The Spoon Theory written by Christine Miserandino

The Spoon Theory

by Christine Miserandino www.butyoudontlooksick.com

My best friend and I were in the diner, talking. As usual, it was very late and we were eating French fries with gravy. Like normal girls our age, we spent a lot of time in the diner while in college, and most of the time we spent talking about boys, music or trivial things, that seemed very important at the time. We never got serious about anything in particular and spent most of our time laughing.

As I went to take some of my medicine with a snack as I usually did, she watched me with an awkward kind of stare, instead of continuing the conversation. She then asked me out of the blue what it felt like to have Lupus and be sick. I was shocked not only because she asked the random question, but also because I assumed she knew all there was to know about Lupus. She came to doctors with me, she saw me walk with a cane, and throw up in the bathroom. She had seen me cry in pain, what else was there to know?
I started to ramble on about pills, and aches and pains, but she kept pursuing, and didn’t seem satisfied with my answers. I was a little surprised as being my roommate in college and friend for years; I thought she already knew the medical definition of Lupus. Then she looked at me with a face every sick person knows well, the face of pure curiosity about something no one healthy can truly understand. She asked what it felt like, not physically, but what it felt like to be me, to be sick.
As I tried to gain my composure, I glanced around the table for help or guidance, or at least stall for time to think. I was trying to find the right words. How do I answer a question I never was able to answer for myself? How do I explain every detail of every day being effected, and give the emotions a sick person goes through with clarity. I could have given up, cracked a joke like I usually do, and changed the subject, but I remember thinking if I don’t try to explain this, how could I ever expect her to understand. If I can’t explain this to my best friend, how could I explain my world to anyone else? I had to at least try.
At that moment, the spoon theory was born. I quickly grabbed every spoon on the table; hell I grabbed spoons off of the other tables. I looked at her in the eyes and said “Here you go, you have Lupus”. She looked at me slightly confused, as anyone would when they are being handed a bouquet of spoons. The cold metal spoons clanked in my hands, as I grouped them together and shoved them into her hands.
I explained that the difference in being sick and being healthy is having to make choices or to consciously think about things when the rest of the world doesn’t have to. The healthy have the luxury of a life without choices, a gift most people take for granted.
Most people start the day with unlimited amount of possibilities, and energy to do whatever they desire, especially young people. For the most part, they do not need to worry about the effects of their actions. So for my explanation, I used spoons to convey this point. I wanted something for her to actually hold, for me to then take away, since most people who get sick feel a “loss” of a life they once knew. If I was in control of taking away the spoons, then she would know what it feels like to have someone or something else, in this case Lupus, being in control.
She grabbed the spoons with excitement. She didn’t understand what I was doing, but she is always up for a good time, so I guess she thought I was cracking a joke of some kind like I usually do when talking about touchy topics. Little did she know how serious I would become?
I asked her to count her spoons. She asked why, and I explained that when you are healthy you expect to have a never-ending supply of “spoons”. But when you have to now plan your day, you need to know exactly how many “spoons” you are starting with. It doesn’t guarantee that you might not lose some along the way, but at least it helps to know where you are starting. She counted out 12 spoons. She laughed and said she wanted more. I said no, and I knew right away that this little game would work, when she looked disappointed, and we hadn’t even started yet. I’ve wanted more “spoons” for years and haven’t found a way yet to get more, why should she? I also told her to always be conscious of how many she had, and not to drop them because she can never forget she has Lupus.
I asked her to list off the tasks of her day, including the most simple. As, she rattled off daily chores, or just fun things to do; I explained how each one would cost her a spoon. When she jumped right into getting ready for work as her first task of the morning, I cut her off and took away a spoon. I practically jumped down her throat. I said ” No! You don’t just get up. You have to crack open your eyes, and then realize you are late. You didn’t sleep well the night before. You have to crawl out of bed, and then you have to make your self something to eat before you can do anything else, because if you don’t, you can’t take your medicine, and if you don’t take your medicine you might as well give up all your spoons for today and tomorrow too.” I quickly took away a spoon and she realized she hasn’t even gotten dressed yet. Showering cost her spoon, just for washing her hair and shaving her legs. Reaching high and low that early in the morning could actually cost more than one spoon, but I figured I would give her a break; I didn’t want to scare her right away. Getting dressed was worth another spoon. I stopped her and broke down every task to show her how every little detail needs to be thought about. You cannot simply just throw clothes on when you are sick. I explained that I have to see what clothes I can physically put on, if my hands hurt that day buttons are out of the question. If I have bruises that day, I need to wear long sleeves, and if I have a fever I need a sweater to stay warm and so on. If my hair is falling out I need to spend more time to look presentable, and then you need to factor in another 5 minutes for feeling badly that it took you 2 hours to do all this. I think she was starting to understand when she theoretically didn’t even get to work, and she was left with 6 spoons. I then explained to her that she needed to choose the rest of her day wisely, since when your “spoons” are gone, they are gone. Sometimes you can borrow against tomorrow’s “spoons”, but just think how hard tomorrow will be with less “spoons”. I also needed to explain that a person who is sick always lives with the looming thought that tomorrow may be the day that a cold comes, or an infection, or any number of things that could be very dangerous. So you do not want to run low on “spoons”, because you never know when you truly will need them. I didn’t want to depress her, but I needed to be realistic, and unfortunately being prepared for the worst is part of a real day for me.
We went through the rest of the day, and she slowly learned that skipping lunch would cost her a spoon, as well as standing on a train, or even typing at her computer too long. She was forced to make choices and think about things differently. Hypothetically, she had to choose not to run errands, so that she could eat dinner that night.
When we got to the end of her pretend day, she said she was hungry. I summarized that she had to eat dinner but she only had one spoon left. If she cooked, she wouldn’t have enough energy to clean the pots. If she went out for dinner, she might be too tired to drive home safely. Then I also explained, that I didn’t even bother to add into this game, that she was so nauseous, that cooking was probably out of the question anyway. So she decided to make soup, it was easy. I then said it is only 7pm, you have the rest of the night but maybe end up with one spoon, so you can do something fun, or clean your apartment, or do chores, but you can’t do it all.
I rarely see her emotional, so when I saw her upset I knew maybe I was getting through to her. I didn’t want my friend to be upset, but at the same time I was happy to think finally maybe someone understood me a little bit. She had tears in her eyes and asked quietly “Christine, How do you do it? Do you really do this everyday?” I explained that some days were worse then others; some days I have more spoons then most. But I can never make it go away and I can’t forget about it, I always have to think about it. I handed her a spoon I had been holding in reserve. I said simply, “I have learned to live life with an extra spoon in my pocket, in reserve. You need to always be prepared.” Its hard, the hardest thing I ever had to learn is to slow down, and not do everything. I fight this to this day. I hate feeling left out, having to choose to stay home, or to not get things done that I want to. I wanted her to feel that frustration. I wanted her to understand, that everything everyone else does comes so easy, but for me it is one hundred little jobs in one. I need to think about the weather, my temperature that day, and the whole day’s plans before I can attack any one given thing. When other people can simply do things, I have to attack it and make a plan like I am strategizing a war. It is in that lifestyle, the difference between being sick and healthy. It is the beautiful ability to not think and just do. I miss that freedom. I miss never having to count “spoons”.
After we were emotional and talked about this for a little while longer, I sensed she was sad. Maybe she finally understood. Maybe she realized that she never could truly and honestly say she understands. But at least now she might not complain so much when I can’t go out for dinner some nights, or when I never seem to make it to her house and she always has to drive to mine. I gave her a hug when we walked out of the diner. I had the one spoon in my hand and I said “Don’t worry. I see this as a blessing. I have been forced to think about everything I do. Do you know how many spoons people waste everyday? I don’t have room for wasted time, or wasted “spoons” and I chose to spend this time with you.”
Ever since this night, I have used the spoon theory to explain my life to many people. In fact, my family and friends refer to spoons all the time. It has been a code word for what I can and cannot do. Once people understand the spoon theory they seem to understand me better, but I also think they live their life a little differently too. I think it isn’t just good for understanding Lupus, but anyone dealing with any disability or illness. Hopefully, they don’t take so much for granted or their life in general. I give a piece of myself, in every sense of the word when I do anything. It has become an inside joke. I have become famous for saying to people jokingly that they should feel special when I spend time with them, because they have one of my “spoons”.
© Christine Miserandino

