Tag Archives: Postural Orthostatic Tachycardia Syndrome

Awareness, chronic illness, chronic pain, Ehlers Danlos Syndrome, health, Rare Diseases, spoonies

To the Doctors That Failed Me and to the Ones That Saved Me

August 23, 2018 Leave a comment

Dear Doctors,

I came to you as a scared, vulnerable teenager. I was in pain and tired all the damn time.

“You’re depressed”, you said.

I walked out with a script for antidepressants. I hoped this would fix everything.

No such luck. I am a zombie. I didn’t care about anything. My friends are scared for me.

Months later I return with the same complaint. My joints hurt. I’m still tired. A new symptom arose. Chest pain.

“You’re stressed”, you said.

“I don’t feel stressed”, I responded.

“Subconscious stress,” you said.

I walk out with another script for a different antidepressant and sleeping pills.

This goes on for years.

It’s all in my head, you said. That the “physical symptoms were a manifestation of something psychological”.

Those words made me genuinely depressed. I wasn’t being listened to. The weight piled on, intensifying my depression. I didn’t feel like this body was mine.

You didn’t listen.

I became pregnant and things escalated.

“It’s just the pregnancy”, you said.

I moved clinics to find a doctor who would really listen to me.

Once again, I explain the pain, the fatigue, the stomach issues, the dizziness, my heart racing and pounding in my chest.

I came to you as a scared, vulnerable mother. I was in pain and tired all the damn time.

“You’re depressed”, you said.

I walk out with yet another script for another antidepressant. The fifth medication of its type that I’ve tried.

I am in too much pain and too tired to function. I can’t muster the energy to get dressed. My friends and family don’t understand. Being judged for being in my pajamas all day. I am just lazy.

I think:

“If this is all in my head, and nothing is working, what is the point in living?”

There were days I looked at my pills and thought that if I took them all, that the pain would end, that I would be free and I would no longer be a burden on my family.

I fight the urge and win.

Three years later, while just about managing an internship, I interview a woman with Ehlers Danlos Syndrome. Her story sticks with me. The doctors didn’t believe her either. Nor were they willing to help.

A year later I speak to this woman again. We get to talking about my issues.

She asks one thing that changed my life forever.

“Are you hypermobile?”, she asks.

“No”, I say.

“I’m stiff as a board!”, I exclaim.

Turns out I am. Very hypermobile. I score 8/9 on the Beighton scale.

The woman gives me the same of a doctor to see. I decided to see what you, my doctors thought.

“Could it be Ehlers Danlos Syndrome?”, I ask.

“Oh God, no! That’s as rare as hen’s teeth,” you say.

I leave, defeated.

One November day, I faint at home. That’s it. I need answers.

I go to see the recommended doctor. My stomach is in knots. That’s normal before I go and see doctors of any type now. I prepare myself to be told the same old thing. That I was depressed, I was anxious, that it was all in my head.

I am examined, I am spoken to with respect, I do not feel like a neurotic child.

“No wonder you have been depressed. Nobody was listening to you”, he says.

Just as I thought; I was diagnosed with Ehlers Danlos Syndrome.

For the first time in my life, a doctor really listened, like no other doctors had done before.

Tears stream down my freckled cheeks with relief.

I walk out, cigarette in my hand, shaking with relief and with disbelief.

Finally! I was listened to. Finally, I have my answers.

A letter is sent to you, confirming my suspicions.

Nothing changes though, I suspect you don’t believe the diagnosis.

I never took another antidepressant after that day.

It was all over, or so I thought.

Then I soon realised, the diagnosis meant nothing without someone to help.

And here I am, six years later. I am bouncing from consultant to consultant. Medicated up to my eyeballs. No real investigations are done and some tests are simply not available in my own country.

To England, I go with family in tow. My two children now facing the same life of disbelief and pain.

But things are different here.

I meet you, dear doctor, for the first time.

You are small and sweet. You’re gentle and kind.

I feel safe with you. You are thorough.

I had no choice but to travel and spend money we did not have, but I am glad I did.

You really listened and didn’t brush me or my feelings aside.

So many from my country have been here before. Desperate for help.

A world expert’s diagnosis, that will shut the rest of them up.

And it did. No more questioning on whether this was the real diagnosis.

I had wondered myself if the diagnosis was correct because all the others, these “experts” made me feel that way.

I return home. The rain pours as we land. It matches my mood.

I am coming back to a country that does not care about my well-being or my children’s.

I jump to another doctor after another again. And again, questions the diagnosis. I am so sick of doctors at this point. But, this next one is different.

You really seem to care.

There is no rush, you have taken the time to listen.

You follow my lead, you let me take the reins on my treatment.

I almost jumped for joy leaving the doctor’s office. I could not believe my luck.

You seemed determined to help.

My pain worsens. I am a ball of tears every time I see you now because I am at the end of my tether. Other doctors want to try these expensive treatments that may not work. They haven’t worked before. I just want relief from the pain.

I just wanted you to tell me you’ll help.

Yet I walk out with a script for antidepressants-again.

The memories flood back, of not being believed. I am now a nervous wreck going to any doctor again.

I take the first pill. It begins again.

I can’t get out of bed, I am more tired than ever.

Zombified again.

Sleeping for three days after taking one of those tiny pills so I don’t take anymore.

I return to you, scared because the pain is as bad as it’s ever been.

Feeling like the tin man, I just need some oil to move with ease.

“Anti-inflammatories will help”, you say.

I take the script, skeptical.

It’s still early days but I just know this isn’t going to work.

Something is very wrong with me.

I can’t keep fighting for help, doctor. I am already too tired.

Is it so much to ask to just want to be normal? To be like my friends.

There is only so much I can do on my own.

I am trying, really, I am to be normal.

Distracting myself with things that I can do.

I just want help with the things I can’t manage alone.

Is not living in pain too much to ask for?

Sincerely,

Someone who just needs help.

chronic illness, chronic pain, dysautonomia, Ehlers Danlos Syndrome, health, Rare Diseases

Ridiculous things that injure people with Ehlers Danlos Syndrome

July 18, 2017 thezebramom 1 Comment

Hey there, hi there, ho there!

Havin’ a bit of fun today with this tongue in cheek article. I was just thinking about all the things that can potentially hurt me-that wouldn’t hurt a “normal person.” The list was pretty funny so I thought I’d share them with you. I try and see the funny side of my illness. Nothing like a bit of comic relief!

Breathing

Breathing is vital to our survival. However, many people with Ehlers Danlos Syndrome suffer from a condition called Costrochronditis. It kind of feels like your having a heart attack and can make breathing very painful.

Eating

Again vital to our survival and yet, many of us have serious trouble with eating. Eating the wrong thing can cause pretty horrible side effects such as Tachycardia. If you suffer from Mast Cell Activation Disorder (MCAD), something you have been previously been able to eat all of a sudden causes an allergic reaction. Then there’s Gut/Bowel Dysmotility and Gastroparesis…

Sleeping

Many people with EDS suffer from Chronic Fatigue. We will spend weeks sleeping like we are hibernating for the long night. Then there are weeks where we suffer with insomnia. Our sleep patterns tend to be all over the place and we tend to be night owls in general.

insomnia GIF

Drinking

I’m not talking specifically about alcohol here. No, even drinking a glass of water can cause issues. We do have difficulty swallowing so sometimes we will either cough and splutter while the water goes down. Then there are times when the water goes down the wrong way or even cause us to bloat so badly that we look pregnant. As for alcohol? Yeah….just no. Tachycardia, MCAD flare, nausea, vomiting, THE APOCALYPSE.

Moving

Moving is like an extreme sport to us. Even on our good days we are afraid to move for fear we might injure ourselves. We are clumsy AF so there’s a pretty solid chance we are going to walk into a wall/table, trip over ourselves or trip over literally nothing.

Exercise

laughing GIF.gif

Working

“God, it must be so great not having to work” *insert eye roll here* We would love to be able to work, some of us can and that’s brilliant for so many of us our working lives are over long before we even get our first grey hair. Being stuck at home is not all it’s cracked up to be, you know?

eyeroll GIF

Sex

Yep. Even sex can be detrimental to us Zebras. Ever dislocated a hip while having sex? Um…no…me neither…

Yawning

Yep, simply yawning can cause pain. Anybody with TMJ Dysfunction will know this.

