Living in The Fire Swamp of EDS

Or

Fire Spurts and Lightning Sand and R.O.U.S.’s! Oh, my!

The Princess Bride is a true classic and one of the best movies ever (IMHO).  You might think me crazy but I have come to realize this fan favorite contains a helpful illustration of life with EDS…

You remember the Fire Swamp – it is where Westley and Buttercup escape from the frying pan into the fire and where Westley tries to convince Buttercup that the very real dangers (Flame Spurts, Lightening Sand and Rodents of Unusual Size, aka, R.O.U.S.’s) they face are no big deal.

 

 

At first, living in the Fire Swamp, like EDS, seems impossible (inconceivable, even) and downright terrifying. You hear all the horror stories about it and you start to believe that it would be impossible to come out of the Fire Swamp alive, let alone unscathed. The dangers, regardless of how Westley gallantly tries to spare Buttercup, are very real and require constant vigilance. They are intimidating and overwhelming when you first encounter them…

There are Flame Spurts – jets of flame that spurt up, setting fire to anything nearby. The Lightning Sand is formidable – patches of quicker-than-quick sand that immediately engulf and bury the unwary. And, last but not least, the Rodents of Unusual Size: enormous, vicious rat-like creatures that attack without warning.

Each of these seemed larger than life as Westley and Buttercup start navigating the Fire Swamp, but, as they encounter each one, they learn how to manage, overcome and defeat each challenge.

Flame Spurts? They make a popping sound before the flame appears, providing ample time to move out of danger. Lightning Sand? Buttercup unwittingly discovers what it is by stepping into it and being instantly buried. She and Westley aren’t likely to make that mistake twice after they both nearly die. R.O.U.S.’s? Yes, they exist. And, while they are admittedly vile, heinous critters that do real damage, they are not immune to flame spurts and the sword.

Oh, it is not easy to be sure – these threats are real and they take a toll. The couple is  battered and bruised as they exit the Fire Swamp and Westley boasts to the evil Prince Humperdinck that he and Buttercup could live indefinitely in the Fire Swamp. The unlikely (definitely inconceivable) fact remains, though: They unraveled the secrets of surviving the Fire Swamp.

The Fire Swamp experience is pretty much our everyday life with Ehlers Danlos Syndrome…

Dislocating joints, crazy injuries, muscle spasms? You quickly learn how to pop the joint back in place (reduce a dislocation), brace an injury, relax a muscle spasm and determine what needs a medical intervention and what can be dealt with at home. Yes, it hurts and it is scary but you learn to manage.

Gastroparesis, constipation, MCAS? You learn what you can eat and what you can’t eat, take Miralax regularly, and carefully manage the symptoms with diet and medications. Yes, it is challenging and can suck you in before you realize what is happening but you can manage the issues and find relief.

The neurological stuff, dysautonomia, seizures and other poorly understood co-morbid conditions that stalk EDSers? Like the R.O.U.S.’s, these are very real and very nasty but they can be defeated – or at least managed – with a great deal of effort.  The right meds, the right doctors and a whole lot of grim determination are the needed weapons.

Somehow, you get used to these unbearable (inconceivable, perhaps?) things and find a way to live with EDS, much like Buttercup and Westley found they could in the Fire Swamp. No, it isn’t a place where you would choose to live but if you have to, well, so be it. No, you probably will not come out of it unscathed but that seems less important than it did as you entered the Fire Swamp; there is something to be said for simply surviving.

Once you figure out the tricks of managing each of the threats, they become more of a nuisance than anything, although their danger is not diminished by that perception. Like anything in life, as we face a difficulty and learn how to manage it, that issue that was once a huge problem now becomes something less in its familiarity – a pain in the butt, an annoyance, a hindrance – something to deal with so you can move on to your regularly scheduled program.

A positive attitude like Westley’s certainly doesn’t hurt as you manage the impossible, although Buttercup’s dogged realism is a necessary counterbalance. There is no place in the Fire Swamp, or in EDS for that matter, for a Pollyanna but if you give up hope, there will be none left to find.  There is also no time to bury one’s head in the sand, Lightning Sand not withstanding, and ignore the dangers that surround you on all sides. Complacency in both EDS and the Fire Swamp is a really bad idea – yes, the dangers can be dealt with and they can be regulated to nuisance status, but they must be faced anew each time.

Life with EDS is a never ending balancing act – you put out one fire and another pops up. Or you fall into a patch of Lightning Sand or an R.O.U.S. attacks.  In the beginning, this is overwhelming and terrifying. Truthfully, I don’t think it ever stops being exhausting – just like living in the Fire Swamp would never cease to be exhausting. But, as you learn to manage, your confidence grows and you know that you are capable of living with the impossible; maybe even thriving with it. And, all of the sudden, one day, you realize that you are a pro and have the confidence to face whatever the swamp can serve up…

So, in the words of Miracle Max, “Have fun storming the castle.” Will it take a Miracle? Probably, but with a little perseverance, a lot of hard work, and a bit of luck, miracles can happen and the Fire Swamp can be survived.

Seeing the Unseen

So many medical conditions are invisible to the normal onlooker – EDS, RSD, POTS, Gastroparesis are just a few that my daughter lives with. It is not easy to live with so many challenges that affect every single aspect of your life, while knowing that people look at you living your life wondering how truthful you are… Are you saying you are sick to get attention? Are you as sick as you claim or are you exaggerating just a bit? How can you look so nice and put together if you are sick? How can you smile and laugh if you are as sick as you claim to be?

As a mom, fiercely protective of this child who has far more to deal with than anyone ever should, I sometimes cringe when I have to tell people that my daughter is ill, very ill, because I know they may not be able to believe me. Not because they are bad people, but because it is hard to believe she could be so ill when she looks so cute and put together and is running around laughing. Or when she is playing her guitar with her friends. Or when she is minding the little ones with a smile and a laugh and a gentle hug. Or when she is acting like a normal teenager, rolling her eyes and flouncing out of the room with predictable attitude.

