Good days, Bad days and everything in between...Living with SPS

It's 3:17am as I start to write this blog post.

I made coffee, took my meds, and didn't tear up as I got out of my chair this morning.  My recliner has become my new "bed".  I miss sleeping next to my husband every night, but it's just not possible anymore.

My husband suggested we get "his and hers" recliners so we can both stay out in the Living Room together.  We've done it before, but the last time we camped out together, he had just gone thru and emergency back surgery due to a ruptured disc, and I was 9 months pregnant with our youngest, Mello.

Back then, it was a full sized futon that we squeezed on to with a needy German Shepherd.

After Mello was born, we moved a pack and play with a bassinet insert right next to the futon, and the Living area became a bedroom. There, I could take care of the middle of the night feedings, and keep my husbands medicine schedule covered.  Our house was less than 700 Square feet!  (I actually miss that.  I could vacuum the whole house from one outlet!!!)

That was 2 1/2 years ago.  Unreal.

Since then, we have lived in 3 different places, survived homelessness, and realized that FAMILY is a bond that isn't always made with bloodlines.  So, I'm no longer a believer in "Blood is thicker than Water"...Sorry!

People have asked about Stiff Person Syndrome almost every day since I was diagnosed.  I research EVERY DAY, and I can't find ONE single "foundation" or "association" to direct them to.  Just Google!

So, what I will tell you is what I deal with personally.  If you don't want to know, don't read this.  This is a very REAL account of parts of typical days, weeks and months for me, and tons of people with chronic illness.

I usually don't sugar coat much, but you will see some parts of this "lightened" a bit, in case a younger person reads this.

LIVING WITH STIFF PERSON SYNDROME

Like anything else, even though I was just diagnosed, I have had this disease for much longer than the month or so that I have carried the title.

When you have symptom after symptom, and already have a  chronic illness, you think of any new "symptom" as just another side effect of what you already have, or medication induced problems, or even stress.

That's exactly what I've done.

I have had a Pain Specialist for my diagnosis of Occipital Neuralgia for over a year now.  About 6 months ago, I explained to my doctor that I have had problems with muscle spasms for years, but that they were gradually getting worse.  Even the doctor thought my back spasms were related to everything else as well.

So, as I grew more uncomfortable, the spasm in my back actually started to bulge.  It looked like someone had taken a paper towel roll and slid it under my skin, parallel to my spine on the right side.  As it got worse, I wasn't able to stand up straight.  I stayed hunched over like a 90 year old woman, and limped around.

It was awful.  I ended up having to ASK for an MRI, because the pain had become unbearable, and I was having trouble getting around my own house.  I couldn't pick up the kids, put dishes away in cabinets above my head...I was frustrated.  I was convinced I had herniated a disc, or pinched a nerve.

I have become somewhat of an "expert" on herniated discs, because my husband had one for years.  After 2 years of conservative treatment, ie; PT, epidural injections, steroids, he RUPTURED his disc. 

How?  Well, we were in an elevator, it went down, and right before the doors opened, it dropped about 3 inches.  My husband went into shock the pain was so bad, and he was in Emergency Surgery 2 days later for a Micro-Discectomy. 

The MRI (L and T spine) came back with just evidence of severe spasms, and 2 herniated discs. (This was ODD to me, because I had no "trauma" in order to herniate discs. 

My C-Spine MRI (my neck) was the most telling.  The doctor said he had "never seen a neck so straight".  Apparently thats a BAD thing.  The spasms went from my neck to my hips, and hurt like hell!

So, I was put on a regular regimen of muscle relaxers, and went in for a few epidural procedures to relieve some of the rigidity.  That helped a little.  I was at least able to stand up straighter!

(need to finish this later....meds are kicking in...)




                                                                                                             

Headache HELL: Part 2 - The people who are supposed to help, that DON'T!

Before I start, I should explain something.

If you are sick, you trust your doctor and their office to make the right decisions for you.  To call in the right medication to the pharmacy, to know what medications you are on, what will interact with your medications, things like that.

Odds are, since a normal person only goes to a doctor once or twice a year, they can't screw much up.

