The week that I can't remember

September is Chiari awareness month and also Intracranial Hypertension awareness month.  With that in mind, I'm going to tell you a story that is ultimately about both.

I don't know about you guys but I divide my life according to a timeline of memories.  There are the things that happened as a kid, the things that happened before Paul (my hubby), life after Paul, life after kids, events before and after college, events that happened while I worked ICU, life after EDS, etc.  Its just how I remember things.  And now there will forever be that notch in my timeline called "That Week".  I remember very few of the events during this particular week or even during the time directly after.  But, I'm going to try to break it down and make a record of it just the same.  This will be a combination of things I do remember, things that Paul has told me, things that friends told me and texts and emails that I've went back and read. 

"That Week" actually began on Friday, April 25th.  This is the last day I truly remember for a few weeks, actually.  For a while I'd had symptoms of intracranial hypertension (IH), a comorbidity to Ehlers-Danlos, chiari malformation and all of our other fun disorders.  I'd been on medications like Diamox and Lasix for a while but we really didn't know how serious my illness was.  I'd never had a lumbar puncture or ICP monitor to actually check.  So, on this Friday I was scheduled for one.  It was fairly simple.  I went in to the outpatient center, signed in, was prepped then put to sleep.  After the procedure I was told that my opening pressure was 60+.  See, our spinal fluid moves at a certain pressure that can be measured just like our blood pressure.  That pressure is usually associated with the volume of fluid or abnormalities that obstruct it.  A normal pressure is around 5-15.  Anything over 20 can be diagnosed as intracranial hypertension.  Pressures in the 30-40s can cause significant damage, pain, headaches, blindness.  Its not often that we hear of pressures that are higher than that.  Mine was one of the highest that my surgeon had saw and he's done this for 30 years.  The tool used to measure the pressure only goes to 60.  My fluid reached that mark and kept going so we don't know how high it was.  That scared me!  He drew off quite a bit of that fluid, dropped my pressures to normal, did a prophylactic blood patch and sent me home. 

That night I was fine.  I was cautious with my activities, used my hated wheelchair.  That night we stayed at the motel (my surgeon is in another state) and the next day we traveled home.  I don't remember the trip home or much after.  Paul says that I started acted odd that morning on the drive home.  He says that I slept a lot more than usual and was a little "off".  He thought it was a result of our trip and all I'd been through.  According to him and verified by my texts to friends that I've read, I was "off" all weekend.  I slept almost the entire weekend.  When I was awake I cried with a headache, I talked out of my head then I slept some more.  If you know me, you know that I don't sleep until I have to.  My phone shows that I texted and emailed my surgeon on Sunday to tell him that I had the worst headache of my life and that I was hallucinating.  Still, we tried to wait it out and see what was happening.  Maybe it was low pressure or a spinal leak, maybe my body was learning to adjust to new pressures.

Then Monday came.  Monday was one of those days that changes your life.  I remember a few things.  Paul had to go back to work that morning and left me with my oldest daughter.  I woke up very early with an even worse headache.  This was a 12 on a scale of 1-10.  I felt like my head was being clamped by a vice and crushed.  I remember that.  I remember taking strong pain killers and getting no relief.  Then the vomiting started.  I took Zofran and it didn't help.  I knew something was very wrong.  I woke my daughter and made her come sit with me.  She and family has helped me fill in the blanks from this day.  She came in and sat with me.  She made me oatmeal and tried to feed me.  Apparently she couldn't keep me awake.  I kept passing out, over and over.  At some point she snuck off to call her dad and tell him that something was very wrong.  He said he was coming home to get me, we were going back to my surgeon and the hospital.  My daughter got me up, helped me get to the shower.  After, she brushed and dried my hair for me.  She packed my suitcase for me.  Just thinking of that now breaks my heart.  I can only imagine how scared she was.  She watched me, kept me talking to her and got me ready to leave. 

Paul loaded me into the car, stopped and picked up my dad and we were off.  I don't remember that.  I do remember showing my dad how to work my blood pressure cuff, how to check my vitals and showing him what meds I was taking, all just in case.  We made a very long trip to another state to see my surgeon because no one local would understand what was wrong with me.  My disorders are too complicated.  Apparently we drove for almost 10 hours.  I was unconscious most of it.  I woke up to cry, hold my head and try to throw up.  That was it.  I don't remember those 10 hours.  It was Monday night when they checked me into the ER.  Paul says that they gave me a lot of meds and I finally seemed a little more stable and had less pain. 

