The newest charity for Ehlers-Danlos Syndrome

Annabelle Griffin is a bright and happy five year old. She likes playing, and music and being a princess. Unlike most five year olds however, she is the driving force behind a newly registered UK charity. Annabelle, or AJ is she as affectionately known, has vascular Ehlers-Danlos syndrome. VEDS is the most serious branch of Ehlers-Danlos, a syndrome that is not widely known of, especially, and worryingly, among the medical community.

AJ is at constant risk of a rupture of one of her internal organs due to a lack of collagen in her body, which could prove fatal. It can happen at any time, with no prior warning for her family. She was diagnosed aged 3. A detailed post on AJ’s condition and VEDS can be read here: http://bend-trend-hms.blogspot.co.uk/2014/02/rare-disease-day-annabelles-story-life.html

It is living with this constant fear and dealing with EDS on a daily basis that led AJ’s family to start a charity- Annabelle’s Challenge. May is Ehlers-Danlos awareness month, and it has seen Annabelle’s Challenge become a registered and official charity in the UK.

Parents Sarah and Jared along with lots of support in their home town, Bury, have already put on countless fundraising events and built up a strong presence on Twitter. Trustees behind the new charity are now calling on local people in Bury and beyond to come forward to help with fundraising and share their skills to help expand their work. Jared says: “As a small charity the trustees will need volunteers to help with fundraising and awareness campaigning and we are very grateful to everyone in the local community who have supported us so far helping us to raise £15,600 over the past year.

It is commonly said among the EDS community that the syndrome, which consists of different types, is not rare, just rarely diagnosed. EDS awareness month has seen a rise in blog posts and online discussion, with people across the globe helping to spread information.

Ehlers-Danlos has a long list of symptoms and complications, and can make life seriously challenging. Jared added: “The worst part of the condition is not knowing when a significant medical event could take place, she could simply be sat watching TV and without warning have a spontaneous rupture of her internal organs which could kill her.”

Earlier this year Annabelle suffered from an accident at home, a trauma injury to her nose required an emergency visit to A&E and a subsequent operation requiring plastic surgery under general anaesthetic. Jared says: “It was a very tense and emotional time for us as we were unsure how the operation would go because her skin is so fragile, the medical team had knowledge of Ehlers-Danlos Syndrome but not Vascular type. Unfortunately not many medical professionals are aware of VEDS – and this prompted our decision to register Annabelle’s Challenge as a charity.”

Annabelle’s Challenge will work with the EDS Diagnostic Service based in London and Sheffield to help provide support to newly diagnosed patients. It will also be raising funds to further the education of VEDS within the medical profession and general public.

You can keep up to date and read lots more about VEDS and AJ’s story here: http://www.annabelleschallenge.org/

EDS awareness month is continuing and by spreading information it can be hoped that people who either are affected directly or indirectly can gain more understanding of this complex condition.

The Ehlers-Danlos syndrome Association website has lots of useful information and links: https://www.ehlers-danlos.org/

Barn dancing, paris and a marriage: One week in hypermobile Hell.

I am lucky enough to sometimes forget I have hypermobility and EDS. That is only sometimes though. Other times it jumps out from whichever rock it has been hiding under and repeatedly slaps me in the face and storms around at night making sure I know it’s there and it means business. One week in the life of someone lacking collagen who’s joints are very grumpy and impossible to please can be a hellish week indeed.

You still have to go to work (if you can) and do everyday things like showering and eating and sleeping, but it is extremely difficult when these things HURT SO MUCH.

I recently had one week full of hypermobile nightmares. The worst I have had in a very long time. To make things more exciting though, the week did involve a wedding, a barn dance and Paris. Sublaxation is not fun. On a scale of one-fun it wouldn’t even make one. I haven’t had an awful lot of experience of subluxation apart from one time when I was walking home and my hip decided to put an end to the walking part. “Hello can you pick me up. I can’t move.”