They don’t see what effort it took to just show up:  How much fortitude it takes to get past the debilitating fatigue and simply get out of bed. How draining it was to get dressed and do her hair so she looks presentable rather than looking like an invalid because, well, no 14 year old wants to look like an invalid if she can avoid it. How she couldn’t eat anything for breakfast because it is one of those days and she knew if she did she would battle the nausea and pain all day.

They don’t see her wince when one of the little ones running around grabs her hand and dislocates a finger and they don’t see her quietly putting that finger back in place without a word or a whimper. They don’t see her periodically roll her shoulder to realign it or swivel her wrist so it is back in place or twist her torso so her ribs go back where they belong or shift her hip so it slips back in the socket. They won’t notice her quietly pull away from a gentle pat on the arm that causes her RSD pain to flair up and they won’t realize that she will endure it in silence rather than pointing out that they just hurt her. They won’t see her head swim and her vision dim as her pulse increases the longer she is upright and they will think nothing of her sitting down quickly because they have no idea she will faint if she doesn’t.

They don’t see the toll trying to be normal takes. They won’t ever see her collapsing into the car seat on the way home because she is exhausted by the hours she spent doing what everyone else does without thought. They won’t see her taking her regularly scheduled meds or taking her break-through pain meds when she is feeling the effects of doing too much. They won’t see her the next day when she is in bed because she has to recover from life. They won’t see her putting on a brave face, dealing with the pain that comes from living, knowing that to not live would be even more painful. They have never seen her at her worst – confined to bed, gritting her teeth against the pain, lying in the dark, nauseated and dizzy – so they have no idea how far she has come in battling the conditions she lives with.

To me though, her invisible illness is not invisible at all… I see the wince when her joints go out and the fidgeting she does to put them back. I see the exhaustion and pain that lurk behind her eyes. I see her pallor and the furrow in her brow when her headache starts and I see the effort it takes her to keep going. I see the sadness that surfaces when she makes a choice to protect herself rather than do something that hurt her and the wistfulness that she hides as she watches others do what she cannot. I see the frustration at all that has been taken away from her. I see the bravery and the occasional bravado that she faces her challenges.

Yes, she lives with invisible illness but it really isn’t invisible if you look. I am sure this is true of everyone who lives with an invisible illness. Of course, it takes more than a casual glance to see it – you have to see that person, really see them. You must see their heart and soul; see them in the same way that you really hear someone, instead of just listening to them. You have to be open to their pain and their challenges and be willing to reach out to them once you see it – for most, living in ignorance is easier, so they will never see… Truly, ‘There are none so blind as he who will not see.’ And that is the problem: most people don’t want to see and don’t want to take the time to reach out.

Living with an invisible illness means suffering in silence; unseen, unnoticed, rarely acknowledged and often questioned. But, if you do more than look and listen – if you really see and really hear, my daughter, and the millions of people like her, won’t be invisible anymore. That is what the campaign to bring awareness is all about. If you open your eyes and your heart, you will be able to see what was once unseen and that will mean everything to a person who is living with an invisible illness.

 

Barbarians at the Gate

As a student of history, I tend to view everything from the lens of the past; even Ehlers Danlos Syndrome at times. EDS is a formidable enemy – a pillaging barbarian of historic proportions. Much of human history involves seeing the barbarians heading for the gate and the populace girding itself for war. Our fight with EDS is no different…

For more than 3 years, my family has been under siege by EDS and its ugly band of cohorts. Our situation is not much different from being in a walled Roman city or a medieval castle, surrounded on all sides by the enemy – the only option is to dig in, figure out who you are fighting and just try to survive.

Unbeknownst to us, the barbarians had been quietly encircling us for years, but the first alarm came in September of 2009 when Em had her first major dislocation. Confused by the sudden attack, we could not identify the well hidden enemy – who the heck was holding us hostage? By the time we identified the invaders [for our purposes we’ll call the EDS army the “Vandals“, one of the barbarian groups that attacked Rome (appropriate, don’t you think?)], they were well entrenched and we knew we were in for the long haul. The weapons of this enemy were hypermobility, dislocations, joint pain, and fatigue – and, in conjunction with its equally nasty allies, generally stealing away everything of importance. Here is what we were up against:

Not who you want to see walking up to your front door…

Then, we found the Vandals had invited their buddies the “Goths” (aka Dysautonomia) to the party, who brought their own special weapons: tachycardia, syncope, fatigue and rapid disability. It looked something like this:

The Goths in action

Following close behind the Goths were the Mongols, with their debilitating combo of neurological symptoms. These guys were bad, BAD news. I am fairly sure I saw Genghis Khan himself, leading the charge against my kiddo.

The leader of the pack, Genghis Khan

Then came the Huns – the thuggish and ill-behaved mast cells pillaging/activating all over the place for no good reason. Little did we know that these were the guys who had stirred everyone else up and would prove to be a wily and evasive foe. Attila the Hun is a total jerk, as you might be able to tell from this image:

The Latin inscription translates as ‘Attila, Scourge of God’. ‘MCAS, Scourge of God’ works too.

The last of the usual suspects were the Vikings, casting a pall across the land with gastroparesis, slow motility and early satiety – using food as a weapon is their MO.

Overwhelming force. Against a kid? Really?

Each of these evil doers brought their own special set of symptoms that interacted with and enhanced the weapons of the other attackers. They were often in cahoots, although each of them were plenty formidable enough on their own – banding together, they can feel unstoppable.

It has taken us many, many months of hard work to make progress against these foes. We were ill prepared in the beginning but had a few tricks up our sleeves – a team of good doctors who have thrown their smarts into the effort, family and friends who have stayed the course with us, our own resourcefulness in figuring out how to get through this crisis and the right medications to combat each of the weapons used against us. The siege has been long and hard and exhausting – there have been moments when we felt like giving up.

