Sleep paralysis

I'm usually not one to try to find rational explanations for things that seem paranormal or otherwise, but I cam across a discussion today that shed some light on something that's happened to me a lot. Now, I have a name for it: sleep paralysis. Like other symptoms, I had no idea it was Chiari related. (Clarification: for me it's probably Chiari related. Other people can have it, too.)

So heres the scenario...

I'm lying there in bed and am half awake, half asleep. Only, I can't move. I'm aware of everything that's going on around me but my body is breathing regularly, like I'm asleep, and my eyes are closed. It's a totally terrifying feeling. Sometimes, it will feel like my body is floating up towards the ceiling.

I thought I had just perfected astral projection. But, apparently, it is sleep paralysis.

Here is your link for more information on the topic: http://www.webmd.com/sleep-disorders/guide/sleep-paralysis

So why does this happen? More symptoms having to do with the blocked flow and crowding in the brain. It's amazing what all that stuff is causing.

It's apparently not dangerous or anything, although I am usually hit with a severe headache when I come out of it. Not that the headache is anything new, though.

Second opinion

So I have my second opinion scheduled before I even have my first.

I decided that since Cincinnati was dragging their feet (I finally have an appointment for the CINE MRI but still no date set for the actual appointment) I should go ahead and get my second opinion scheduled. What if I don't like Dr. Tew? What if I don't like what he has to say? This is serious business. I don't want to go on one opinion alone.

So for my second opinion I am not going with a Chiari specialist, just a neurosurgeon who has done decompressions before. Normally, you should do with a specialist. (And I ultimately probably will.) But a second set of eyes wouldn't hurt.

So I scheduled an appointment with a neuroscience center in Huntington, West Virginia for May 30th. That's a long time off, I know, but hopefully I'll have the appointment with Cincinnati before then so the WVa appointment really will be the second opinion. If I had waited to book with them until after I saw the first NS then it might have been August before I got in. 

But he seems smart...

I've had a lot of battles with SIDS in regards to people having inaccurate information and now it seems like I am going to have to battle the same kind of mindset with Chiari. Between the doctors that don't know anything about it and the people who just assume that I can somehow turn the symptoms off and on at will it's very frustrating.

Perhaps this is the most frustrating incidence so far, though...


The odds of one of my children having this are great. We know Iris doesn't have it because she's had a MRI. We don't know about Toby. So then there's Sam...

In telling a friend about my concerns she said, "Oh, I'm sure he's fine. He seems pretty smart."

I thought I had done a pretty good job of educating the people in my life about this matter. Clearly, I failed on at least one level. So, for the record, Chiari does not affect intelligence. It might mess with out memory, our balance, and our ability to hear clearly but it does not affect our intelligence.

Also, what the heck was that supposed to imply-that I don't seem smart? Good grief. What a thing to say to someone. Even if you think it, that's no reason to voice it. 

CINE MRI

I finally got a call from Dr. Tew's office. No appointment scheduled yet, but they are sending me to northern Kentucky next Tuesday to have a CINE MRI to measure the fluid to my brain. At least they're taking some initiative now. Hopefully, once he gets the results from that, it should just be a matter of days to see me. 

Ehlers-Danlos Syndrome: Why My Heart Might Explode

I mentioned in another post that there can be other conditions that go along with Chiari. Well, now I can welcome myself to the world of Ehlers-Danlos Syndrome.

Ehlers-Danlos is a group of connective tissue disorders that can cause a lot of problems. The symptoms are strange. Here are mine:

- Double jointedness (You should see what I can do with my pinkie.)
- Soft skin (I thought it was the Bath & Body Works all this time!)
- Short stature (I am barely 5 feet tall)
- Joint pain (check!)
- Fine hair (forget trying to brush it-the minute I do it's in tangles again)
- Visible veins (I'm pretty stringy on top and in my legs)
- Difficulty healing from wounds and surgeries (I still have cat scratch scars from the 90's)


The funny thing is, I had NO idea any of these were related.

This syndrome also explains some of the problems I had with my hysterectomy. When the surgeon was in there he said that my tissue was in very poor condition and that it tore and disintegrated whenever he tried to clamp it or move it. He thought I might be a vegetarian. (I'm not.) It turns out, it's part of this nonsense.

