EDS Awareness: Coming to terms with chronic pain

As ever, My EDS Awareness month post sneaks in on the last day of May. It's been a big month, including a short, but amazing, trip to Barcelona to celebrate my and my husband's 50th birthdays (wtf?!). His was in April and mine is on 6th June (666... really rather fitting.)
I wasn't sure how I would manage it, given the uncertainties of my Ehlers-Danlos syndrome, but we had booked assistance at the airport and the train station and I took my own wheelchair.
The weather was perfect for me, warm, but not too hot for walking/wheeling around the very accessible city, visiting the wonderful architectural sights of Gaudi, including the incomparable Sagrada Famiglia, the stunning mosaic quirkiness of Park Güell and the stirring originality of La Pedrera and Casa Battló. It made my heart sing and I felt so alive and joyful to be there.

At Gaudi's Park Güell

Because of the limitations that Ehlers-Danlos syndrome places upon me, I had carefully planned the trip to include rest times, using taxis to get around so as not to use precious energy on getting between destinations. Slow walking with a stick, lots of breaks to watch the people go by and an afternoon rest while my husband visited the Picasso Museum were all factored in. Still, by the last day, I was exhausted and had to take to my wheelchair with the planned visit to Montjuïc being left for a future visit.
It's not how I used to go on holiday pre-illness but, after four years, I feel I have come to terms with structuring my life around it.

ACCOMMODATING EHLERS-DANLOS

It took more than two years to come to the realisation that this is how my life now is, restricted, stilted by fatigue and, if I forget to take my pain meds, in agonising pain. I had to decide if I was going to be angry that this had happened, or to accommodate it as an unwelcome guest, planning your life around their convenience.
At the same time, the years were inexorably moving forward, my children would soon both be adults and no longer so reliant on me. And, as I began staring down the barrel of 50, I have had to decide what kind of life I wanted to have, given what I was physically able to do.
I have seen any number of YouTube and Instagram videos of young women with EDS talking or complaining about their conditions; they're hard to watch and I'm grateful that my own illness didn't blow up until much later in life. Although I was always plagued by various bodily pains, digestive problems, dizziness and so on, I had no diagnosis and I wasn't a regular joint dislocator. I just got on with it. I was lucky.

But coming on, as it did, just after I was getting back into working after caring full time for my autistic sons was a huge blow. Realising it was here to stay plunged me into depression, always something that has clung to my event horizon, never quite able to escape completely.

A VICIOUS CIRCLE

One of the problems with EDS is that it can become a vicious circle of debilitation. You are tired and faint and in pain so you don't - can't - move about much. As a result, your body becomes weaker and "deconditioned". It is the cruel partner of any chronic illness.
At my age, this was even more of a danger that had become my reality. Going anywhere took extreme effort and the payback was several days in bed just to get back to the usual poor state.
It was going on the RNOH Stanmore Hypermobility Rehab course that gave me the confidence to start to try to push myself a little bit more. Armed with skills for pacing (that came in very useful in Barcelona) I began to look at my work schedule to try to ensure that I had energy for both the things I wanted to do and the things, like caring for my boys, that I needed to do.

Serendipitously, I found a fitness expert who now also has a diagnosis of EDS. She puts me through weekly (or so) gentle one2one Pilates using a Reformer and, over time, my strength has built up to a level where I can go to a meeting in London and take a bus from the train station, if there is a stop within a few metres of my destination. It's a big money saver from always needing a taxi, although it does exact a greater physical toll. Of course private Pilates is a financial investment that not everyone can afford. Once again, I'm lucky.
Taking up mindful meditation, stopping eating meat and developing a keener understanding of myself, via my own autism diagnosis, have all helped. It's important to do my best to ensure that I, and not Ehlers-Danlos, am in control of my future.

