Saturday, August 29, 2009


Here is the latest edition of my medical overview:


Chiari Malformation: A skull malformation. My skull was formed too small, causing pressure in the back of my head, and a blockage of fluids that should flow freely in and out of the brain.

Ehlers Danlos Syndrome (EDS): A connective tissue disorder. My ligaments are looser than they should be, causing certain joints to easily dislocate. My veins and arteries are also lax, making it difficult for blood to be pumped the way it should. I also have associated heart valve defects.

POTS: An autonomic dysfunction. My autonomic nervous system malfunctions, causing problems with all involuntary functions of the body. This causes difficulty standing, due to my heart rate and blood pressure not reacting properly to the effects of gravity.

Tethered Cord Syndrome (TCS): A spinal cord malformation. My spinal cord is anchored in place, not allowing for it to move freely within the spinal column.

Cranial Settling: When EDS and Chiari join together, the skull-spine joint often becomes unstable. The skull begins to settle on the spine. This causes trouble swallowing, nausea, heartrate abnormalities, and other autonomic dysfunctions, including POTS.

Vitamin D Deficiency --> Hyperparathyroidism --> Osteopenia: Lack of vitamin D absorption causing excess PTH, reducing the amount of calcium in the bones, which results in low bone density.

Major Symptoms:

Headaches--I have a constant pressure headache. This pressure sometimes reaches a debilitating level. I also get migraines and more acute, sharp headaches. These are from Chiari and Cranial Settling.

Nausea--Nausea can be caused when there is increased pressure on a certain part of the brain. It is often worse when I am upright, and relieved when I lie down, but it never goes away. It is a symptom of Chiari and Settling

Neck and back pain--I have pain throughout my spine. This is caused by loose ligaments in the spine, allowing the vertebrae excessive movement, causing arthritis of the spinal joints.

Fatigue--I require a great deal of sleep. If I don't get enough sleep, my symptoms will worsen, leading me to return to bed and get more sleep. I also sometimes fall asleep at inappropriate times or have insomnia at night.

Tachycardia/Low blood pressure/fainting--After standing for too long, my heart races and my blood pressure drops. Sometimes for no reason at all, my blood pressure gets very low, and I can fall into a state of reduced consciousness. I get short of breath very easily. These are the main symptoms of POTS.

Heavy head--Due to the settling, I have difficulty holding my head up. This is very tiring and leads to severe muscle spasms in my neck from trying to support my head. All of my symptoms get worse if I don’t wear a collar to keep my head from settling too much.
Joint pain--I have pain in all my joints, especially my knees and hips. This is sometimes severe enough to prevent me from walking. It is caused by EDS. It is gradually causing early osteo-arthritis in all of my joints.

Flu-like symptoms--I have have low-grade fevers, cold sweats, and overall body aching, very similar to the flu.

Vertigo and Balance problems


I take medication to constrict my veins, in an attempt to help my blood flow improve and raise my blood pressure. I also take salt pills regularly to force my body to retain more fluid, which can help keep my blood pressure up. Salt pills are also the only thing that helps when my level of consciousness lowers. I drink a LOT of water.

I have medications that help me stay awake during the day. They usually work, but they sometimes make me a little too talkative and hyper.

I am very cautious about taking pain medications. I treat pain with ice packs, heating pads, and rest, when possible. Any pain medications I take a carefully monitored by my pain management doctor, whom I see regularly.

I've already had several major operations, but obviously, I'm not better yet.

In July 2002, at the age of 18, I had a posterior fossa decompression surgery. This involved removing part of the base of my skull and the back of my C-2 vertebra. I felt much better at first, but started to get worse again after a couple months.
When my symptoms continued to worsen, I began to search online for more answers. I sent my MRI’s to the doctors at The Chiari Institute in New York. They found that I had cranial settling, caused by cranio-cervical instability.

In December 2003, the day after my 20th birthday, I had a cranio-cervical fusion in New York. Titanium rods were bolted to my skull and screwed into my top 4 vertebrae. This fused my skull in place atop my spine.

After that surgery, I worked very hard to recover. I struggled to keep living my life, but my symptoms persisted. This led me to have another set of MRI's. I sent these to my doctors at The Chiari Institute in New York. It was confirmed that I had an occult tethered spinal cord. This is a hidden form of Spina Bifida that isn’t found until later in life. It was also found that I have an inadequate fusion.