You: “What was that?!”

Me: Oh just my jaw popping out of place.

You:

shock GIF

Stretching

You’ve finally managed to have a good night sleep, you wake up, have a lovely big stretch and then….POP, POP, POP, POP.

crispies gif.gif

Sneezing

See above.

Laughing

Side effects of laughing include:

Headaches

Dislocations/subluxations

Fatigue

Your funny bone may get tickled but it also might pop out of place.

Bathing

Yes, even bathing cause some pretty nasty symptoms such as our POTS and Hypotension to flare up. Do you really need to wash your hair today? Like, is it a special occasion?

faints giphy

Hobbies

I’m not talking about bungee jumping or horse riding here, people. I’m talking about sitting on your butt, watching TV and KNITTING. Yep, even knitting can cause an injury. Dislocated wrists will put you off knitting for life.

Taking our meds

Never mind the difficulty having to swallow giant pills or multiple pills. Most of us can do that dry after awhile but when the side effects make you feel worse than the symptoms you are actually treating, you know you have a problem.

The weather

Is it hot and humid? Pain in the muscles. Cold? Pain in the joints.

Have anything to add to my list? Let me know below!

Until next time,

Z.M

Don’t forget to follow me on Facebook, Instagram, Twitter and Pinterest!

chronic illness, chronic pain, dysautonomia, Ehlers Danlos Syndrome, health, reviews, spoonies, tips

The Zebra Mom Trials-Oska Pulse

July 16, 2017 thezebramom 2 Comments

I have been given this product as part of a product review through the Chronic Illness Bloggers network. Although the product was a gift, all opinions in this review remain my own and I was in no way influenced by the company.

Hey there, hi there, ho there!

I contacted Oska Wellness a few months back as I had heard about the Oska Pulse and had seen some reviews. I’ll be honest, I was skeptical. But, my curiosity and desperation got the better of me so I made contact with Steve Collins one of the founders of Oska Wellness. He requested a Skype call and what a call it was! I felt as though I was talking to a friend, we spoke about music, Ireland and his family heritage. We spoke about the device and I was carefully hopeful after hearing about some of Steve’s stories. Steve told me “be skeptical, but don’t be surprised if it works. You won’t even realise it’s working, one day you’ll find you’re doing something you haven’t been able to do for a long time”.

So, what is the Oska Pulse?

According to oskawellness.com:

“Oska Pulse mimics the body’s own recovery processes to relieve pain, muscle stiffness and inflammation, using optimized pulsed electromagnetic field technology (PEMF) to encourage recovery at a cellular level.” It is a first grade, FDA approved medical device.

What are Electro Magenetic Fields?

Sounds a bit Star Trek, right? Ok, so I’ll break it down a bit.

So first of all, I think it’s important to know what electromagnetic fields are and how important they are to our survival.

The Earth has it’s own electromagnetic field and without it, we wouldn’t be here. The Earth’s magnetosphere is a shield that protects us from the powerful solar winds given off by our sun. Without it, our atmosphere would be blown away out into space.

We all use this technology on a day to day basis. Our phones, microwaves and computer screens all emit electromagnetic fields.

So how does this technology work as a therapy?

PEMF therapy is applied by running an electrical current through a copper coil which creates an electromagnetic field.

This type of therapy is said to improve circulation. With improved circulation, your cells are in a much better condition allowing your body to heal more effectively.

Electromagnetic field therapy has been in use since the invention of electricity. It was widely adopted in East and Western Europe but its use was restricted to animals in North America until recently. Veterinarians became the first health professionals to use PEMF therapy, usually to heal broken legs in racehorses.

In 2004, a pulsed electromagnetic field system was approved by the FDA as an adjunct to cervical fusion surgery in patients at high risk for non-fusion.

Although electricity’s potential to aid bone healing was reported as early as 1841, it was not until the mid-1950s that scientists seriously studied the subject.

Ok sounds great, right? But if you’re like me, you’ll want some hard core proof this technology works. So I did a bit of reading about PEMF. I read some articles and reviews and it gave me hope but, I was still a bit skeptical about whether it would work for Ehlers Danlos Syndrome. After all, it’s been noted as one of the most painful conditions.

So it arrived after a couple of weeks. I charged it for awhile and used it that evening. My science fanatic husband used an EMF detector on the device and sure enough, the Oska Puse was emitting electromagnetic pulses every few seconds.

A few weeks later my husband was refilling my Pill Drill and he called into me ” Babe?! You know you haven’t been taking your painkillers, right?”

I stopped and thought about it. Lo and behold I realised I was only taking two Tramadol in the morning. I had previously been taking the max dose (400mg per day).

I upped my use of the Oska to see if it would help my Dysautonomia symptoms as Steve had told me a young woman with POTS had noticed an improvement with her symptoms. Once I upped my usage, I did notice a difference with my POTS symptoms.

So how do you use the Oska Pulse?

Please note that if you have a pace maker or internal defibrillator you will not be able to use the Oska Pulse.

The device is about as small as a phone and can easily fit into a pocket or handbag. A strap also comes in the package so you can simply strap it on to you as you get on with your day. I use mine first thing in the morning for an hour. I use it again in the afternoon for about 2 hours and then finally another 2 hours in the evening. You can use it while on the computer working, reading a book or even making the dinner.

Skeptics might say this device is producing a placebo effect but we were both skeptical from the beginning. I went to my pain specialist just last week and told him about the Oska Pulse. He was in absolute agreement that this technology works and he was not surprised at all that the device was working for my chronic pain. This pain specialist has an amazing reputation and is highly regarded in his field here in Ireland so for him to believe in this product and technology, it says a lot. I know this isn’t a placebo effect.

I’m now thinking about possibly working outside of the home, something I haven’t done since 2012. I’m pushing my daughter on the swing and playing hide and seek with the kids. My day to day pain has all but vanished.

Now, of course no tablet or device is going to stop me from dislocating and stop the pain that goes with it. But knowing that the horrible day to day excruciating pain that I normally experience is not going to stop me from living my life, has given me hope. Once I start getting my injections into my hips and spine, I should hopefully be in a position to throw myself into physiotherapy. Getting myself strong should reduce dislocations so between the Oska Pulse, injections and physiotherapy, I will hopefully be able to go back to the old Evie who danced, played tennis, went horse riding and a social life.

The Oska Pulse has put in me in a position to start my recovery. I can start seeing the light between the trees. Not using pain killers has also made me feel more clear headed and the rebound pain I often get from using Tramadol has disappeared. Using Tramadol over a long period of time can have a number of pretty nasty side effects which I started noticing.

If you are like me living with chronic pain that has stopped you living your life, you should definitely try the Oska Pulse. You have nothing to loose. Oska Wellness offers a 90 day money back guarantee so if it doesn’t work for you, you can simply send it back.

Product rating: 5/5

If you would like to try the Oska Pulse and receive a $55 discount click the link here. Or you can use the coupon code 180387 at the checkout! For more information, see the Oska Wellness Facebook page.

Don’t forget to follow me on Facebook, Instagram, Twitter and Pinterest!

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Awareness, chronic illness, chronic pain, dysautonomia, health, Rare Diseases, spoonies

Autonomic Testing: What you need to know

June 19, 2017 thezebramom 3 Comments

Hey there, hi there, ho there!

So a few weeks back I returned to London for some autonomic tests. While I was fairly prepared, there are a few things I would do differently. Even though I had been briefed by the investigations unit, I feel that some people who are due to have these tests would benefit from a few extra tips.

These are the tests that I had:

Autonomic Function Screening tests, plasma Catecholamine blood samples, head up tilt test (Autonomic Protocol), liquid meal test with autonomic responses, modified exercise test with autonomic responses to gravitational changes, cardiovascular autonomic responses to arm movements and a 24hr blood pressure monitoring using the autonomic protocol & analysis.

How do you prep for these tests?