FIFTY PLUS

Although my caring duties are far from over, I have reached a point where they are not as all-consuming as they once were. In theory, I have more time to find or create work and earn money.
Luckily, again, over these years, I have developed my special needs website, worked as a volunteer and honed my skills as a social media specialist for social good. Mostly from my bed. But is this what I want to do for the next 20 years?
50 is nothing to be afraid of; it is a time, for many, of new freedom from parental duties if you've had your children by your early 30s.
But of course, Ehlers-Danlos doesn't like to release its grip quite so easily. Just because I've reached a turning point in age and mental strength doesn't mean it's going to pack its bags and bugger off. I may have learned to accommodate its threatening ever-presence, but I am mindful that it is always a precarious balancing act. One missed or too-late dose of pain meds, too little movement because I'm not feeling well, eating something my EDS digestive system doesn't like or just doing too much (what is too much varies daily), can destroy the delicate equilibrium I have acquired. Even emotional upset or stress can tip the scales into a flare up.
But I never forget that despite all these challenges, I am lucky to be where I am right now. Ehlers-Danlos syndrome is a greedy bastard. It knows that no matter how hard you work to regain strength, one slip and it will have you back in its painful embrace.

READ MY OTHER POSTS ON EHLERS-DANLOS SYNDROME

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Sucked into the black hole of a rare disease

It's fair to say that my life has changed completely in the last year and a half.

Not just my day to day life, but also my hopes and my expectations of how life would be once my children left home - if they ever do.

I am blessed with not one, but three rare diseases, Ehlers Danlos Syndrome, Postural Orthostatic Tachycardia and an extremely rare eye condition called Punctate Inner Choroidopathy (PIC). 

I have endured constant pain, the inability to stand for long or to walk far, unrelenting fatigue and at times, like now, impaired vision with the added delight of having things floating in my eye that make me convinced insects are crawling near me.

I have cried and grieved and complained and felt sorry for myself. I have often contemplated whether it is worth continuing.  Sometimes it seems like the only point to staying alive is so that my children are not left with the burden of my no longer being here. I yearn to run away from myself.

But that's all on the inside, as invisible to onlookers as the illness itself. I have almost become four people. The one people can see, the one I am inside, the one that has accepted my limitations and tries to work with them and the destructive and overpowering me that will push forwards with my dreams, whatever the cost to my health. 

On the outside, I am the plucky trier, bravely battling on within my limits, finding ways to get things done, finding help and treatments. Helping others affected as best I can and continuing and expanding my special needs website.

Aren't I amazing? And I look so good considering everything! This is what I am told - I'm never sure if it's meant truthfully or if it's just to gee me up. Whichever, the words are full of good intentions and I am grateful for them.

But the daily irritations take their toll. The uncontrollable perspiration when I drink or eat something hot. Not every time; it likes to surprise me. The fatigue that creeps up like a car slowing to a halt as it runs out of petrol, while knowing I still have to get in the car to collect the boys from their school, five miles away.

And then there is my constant companion, the pain. 

The pain is multi-faceted. There is the all over body pain that begins to buzz, quietly at first when the Tramadol runs out if I have forgotten to take it, then quickly building into pulsing through my calves, my thighs, arms, fingernails. There is the joint pain, controlled by a different drug but when it has kicked in, it's hard to even pick up a cup of tea. Then there is the gastrointestinal pain of stretchy insides and now a new pain, a labral tear in my hip that is likely to need surgery. No drugs work on this pain.

Plucky me has joined Facebook groups, forums, charities because knowledge is power and helping others helps me feel less like an empty space.

But the worst pain is on the inside. The pain of feeling that I am letting people down. That I forget things I am supposed to do because of the medications; that I cannot do enough around the house and that falls on others; that I need to sleep when I should be being useful. That my husband spends the weekend after a hard work-week running around after the boys and fetching me cups of tea.

The emotional pain of wanting things to happen that are never likely to be possible. Relationships have changed forever. I have changed forever. 

But the me I cannot quiet is the one that still comes up with creative work ideas and plans that I really want to do, if only I had the energy. I push myself to make things happen at the cost of exhaustion because I am not ready to accept defeat. I am not willing to accept the truth of my new reality. I am not sure I ever will. This me is my downfall. 

She's the one that wants everything with no limits. The one with dreams who wants to be living and laughing. The one that wants to go places and meet people and have fun.  The one who wants to love and be loved passionately, absolutely, energetically. The one with hopes and ambitions.