So, there are a few problems this can cause. For one, healing after surgery becomes an issue. That's probably why it takes me so long to heal and why my surgery scars remain a deep purple for years afterwards. It also means that for the brain surgery I will probably have to use my own tissue instead of that of a cadaver. Which, of course, is perfectly fine by me.

This might account for my joint pain. Then again, it might not. It probably does account for the fact that every time I move my body cracks and creaks like old floorboards.

The pain can be managed and surgeries can help with the ligament problems. (Essentially, my body is literally falling apart.) However, there is a dark side to this.

Vascular Ehlers-Danlos (or, Type IV) is very serious. It affects the heart and other internal organs. More importantly, it can cause the internal organs to rupture. Now, who wants to think about their intestines exploding? Not me. It could explain why I had a placental abruption. Generally speaking, women with the vascular type are not recommended to get pregnant. Oops.

The bad news is that life expectancy for this is not great. It hovers around 48 years of age with 80% of people having life threatening problems before they are 40. Now, I know that realistically I could step out tomorrow and get hit by a car and die. However, having someone actually tell you that you might not live very long is not a very nice thing to hear. There is no "cure" for this and not even any treatment. If the heart  ruptures then there's very little they can do about it.

I'm kind of pissed off about this.

At any rate, it's now off to the cardiologist. 

If Not Surgery

Nobody REALLY wants to have their brain operated on. So what are your options if you don't have the decompression?

Here are some of the things that I have tried:

- Topamax
- Lamictal
- Maxalt (for headaches)
- Gabapentin (for nerve pain)
- Amitriptilyne (for nerve pain)
- Klonopin (when they thought it was anxiety)
- Celexa (when they thought it was depression)
- Physical therapy
- Chiropractor


Some of these worked for a little while. Some didn't work at all. The last dose of PT actually made it worse. From the online world, I have found that some people use Lyrica and have had mixed results.

Basically, you're treating the symptoms. You might be able to find something that helps the headaches. Or, you might be able to find something that helps the nerve pain. If you're having really troublesome symptoms like trouble swallowing, vasovagal syncope, memory loss, or cognitive issues then there doesn't seem to be anything out there that helps. You can't change your fluid flow without surgery.

Decompression surgery does not cure Chiari but it can help manage the symptoms. If you have a syrinx then it might even be necessary. 

My Chiari

This was an entry on my other blog...

After being given the runaround by insurance I was finally able to straighten things out and hopefully will be seeing Dr. Tew at the Mayfield Clinic in Cincinnai. We got all the stuff faxed into him today. He is a true Chiari specialist and apparently knows what he is doing. 

I was also able to get my MRI disk from the hospital. Here are some of the images from it. What you're looking at is the cerebellum, where it's situated at the top of my spinal column. Mine is very thick and herniated. (Meaning that it sinks down lower than it should.) Some people have very long herniations, like 20 or more. 

Mine is not actually that long, but it is thick. It corks me up so that spinal fluid is not able to move like it should. That, in turn, has caused the syrinx (or cyst) lower down in my spinal column. 

The reason that it's important to find a Chiari specialist and not just any old neurosurgeon is because a) you don't just want any old person operating on your skull and spinal column and b) there is a lot of outdated information about Chiari and you need someone who keeps up with it. Some doctors won't bother with the surgery unless the herniation is a certain length. It's not the length that always makes a difference, however, but the width that can cause the spinal fluid flow problem. 

SYMPTOMS:

Chiari symptoms vary. There are several official symptoms that you can find on different websites, but i total there are more than 40 different ones associated with Chiari and even more when you take in the related conditions. 

Although headaches are one of the most common, they're not the only symptom. I have been treated for migraines for around 12 years now and no migraine medication (Maxalt, Imitrex, Excedrin migraine, etc.) helped. Chiari headaches are different from even regular migraines.

Balance is also something that can be affected. Running into walls, having trouble with patterned carpet or tiles, and being able to walk down steps correctly (without falling) are all things that some people with Chiari have trouble with.

Nerve pain is one of the worst symptoms. My pain starts in my neck and slowly radiates down the left side of my body. This is not pain that can be alleviated with a visit to the chiropractor or physical therapist (if you do go to one then make sure they have worked with Chiari before) or something that can be easily massaged away.