But, the thing about a siege is… It eventually ends. It may take years, but it eventually ends and, often in history, the advantage is with the home team. The invading army, if it cannot gain a victory, gives up and heads home. Usually weaker than when it arrived. And, better known to the inhabitants than when it slunk onto the scene undercover of darkness. Moreover, by the end, the townsfolk have been battle hardened and have fortified their defenses. They may be attacked again by the same enemy, but it will likely not be a surprise and they will be ready to fight.

So, if you can just hold out long enough and bear the horrible deprivations a siege brings, the enemy can be beaten back. Perhaps not defeated entirely, but once you know the ways of your enemy, you have a chance to to beat him. I know that sometimes EDS is not beatable – sadly, too many good people have been lost to it. But, every person who fights back and wins weakens the enemy so that fewer are taken. We just have to hold on.

 2 Corinthians 4: 8-9 

We are hard pressed on every side, but not crushed; perplexed, but not in despair;

 persecuted, but not abandoned;struck down, but not destroyed. 

Breaking up is [not] hard to do…

Dear Dr. B,

I know we have been seeing each other for a long time – you have been my kids’ doctor for 16 years, in fact. Because you have been such an fixture in my life, I don’t quite know how to say this, so I will just come right out with it:

I am breaking up with you. It’s not me, it’s you. I am just not that into you anymore. No, I really don’t want to remain friends. I am quite sure there are other fish in the sea and I am willing to take my chances.

I stayed with you all of these years for the sake of the children, thinking that consistency would be good for them and for me. It was rocky at times – we had our moments, even early on. But, when it was good, it was really good. Those times that we actually worked as a team felt so right. Unfortunately, it hasn’t felt right for quite a while now and I need to move on.

I have to be honest: I am sick and tired of doing all of the work in this relationship. For years, long before EDS came into our lives, I have been the one who researches and brings my findings to you, hoping you could help with whatever was going on. Let’s be honest, I have figured out pretty much every single issue my kids have ever had and had to go out of my way to prove it to you, each and every time, when you should have figured it out on your own without me saying a word. And I am getting darn sick and tired of paying you for this privilege. That you abuse me with your rolled eyes and derisive snorts when you are in your I-am-the-doctor-and-you-are-just-a-silly-little-mom mode is wrong and I am done taking your unprofessional crap.

Then there is the inescapable and unforgivable fact that you missed some really important things that were right in front of your nose the whole time. Like, for instance, when you routinely told my kid for years that she was ‘obscenely healthy’ although she had a serious genetic condition that you should have caught and didn’t. Like the fact that every single time the nurse recorded Em’s head circumference from the time she was 6 months to 2 years old, the evidence of externally communicating hydrocephalus was right there and you ignored it, to my child’s everlasting detriment.

I have had a resentment regarding these and other issues that initially just quietly bubbled up at times, then it began simmering – now it is boiling furiously. I will no longer give you a pass for what you missed. You had a chance to make my kid’s life better. You didn’t. Now I have to try to clean up the mess you made. I will do that without you. I won’t sue you, although I am starting to think you deserve it, but I will forever resent you for what you could have done and didn’t.

Our time together Wednesday was the last straw – you had one last chance and you blew it. I bring my daughter to you, worried and requesting help. You know she has a whole lot going on medically, not that you have contributed much to her care. Her vitamin D level is a 6.1 – severely, dangerously deficient – and you give her a measly 2,000 IU of vitamin D a day. 14,000 IU a week, when she should be taking 50,000 a week to try to aggressively bring her level up. You were  absolutely right about one thing:  “this process will take a while”-  obviously,  if you only give her a quarter of what she should be given, she will never get to a therapeutic level, she will continue to feel horrible and be at risk for serious health issues. Another 6 months or more of her life wasted. Thanks for nothing. Again.

When we talked about GI issues and discussed her poor absorption and  how she has lost 20 pounds because she has a very poor appetite and nausea – you tell her that she is in the upper range of weight for her age so she has room to lose weight and there is no need for concern. (Which is total nonsense in every way) The worst is this: Translated into the language of a 13 year old girl: You are totally, horribly fat. Thanks so much for the opportunity to develop a poor body image based on a thoughtless, passing comment – we totally needed that on top of everything else. The everything else being gastroparesis, which I am not positive you have ever heard of.

Then there is the fact that I mentioned that my child has pectus carinatum, her ribs and back are wrong and she swears her leg bones are not right – in the context of severe vitamin D deficiency which comes with softening of bones and bone deformities. And you just look at me. And say we just need to supplement her vitamin D a bit. You never even offered to examine her. You allowed a child with a debilitating medical condition to walk away having never examined her. I have no polite words for what I think of this.

When I asked for a referral to a pain psychologist – someone who specializes in chronic illness to help us navigate this journey we are on, you tell me to find someone who can help – you would be happy to refer us, but you would have no idea where to start looking. Really? Isn’t that what I am paying you for? Isn’t that your job? To know precisely where to start. You are the doctor and I am ‘just’ the mom. I come to you for help with the things I can’t do on my own. This isn’t the first time you have let me down – it is, however, the last.

I could go on and on, but, really, what is the point? This has been coming for a long time. We have irreconcilable differences and we simply need to part company. I deserve better, my daughter deserves better and I promise you, we will find it elsewhere. I suspect that you will be happier without me anyway. We have stayed together out of habit and it is time to make some changes.

Lest you think I do not see my own part in this, let me be clear-  I know I have some issues that I have developed while we have been in this relationship: I truly am not the same woman I was when we first met. I have developed some major trust issues and I have been reluctant to ask you for help at times. I sometimes do not communicate well enough with you. I tend to give up when you are being difficult, rather than hanging in there to fight for us. But, now I realize that is because I just don’t trust you to listen or to know enough to help us.  And I also know that you are to blame for most of the baggage I am lugging around right now. I have enough to juggle in my life and I cannot carry anymore. So, I am going to find a doctor who will help me carry the load, instead of adding to it.

Please do better with the next EDS kid who walks through your door. You could have learned so much from Em and could have become a better doctor. This is your loss, you know, not mine.