Food and water

You have to refrain from any food four hours prior to testing and you can not drink anything until the lead up to the tests. So, I would recommend buying a breathe freshener spray as my mouth gets really stinky when I don’t drink water for awhile. So if that happens to you, you might want to bring some with you.

Luckily, my appointment was at 11am so I did have time to get up and eat something before we left. For 48 hours prior to testing you must refrain from eating anything with caffeine (coffee, tea, coca cola etc) bananas, chocolate, cocoa, citrus fruits & vanilla.

Clothing

You should wear loose fitting clothes for testing as the hospital informs, they suggested shorts in the documentation but London in spring is cold! I brought a few different things to wear and knew what would work for the second day of tests. What worked best was a loose t shirt (make sure the arms are loose or short too) and leggings. They only attach the vast array of wires and monitors on your upper body. The lowest they go is your hip area.

Medications

I wasn’t sure whether I should take my meds or not and it didn’t say what to do on the documentation so I refrained from taking my Midon but took my pain killers as the mornings are the worst time for me, pain wise. Luckily, I had booked a wheelchair to travel around London in so there was no chance that if I did faint, that I’d crack my head on the pavement. If you’re unsure yourself, give your investigation team a call or send an email to ask what to do in regards to your medications. It didn’t dawn on me until that morning on whether I should’ve taken my meds or not.

Punctuality

If you’re travelling abroad or far from where you live, make sure that you know which train/bus etc you need to get or what route you need to take if driving. I would advise for anyone having these tests in London, to check online whether there are any delays on the tube line you’re taking. I have a tube map app that was very useful for our trips around London. Give yourself plenty of time to travel. Be sure to get to the hospital in plenty of time so you can fill out any paperwork. Because we had the two kids with us, we did get delayed a bit so I was late for my tests but what we didn’t have time to do on the first day we did on the second.

How do these tests work?

Autonomic Function screening tests

This comprises of six tests. They are performed whilst you are lying flat, which will assess cardiovascular autonomic function.

The exercises vary in length, ranging from 15 seconds for the shortest and 3 minutes for the longest. The exercises examine blood pressure and heart changes in response to various stimuli: breathing exercises, cold, problem solving and isometric exercise. There

is a rest period between each test to ensure that your blood pressure and heart rate returns to baseline before commencing the next test. The autonomic function screening test also contains an active stand test. You will be asked to stand for a maximum of 5 minutes, whilst measuring your blood pressure and heart rate.

So, the breathing exercises were very straight forward. You are asked to breathe in deeply and then breathe out slowly for 15 seconds into a small tube. Then you’re asked to breathe quickly like you are hyperventilating.

The cold test was not pleasant at all. You’re asked to place your hand on an ice pack for 1.5 minutes. It doesn’t sound very long but trust me, it feels like forever. I won’t lie, it does hurt but as soon as that 1.5 minutes is up, you take your hand off and feel instant relief.

The problem solving test was tricky for me as I have a Dyspraxia diagnosis and people with this condition tend to perform very poorly when it comes to mathematics.

You’re asked to do fairly basic maths of adding and subtracting but when we got to the big numbers, I ran into trouble. Plus the pressure of having to perform and brain fog thrown in, the odds were stacked against me but, they’re not measuring your math skills or intelligence, they want to see how your autonomic system reacts to these particular exercises.

Finally we did the isometric exercise test and this was simply having to squeeze a ball. With my weak hands and the wrists that are susceptible to dislocation, it was difficult for me but I was assured I did just fine by the lovely woman, Kiran who looked after me so well.

Plasma Catecholamine blood samples

Blood samples were taken from my arm using a small butterfly needle. Catecholamines / Metanephrines are a group of hormones (adrenaline, noradrenaline and dopamine) which circulate in the blood and help regulate blood pressure and heart rate. The levels of catecholamines/ Metanephrines in the blood changes with posture (the levels increase from lying to standing). Blood samples were taken while I was lying, following the 10 minute rest period, and while I was on the tilt table in the standing position.

On some occasions, a spot catecholamine/ Metanephrines blood sample is taken during testing. This usually occurs if the clinical autonomic scientist observes a sudden marked increase in blood pressure and / or heart rate which correlates to certain symptoms. This test is performed to determine whether there are any sudden increases in the catecholamine levels, which could explain the symptoms.

Tilt table test

A tilt table test is a non-invasive diagnostic test to determine whether your symptoms: dizziness, light headedness or loss of consciousness; are due to changes in the blood pressure and / or heart rate. At the beginning of the test, you will be asked to lie flat on a table. Two straps are put around your body to hold you in

place. After about 10 – 15 minutes of lying flat, the table is then slowly tilted to raise your body to a head-up position – simulating a change in position from lying down to standing up. The table will then remain upright for

either a maximum of 9 or 45 minutes (maximum time depends on the test requested by the doctor), while your heart rate and blood pressure are monitored continuously. This allows doctors to evaluate your body’s cardiovascular response to the change in position.

Liquid meal test

This is a procedure that’s used to examine the effects of food on blood pressure (BP), heart rate (HR) and / or symptoms.

A liquid meal is used as the effects on BP and HR can be observed after a 45 minute period. Solid foods can take a lot longer to digest (up to 7 hours). There are two options for the liquid meal:

1) complan (original flavour) + glucose + milk

2) glucose + water

Most patients will have option 1 during the test. However, I had the glucose solution as I am lactose intolerant. It is so sweet, like sickly sweet. I would love my sweet treats but this was just gross. Both meal options are suitable for vegetarians and are gluten free.

So at during this test I was asked to lie flat on the examination couch for 10 minutes to get a baseline of my BP and HR. Then I had a tilt table test. Then I had to rest again until I was asked to drink the liquid meal. Then came by favourite bit, resting for 45 minutes. It was pure bliss, no noise, no lights, nobody crawling on me or calling for me every two minutes. It was every mother’s dream. I’m pretty sure I dosed off for awhile too.

During the 45 minute rest period, my BP and HR will be monitored continuously. During the 45 minutes however, my heart rate (HR) dropped very low. My usual HR is 80-90 BPM but mine dropped to 50 for awhile. The on call doctor was called to check my stats but no intervention was needed as my HR came up by itself. After 45 minutes, I had another tilt test to see if there have been any changes to BP and / or HR following the meal.

Modified exercise test

I was asked to lie flat on an examination couch for 10 minutes or until a baseline of

my BP and HR was established. I then stood for a about 2-3 minutes. I was then again asked to rest prior to performing the exercise. For the exercise, I cycled on a bike whilst lying flat. It was tough but I needed to cycle at a continuous set speed, with the

resistance of the bike gradually increased over a set period of time. I performed the exercise as long as I could which was just 3 minutes. My knee and hips were giving out.

Following the exercise, I had yet another period of rest before standing again for 5

minutes. I couldn’t last that long either. My BP plummeted making me feel dizzy.

Cardiovascular autonomic responses to arm movements

So I basically had to hold my arms out in front of me and then above my head for a few minutes. I think I only lasted a minute. My body has become so deconditioned that it physically hurt to hold my arms out in front of me. I could feel myself getting tachycardic during the tests.

24hr blood pressure monitoring using the autonomic protocol & analysis.

I had to wear a small portable blood pressure monitor, which is on a belt and a blood pressure cuff, which was fitted to my upper left arm. The monitor will takes your blood pressure and heart rate every 20 minutes during the day and every hour during the night.

You are advised to continue with your normal activities, which will help to show your investigations team a typical profile of your blood pressure and heart rate during a 24 hour period. The monitor can be removed for a bath or shower during this period. I was also given a diary. The diary contains a list of extra activities that my team wanted me

to perform whilst wearing the monitor (these activities including measuring your blood pressure & heart rate responses to mild exercise, food and postural changes). I couldn’t do all of these activities as we were either out of the hotel, I was tending to the children or having a bath.

During the monitoring, you can eat and drink as normal prior to your appointment. Your doctor will advise you whether you need to stop any medication prior to and during testing.

After the testing, I did feel exhausted and a bit sick. Booking my wheelchair was the smartest thing I did in preparation for my trip.