Along with nerve pain, you might feel tingly or even numb. I get both and they seem to alternate regularly. Weakness is a HUGE symptom and may or may not affect both sides of your body. (I get it on the left side.)

Lastly, sight and sound can be affected. Many Chiarians complain of tinnitus. To me, it sounds like a wooshing sound inside my ears but can also sound like a small owl hooting at times. Again, the left side is affected more than the right in me. My eyesight, however, is worse on the right.

CSF flow is responsible for a lot of the symptoms so it's important that you get a CINE MRI to study the flow to make sure you're getting the right stuff to the right places.

In a previous post, I wrote about my personal symptoms. You can find it here: Falling Apart. 

RELATED CONDITIONS:

There are many related conditions that you might experience with Chiari. These include POTS,Ehlers Danos, retroflexed odontoid, Tethered Cord Syndrome, and Syringomyelia. You should be tested for all of the related conditions by a specialist to ensure that you're getting the proper care. 



I am excited about my visit with Dr. Tew. So, I am on the right track now. If that doesn't work out, though, then I will probably try the clinical try in Maryland.

Here is some MRI information...

This is a normal MRI.

See how the cerebellum is kind of shaped like Pac Man?



 Now, this is my MRI.



You can see that my Pac Man seems to have an extra part on it, hanging down. That is sometimes referred to as the "tonsils." 






























Here's a better look...

The size of the herniation isn't necessarily what makes the difference. In my case, the herniation isn't long but it is thick. This cuts off fluid flow which is really what is causing the problem. Also, you'll notice that different slices on the 

MRI show different views so in some the herniation looks bigger than in others.

Dealing with Pain

While waiting for my appointment with my neurosurgeon to be scheduled, I've been trying to figure out how to deal with this pain. What pain, you ask? It seems to differ.

Sometimes, it's the pain in my left arm and hand. That's a shooting, stabbing pain. It makes me hard to hold anything. When it's not hurting, it's tingly and numb. The pain is almost better.

Then, there's the pain in my back. That's usually the worst pain. It affects my mid-back mostly, although it can be my lower back. This pain is also sharp. No amount of rubbing helps it. It does give me reprieve now and then but during those times it also goes numb and that's scary as hell because I'm sure I'm being paralyzed.

The pain in my hips is what makes walking and functioning difficult. It feels like my hips should pop back into place or something . They do creak a lot. They don't hurt constantly, but when they DO hurt it's excruciating.

Lastly, there is the pain in my legs. This is normally a cramping pain although it's not uncommon for it to be shooting, too.

I have tried the following for pain:
- heating pads
- heat patches
- warm showers
- hot tubs
- Tylenol PM
- Ibuprofen
- massages
- Gabapentin

I am currently on Gabapentin. It takes the edge off the arm pain and head stuff but does nothing for the joint type pain in the rest of the body. Some days, it feels like my whole body is breaking. 

Getting Scheduled

Actually getting scheduled to see the neurosurgeon has been a thorn in my side. Here's the Cliff's Notes version of my journey...

First, I had the requisite number of friends telling me about their cousin or friend or aunt who had a great NS and how I should go to them. That might be fine under any other circumstance but with Chiari you REALLY need to see a specialist. Don't believe me?

Three of the five neurosurgeons I called had never even done a decompression before but "knew what it was." Folks, don't let them hack into your brain unless they have experience. Only get a recommendation if the person you're talking to actually has experience with Chiari.

I did find someone with Chiari experience in Louisville so I made the appointment. They could get me in within the week. Yay! I could bring the referral form, too, so the doctor didn't need to fax them.

Unfortunately, as soon as I got off the phone with them and started reading some reviews I was not impressed. In fact, the reviews were horrible. They scared me so much that I ended up canceling that appointment.

A friend who has Chiari and has been through the surgery told me about Dr. Tew and his new Chiari Center. I researched him and it appears that he is one of the top in the field. A call to his office revealed that he did not take my insurance. (He's in Cincinnati.)

I finally found someone (Dr. Choutka in Ashland) who had it on his resume and came with good reviews. I called his office and asked them if he took my insurance. He did! So, I called my PCP with the phone number and information and asked them to refer me.