Sadly and very sincerely,

Em’s Mom

Down the Rabbit Hole: Life with an Invisible Illness

In honor of National Invisible Illness Awareness Week (September 10 – 16, 2012), I have decided to repost an essay I shared a few months ago. This essay sums up what life has been like for my family since my 13 year old daughter was diagnosed with Ehlers Danlos Syndrome .

I have come to realize exactly how much living with a chronic illness is like Alice falling ‘down the rabbit hole’. Having a child with Ehlers Danlos Syndrome, a rare genetic condition, is strangely akin to Alice’s experience of unexpectedly finding herself in a surreal  land. Although Alice in Wonderland has never been one of my favorite stories, I find that I now have a bit more empathy for Alice and her adventures, having fallen down the proverbial rabbit hole myself!

For my family, like Alice, it was a long, disorienting fall before we realized where the rabbit hole was going to transport us. The fall was long enough to be conscious of the change we were undergoing and to feel, in turns, anxious about what was happening and just plain ready for it to end. Finally, landing with a nerve-wracking thump, we got a diagnosis of EDS and we had to face the alien world we found ourselves in.

We found ourselves in a topsy-turvy world where little makes sense and riddles abound. Where my own personal ‘Pool of Tears’ is topped off on a regular basis as I watch my daughter carving out a life amidst her challenges. Down the EDS rabbit hole, I have the surreal task of being thankful that the particular genetic mutation my daughter has won’t kill her, but will just make her suffer for the rest of her life. In this upside-down world, the doctors often know less than a mom with absolutely no medical training.  Here, the  simplest actions (like picking up a bottle of water or rolling over in bed) can, and frequently do, cause significant pain and even serious injuries. Body parts that ‘can’t’ dislocate, do; the ‘impossible’ is all too possible and ‘normal’ is a moving target. It is a land where nothing is as it seems and even necessary things like nutritious food can be harmful; where walking across the room is as challenging as running a marathon. 

We haplessly follow the White Rabbit to one specialist after another, as we deal with the host of co-morbid conditions that come along with Ehlers Danlos Syndrome, few of which the doctors seem to know anything about. Gymnastics, dance, soccer – all the trappings of a normal childhood have vanished, bit by bit, like the Cheshire Cat, and we have now a wheelchair and physical therapy in their place.  Tea is served daily here, with large helpings of madness, as we learn to live with an invisible illness, in which my daughter and others like her, often look perfectly normal – young, beautiful and seemingly healthy – while actually being very, very ill. Instead of the Queen of Hearts shouting ‘off with their heads’, we have doctors who pat themselves on the back while spouting inane things like‘You’re so flexible you could be in the circus’ or ‘Just eat your green vegetables and everything will be ok’. If only I could do as Alice did: scatter them to the wind while defiantly shouting ‘You are nothing but a pack of cards!’ Alas, this is life with Ehlers Danlos Syndrome and there are no such simple solutions.

We have now more or less adjusted to this odd place, this decidedly UN-Wonderland, we find ourselves in and have settled in for the duration. Because, unlike Alice, we won’t ever get to wake up from the dream and go back to reality. This weird world of EDS is our reality and we are stuck here, like it or not. Our task now is to make the best of the situation that we are in – to mold and shape this world into a pleasant, habitable place. We will make it make sense and leave it a better place for the next generation, by educating the doctors and sharing awareness about this rare condition, by finding answers to the riddles of EDS, and by befriending other families who have tumbled down the same rabbit hole. For, although this is a lonely place at times, we are not alone – we share this journey with other EDS families who help us and whom we are blessed to help.

And perhaps, in time and with some hard work,  we will eventually come to view this as Wonderland after all. Because, once you get used to the ‘upside-downness’, life here is somehow a bit, well,  more real  – more meaningful and precious than it was before we took our tumble. Perhaps, in some strange way, when we fell down the rabbit hole, we accidentally found reality instead of losing it.

If this is reality, I’d honestly rather not be here at all. Life with an incurable, chronic illness is hard and terrifying and exhausting. But, sometimes it is not only good, it is wonderful and amazing and brilliant. No, this isn’t the life I envisioned for my family, but it is our life and I have learned enough on this journey to be boundlessly grateful for it. Falling down the rabbit hole has a way of permanently rearranging your life and your priorities. Just ask Alice – or, better yet, anyone who lives with EDS.

Balance is the Key Part 2

Starting where I left off in Part 1…

So, it took me a few days to identify exactly why I felt uncomfortable at certain points during the conference – this whole ‘don’t let fear hold you back’ thing was a little perplexing to me. It seemed so absolutely clear to me that ‘balance is the key’ that I was quite frustrated at times that we were being given a different message. Once I figured out that most of the advice being given was most applicable for the mildly to moderately affected EDSer and not terribly helpful for the severely affected, like my kid, I  became even more frustrated.

Before I go any further, let me say that I don’t mean to be [too] critical of the EDNF or the roster of speakers. My family benefited greatly from the conference and I am deeply appreciative of everyone who contributed. And I totally get that the individuals who spoke are not just doctors, they are humans, and had real limitations:

• Time  Those 45 minute sessions flew by. The speakers had very limited time in which to share their knowledge with and answer questions from a very intense crowd. There was simply not enough time to say everything that needed to be said, so the speakers had to just do the best they could.
• The Audience  Any speaker has to know their audience and speaking to the EDS population is no exception. The majority of people attending the conference, by default, were on the milder end of the spectrum and that is who was spoken to for the most part.
• Not enough research  While the roster of speakers included the best minds working on EDS and who are, by all accounts, very dedicated to the EDS cause, the fact remains that we just don’t know everything we need to know about EDS to effectively treat it. The speakers are not to be blamed for that – they are doing the best they can with what they have. Until we know more, we are all groping blindly for answers.
• Caution  They have promised first to do no harm to the patients they are trying to help, and so, they parse their words and try to give advice that makes sense while not rocking the boat. Of course, the definition of caution seemed to vary from individual to individual, so that wasn’t terribly helpful.

