Thursday, January 14, 2010

The Lie Is...

At the moment, my bedroom has no electricity. I open the blinds wide during the day and light six or seven candles every night. It's just enough to read by. I called the new rheumatologist who is managing my Ehlers-Danlos syndrome and told her I was in my second week of physical therapy, but I was still being overwhelmed by pain.

She recommended Icy Hot patches for pain in shoulders and back and said to let her know how they worked, because she was willing to wage war with my insurance company to get me Lidoderm, patches impregnated with lidocaine, a local anesthetic. I've heard good things, so I hope they work out.

In the meantime I've been losing a lot of sleep, taking Tylenol around the clock and even sneaking extra anti-inflammatories when I can.  The pain orders me around, determines when and if I do anything, and makes it impossible to plan for the future.

In a week I'll be in Chicago and I worry now about burdening my friends with my pain or possibly getting sick and having to go to the hospital. I HOPE this does not happen.

I cannot convey to anyone how it feels to have this awful disease. Not to my professors, not to my friends, not even to my doctors or my parents.  So, I feel as I did as a child, feeling that unique disenchfranchisement all over again, the distance between you and other people.

Going back to Chicago next week, I dimly remember the feeling of listening to my iPod on the L...watching the light cartwheel between the branches, the sound of the rails. I liked to sit backwards. I loved Chicago so much.

I had a grace period, where my body could compensate for my disease and I could live relatively comfortably. But as I aged past my prime, into my mid and late twenties, the system began to fail little by little. I could feel it happening and was terrified. What on earth was happening to me? I got short of breath, my heart would race, my vision would blur...I couldn't concentrate on anything and when people spoke to me, I couldn't understand what they were saying. The words were just sounds, the inflections preserved, but the meanings lost.

My doctors told me I was just having panic attacks, but that didn't seem right, and the medications didn't help.  And no one could explain the pain that started as a weak gnawing and at its worst left me writhing and sobbing.

This is such awful, self-fixated stuff. My mind should be a billion other places, but unfortunately it's tethered to my body securely and my body is in crisis. I long to work on poetry, but it's difficult to write in a crisis. Being sick yanks me this way and that, leaving little energy left over for anything else.

I read yesterday, "The lie is that the world is good." It certainly isn't good. Or fair.


SJ said...

Hi Yvette:

Your writing touched me. I'm sorry you are suffering.

I am an EDSer too; twice your age and still swordfighting the pain monster. Sorry to say, it doesn't get easier over time, but you do develop favorite techniques that help you stand your ground. If there were degrees awarded for living with pain, I think EDSers would earn Ph.D.s.

I'm writing because you mentioned some of your symptoms and they seemed to indicate a more complicated scenario than EDS alone. Do you know much about the connections between EDS and Chiari 1 Malformation and Syringomyelia and Tethered Cord Syndrome? If not, you may want to research them online.

My symptoms were similar to yours for many years until I went to some experts in EDS at the NIH and got the whole story. They were just figuring out the connection and they included me in their study.

The good news is that there are effective treatments for CM, SM, & TCS. (The not-so-good news is that they are surgical.) There is a good online support group you may want to know about that is helpful to people with various combinations of these conditions. It's a yahoo group called Chiari Connection International.

Also, the best EDS expert is Dr. Clair Francomano in Baltimore. She and her team are the ones who figured out all this stuff.

I wish you all the best, Yvette.
Be well, and keep writing.-- you have talent.


Yvette. said...


Thanks for your comment! And I'm glad you enjoyed my (somewhat pitiful) writing. Dealing with pain is probably the hardest aspect of having EDS it's nowhere near controlled, so I live in fear of it.

I am familiar with CM1 and syringes and TCS. For the longest time I thought I had one (or all!) I had an MRI in May when I still lived in Chicago, but it was all clear. (Or so they told me) I was still suspicious, but after finally getting diagnosed with POTS in July and being treated with Florinef most of my symptoms like numbness and complete inability to concentrate disappeared.

Based on that, I assumed that I was dealing with POTS alone and not any other hangers on like CM1 or TCS.

I too am trying to get to Baltimore to participate in a clinical study. They are only looking for ethnic minorities and males at this point (lucky for me.)
If that falls through, I probably will schedule with Dr. Francomano.

I really do want to learn as much as I can about EDS, since most doctors beyond geneticist and the rare rheumatologist know next to nothing.

Thanks again for commenting! It makes me feel better to hear from people who struggle with the same thing.


SJ said...

