Ehlers-Danlos Q&A

Updated: Feb 4, 2019

I thought it would be rather nifty to include some of my pals in a blog post, while also helping to spread awareness of Ehlers-Danlos syndrome. If you would like to write a similar blog post, go ask your friends to join in by thinking up some questions to ask you, and we'll publish it here!



Through the years I've been diagnosed with several health issues, including cancer, and so I know as things haven't been as linear as they probably should be (my diagnoses journey has actually been really and truly all over the place), that perhaps things can get mixed up. I explain symptoms and issues  of course, but my conditions (especially EDS) can seem so broad, vague and I'm sure confusing to loved ones.

As time has progressed I'm unfortunately unable to spend as much time with friends as I wish. I told some friends that they could ask me any question about EDS -particular to my case or just in general, something maybe they have always wanted to know or were afraid to ask, or just simply never had the opportunity to ask. No silly questions. Although silly questions are also welcomed -haha, funny is also good!

So here is post one of my friends corner: Ehlers-Danlos Q&A. These are some of the people who made me who I am... Please don't judge me too harshly based on them -I can't get out enough to make new ones 😛

Tasha

I met this hot mess around 2010 through mutual friends, and our friendship has remained strong since -mostly based on the fact that we are both absolute (hot) messes, non-deliberately anti-social weirdos who hate sunlight and love naps. I.E. we both live in the realm of chronic illness and as a result, have less contact with the outside world than either of us would like. But the interstnetz and phone calls have helped keep the magic alive!

She stars with me in this most epic video of all time:

Her kids think I'm really, really cool (so we can just mark that down as fact, as they're teens and they know cool when they see it...  No, no, you don't need to ask them, they're eh, busy at the min. So just take my word on this one. Mark that shit down).

Q. What's the best thing friends and family can do to help you?

The main thing I request from friends and family is understanding. Which sounds basic in itself, but it really isn't. My condition is technically seen as non-progressive, but due to general age issues, and not receiving medical help that I need when I need it -Things are getting worse, my condition may continuing deteriorating. Each day is also different -one day I can walk, another day I can't. One day I can walk around town, do some cleaning, play with the dogs, and the next day I can barely move. I think most friends and family and even acquaintances understand my life under those conditions. They get used to those limitations. And until recently I could leave the house and travel in a car once, maybe twice a week.

But I think it's harder for people to understand progression of illnesses. I'm now at a stage that travelling is agony. And while I am in the process of putting things in place to make travel less painful, I still won't be able to visit friends weekly. And that's just that. But sudden changes are, and will, be hard for people to deal with. You get used to a friend being one way, it takes time but you get used to it -then everything changes, their illness progresses, you're probably a bit annoyed (at the illness, not them), frightened, confused. But stop and think. There is nothing they can do to change certain circumstances. It's harder for them -a million times over.

But your unwell friend or family member will understand you are finding it hard to cope with such changes. Talk to them -or sometimes even better, talk to a mutual friend who seems to be dealing with it well.

Secondly, I just want friends and family to know the basics of my condition(s). Nothing fancy, nothing overly medical, but the raw basics. Ehlers-Danlos syndrome and dysautonomia are hella confusing and complex, I know that. I don't know everything, I'm still learning every day. But if I have to repeat the very, very basic stuff, it really is tiring. Read up on the main EDS websites, or look for a YouTube video. If you are lost, ask me (or your unwell loved one) for a couple of links -I would be happy to oblige, and most disabled people would feel the same way. 

Q. Is there any "helpful" things you wish they wouldn't say or do? 

Sometimes hearing "get well soon" makes me giggle... I'm never getting well, lol, that's kind of the point. I try to give the benefit of the doubt, I think they mean maybe my current pain (if it's a bad shoulder day, for example). The old wives tales of turmeric, lemon juice, and a whole assortment of foods to eat, baste yourself in and stick up your ass, is EXTREMELY eye-rolly. Healthy people try all sorts of crazy shit. Prayer, fairy wishes, etc. I just ignore it. People mean well, but I would be lying if I said it wasn't annoying and silly and sometimes ridiculously condescending. But people are desperate and will place hope on anything -especially healthy people. Mostly people in my life are pretty cool though, and if they said anything silly I know we could laugh about it.