I can't shut her up. I can't cram her into a box and sit on the lid until she suffocates. Her spirit is too strong, her heart beats too loudly but she is trapped in a body that will not comply; the body that has never complied if I am honest. The one that always got me so far before it collapsed from exhaustion while my brain failed to shut down too.

I am no closer to peace than I ever was. I am very good at what I do and I don't want to give it up despite doctors, friends, family telling me I must so I can cope.

But I can't. I want it all. I always will. So I will live with the grief because the truth and acceptance doesn't fit. It never will.

So if you love me, you'll just have to love me for who I am and the way I am. Flawed, sometimes flaky but maybe worth caring about. Maybe.

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The vicious three headed dog of EDS, POTS & Chronic Pain

I'm writing this post as part of Ehlers Danlos Syndrome Awareness Month, with which both Youngest and myself have recently been diagnosed, along with Postural Orthostatic Tachycardia Syndrome (POTS) and Chronic Pain.

It's a fearsome triumvirate rolled into one, like Cerberus, the three-headed mythological dog that guards the entrance to Hades.

I'd like to write something positive about it but there isn't anything, as far as I can see.
In the last six months- in fact, I can now see it was building up for months before that- I have gone from being busy, capable and productive to feeling like a hollowed-out wreck.

I can no longer work a job as my health is too unpredicatable. I have to use a wheelchair to go further than a few yards, which embarrasses my kids, the rest of the time I need a stick to lean on. The simplest tasks use as much energy as a mountainous trek.

Not that I've ever been on a mountainous trek - I now know that I've always had EDS at a low level, undiagnosed, but I thought being low-energy, often dizzy and in a certain amount of pain was pretty normal.

Weak ankles? Slipped Disc and chronic back pain? Painful adhesions? Sore joints? Didn't everyone get this type of thing? You just had to keep going, didn't you, using whatever made you feel better. Which, in my case, is a glass of hot whisky. Sometimes two.

I'm a lifelong gym abandoner. I love the idea, but ten minutes in I'd get dizzy and nauseous so I'd eventually stop going. Now I know this is part of Postural Orthostatic Tachycardia Syndrome, a feature of EDS.

Still I got this far. 46 in a couple of weeks or so. 6th of the 6th (no surprise there, many will say). Married to a very, very understanding man. Had two kids, both, as you know, with Asperger's. I almost certainly have that too. There is some anecdotal evidence the two have some links.

Keep going. Don't give up. Move forward. One day at a time. It's how I was brought up, to just get on with it.

Six months ago, it all blew up in my face. Doing too much, an over-reliance on adrenaline to get through the day, gradually feeling like things were spinning out of my control. Trying to keep on top of everything. I wrote a post here about the Tumble Drier breaking and it being the final straw. I didn't know then how true that was and how that was just the start of my life crumbling catastrophically before everyone's eyes.

I didn't realise back in 2009, just how apt the title of this blog about a life 'Not As Advertised' would become.

I've tried to make adaptations. Put a brave face on it. Laugh at myself so people don't pity me or feel uncomfortable. Pace myself (ha!).

I did a few things last week: Spoke on an SEN panel, hobbled through parents' evening having to explain over and over why I need a stick. Actually I needed a wheelchair, but the venue was on two levels and I was too embarrassed to get it out. I knew it would be a hassle for everyone, so I made do with the stick. Went for dinner with the family on Saturday night, was quite cheerful.

Paid for all that by spending the last three days in bed on Tramadol. Which is usually followed by throwing up, but a period of no pain is worth one session of vomiting.

Everything I try to do takes at least double the time. Talking to people, whether on the phone, online or in person is exhausting, because I put too much into it. It's not the kind of thing you can pace, talking. So I avoid the phone. I avoid people. I prefer to message or email.

I open up my laptop and see many, many unanswered emails. Feel tired all over again. Decide to pace myself by shutting the laptop again.

I can still do some things easily: enjoy listening to music (what a life-saver Spotify is for me to find new things to listen to instead of getting stuck in a middle-aged rut. It's not overly social though as most of my friends aren't on it!).

I still write, though less, and I can still read, though for shorter periods.

I can do some things with help: Cook and do some things in the house (see this post)

Now I've got a disability parking badge, I can go to occasional SEN meetings more easily. But the fact that I had to apply for one in 2013 when in 2012 I was "flying" is almost incomprehensible.