POTS is fairly common with EDS; so are other forms of Dysautonomia that arise when the tip of C2 hits the brainstem, or when there is cranial-cervical instability, or when a Chiari occlusion forces high-pressured cerebrospinal fluid up the front side of the brainstem and into the pituitary gland. It's rough on a person to feel as if she is dying several times a day. Also, these processes are nearly invisible to others, so there's not much sympathy or real help.

EDS pain is notoriously tough to treat. It's refractory to most meds. Typically for classical EDS, HEDS, and the newer crossover types, including the kind called EDS+ that often accompanies CM1, local anesthetics don't work well or at all. (That's why having dental work and getting stitches put into skin are often royal nightmares. Add poor wound healing or atrophic scars and it's really challenging.)

Many EDSers tend to metabolize meds very quickly, so the pain comes and goes all day long. Toughing it out is not a good idea. It's dangerous for the brain; chronic, unresolved severe pain destroys about a cubic centimeter of gray matter per year.

EDS experts now prescribe low doses of long-acting oral opiates or opioids (that are supposed to last 12 hours in normal people) and ask the EDS patient to take them every 6 - 8 hours. The coverage is more even, and less like a roller coaster. Although this approach takes the edge off but does not entirely eliminate pain, the sense of consistent relief it grants makes it possible for many EDSers to function very well instead of fearing and avoiding pain triggers.

I hope you get to do both: join the study and see Clair. I also hope they get your pain under control and revisit the CM1/TCS issue.

If you were to get a copy of your MRI scan, you could read it yourself and answer the question. Usually radiologists miss or ignore smaller herniations, and those are the ones that accompany EDS and TCS. Also, newer research says to look for elongation of the brainstem and clumping of the cauda equina roots as signs of cord tethering, since in EDSers, the conus tends to be positioned normally. Most radiologists haven't heard of that, so they miss it.

Let me know if you want any help when the time comes...

Best of everything good!

~Sandy in Oregon

Yvette said...

I hear that POTS/dysautonomia often runs along with EDS but I'm not sure why in my case. Or, I know that I have a loose neck. It's easy for me to pop my own vertabrae around and I have bone spurs rumbling around in there and have had doctors catch my cervical and thoracic vertabrae being out of place (they were nice enough to help put them back.)

At the same time I also have autonomic neuropathy with little or no vasomotor or sudomotor reactivity in my feet (my feet don't sweat or pump blood anymore.) This seems to have happened after a bad virus I had. Is my brain stem really being crunched up by my neck vertabrae? Or do I just have such bad POTS because of my neuropathy? Inquiring minds want to know (like, really badly.)

After looking at the results of my tilt table test where my blood pressure falls, then spikes, then falls then spikes again, my neurologist came up with a list of reasons it might be happening in that unusual pattern. Most had been eliminated during previous work-ups (pheochromocytoma) or seemed unlikely (mast cell disorder). Only two options seemed reasonable: denervation supersensitivity from early small fiber neuropathy (or my damaged nerves flipping out) or CSF flow abnormalities (is this like what you mentioned with Chiari and CSF going places it doesn't belong?)

The only thing I can fall back on is the fact that I don't have the very severe headaches that seem to accompany Chiari foremost. I assume that if my POTS flares up again we'll look into it with more focus. I hate to think there's something more I could be doing.

That's why I hope I can get in at Baltimore. I want to know as much as I possibly can.

I was planning on requesting my full-body x-rays and MRI to give to my new rheumatologist. Of course, I'll take a look at them too.

The pain is really its own animal. No doctor will give me opiates. The closest I can get is tramadol and for the longest time I couldn't even get that and so would have to use the ER if I needed something stronger than Advil and Tylenol. Since then, I've added meloxicam and Lidoderm to the arsenal, but I still can't really do much in a day at all, or I'll pay for it. It doesn't have to be anything drastic, just walking around will do it. I could never work even part time with the level of pain I have. This really grinds my gears, because all I want is to do the things regular people do like work to support myself! I don't want to get high or sell them for cash...I just want to live my life without being in excruciating pain! I feel like that's a right.

Luckily, I'm one of those people who isn't insensitive to local anesthetics. My Lidoderm actually works! But then I can't put Lidoderm patches everywhere it hurts, only where it hurts the most. There's a level of pain (the level which I try to frantically avoid) that nothing I have can touch. This is where the opiates I can never get would step in, I assume.

I'm just afraid to put my foot down because I've been thrown out of practices for being stubborn and mouthy before.

Thank you so much for discussing this with me, Sandy. No one else seems to get it.