Breanainn


Breanainn is Tasha's partner in crime, although I met him a couple of years before I met Tasha (maybe 2007/ 2008?). We lost contact, only saw each other randomly throughout the years, until himself and Tasha became close.

He's also a fellow spoonie/ disabled person, so we have many a laugh over that. We are super annoying to be around in small cafes -me with the rollator, him in the wheelchair. We take up all the space. So being in the way and annoying is kind of our thing. We are a complex and diverse bunch! And in case you're wondering, as I'm sure you are -I'd totally win in a cripple race. On that note, I realised that we have like zero photos together. Sick recluses are terrible at getting photos together apparently! However, I do have ones that I took of him, as you'll see from above.


Q. Are there any celebrities with EDS, That could help out with awareness?

Cherylee Houston is the one that first springs to mind. She plays Izzy in Coronation Street, and is also the patron of Ehlers-Danlos UK.

Other than that I've heard only hearsay really about other famous people. The thing with any hypermobility condition is that not everyone has it to the same extent -some people are flexible but rarely dislocate! So they might be only in moderate pain and never think anything of it -But other people with EDS may notice it for them. Plenty of people are also double jointed and have absolutely no pain or issues. It's important to see EDS hypermobility type on a broad spectrum, but it's also important to note that not all people who are hypermobile have an illness.

There has also been speculation about historic people such as Anne Frank and Abraham Lincoln having either EDS, Marfan's syndrome or something closely linked.

Cherylee Houston


Sarah


Q. How do your illnesses effect pregnancy and child birth?

With trying to conceive, there seems to be a slight increase in miscarriages for Ehlers-Danlos patients when compared to the general population, as skin issues such as fragility can also occur inside the body.

However, the type and severity of complications really depends on what type of EDS the patient has -vascular EDS tends to present with far more likelihood of severe problems. I'm waiting on testing to rule out both vascular type and classical type, but I'm pretty confident that I have hypermobility type, or some form of hypermobilty spectrum disorder -criteria change came into place this year which has complicated previous diagnoses. So hopefully both London and genetic testing can clear up which condition or sub-type I do have. Here's some further information on EDS types.

The main issue with Ehlers-Danlos syndrome in pregnancy is the obvious one -that with such sudden, extra weight, I (or any sufferer) may be prone to dislocations even more frequently than usual.

For me individually, due to hip dysplasia/ shallow hip socket, I will more than likely require a C section. I'm perfectly happy with this. However, many people with EDS have issues with anaesthetic not working the way it should. Some people need extra at the dentists, hospital procedures and of course that includes epidural during childbirth. I'm not 100% sure why, I guess faulty collagen doesn't play by normal rules when it comes to anything. Luckily I've only had this inability to be sedated occur once in my life so far... But I imagine epidural failing during a C section is the very worst time that this can happen. I've heard of some women needing complete knocking out due to epidural failure. So that is something that I will need to stress to my doctors at the time.

Another possible problem with C section -People with EDS can take longer to heal after surgeries (my biopsy scar took longer than it should), we scar more easily (like that tiny scrape I got on my neck when I was eight that is still present) and our scars can burst open (like the mess on my leg), and of course this can't be great for such large scars. As I said above, skin fragility is common with EDS patients. If you have Chiari Malformation this also brings about possible complications during birth.

Many people with forms of EDS or HSD have mentioned that they felt their pain became worse after pregnancy/ giving birth. I'm not sure if this is down to a natural decline or if it's directly linked to pregnancy in other ways. Nine months is a long time, I know that my issues have all worsened over less time frames and naturally during pregnancy patients are less active. So I suppose it may be a mixture of all those elements combined. However, other people don't feel a noticeable increase in their pain and symptoms -it's very much a wait and see situation, that no one can predict.