A couple of weeks later Prof Mathias spoke with me on the phone (this consult does cost extra on top of the couple of thousand pounds I paid for the tests) and I received a diagnosis of Postural Orthostatic Tachycardia Syndrome, Autonomic Mediated Syncope and my Orthostatic Intolerance/Hypotension was confirmed.

If you have any questions, you can comment below or message me on my Facebook page: The Zebra Mom

So until next week,

Z.M

Awareness, chronic illness, chronic pain, dysautonomia, Ehlers Danlos Syndrome, family, Friday Feelings, Guest Posts, health, Rare Diseases, spoonies

Friday Feelings with Pain Pals Blog

May 19, 2017 thezebramom 2 Comments

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.

“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 nursing and I trained and worked in various London hospitals, and then worked as a palliative care nurse in a local hospice – a fantastic, rewarding job that I loved. I was medically retired 9 years ago when my back gave up on me. Now I am kept busy with the family (more needy as young adults than they were as toddlers).

I am a school governor at a local primary school, and I am about to become Chair of my old school’s friends’ committee. We keep old pupils in touch as well as arranging careers events for current pupils. I love meeting new people and interacting with the pupils at both schools. I love reading and belong to several book clubs, posting reviews on the blog, and when I can get there I love the cinema and streamed theatre live events.”

So now that we know a little about Claire, let’s have a read of her Friday Feelings entry.

“Dear Diary,

It’s Friday, many people will go out tonight for a few sociable drinks with their friends. On a typical Friday night I will usually be found curled up in my PJs! How much of the evening meal I help with really depends upon what sort of day I have had and how fatigued I am. These days my hubby, Duncan is based at home with me, and in theory the kids can take over the kitchen duties but, it is usually like Piccadilly Circus as they come and go, leaving us to keep pace with who is eating or not! Some weeks I will try to join in with Spoonie Speak – a chat set up on Twitter for those of us who can’t go out, but like a chat in our PJs. Like lots of spoonies Netflix, Amazon & TV play a big part in my life but, this week I am actually going out on Friday for the leaving dinner of the current Chair of the friends committee.

I really want to go but do get a bit anxious about making arrangements in case I have a bad day, can’t go and let others down. Not to mention missing out.

My physical health has really deteriorated recently and since having a spinal cord stimulator implanted for the chronic pain, my POTS symptoms have really gone mad. I feel frustrated, tired and worn down by the constant pain, fainting, dislocations, drug side effects, losing my independence, the uncertainty of how I will be from hour to hour and struggling to make plans. Something I really struggle with is the fatigue and the subsequent guilt at needing to rest. On a beautiful day I can’t stand not being to jump up and just go for a walk in the park.

Some days I feel like I am constantly saying sorry for needing help to have a shower, for fainting, for being unable to cook, for being pushed in the wheelchair, even for not being able to lift the iron.

I try not to think too hard about the future for me – on bad days I can’t think about living with the pain, particularly the chronic back and leg pain, for another week let alone another 40 years! Sounds dramatic but that is how it feels. I also know that there is very little that can be done for the extreme joint pain – I have been told I need shoulder and hip replacements, but that it isn’t possible because of the dislocations and I’m still too young.

I don’t want to “give in” to the illness any more than I have to. Sometimes this is easier said than done – and at times the future feels uncertain.

But I do tend to do what I can when I can and probably don’t pace. I want to keep as active as I can for as long as I can, but the severity of POTS symptoms this last 6 months has left me feeling really low and really ill. Hopefully the different consultants can sort me enough to get me back on an even keel – but the lack of “shared” care is frustrating; repeating the same story over and over, no one knowing what other specialities are doing!

What does worry me for the future is the kids – the younger two anyway. The 18 year old has bad and frequent migraines, with neck pain, hand and arm pain. The youngest is now subluxating and dislocating, has daily pain in joints, can’t hold a pen in a normal grip, writes slowly and has pain. All 3 are dizzy on standing and lose vision – and all three are overly anxious. Two have had counselling, one self harms, one is on anti depressants, we are well acquainted with CAMHS!

I do accept that hardly anyone will have heard of EDS and have got used to suggestions of having collagen injections but what is tougher is the lack of understanding about what chronic illness means. No, I won’t get better. It is genetic so no there is no cure, not even for the kids! I feel that I have to justify myself for being tired, cancelling plans, etc. I hate having to ask for help and would love it if friends would just remember that I can no longer drive and offer a lift without me having to ask. Some people can’t understand why I use a wheelchair when I can stand and walk – pain is invisible, as are the implants in my back, and damage to my joints, but these are what making standing up and walking a near impossibility some days. I feel that other people’s lives are so busy and that I should be fitting in with them, after all I’m at home all day.

It is frustrating when I’m told how well I look and this happens when I’ve put on some lippie and blusher.

You don’t see me at my worst because I can’t actually get out of the house then! But I would probably rather people think I’m putting best foot forward and being positive – chronic illness doesn’t stop you wanting to look good, go out, have a laugh; even if you can’t always actually do this.”

A big thank you to Claire for taking part in Friday Feelings and being so open with us. Can you relate to Claire? Do you feel guilty not being able to perform certain tasks? Let us know in the comments below!

Want to write your own Friday Feeling entry?

Send

A high res photo

A short paragraph about yourself

What illnesses you have

Your diary entry with the following topics in it:

It’s Friday, many people will go out tonight for a few sociable drinks with their friends. What do you do on a typical Friday night?
How are you feeling at this moment about your chronic illness?
How do you feel about the future in regards to your illness?
How do you feel about the way people view your illness?

and links to your blog and social media to evienevin87@yahoo.ie

Be sure to put “Friday Feelings” in the subject bar.

Until Sunday,

Z.M

Awareness, chronic illness, chronic pain, disabiliy, dysautonomia, Ehlers Danlos Syndrome, family, health, Rare Diseases

The Zebra Mom in the News

April 8, 2017 thezebramom 4 Comments

Hey there, hi there, ho there!

So this week’s blog is coming a little early because we are flying off to London tomorrow. This week has been very tough on me because my legs have become so weak that I can not stand up without help. I am on day two of a migraine and nausea too. So driving 3 hours up to Dublin and flying to the UK is going to be very hard on me. This will my third trip in a year going over to London for treatment and testing.

This trip I am having autonomic testing and physiotherapy. The children are also going to see their physio on this trip.

The last couple of weeks have been mental though. We have been on three newspapers, another on the way and I have been on the radio too talking about the lack of care in Ireland for patients with EDS. I am also lobbying for medical cannabis to be granted for those with chronic pain. I found out just yesterday that one of the newspaper articles below was read out in the Dail (Irish Government building) during a presentation about medical cannabis. Apparently my story of just wanting to be better for my children moved a lot of people. Medical cannabis could make that a reality.

Article from The Southern Star by Kieran O’Mahony

‘I’m left helpless to ease their pain’ says Clon dad in bid to get UK treatment

A CLONAKILTY man who says it pains him to see his wife and two young children suffer from a cureless condition, has set up a funding page to help them access treatment abroad.

Martin Nevin set up a GoFundMe page for his wife Evie (30) and their two children Alexander (7) and Olivia (2) who suffer from Hypermobile Ehlers Danloe Syndrome (EDS) and Dysautonomia.

The chronic, multi-systemic conditions have left his wife and children susceptible to joint dislocations and chronic pain and fatigue, as well as many other issues which can severely affect their standard of living.

In a poignant post on the page, Martin outlined why he set it up.

‘It pains me to see these three fantastic people suffer, to watch a woman with so much talent restricted from sharing it with the world, to see a boy with the will to climb a mountain withdraw from fatigue and to see the sweetest and the happiest little girl ever cry with agony due to dislocated joints.’

‘I can do nothing but watch their symptoms flare up. I’m left helpless to ease them and that is why I’ve set this page up so my family can get the treatment they need,’ said Martin.

Although the condition is slowly being recognised here, there are no specialists or facilities in the country to help sufferers, leaving the Nevins with no choice but to seek treatment abroad.

‘The Ehlers Danlos Syndromes are a group of connective tissue disorders which cause the body to produce faulty collagen and collagen is the glue that holds our bodies together. We are also affected by Dysautonomia, which means out autonomic systems don’t work properly. While Alexander and Olivia are not very affected by this condition, it does affect me and it can cause me to faint by simply cooking dinner,’ Evie told The Southern Star.