And there my patience and understanding wanes. Because the big issue at hand here, I believe, is a thorny one that isn’t being addressed for a variety of reasons. The biggest of which is politics, which I have no tolerance for whatsoever. I don’t want to delve into the politics of this and name names, but I know for a fact that it exists. I’d rather focus here on the results of this attitude…

There are lots of people with EDS who manage pretty well. They have issues that they manage and they get on admirably with their lives. The message of ‘don’t let your fear hold you back’ probably resonates, I think, with these folks.

But, there is a significant segment of the EDS population for whom the condition is not easily managed – they have a nasty constellation of symptoms that are challenging  and fairly resistant to treatment. The doctors have had precious little to offer these patients who are so very ill up to this point. Autonomic Dysfunction? Drink lots of water and up your salt intake. Severe, intractable headaches and other neurological symptoms? Got to be craniocervical instability – you need a fusion. Hmm, it didn’t work well? Ah, that is unfortunate. About the rest of your weird symptoms? No clue, sorry.

Because there has been little to offer this group in the way of effective treatment, they get essentially ignored. Which I suppose was perhaps understandable when there was nothing to offer them, but since those elusive answers are now within our grasp, thanks to the Driscoll Theory, I have a real problem with this group being swept under the rug.

Dr. Diana Driscoll has been working tirelessly and has hit upon real answers which she has generously shared with the EDS population. Many of us have experienced the life changing answers she has shared – in the form of Diamox, mast cell treatment and CCSVI – and would have appreciated the opportunity to discuss it at the conference. It would have been lovely if we could have heard Diana speak, to ask questions, to have a chance to discuss it. To have these issues addressed by anyone instead of ignored. But Dr. D was not invited, the desperately needed answers were not shared or even discussed, we were just told to not allow our fear to control us and this group of very ill EDSers were swept under the rug. Again.

If you tell me, the mother of a very ill child, to not let my fear hold me back , I am going to look at you like you are nuts. I will not thank you for your homey wisdom and trite cliches. Seriously.

As are so many others like her, my kid is dealing not just with constant, painful dislocations and high levels of pain that are so common in EDS, she also has debilitating neurological and autonomic dysfunction  symptoms (admittedly vastly improved since starting the Diamox), life altering food and environmental sensitivities (admittedly vastly improved since starting the Zyrtec/Zantac combo) that indicate mast cell disease, ‘typical’ EDS GI motility issues, anxiety,  pectus carinatum, TMJ issues, eye issues and the list goes on.

We – SHE – deserves answers and if you don’t have the answers to our problems, don’t patronize me by saying I should rise above my fear. Don’t tell my kid she should push through and ignore her problems and not be afraid. Because the advice to not let fear hold us back starts to sounds an awful lot like ‘you are imaging how bad it is’. Like ‘you are exaggerating the magnitude of your troubles’.  Like ‘it couldn’t possibly be that bad’. It feels pretty darn condescending, to tell you the truth. We have enough disbelief from the medical community, we don’t need it from our EDS experts.

In Part 1, I talked about how balance is so important for EDSers in addressing their myriad of problems and I am truly convinced it is the key. Well, now I am going to suggest that the medical folks who are going to bat for EDSers to apply a little ‘balance is the key’ to their thinking. Why not look at the possibility that Dr. D has hit upon the answers to help us? Why does it matter who figured it out if people are being helped? Is there not room for diverging opinion? (You proved that there is on Saturday when you all sat on a panel for the Q and A and disagreed with one another right and left. Why can Dr. D not be part of those diverging opinions?) A little balance would do us all a world of good in this matter, in my opinion.

Hmm, for that matter, I think ‘Don’t let your fear hold you back” could apply here as well. Maybe they need a dose of their own medicine… I get that they are being cautious for good reason, I really do. But, the same people who are proponents of cervical fusion and appear to view it as having approximately the same risk as eating candy, are too afraid to consider or even discuss a little pill which has been used for years and is helping people avoid surgery. That seems a wee bit insane to me, I must confess – that the least invasive option is demonized and the most invasive option is lauded makes no sense to me whatsoever. Is fear holding them back? Or is their concern valid? Perhaps, and here is a novel idea, we should be able to discuss it like adults?

I got a lot of good out of the conference and I don’t want to minimize that, really I don’t. It was a wonderful experience overall. With anything in life, there is both good and bad. But, this whole ‘don’t let your fear hold you back’ thing clearly has stuck with me. It stuck with my daughter as well and I have to say that I am not loving that. She and I have talked extensively about the conference and she has shared that her experiences were similar, so I don’t think I am really on the wrong track here.

All I want is for the powers-that-be to acknowledge the real possibility for viable treatment options for  the most challenging problems EDSers face. To acknowledge that Dr. D is on to something and simply be open to considering her work. A little bit of balance. And for their fear to not hold them back from offering help to those who really need it.  I don’t think that is too much to ask…

Balance is the Key Part 1

The official theme of the 2012 EDNF Learning Conference was Living With EDS. And don’t get me wrong – it was great,amazing, awesome. We learned a lot and I am very grateful for the opportunity to attend. I appreciate the efforts of everyone who worked to make the conference a reality – EDNF staff, volunteers and speakers alike. But, (and there is always a ‘but’, isn’t there?) I have some mixed feelings about some of what was put forth.

If the official theme was Living With  EDS, then the unofficial theme was Don’t Let Fear Hold You Back. Which is great and all and I would have a hard time disagreeing with it on paper. I just found it a little strange to be bombarded with that concept because the recurring theme that I kept coming back to after each session was Balance is the Key.

I have to be honest, I didn’t totally get the direction that some of the speakers were taking us. And, to be fair, most didn’t go there and even those who did, I think, would readily agree with the concept of balance. There is a pretty wide range of opinion among the EDS experts – which is somewhat entertaining (particularly when they are all in the same room answering questions) but also somewhat unnerving. I suspect that they would be more in agreement than not, but I heard enough contradictory advice that really made me doubt that. What each speaker actually says and what the listeners actually hear, really matters. And, what came through for this listener was a consistent emphasis on issues and concepts that were, perhaps, not totally helpful and somewhat confusing.

