Worried about working

My headache is about a 6 or 7 on the pain scale today. My baseline headache is a 4, so it's significantly worse today. It feels like a high pressure headache to me. The pressure is allover my head, and somewhat relieved by applying external pressure to my temples, cheekbones, or even the top of my head. I still seem to have temperamental intra-cranial pressure. It can't help that I take salt pills to keep my blood pressure up. Because many people will treat high pressure with a diuretic, which is the opposite. So I'm sure the excess sodium doesn't help my head pressure. Ugh.

Also, standing up for even a short time has been making me so winded.

And the nausea from sitting up continues.

These symptoms are getting noticeably worse. It really scares me. This is my spring break week, meaning my piano students are off from school, so I gave them (and myself) the week off from lessons. But it really makes me wonder how much longer I'll be able to pull this off. I work around 3 hours a day, 5 days a week, normally. At least I have been since last fall. But lately, I've had to cancel more frequently. And with symptoms worsening so quickly, I'm afraid I won't be able to teach at all much longer. I was hoping to last until May 15th, when I go to The Chiari Institute in New York. Then, I thought I could give the students the summer off and try to have any surgery done, then. But what if I can't do it? What if I can't wait until summer? And what if I don't recover in time to restart in the fall? I know: it wouldn't be the end of the world. I just wish it weren't so complicated.

New Stuff posted

Just wanted you all to know that I've posted a new set of links to the right. They include two pages full of my medical explanations, in easy-to-understand terms. Also, I've included links to my medical history, including information on the surgery I've already had. I'll probably get that information transferred over here eventually. Also, I'll try to get some pictures up soon.

In bed another day/IBS problems

Well...I'm in bed again today...it's been the norm for so long. But sometimes it's all I can do.

My digestion has gotten worse the past few days. No matter what I eat, I'm getting horrible abdominal pain after each meal. I've been following the diet recommended by Heather at helpforibs.com for a year now. And it's a wonderful thing. But sometimes, it isn't quite enough. I take soluble fiber supplementation and peppermint oil pills and I have several prescriptions for stomach spasms. But some nights nothing helps but curling up with a heating pad. My digestive difficulties are most likely caused by my dysautonomia, as digestion is a function of the autonomic nervous system.

Also, it's gotten harder to sit up lately. The nausea becomes so strong and I feel a lump in my throat when I swallow. These are symptoms of cranio-cervical instability and retroflexed odontoid. I went to a movie with my mom yesterday, and I barely made it through. I tried wearing my old Aspen collar. Unfortunately, it doesn't really fit anymore, which is just another sign that my fusion didn't really hold.

This is in addition to my usual headache, which hasn't left me for many years. I got a short break after my first decompression surgery. After that, it just kept getting worse.

New research articles posted

The new link I posted, under EDS sites (New Article...) has some really great information about a study done by Milhorat, Bolognese, Nishikawa, and Francomano. It explains that people with EDS and chiari have distinct changes in the position of brain structures upon upright posture. These changes result in lower brainstem compression and symptoms. This helps to explain why I need to lie down so much of the time.

In addition there are several new links under TCS sites that report on the outcome of tethered cord surgery for treatment of chiari. It's good to know that this information is being reported, and coming from multiple places. For example, a doctor in Barcelona has been successfully performing surgery on occult tethered cord.

Just in case you're interested in the newest information.

Dos and Don'ts AND explanations from Dr.B

The following site has a great list of Dos and Don'ts for dealing with a person living with chiari. If people would read this page, maybe they'd have an easier time understanding my struggles.

Dos and Don'ts

Also Dr. B wrote an explanation of craniocervical instability in EDS patients, the incidence of TCS, and information about the use of Botox in the fusion surgery.

Dr. B on CCF

Questions, Concerns, and Worries

I did work out again tonight, but I'm not feeling quite so confident...about anything. Yesterday was Easter, and amazingly, I was able to play the keyboard at both of the morning services, with just rest in between. Normally, I actually fall asleep and cannot be woken up to play. It makes it hard to decide if I should keep the job or not. I'm certainly not in it for the money. But I don't want to abandon everyone. But I'm not really capable of doing the job right now. Anyhow, I guess I got lucky yesterday. After services, I went right to bed for about 2 hours. Then, I went to lunch with the family, which I also made it through. Gus took me home a little early. I spent the next 24 hours in bed, many of them sleeping. So of course, I did eventually pay for my over-doing it. Luckily, tonight I made it to the gym. But I felt pretty awful afterward.

I saw someone at the gym who I've seen only a couple of times since high school. That made explaining about my continuing illness very complicated, considering I was at the gym. It's one of those moments where I actually feel like I'm lying or exaggerating about my illness, which is really not fair. As soon as I got home (barely), my symptoms were horrendous. And even while I was at the gym, I was watching my heart rate very carefully, as it tends to skyrocket with all of the upright activity. My trainer has helped me learn to exercise safely by mixing supine exercises with upright ones. I only really exert myself if my body is near horizontal. This makes it a little easier to handle. But it is still such a challenge.

