Rising Up to Fight

My life is an odd balance of good and bad.  I never thought I was too superstitious, but when "the good" came in heaping piles earlier this year, I knew something was coming.  I wasn't wrong.  The last three weeks have been some of the roughest of my life.

I am, unquestionably, lucky and blessed in most aspects of life, which allows me to say (and mean) that I live happily and have a good life.  It pretty much goes wrong with my body and health.  I am up at 0330 writing this, after all.

Over all the years I used this blog as a platform to keep family and friends updated, I guarantee I've never listed more than half of what is wrong with me.  So, I won't start now.  But if you've followed me, then you know the biggies:  Multiple Sclerosis, Breast Cancer, Valvular Heart Disease, and now Ehlers-Danlos Syndrome (EDS).

EDS is a rare genetic disorder that causes a defect in how the body makes (or doesn't make) collagen.  It's a connective tissue disorder.  There are 13 subgroups with a lot of overlap between them.  Some are diagnosed by symptoms and some by genetic testing.  I am pending genetic testing, but I've been confirmed for Hypermobility Ehlers-Danlos Syndrome (hEDS) and I have visual and historical symptoms for Vascular EDS (vEDS).  Vascular EDS has a high accuracy rating on genetic testing, so that diagnosis isn't confirmed yet.

I've been given so much to read, it's overwhelming.  I think, if I was healthy, it might not seem like such a blow, but given everything else, this is hard.  To put it into perspective, when I learned about Breast Cancer, I was home alone.  I called my neighbor, who was like a sister, and she rushed over with shoulders to cry on.  And then we fought.  And we won.  I went at the doctors like, "Let's do this!  Cure me!"  I can't say I didn't cry in the years that followed, but cancer didn't break me.  I beat it.

Multiple Sclerosis didn't break me.  The uncertainty of my future almost did, but I learned how to reframe my thinking and planning, and that made a huge difference.  I've also been in weekly therapy for years to help manage my symptoms.  After I first learned I had MS, I went home, put headphones in, and walked around my garden for a while.  I'd stop and look out over the field, the back of the house, or just stare blankly at the sky.  I was mourning the life I thought I'd have, which I had to do to accept my new life.  I can't win against MS, but I can actively fight it.  So I do.

EDS . . . wow.  I just - I don't know where to start.  I've known for the last two years that the doctors were missing something, and that's not a slight against how good they are.  I have great doctors.  It's a testament to how rare this disorder is.  One of my doctors says I'm his "most monitored patient" and he usually sees people twice my age (seriously).  So, of course, the first question everyone asks me is, "How did they miss this your whole life?"  Because, yes, I was born with this.  It's a genetic disorder.  Whether I inherited it from one of my parents, both of them, or my genes mutated when I was a fetus, I was born with it and have unknowingly been adapting to it all my life.

EDS answers questions about my life that I didn't even know I had.  It's taught me that things I thought were normal about my body are totally not.  And that some seemingly benign things, like having super soft skin, are actually symptoms.

So the next question is why did they find it now?  I am stubborn with my pain levels.  I live in constant pain.  I don't want to depend on controlled substances to the point of resistance or dependency, so I will push it until I can't take it anymore.  Several of my providers think I'm too hard on myself in that regard, and (responsibly) advise that I treat my pain more than I do.  That said, I've been suffering agonizing joint pain for most of this year.  It started in my left elbow and then my right knee suddenly got bad, fast.  At times, I couldn't put weight on it.  Then I woke up a few mornings in a row unable to move my right shoulder without manually manipulating it first.

When I woke up and couldn't move my right shoulder or stand up, I knew I'd pushed it too far.  I got in with Rheumatology and was lucky enough to get a doctor who knew what I had by just pressing on my knuckle.  There was no collagen between the bones.  Also luckily, my health care system has one of three geneticists in the state and she's an EDS expert.  They got me in with her the next day.  Unfortunately, having a connective tissue disorder with a hypermobility aspect and  MS is going make "things very hard" for me.  EDS is not understood well, not many doctors know anything about it, and there's not really anything you can do for it other than preventative maintenance like physical therapy or massage therapy, and pain management.

I'm sure you're asking, "Lucky?"  Well, yeah.  I have answers now.  I've been poked and prodded and tested as  a medical mystery for so long, it's my normal.  My doctors have answers.

My friends and family have questions.  Too many questions.  It's been overwhelming to get a diagnosis like this with the expectation that I read up on it so I can have an educated conversation with the Geneticist after my test results are back, to process what I'm reading (because it's not great), and to field a thousand questions coming from every angle.  I've been filtering info out to family privately and on Facebook as I'm feeling up to it.  I have to be ready to tackle EDS to talk about it and I'm still not sure that this isn't the one that breaks me.  I certainly feel broken.