Last week however, while at a wedding, wearing VERY sensible shoes might I add, my hip started to throb.  I tried to walk it off at first as sometimes unexplained random hip pain does occur, but to no avail. Fortunately the food, wine and speech section soon kicked in which meant excessive sitting and excessive wine. I’m not sure if the excessive wine was an advantage or not, but my hips soon became much less of a focus.

It’s not every day a wedding includes a barn dance, but on this occasion it did. I think barn dancing was the final straw. That is definitely not something I ever imaged playing a factor in my joint woes but hey, it’s actually quite fun (when you have had 1 or 15 drinks) and clearly good exercise, as my hip can attest to.

So my hip sublaxed, I had to limp around a wedding, then get a four hour train home the next day in the company of a hangover and a killer back ache and on Monday I was due to do a retinal screening clinic and stand for the best part of nine hours. I also live in a house with LOTS of stairs, so becoming thirsty or needing food pretty much turned into the Hunger Games. Perfect! Obviously the next day didn’t happen and I had to retreat to my parents to lay in a burning hot bath and watch tv with my dog via a painful physio session and lots of limping. Every cloud.

Of course, because hypermobility waits for no one, this all coincided with me popping across to Paris with a friend for a quick break. Paris involves A LOT of walking. A lot of museums. A lot of steps. My hip behaved for most of it. I only had to limp and complain a couple of times a day, but then after day one my shoulder began to ache and click and crack. I mean why not, it’s not like I was trying to sight see or anything. I clearly rewarded myself for putting up with life with ALL OF THE PASTRY.  But it was still a massive pain, in every sense of the word.

 

I am now in urgent need of loads of physio, my hip is cracking constantly and my shoulder is doing a routine where it kicks in with full on pain every hour or so. Throw starting a new job into the mix and developing massive brain fog, and you pretty much have it all. I definitely forgot just how bad it can be sometimes, and it makes me wonder exactly how people who suffer in this way constantly can face the day. But they do, as I seen on facebook groups and on forums and through chatting to people on Twitter.

Personally, I think someone somewhere needs to start handing out ‘congratulations you live with HMS/EDS’ certificates along with wine and brownies. And maybe puppies?

The above is basically just because this is PRETTY.

The beauty of tea

There are few things in life more comforting than a cuppa. There are few things in life less comforting than painful, stiff joints. So it isn’t it relieving to know that a nice cup of tea can help out with the latter? Since working in an office of dedicated tea lovers I have become a lot more experimental with my choice. I never used to stray from a traditional cup with milk and a sugar (about ten cups a day), but now that has taken a firm back seat. There are SO many flavours of tea, it’s almost overwhelming. I have tried hard to get into green tea for ages because of how many people rave about its health benefits. Once used to the taste, it really isn’t bad at all. You can also go for infused green tea in pretty little boxes. Raspberry is particularly lovely.

For those who suffer from bad joint pain, be it with hypermobility or arthritis, green tea should be considered. There is of course no guarantee that it will instantly wade in and evict pain from sore joints, but there is a large amount written about it that suggests it can lend a hand. As do most things associated with joint pain relief, it is a lot to do with anti-inflammatory properties. Green tea can reduce the production of chemokine in the body, which are proteins that promote inflammation.

Another regular on the joint pain circuit is ginger. Ginger tea has become increasingly popular lately. Used to combat colds and boost the immune system, it can also pack a punch when it comes to inflammation. Ginger features in loads of flavours of tea and is particularly taste bud-friendly when paired with lemon. You can of course make your own if you are that way inclined. Tea is consumed so widely in the UK, that a simple swap to one of these for the many sufferers of joint pain could make a difference. Of course if you feel married to and uneasy about betraying your standard Tetley, swapping one cup a day to start with is fine.

There is a short post about a research study into ginger and joints on the Arthritis Foundation website: http://www.arthritistoday.org/what-you-can-do/eating-well/arthritis-diet/ginger-benefits.php 

I’ve also discovered that apparently rubbing green tea on your face makes you look healthy. But that is another road to go down later.