For instance, in the Parents of Children with EDS Discussion Group, which was facilitated by a very knowledgeable doctor/mom who has EDS and which ended up being more of a Q and A with her instead of being a discussion group, a question came up about a basically asymptomatic child with a strong family history of EDS, who was having foot pain while playing the goalie position on his ice hockey team. The answer below came among other answers to other questions like ‘your kids are manipulating you and playing the ‘I am tired’ or ‘I hurt’ cards to avoid doing things they don’t want to do’ and an apparent lack of concern for the child who has had multiple fractures but continues to cheer. While I firmly believe that each family living with EDS must answer the sports question based on their own situation, there being no one right answer, I was a bit flummoxed by this and was stunned by the departure from the cautions we personally have been given by most of the doctors we have seen. I was sitting there thinking about all of the adult EDSers who were irreparably damaged because no one intervened when they were children and wondering why caution was absent in 2012…

The Don’t Let Fear Hold You Back answer to the hockey question: It is true that the initial statement given in response was that perhaps hockey is not the ideal sport for a kid with possible EDS, but that was quickly followed up with the suggestion that you can’t put  kids on a shelf, you gotta let them live, just give him orthotics and wait and see what happens.

The Balance is the Key answer (aka my opinion): No, you can’t put EDS kids on a shelf, but as a parent you sometimes have to make really hard, unpopular decisions. At the very least, the child in question should be given opportunities to enjoy a variety of activities, athletic and otherwise – all children should be well rounded and that is particularly crucial for EDS kids. If the time comes when hockey is no longer feasible, the child should have other activities he loves to fall back on. I speak from personal experience: it is devastating to watch a child lose the activity which defines them. Parents have the power and authority to guide their children into the least damaging activities and, for goodness sake, if you know about EDS before your kid has a life altering injury, you have the chance to protect them and teach them how to protect themselves at the same time! A beloved sport does not have to be an all or nothing proposition: you can tweak and make adjustments, avoid competitive versions and so on, but sometimes you have to walk away for your own good. Figuring out which is the right answer at any given moment is a valuable lesson for a child with this life long condition. It really is about finding the right balance – you might have to fight to find that balance, particularly since it is constantly changing but that should be the goal. 

As I already mentioned, in the ‘discussion group’ the topic of  ‘how do I know when my kid is playing me’ came up. The question involved my kid ‘miraculously’ is able to do fun things, but avoids the not-so-fun things.

The Don’t Let Fear Hold You Back answer: Yes, your kid is playing you. You have to be tough, make them get up off the couch. They don’t get to do anything fun until they manage to go to school all day. I went to medical school, I had 4 kids. You have to push them to achieve. (Admittedly, this is a simplification of what was said, but I don’t think it is inaccurate – at any rate, it is what I heard from this conversation, which goes to my earlier point that what is actually said, matters. I only heard the tough love attitude until a lovely mom in the group spoke up and her comments are shared below.)

The  Balance is the Key answer (via the very wise mom who happens to be a reader and who was brave enough to speak up in the climate of tough love):  You might have to make adjustments to their schedule so they can do more, don’t necessarily assume you are being played. Shortening their school day can make it easier for them to do both the fun and not-so-fun. Be willing to look for solutions.  She and I spoke afterwards and agreed, that for these kids who are really ill, they deserve a chance to have fun and be normal sometimes. It is totally unrealistic to demand that they spend their limited energy solely on school. What kind of life is that? Again, balance is the key. Yes, kids – even sick ones – need limits, but that must be tempered with an understanding of the condition they are living with. As parents of EDS kids, we have just a few years to get up to speed with knowing everything we need to know about EDS so we can teach our children everything they need to know about it before they are grown and have to manage their condition on their own. It is our job to teach them to take care of themselves and when we tell them to just push through, we are doing them a grave disservice. Too many parents come to the EDS diagnosis with unhelpful, clueless attitudes that were passed on from their own childhoods. Often, they don’t fully understand how damaging those messages are to an ill child. They are not lazy, bad, attention seeking or crazy. They are ill and they need help managing this life long condition. Sometimes they need a kick in the butt and sometimes they need a hug. Balance is the key! (Tracy, if I misrepresented your thoughts, feel free to correct me!)

Another instance that comes to mind is Dr. Levy’s talk on ‘It’s Not in Your Head – Or is it?’  He said a number of times: Don’t let fear hold you back… And again, I mostly agree and yet I was a little confused because some of what he said sort of contradicted the typical advice we get. Is it joint protection at all costs or don’t let your fear hold you back. Or something in between?

The Don’t Let Fear Hold You Back approach: The gist of his talk was that pain is hugely impacted by your mind – which is a no brainer (no pun intended) for most of us. He spoke of not allowing fear of dislocating keep you from an activity. That you should not let fear hold you back from living life.

The Balance is the Key approach:  I know that reality gets a little skewed for the EDSer and they can dwell in an unhealthy fashion on pain and fear, but sometimes pain still is an important signal to be heeded and sometimes fear can protect us from doing something that could be harmful. Telling an EDSer to ignore pain and fear might be equally as harmful as encouraging them to wallow in pain and fear. I don’t think the answer is one or the other, rather, it is finding the correct  balance so you can live to the fullest extent possible. I do not think if you say, if I do that particular activity, I will most likely dislocate and it will hurt, that it means you are living in fear. That may be a practical assessment of reality that leads you to either making a different decision or tweaking the activity in such a way that you can safely do it. On the other hand, you don’t want to look at the world from your bed and say I can’t do anything. I happen to think that is a terribly rare attitude – the vast majority of EDSer want desperately to live to the fullest.  EDSers should be taught to listen to their bodies and analyze the risks and make wise, informed decisions so that they can live life fully.