Things are different for people like me.

I see a Pain Specialist 3 times a month.
I am in the Infusion Center every week, for 2 days in a row, 6-8 hours a day.
I have 2-3 days to recover from my infusions, due to the fact that I'm allergic to them. (Aseptic Meningitis)
I have to see my Neurologist 2 times a month.
Then, add in procedures for my back and neck, visits to my Regular Doctor, and time spent in the Pharmacy, and guess what?

It feels like I have a full time JOB.

The point I am attempting to make is, I think I had more "down time" when I ran a small business!  Being chronically ill is exhausting.

So, that point made, imagine spending your entire day in pain, just leaving the doctor's/pharmacy/hospital, picking up your kids from school, and when you check the mail, you get a notice that the ONLY pain medication that actually works has been denied by your insurance company.

No biggie, right?   I should just be able to call the doctor's office, tell them what happened, and they will send over some magical paper that explains that I NEED this certain medication, and poof, everything will be fine in a week or so.

In a perfect world, that would be the case.

Here's how the process of getting an "expensive" medicine approved is supposed to go:

1. Take the medication to the pharmacy, and they tell you that your insurance company has denied it.
2. The pharmacy is SUPPOSED to fax a letter to your doctor's office, informing them of the denial, and they can either give you your prescription back, or keep it until the medication is approved.
3. The doctor's office SHOULD replace your prescription with something that IS covered, and then start the "Prior Authorization" (PA) process for the medication you were originally prescribed.. 
4. Within 5-7 days, you get a call from the pharmacy letting you know that your prescription is ready.

That's the "poof" I was talking about.  Seems easy, right?

The problem I have been having is, depending on people to do their job.

Okay, let me get back to the headache, and try to explain how all of this comes into play.

Where was I?

Ah, the RHIZOTOMY.

After 2 cervical facet injections, one of which nearly scared me away from the pain specialist altogether, I was ready to fix this headache.

It had been an entire year of pain.  Unreal.

There is no long descriptive story about the Rhizotomy.  I was heavily sedated, and don't remember anything until I came out of the room.

I was told that I would know the SECOND that I was done whether or not it worked.  

I thought I knew what to expect, but yet again, as seems to be the case when I get my hopes up, I was wrong.  Again.

When I came out of the room after the Rhizotomy, I could still feel my headache pain.  I was worried sick, because I actually had a new pain.

The right side of my head was now ON FIRE.  A horrible burning sensation had stemmed from the procedure.

I panicked.  Not a little bit, either.

I started to cry, uncontrollably, in the recovery chair.  This drew the attention of the anesthesiologist, who came to check on me.  When I explained that I was still in pain, and thought that I was supposed to feel better immediately, he looked confused.  He sent the Medical Assistant over to see me.

I got the "You have to give it time..."  and when I asked how MUCH time???  The answer I got sent me into a downward spiral.  "4-6 weeks."

My poor husband.  I was discharged a weeping mess.  AGAIN.

This time, he didn't wait for me to calm down to ask me what was wrong.  He could tell I was still in pain, and got my verification.  See, he was under the same impression I was.  That once the procedure was done, I would absolutely KNOW that it worked.

So, he did what any loving husband would do.  He wanted someones head on a platter.

He asked for the doctor, or anyone that could tell him what went wrong.  He wanted an explanation, anything that would explain my pain.  He got me comfortable in the car, turned on the AC, and paced back and forth while the office staff got more and more uncomfortable.  When they realized he wasn't leaving until SOMEONE talked to him, they sent out the Medical Assistant.

The MA explained that in "most cases" the nerves react immediately, and a patient can tell that the procedure worked.  In other cases, it can take 4-6 weeks to work.  She folded and rung her hands, as I crawled out of the parked car.  I had heard what she said, and wasn't happy.

I apologized for being so upset, and told her that I had looked forward to this day for so long, because I thought I would be out of pain.  That was all I wanted.  I had gotten my hopes up, and really shouldn't have.

My husband went into the office, made another follow up, and we left.

An entire year had gone by, with no pain relief.