I saw my surgeon the next morning.  At first, he wanted to do an emergency decompression.  Then he decided to do a VP shunt (a shunt that goes in your brain to drain fluid).  Then he decided to put in a lumbar shunt (this shunt goes in your back and drains fluid).  This is what I eventually got.  I was in the hospital for one week, Monday-Monday.  I remember seeing a few friends, I remember talking to dad and Paul a couple of times, I remember seeing my doctor once.  That's it.  On Thursday, May 1st, they took me to the OR and put a lumbar shunt in me.  This is a small rubber tube that is in my spinal column.  It runs around my body and drains excess fluid into my abdominal cavity where my body absorbs it and moves it out.  I don't know what day we left and came home.  I don't remember much for another couple of weeks.  I was very sick during that time.  I made a bed on my couch so that I could be with everyone and I didn't move for those two weeks or so.  I was still beyond sick.  I was still hallucinating, still sleeping all day.  Paul has told me that he was very worried about me during that period.

Eventually my body healed some.  Or adjusted.  I don't know which it was.  I'm better now than I was then.  I'm not well but I'm not seeing some of the crazy things I was then! I'm not sure that the shunt has done much for me.  I still have a lot of symptoms of high pressures.  I'll live with it for now, though.  I still don't remember much of that three week period but my dear husband fills it in for me.  I know in my heart that I am lucky to be alive, that I beat the odds.  With pressures that high then the complications that followed, I should be gone.  Those aren't the kind of things that people survive.  But, here I am to tell you about it now.

And that is the long winded story of the week (or three) that I can't remember.  That definitely becomes a landmark on my life's timeline.  Things will now be remembered as before I tried to die and after I tried to die.

The Five Stages of Grief

If you were ever a medical or nursing student, or majored in any other subject that required a psychology class, then you've heard of the five stages of grief.  We are taught that these are the stages that we go through when we lose a loved one, when we face death.  However, we are not always taught that those who live with chronic disease also go through these phases.

What are the five stages of grief?  They are Denial, Anger, Bargaining, Depression and Acceptance.  We can go through them in any order, we can go through some stages more than once, we make take years to move on to the stage of acceptance.

I have so far moved through denial, anger and depression and acceptance.  I constantly deny my illness and disability.  When I hit this one hard I will usually try to work too much.  I will clean, scrub, cook horrific amounts of food, craft, shop, work outside, anything to deny that I am sick.  I'll go until I can't.  Until my heart won't let me.  Until my body begs me to stop.  Until my head is pounding.  And, for a few days after, I have a hard time going at all.  And then I get angry.  I'm mad at the doctors for not fixing me, for taking so long to diagnose me, whatever reason I can find.  I get mad at my family for not pitching in more, not listening, not taking me serious.  My poor husband bears the brunt of this stage.  Then, I'll often become depressed.  I become afraid of dying, upset about the things that I can't do.  I have to work on who I am again, find my value.  Then, for a few days I move into a stage of acceptance and am at peace with who I am and where I am.  And before long I change and it starts all over.

So, what I want you to know is that these feelings are normal.  If you are dealing with a terrible illness, if you've lost a family member, if you've been through a huge change in your life, then you'll go through these stages.  Recognizing them helps you to understand what you are feeling and why.  It helps bring a bit of perspective so that you can better live with it and maybe move on.

I never knew you were sick....

How many of us have heard that?  If you have a chronic illness, a genetic anomaly, then you've probably heard it at least once.  I never know how to reply.  Should I feel defensive?  Should I try to explain how I was born with EDS but wasn't symptomatic?  I try to be honest about these things so that people learn from it.  There is no easy or right answer to this comment.

See, Ehlers-Danlos is part of me.  Its in the very cells of my body.  Its in my genetic makeup.  As a kid, I wasn't sick.  But I was bendy.  No, I was freakishly double jointed.  I could bend in all kinds of neat ways.  I was also horribly clumsy.  I ran funny and remember kids laughing about it.  When I was in elementary school, gym class was torture for me.  Our gym teacher was athletic and insisted that we tumble, roll, somersault and cartwheel our way around the gym.  I couldn't.  Some bendy people make great gymnasts but not me.  My body doesn't hold together and move the right way and I could never do it.  Poor Mr. Lunsford could never understand.  I know that he thought I wasn't putting in effort.  I wish that now I could go back and tell him the truth of it.