I feel so lazy when I'm spending 90% of my time in bed. It's hard to explain the reason for this, even to people who know about my conditions and see me quite frequently. I still get asked "Why don't you get out of bed?", "Maybe it would help to get dressed and ready", etc. It just makes me feel guilty for being lazy, when really, I don't feel like my body gives me a choice. Believe me, lying in bed is boring. This is not the life I want. But it is the life I have.

I've been questioning surgery a lot lately, after talking to some people about less successful outcomes to these operations. What if I get worse? So many things could go wrong with such invasive surgeries. I've dealt with recovery before, and I know that things don't always go as expected. For example, the muscle spasms in my neck and shoulders haven't ended since my fusion. What if the tethered cord surgery creates permanent lower back pain? It seems like a definite possibility. And if it doesn't help other problems, it certainly wouldn't be worth it. But on the other hand, it could prevent the deterioration of my bladder control.

Well, my mind has been so busy. I feel like I never really think about anything else. Because it is such a huge decision on my shoulders. Maybe I'll feel better after talking to Dr. B. If only May 15th would come a little sooner...

Bring It On

I'm feeling proud of myself, because I've been working out hard with an amazing trainer. I barely ever miss it. No matter what else is going on, I work out once a week. By doing this for five months, I've been able to decrease my body fat by 5% and build a lot of muscle. Even though I lay in bed most of the day, I've still been able to improve the shape I'm in significantly. I can do some aerobic workout and a decent amount of weightlifting.

This makes me feel very optimistic about my body's ability to heal from surgery this time around. Last time I had surgery, I weighed about 90 lbs and was essentially bed-bound. Needless to say, recovery was incredibly slow. I had to rebuild my entire body. But this time, my body is strong, so I should be able to recover much more quickly.

It's nice to have a reason to be hopeful. I still dread the surgery and the pain, but I believe this time will be easier than last. So I say, bring it on.

Current Symptom List

1. Headache: My baseline pain level is a 4, with a constant feeling of pressure in my head. At times, the pain worsens to a more debilitating headache. I become hypersensitive to light and sound, and I need to rest for quite a while to have any relief from the pain. It does not improve with massage therapy or pain medications.

2. Fatigue: I require 12 hours of nightly sleep plus frequent naps. I spend much of the day in bed, but I am often able to work a couple of hours in the afternoon. I have more energy at nighttime, if I've rested during the day, so that is when I exercise, if possible. Overall, I feel very lethargic.

3. Neck/back pain: I have regular neck pain, as well as frequent pain between my shoulder blades and in my lower back, always within the spinal region. My head often feels very heavy. I also have developed increasing mobility since my fusion to C-4. I have a fairly normal range of motion now. It feels like my head is no longer being supported by the fusion, like it's no longer sitting on my spine the right way. It just feels off. I also have severe muscle spasms and painful knots in my neck and shoulders that always return soon after my frequent massages.

4. POTS symptoms: I have frequent tachycardia and low blood pressure spells. When my blood pressure is low, I can barely move. I am not able to walk very far most of the time, despite treatment and regular exercise. I have palpitations and arrhythmias.

5. IBS symptoms: These have developed in the last couple of years. I have severe abdominal pain and bloating after eating as well as chronic constipation. These symptoms continue despite treatment and drastic restrictions on my diet.

6. Signs of elevated intra-cranial pressure: sore throat, dripping nose, earache, feeling of fullness in the head with pulsing around the area of the decompression.

7. EDS symptoms: Joint pain. My knees and fingers hurt in the cold. My shoulders and hips can hurt anytime, sometimes restricting activity. In addition, my spine causes me the most pain.

8. Signs of cranio-cervical instability returning: nausea in waves when I am upright; choking on food, water, anything.

Abbreviations

CM, CMI, or ACM - Chiari I Malformation, Arnold-Chiari Malformation
TCS - Tethered Cord Syndrome
POTS - Postural Orthostatic Tachycardia Syndrome
EDS - Ehlers-Danlos Syndrome
CCJ - cranio-cervical junction
CCF - cranio-cervical fusion
ICT - invasive cervical traction
SFT - Section Filum Terminale
OTFT - Occult Tight Filum Terminale
C1-C7 - the 7 vertebrae of the cervical spine
T1-T12 - the 12 vertebrae of the thoracic spine
L1-L5 - the 5 vertebrae of the lumbar spine

Symptoms of Tethered Cord Getting Worse

I'm starting to realize that I really do have lower body symptoms that are getting worse. I've had achy legs for a long time, but that's nothing new. Also, I'm having trouble getting comfortable in bed. My upper and lower body refuse to be comfortable, even in my super-expensive sleep number bed with the specially-designed chiari pillow. I think I'll have to go to the sleep number store to try a new pillow. They have expensive pillows, but it will be worth it if I can get comfortable in bed...ever.

