I thought I'd write about this disease here so that those interested could learn about it and understand how it affects my life. I have this disease, like I have blond hair or allergies- it doesn't have me! (The accompanying photo shows the effects of MG on my smile. Note how asymmetrical it is- more about that later...)
The facts;
Myasthenia Gravis (MG) is an autoimmune disease (usually inherited- thanks mom!) that occurs when the immune system, in the form of antibodies, attacks and damages the junction between nerve and muscle. These rogue antibodies target the acetycholine receptors, proteins on muscle cells required for muscle contraction. The ensuing damage to these receptors reduces the muscle's ability to respond to nerve signals. This results in muscle weakness. Muscles that control the eyes, face, throat, and limbs are affected. Symptoms in MG typically occur when the reduction in available acetylcholine receptor sites reaches 30 percent. Most symptoms are related to weakness of skeletal muscle groups, for instance, the muscles involved in climbing stairsor flexing the hands. Cardiac and smooth muscle are not affected. In about 20 percent of patients, vision alone is affected. Typical visual disturbances in MG include diploplia (double vision), ptosis (eyelid drooping or sagging) and blurred vision. I win the booby prize- I get all three! Other symptoms I have include weakness of neck muscles and proximal muscles such as the biceps and thighs, slack facial muscles, slurred speech, uncontrolled facial expressions, unstable or wobbly gait, shortness of breath, weakness in arms, hands, fingers and legs, vocal (nasal tone) changes, difficulty swallowing, chewing or talking, generalized weakness and limpness, absent gag reflex (could be a plus…), and respiratory weakness. About 75 percent of patients with MG have thymus gland abnormalities, thymus gland tumors (thymomas occur in about 15 percent of cases. About 10 percent of patients have thyroid gland disorders). Yes, I'm a cancer survivor too!
Treatment for MG targets different stages of the disease. Thymectomy -surgical removal of the thymus gland- located above the heart- through the sternum- ouch! I underwent this treatment three years ago in hopes that the abscent thymus, provider of the antibodies, would eventually result in a remission of the disease in 5-10 years. Cholinesterase (the antibody) inhibitors, like pyridostigmine bromide- which, by the way, is the same drug that our glorious government administers to victims of Gulf War Syndrome for toxic nerve gas damage. I take it every four hours so it can delay the breakdown of acetylcholine when it's released from the nerve endings. With me so far?
Intravenous immuno-globulin and plasma exchange are used to remove circulating antibodies and the cells that produce them from the blood. It's a bitch- like dialysis- and comes with extreme feelings of nausea and fatigue with a hint of pincushion! The mainstay of treatment (which I plan to avoid) is long-term immuno-suppression through the use of drugs like CellCept (originated for use in transplant patients to avoid organ rejection. One of the side effects..."vomiting coffee ground-like granules". Say wha...?), corticosteroids (can you say "moon face" and "shrunken testes" and "mood swings"?), Azathioprine (side effects equal upset stomach, vomiting, diarrhea, major liquid blowouts from both ends!, muscle aches, mouth sores, cough, lack of energy, loss of appetite, pain in the upper right part of the stomach, yellowing of the skin or eyes, flu-like symptoms, rash, blurred vision, stomach pain).
As per the "Steroid Therapy Consent Form" from my doctor, side effects of glucocorticoid therapy include, but are not limited to;
fluid retention, potassium loss, hypertension, steroid myopathy/muscle weakness(!!!), osteoporosis, bone fracture, hip joint degeneration, ulcers, nausea (like I don't have enough of that with the drugs I'm already taking...), abdominal distention, impaired wound healing, thin, fragile skin, bruising, increased sweating, dizziness, headache, hallucinations, personality change, insomnia, menstrual irregularities (no problems there), Cushing's syndrome (aka "moon face", decreased carbohydrate tolerance, manifestations of diabetes mellitus, cataracts and glaucoma. The doctors say this stuff is supposed to make me feel better! I mean, are they kidding?!
You'd be surprised how many of my friends don't ever ask about it, or the number of friends whom I've "lost" because of it. I've come to realize that seeing someone you know with a disease makes it all to difficult to pretend that bad things won't happen to good people if they don't see them. I can't blame them, I don't want to see it either.
Stress is the enemy of MG! Heat and cold are big stressers. It brings on all my symptoms.
Myastheniacs, oh, we lucky few, should not be offered gin & tonics- the quinine in the tonic can cause a toxic shock. And I'll have to grow old with all my wrinkles because Botox is lethal to myastheniacs ;o)
Effecting all voluntary muscles (that leaves only the heart unaffected) it really just makes it impossible to do just about anything. Buttoning shirts, tying shoelaces, holding a toothbrush, combing hair, using scissors, writing letters and filling out forms. I've rechoreographed my daily life to work around these- like keeping my shirts buttoned on the hangers and using them like pullovers- and laceless shoes or asking Robert to tie my shoes for me. Talk about humbling.....
It's eclipsed all my hobbies (long distance bike riding, calligraphy, gardening, long walks in the woods or the park). Dancing is out too- did you know that I was a professional ballet dancer? For nineteen years- all over the world. That's gone too. I can't watch a ballet without just bawling. It's not just a psychological thing. It's visceral, kinetic.
Myasthenia Gravis has made a "flake" out of me. I can't be counted on to do anything physical come a certain date or time. I may have a good day, but just as likely the MG will have its way. It's difficult for most folks to remember that I'm "sick” because I don't look diseased. Folks see me on my best days or I just don't let them see me at all. If I've planned to go out with friends, I'll stay home and take it easy to store up some reserves before hand. I know that vanity has got the best of me in this way, but it’s just too painful to hang with friends while only half of my face is working and the beer is dribbling from the corner of my mouth.