I had horrible headaches all of my life but we didn't know that was anything more than just that.  Headaches.  They called them migraines.  They blamed them on inheritance (my dad has them).  They put me in glasses for the mild farsightedness and astigmatism and said it would help.  That's all that anyone did for headaches when I was a kid.

When I was a teenager I began experiencing hypoglycemia.  My blood sugar would drop severely.  I didn't eat too well so I'm sure that contributed but much, much later in life I learned that I my adrenal glands don't work correctly and low blood sugar is one of the signs of that.  We didn't know all of this, then.  I remember a doctor telling me once that its just low blood sugar.  When I feel it coming on to eat a protein and carb combo snack. He also told me that it would switch and become diabetes later in life.  He was wrong.

As I moved through my 20's, I started to become more symptomatic, lived with more pain.  I wasn't diagnosed with EDS until my mid-30's, after my daughter.

So, yeah, many people had no idea that anything was wrong with me, including me!  But, it was.  I was born this way, as were most of my children.  We may look normal, we may act normal sometimes.  That's why they call so many chronic disorders an "invisible illness". 

The Hardest Places to Live in America???

Tonight, my entry is not about chronic illness, EDS or any of the normal things.  Tonight, I want to talk about home.

I read a news story about some of the most difficult places to live in the United States.  My small corner of Kentucky was on that list.  They considered the median income, the life expectancy and unemployment rates.  So, I'm going to talk about this.  After all, who is a better resource for the truth than someone who has lived here for 37 years, someone who has written many community assessments in grad school about this region?  Many people in other parts of the world do not understand the culture, the lifestyle, here.  They do not understand how Appalachia has been suppressed, deprived and forgotten. 

First, lets talk about the income.  Now, the median income in Jackson county, Kentucky varies, according to the source that you use.  According to the Census Bureau, the median income is about $22,000.  Lets give you something to compare that to.  The median income in New York City is $51,000.  Our average income is low because there are few employment opportunities.  In Jackson county there are a couple of very small industrial settings, and the occasional gas station and restaurant.  Because of the isolation of these mountain towns, industry does not settle here.  If they do settle, there is no competition so they can keep wages at or near minimum wage.  Here you have the cause of low median income AND high unemployment rates.

If the median income is that low, then of course the life expectancy is lower.  The people in this area are not as healthy.  If you are budgeting $22,000 to pay your bills, your mortgage, insurance, medical bills, etc, then how do you afford healthy food and proper medical care? Our utilities are not cheaper, our medical bills are not cheaper and our insurance is actually higher!  Food is not cheaper here.  Some things are even higher because of location.  So, lets say you have a family to feed on an extreme budget.  A bag of oranges is $7 and a bag of chips is $2, a gallon of 1% milk is $3.50 and a gallon of kool aid is just over $1.  Your ultimate priority is to ensure your children have food and drinks.  You don't want them to be hungry.  The fruit and milk would cost just over $10.  The chips and kool aid are just over $3.  What choice will you make?  Buying the unhealthy choice means that you're budget will stretch a little farther to cover a little more food.  A poor parent can't worry about the long term consequences.  They live in the Now.

These same people may have a more difficult time finding ways to exercise.  There are no gyms, no walking tracks, no YMCA.  You find a way to work out at home, usually.  This is more difficult.  Even walking is a difficult option.  Many of us live on single lane country roads.  You may be taking a huge risk walking out those.

Health care....if you can't afford groceries, you probably can't afford doctor's bills.  Many have no insurance.  I don't care what the Affordable Care Act says.  It is not an affordable option for these people.  Many, many times I cared for patients in the hospital who were admitted and readmitted because they had no way to pay for the medications that they needed to remain healthy and functional.