And I waited.

And waited.

Waited some more.

After 5 days I called them. They hadn't done it yet. Said it wasn't a priority. I asked them to please make it one. The next day, they called me back. Come to find out, he did NOT take my insurance. (Then who the heck had I spoken to on the phone?) So, I called my insurance provider and asked to switch since I was still in open enrollment. She said sure, but that it wouldn't take effect for 7 weeks. If I had called the day before, it could have happened the next week.

So now I was looking at 7 weeks before I could even get the referral in. That sounded like too long.

Off to a new plan!

Frustrated, I called Dr. Tew's office back. After all, I had this new insurance now. Maybe his office would make me an appointment for April 1st when my insurance went into effect. A new call to his office revealed that he DID take my insurance-only it was the one I had just canceled.

So, I called my provider BACK and asked to switch back. Shew.

I then called my PCP and asked for a new referral, this time to Dr. Tew.

And I waited.

And waited.

And waited some more.

Two weeks later, I called my PCP. They said they hadn't heard any news yet.

I waited another week. Then, I called again. The office worker I talked to this time told me that it wasn't even in my chart or notes to make the referral.

Frustrated, I went in and got the referral myself. I faxed it and my records to Dr. Tew's office.

Two days later, I called his office. They didn't get it. Unbelievably frustrated at this point, my husband faxed it for me. I called the next day. They STILL didn't get it. So, I emailed it to them and asked my PCP's office to fax it as well. As in THAT DAY. They finally received it.

The guy in Dr. Tew's office called me the next morning and got a history. He also said that I would need to send him a copy of my MRI. My husband went out that day and mailed it. That was four days ago. Once they receive everything he said I should hear something about my appointment date within 4-5 business days.

Some people have to see several neurosurgeons before they find one they are comfortable with. I hope to goodness this one works. 

Visiting the Neurologist

After the initial shock of being diagnosed with Chiari wore off, I made an appointment with my neurologist. After all, it deals with the brain so the NL seemed like the place to start and since I already had one...

Well, that didn't get me anywhere.

Let me start by saying that I wasn't real confident that it was going to be a successful visit to begin with. Over the past few years I've been getting more and more frustrated with my NL but kept giving him the benefit of the doubt. My migraine meds stopped working years ago but his reaction was to simply increase my anti-convulsants. Eventually, I stopped taking those because I was no longer even convinced that I had epilepsy. I noticed no change in my seizure like activity and when I tried to ask him questions about the type of seizures I had (my OBGYN wanted to know) he seemed to get angry and couldn't answer me.

Anyway, he immediately went into defense mode when I showed him the MRI report. He told me I "might" have had a malformation but that it wasn't causing me problems. That, more than likely, I just had a vitamin B12 deficiency and restless leg syndrome. Yes, restless leg syndrome CAN be a part of Chiari but RLS does not explain the other symptoms. You know, the ones above my legs.

I told him that I'd had a full panel run and that my levels were normal. His reply was that they said that anything less than 300 were normal but really the normal range was 350. When I told him that my levels were 392 he corrected himself and said that anything under 400 was normal. So I can only assume that if I had told him my B12 was 500 he would have told me that it needed to be 550.

I left feeling frustrated.

Obviously the NL does not do the surgery but I will still need one for post-op and continued Chiari management. Unfortunately, it's not going to be this NL. The time has come for us to part ways.

This is the same NL who shrugged and told me that I'd be fine and that my headaches weren't anything to worry about when I was pregnant. Three days later, I had a complete placental abruption, delivered 4 weeks early, and was found to have preeclampsia. So it's not the first time he's dropped the ball. 

My Story

I figured I'd start this blog by giving my story and how I got started on this road to Chiari Malformation...

1980-1987:

For the first 7 years of my life, I was thought to have a heart problem. I began having spells at around 7 years old and nobody could figure out what they were. My heart would race, I'd get very sweaty, and I'd pass out. I went to a couple of cardiologists and had a bunch of tests run. The verdict? A murmur.