My personal main concern in preparing for children is the effect on my body without my medication, particularly my antispatics (Baclofen).Without this medication my muscles tense to the point of all over body tremors, and I fear what way my body will handle these fits once again. This medication was an absolute life changer for me, and without it my body is out of control. I have concerns about how rigidity/ spaticity fits will effect a growing foetus. It might be safer to stay on a low dose, and this is something I will be discussing with my GP in the near future. I'm prepared for extra pain, and can handle that, but the fits are unbearable to the point of no quality of life (or bladder control! Too much info perhaps, but I'm trying to make these posts as educational as possible, even if that means embarrassing). 

From discussions in POTS (postural orthostatic tachycardia syndrome)/ dysautonomia groups, I've heard mixed results of how pregnancy effected each case. Some women actually noted that their symptoms eased up quite a bit during pregnancy. Others worse, and some had no dramatic differences. I suppose the important thing to note is that every pregnancy is different, even for those who have no health issues. Doctors can't predict how things will go for me, in the same way they couldn't tell how things might go for a healthy person.

Planning to have kids is a risk, but it's something we've (myself and Bicky) have been discussing and organising for the last couple of years. From getting advice in support groups of all sorts, to normal things like choosing schools (trying to access a multi-denominational school in Ireland is a major challenge all on its own!) and decorating the spare room into a nursery. At this stage we are as ready as we can be, and are also considering the many issues that will arise after the pregnancy -raising a child as a disabled woman. But millions of disabled people do it all the time. It's something we both want and we feel prolonging it will only equal more problems.  For further information, here's a really well written and detailed piece on Facebook: Pregnancy in JHS and EDS-HT  (Note: JHS = joint hypermobility syndrome, the old phrase for HSD = hypermobility spectrum disorder. And EDS-HT = Ehlers-Danlos syndrome hypermobility type). 



Gabriel

Gabe is one of Bicky's (my husband) oldest friends, although I actually met him shortly before I met Bicky. I think around 2008/ 2009.

Over the years we've bonded over our passion for garlic bread memes, love of Buckfast (you will notice that crops up as a mutual bond for most of my friends) and our shared inner spirit similar to that of a grumpy, old farm cat.


Q. What was the most inconvenient time that a joint has dislocated?

I'll try keep this relatively PG, as err, fam possibly reading, but the number one obvious answer is the most obvious answer (cheers for the question, horsebox). Nothing ruins the mood more than constant injury.

Besides that, I would have to say in the gym. Obviously exercise equals dislocating, so it's never of any surprise, but on one occasion I was sitting on a machine when my hip went out. Quite badly. I was in agony, trying not to scream/ make a show of myself, but also kind of laughing -the thoughts that I would not be able to get it back in, the horror of gym staff ringing an ambulance. The place was hopping. As stupid as it sounds, I was most concerned about people finding out and being mortified. I would forever be that girl that had to get paramedics to help her off the gym machine. The girl whose hip fucking fell apart, another massively clickbaity title in the Democrat, forever and ever and ever... So I sat there. In agony, panicking, and pretending that everything was fine and I'm a normal person doing normal gym stuff. Just casually taking a break, sitting on the machine, like a normal 30-ish person. I eventually got it back in and was so relieved.

Not sure if that's "inconvenient" or just tagged under embarrassment. I'm positive that there are a million other stories that I just can't recall right now! I guess if you enjoy walking, bathing, eating, breathing, then all the dislocations are in some way really inconvenient. 