So, I don't mind saying this now, since I survived it, but I got depressed.  Pretty badly.

My husband packed me in the car, and we disappeared to Assateague Island for a day.

Alright, earlier I mentioned the medicine.  Here's the HELL part I'm talking about.

At any doctor's office, there is a staff member, (presumably more than one) that is in charge of PA's (Prior Authorizations), referrals to other doctors, and getting procedures approved by your insurance company.

At my neurologist's office, I have no issue with this.  The get me where I need to be, when I need to get there.

My problems started back in July.  Since I have to be on pain medication 24 hours a day, I have a Pain Specialist for  reason.  This is what they do for a living.  I was hoping for procedures to lessen or eliminate my pain, with the goal to come off of every pain medication I am on, so my body didn't develop a physical dependence. 

Since the procedures didn't work, I asked to go on "Long Acting" pain meds instead of short acting  (ie. Percocet, Norco, Morphine, etc.)  I hate having Percocet (oxycodone) laying around my house with kids around!

So, I was written a scrip for long actings, and a small scrip of short actings for breakthrough pain. I picked it up at the pharmacy with no issue.

Since it has to be titrated upwards, every month the prescription has to be upped, until a good level of pain relief is achieved.

Well, the next month, my prescription was denied.  With the cost being over $900 for the month, I couldn't afford to just pay cash for it, so they faxed a denial letter over to the Doc, who is supposed to send a PA over to the Insurance company, and within a week, I should have my scrip.

This DID NOT happen.  The pharmacy did their job, but when I called the doctors office, they explained to me that they had not sent over the Prior Authorization form yet.

Here's the timeline of how this went:

July 18- Received the prescription for LONG ACTING pain medicine.
(I was denied the same day, and contacted the Pain Doc's Office)

July 21-Called the PS (pain specialist) to confirm that they had sent over the PA form.
(I had to leave a message, because NO ONE gets to talk to the girl in charge)

August 10th- Received a denial letter in the mail from my insurance company, explaining that I could "APPEAL" the decision.  I opted for and "EXPEDITED APPEAL" which would give me an answer in 72 hours....IF, and only IF the doctor's office got the right paperwork faxed over to my "APPEALS COORDINATOR" 

August 18th- Made a call to the Appeals Coordinator, and she had received NOTHING from my doctor's office.  Nothing. 

***At this point, it had been a month since I was written the prescription.  I was in horrible pain, and taking MUCH less medication than I should have for 2 reasons.  1) I hate pain medication. 2) I had to spread 2 weeks of medication out over a MONTH.

So, pause on the timeline for a minute.  I am doing all of this fighting with the insurance company, and don't have any information as the MIDDLE MAN in the transaction. 

So, at my next appointment, I involve the doctor.  I show him my notes...( I am an AVID note taker) Names, dates, phone calls, etc. 

I explain that I don't have the fight in me when I am in THIS MUCH PAIN to do all this work.  It's bordering ridiculous. 

So, I thought to myself, "How do I get OFF of the pain meds altogether, so I don't have to do this anymore?"

FIX THE UNDERLYING PROBLEM!

So, my husband and I discuss other options for this headache.

How do we just MAKE THE PAIN GO AWAY?  That's all I want.  No pills, no doctors, just get rid of the headache. 

It's horrible!  I spend my days sensitive to light, I can't brush my hair, and by now, I'm avoiding taking a shower to wash my hair.  I used to LOVE my hair.  It has now reached my waist, and is a dreaded up mess, because I can't take care of it at all. 

I don't even remember now what month it was, but in the middle of all of this, I lost sight in my right eye.

Yup, JUST WENT BLIND.  Scared to death, I was sent to the infusion center immediately, to dose me full of steroids, in hopes that it was just inflammation. 

Okay back to the timeline:

Now Blind in my right eye, dealing with a full blown migraine 24 hours a day, unable to sleep in my bed, and I can't even have the window down in the car, because the wind MOVES my hair and puts me in excruciating pain....

August 20th: We decide to find a surgeon that will cut the nerve. 