It is true that fear is a real part of living with EDS. There are some very real things to be afraid of. So real, oftentimes, that it can be paralyzing. And, that we are sent such contradictory messages from our experts, is part of the problem. At the same conference, we heard both sides of various arguments. Have surgery, don’t have surgery. Avoid high impact activity, go ahead and play that sport which has injured you multiple times. Rest, push yourself. It was confusing and frustrating at times.

I finally have realized that part of the frustration I was feeling was that there is such a huge range of impact within the EDS population – and any given set of comments was being  broadcast to everyone, regardless of whether it was applicable or not. The barely impacted and the severely impacted got the same messages – with the mild to moderate folks getting the most advice. And those bits of advice just don’t always fit everyone. So, the discussions of POTS mattered a great deal to those disabled by it, but were rather meaningless to those who aren’t dealing with it. Discussions of which sports should kids play and under which limitations mattered a great deal to kids who are still well enough to play, but fell on deaf ears for those of us for whom playing sports is a distant dream. EDS treatment/ advice is never, ever one size fits all. And we all need to keep that in mind. The parent of the mildly affected child should cling to the knowledge that not all EDSers are functionally disabled. The parent of the severely affected child should cling to the knowledge that many do get better, particularly with the right treatment. But that is hard to do when you are hearing a one size fits all message and trying to figure out how it fits in your life. And, for those of us whose lives have been violently turned upside down by EDS, it was frustrating to feel that our very real concerns were not fully addressed and even minimized at times.

In part 2, since part 1 is already way, WAY too long, I want to talk about how a discussion of what we know about why some EDSers are so terribly ill would have been beneficial to have at the conference. And probably other stuff like how this balance is the key business is easier said than done, as well.

If you are with me this far, pat yourself on the back and congratulate yourself for plodding through! Do something nice for yourself because you deserve a reward! Part 2 is on the way…

The Key

The Olympics, EDS and stolen dreams

I thought/ hoped that I was past all of those painful firsts since we are beyond our first year after the official EDS diagnosis. But, there is one I forgot to prepare for, because it only occurs every 4 years and it is a rather big deal to me.

The Olympics.

You have to understand…I love the Olympics. Love, love, love them. I love the history and the tradition and the pageantry and the patriotism and two weeks of the youth of the world being united. I love the underdogs and the shoe-ins and the winners and the losers and each touching story of the blood, sweat and tears it took to get to the Olympics. Simply put, the Olympics are about dreams and dreams are powerful things. For me, that is what it is about.

Truly, I am the most non athletic person in the world and I really don’t care much about sports one way or the other most of the time. But, every 4 years (well, really every 2 years because I love the summer and winter games equally), I become a Fan of Sport for 2 straight weeks – all sport, doesn’t matter which one. If it is in the Olympics, I will happily watch it. Curling? Pop some popcorn. Badminton? Turn it up. Hockey?  Yeah, sure. Ya Betcha. The Marathon? Woohoo!

While I will gladly watch any sport with an official Olympic stamp of approval, I do have my favorite Summer Olympic sports… Swimming, diving, track and field. Gymnastics.

And there is the rub. Gymnastics has always been my favorite – I think every little girl watches the US gymnasts compete and thinks how great it would be to do that. But, the last time we watched the Summer Olympics – in 2008 – Em was ‘fine’ and still in gymnastics and we had never even heard of Ehlers Danlos Syndrome. She and I sat and watched the Olympic gymnasts together – and dreamt of things larger than ourselves. We had no idea that those dreams would soon be stolen by a thief lurking in her genes.

Now it is 2012 and so much has changed. Em is now unable to do the sport she dearly loves – unlike me, her love of gymnastics was more than just as a spectator. She, like every other little girl who has ever put on a leotard, harbored Olympic dreams. While actually going to the Olympics was probably never in the cards for her, even without EDS, she was still the real deal – she had real talent. And those dreams were stolen from her. Now, even watching gymnastics is painful for her and watching athletes in any sport do their thing is just a reminder of what she has lost. And I have to weigh my love of the Olympics against my kid’s emotional well being.

I generally try to have a positive attitude, but it is hard at times and this is one of those times. It is in moments like this that I hate EDS. It has taken so much from us – from Em in particular. For it to taint my beloved Olympics just isn’t fair. But, to go from Gymnast to a wheel chair in a year’s time is a brutal and breathtaking change for a kid to live with. To say THAT is unfair, doesn’t even begin to describe it.

The opening ceremony in London is tonight. I will be watching because I cannot NOT watch. But, there will be a shadow over the games for me. A little of the joy will be gone: the dreams have dimmed a bit and the torch will not burn as brightly for me in this first Olympics since Em was diagnosed. Perhaps future Olympics will be easier, but this one will certainly be bittersweet and I sadly suspect it will never quite be the same.

Grief Triggers

For anyone who suddenly finds their life turned upside down by a diagnosis of an incurable condition like EDS, there is a real and unavoidable and highly individualized grieving process to experience. One aspect of this grieving process is the Year of Firsts – the first year after a traumatic event (like a diagnosis), in which you have to painfully face the first time for, well, everything. The first Christmas, the first birthday, the first day of school, the first family get-together – any first is difficult. Eventually, there are no more painful ‘firsts’ to deal with and life can get back to something resembling normal. For me, the year after our official diagnosis has been a Year of Firsts. Frankly, it has been a really rough year.

As our Year of Firsts winds down, my life is increasingly filled with breathable air and less filled with grief. I no longer feel as if I am wearing my skin inside out, as I have for the past months. I am finally to a point where I can take life one day at a time, rather than only managing one hour or moment or breath at a time. But, still,  I am sometimes blindsided by a deep sadness that comes out of [seemingly] nowhere.

I don’t know about you, but I find that certain places or events trigger a fresh round of grief over what has been lost, even after I think I have worked through my grieving process. It took me a long time to figure out what was going on and to honestly recognize my triggers but I eventually got there: it took me even longer to get to a point where I could write about it. Hopefully, facing it will grant me an awareness that will make it easier.