Get worse with activity and improve with rest Myasthenia Gravis September 30, 2015 Lussy williams

Myasthenia Gravis (MG) can affect any of the muscles that you control voluntarily. It can affect muscles of the face, hands, eyes, arms and legs and those muscles involved in chewing, swallowing and talking. Muscles that control breathing and neck movement also can be affected.

MG does not affect involuntary muscles such as the heart, smooth muscles of the gut, blood vessels, and uterus.

The hallmark of weakness related to MG is that is worsens with sustained activity of the involved muscle(s). Eye muscle weakness worsens with reading, and double vision may improve with a brief rest. When speech is affected, symptoms worsen with prolonged talking. Extremity weakness if often noticed when holding arms over the head. Drooping of the eyelids, neck weakness and other symptoms are usually worse at the end of the day.

Symptoms, which vary in type, severity and combination, may include:

• Drooping of one or both eyelids
• Double or blurred vision
• Weakness in arms, hands, fingers, neck, face or legs
• Difficulty in chewing, smiling, swallowing or talking
• Excessive fatigue in exercised muscle groups
• Shortness of breath, difficulty taking a deep breath or coughing

MG is often called the “snowflake disease” because it differs so much from person to person. The degree of muscle weakness and the muscles that are affected vary greatly from patient to patient and from time to time.

For most people, the first noticeable symptom is weakness of the eye muscles causing drooping eyelids or double vision. In others, difficulty in swallowing and slurred speech may be the first signs. The onset of the disease is usually gradual over weeks or months, but may be more sudden. Symptoms may come and go over time, and even resolve completely for months or longer. Symptoms often are not immediately recognized as MG, especially if they are subtle or variable.

Most individuals do not develop all of the symptoms of MG. In some patients weakness remains limited to the eyes for entire course of the disease. These patients have ocular MG. Most patients have generalized MG where symptoms also involve muscles besides the eyes. When muscles involving speech or swallowing are involved, this is sometimes called bulbar weakness.

MG symptoms differ somewhat for MG patients who test positive for MuSK antibodies. Most MuSK MG patients are women, and tend to have more severe symptoms. Researchers have found that this group of patients often require higher doses of prednisone, and tend to improve more with plasmapheresis than IVIg treatments.

The course of MG during pregnancy is hard to predict. For some women, symptoms worsen; in others symptoms stay the same or improve. Because MG increases risk during pregnancy and MG medications pose risks to an unborn child, a woman with MG should discuss pregnancy with her doctor in advance.

If a person’s ability to breathe, cough, or protect their airway becomes insufficient, it’s called a myasthenic crisis. These patients require prompt treatment, and may need mechanical breathing assistance in a hospital for a period of time until their strength improves. While most myasthenics never experience a crisis, those who have trouble swallowing and talking are the ones most likely also to have trouble breathing. Progressive warning signs that swallowing, talking, and breathing are becoming compromised should be addressed immediately.

Myasthenic CrisisAdded by Matthew M Hastings , last edited by Matthew M Hastings on Feb 01, 2012

Monitoring

• IF IN ANY DOUBT, GO SEE THE PATIENT!!!

• Monitor respiratory parameters every few hours.
  • NIF (it should be more negative than -30 in general)
  • Vital cap (concerning when <10-12 mg/kg)
  • Other ways to assess respiratory status
    • Quality of cough
    • Ability to count out loud in one breath
  • These are partially driven by patient effort, so use these in combination. No single number or sign can substitute for a good exam and your general impression.

Time to move to the ICU?