But worse than that, I've noticed a progression in my bladder symptoms, which are a hallmark of tethered cord syndrome, since your bladder is controlled by the nerves at the base of your spinal cord. I am fortunate enough not to suffer from incontinence. But I do have incomplete emptying of the bladder. This means that I'll wake up during the night multiple times to have to go again and again.

It's annoying, but the scary part is that it's a sign of progression, which eventually does lead to incontinence. So, the sooner the detethering can be done, the better the chances of making a full recovery without any further nerve damage.

It's so hard to wait to see Dr. B. I want to get all better. Well, I want to get any painful things over with so I can start recovering.

Upcoming surgical possibilities

I've recently completed a batch of tests and Dr. Bolognese has concluded that I need at least one more operation, to be done this summer.

I've been diagnosed with a severe case of Occult Tight Filum Terminale, which is a form of Tethered Cord. He proposes performing a section filum terminale surgery to detach my spinal cord from my tailbone, since it is being pulled too tightly, elongating my brainstem and increasing tension and pressure. A full explanation is included below.

In addition, he says that it is quite likely that my cranio-cervical fusion has failed. In order to determine this with certainty, he proposes another round of cervical traction (We all remember how fun that was the first time around.) If it has failed, it would be necessary to re-fuse my cranio-cervical junction, but only after the detethering surgery has been done.

My Chiari Journey

My chiari journey has been long and painful. I started getting sick in junior high, with stomach aches, fatigue, and headaches. During high school, the headaches worsened, and I developed worsening fatigue and exercise intolerance. No one knew what was wrong with me. I got tested for many things. They only found that I had mono one year, that never seemed to go away. By my senior year of high school, I was home-schooled and so sick all the time. It was a miserable existence. I was finally diagnosed with a chiari malformation in the spring of that year. I had a decompression surgery at Children's Memorial Hospital that summer, July 2002. The surgery went very well, as did my initial recovery. It was the first relief I'd felt from my headache and nausea for as long as I could remember. But when I went to college that fall, I started getting worse, and kept getting worse. I developed POTS, which caused a dizzying array of symptoms. I had blackouts, low blood pressure spells with decreased consciousness, chest pain, extreme fatigue, cold sweats, sleep difficulties, and more. Finally, I went to the chiari institute, and they determined that I needed further surgery. I had a cranio-cervical fusion in December of 2003. The recovery was so difficult that I was bed-bound for over a year. But my symptoms have stuck around. I now have been diagnosed with tethered cord syndome, and am considering multiple more operations.

Living with chiari has been a living hell. No one understands the kind of pain I am in every moment of my life. I work very, very part time, and even then I often have to call in sick. The constant headache is so draining, I just don't know how to handle it. Due to my illness, I deal with a lot of depression and anxiety issues. I've been suicidal multiple times. Anything to escape the horrendous pain. I just never know what symptom to expect next. Constant headache, back and neck pain, pain anywhere in my body, dizziness, nausea, extreme fatigue, fainting spells, heart arrhythmias, choking on my food...it never ends. And no one seems to understand or know how to help my symptoms.

Tethered Cord Explanation

I found a great definition of my form of tethered cord syndrome at http://www.btinternet.com/~tetheredcordresources/

"The filum terminale is like a thin elastic band, about 8 inches long. At the top, it is formed from one of the layers of tissue surrounding your spinal cord, and extends from the bottom of your spinal cord to the tip of your tail bone. The outside of the "elastic band" has a few nerve fibres sticking to it.

The filum terminale works as an anchor for the spinal cord. For people with normal anatomy, the filum stretches when they bend over to allow the spinal cord to move up in the spinal column and then goes back to normal length when the person straightens up and gently pulls the spinal cord back to its normal position.

When the filum is fat-filled, fibrous and tight, it will not allow the spinal cord to move up and down within the spinal column, and so the spinal cord and the nerves end up being stretched instead of the filum. In most people this causes nerve damage".

Everybody has a filum terminale; it is a threadlike piece of tissue that connects the end of the spinal cord to the sacral end of the spinal canal in the pelvic area. In patients with a thickened filum (defined as more than 2 mm in diameter) [Yundt, 1997], the filum is shorter or lower-lying than normal and is thickened with fatty or fibrous tissue. This abnormality causes the filum to become relatively inelastic (a bit like a rope or cord, rather than an elastic band) and the spinal cord becomes tethered at an abnormally low level, thereby giving rise to the recognised signs and symptoms of a tethered spinal cord."