Evie and Martin have been told by the HSE that there are no plans to help patients with EDS in Ireland, so they are looking to London for treatment. ‘It’s a little too late for me now, as my body was neglected for almost 30 years, but the children are lucky to have been diagnosed so early,’ explained Evie. ‘So with help in the UK, we hope that they won’t become as affected as me.’

Evie said she is almost house-bound seven days a week, and she has been unable to work for several years due to her condition.

‘We want to give our children the best possible chance at a normal life and it’s really only now that I am getting tests and treatments for myself.

‘While we look to getting treatment abroad, we’ve been told that the Treatment Abroad Scheme won’t cover the costs to the UK, because our doctors are private consultants and the Cross Border Directive won’t cover our tests because they need to also be available in Ireland, which they are not.’

Evie and Martin also received another big blow with the recent announcement that chronic pain won’t fall under the Medicinal Cannabis Bill.

‘Medicinal Cannabis is often recommended for EDS patients in the US because patients are often resistant to pain medications, particularly opiate-based pain killers,’ added Evie.

Evie herself has been working tirelessly to get this condition recognised in Ireland and has written extensively on this for many newspapers and journals, as well as spearheading a campaign for all EDS sufferers.

The family recently attended the Hospital of St John and St Elizabeth in London where they met with professors who specialise in EDS and Autonomic Dysfunctions.

‘We have had to break up the trips over to London which is more expensive, so every time we go back they add on other tests and consultancies, so we are going to be fundraising for the foreseeable future.’

The family have set up a Go Fund Me page to raise the vital funds to avail of the treatment in London and they and their friends have already been fundraising by holding events in Clonakilty.

See www.gofundme.com/2befu24c or see ‘The Nevin Family Treatment Fund’ on Facebook.

Interview on C103’s Today show

Play from 56:20 to hear my interview below

https://soundcloud.com/cork103/corktoday-4th-april-2017

Article on The Irish Examiner by Sarah Slater

Cork family pleads for help to battle rare illness

A young mother and her two children are battling an illness that is consuming all of their short lives.

Evie Nevin, aged 30, her son Alexander, 7, and daughter Olivia, 2, have the rare and debilitating condition, hypermobile Ehlers-Danlos syndrome (EDS).

Although EDS is slowly becoming more widely known in Ireland, there are no specialists or facilities in the country to help people with this condition.

There is no cure for EDS, but with careful management and specialised physiotherapy the quality of living for someone with EDS can be improved.

Most Irish EDS sufferers are referred to Rodney Grahame, consultant rheumatologist at the Hospital of St John and St Elizabeth, London, who has said: “No other disease in the history of modern medicine has been neglected in such a way as EDS.”

Members of the EDS community have campaigned for specialist treatment here, to no avail. They recently learned that there are no plans by the Government or health services to implement any.

Evie, from Clonakilty, Co Cork, is prone to hip and wrist dislocations on almost a weekly basis. She is extremely effected by changes to pressure and can be left bed-ridden with pain from something as small as a rain shower.

I went from getting up at 6.30am and getting home at 9pm working as a journalist in 2012 to being someone just existing in 2013,” she explains.

“I remember being very self-conscious all the time because I rarely had the energy to get dressed and people only really saw me in my pyjamas. I got called lazy a lot. That hurt. I didn’t know what was wrong with me, but I knew it wasn’t laziness.

“When Alex was diagnosed my heart broke. I felt so guilty. My genes did this to him but, thankfully, I’ve moved past that now because what’s the point in feeling guilty? He’s lucky that he has been diagnosed so young and that he has an excellent school who support him by providing resource hours, an SNA and movement breaks.

“It was confirmed for me before Olivia was born. I felt her skin and saw the hint of blue in the whites of her eyes when she was born.

“My heart goes out to Martin. He didn’t ask for this at all. When we first met I was still relatively ‘normal’.”

Evie’s husband Martin, 29, has thrown himself into fundraising to get his family the specialist treatment they need, as well as being a videographer. He’s also determined to keep their spirits up.

“I feel like such a burden to him, but he never makes me feel like that,” says Evie. “Even on days when I’m in agony and not so pleasant in my manner, he doesn’t complain. He has never once made me feel bad for being sick or for the kids inheriting my genes. It’s all down to me and my issues.

“He took his ‘in sickness and in health’ vow very seriously! He’s a brilliant dad and husband and treats us when he can so we have things to look forward to. When you’re stuck at home six days out of the week, most weeks, looking at the same four walls, you need something to keep you looking forward.”

For now, the focus is on fundraising, but the Nevins are facing the fact that they may have to emigrate to properly deal with the condition. They have been told they cannot access either the Treatment Abroad Scheme (TAS) or the Cross-Border Directive (CBD).

“The TAS only covers treatments available on the NHS,” says Evie.

“The Hypermobility Unit in St John’s and St Elizabeth’s in London is a private hospital so our consultancies and treatments won’t be covered.

“The CBD will only cover medical issues that are also available here in Ireland and the tests and scans I must have in London are not available in Ireland.”

In a statement, the HSE said: “EDS is treated across Europe by multidisciplinary teams, often led by physiotherapists, with extreme cases being sent to orthopaedic for corrective surgery. EDS is readily treated in Ireland by physiotherapists. There is no specialist centre in Europe or the UK.”

Evie refutes this: “There is no treatment available here, no matter what they say. The people in charge will say rehab in Harold’s Cross is an option, but they are in no way experts and the reviews from my community haven’t filled me with any confidence that they could take care of me and my complex conditions.

“Right now, we are aiming to move abroad somewhere where there is better care and where the air pressures are more stable than Ireland. The kids are becoming affected by the pressure changes too. Both of them wake in pain when we have particularly nasty weather.

“Obviously I can’t blame the Government for the weather, but I do blame them for not setting up a clinic for people with connective tissue disorders.

“We need somewhere with a multi-disciplinary team like in London. We need consultants that communicate with each other. Things slip through the cracks because of the lack of communication and a holistic approach.”

One development that could make a huge difference to the family’s lives is medicinal cannabis. Those with EDS are often resistant to pain medications and they find it difficult to manage their pain. Medical cannabis is recommended for EDS patients in the US.

There is an increasing call by medical support organisations for greater access to medicinal cannabis for patients with debilitating conditions.

A Health Products Regulatory Authority report published last month advised that, if a policy decision is taken to permit cannabis under an access programme, it should be for the treatment of patients with three conditions — spasticity associated with multiple sclerosis; intractable nausea and vomiting associated with chemotherapy; and severe, treatment-resistant epilepsy that has failed to respond to standard anticonvulsant medications.

“When health minister Simon Harris announced that only three conditions would be approved, none of which are related to chronic pain, I was devastated,” says Evie. “I had been hopeful and excited at the idea of having some normality back in my life.

“Medical cannabis could be seriously life-changing for my family and me. In the US, EDS experts highly recommend cannabis as a form of pain relief as many patients are resistant to painkillers, specifically opiate-based pain medications.

“I have changed pain medications so many times because I’ve become resistant pretty quickly to them. This is now starting to happen with my current medication, Tramadol.

“I’d rather have medical cannabis over my Tramadol because taking 5,000 painkillers a year can have serious repercussions on my body. If I can access cannabis safely and with help from the HSE, I may not develop symptoms of long-term opiate use such as depression, hormonal dysfunction, and respiratory depression, to name just a few.

“I have used cannabis-based products to manage my symptoms. I use a MediPen, which has been helpful for my chronic fatigue, but not for my pain. I have smoked cannabis and I find that, on days when my Tramadol isn’t working, it’s the only thing that helps manage the pain.

“I also find it good for my appetite because I don’t eat a lot when I’m in a lot of pain. I’m so nauseas and I find Tramadol suppresses my appetite sometimes. There are days I’d be lucky to get 800 calories into me. I would like to be able to access cannabis legally so that I can function a bit better in my day-to-day life and be a better mother and wife to my family.”