*Of course, the one Doctor that does this, DOESN'T TAKE MY INSURANCE*

So, we look farther, and decide to get me into Johns Hopkin's Neurosurgery department, and pray they can do it.  I live 20 minutes from Hopkin's, so this should be EASY, right????

NOPE.  The same "Prior Authorization" girl in the office is in charge of this department as well.

Okay, so, I will skip over the bad parts.  I will fill them in later, but I am getting ANGRY just thinking about it. It's been 4 months.

Time on Deck: November 15th, 9:18am.  - I still don't have an appointment with Hopkin's. 

FOR MY HEADACHE, ALL I NEED IS *ONE* SURGEON TO CUT A NERVE.

I know it's a little more involved than that, but dammit, with 2 other diseases, my life would be SO much better if I could just get one guy to cut a hole near my spinal cord, and destroy this nasty little nerve that is making my life hell. 

So, I will call every day, until I have an appointment.

Oh, and I've decided to "name" the Occipital Nerve that has gone haywire, after the girl that hasn't done her job.  Yup.  I'm that pissed off.  I won't let you know her name just yet, but if I find out that she could have done this MUCH sooner, I will call them all out on the carpet.






 

The 5 stages of GRIEF, are not necessarily done in order.

I have been working on Part 2 of my headache post, but was rudely interrupted by this new diagnosis.

I actually think I might have flip-flopped through the stages of grief, sometimes having more than one stage visit me a day.

Okay, so I have had to go far beyond Google for the answers I needed to what exactly "Stiff Person Syndrome" is.

Over the years, I have collected Medical Journals, and have multiple paid subscriptions to all of those "scientific study" websites that only give you abstracts.  (unless you pay)

I started doing this when, 5 years ago, I was told I had some "female disorder" that ended up being Chronic Appendicitis!  I ended up diagnosing myself after almost 2 years, and 19 doctors.  I had an exploratory abdominal laparoscopy, where the doctor found Stage 4 Endometriosis.  It was ablated, and I was sent home to recover.  I never did.  More doctors later, I had another one, more endometriomas were found, and removed.  I tried to explain to these doctors that I was still in pain, but no one would listen.

So, I did what anyone with my personality would do.  I made a list, and forced my regular doctor to perform every test I could think of to rule stuff out.

Finally, I drew up a legal paper, explaining that I would not sue, if a surgeon would just open me up and LOOK at my appendix.  That was on a Tuesday.  I took it to the head surgeon at BWMC, a hospital that didn't have any part of my previous surgeries, and I was in surgery AGAIN 2 days later.

I now have 9 small scars in my abdomen because of this.

When I was wheeled out of surgery, I was groggy, in pain, and the surgeon was actually waiting for me.

He looked sad, and apologetic.  He apologized for every other doctor that didn't take the time to explore my intestines.  He said that sometimes they don't, because there are miles of them.  I was a lucky girl, because my appendix was moments from rupture.  He was surprised I wasn't septic, and the reason he looked sad, was he knew that if it had ruptured, I would have ignored the severe pain, popped a couple of pills, and died.  (I will leave out the awful part of not knowing that stopping pain meds would make me sick...)

So, anyway, this is how I learned to become my own advocate.  I've said it before, and I will say it again.

They call it the PRACTICE of Medicine for a reason.  No one is perfect.  I understand that.

So, this brings me back to my original point, I have medical resources that most people don't have.

Sometimes, I wish I WAS limited to Google! 

When I was first told by my neurologist I had Stiff Person Syndrome, I didn't flinch.  It really doesn't sound that scary. I just figured it was why I couldn't stand up straight, and why I kept getting muscle spasms without any trauma.

It actually took me over a week to look into it.  When you have an auto-immune disease, it tends to spawn others, so, I wasn't that worried.

Then, when I went to my regular Pain Specialist appointment, he saw the new diagnosis, and looked at me like he wanted to hug me.  He talked about some pump for Bacolfen, once we titrated my meds to a certain level.  THAT was when I had questions.  He said he has only met one other person with it, but it was bad.

Not many DOCTORS even know what the hell it is...So, the research started.