• If you are worried about the patient's respiratory status at all, get them to the ICU. Myasthenia gravis patients can crash quickly and without much warning!

• Do NOT rely on the O2 sat or ABG to predict a crash. This may not change until it is too late!

• Consider BiPAP in ICU to try to avoid need for intubation.
  

  Work-up

• Perform infectious work-up for every myasthenic crisis with:
  • Blood cultures
  • Urine cultures
  • CXR
• Consider non-contrast chest CT for thymoma

  Treatment

• Acetylcholinesterase Inhibitors
  • Pyridostigmine (Mestinon and Mestinon Timespan (extended release))
    • Symptomatic treatment only.
    • Unnecessary in intubated patients; in fact, increase secretions.
    • You can provide mestinon, but not mestinon timespan, via dobhoff
    • Conversion of PO pyridostigmine:IV pyridostigmine is 30:1
• Glycopyrrolate (Robinul) to decrease secretions
• Steroids
  • High dose steroids can initially cause worsening! But later can help suppress immune response.
  • If already intubated, start high dose steroids (prednisone 60 mg PO qAM or solu-medrol 500 mg IV qAM)
• IVIg
  • Used acutely in crisis or for maintenance
  • Typical load for MG
    • If old, renal insufficiency, or severe cardiac disease & hypertension: 0.5 gram/kg over 4 days
    • If young and no renal or cardiac disease: 1 gm/kg daily x 2 days
  • If the patient has known renal disease and you must use IVIg:
    • Use the lysine based Gammagard formulation
    • Avoid concurrent use of nephrotoxic medications
    • Avoid concurrent IV contrast
    • Aggressively hydrate the patient during IVIg treatments
• Plasmapheresis
  • Used acutely in crisis or for maintenance
  • QOD x 5 treatments, and then reevaluate for need for further series
  • See AIDP section for more information on apheresis.
• Other immunosuppressants for maintenance
  • Mycofenolate mofetil (Cellcept)
  • Azathioprine (Imuran)

Myasthenia Gravis Early Sings and Symptoms October 5, 2015 In category Womens Health

Myasthenia gravis (MG) weakens and fatigues the body’s voluntary muscles (those we can move at will). It doesn’t damage the musculature of the heart or the gastrointestinal tract.

Early in its course, MG tends to affect the muscles that control movement of the eyes and eyelids, causingocular weakness. Consequently, a partial paralysis of eye movements (ophthalmoparesis), double vision (diplopia) and droopy eyelids (ptosis) are usually among the first symptoms of MG.

Weakness and fatigue in the neck and jaw also can occur early in MG. This bulbar weakness — named for the nerves that originate from the bulblike part of the brainstem — can make it difficult to talk, chew, swallow and hold up the head.

Bulbar weakness tends to give speech a slurred, nasal quality. It also can lead to frequent choking spells, and make eating unpleasant and tiresome.

In generalized MG, weakness tends to spread sequentially from the face and neck to the upper limbs, the hands and then the lower limbs. It may become difficult to lift the arms over the head, rise from a sitting position, walk long distances, climb stairs or grip heavy objects.

In some cases, weakness may spread to muscles in the chest that control breathing.

Disease course

Weakness and fatigue in MG tend to fluctuate from day to day, and even during a single day. People with the disease are often strongest in the morning after a full night’s sleep and weakest in the evening.

Over a longer term, the symptoms of MG usually progress, reaching maximum or near-maximum severity within one to three years of onset in most people. In about 15 percent of people, the disease remains ocular, but in most it becomes oculobulbar or generalized. If the disease remains ocular for three years, it usually doesn’t become generalized.

Weakness serious enough to require full-time wheelchair use is not common in MG. Most people, when properly treated, find they can remain physically active.

Remission, a reversal of some or all symptoms, occurs in about 20 percent of people with MG. Usually, the remissions are temporary, with an average duration of five years, but some people experience more than one remission during their lifetime. A few people have experienced apparently permanent remissions, lasting more than 20 years.

Compared to adult-onset MG, juvenile MG tends to progress more slowly and has a higher incidence of remission. Historically, many children given diagnoses of juvenile MG turned out to have a congenital myasthenic syndrome.

21 Energy-Saving Tips to Ease Life With Chronic Illness

By Lynda Shrager

Published Jul 28, 2014

Sometimes, even the simplest of tasks may seem daunting.
Especially for people living with a chronic illness, or those just coming home after hospitalization, weakness, limited joint motion, compromised breathing, and decreased endurance are common.
As an occupational therapist, my focus is to teach my patients how to combat fatigue so they can participate in more activities within their physical limits. One method I like uses energy conservation and work simplification techniques to fight fatigue.

Conserve Your Energy for the Important Things in Life

The first thing you need to learn is how to pace yourself.  You can do this by planning ahead and organizing your daily schedule with a balance of strenuous and easy tasks. Leave ample time to complete your tasks so you don’t feel rushed and under pressure.
For example, I suggest to my patients that they avoid early-morning doctor’s appointments as the pressure of getting up, bathing, eating breakfast and getting to the doctor on time can create a stressful scenario. Rather, try to get the first appointment following the staff’s lunch break as they won’t be running behind schedule yet. This gives you plenty of time in the morning to get everything done and still get there on time.

Work Simplification: Sit Wisely, Prioritize, Delegate

The principles of work simplification include sitting to work as much as possible.  When you’re sitting, choose a firm surface with arm rests.  Soft couches and chairs, although tempting, are hard to get out of.  Organize work areas so the bulk of activity occurs at waist height and close to your body, so you can avoid bending, over-reaching, and stooping.  Instead of lifting heavy objects, slide them.  Avoid strenuous arm motions.  If you must pick up objects, bend your knees or squat rather than bending from your back.
Prioritize important tasks, and delegate to others whenever possible.  Remember to save your energy for things you most want to do.  Get to know your own activity tolerance, and stop if you feel symptoms of fatigue such as muscle stiffness, weakness, and shortness of breath.