I finally recognized that one of my biggest grief triggers is our local YMCA… Both kids took classes there since they were little – Luke did/does Tae Kwon Do and Emily did gymnastics and ballet; usually their classes were on the same night. For years, we spent every single Tuesday evening at the Y. Em’s big injury happened at the Y – there was no way of knowing at the time that it would be the last time she would walk into the Y as an athlete or that her life would be so utterly changed afterwards.

Luke still does TKD at the Y, although he is now an instructor after earning his black belt. So, I am often back at the Y to drop him off or pick him up – but Em’s absence is a striking void. When I am there, I walk through the halls that I walked so often in happier times, and I am weighed down with sadness that she is not skipping happily off to class or cartwheeling down the hall after class. When I see the gymnastics area and equipment, my heart is painfully squeezed – nothing has changed there, except her absence.  When I see the excited preschoolers clad in pink leotards or the laughing, chattering competitive gymnasts, I hurt unbearably and, sometimes, am even angry that my child cannot be with them, that her chance was stolen. I cannot avoid seeing the blue mat where she dislocated her knee, and when I do, I am carried back to that night and cannot help but think of where she is today. Every single time I am at the Y, the wound is reopened – I don’t think I have even healed enough for that wound to scar.

So, now I understand that it is painful to walk into the Y because it makes me remember what we have lost because of EDS. I feel overwhelming sadness at times and I occasionally feel really angry. And, now that I know, I think it will be easier to deal with.

My other grief triggers: healthy, happy children who take their health for granted, casual mommy talk of our children’s futures, various holidays and family get-togethers, and events that my daughter misses out on. Oh, and any and all references to gymnastics. And happy people in general. And babies. The trigger can be big and noteworthy or tiny and inconsequential – and it certainly doesn’t have to be reasonable. I don’t believe ignoring or covering up feelings of grief is helpful, so I try to face it forthrightly with the prayer that I am able to eventually find  acceptance and maybe even peace.

What about you? Do you have grief triggers? What are they and how do you deal with them? If you want to share and feel it would be helpful, please do!

The Stages of Grief

Down the Rabbit Hole

I have come to realize exactly how much living with a chronic illness is like Alice falling ‘down the rabbit hole’. Having a child with Ehlers Danlos Syndrome, a rare genetic condition, is strangely akin to Alice’s experience of unexpectedly finding herself in a surreal  land. Although Alice in Wonderland has never been one of my favorite stories, I find that I now have a bit more empathy for Alice and her adventures, having fallen down the proverbial rabbit hole myself!

For my family, like Alice, it was a long, disorienting fall before we realized where the rabbit hole was going to transport us. The fall was long enough to be conscious of the change we were undergoing and to feel, in turns, anxious about what was happening and just plain ready for it to end. Finally, landing with a nerve-wracking thump, we got a diagnosis of EDS and we had to face the alien world we found ourselves in.

We found ourselves in a topsy-turvy world where little makes sense and riddles abound. Where my own personal ‘Pool of Tears’ is topped off on a regular basis as I watch my daughter carving out a life amidst her challenges. Down the EDS rabbit hole, I have the surreal task of being thankful that the particular genetic mutation my daughter has won’t kill her, but will just make her suffer for the rest of her life. In this upside-down world, the doctors often know less than a mom with absolutely no medical training.  Here, the  simplest actions (like picking up a bottle of water or rolling over in bed) can, and frequently do, cause significant pain and even serious injuries. Body parts that ‘can’t’ dislocate, do; the ‘impossible’ is all too possible and ‘normal’ is a moving target. It is a land where nothing is as it seems and even necessary things like nutritious food can be harmful; where walking across the room is as challenging as running a marathon. 

We haplessly follow the White Rabbit to one specialist after another, as we deal with the host of co-morbid conditions that come along with Ehlers Danlos Syndrome, few of which the doctors seem to know anything about. Gymnastics, dance, soccer – all the trappings of a normal childhood have vanished, bit by bit, like the Cheshire Cat, and we have now a wheelchair and physical therapy in their place.  Tea is served daily here, with large helpings of madness, as we learn to live with an invisible illness, in which my daughter and others like her, often look perfectly normal – young, beautiful and seemingly healthy – while actually being very, very ill. Instead of the Queen of Hearts shouting ‘off with their heads’, we have doctors who pat themselves on the back while spouting inane things like‘You’re so flexible you could be in the circus’ or ‘Just eat your green vegetables and everything will be ok’. If only I could do as Alice did: scatter them to the wind while defiantly shouting ‘You are nothing but a pack of cards!’ Alas, this is life with Ehlers Danlos Syndrome and there are no such simple solutions.

We have now more or less adjusted to this odd place, this decidedly UN-Wonderland, we find ourselves in and have settled in for the duration. Because, unlike Alice, we won’t ever get to wake up from the dream and go back to reality. This weird world of EDS is our reality and we are stuck here, like it or not. Our task now is to make the best of the situation that we are in – to mold and shape this world into a pleasant, habitable place. We will make it make sense and leave it a better place for the next generation, by educating the doctors and sharing awareness about this rare condition, by finding answers to the riddles of EDS, and by befriending other families who have tumbled down the same rabbit hole. For, although this is a lonely place at times, we are not alone – we share this journey with other EDS families who help us and whom we are blessed to help.

And perhaps, in time and with some hard work,  we will eventually come to view this as Wonderland after all. Because, once you get used to the ‘upside-downness’, life here is somehow a bit, well,  more real  – more meaningful and precious than it was before we took our tumble. Perhaps, in some strange way, when we fell down the rabbit hole, we accidentally found reality instead of losing it.

If this is reality,I’d honestly rather not be here at all. Life with an incurable, chronic illness is hard and terrifying and exhausting. But, sometimes it is not only good, it is wonderful and amazing and brilliant. No, this isn’t the life I envisioned for my family, but it is our life and I have learned enough on this journey to be boundlessly grateful for it. Falling down the rabbit hole has a way of permanently rearranging your life and your priorities. Just ask Alice – or, better yet, anyone who lives with EDS.