UNDERSTANDING MYASTHENIA GRAVIS: A GUIDE FOR NEWLY DIAGNOSED SNOWFLAKES
Understanding Myasthenia Gravis after you’ve just been diagnosed is honestly overwhelming. From symptoms to triggers and treatments and all the terms, many are lost with no idea of what they should really do while embarking on their chronic illness journey.
I know because I was once there.
I was the young woman sitting in the doctor’s office stunned by the news and left with 1,001 questions. Turning to google was my only option (50/50 split on whether that was good or bad).
If you are looking for a comprehensive guide to help you understand everything around your diagnosis, you are in the right place.
UNDERSTANDING MYASTHENIA GRAVIS FOR THE NEWLY DIAGNOSED TABLE OF CONTENTS
- What is MG?
- MG Facts and Myths
- Who is at Risk?
- Causes and Triggers
- MG Crisis
- Living with MG
- Support Systems
- Pregnancy and MG
- Traveling with MG
- Talking about MG
- Cautionary Drugs
- Common MG Terms
- Helpful Resources
What is MG?
Myasthenia gravis, better known as simply MG, is a rare neuromuscular chronic illness that affects the voluntary muscle groups.
The name myasthenia gravis is Latin and Greek in origin and means “grave muscle weakness”.
Myasthenia gravis is also referred to as the “snowflake disease” because everyone’s MG journey is unique.
What happens in the body when someone has MG?
Your voluntary muscles are controlled by nerve impulses that come from your brain. You think to move ___ body part and the impulse travels down the nerves to meet the muscle fibers in the neuromuscular junction (a fancy name that means space where nerves and muscles meet).
Once the impulse reaches the junction, it releases a chemical called acetylcholine. Acetylcholine travels to the muscle fiber side of the junction where it attaches to and activates the receptor sites.
When enough receptor sites have been activated, the muscle moves.
Muscle weakness occurs when acetylcholine cannot activate enough receptor sites at the neuromuscular junction. When you have MG, there is something interfering with the acetylcholine activating the receptor sites- an antibody. The antibody is destroying or blocking the receptor sites.
Antibodies are soldiers for the immune system. They typically attack foreign antigens in the body like bacteria and viruses. For some reason, when you have MG, your immune system makes antibodies that mistake your receptor sites as enemies.
Understanding Myathenia Gravis Types
Ocular myasthenia gravis is when the muscles that move the eyes and control the eyelids are easily fatigued and weakened. Approximately 15% of people with MG have ocular mg only. Ocular symptoms are often the first symptoms of MG, and many people may develop other generalized muscle weakness issues later on.
Generalized myasthenia gravis is when all the voluntary muscles of the body become weakened.
MuSK positive generalized myasthenia gravis categorizes a small percentage of folks with generalized MG will test positive for anti-MuSK (muscle-specific kinase) antibodies.
The presence and type of antibody present in blood can help guide treatment choices. People with anti-MuSK antibodies may respond differently to medications than people with AChR antibodies.
Seronegative myasthenia gravis is a form of MG where autoantibodies (anti-AChR and anti-MuSK autoantibodies) are not detectable in the blood.
Autoimmune myasthenia gravis is the most common form of MG, characterized by fluctuating weakness of the voluntary muscle groups. In autoimmune MG, the immune system mistakenly attacks and destroys the body’s own tissues by forming autoantibodies.
Transient neonatal myasthenia sometimes occurs in infants born to mothers with autoimmune MG. As the maternal antibodies degrade in the infant’s body, symptoms gradually disappear within a few weeks. Infants with transient neonatal myasthenia gravis do not have an increased risk for long-term or future MG.
MG Facts and Myths
Facts and Stats about MG
- The most common form of Myasthenia Gravis is a chronic autoimmune neuromuscular disorder characterized by fluctuating weakness of the voluntary muscles.
- Myasthenia Gravis is considered to be a rare disease. In the U.S., it is estimated that 20 in 100,000 people have MG. However, myasthenia gravis is often underdiagnosed, so there is a change the numbers are higher.
- The first case of MG was documented in 1672 by Thomas Willis, an Oxford physician, not much was known or understood about it. 400 years later we are hitting the tip of the iceberg.
- Myasthenia Gravis can occur in anyone regardless of race, gender and age.
- MG is not thought to be directly inherited nor contagious, but can occur in more than one member of the same family.
- MG is not transmitted via intimate or any form of contact.
- At the time of this guide creation, there is no known cure for Myasthenia Gravis.
- June is Myasthenia Gravis Awareness month.
- Teal is the awareness color of Myasthenia Gravis.
Debunking some Myths about MG
If you suffer from MG, there are well intentioned and sometimes ill intentioned e doctors who swear they know what’s good for you. They will offer advice or make comments that are often wrong.
I hope that this will help debunk some of those misconceptions:
Who Is At Risk for Developing MG?
Myasthenia Gravis occurs in all ethnicities and genders. It most commonly affects men over 60 and women under 40 but it can occur at any age.
In cases of neonatal myasthenia, symptoms are temporary and disappear within a few months after birth. MG in children is rare but possible and is identical to adults with MG.
MG is not inherited however, having a personal or family history of autoimmune diseases may increase your risk.
Understanding Myasthenia Gravis Diagnosis
MG is pretty difficult to diagnose for a number of reasons. One being that weakness and fatigue is a common symptom of many illnesses. Another is that MG symptoms tend to fluctuate daily. A diagnosis can take over a year in some cases.