Try These Strategies to Save Time and Energy

Use the following organizing tips to conserve energy and complete daily tasks and self-care in the most effective way:
Self-care activities in the bathroom:

1. Sit in front of the sink for hair care, makeup, and shaving.
2. Use a long-handled bath brush for your back and feet.
3. Wrap yourself in a terrycloth robe rather than using towels to dry yourself.
4. Sit on a shower bench and use a hand-held shower hose.
5. Keep your hair in an easy-to-care-for style.
6. Use a raised toilet seat and/or toilet safety rails.

When you’re dressing:

1. Gather all of your clothes before you start getting dressed.
2. Sit on a bed or a chair to dress.
3. Dress your lower body first as this takes more energy.
4. Put your underwear on, then pull it up to your knees; put your pants on, then pull them up to your knees; stand once and pull both up at the same time.
5. Choose front-opening, loose-fitting clothes.
6. Choose clothes with elastic waistbands because they’re easiest to manage.
7. If you can’t use slip-on shoes, consider elastic shoe laces.  Bring your feet up to your tie laces; footstools are helpful for this.  Use long-handled shoe horns to avoid bending.
8. If a limb is sore, weak, or otherwise compromised, put that arm or leg into the shirt or pants first and take it out second when you’re undressing.

In the kitchen:

1. Store items according to use, putting most-used items at waist level or above and less-used items below waist level.
2. Consider leaving your most-used items out on the counter.
3. Plan one-dish meals, consider using a Crockpot or other slow cooker, and try prepared mixes and frozen foods.
4. Use a wheeled cart for carrying food from the kitchen to the table and cleaning up after.
5. Slide filled pots, mugs, and other containers along countertops or the stove rather than lifting them.
6. Ease food prep using electric appliances.
7. Use disposable paper goods and utensils to minimize clean-up.

Top Detox Foods To Help The Immune System Help Myasthenia Gravis October 2, 2015 In category Womens Health

Myasthenia gravis is a chronic autoimmune neuromuscular disease, with symptoms of weakness and fatigue of the skeletal (voluntary) muscles of the body, in particular, the eye muscles, the face and throat muscles, as well as muscles in the arms and legs. Oddly enough, after periods of rest, some of the symptoms may improve. Having said that, myasthenia gravis affects the way of everyday living, such as vision, facial expression (facial muscles), breathing, talking, chewing and swallowing, as well as body movements.

Myasthenia gravis is only one of the many autoimmune diseases affecting humans. At present, the medical community is still very much uncertain about how an autoimmune disease may develop in an individual, although there is increasing evidence linking environmental agents to autoimmune diseases. These environmental agents include infectious agents, such as viruses, pharmaceutical and chemical agents, heavy metals, dietary factors, as well as a number of biological agents, including the genetic factor.

The human immune system is complicated, and, as such, affects the whole body system in many different ways. A healthy immune system protects the body, while an impaired immune system opens the door to diseases and viruses. It is, therefore, important to have a healthy immune system, without which the body’s own innate self-healing mechanism may become dysfunctional. It is believed that an autoimmune disease is caused by a dysfunctional immune system attacking the body system, instead of attacking external invaders into the body.

The first step to protect the immune system is to detox the body. You may have a toxic body when you often experience fatigue, headache, insomnia, indigestion, and all types of physical pain. Even if you do not have those symptoms, strengthening the immune system will reduce many of the symptoms of myasthenia gravis.

Some of the top detox foods to help the immune system help myasthenia gravis are as follows:

Seaweed: It contains high doses of minerals, such as calcium, iodine, iron, and magnesium, which bind with radioactive wastes from polluted soils and waters, according to research at McGill University in Canada. Seaweed is an inexpensive sea vegetable. Use seaweed in soup.

Avocado: It is rich in glutathione antioxidant, which is effective in removing toxins from too much alcohol consumption. Eat an avocado for breakfast; it is filling and saves you time.

Kiwi fruit: Like avocado, kiwi fruit is loaded with glutathione. In addition, it is rich in vitamin C. It is a powerful antioxidant to protect the immune system.

Apple: An apple a day keeps the doctor away. There is truth in that: apple is a powerful antioxidant and toxin remover due to its vitamin C and its fiber, pectin. Eat an apple everyday to keep your immune system healthy.

Beet: It contains methionine, which purifies natural waste products from the body. Beet has been used for centuries to purify blood. Make beet juice with carrot, or eat it raw in a salad.

Garlic: It contains allicin, which is a potent purifier of mercury and other toxic chemicals found in food additives. In addition, garlic alkalizes the body, making it more efficient in resisting disease. Put crushed garlic in all your cooking. If you wish to remove the odor from the breath due to garlic, chew some fresh parsley for a fresher breath.

Artichoke: It increases bile production to facilitate bowel movement. Steam artichoke and serve with meted butter.

Watercress: It increases detox enzymes. Watercress is especially effective in removing carcinogens from smokers, according to a UK study. Steam or put watercress in soup.

Cruciferous vegetable: Brussels sprouts, cabbage, cauliflower, and spinach are all effective in enhancing the liver in its production of enzymes for digestion and elimination. Eat them as much and as often as you can, either cooked or raw.

Strengthening the immune system is better than taking medications to deal with a compromised immune system.

Myasthenia Gravis Fact Sheet October 11, 2015 Lussy williams

What is myasthenia gravis?

Myasthenia gravis is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body. The name myasthenia gravis, which is Latin and Greek in origin, literally means “grave muscle weakness.” With current therapies, however, most cases of myasthenia gravis are not as “grave” as the name implies. In fact, most individuals with myasthenia gravis have a normal life expectancy.
The hallmark of myasthenia gravis is muscle weakness that increases during periods of activity and improves after periods of rest. Certain muscles such as those that control eye and eyelid movement, facial expression, chewing, talking, and swallowing are often, but not always, involved in the disorder. The muscles that control breathing and neck and limb movements may also be affected.
 

What causes myasthenia gravis?