For now, another round of tests and physiotherapy for Evie, Alexander, and Olivia at St John’s and St Elizabeth hospital in London, as well as flights, accommodation, and private medical consultations in Ireland, mean the young family need to raise in the region of €10,000 for the first trip.

Martin says: “It pains me to see my family suffer. I can do nothing but watch when their symptoms flare up. I’m left helpless.

“So I’m asking the public for their help, as a father and as a husband, to send my family to get the treatment they need. Anything you can spare, even a few euro, will make a massive difference.”

You can find out more on their GoFundMe page, or on Facebook.

The symptoms

Ehlers-Danlos syndrome is a genetic connective tissue disorder that affects the joints, skin, and blood vessel walls.

Sufferers usually have overly flexible joints and stretchy, fragile skin. This can become a problem if a wound requires stitches.

There are 13 types of The Ehlers Danlos Syndromes and symptoms vary.

Overly flexible joints can result in dislocations and early-onset arthritis. Fragile skin may develop prominent scarring. Those with the vascular disorder are at risk of often-fatal ruptures of major blood vessels. Some organs, such as the uterus and intestines, may also rupture. Pregnancy can increase these risks.

In other forms of the disorder, fainting and low blood pressure occurs because blood vessels stretch. Stomach wall lining is fragile and bowel ruptures can occur. Constipation and other motility (movement) disorders feature, as does gastroparesis (paralysis of stomach; inability to process food).

Excessively loose joints are the hallmark of hypermobility disorder as suffered by Evie, Alexander, and Olivia. Large joints and small joints are affected. Partial and total joint dislocations are common. Many experience chronic limb and joint pain.

ENDS

It has been a crazy couple of weeks and thanks to these journalists, we have reached our first goal of 10,000 Euro! When the Irish Examiner piece came out over 2,000 was raised in that day alone. I am truly blown away by stranger’s generosity and kindness.

Another good thing that came out of all of this is that I have had parents from all over the country ring me to ask for advice regarding their children. Sadly, and also not surprisingly, many families are given diagnosis but then offered no support or solutions. In Ireland we have to fight for every support we get. This is no country for disabled people. The entire health system is lacking in every sector.

Standing up and speaking out does work. You just got to keep chipping away at the block. I truly believe my story has made a difference in regards to awareness and lobbying for medicinal cannabis.

Next on our press list is an article with the Evening Echo. We hope that some more newspapers, radio stations and TV stations get in touch. Not so that we can fundraise, but to raise awareness and give chronic pain patients a voice.

Until next week,

Z.M

Awareness, chronic illness, chronic pain, dysautonomia, Ehlers Danlos Syndrome, Guest Posts, spoonies

My Guide to Dysautonomia featured on Irish Dysautonomia Awareness

March 12, 2017 thezebramom 3 Comments

Hey, there, hi there, ho there!

This week I guest blogged on Irish Dysautonomia Awareness with my Guide to Dysautonomia. Go check it out!

I’m putting together an e-book of all my guides of conditions relating to invisible illnesses. Want you illness included? Comment below!

Until friday,

Z.M

x

Irish Dysautonomia Awareness

I’m Evie and I come from Cork, Ireland. I’m a 29-year-old mother of two baby zebras. Alex is 7 and Olivia is almost 2. I am diagnosed with Hypermobile Ehlers Danlos Syndrome (hEDS), Orthostatic Intolerance and Vasovagal Syncope. I first heard of EDS after interviewing a young woman with EDS for the paper I used to work for. Something about this woman’s story stirred something inside me and I became passionate about raising awareness of the condition. A year later I was diagnosed with EDS. When I’m not blogging, looking after my two children or lying in bed ill, I help my husband run our wedding videography business and co-host a radio show on Saturday evenings from 7pm (Irish time) on www.clonlineradio.com.

evie blog

I write about Ehlers Danlos Syndrome an awful lot and with where I am guest posting today, I decided to focus on Dysautonomia. I recently wrote A Simple…

View original post 1,780 more words

chronic illness, Friday Feelings, Guest Posts, health, spoonies, tips

Friday Feelings with Living With POTS

March 3, 2017 thezebramom Leave a comment

Hey there, hi there, ho there!

This week I spoke to Caroline from Living with PoTS . Caroline suffers from Postural Orthostatic Tachycardia Syndrome (PoTS). PoTS is a debilitating type of Dysautonomia and is often found in people with Ehlers Danlos Syndrome. You can find Caroline on Twitter.

I’m Caroline, I’m a final year Uni student in Yorkshire, studying Psychology and Education. I love reading, especially Harry Potter, baking and crafting, and my dream is to live abroad and work with disadvantaged children.
I’m relatively new to both the chronic illness and blogging communities, having suffered with PoTS like symptoms all my life, but only just figuring out what it is. I’ve been amazed by how supportive everyone is.

So now that we know a little about Caroline, let’s look at her Friday Feelings entry.

“Dear diary,

Friday night again, well 4pm. I’m in bed, exhausted from the week. Tonight is worse than most, Friday is food shop day, and dragging a week’s worth of stuff back on the bus means the pain is worse than usual. Later my flatmates will be going out, I’ll be lying in bed trying to sleep, constantly being woken up by them. Eventually I’ll probably give up and turn my laptop on. If it’s been a bad week, I’ll stick a DVD on, if it’s been a better week I might try get through some of the mountain of work I’ve been avoiding.

Right now I’m still getting used to my chronic illness. I’ve been suffering for years, but always just assumed I was lazy, or hadn’t eaten enough, or a million and one other excuses. Although having a reason is a relief, it’s scary. It’s here to stay and I need to learn to live with it. I’m sure one day I’ll know where I stand with it, I’ll have a diagnosis and a symptom management plan, and I’ll feel more in control, but that day is not yet.

The future is overwhelming. In July I’ll graduate, and have to enter the real world. I’ve always known what I wanted to do, I never even considered that I might be too ill to do it. So right now it’s back to the drawing board, desperately searching for a job I can do, without having to move away from the doctors I’ve only just met, who will be supportive of my illness. My degree has 3 contact hours a week, so I have no idea how I’ll cope with work, I don’t even know how I’ll cope making tea tonight, or having a shower. Long term planning is nothing short of impossible.

I wish people would stop telling me to be positive. I am positive. I’m also realistic. When I say this illness isn’t going away, it’s not. That’s a chronic condition. When I say it limits what I can do, it does. That doesn’t mean I’m not ambitious. I will find a job and a life that I love, it just won’t be what I was planning, but that’s okay. My condition is part of me, it does not define me.”

A big thank you to Caroline for reaching out to take part in our Friday Feelings blog.

Do you relate to Caroline’s entry? Do you constantly hear that you should be more positive from those around you? How do you deal with it? Comment below to offer some advice to this new Spoonie.

Want to write your own Friday Feeling entry?

Send

A high res photo

A short paragraph about yourself

What illnesses you have

Your diary entry with the following topics in it:

and links to your blog and social media to evienevin87@yahoo.ie

Be sure to put “Friday Feelings” in the subject bar.

So until Sunday (I know, I know I said I’d have it up during the week),

Z.M

Awareness, children, chronic illness, Ehlers Danlos Syndrome, family, health, Rare Diseases, spoonies, Uncategorized

A Simple Guide to The Ehlers Danlos Syndromes

December 8, 2016 thezebramom 14 Comments

UPDATE: On March 15 2017, criteria and classifications of The Ehlers Danlos Syndromes were updated for the first time in 20 years. In light of this, I will update my guide (with the new information made available) to highlight new diagnostic criteria and classifications. You can read more about the changes here.

Because there are now 13 types of EDS, I have only covered Hypermobile Ehlers Danlos Syndrome (hEDS), Vascular Ehlers Danlos Syndrome (vEDS) and Classical Ehlers Danlos Syndrome (cEDS). If you would like me to do another guide to the rarer types, please comment below or email me. I would be more than happy to oblige!

“You’re suffering from Fibromyalgia!” “You’re depressed!” “You’re imagining it!”

“You’re malingering!” “You’re attention seeking!-”

“No I’m not – I have an Ehlers Danlos Syndrome!”

