Thursday, April 12, 2012

Today is a bad day.

Today is a bad day for a few reasons.  I came down with a cold yesterday, and any type of illness makes all my symptoms worse.  I had tachycardia during the night last night while I was trying to sleep.  Very unusual for me to have while lying down.  I got a migraine yesterday and it has continued through all day today.  I also developed an intestinal obstruction yesterday...second one in a month.  So, I knew this time I had to take a stimulant laxative if I wanted to avoid the hospital.  They would do the same thing there.  It worked, but of course it worked too well, so I'm in a lot of pain today.  So my head pain level is about an 8 and my abdominal pain level is about a 7.  I still have a cold, but it mostly seems to have messed up my POTS.

I still taught my piano lessons today.  It was especially hard to make it through, but I'm still alive and no one quit, so I guess I did well enough.

Now that I'm done teaching, I'm resting with an ice pack on my head and a heating pad on my stomach.  No relief yet.

I think I have confirmed that a small amount of dairy is what caused my obstruction.  I just used a greek yogurt dip with my carrots yesterday.  But that small amount seems to be the cause of my problem, as it's the only thing that changed.  If I can be stricter about my dairy-free diet, maybe I can avoid this in the future.

Wednesday, April 11, 2012

Good Days and Bad Days

A Good Day for me:

Lately, a good day is pretty good. On a good day, I might have no GI problems, or mild GI problems that are managed with medicine. (I follow my regular diet daily.) I might have just my "baseline" headache--generalized head pain, achy feeling, mostly in the back around the rods--the pain level can be as low as a 2 (although this is based on my new, modified pain scale). My energy may be high enough (with the help of caffeine pills) to be active several hours--up to 5. By active, I mean not resting in bed or the recliner. And my POTS symptoms may be well-managed with salt tablets and Midodrine, along with high water intake. This means that I am alert and able to walk around much of the day. My joints may only hurt when walking or standing in place. On a good day, I may have no trouble concentrating or coming up with words. Looking at all of this, I'd say I have a good day about once a week.

A Bad Day for me:

A bad day is still pretty terrible. Any number of things can cause a bad day, and often it's more than one symptom combined. It could be a migraine that doesn't abate with medicine--pain levels can really max out with this. I may pretty intense GI problems, including severe bloating, pain, or even intestinal obstruction. It could be severe POTS, where I am out of breath just from sitting up. And despite regular caffeine intake, I may have severe lethargy. Also from the POTS, I may be completely out of it, and go into a state of reduced consciousness for any length of time. I can have full-body muscle aches and joints that hurt with every movement. Bad days happen about once a week, too.

An Average Day for me:

So I guess the other five days are average days. I'll usually have mild GI problems, despite the usual diet and medicine. I often have a migraine, but many times the rescue medication I take will help it to go away in 2-6 hours. On an average day, my POTS symptoms force me to rest for the first 5 hours of the day...yes, 5 hours. That usually means I wake up around 10:00 and have to rest until 3:00-ish. I walk like a drunk person in a stupor all morning. I have trouble coming up with words or focusing on much in the mornings. And my energy level can be pretty decent later in the day, beginning around 4:00. Finally, on an average day, my joints, especially my knees and hips hurt a lot of the time. My spine often hurts. I get pretty bad muscle contractures in my neck and shoulders. Sudden vertigo spells are still common. If I have been somewhat active for even a few hours, I crash hard by 9:00 pm. Those are common symptoms on an average day.

I recognize just how improved I really am since my fusion revision surgery in the summer of 2010. I couldn't be more grateful for the good days, and even the average days. Having less of the bad days is incredible. So, even though I have plenty of symptoms, sometimes all at once, I am so grateful I had that surgery. I know I could be better, but I am so grateful to Dr. Bolognese for giving me another chance at living my life. I don't want to think about what I would be like if I had continued to decline.

So, I have plenty of limits, lots of meds, and a pretty regular, restricted schedule to my days. I can't exercise in any of the ways I would like to. I can't stay out late or drink alcohol. I still use a wheelchair at times and can't stand in one place for too long. I feel really out of it, and I have pain every moment. I deal with some truly horrific days, that feel completely impossible to survive. I visit doctors a lot. I can't work more than a few hours a week. I am constantly balancing and managing my symptoms. And yet, I can live independently most days of my life! Due to this, my quality of life has improved so much from where it was just two years ago. I would love to improve even more. I'd love to be able to work even 20 hours/week and exercise regularly. I'd love to be confident that I'll be able to raise a child one day. But if this is as good as it gets, I can accept it. And that's an amazing place for me to be in. :)

Wednesday, April 4, 2012

You're sick? What's wrong with you?

It's getting hard to keep track of the answer at this point. Even people that are aware of my illnesses don't understand how they cause my long and varied list of symptoms.

So, I'm updating the list of things wrong with me and what problems they cause me.