Symptoms:
- Racing heart
- Sweating
- Fainting

1987-1998:

The "spells" continued on through the rest of my childhood and were ever resolved. Stress seemed to make them worse but not bring them on. As a teenager, I began developing headaches and one sinus infection after another, too. I would do a course of antibiotics, only to have the sinus infection come back. I went back to the cardiologist for my heart and wore a heart monitor. Nothing.

Symptoms:
- Chronic sinus infections
- Headaches
- Fainting
- Racing heart

1998-2001:

For about 3 years I had more than 2 sinus infections. In addition, my headaches were terrible. After one week of constant pain my PCP sent me to a neurologist. He diagnosed me with chronic migraines. I was also sent to an allergist. He gave me Advair for asthma (that was new to me) and Rhinocort Aqua for sinus infections. I was very fatigued at this point, too, and sleeping a lot. The neurologist thought once we got the migraines under control this would change. However, along with the fatigue, I grew very winded at simple activities and felt weak.

Symptoms:
- Headaches
- Chronic sinus infections
- Weakness
- Asthma
- Fatigue

2001-2005:

On a trip to Europe (I had done several backpacking trips) I had a really scary experience. I completely lost my sight for about 5 minutes. I then passed out and woke up sweaty and disoriented. I also started experiencing a lot of back pain. When I came back, my neurologist ran me through a bunch of tests and diagnosed me with epilepsy and said I was having seizures. He put me on Lamictal. I did not notice any changes. He increased it, but still no changes. I also noticed that whenever Iflew the pressure in my head grew unbearable and I would always get a sinus infection upon landing. It ruined the start to quite a few trips. Fatigue got worse. I started an exercise program. I went on a diet. Nothing seemed to help. Around 2005 I began having a ringing in my ears that started suddenly. An ENT diagnosed me with tinnutis. After about a year it eased up.

Symptoms:

- Fainting
- Heart racing
- Fatigue
- Back pain
- Chronic sinus problems
- Head pressure
- Tinnutis

2005-2009

I got pregnant in 2006 and had a few problems during my pregnancy. I had horrible back pain that continued after the pregnancy. The OBGYN thought it might be pregnancy related and would go away. It didn't. I went to a chiropractor and physical therapy. I also went to PT for my migraines which seemed to get worse. The fatigue continued after the pregnancy. We blamed it on having a newborn.

In 2008 I began having what was eventually diagnosed as anxiety. Once, I was driving for work and ended up in the library parking lot. I had no idea how I got there or what I was doing. My husband had to come and get me. I tried to read the clock, read the phone numbers on my phone, etc but nothing made sense. My neurologist called it situational anxiety due to the nature of my work. The pressure in my head continued to increase. The headaches became more intense and no longer responded to medicine. My back pain was increasing. At this time, I also started developing bowel problems. I would get painful cramping during the day and night which would sometimes result in a bowel movement and sometimes not. I was diagnosed with adenomyosis and we figured that was the cause, along with endometriosis, for the bowel issues.

Symptoms:

- Cognitive issues
- Memory problems
- Back pain
- Bowel problems
- Fatigue
- Head pressure
- Migraines
- Sinus infections

2009-2011

I got pregnant in 2009 and had a whole bunch of complications. They included hyperemisis, preeclampsia, and a complete placental abruption. I also had excruciating pain from my back and down my hip and leg. The OBGYN said it was probably sciatica. I was set to a PT which made it worse. The fatigue was chalked up to pregnancy and the hyperemisis. I gave birth at 36 weeks due to the placental abruption. It required a blood transfusion.

Not long after giving birth I began having what we thought were extreme panic attacks. The room would close in, I'd forget where I was, and I couldn't function. The doctor thought I had PTSD due to the traumatic events of the birth. (I hemhorraged, it required a blood transfusion, etc.)

Seven weeks after birth, my son died from SIDS. (See my SIDS blog at www.lifeaftersids.blogspot.com) My anxiety (or so it was called) increased. I began having cognitive problems and could no longer follow detailed conversations. My memory faltered. The pain in my back increased. The fatigue kicked in. I had trouble leaving the house. The doctor thought it was depression.

