Only the best can touch my brain

Why do we travel across the country to have brain surgery?

I have chiari malformation and a list of comorbid conditions such as Ehlers Danlos, intracranial hypertension, mast cell activation disorder, etc.  If I had ONE of these issues, then maybe I wouldn't have to travel for treatment, maybe I could find help here at home. But, each of these conditions feed on each other. One affects the other.  A surgeon may tell me that he can fix my brain, correct the chiari. But, will he understand that having EDS means that if he removes the tiniest bit too much bone that I may develop severe instability between my skull and upper vertebrae and that it may be as deadly or more so than the chiari? Will he understand that I may react badly to all of the normal medications and anesthesia because of the mast cell issues?  Will he be familiar with the fact that we are more prone to have clotting disorders, more likely to stroke with these surgeries? I could go on and on but I won't.  Lets just say that no, the majority of neurosurgeons do not see or accept the entire picture.  But you have to if you want to live.  There are a small handful of surgeons who have put time, hours, years into studying us, learning how to help us.  And some of us are only willing to trust our lives to them.

I found a surgeon who understands me and what I live with. Twelve days ago he decompressed me, cut out the herniated part of brain, made room in my skull. Then he opened the top of my head and inserted a shunt. Then he closed off the failed shunt in my lower back. Twelve days. And I am here to write about it, tell you about it, advocate for all of you like me. I am already healing, thinking more clearly, moving better. Twelve days after a major ten hour brain surgery, I am home and living again. I believe that its because I traveled to one of those surgeons. I traveled from Kentucky to New York to regain my life. And I am here now to tell you all about it.

A New Surgery, a New Me

I am not very good at sharing my feelings, my emotions, but for you all I will try. I’m going to try and give an unedited view of my new life.

 

Healing. It seems like such a simple word. Nerves regenerate, tissue grows, and symptoms ease.  But, it’s not as easy as all of that. There is so much more to it, aspects that we don’t know about, sides that we don’t expect.  Healing, especially when a chronic disease is involved, is a multidimensional process. 

I have been sick in some way as long as I remember. I was so broken that I didn’t even know I was. My pain, my aches, my difficulties were my normal.  After learning that I was sick, that my body was broken, I began the fight for healing.  I fought hard for a long time. I used every resource, any medical knowledge I had, and any strings that I could pull.   And still it was a heartbreaking and difficult journey.

Last week I finally had the “big” surgery, the major one that I have waited so long for. Last week I had brain surgery. I had some pieces reshaped, some cut out, some added. My head is now different. It is shaped different, it has spare parts.  And it is bald.  I have had some adjustments to make

First, I watch every day to see what is new and different. My new normal amazes me. I just can’t get past how this feels. I’m not sick, my head is better, the physical healing has begun.  It’s so much to wrap our mind around. There are the ecstatic moments when I realize that I have no pain. There are the somber moments when I see that some other aspect hasn’t healed yet.  This is an emotional roller coaster.

And then comes the support, and sometimes lack of. I felt like people weren’t listening, didn’t believe that I was sick. I have an invisible illness. On the outside I looked normal. And then I had surgery. I came out with three large cuts into my head, down my neck and two large ones across my abdomen. My invisible illness could finally be seen. This is the time to teach and to fight and to show the world what we live with. I am tired, recovering, but I have to use this time well.  Suddenly, I have friends and family calling, texting, sending me messages and well wishes. I’m hearing from everyone that I know, people that I’ve not heard from in so long. This is a new and unexpected turn in everything.

And now I look different, too. I wasn’t a very vain woman, not especially pretty. But, I am a woman. I went to sleep looking like me, with long red curls that everyone touched and talked about. I woke up with no hair. None. It’s OK. It’s worth the loss for my health.  But, I see myself differently and I wonder how others see me. I look in a mirror and that woman isn’t me. I walk through a store and am self-conscious. I want to dress up and go celebrate but what do I do with my head? It’s such a silly thing but its one thing that we deal with, still.

And I’m tired. Just tired. I want to jump in and be a mother again, take care of my family. I want to be a wife, wanted and important. I want people to see me as they did before but I know they won’t. That’s part of this.  I am not the same person. Why would they see me as her?