I could write a book about the problems the people of Appalachia face.  It is a difficult place to live.  But, most of us don't know that.  This is home, this is the life that we know.  We are a hardy people.  And, let me tell you, there is plenty that this story doesn't tell you.  It doesn't tell you about the clean fresh air that we have here.  It doesn't tell you about the open fields, the beautiful forests, the lakes and rivers.  It doesn't tell you about families that stick together, friends that sit on front porches or neighbors that check on each other. This story paints the typical portrait of Eastern Kentucky.  I've been a few places and, let me say, no where is like Jackson county.  This is home.  So, maybe, instead of trashing and bashing our area, people could take a little time to understand it.  And, maybe something as big as the New York Times could help find ways to help, to spotlight us and our plight, not stereotype us.

Charity starts at home

I've not posted anything in a month but tonight I'm thinking about something.

Fundraising.

 

That has been on my mind.  I don't know what its like for other people with other chronic diseases ad won't pretend to.  I only know what its like for us, the EDSers, the chiarians.  There is an order to the things that happen to us.  We get sick.  We search for years while doctors tell us we aren't sick, we are fine, we are misdiagnosed.  Finally, we find that one doctor who puts the puzzle together and sets us on the path to more diagnosis.  We learn that we have a collection of rare disorders.  We go through denial, anger, grief, more anger and sometimes, if we are lucky, acceptance.  Then we learn that there is no cure, there are only medicines and surgeries to repair the damage to our bodies.  Next, we begin the hunt for a neurosurgeon that knows what to do to our broken spine, skull, brain and nervous system.  We finally find others like us and connect and learn that there are only a handful of surgeons in the world that know what to do to us.  Going to the right surgeon, one who understands our complications, can mean the difference between life and death.

 

 

So far, so good, right?  We know what we have, we know what we need to repair our bodies and we've found the doctors that know how to save us.  Now for the problem.  These doctors are most often hundreds, if not thousands, of miles away from us.  Seeing them means we need to pay travel expenses, motels and food.  We need specialized tests, sometimes.  Then comes the surgeries.  Often our miracle doctors are not contracted with insurance.  This means that we don't get to roll in for surgery and pay a deductible or a copay.  We usually have to pay deposits in the thousands.  Its worth it to live, right? But, how do you pay so many expenses and deposits if your sick and can't work?

 

This is where fundraising comes in.  This is the biggest obstacle for most of us.  When you've lost so much, your health, your income, your independence, how do you let go of your pride as well?  Its so hard.  We were workers, achievers, go getters.  Now, we are beggars.  This was nearly my breaking point.  I was always proud of my strength, my independence.  For each surgery I have had, though, I've had to ask for and accept help.  It kills a piece of you, eventually.  But, its better than letting the disease kill all of you.  Sometimes a very kind person hosts a fundraiser for you just as someone did for me last year.  Sometimes we fundraise online (see my link in the top right corner).  Sometimes some angels out there leave a check in your mail.  We learn to swallow our useless pride and say thank you to these great people. 

 

How much is too much, though?  I have had three surgeries and  many, many trips to MD to see my surgeon.  I still need another surgery if he will do it.  I still need at least one or two more trips to MD.  I can't drain my friends, though.  I can't work, either.  So now what?  Let this take me or beg some more and deal with my suffering soul later? 

 

This, my friends, is what its like to be us.  We want to live and will ultimately do whatever it takes.   When you give money to charity, think of those who might not live without it.  And think locally if you get the chance.  And know that those like us, those who know what its like to try so hard, will often pay it back or pay it forward.  Just saying....                                                                                                                                                                                                                                                                                                          

A different kind of Mother's Day

All of our lives we are shown perfect mothers.  I grew up watching the Brady Bunch, then Growing Pains and Wonder Years.  All of these had different kinds of moms but they were all perfect.  They were self sacrificing women, full of infinite wisdom and they made all of the right choices for their children.  Their homes were spotless, their meals were on time.  They balanced everything and still had time to do their hair and makeup.  More than anything, I wanted to be one of those moms.  I wanted to be perfect.  I wanted to give them everything, to do things right, to have healthy meals and no dust bunnies.  But, reality doesn't work like that.

Today, I am thinking of all of the time that I lost with my kids by trying to be perfect.  I had my kids while I was very young and very broke.  I tried to spend time at home, cooking good meals, cleaning up after them, watching episodes of Barney and singing ABC songs.  But, we were poor and I felt like they deserved so much more.