As with any diagnosis, in the beginning everyone tries to cram hope and sunshine down your throat.  It was the same with MS.  From personal experience, that's a shitty thing to do.  Giving people hope isn't a bad thing, but the way it's done dismisses the patient's fears and isn't realistic.  I've been through it with MS.  I mean no disrespect, but I don't need unrealistic hope.  I see through the bullshit.  I live in my body.  I'm not a pessimist.  To me, the glass isn't half full or half empty - it can be refilled.

EDS adds the certainty that MS lacks.  Those two together are formidable.  I am looking at a future of debilitating pain, physical disability, worsening mobility, and no real way to manage any of it.  That makes researching this so much worse.  I try to stick with facts, but it's good to get an idea of what EDS patients live with and their stories are heartbreaking.  At this point, I'd like to acknowledge that this is long and that you have no idea how many times I've had to stop typing because I can't deal with this.  Taking even just a second to take in a breath and close my eyes, helps.  Like I can lose this reality in that brief dark, respite.

So that's hEDS is a very small nutshell.  There's so much too it.  It's connective tissue.  It's your skin, your tendons, your organs, everything in the soft or connective tissues.  I can literally say that I'm literally falling apart at the seams.  It's everything.

And that doesn't include the fact that I glossed over vEDS.  It's not just me saying that I likely have it.  It's my cardiac history, it's my doctors who know about vEDS asking about my heart in that gentle voice that doctors use for delicate issues, it's knowing that I have leaky valves - the valves in the heart that are made of connective tissue.  The one, big complication of vEDS (and rarely in hEDS) is that the aortic valve will "slip" and cause massive internal blood loss that leads to a fatal heart attack.  Until the geneticist saw the vascular signs, she had started talking about how EDS generally isn't fatal and that hEDS will be minor compared to MS.  That speech disappeared quickly and life expectancy wasn't brought up again.

The median life expectancy for vEDS is 48.  When MS is the cause of cardiac issues, the median life expectancy is 45.  One of those two things is responsible for what's going on with my heart.  If that is all true, I have 3-6 years, maybe.  And it'll probably happen fast.  I've taken many breaks trying to get through this paragraph.  I can't stop the tears.  I thought I was mourning my life with MS?  Wow.

I don't put any of this stuff out there for sympathy or pity.  I'm an odd duck with weird things that people don't understand.  I try to help them understand.  I try to answer questions in one fell swoop instead of repeating myself over and over - that's so stressful.  I try to find others like me that I can learn from.  I want to share my experiences.  I suppose like any of us, I want to know that my life will have mattered after I'm gone.  And facing mortality, again in a matter of three years, is a big deal.

I don't know if it ever really occurred to me that I could've died fighting cancer, but I do know that I prepared for the possibility on the surface.  I got powers of attorney, a living will, and a DNR order.  I made sure that people knew what to do if something went wrong in surgery.  I asked my best friend to help my husband unload my geeky collections when he was ready.

But now?  This is so different.  This is looking at unread books and not knowing if I'll ever read them.  It's wanting to research Batman because there are gaps that I want to fill.  It's hoping I can go back to Florida one more time before I die.  It's hoping I get to finish writing my books so I don't leave my readers hanging.  It's truly getting my affairs in my order.  Reinstating my DNR.  Talking with my husband about what I need to do now to make it easier on him when I'm not here anymore.  I can't scratch the surface of this on a deeper level...my husband, my daughter, my granddaughter - I just can't face that yet.  So yes, right now, it might seem superficial, but don't judge until you're sitting in my shoes - which I don't like to wear because they make it harder to walk.

I know.  It's easy to sit on the other side and say that I don't know what's going to happen.  I don't.  I could die in a car crash on the way to one of my multiple doctor appointments tomorrow (which actually makes a stronger case for having your shit together to help your loved ones when you pass).  I could defeat the odds and live to 92.  Most of the people in my family live ridiculously long lives and are tough.  But I'm only human, with human fears and emotions, and I can only take so much in stride before I break.  I don't like to play what ifs.  It's actually something I bring out in my writing.  My characters will say that what ifs mean nothing and waste energy.  For the most part, that's true.  But there is a time and a place where using them to plan is necessary, and this is the time.

Maybe those tests will show I don't have vEDS.  Honestly?  I'm not going to believe it.  There's a 1-2% error margin.  That should be a relief if it comes back negative.  But I know what symptoms I show and my history.  Even if that test comes back negative, it is possible to overlap with hEDS, which I DO HAVE.  The good news there is that if we can manage it, I can add a decade or two back (from a cardiac standpoint), and the aortic valve slippage is possible, but rare with hEDS.  Which brings me back to pain and disability and a dark hole.  Do I want to live longer in agony?  Or die sooner?  It doesn't really matter what I want.  I'm not in control of that.

I control whether I fight or not.  And I fight.  Pain or agony be damned.  I'm a survivor and that's because I fight.  Death will win, eventually.  None of us can win that fight.  But we can fend him off.  That's our choice.

Even in a dark hole, I will eventually find light.