The Ehlers Danlos Syndromes (EDS) are a group of conditions that are poorly understood, even by many in the medical professions. It is essentially a defect in the production of collagen, an essential component of connective tissue.

Many articles about EDS contain medical terminology that can be difficult to understand. The purpose of this guide is to put the medical terminology in plain language and help non-affected family and friends understand exactly how EDS affects people and their day-to-day lives. The medical terminology is included in italics. Links to web pages are included throughout the article if you want to conduct your own research.

Why are they called The Ehlers Danlos Syndromes (EDS)?

The name of the condition itself is quite a mouthful! Ehlers Danlos Syndrome (Eylerz-Dan loss Sin-drome) is named after the two physicians, Dr Ehlers and Dr Danlos, who first described this group of connective tissue disorders.

What is EDS?

People with a type of EDS will produce faulty collagen. Collagen is essential for healthy connective tissue, which is found throughout the body supporting and connecting the different types of tissues and organs, including tendons, ligaments, blood vessels, internal organs, bones, the blood and skin.

Imagine a healthy person’s connective tissue as being like regular household glue. People with EDS have collagen that is more like chewing gum; stretchy and not very good at keeping things in place.

What causes EDS?

There are a number of different genes responsible for making collagen and connective tissue, so there are different types of EDS depending on which genes are faulty. There are 13 types of The Ehlers Danlos Syndromes

How did I get a faulty gene?

It is possible that the faulty gene may have been inherited from one parent, or both parents, or not inherited at all. It may be that the defect has occurred in that person for the first time. This happens in 25% of cases.

How I explained it to my 7-year old son.

A carpenter makes a wooden chair. Instead of using wood glue to place the joints of the chair together, he uses chewing gum. Once finished, the chair looks fine. But, as time goes by and the chair is used, the chewing gum doesn’t work very well at keeping the joints together. Without proper glue the chair can begin to get wobbly. I went on to explain that with proper exercise he could help to strengthen his muscles so that they acted like binding around the joints to help support them.

What does EDS feel like?

Having an EDS feels different from person to person, depending on their type, but many describe it as having a lifelong flu. Have you ever had the flu? Do you remember how painful it was having those aches and pains in the joints and muscles? Do you remember how tired and run down you felt? That’s what it’s like for people with EDS only worse and it never goes away. In addition to the daily aches and pains people with EDS also have to deal with very painful headaches, gut issues and then of course there’s the issue of dislocation. Many EDSers can’t go a day without a joint popping out. It can happen simply by stepping off a footpath or picking up a pot when cooking. A lot of people with EDS are also affected by the weather. When it is damp or when the air pressure changes their pain can increase.

How does EDS affect people?

Because collagen is everywhere in the body, there are hundreds of ways EDS can affect people. Any two people with EDS may have very different signs and symptoms, this includes people with the same type. In som,e the condition is quite mild. For others it can be disabling. Some of the rare severe types can be life-threatening.

One of the problems with diagnosing EDS is that many diseases share the same symptoms. As a result, EDS can be easily confused with other conditions and it may be difficult for doctors to recognise. But there are ways to tell if someone may be affected by EDS and need more thorough investigation. Some of the investigations available are listed later.

The most common symptoms of EDS (hEDS and cEDS) are:

“Double jointed” – Hypermobility: joints that are more flexible than normal.
Loose, unstable joints that dislocate easily.
Clicking joints.
Joint and muscle pain

In addition there may be

Fatigue (extreme tiredness).
Injuring easily.
Fragile skin that bruises and tears easily. The skin may also be stretchy.
Digestive problems
Dizziness and an increased heart rate after standing up. (Postural Orthostatic Tachycardia Syndrome or simply POTS for short)
Incontinence of urine in women

Digestion.

If food in the stomach doesn’t move through the body to make its way out it may just sits in the intestines and can cause a feeling of fullness, nausea, vomiting, stomach pain, to name just a few symptoms. This condition is known as Gastroparesis. (gas-tro par-eesis).

Nervous System

Another condition than often affects people with EDS is a fault with that part of the nervous system controlling the “automatic” functions of the body; things like blood pressure, breathing, heartbeat, digestion, how hot or cold you feel and the way your organs work and so on. This is called the Autonomic Nervous System. When it doesn’t operate as it should the conditions is called Dysautonomia (Dis-auto-no-me-a). Common symptoms of this are trouble with digestion, dizziness and fainting.

Dysautonomia affecting the heart.

The most common type of Dysautonomia causes dizziness and an increased heart rate after standing up. This condition is called Postural Orthostatic Tachycardia Syndrome or simply, POTS for short.

Some sufferers have fairly mild symptoms and can continue with normal work, school, social and recreational activities. For others, symptoms may be so severe that normal life things like bathing, housework, eating, sitting upright, walking or standing can be very difficult. They may feel dizzy or even faint from doing these things.

What are the symptoms for POTS?

People with POTS experience fatigue (extreme tiredness), headaches, lightheadedness (feeling dizzy), heart palpitations (when their heart beats so hard you can hear and feel it), exercise intolerance (feel ill when exercising), nausea (feeling sick), diminished concentration (hard to concentrate), tremulousness (shaking), syncope (fainting), coldness or pain in the arms, legs, fingers and toes, chest pain and shortness of breath. People with POTS can develop a reddish purple colour in the legs when standing; this is believed to be caused by blood falling down in the body because of weak veins. The colour change subsides upon returning to sitting or lying position.

Can you tell someone has EDS just by looking at them?

The short answer is no. Some may have typically blue sclera (whites of the eyes), they may have translucent skin (see through) and you may even notice how bendy they are. But some people may have some of these things and not have EDS.

Many people with the type of EDS that affects blood vessels (Vascular Ehlers Danlos Syndrome or simply, vEDS) do have some facial characteristics. Notice in the picture below that the people have big eyes, thin nose and lips.

Can EDS kill people?

Some people think it can’t but actually, EDS has led to the untimely death of people all over the world. vEDS is considered the most serious form of EDS due to the possibility of the heart or organs tearing.

Many EDSers live a life of constant pain. This pain and misunderstanding from their medical teams, families and friends can make a person feel very sad and alone which can lead to depression and even suicide.

What treatments are available for people with EDS?

Because EDS is considered “rare” there are not many doctors willing to learn about it. Types such as hEDS and cEDS can be somewhat managed through specialised physiotherapy. Joints with weak connective tissue are more likely to dislocate. Exercises to strengthen the muscles around a joint can help stabilize the joint. Your physical therapist might also recommend specific braces to help prevent joint dislocations. Occupational therapy is also useful to help manage everyday life. Pain relief is very important for people with EDS.

EDSers should also be under the care of a Rheumatologist (a doctor who looks after bones and joints), a Cardiologist (heart doctor). There may also be a need for more specialised doctors such as Neurologists (doctors who look after the nervous system) or all of the above plus many, many more. Sometimes operations are required to repair joints that have dislocated frequently and haven’t healed properly.

Do all people with EDS need wheelchairs?

Not everyone will experience EDS the same way, some people can live normal lives and manage very well with physiotherapy and pain relief. Others may need to use wheelchairs or walking sticks to help them get around. Some people with EDS also have Gastroparesis which we discussed earlier and may need to be fed using a tube. Others may only have mild tummy problems. Some people with EDS may have to go to hospital a lot while some may only go to their GP every few months. But, just because one person can live their lives fairly normally, it doesn’t mean they don’t have EDS or that their pain shouldn’t be taken seriously.

Can you catch EDS, POTS or Gastroparesis?

No. EDS and other sub conditions are not contagious. If you know somebody with EDS, don’t be afraid, you’re not going to catch anything from them. So, if you’re avoiding someone with EDS, go make friends with them.

How can I help someone with EDS?

Be there to listen if they want to talk about it. Some people are afraid to tell you how they feel because they think friends and family don’t want to hear them complain. Ask them how they are and if you can do anything to help them. Doing shopping or household chores can be a huge help and it would be most appreciated. If you’re friend or family member has EDS and can’t access appropriate treatment like here in Ireland, write to your local representatives to tell them about EDS and the lack of care that is available. Help raise awareness in the public by sharing articles or pictures about EDS. Experts believe that EDS is not rare, just rarely diagnosed.