In June 2008, I had my filum terminale clipped to de-tether my spinal cord. This released the tension in my spinal cord. Mild lower-body symptoms, such as leg pain, low back pain, and bladder control improved. These symptoms probably would have progressed without this operation.

I still require a revision to my current cranio-cervical fusion. This will reset the position of my head, allowing for greater stability. This surgery cannot be scheduled until my bone density has improved. My bone density scan will be done in November 2009. If my bones are stronger, I will be approved for surgery, to be done early in 2010.


Currently, I sleep about 12 hours a night. I have medication that helps me stay awake the rest of the time, usually. This is a great improvement over a few years ago.

I still need to spend most of the rest of my time resting, either in bed or on the couch. About 4 hours a day, I am able to be somewhat active, most days.

I use a wheelchair or a walker for assistance at times. I also wear a rigid cervical collar at all times. These things make it easier to get by.

I am in pain all of the time, but I have learned to live with it. I am expecting my next (and last) surgery to decrease my overall pain and increase my level of functioning, so that my life is slightly more livable.

Thursday, August 13, 2009

More thoughts on today

I think the reason I'm so upset today is that I am certainly having a sick day, I'm noticing the downward pattern in my health, I am worrying about my ability to keep pushing through...and I don't feel that I'm allowed to be feeling this way!

The ones that love me do have other things going right now. But I don't know where and when is appropriate to discuss all of these feelings, and more importantly, have these feelings validated.

I have serious fears about my ability to continue working, to continue commuting back and forth to Milwaukee, and to handle the severity of my symptoms.

I have been sicker than this before, but then, I was bedridden. There was no pressure on me to do anything. This time, there's tons of pressure. I need to keep doing everything.

And the constant struggle to do what is needed, despite horrible pain and serious worries, is turning me into a witch. I'm not the person I want to be. I don't know how to keep being nice when I'm struggling just to exist. Even just laying down is a struggle. The guilt is immense. Guilt that I'm not doing what's expected of me. Guilt that I am making other people's lives so much harder. And guilt for being so mean and irritable.

I also have serious concerns about if my bones will be ready in time for my December DEXA scan. I haven't had any actual medication for it, just supplementation. And people with EDS do NOT absorb the way we should. But my doctor wouldn't prescribe an actual medication to help rebuild bone. How do you think my bones got like this in the first place? I don't absorb properly!!! Adding more won't make me any better at absorbing! So it's quite likely that this year long wait will be pointless. The bones have to get better, or I can't have this surgery. And I've already waited so long. It's unfathomable that I may have to wait even longer.

I am overwhelmed by all of this. I can't stand this existence. I just can't handle all of this.

So that is what is upsetting me. I suck at this life. I need it to change. But I don't think anything can change for a while. Who knows how long? It's an endless wait.

I know, I just have to do my best. And not every day will suck this much. But for today, it just seems like more than I can handle.

I just wish it could be different. I wish I could be different. I wish it didn't have to be this way. I want to be better for everyone I love. I really hope that one day I will be.

Wednesday, August 12, 2009


I had a new post written. But it was very raw and dark. The point of it is that my body is deteriorating, and the failings of my body are depressing. Sorry if these posts are ever too much. These are simply the truth behind the smiles. But I've re-written the post to make it a little easier to handle.

I have a new pain doctor. Dr. Wilkin is great, but I'm having a hard time with the insurance company approving medications. Ugh. Hope this gets worked out soon. I'm in so much pain. I have another appointment next week, and I hope to try something different and get the new medication approved.

I need help. I need to find a way to get through until surgery, which is at least 5 months. I have many new and worsening symptoms. But most importantly, I need adequate medication for my energy and my pain to help me get through until then. But my determination that I need this surgery has never been stronger. I just hope that once my bone density levels are improved, there is no further delay. I hope that things are settled at The Chiari Institute and I can get surgery scheduled early in the new year.

I need to stop burdening those around be the person I should be and live the life I should be a positive force in the world. I want to have this surgery so that one day, it will be worth all the trouble. Worth the trouble we've already been through, and all of the trouble that is ahead. It is such a huge burden to ask someone to help me bear. How can I ask someone to take care of me, to spend time with me, to watch so much more suffering? Only if they too have the hope that one day, it really will be worth their while. This is the only hope I have to offer, to hold on to. That after recovery from this surgery, I'll feel well enough for it to be worth everyone's trouble.