While they were all tiny, I started college.  I spent a lot of hours in class and at home doing homework. We ate a lot of pizza and my house was not clean.  I started working weekends so that I could pay a babysitter during the week.  There was no way around that.  I was sure I was doing the right thing.  We'd all sacrifice a little time but they'd have a better life.  So, it took me forever but I eventually got that nursing degree.  I thought that I'd shown my kids how important an education was, how they can never quit.

Then, we started the next stage.  I was a hospital nurse.  This means that holidays and weekends don't exist.  I worked all that I could so that they had better clothes, better shoes, more things.  They had vacations, trips to the theatre, dinner out once or twice a week.  These were things they couldn't have had before.  But, the sacrifice is that I worked holidays, some birthdays, lots of weekends.  And, again, I thought it was the best thing.  It meant that I'd be able to help them more as teenagers and adults.  I'd be able to buy cars, help with college, loan money when they moved out. 

The world has a way of flipping everything over, though.  A year or so ago, I got sick.  And I got worse and worse.  Eventually, I had to stop working.  My treatments and surgeries have drained all of those resources that I was saving for my kids.  Now, I am home with my kids.  I don't miss holidays and birthdays anymore.  But, I'm broke again.  And I'm not able to do much.  They have to watch me suffer, watch me change.  They stay behind while I'm in the hospital. They wait with me to see what's next. 

So, I'm looking back at all of the good intentions that I had but all of the time I lost.  It doesn't mean that I did anything wrong.  Or that I did it right.  I'm not sure.  I guess there is an in between there.  I'd give anything, now, though, if I could have those missed days back.  I'd give anything to remember, now, how it felt to have my babies sleep in my lap.  I wish that I could remember a time when we weren't rushed to the next moment.  And, I know its not just me.  I guess that this is how the world is now.  We are the new Carol Brady's. I can look back, though, and tell you to hold on tighter.  Take more pictures.  Let your babies sleep with you a little longer.  Don't push yourself or them so hard.  In the end, it doesn't make everything better.  And all of that time can never be reclaimed.

Happy Mother's Day to all of you mothers out there, no matter what kind you are.  Loving them is the important part in the end.  Make sure that they know it, no matter how you do it.

Drain my Brain, please!

One of the complications that many of us EDSers/chiarians deal with is high intracranial pressure.  The cerebral spinal fluid accumulates in our brain, builds up and puts too much pressure around brain tissue.  There are different treatments, depending on the cause.  For some, having surgery to correct whatever is blocking the flow will lower the pressures.  For some, medications such as diuretics will lower the pressures.  And, for some, shunts are inserted to drain the excess fluid and keep the pressures down.

I have high intracranial pressures, also known as intracranial hypertension (IH).  I have too much fluid circulating in my brain and its making me more sick.  I take 2 different medications, Lasix and Diamox, for this.  There are plenty of risks to both of these meds.  And, there are side effects that come with Diamox, including neuropathy, that drive me crazy.  But, I'd rather have neuropathy in all of my extremeties than deal with high pressures.  Why?  Let me tell you what it feels like when the pressure is up.

Have you ever had a migraine?  One of those miserable headaches where you feel every heart beat and want to puke and just need a dark room?  That's sorta what this feels like to me.  I get a headache.  It starts like a migraine.  But, then it grows.  My entire head pulses.  It feels like something is pressing against my skull, my face, from the inside.  Eventually, I have trouble holding my eyes open.  I can't stand to eat and I want to puke but know that would make me worse.  I have a lot of trouble thinking clearly.  My reflexes slowly leave.  As it gets worse, this "migraine" moves through my body.  Everything else starts to behave a little bit wrong.  My heart and vital signs are affected, I breath much deeper, I develop tremors and twitches.  It starts to feel like I really am dying.  Or, hurts bad enough that I want to.  I've tried everything from migraine meds to pain pills to ice and heat and dark and sleep.  Nothing fixes this pain except one thing....diuretics.  Pills meant to pull the excess fluid from your body.  They move some of that fluid from my body and my pressures come down and I can function again. 

So, when you hear mention of someone's pressure in their head, this is sometimes what they are talking about.  High intracranial pressures, aka intracranial hypertension, aka idiopathic intracranial hypertension, aka pseudotumor cerebri.