Things started to make sense.

He's the "in a nutshell version".  And no, I am not writing this to make you feel sorry for me.  I want to educate people, and fight the odds.  Even though the odds are stacked against me.

 Stiff Person Syndrome is a VERY RARE disease.  Approximately one person per million is diagnosed, and it's not very widely understood.  Not many studies can be done, because there aren't enough of us to go around!

The good, the bad and the ugly;

•  The only "GOOD" I have found so far, is that I was already on IVIG for the CIDP. 
• Sudden Stimuli, (Loud noises, lights, doors slamming, honking) cause horrible spasms throughout my entire body. (IVIG helps lessen this)
• Muscle spasms aren't your typical spasms.  My entire body is involved.  Imagine doing a sit-up and staying in the "up" position.
• I have to take Valium or Baclofen daily, just to uncurl out of bed in the morning.
• I have to try to explain to a 2 year old that screaming actually HURTS Mommy.  Not easy.
• I can attempt to get the disease into a "plateau" phase, but have to get up to 80 mg a day of Bacolfen first.
• I am now stuck on IVIG for the rest of my life.  No remission. No cure. No pain relief.
• I will eventually have spasms so bad that the muscles will rupture, and need a surgical repair.
• The spasms will eventually try to break my bones, including my ribcage and spine.
• My life expectancy is now 15 years from date of diagnosis.

Now, that last one- There isn't a ton of "Google" research that explains it.  The studies that have been done by universities are grim.  The description of some of these poor people make me cry when I read them, because only 25% ever finished the 10 year study.  Half of the people died accidentally, because they stopped taking the medication.  That's a big NO-NO.  Once I am up to the amount needed, I can NEVER EVER stop taking it, or my body will shut down systematically, VERY quickly.

Luckily, I am the stubborn Bitch that I am.  (Yes, with a capital "B")  I will never stop fighting, even though I have gotten so sad that I considered it.

The way I see it, if I am one in a million, I am already an "odds breaker".  Why?

• I am a 31 year old female, who has ALWAYS done what I was told I COULDN'T.
• I worked in Construction my entire adult life, because someone told me I couldn't.
• I became a singer, even though I thought I couldn't.
• I was able to get back up and walk after being completely paralyzed.
• I had 2 daughters, even though multiple doctors told me I couldn't.
• I married the man of my dreams, even though LOTS of people told me I couldn't.
• I have managed to stay HAPPILY married to said man after being told I couldn't.
• I became a good mother, even thought I thought I couldn't.   

And there are SO MANY MORE!

I don't believe that I will only live 5-15 years.  I REFUSE to believe it. I am Penni Lynne.  If you know me, that's enough of a reason.  I have always been a tough bitch, and I will not let anything kill me.  I still have something left for this world.  I just don't know what it is yet.

My BUCKET LIST is still being written.  I have actually already crossed off a few things!

• I sang on stage...(Thanks to Doug, now I actually have my own fans!) (Oh, and I ROCK THE SHIT out of "Bobby McGee")
• I went skydiving...(Thanks to my friend Bill)

I still need to do things though, and now I will work on them a little faster.

• I WILL have full sleeves of tattoos.  (Sorry to my parents, but dammit, I love them.)
• I WILL record an entire album of my own songs.
• I WILL go back to school to become a nurse, or Patient Advocate.
• I WILL watch both of my daughters grow up and graduate High School.
• I WILL eliminate all of the negative shit heads in my life.  (even the ones related to me)
• I WILL help people every day.  
• I WILL teach people to help themselves.
• I WILL buy a house one day.  (Just have to figure out how to get a mortgage on SSI!)
• 
  
• I WILL have a 50th Wedding anniversary. (You just watch me....)

The list goes on.  I add to it every day, and Doug and I both are going to go to school together.

Now, to the 5 stages of Grief that I started this post with.

1.) Denial and Isolation;

 Yup, I already did that.  I was diagnosed a month ago, and I still want to deny that I am as sick as they say, but when I end up on the bathroom floor, clutching for a bottle of muscle relaxers, it it a pretty good reminder that I am sick.