And, for whatever reason, I want to share my store, the hard and the easy parts. I want my friends, my loves, others like me to know that they aren’t alone. I want people to know that there are more of us that they can reach out to, those of us that can help, lend a shoulder and just understand.  In that way, maybe I can make an even greater difference for someone else. Maybe I can bring something good from my story.

An overdue update...

I've not posted in quite a while! It doesn't mean that I'm well or healed, that this journey is over.  It just means that life has been busy, pulling me other directions.  I find a lot of things to keep me busy.  I've been putting a lot of time into my daughter who is very sick.  There is always something else to be done, someone else needing us.

Where are we on this journey?  I've not had any more procedures, any more visits out of state, since last summer.  I am very much due a follow up with a neurosurgeon, though.  So many things have been worked on, some are better.  But, so many more are falling apart.
Currently, I believe that my biggest problems are in my neck and head.  I have instability in my upper vertebra, between c1 and c2 (also called atlantoaxial instability).  These are the vertebra that support the skull and the motion when it turns.  Like so much of our EDS bodies, our vertebra slip apart and/or overextend.  When mine was last measured, there had been some significant change.  Now, I am becoming much more symptomatic.  So, what are the symptoms of upper instability?  They are vertigo, dizziness,  neck pain, balance problems, blurred vision, pain in shoulders, poor gait when walking, and more.  I have all of these thing and the more that I mention. :D 
Now, to accompany my instability, I have progressive chiari and intracranial hypertension symptoms.  My headaches have increased in frequency and severity.  I have much more pain in the back of my neck and head.  I have high pressure when I wake every morning along with difficulty with my vision.  I have developed spots in my peripheral vision.  I have near constant nausea that is caused by neurological damage.  I have tremors and occasional contractions in my hands.  I have, I have, I have....

I still need to see a neurosurgeon but there are other things that come first for me right now.  I am thankful that I'm not confined to a bed, that I can still enjoy my family.  I know that there are bad days, hard ones, sick days, dark days.  But, you'd be surprised how tough we Zebras can be. 

A December Update

I just realized that I've not posted an update in quite a while. 
Where am I now on this long journey?

Fall is here and Winter is around the corner.  As the temperatures dropped, my ICP (the pressure of fluid in my brain) climbed up.  I became more sick, experienced increased symptoms.  The vertigo became worse, the headaches are constant, the nausea is nearly constant, my memory is like swiss cheese, full of holes, and my vision is blurry and often doubled.  So, I started taking Diamox again.  It is a mild diuretic that is used to pull fluid from the body and it helps a bit with the symptoms.  However, it only helps a bit, not quite enough.  So, whenever I get around to calling my doctor's nurse, they are doubling my dose.  Yay for yucky side effects.

I've been trying to find a neurosurgeon that is at least within my home state who is not afraid of me and willing to take over my care.  Traveling has become too much.  I've not had much luck so far.  I found one who will care for me in a "shunt emergency" but that's it.  Its an uphill battle to get my primary physician to make referrals so that I can check out other doctors.  I'm not sure why.  It doesn't cost them anything.  And, when they finally give in, I have to wait months for that appointment.  My physician refuses to help me schedule appointments with more than one NS at a time.  They insist that I have to see one before they will help me schedule with the next.  Between the wait time for them to make the referral and the wait time to get in and see the surgeon, I may manage to see three in a year.  I'm only interviewing them, not asking for surgery!  Its ridiculous.
In the meantime, I do have a follow up appointment with my doctor in Maryland in February.  I'm not sure how I'll manage it, yet.  I've drained all of our resources this year with my surgeries, trips, emergencies, medicines, etc.  I no longer have backup plans.  I'll save what I can but it will pretty much take a miracle to fund another trip.

At this point, I don't really have new symptoms, just an increase in some of the old ones.  Of course, some problems were resolved with surgeries.  I'm guessing, based on my symptoms and limited knowledge, that I still need a little repair work.  We'll cross that bridge as we come to it.

For now, that's where I am.  There is nothing more exciting to report. 

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".