Myasthenia gravis is caused by a defect in the transmission of nerve impulses to muscles. It occurs when normal communication between the nerve and muscle is interrupted at the neuromuscular junction—the place where nerve cells connect with the muscles they control. Normally when impulses travel down the nerve, the nerve endings release a neurotransmitter substance called acetylcholine. Acetylcholine travels from the neuromuscular junction and binds to acetylcholine receptors which are activated and generate a muscle contraction.
In myasthenia gravis, antibodies block, alter, or destroy the receptors for acetylcholine at the neuromuscular junction, which prevents the muscle contraction from occurring. These antibodies are produced by the body’s own immune system. Myasthenia gravis is an autoimmune disease because the immune system—which normally protects the body from foreign organisms—mistakenly attacks itself.

What is the role of the thymus gland in myasthenia gravis?

The thymus gland, which lies in the chest area beneath the breastbone, plays an important role in the development of the immune system in early life. Its cells form a part of the body’s normal immune system. The gland is somewhat large in infants, grows gradually until puberty, and then gets smaller and is replaced by fat with age. In adults with myasthenia gravis, the thymus gland remains large and is abnormal. It contains certain clusters of immune cells indicative of lymphoid hyperplasia—a condition usually found only in the spleen and lymph nodes during an active immune response. Some individuals with myasthenia gravis develop thymomas (tumors of the thymus gland). Thymomas are generally benign, but they can become malignant.
The relationship between the thymus gland and myasthenia gravis is not yet fully understood. Scientists believe the thymus gland may give incorrect instructions to developing immune cells, ultimately resulting in autoimmunity and the production of the acetylcholine receptor antibodies, thereby setting the stage for the attack on neuromuscular transmission.

What are the symptoms of myasthenia gravis?

Although myasthenia gravis may affect any voluntary muscle, muscles that control eye and eyelid movement, facial expression, and swallowing are most frequently affected. The onset of the disorder may be sudden and symptoms often are not immediately recognized as myasthenia gravis.
In most cases, the first noticeable symptom is weakness of the eye muscles. In others, difficulty in swallowing and slurred speech may be the first signs. The degree of muscle weakness involved in myasthenia gravis varies greatly among individuals, ranging from a localized form limited to eye muscles (ocular myasthenia), to a severe or generalized form in which many muscles—sometimes including those that control breathing—are affected. Symptoms, which vary in type and severity, may include a drooping of one or both eyelids (ptosis), blurred or double vision (diplopia) due to weakness of the muscles that control eye movements, unstable or waddling gait, a change in facial expression, difficulty in swallowing, shortness of breath, impaired speech (dysarthria), and weakness in the arms, hands, fingers, legs, and neck.

Who gets myasthenia gravis?

Myasthenia gravis occurs in all ethnic groups and both genders. It most commonly affects young adult women (under 40) and older men (over 60), but it can occur at any age.
In neonatal myasthenia, the fetus may acquire immune proteins (antibodies) from a mother affected with myasthenia gravis. Generally, cases of neonatal myasthenia gravis are temporary and the child’s symptoms usually disappear within 2-3 months after birth. Other children develop myasthenia gravis indistinguishable from adults. Myasthenia gravis in juveniles is uncommon.
Myasthenia gravis is not directly inherited nor is it contagious. Occasionally, the disease may occur in more than one member of the same family.
Rarely, children may show signs of congenital myasthenia or congenital myasthenic syndrome. These are not autoimmune disorders, but are caused by defective genes that produce abnormal proteins instead of those which normally would produce acetylcholine, acetylcholinesterase (the enzyme that breaks down acetylcholine), or the acetylcholine receptor and other proteins present along the muscle membrane.

How is myasthenia gravis diagnosed?

Because weakness is a common symptom of many other disorders, the diagnosis of myasthenia gravis is often missed or delayed (sometimes up to two years) in people who experience mild weakness or in those individuals whose weakness is restricted to only a few muscles.
The first steps of diagnosing myasthenia gravis include a review of the individual’s medical history, and physical and neurological examinations. The physician looks for impairment of eye movements or muscle weakness without any changes in the individual’s ability to feel things. If the doctor suspects myasthenia gravis, several tests are available to confirm the diagnosis.
A special blood test can detect the presence of immune molecules or acetylcholine receptor antibodies. Most patients with myasthenia gravis have abnormally elevated levels of these antibodies. Recently, a second antibody—called the anti-MuSK antibody—has been found in about 30 to 40 percent of individuals with myasthenia gravis who do not have acetylcholine receptor antibodies. This antibody can also be tested for in the blood. However, neither of these antibodies is present in some individuals with myasthenia gravis, most often in those with ocular myasthenia gravis.
The edrophonium test uses intravenous administration of edrophonium chloride to very briefly relieve weakness in people with myasthenia gravis. The drug blocks the degradation (breakdown) of acetylcholine and temporarily increases the levels of acetylcholine at the neuromuscular junction. Other methods to confirm the diagnosis include a version of nerve conduction study which tests for specific muscle “fatigue” by repetitive nerve stimulation. This test records weakening muscle responses when the nerves are repetitively stimulated by small pulses of electricity. Repetitive stimulation of a nerve during a nerve conduction study may demonstrate gradual decreases of the muscle action potential due to impaired nerve-to-muscle transmission.
Single fiber electromyography (EMG) can also detect impaired nerve-to-muscle transmission. EMG measures the electrical potential of muscle cells when single muscle fibers are stimulated by electrical impulses. Muscle fibers in myasthenia gravis, as well as other neuromuscular disorders, do not respond as well to repeated electrical stimulation compared to muscles from normal individuals.
Diagnostic imaging of the chest, using computed tomography (CT) or magnetic resonance imaging (MRI), may be used to identify the presence of a thymoma.
Pulmonary function testing, which generally call for immediate medical attention—may be triggered by infection, fever, or an adverse reaction to medication.

What are myasthenic crises?