Damien

Me and Damien met all fresh faced and bright eyed during our teens, I think about 16, the year being... None of your business. There's literally too many memories, laughs and tears to mention in one paragraph, but he's the son my mother wishes I was. Lol. Like seriously, she prefers him over me... Now she is reading this both laughing but denying it, and he is reading this both laughing, and well, sneering I'm sure. He loves a sneer. And a grump. Think like Roger The Alien From American Dad meets... Well that is really all he is. In all seriousness, he lives far away the last few years but I know he is only an e-mail away, and has been a big part of my EDS journey. He has watched me go from "oh that's an odd pain"/ "why is my hand hand doing that?"/ "This doesn't seem normal", to bandages and wheelchair. He's forever asking questions about my conditions, so I knew he would want to take part this blog post.

Q. Do all people with EDS have dysautonomia, or can you have EDS without dysautonomia? 

A large amount of people with Ehlers-Danlos syndrome also have some form of comorbidity, a closely liked secondary condition. And for many, that's a type of dysautonomia like POTS - Postural Orthostatic Tachycardia Syndrome.

But EDS and POTS are not the same thing, and each condition exists in their own right. Not everyone with EDS has POTS, and not everyone with POTS has EDS. While both illnesses generally cause widespread pain, EDS is a fault in collagen while dysautonomia is a fault in our autonomic nervous system (autonomic dysfunction). I.E. all the things our body does without conscious thought -breathing, digestion, heart-rate, blood pressure, and so on. Because of this, the symptoms are widespread and can seem very disconnected. Like EDS, it can be hard to diagnose -although it does not cause dislocations like EDS does. They are both multi-systematic conditions. I'm currently waiting for further testing to see if I have POTS, or another form of dysautonomia called inappropriate sinus tachycardia. My main issues (well, the ones that are most bothersome) are low BP and tachycardia.

Here's a video about POTS that I absolutely love, it explains everything about it perfectly: 


Philip


This miniature wizard came into my life as a very shy, non-drinking, long-haired metalhead. Our first encounter involved him tagging along with Bicky to a very exclusive (haha) nightclub called Amber. Which now doesn't exist, but it was the go to dive for anyone in Dundalk around 2009. I asked him if he wanted a drink -he said no, panicked, and looked at me like I had two heads... He's learned a lot of social (and alcohol) skills since then! Haha. Himself, Bicky and Gabe are a package deal (package of nerds, snort).

He now has short hair, and continues to be short in general. He's now counting how many times I wrote the word short and related synonyms in this one paragraph. We are both quite short and discuss it in-depth. We both also enjoy cider.

Here he is in his early wizard school days -rockin' out with Bicky, haha:


Q. What is a "spoonie"?

A "spoonie" is a term used by the chronically ill that relates to the spoon theory -a way to express energy levels and fatigue to those who have normal energy and fatigue levels. You may hear someone say "I'm all out of spoons today", and similar phrases. Basically spoons are levels of energy, so for example you might have five spoons for the whole day and so have to decide how best to use these spoons. Remembering that you only have five, means you probably have to pick between tasks by importance. So showering might take two spoons, cleaning the house might take three, and so on. Obviously everyone differs.

So the term "spoonie" really just refers to anyone who is chronically ill.

I think it's important to note that many people in the community are opposed to the idea of spoon theory, as they feel it oversimplifies the levels of fatigue we actually endure. And I do kind of agree with that notion. The idea is almost centred around the belief that we may be able to control our energy levels and some how manipulate them. While on a "good day" that might be plausible, things are generally never so easy. For example, if I have a big event coming up I will try and limit my movements and save my energy, in the days proceeding the event. Which coincides with the theory -saving up my spoons. But unfortunately, it just doesn't always work like that. I can try my hardest, yet wake up the day of the event with zero energy and limited mobility.

BUT I do quite like the word spoonie, and I can see its appeal -it sounds cute, almost young. It rolls of the tongue easily. It gives us a title that sounds nice. It's not associated with the elderly, it's not as cheesy as "special needs", or as stern as "disabled". It's more socially acceptable than "cripple" (one of my faves, obviously -but the whole movements of crip the vote and such are not well known outside the community). So for me and for others, even if I don't agree with all the points of the theory, the title is up there with one of my most liked.