One thing I am VERY guilty of; Hiding my symptoms.  Even from my husband.  Especially my kids.  Luckily, my husband is sweet enough to let me think I'm getting away with it...But I compare it to when people have the flu, and have to vomit, they don't want anyone to see them like that!  So, when I feel a spasm come on, I "have to take a shower", or "go to the bathroom",  or just plain old, "Mommy will be back in a minute". 

Right now, my kids are too young to understand the severity of the situation, and I just want them to be kids. My oldest has already seen me in a wheelchair, and it has made her a better person. She treats people with disabilities with respect and courtesy.  Other kids at school make fun of the "Special Needs" kids, and Patience has befriended them all.  She even went as far as to borrow my duct tape to bring to school one day, and made a drink holder for a little girls walker.

I don't let them see the bad stuff though.  I used to have home infusions, and it scared the hell out of her watching a lady shove needles into my arms, so I have them at the hospital, and will continue to do so, until it becomes a pain to get out of the house.

2.) Anger;

Now, this one comes and goes.  It can be pretty destructive as well. I get angry for lots of reasons;

• Why me?  Is this Karma?  
• No one understands my pain, my struggle.
• I can't open the peanut butter jar.
• I'm only 31.
• I can't sing today because of all of the medication I have on board.
• My husband doesn't deserve this.
• I don't want to die young.

and the list goes on...

I am SO LUCKY to have married the wonderful person that is my husband.  He is the POLAR opposite of me, and is an Optimist.  He keeps me grounded, and I am a better person because of him. Honestly, a LOT of people of better people because of him.  When you are around him, you CAN'T be angry.  He makes you want to be more like him.

I hate to admit this, but the reason I keep this blog is to educate, and help other people with my disease...I have HORRIBLE fits of rage.  Sometimes it's the medication ups and downs, and sometimes it's hormonal, and sometimes, I just need to be angry!

I want someone to blame, someone to take it out on, and just want to go back to being the person I was before all of this started.

I used to be a bad bitch.  I could carry 8-10 sticks of pressure treated lumber on one shoulder.  I could paint a room in a day, I could organize multiple crews of employees and always stay on track and under budget on jobs.  I was awesome at sales, and up-selling a job.  I was an incredibly talented painter, and I could make a wall look like glass.  I could build decks, install doors, and lay tile flooring like a beast.

I can't go back to that, ever again.  I can barely hold a pencil now.

3.) Bargaining;

I do this a lot.  If I had just gotten to the doctor sooner, If I had just worn a better respirator while sanding drywall, maybe if I went to church more, if, if, if...

Maybe if I do this, it will go away...I want to change the past, but I can't.

4.) Depression;

This is one of the stages that comes and goes.  I'm not depressed today, but I may be tomorrow.  And when it takes hold, it's hard to shake.  Sometimes it goes hand in hand with anger.  Usually, when I get depressed, I get angry that I am depressed.  Then, when I get angry, I sit and sulk, sometimes for days, which is horrible.  My husband tries everything he can think of, and sometimes it works, sometimes it doesn't.

Sometimes, when it circles back to anger, it can be productive!  I get so angry that I start trying to prove that I CAN do stuff, and my house will get cleaned, or the laundry will get done, or something to that nature!

I am actively searching for a Therapist right now, for both my husband and I, so he can understand better what happens in someone who is chronically ill.  It's hard for him, because he has never had anxiety, or phobias, or any kind of severe depression, so without personal knowledge, you can't explain it.

5.) Acceptance;

I think I am finally here, hence the blog post.  I have accepted the possibility of a pretty rough ride, and am preparing for it the best I can.

I am drafting a will, and my wishes for my kids, and keeping a journal for them, so they know how loved they are.

I am staying on top of my doctors appointments, and not avoiding them anymore, and taking my meds exactly as I should, and not fighting the fact that I have to take them.

I am stocking up on the things I will need when I am unable to walk, so I don't have to fight for them when the time comes.

I will work on my bucket list, get tattoos, not take any shit from bad medical staff or nurses, and live life.

I will not lose this battle without a fight.