A myasthenic crisis occurs when the muscles that control breathing weaken to the point that ventilation is inadequate, creating a medical emergency and requiring a respirator for assisted ventilation. In individuals whose respiratory muscles are weak, crises—which generally call for immediate medical attention—may be triggered by infection, fever, or an adverse reaction to medication.

h measures breathing strength, helps to predict whether respiration may fail and lead to a myasthenic crisis.

How is myasthenia gravis treated?

Today, myasthenia gravis can generally be controlled. There are several therapies available to help reduce and improve muscle weakness. Medications used to treat the disorder include anticholinesterase agents such as neostigmine and pyridostigmine, which help improve neuromuscular transmission and increase muscle strength. Immunosuppressive drugs such as prednisone, azathioprine, cyclosporin, mycophenolate mofetil, and tacrolimus may also be used. These medications improve muscle strength by suppressing the production of abnormal antibodies. Their use must be carefully monitored by a physician because they may cause major side effects.
Thymectomy, the surgical removal of the thymus gland (which often is abnormal in individuals with myasthenia gravis), reduces symptoms in some individuals without thymoma and may cure some people, possibly by re-balancing the immune system. Thymectomy is recommended for individuals with thymoma. Other therapies used to treat myasthenia gravis include plasmapheresis, a procedure in which serum containing the abnormal antibodies is removed from the blood while cells are replaced, and high-dose intravenous immune globulin, which temporarily modifies the immune system by infusing antibodies from donated blood. These therapies may be used to help individuals during especially difficult periods of weakness. A neurologist will determine which treatment option is best for each individual depending on the severity of the weakness, which muscles are affected, and the individual’s age and other associated medical problems.

What is the prognosis?

With treatment, most individuals with myasthenia can significantly improve their muscle weakness and lead normal or nearly normal lives. Some cases of myasthenia gravis may go into remission—either temporarily or permanently—and muscle weakness may disappear completely so that medications can be discontinued. Stable, long-lasting complete remissions are the goal of thymectomy and may occur in about 50 percent of individuals who undergo this procedure. In a few cases, the severe weakness of myasthenia gravis may cause respiratory failure, which requires immediate emergency medical care

What research is being done?

Within the Federal government, the National Institute of Neurological Disorders and Stroke (NINDS), one of the National Institutes of Health (NIH), has primary responsibility for conducting and supporting research on brain and nervous system disorders, including myasthenia gravis.
Much has been learned about myasthenia gravis in recent years. Technological advances have led to more timely and accurate diagnosis, and new and enhanced therapies have improved management of the disorder. There is a greater understanding about the structure and function of the neuromuscular junction, the fundamental aspects of the thymus gland and of autoimmunity, and the disorder itself. Despite these advances, however, there is still much to learn. Researchers are seeking to learn what causes the autoimmune response in myasthenia gravis, and to better define the relationship between the thymus gland and myasthenia gravis.
Different drugs are being tested, either alone or in combination with existing drug therapies, to see if they are effective in treating myasthenia gravis. One study is examining the use of methotrexate therapy in individuals who develop symptoms and signs of the disease while on prednisone therapy. The drug suppresses blood cell activity that causes inflammation. Another study is investigating the use of rituximab, a monoclonal antibody against B cells which make antibodies, to see if it decreases certain antibodies that cause the immune system to attack the nervous system. Investigators are also determining if eculizumab is safe and effective in treating individuals with generalized myasthenia gravis who also receive various immunosuppressant drugs.
Another study seeks further understanding of the molecular basis of synaptic transmission in the nervous system. The objective of this study is to expand current knowledge of the function of receptors and to apply this knowledge to the treatment of myasthenia gravis.
Thymectomy is also being studied in myasthenia gravis patients who do not have thymoma to assess long-term benefit the surgical procedure may have over medical therapy alone.
One study involves blood sampling to see if the immune system is making antibodies against components of the nerves and muscle. Researchers also hope to learn if these antibodies contribute to the development or worsening of myasthenia gravis and other illnesses of the nervous system.
Investigators are also examining the safety and efficacy of autologous hematopoietic stem cell transplantation to treat refractory and severe myasthenia gravis. Participants in this study will receive several days of treatment using the immumosuppressant drugs cyclophosphamide and antithymocyte globulin before having some of their peripheral blood cells harvested and frozen. The blood cells will later be thawed and infused intravenously into the respective individuals, whose symptoms will be monitored for five years.

Myasthenia Gravis Overview, Types, Incidence and Prevalence October 13, 2015 Lussy williams

Overview of Myasthenia Gravis

Myasthenia gravis (MG) is a chronic autoimmune disorder that results in progressive skeletal muscle weakness. Skeletal muscles are primarily muscle fibers that contain bands or striations (striated muscles) that are connected to bone. MG causes rapid fatigue (fatigability) and loss of strength upon exertion that improves after rest.

In early stages, myasthenia gravis primarily affects muscles that control eye movement (extraocular muscles) and those that control facial expression, chewing, and swallowing. If untreated, the disorder may affect muscles that control breathing (respiration), causing acute respiratory failure.

Types of Myasthenia Gravis

pr> Myasthenia gravis can be classified according to which skeletal muscles are affected. Within a year of onset, approximately 85–90 percent of patients develop generalized myasthenia gravis, which is characterized by weakness in the trunk, arms, and legs.

About 10–15 percent of patients have weakness only in muscles that control eye movement. This type is called ocular myasthenia gravis.

Other types of MG include congenital, which is an inherited condition caused by genetic defect, and transient neonatal, which occurs in infants born to mothers who have MG. Congenital MG develops at or shortly after birth and causes generalized symptoms.

Transient neonatal MG is a temporary condition that develops in 10–20 percent of infants born to mothers who have MG. Transient neonatal MG is caused by circulation of the mother’s antibodies through the placenta and it lasts as long as the mother’s antibodies remain in the infant (usually a few weeks after birth).

Incidence and Prevalence of Myasthenia Gravis

Myasthenia gravis affects approximately 2 out of every 100,000 people and can occur at any age. It is most common in women between the ages of 18 and 25. In men, the condition usually develops between 60 and 80 years of age.