Symptoms:

- Fainting
- Fatigue
- Hip pain
- Leg pain
- Back pain
- Memory problems
- Cognitive problems
- Panic attacks
- Migraines
- Sinus infections
- Head pressure
- Bowel problems

2011-2012

After losing our son, we got pregnant again. I had more problems with this pregnancy (partial placental abruption, subchorinoic hematoma, preeclampsia, gestational diabetes, hyperemisis, and intrauterine growth restriction). So, of course, any symptoms I had were blamed on pregnancy.

After giving birth, I was hit with a ton of other things. The back pain never went away. When it wasn't hurting, it was numb. I grew so weak that walking became difficult. My left arm had throbbing pain and then turned to tingling and numbness. The "wooshing" sound in my head from a few years back returned. My bowel trouble made it difficult to go out anywhere.

My PCP suspected MS. She ran some bloodwork which came back fine. She then sent me for the first MRI in 10 years.

And, there it was, the Chiari.

Thus begins my journey as a Chiarian.

Falling Apart

I have imported this from my other blog...

The post on my SIDS blog explains a lot about what happened last week. I am still in shock. I am out of state at the moment but when I get home I'm going to do what I can. I am so incredibly saddened for the people of my hometown.

I'm getting an insurance runaround at the moment, but might be the first person in history looking forward to getting my brain and spinal cord operated on. I've heard the whole spiel about your body failing you, but only now do I understand it. 

Starting with my head...I now have a dead spot on my right eye. I have some sight in it, but there's a big spot that's just blurry. It makes seeing very difficult. I know this is from the fluid and pressure because the rushing, pulsating sound in my ears is a sign of that. Hearing has become difficult. The pain in the back, left side of my head is burning when it's at its best, throbbing when it isn't. The dizziness and vertigo make it hard to walk down stairs. I am losing depth perception. 

The stiffness and pain in my neck make it hard to move. When the pain that radiates down my left arm is bad, I can't deal with it. When it goes numb and my hand forms into a claw, however, it's far worse.

My back pain reminds me of labor. Sometimes, it's shooting pains. Other times, it's spasms. It always aches. When it's not really painful it goes tingly and numb and for that reason I get terrified. Paralysis is a huge outcome of syringomelia if you don't address it in time and ever time it goes numb I"m afraid it might have finally passed the point of no return. Obviously, it hasn't and I am okay but it still scares the hell out of me. 

Then, there's the digestive issues. I'll eat and feel fine. Then, we'll get in the car. A little while later I'll get it with mind numbing cramps that will make me break out in sweat and sometimes tears. Pete or Mom will have to find me the nearest bathroom. it doesn't always happen but it's made me leery of eating out in public. Sometimes, the cramps are so hard that I vomit. 

The pain continues through my hips. It hurts to stand for very long, it hurts to sit, and lying on my side is almost impossible. The best position is flat on my back but who the heck wants to spend all day like that?

Standing isn't great anyway thanks to the weakness in my legs. I'm afraid to carry Iris for long because sometimes they buckle. 

All of that might be able to be dealt with if it weren't for the memory issues. There are literally times that I ill be sitting in the living room and have no idea where I am. I'll see my walls and things and none of it will make sense. 

This is not a fun way to live. It makes me an awful parent and spouse. My quality of life is slowly dwindling. I have met others who have these problems (Chiari Type I and a syrinx) and we all have similar issues. The surgery itself is not a cure, just a treatment that will help the symptoms. Death rates with the surgery are high. In some cases, not having the surgery will cause death anyway. (Because of the spinal cord issue.)

There are days that I really question what I am doing. On days that I am having a pretty decent time I try to do as much as I can. I go for walks with Iris, play games with Sam, and try to clean. 

The list of things I can no longer do (even with the surgery) is long and depressing. Goodbye rollercoasters. 

I am also angry. I am angry that my symptoms have progressed to this point because I was misdiagnosed so many times. I am angry that nobody ran a test in the past 10 years and just assumed that it was stress or anxiety. I should NOT have given birth vaginally. No wonder my pregnancies were so hard. This has complicated my life in so many ways and we just didn't know I had it. A simple MRI would have shown it immediately. I am thankful my doctor finally listened to me and put all my symptoms together. I just wish it had been earlier. 

I am not looking forward to shaving my head. I am not looking forward to spending so much time in the hospital. I am not looking forward to the recovery time. I am not looking forward to having a zipper up the back of my head. 

But I am definitely ready for a change.