5 normal things that make you realise how hypermobile you are:

1.       Your morning stretch. There are few greater things in life than waking up and stretching out in bed. However, the joy is slightly hampered when you feel your shoulders moving around in ways they definitely shouldn't be and hear about 35 cracks and clicks that could be coming from anywhere. Hips, toes, knees, they’re all at it. Hypermobile joints can definitely give Rice Crispies a run for their money in the snap, crackle and pop stakes over breakfast.

2.       Hair washing. I have long hair and whenever I reach up to the back of my head to go to work with some shampoo the clicking in my arms and shoulders goes wild. I also start to ache after about 30 seconds of head massaging. The one good thing about this situation is standing under the hot shower and letting it massage your joints a bit (until it goes arctic without warning, which mines really enjoys doing). The downside is once you have recovered, you have conditioner to contend with. Being clean and tangle free is hard.

3.       Cooking. This must apply to lots more people than just me. Chopping, peeling, mixing, grating and rolling cause my already grumpy shoulders to go into full strop-mode. They hate it. I hate it. We all hate it. Last week making fairy cake mixture turned into something not dissimilar to a physio appointment. My shoulders came in and out and cracked and clicked and that was only mixing butter and sugar. Dinner time hypermobility flare ups are not ideal. 

4.       Photos. My sister’s birthday at the weekend provided a wide selection of drunken and ridiculous photos and in most of them my arms look a bit like they have been taken off and sewed back on the wrong way round. Holiday photos where everyone is half naked and looking really pleased on a beach are also often joint shots of shame. It’s a bit like the daily mail side bar of cellulite and things that could be spots but probably aren’t, but for hypermobile people.

5.       Any kind of travel. My morning journey to work in central London involves being squashed into a tube carriage with bits of coat and rucksack in my face. It also often involves holding onto the bars above my head, which my wrists and shoulders love (they don’t) and is always followed by pain. Also the obscene amount of escalators that you have to traipse up and down do absolutely nothing to please hips. Planes are also quite an ordeal. Sitting in small seats with no leg room usually ends in limping off of the plane and being stiff for a few days. Dragging suitcases gets harder every time, but it’s quite nice also being able to off load your luggage on a lovely friend while you float around departures with your YAY holiday face on. 

I’m getting ready to go around southern Thailand in 7 weeks. With a backpack. A large backpack. We shall see.

Snacking for joint pain

I’m having a day where I finish eating and immediately begin looking for the next snack victim. I blame catered meetings and knowing where the treats are kept at work. I was told earlier that eating strawberries is good for joint pain, as I sat at the kitchen table shoving them in my mouth and nursing a painful shoulder. Multi-tasking is key. Apparently the science behind this is that strawberries may reduce levels of C-reactive protein which is a sign of inflammation. Most sufferers of hypermobile joints will know how painful they become when inflamed. I’ve also been trying anti-inflammatory smoothies after exercise lately which I have been quite impressed with. http://bend-trend-hms.blogspot.co.uk/2014/03/liquid-sunshine-smoothie-for-your-joints.html 

Another equally tasty snack is nuts- namely almonds, walnuts and seeds which are high in omega-3 fatty acids and also help with inflammation.

Something I wasn’t aware of before is that an imbalance in omega-6 and omega-3 fatty acids can lead to pain and inflammation in joints. Health conscious people who keep on top of their veg consumption will be keeping their omega-6 down, as veg helps to reduce levels. Heartbreakingly of course junk food increases omega-3 fatty acids, so if you feel like your nutrition could do with some tweaking these are good things to keep in mind.

Having hypermobility syndrome or EDS Hypermobility type can mean that you have seriously alter your way of life to help deal with pain and joint problems. Diet, along with some willpower if you struggle with that sort of thing can really help.

Blueberries and raspberries along with strawberries may also be helpful as they are packed with vitamin C, which is good for keeping joint wear and tear at bay.

There is also a vitamin B-3 (some sort of secret vitamin perhaps as I only found out about it a week or so ago) that is also known as niacin. Word on the internet is that it helps protect your joints for the future, reducing the risk of developing nasty joint and pain issues like osteoarthritis. Good food sources for this are crimini mushrooms and tuna, chicken breast, asparagus and salmon.