 

Why Myasthenia Gravis An Autoimmune Disorder?

Artem is a doctor of veterinary medicine and has taught science and medicine at the college level.

This lesson will go over a condition called myasthenia gravis. We’ll talk about why it occurs, how your immune system is involved, how it can be treated, if it’s curable, and what typical signs it produces in a person.October 15, 2015 Smith

 

Autoimmune Disease

Most every one of us has played a game called ‘monkey in the middle’, or something along those lines. It’s basically where at least one person stands in between at least two other people who throw or kick an object like a ball to one another. The role of the monkey in the middle is to block the ball from getting through to the other person. Typically, as was the case when I was the monkey in the middle, the person in the middle has a hard time intercepting the ball.

But in some cases of autoimmune diseases, diseases where your own body attacks itself, the monkey in the middle is devastatingly effective. So effective, in fact, that it causes serious problems, as this lesson will address.

What is Myasthenia Gravis?

The terrible game that our body plays with us isn’t called monkey in the middle, it’s called myasthenia gravis. This is an incurable autoimmune disease that leads a person to develop severe muscle weakness and fatigue.

Although, as with many diseases, myasthenia gravis can occur at almost any age, it seems to affect more women under 40 and more men over 60 years of age, and in the United States affects about 20 individuals for every 100,000 members of the population.

Some of the most famous people in the world have suffered from this terrible disease, such as Aristotle Onassis, the billionaire Greek shipping magnate who married Jackie Kennedy after JFK’s assassination.

Why Does Myasthenia Gravis Occur?

Scientists are still working out the details of why myasthenia gravis may occur. We know that everything from genetics to immune reactions to drugs may increase the likelihood of developing this condition. Some researchers also believe the thymus , an organ located underneath your breastbone that is responsible for the proper development of the immune system, may play a role in the aberrations that occur in this disease. It has been noted that certain individuals with myasthenia gravis have a thymoma, or a tumor of the thymus.

What we do understand a bit better is the end result of most cases of myasthenia gravis. In this condition, your own body produces little proteins called autoantibodies, which are antibodies that target an individual’s own body, organs, cells, and receptors for destruction or inactivation.

Normally, antibodies are supposed to target foreign invaders such as bacteria and viruses for destruction or inactivation. But in an autoimmune condition such as myasthenia gravis, the antibodies become directed against auto, or self.

Specifically, these autoantibodies attach themselves to the receptors on the skeletal muscles of your body; these are the muscles responsible for locomotion. By doing so, the antibodies prohibit the receptors on the muscles from receiving a specific signal or destroy the receptors outright. Namely, this signal comes from nerves innervating the skeletal muscles. These nerves release a neurotransmitter that activates muscles of locomotion, called acetylcholine.

Under normal conditions, the nerves release this acetylcholine and it lands on its receptor on the muscle the nerve innervates. Once it lands on the receptor on this muscle, the muscle gets all excited, contracts, and allows you to move. The release of acetylcholine occurs at the neuromuscular junction, the place where nerves meet the muscles they innervate. The space between the nerve ending and the muscle it controls is known as the synaptic cleft.

That’s what happens normally. However, in people with myasthenia gravis the autoantibodies bind to the receptors on the muscle cells and thereby destroy or inactivate them.

You can liken the autoantibodies to our monkey in the middle, the nerve and muscle to our two players, the space between the two players as the synaptic cleft, the acetylcholine to our ball, and the receptors on the muscle cells to the hands of our player called ‘the muscle.’

The nerve player wants to pass the ball to the muscle player, but the monkey latches onto the hands of the muscle player and blocks the muscle player from ever catching the ball. If the ball can’t pass from one end to the other and can’t land in the muscle’s hands because those hands are blocked by the monkey, then the muscle player cannot become all excited about catching the ball, becomes depressed instead, and refuses to move because the game is no fun anymore.

Clinical Signs, Symptoms, and Diagnostics

Since the muscles are no longer excited to move, you shouldn’t be shocked by the typical signs and symptoms associated with myasthenia gravis, which means ‘grave muscle weakness’ through its ancient Latin and Greek roots. These signs include:

• Difficulty walking, eating, speaking, smiling, and swallowing
• Droopy eyelids and double vision
• In serious cases, respiratory failure, when the muscles of respiration can no longer work.

What is it like to have Myasthenia Gravis?

What is Myasthenia Gravis?

Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of voluntary muscles, which often improves with rest and worsens with activity. The condition is caused by an abnormal immune response.

What is it like to have Myasthenia Gravis?

Common symptoms		What people are taking for it
Pain		Pregabalin, Hydrocodone-Acetaminophen, Codeine-Acetaminophen
Insomnia		Zolpidem, Clonazepam, Gabapentin
Fatigue		Amphetamine-Dextroamphetamine, Wheelchair (powered), Amantadine
Anxious mood		Alprazolam, Paroxetine, Lorazepam
Depressed mood		Bupropion, Diet and exercise, Escitalopram

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What do patients take to treat Myasthenia Gravis and its symptoms?

Treatments reported by members
Pyridostigmine (Kalymin 60N, Mestinon)
Prednisone (Pulmison, Pronizon, Deltasone, Encorton)
Intravenous Immuno Globulin IVIg (Carimune, Privigen, Pentaglobin, Sandoglobuline)
Azathioprine (Imurek, Azasan, Azapress, Imurel)
Plasmapheresis

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These charts show data from Myasthenia Gravis patients’ latest treatment evaluations

Last updated: June 20, 2014

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• Major
• Moderate
• Slight
• None
• Can't tell

• Severe
• Moderate
• Mild
• None

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Who has Myasthenia Gravis on PatientsLikeMe?