I will update the Diagnostic Criteria for cEDS, hEDS and vEDS in the coming days.

*Special thanks to my Dad who helped me edit this guide.*

Do you think anything else about EDS needs to be explained? Let me know in the comments!

Z.M

Awareness, chronic illness, Ehlers Danlos Syndrome, family, health, Rare Diseases, spoonies

Our medical trip to London. Part 1

September 28, 2016 thezebramom 2 Comments

So, I’m sitting on a plane at Heathrow airport. As I stare out the window and listen to the rumblings of the engines preparing to take us back home, I reflect on the last few days.

I have been running on adrenaline, will power and strong cups of coffee to let my family enjoy the experience of everything London has to offer. I know they wouldn’t have gone sight seeing if they knew just how unwell I was. I can’t hide it now though. My pelvis has separated, which it does every few days or with exertion. My wrist popped out and is now painfully bruised. I am emotionally and physically drained.

We arrived in London on Tuesday evening. Weary after our drive from Cork to Dublin, I was looking forward to getting to our hotel in the Premier Inn Earl’s Court and hopping into the bath for a soak. Ollie Pops N’Clicks had other plans..

In addition to inheriting all my wonderful genetic gifts, she also inherited my inability to travel without some form of sickness cropping up. Yup. Right there on the packed tube, close to me in the sling she vomited. And I mean vomited. Like ‘Team America’ vomited. It just kept coming! How could someone so little bring up that much puke?

The smell. Christ. Just what I needed. I look over at my husband only to see him laughing. Then everybody else in the tube noticed what happened and began to laugh too. Frickin’ hilarious, lads.

We hopped off the tube so I could clean myself up as much as possible. We eventually made it to the hotel in one piece, just. Ravenous, we dropped off our bags, got washed up and went to the restaurant for a pleasant dinner.

I didn’t sleep so well that night. The next afternoon Bendy Boy and I would be meeting the Professor Grahame. I met him once at a conference in Cork. He was just as sweet and gentle as I had remembered. The Professor knows all too well the struggle Irish zebras face, almost total abandonment from our own Government and healthcare system. No specialists and the majority of tests needed are simply unavailable. We don’t even have an upright MRI machine.

After an examination and a very long chat, the Professor confirmed Bendy Boy’s diagnosis of EDS Hypermobility Type. It was also noted that the six year old shows signs of Postural Orthostatic Tachycardia Syndrome. When Bendy Boy stands up, his heart rate rises and his feet pool with blood. I was shocked to learn of the POTS signs as he has never complained of feeling dizzy except when he gets out of the bath. I was surprised I didn’t notice the pooling.

The Professor seemed impressed with my knowledge and understanding of medical terminology. When there are no experts available to you, you have to become your own expert.

Here is an excerpt from my own medical report:

“On examination there is evidence of widespread joint laxity with a hypermobility score of 8/9 on the hypermobility scale. Outside the scale her shoulders and hips (borderline) are also hypermobile, as are her feet which flatten and pronate on weight bearing. There is a non-significant 2° scoliosis on the Bunnell scoliometer, but no other features of a marfanoid habitus. Her skin is soft and silky and semitransparent, and shows increased stretchiness in the phase of taking up slack. There are numerous thin scars from knee scrapes acquired in childhood and similar over her elbows. Striae atrophicae were first noted by her at the age of 18, and she has minimal striae gravidarum despite having had two full-term pregnancies, a pointer to EDS. Gorlin sign, ability to touch the nose with the tip of the tongue is positive, and the lingual frenulum is rudimentary, both pointers to EDS. She scored very highly (25/30) on our checklist of symptoms compatible with autonomic dysfunction, known to be a common feature of EDS. Her blood pressure lying was 124/84, pulse rate 66; standing 124/84, pulse rate 80. This rise of 14bpm on change of posture is suggestive of postural tachycardia syndrome (PoTS), the most frequently encountered form of dysautonomia seen in patients with EDS. The evident pooling of blood in her toes on standing is further evidence in favour of PoTS.

On the basis of the clinical findings I have formed the conclusion that Yvonne is suffering from a heritable disorder of connective tissue, the Ehlers-Danlos syndrome hypermobility type, a diagnosis that was established by Dr Mulcahy in 2013. I explained the nature of the condition to her, in particular its genetic basis and the vulnerability it confers on soft tissues to the effects of injury and overuse. In her case it has resulted in longstanding widespread joint and spinal pain. Since the time of her first pregnancy she has suffered a secondary chronic pain syndrome, a frequent occurrence in this situation. It is likely that her bowel symptoms represent an EDS-related intestinal dysmotility, and almost certainly she has PoTS.

There is a concern about the possibility that she might have craniocervical instability on the basis of left-sided weakness, headaches, and paraesthesia in her arms and legs. In addition she feels that her head feels too heavy for her neck. With this array of suggestive symptoms I have agreed that we should proceed to an upright MRI examination, and I will be requesting this at the Medserena Upright MRI Unit for her to have one on a future visit.”

And Bendy Boy’s report:

On examination there is evidence of widespread joint laxity with a hypermobility score of 8/9 on the hypermobility scale. Outside the scale his shoulders, cervical spine, hips, fingers and big toes are all hypermobile, as are his feet which flatten and pronate on weight bearing. There is a non-significant 3° scoliosis on the Bunnell scoliometer. Other features of a marfanoid habitus include a pectus excavatum, and hand-height and foot-height ratios both elevated to within the marfanoid range. I interpret these findings as indicating an incomplete marfanoid habitus, which may become more obvious as he completes his adolescent growth spurt. This should not be taken to imply that I feel he has the Marfan syndrome as the habitus is widely distributed throughout the family of heritable disorders of connective tissue. His skin is characteristically soft, silky and semitransparent, and shows increased stretchiness in the phase of taking up slack. There are no paper-thin scars of note. Gorlin sign, ability to touch the nose with the tip of the tongue, is negative. The lingual frenulum is present (normal). He scored moderately highly (12/30) on our checklist of symptoms compatible with autonomic dysfunction, known to be a common feature of EDS. His blood pressure lying was 96/53, pulse rate 75; standing 102/62, pulse rate 85. This rise of 10bpm on change of posture is suggestive of postural tachycardia syndrome (PoTS), the most frequently encountered form of dysautonomia seen in patients with EDS.

On the basis of the clinical findings I confirm that Alexander shares his mother’s phenotype and diagnosis.”

While I was being examined, Ollie Pop (16 months) decided to stand up on her own for the first time! And I missed it. Thank You, EDS!

Receiving the confirmation of EDS HT and the noted symptoms of POTS given by Professor Grahame will hopefully bear weight in accessing services here in Ireland. Although, I won’t hold my breath. My GP was happy to hear that I took the plunge going to the UK and she’s very interested in my case. It took a long time to find a GP that genuinely cares. While a weight has been lifted knowing that I definitely have EDS and haven’t been misdiagnosed for the hundredth time, there is a fear. The idea of having cervical instability or Chiari freaks the sugar out of me. This last trip cost roughly 5,000 Euro. The next trip will be double that again. If Chiari is present and significant it may mean I will have to take a trip to the US to have surgery. We will just have to wait and see.

Coming home to Ireland, it is wet and windy. It’s miserable. The weather here reflects how I feel about Ireland and it’s healthcare system.

Now that I’m home I don’t have access to the fantastic services and more importantly the compassion I felt in London. I felt so at ease.

I am fundraising to get back to the UK in the new year for further testing. I will give details of these in Part 2 along with the rest of my tale. If you can donate anything at all, just click on the link below. Even sharing our story would be a massive help.

https://www.gofundme.com/2befu24c

So, until next time,

ZM.

Have anything to add to my list? Let me know below!

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Don’t forget to follow me on Facebook, Instagram, Twitter and Pinterest!

Want to write your own Friday Feeling entry?

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Interview on C103’s Today show

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Want to write your own Friday Feeling entry?

I will update the Diagnostic Criteria for cEDS, hEDS and vEDS in the coming days.

One mother's experience of life with Ehlers Danlos Syndrome