But my glass is still half full. Hell, I have two glasses and they're both full!! I still love life and I'm so happy to be alive!
The facts;
Myasthenia Gravis (MG) is an autoimmune disease (usually inherited- thanks mom!) that occurs when the immune system, in the form of antibodies, attacks and damages the junction between nerve and muscle. These rogue antibodies target the acetycholine receptors, proteins on muscle cells required for muscle contraction. The ensuing damage to these receptors reduces the muscle's ability to respond to nerve signals. This results in muscle weakness. Muscles that control the eyes, face, throat, and limbs are affected. Symptoms in MG typically occur when the reduction in available acetylcholine receptor sites reaches 30 percent. Most symptoms are related to weakness of skeletal muscle groups, for instance, the muscles involved in climbing stairsor flexing the hands. Cardiac and smooth muscle are not affected. In about 20 percent of patients, vision alone is affected. Typical visual disturbances in MG include diploplia (double vision), ptosis (eyelid drooping or sagging) and blurred vision. I win the booby prize- I get all three! Other symptoms I have include weakness of neck muscles and proximal muscles such as the biceps and thighs, slack facial muscles, slurred speech, uncontrolled facial expressions, unstable or wobbly gait, shortness of breath, weakness in arms, hands, fingers and legs, vocal (nasal tone) changes, difficulty swallowing, chewing or talking, generalized weakness and limpness, absent gag reflex (could be a plus…), and respiratory weakness. About 75 percent of patients with MG have thymus gland abnormalities, thymus gland tumors (thymomas occur in about 15 percent of cases. About 10 percent of patients have thyroid gland disorders). Yes, I'm a cancer survivor too!
Treatment for MG targets different stages of the disease. Thymectomy -surgical removal of the thymus gland- located above the heart- through the sternum- ouch! I underwent this treatment three years ago in hopes that the abscent thymus, provider of the antibodies, would eventually result in a remission of the disease in 5-10 years. Cholinesterase (the antibody) inhibitors, like pyridostigmine bromide- which, by the way, is the same drug that our glorious government administers to victims of Gulf War Syndrome for toxic nerve gas damage. I take it every four hours so it can delay the breakdown of acetylcholine when it's released from the nerve endings. With me so far?
Intravenous immuno-globulin and plasma exchange are used to remove circulating antibodies and the cells that produce them from the blood. It's a bitch- like dialysis- and comes with extreme feelings of nausea and fatigue with a hint of pincushion! The mainstay of treatment (which I plan to avoid) is long-term immuno-suppression through the use of drugs like CellCept (originated for use in transplant patients to avoid organ rejection. One of the side effects..."vomiting coffee ground-like granules". Say wha...?), corticosteroids (can you say "moon face" and "shrunken testes" and "mood swings"?), Azathioprine (side effects equal upset stomach, vomiting, diarrhea, major liquid blowouts from both ends!, muscle aches, mouth sores, cough, lack of energy, loss of appetite, pain in the upper right part of the stomach, yellowing of the skin or eyes, flu-like symptoms, rash, blurred vision, stomach pain).
As per the "Steroid Therapy Consent Form" from my doctor, side effects of glucocorticoid therapy include, but are not limited to;
fluid retention, potassium loss, hypertension, steroid myopathy/muscle weakness(!!!), osteoporosis, bone fracture, hip joint degeneration, ulcers, nausea (like I don't have enough of that with the drugs I'm already taking...), abdominal distention, impaired wound healing, thin, fragile skin, bruising, increased sweating, dizziness, headache, hallucinations, personality change, insomnia, menstrual irregularities (no problems there), Cushing's syndrome (aka "moon face", decreased carbohydrate tolerance, manifestations of diabetes mellitus, cataracts and glaucoma. The doctors say this stuff is supposed to make me feel better! I mean, are they kidding?!
You'd be surprised how many of my friends don't ever ask about it, or the number of friends whom I've "lost" because of it. I've come to realize that seeing someone you know with a disease makes it all to difficult to pretend that bad things won't happen to good people if they don't see them. I can't blame them, I don't want to see it either.
Stress is the enemy of MG! Heat and cold are big stressers. It brings on all my symptoms.
Myastheniacs, oh, we lucky few, should not be offered gin & tonics- the quinine in the tonic can cause a toxic shock. And I'll have to grow old with all my wrinkles because Botox is lethal to myastheniacs ;o)
Effecting all voluntary muscles (that leaves only the heart unaffected) it really just makes it impossible to do just about anything. Buttoning shirts, tying shoelaces, holding a toothbrush, combing hair, using scissors, writing letters and filling out forms. I've rechoreographed my daily life to work around these- like keeping my shirts buttoned on the hangers and using them like pullovers- and laceless shoes or asking Robert to tie my shoes for me. Talk about humbling.....
It's eclipsed all my hobbies (long distance bike riding, calligraphy, gardening, long walks in the woods or the park). Dancing is out too- did you know that I was a professional ballet dancer? For nineteen years- all over the world. That's gone too. I can't watch a ballet without just bawling. It's not just a psychological thing. It's visceral, kinetic.
Myasthenia Gravis has made a "flake" out of me. I can't be counted on to do anything physical come a certain date or time. I may have a good day, but just as likely the MG will have its way. It's difficult for most folks to remember that I'm "sick” because I don't look diseased. Folks see me on my best days or I just don't let them see me at all. If I've planned to go out with friends, I'll stay home and take it easy to store up some reserves before hand. I know that vanity has got the best of me in this way, but it’s just too painful to hang with friends while only half of my face is working and the beer is dribbling from the corner of my mouth.
But my glass is still half full. Hell, I have two glasses and they're both full!! I still love life and I'm so happy to be alive!