• 266 patients have this condition
• 3 new patients joined this month
• 192 say Myasthenia Gravis is their primary condition

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Age

Age	Proportion	# of patients
<20	3	3
20s	12	12
30s	36	36
40s	58	58
50s	75	75
60s	34	34
70+	15	15

Gender

Distribution of females vs. males

74%	Females
26%	Males

Age at first symptom

Age at first symptom	Proportion	# of patients
0-19 years	17	17
20-29

Genetic Research Reveals Possible New Targets For Treatment Of Myasthenia Gravis October 15, 2015 Smith

NEW YORK – A new genome-wide association study suggests that immunomodulating drugs already approved by the U.S. Food and Drug Administration (FDA) could benefit patients with myasthenia gravis.

Dr. Bryan Traynor, chief of the Neuromuscular Diseases Research Section at the National Institutes of Health in Bethesda, Maryland, and colleagues, found three different disease-associated loci in myasthenia gravis patients. One locus was at CTLA4, and the FDA has approved two CTLA4-targeting treatments, abatacept and belatacept, for treating rheumatoid arthritis and renal transplant patients, respectively.

The findings also showed two distinct, but overlapping, disease-associated loci for the early- and late-onset forms of the illness
 

“The discovery of a genetic locus is always an exciting thing, but often times it can take 10 to 15 years between the discovery of a particular locus and a first-in-humans clinical trial,” Dr. Traynor told Reuters Health in a telephone interview. The fact that drugs targeting CTLA4 are already FDA approved should accelerate that timeline, he added.

In their study, published online February 2 in JAMA Neurology, Dr. Traynor and his colleagues looked at DNA from more than 1,000 white, North American patients with acetylcholine-receptor antibody positive myasthenia gravis and nearly 2,000 controls.

They investigated associations between more than 8 million variants and myasthenia gravis risk. Significant association signals were found at CTLA4 (odds ratio, 1.37); HLA-DQA1 (OR, 2.31) and TNFRSF11A (OR, 1.31).

The CTLA4 and HLA-DQA1 associations were replicated in a cohort of 423 myasthenia gravis patients and 467 controls from Italy.

The analysis also confirmed past observations that myasthenia gravis strikes younger patients, who are usually female, as well as patients 60 and older who are usually male.

We were able to find different genetic loci that drive genetic susceptibility in each case,” Dr. Traynor said. “We genetically proved that they are two fundamentally different conditions, but overlapping conditions as well.”

The CTLA4 associations were seen in both early- and late-onset patients, the researcher noted. It’s likely, he added, that clinical trials testing CTLA4-targeting drugs in myasthenia gravis would enroll all patients with the illness, regardless of their genotype.

Even in patients who don’t have the CTLA4 variants he and his colleagues identified, Dr. Traynor noted, “it’s possible that in the other patients there are other variants in that region that are altering CTLA4 function, but we are not powered to pick it up.”

While effective therapy for myasthenia gravis is available, the researcher added, some patients with the disease do wind up having significant problems. “I think there is considerable room to grow new treatments,” Dr. Traynor said.

Dr. Robert Lisak of Wayne State University School of Medicine in Detroit co-authored an editorial accompanying the study.

“As in other complex immune-mediated disorders, there is an important influence of minor changes in multiple genes, likely interacting with the environment and with one another and their protein products, in the pathogenesis of myasthenia gravis,” Dr. Lisak told Reuters Health by email. “And in an autoimmune disease it is not surprising that the minor changes occur in genes for proteins that are critical in the immune response.”

“Identifying changes in genes in patients with different types of myasthenia gravis has the potential to help us understand which molecules and pathways in the immune system are the most important in development of disease,” he added. “In theory that might help in development of more-focused treatments for patients with myasthenia gravis. Only time will tell if this ultimately happens.”

APPETITE AND CORTICOSTEROIDS

Corticosteroids like prednisone and deflazacort, often prescribed to treat DMD and other neuromuscular disorders — particularly myasthenia gravis (MG) and polymyositis (PM) — are known for causing unwanted weight gain. Managing hunger with small, frequent servings of high-fiber foods that are low in calories and developing an eating schedule are some helpful hints. To learn more, read War on Weight Gain and Nutritional Considerations While on Corticosteroids.

Side effects of prednisone and other corticosteroids include increased appetite and weight gain, as well as osteoporosis (porous and weakened bones). Appropriate nutritional supplements while being treated with prednisone include calcium and Vitamin D.

Meal planning suggestions

Weight gain is a side effect of prednisone regardless of how much a person eats. However, since many people on prednisone also have an increased appetite, they may gain additional weight from overeating. This weight gain may result in further difficulties with mobility.
Therefore, recommendations for individuals taking prednisone suggest following a healthy eating plan to maintain your weight, avoiding excess weight gain, and avoiding high blood sugar. It may be helpful to develop an eating schedule, so that you won’t overeat from becoming excessively hungry.
One of the culprits in this weight gain is fat. Fat is a concentrated source of calories and can add unwanted pounds quickly. Therefore, your intake of fat should be limited (to less than 30 percent of total calories consumed).
Furthermore, if you’re watching your weight, you’ll want to limit your intake of sugar. Sugar and concentrated sweets are usually high in calories and low in vitamins and minerals.

Low-fat, low-sugar foods

Breads:	whole wheat breads, long grain rice, whole wheat tortillas, pita bread carrot/bran muffin, wheat English muffin, wheat crackers granola bars without frosting, rice or corn cereal without sugar fiber cereals, grain cereals without frosting
Meats:	lean turkey, fish, chicken, beef, pork, tuna in water, eggs (no more than 3 per week). These should be steamed, baked, broiled, grilled, not fried.
Dairy:	skim or 1 percent milk, low fat or nonfat cheese, ice milk, low fat frozen yogurt, lite cream cheese
Vegetables:	all fresh or frozen that are unbreaded, including starchy vegetables, such as peas, lima beans, corn, squash, lentils, beans vegetable soups
Fruit:	all fresh or frozen fruits Limit fruit juice to less than 4 ounces per day.
Fat:	low calorie salad dressing, lite mayo Limit to three teaspoons of added fat per day.