Happy munching.

Dysautonomia and EDS

Dysautonomia is a word that I have great difficulty in pronouncing. I would actually put it up there with millennium. It is a condition that shows up a lot on EDS forums and leaves a lot of people confused.

It is all to do with your autonomic nervous system, which is a network of neurons. This is a particularly busy network, responsible for controlling blood pressure, breathing, sweating, the bladder, heart rate, gastrointestinal tract mobility, pupil size and sexual function. It is also not largely under voluntary control. Any malfunction or disease of the system means you have dysautonomia.

It occurs in a number of disorders; one of which is Ehlers-Danlos syndrome. The abnormal connective tissue in EDS sufferers is the believed reason that dysautonomia rears its unwanted head. Hypermobility type EDS appears to be the most commonly associated form with dysautonomia. Not all patients with EDS develop problems with their autonomic functions, but it is quite widely reported.

Symptoms vary widely from person to person and can be very different depending on what your main issue is. The primary symptoms include excessive fatigue, thirst, dizziness or vertigo, heat intolerance  and gastroparesis (delayed gastric emptying). Sufferers may also find that they crave salt, have headaches often, are flushed in the face and have abnormally large pupils.

From what I have seen online and from contact I have had with EDS patients, gastroparesis (another delightful word) is one of more the common complaints. In short, it means that food remains in the stomach for longer than it should. The stomach contracts, controlled by the vagus nerve, to move food along to the small intestine for further digestion. When the vagus nerve is damaged, this process of contracting and moving food does not happen properly. The food journey is slowed or simply stops moving through the digestive tract.

Symptoms associated with this are not pleasant. Often chronic nausea and vomiting, with abdominal pain and feeling full very quickly create the perfect storm of suffering. Other complaints can include heartburn, bloating, erratic sugar levels and weight loss to name a few.

There are ways of having this diagnosed with x-rays and scans and it is worth chasing up if you are unsure or only think you are suffering with this condition. Better to know and deal with it accordingly, as I have found with EDS in general.

Lots more detailed information can be found by visiting DINET- the dysautonomia information network.

http://www.dinet.org/

Liquid Sunshine- A smoothie for your joints

Joint inflammation in a nut shell: It’s not fun. Exercise is an important part of looking after yourself when you have hypermobility syndrome. Inflamed joints often show up uninvited after doing physical activity, which is a shame as the pain often overrides the feeling of ‘yes I can eat another dinner because of all the calories I have burned.’ I find anti-inflammatory creams often don’t cut it, especially after running or lots of swimming. I was told by my last physiotherapist that most people crave something sweet after exercising (or all the time!?), which is where the anti-inflammatory smoothie comes in. Sweetness in a cup that is a pretty colour and good for you. BRILLIANT.

The first one I have tried is pineapple and ginger. Pineapple is not loved by all, whether or not that is the extremely tangy taste, or the fact that is SO yellow I am not sure, but it does have good anti-inflammatory properties. Prepare for science:

 Pineapples come from the bromeliad family of plant, which rarely produce edible fruit, not entirely surprising as pineapples don’t look particularly mouth-friendly, but they are the exception. Bromelain is an enzyme which is used to help clear up all sorts of health issues. Included in this is asthma, breathing problems, a boost to the immune system and reducing inflammation. It is also famed for its pain relieving element.

Ginger is pretty much as super a food as you can get. In terms of anti-inflammation, it contains gingerols, substances which are believed to be behind the reduction in pain for sufferers of arthritis who regularly consume ginger. It is also, in my opinion, absolutely delicious.

With smoothies I often just use enough fruit to make myself one large glass, because I think they taste best when they are fresh. For this yellow delight chuck in:

-          3-4 rings of fresh pineapple

-          A handful of chopped mango

-          A medium piece of ginger, about 2 inches long

-          A few blueberries (because they are just generally SO good for you)

-          1/4 pint of coconut water (or more or less depending on your stance on coconut)

-          About a tsp. of fresh vanilla

Warning: results may look a bit like liquid sunshine, don’t be alarmed.