I have Chiari Malformation. This was diagnosed at age 18. It's a congenital birth defect. My skull is too small for my brain. This causes compression of the cerebellar tonsils and the back of the brain. The brain is compressed into the top of the spinal cord, which causes a blockage of craniospinal fluid (CSF). This causes increased pressure in the brain, which causes many neurological symptoms, and a heck of a headache. I've had two operations to treat this, at age 18 and 20. They removed part of my skull and my C-1 vertebra. It is as treated as it will ever be. Surgery is not a cure, but it prevents progression of symptoms. The neurological symptoms of this condition will be lifelong. This includes problems with balance (walking like a drunk person), coordination, cognitive function (commonly referred to as brain fog), memory, concentration, visual disturbances, neck pain, eye pain, jaw pain, spontaneous vertigo, changes in reflexes, etc. etc. etc. A list of symptoms can be found here:

I have Ehlers Danlos Syndrome. It was diagnosed at age 25. I have features of the hypermobile type and the classic type. It is a genetic connective tissue disorder. The collagen in the tissues is weak, fragile, and extra-stretchy. Symptoms of this are known to progress throughout life. Symptoms are seemingly endless, due to the important role that collagen plays in holding the body together and maintaining normal function.
This includes:
-Loose, unstable, and painful joints
-Early onset osteoarthritis
-Easy bruising
-Fragile and weak blood vessels--this causes difficulty maintaining blood pressure and oxygenation of the brain and other parts of the body
-Hypotonia (crappy and weak muscles)
-Muscle and joint pain
-Gastrointestinal dysfunction due to weak lining of the intestines
-Osteopenia/osteoporosis (crappy bones)
-So much pain throughout the body!!!
Wikipedia has a good article on it:
A more scientific explanation can be found here:

I have a form of dysautonomia called Postural Orthostatic Tachycardia Syndrome (POTS). I was diagnosed at age 19. Dysautonomia is a dysregulation of the autonomic nervous system. The autonomic nervous system regulates organ function throughout the body. Heart rate, blood pressure, temperature, digestion, and every other automatic function of the body can be affected. POTS is a common secondary condition related to EDS, due to the crappy blood vessels. It can also be related to Chiari, due to damage to the brainstem.
Symptoms of this condition are also widespread throughout the body. They include:
-Low blood pressure
-Shortness of breath
-Excessive sweating
-Functional bowel disorders
-Bladder dysfunction
-Pupillary dysfunction
-Exercise intolerance
-Body temperature control problems
-Generally feeling sick
A great explanation of this condition can be found here:

I have cranio-cervical instability. This was diagnosed at age 19. When someone with Ehlers Danlos Syndrome has surgery to treat Chiari, the skull becomes unstable. The treatment for this is a surgery called a cranio-cervical fusion. This surgery involves implanting titanium rods, screws, plates, and wires to support the skull by attaching it to the spine. My fusion goes from the skull to C-4. My first fusion was done at age 20. My second was done at age 26. The symptoms of this are devastating, causing complete disability. Symptoms include:
-Headache, dizziness, and nausea upon upright posture
-Severe autonomic dysfunction, particularly upon upright posture
A journal article explaining the connection between Chiari and EDS can be found here:

I have chronic migraines, which are distinct from my other types of headaches. this problem has worsened recently, and is being managed with medications, with some degree of success. Migraines can cause intense headaches, sensitivity to light and sound, pain upon exertion or movement, nausea, and generalized body pain.

I have a tethered spinal cord, specifically Occult Tight Filum Terminale. This was diagnosed at age 24. It means that the bottom of the spinal cord is pulled too tight. This causes nerve damage to the surrounding areas, especially the bowel and bladder. It was treated with a surgery called a section of the filum terminale. They removed part of my L-4 vertebra to access the base of the spinal cord, where they cut the spinal cord to separate it from the spine and relieve the tension. Again, surgery stops the progression of symptoms, but nerve damage is generally not reversible, so symptoms continue.

Sometimes my life is about trying to get better, and sometimes it's about learning to accept limitations and new and ongoing symptoms. Constant management of symptoms, through lifestyle changes, medication, and sometimes surgery is needed.

A problem I find when trying to explain this to people is that these conditions are just not heard of, so people really don't understand how much they affect me.

One way I've been thinking of it lately is comparing it to more common conditions where the symptoms are better understood by the majority of people.

These conditions have similar symptoms to my conditions:
Heart Disease
Irritable Bowel Syndrome
Chronic Migraines
Various neurological problems
Chronic Fatigue Syndrome
Chronic Pain Syndromes
etc. etc. etc.

So, my life is a lot like living with all of those conditions at the same time, plus some other more unique symptoms.

All of this, and many people in my life have no idea that I'm sick at all. I modify my lifestyle to deal with changes. I have to miss out on plenty of activities. But when I'm around people, I usually cover up the symptoms and slap on a smile. I try to hide my shaky hand, my dizzy spells, my racing heart, chills, hot flashes and constant pain. It's better than having to explain all of this to everyone! But it does get exhausting pretending to be healthy.