in EDS patients. He believed at the time that the upper abdomen tube to irrigate through could be an option before bowel removal however it has taken nearly 6 months to convince my GP to refer me to him. My lower GI problems are much worse now but I hope that this is still an option by the time I finally get to his clinic.

I also have upper GI problems now. Mainly I have no appetite now. When I do force myself to eat I can only manage very small amounts and the food takes its time getting down into my stomach. I sometimes have a hard time swallowing and can feel and hear 'clunking' in my oesophagus when I have to gently squeeze and rub my neck to encourage the food down.

I had an endoscopy last week to check for anything none-EDS. I am very scared of my upper GI system failing in the way my lower system hasso I was hoping they would find something non-EDS that is easy to treat. However all they found was some inflammation in my stomach which could be caused by a bacteria they also found. Neither of which would cause my symptoms.
It looks likely that my upper GI symptoms are EDS, particually muscle weakness.

I have a couple of friends with EDS who have serious GI problems. One has the hypermobile form of the condition like me, the other has the vascular. Both have feeding tubes and have had their bowel removed.
I have lots of other eds friends who have GI problems but none have got to needing irrigation treatment or it failing like me.
It does worry me that I may be coming close to the end of the line with options in terms of what can be done for my GI problems.

This issue of my blog was intended to raise awareness of just SOME of the GI issues that can happen with EDS. I hope that it has helped others understand just how serious this part of the condition can get and encourage those with EDS who are experiencing GI problems to look into this area and ensure that your Dr understands that your GI issues could be due to your EDS and should not be ignored.