I still have my boys and my husband. There are no words to describe how wonderful my husband has been and how bad I feel for being so useless in return.

I have a few friends and even fewer family members who manage to be supportive without being pitying, which I would hate. Those few people (who should know who they are) manage it perfectly. I'm sure they feel bad for me, but they don't treat me as a sad case and they allow me to feel I still have something to give to them too. This is important to me.

My kids, well who knows? Eldest has made no allowances. He doesn't like change and so is ignoring that anything has changed.

Youngest also has EDS and POTS and, like me, is still going through testing/treatment. Youngest was diagnosed first although I knew I had similar symptoms. It was only when they blew up in my face that I was forced to do acknowledge that this wasn't normal, actually.
I need to protect both my children and ensure they get everything they need. Ah, I still have a purpose, a reason not to give up.

But living for myself? That involves finding 'new ways forward', I have been advised.
I'm an old dog; not sure new tricks are my thing. Is the alternative worse? For others around me, yes. Not for me. For now, that will have to be enough.

Some useful links:
Join the RareConnect EDS Community
Join EDS-UK
Join The Hypermobility Association

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Chemo for little Dylan's Nephrotic Syndrome

I wrote a while ago about my nephew, Dylan and his fight against Nephrotic Syndrome, a rare disease that affects his kidneys.

Next week, Dylan, who is four, is starting chemo.

His mum, Sam, will be giving an update about NS, Dylan's treatment and how it affects his education on my Special Needs Jungle site as part of a series of articles in the run up to Rare Disease Day.

In the meanwhile, this is an article in the Flintshire Chronicle about him.

***

A BRAVE little boy battling a rare disease that causes his immune system to attack his kidneys is preparing to have chemotherapy.
 Four-year-old Dylan Davies-Abbott, who was diagnosed with Nephrotic Syndrome a year ago, will undergo the eight-week treatment from February 4 with the aim of limiting the number of attacks he has.

 His mum Sam admitted it was a “difficult decision” to go down the chemotherapy route as there is a small risk of Dylan becoming infertile. But having had several relapses at the end of last year, Dylan has now been classified as “steroid dependent”, which is the medication used to treat his condition. Steroid use can have damaging long-term effects on his bones and growth, and could also result in him becoming vitamin B deficient.

Nephrotic Syndrome, which is most common among boys aged two to six, affects about 10,000 people in the UK. When the immune system attacks the kidneys, it damages them and causes large amounts of protein to go from the blood into the urine.

 When this happens, it can cause Dylan to become swollen and very tired, as well as causing scarring to his kidneys which could one day lead to him requiring a transplant.
Sam, from Holywell, North Wales, said: “The ideal scenario with the chemotherapy is that we would get a long period of remission without medication and to get Dylan off the steroids.
 “When he has a relapse he does respond to the steroids within five or six days. “We’re grateful that it works but it’s not a long term solution.”
 She added: “We don’t know if the chemotherapy will work but it’s a better option as it’s a short-term intervention.
 “It’s been a difficult decision. Potentially it could have big effects on him.
“There is a very small risk of him becoming infertile.
 “I know the risk is small but it’s still a risk. It’s impossible to say if the chemotherapy will work, it’s very much trial and error.”

 Sam had taken Dylan to hospital in January last year with a suspected sickness bug – what she wasn't expecting to hear was that her son actually had a rare illness and that he may need a kidney transplant in the future.
“They don’t know what causes it and we don’t know why it was triggered in Dylan because he had no underlying health problems”, she said. “It came as quite a shock to us.
 “The worst case scenario is that he could at some point need a kidney transplant but that wouldn’t cure him long term because the problem isn’t with his kidneys.
 “We hope he might grow out of it in late adolescence – maybe 15 years or so.”
Since Dylan was diagnosed Sam and her husband Ian have been fundraising for the Nephrotic Syndrome Trust who carry out research into the illness.

 On March 17, Ian will run the Liverpool Half Marathon to raise awareness of his son’s condition. Sam said: “When we found out we didn’t know any one who had heard of it.”
 For more information visit nstrust.co.uk/pages/home.

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