A diagnosis of MG is confirmed via several methods, including the following:
- Acetylcholine receptor (AChR) antibody testing– Blood test to detect abnormal antibodies. Approximately 85% of people diagnosed with MG test positive for this group of antibodies.
- Anti-MuSK antibody testing—Blood test for MG patients who have tested negative for the acetylcholine antibody (AChR). Approximately 6% of MG patients without AChR antibodies test positive for the anti-MUSK antibody.
- Ice Pack tests—Ice Pack tests are examinations performed by specialists to evaluate strength and recovery responses that could be consistent with an MG diagnosis.
- Imaging – a CT or MRI is done to identify an abnormal thymus gland or thymus gland tumor (thymoma)
- Edrophonium test– Edrophonium is a drug that prevents the breakdown of acetylcholine, allowing the clinician to understand your muscle response and whether the results are consistent with an MG diagnosis.
- Electromyography (EMG)— Repetitive nerve stimulations (RNS) applying electrical shocks are often used to gauge muscle response and action potential and whether results are consistent with an MG diagnosis
- Neurological Exam – Your reflexes muscle strength, muscle tone, senses of touch and sight, gait, posture, coordination, balance, and mental skills are tested for weakness. fatigue with repetition. Impaired eye movement or muscle weakness may prompt a doctor to evaluate further.
- Single-fiber electromyography (SFEMG)—Detects neuromuscular transmission defects by recording action potentials from individual muscle fibers using a small needle electrode.
Possibly the most frustrating part of diagnosis is that results from some (or all) of these tests can be negative or inconclusive, even when a person does have MG. A doctor familiar with MG is important in determining a proper diagnosis.
Understanding Myasthenia Gravis Causes and Triggers
As mentioned earlier in the breakdown, when you have myasthenia gravis, your antibodies block, alter, or destroy the receptors which prevents the muscle from contracting. This is most often caused by antibodies to the acetylcholine receptor itself, but antibodies to other proteins, such as MuSK (Muscle-Specific Kinase) protein, also can impair transmission at the neuromuscular junction.
The thymus gland
Going a little deeper, the thymus gland controls immune function and may be associated with myasthenia gravis. It is responsible for producing T cells that protect the body from viruses and infections. As you get older, your immune system is stronger so the thymus grows until puberty then shrinks and is replaced by fat.
In many adults with myasthenia gravis, the thymus gland remains large and sometimes develops tumors called thymomas. Thymomas are typically harmless, but they can become cancerous.
It is thought that the thymus gland is the culprit behind causing the immune system to attack its own cells and tissues and produce acetylcholine receptor antibodies.
Myasthenia Gravis Triggers
The following factors may trigger or worsen exacerbations:
- Warm weather
- Concurrent illness (eg, viral infection)
- Tapering of immunosuppression
- Pregnancy and postpartum period
- Worsening of chronic medical illnesses (cardiac, renal, autoimmune, etc.)
- Certain Medications
Learning your triggers is key for symptoms and lifestyle management.
Understanding Myasthenia Gravis Symptoms
MG can affect any of the muscles that you control voluntarily. Weakness related to MG worsens with activity.
Common Myasthenia Gravis Symptoms
- Chewing difficulty
- Difficulty breathing
- Double vision
- Droopy eyelids
- Dysphonia, or voice disorder
- Dysarthria, or slurred speech
- Vocal Fatigue
- Difficulty with Pitch, or monotone
- Limb weakness (arms, legs, neck)
- Difficulty Smiling
- Swallowing difficulty, or dysphagia
MG symptoms vary and fluctuate throughout the day. At times you may experience little to no symptoms and other times you’re symptoms will flare.
(An exacerbation, or flare, is when your symptoms increase in frequency and/ or become more severe.)
Understanding Myasthenia Gravis Crisis
While a Myasthenia Gravis diagnosis is not terminal, some of the complications can be life-threatening. Myasthenic crisis is a life-threatening condition that affects breathing and requires immediate treatment.
A Myasthenic Crisis is a complication of MG characterized by worsening of muscle weakness resulting in respiratory failure. This happens when your respiratory muscles get too weak to move enough air in and out of the lungs. Basically you have a very difficult time breathing.
If you’re experiencing severe shortness of breath seek medical attention immediately. Almost all patients with MG crisis or impending crisis require specialized medical treatments administered in a hospital setting. Most often the course of action will be to put you on a ventilator to help you breathe.
Understanding MG Treatments
What kinds of doctors treat MG
It is recommended that you seek a doctor that is at bare minimum trained in nervous system conditions such as a neurologist. Personally, I think an actual specialist that has extensive experience in diagnosing and treating myasthenia gravis is better but there aren’t a lot of them around.
If you’re looking for a list of specialists, the MGFA has a Physician Referral List on their website.
While there is no known cure for myasthenia gravis (MG), there are many effective treatments. Every case of MG is unique, so you and your doctor will work together to decide on a treatment plan for your specific needs.
Here is some Information on currently available treatments:
- Anti-acetylcholinesterase agents – Mestinon® (pyridostigmine bromide)– allows acetylcholine to remain at the neuromuscular junction for a longer period, which in turn allows activation of more receptor sites, resulting in increased conductivity and muscle engagement. Mestinon® comes in two forms, fast-acting 60 mg tablets and long-lasting slow-release 180 mg capsules known as Timespan®, which delivers pyridostigmine bromide over a 12-hour period.
- Corticosteroids- Prednisone suppresses your body’s production of antibodies that may be blocking or binding onto your body’s acetylcholine receptors. This blocking or binding of the acetylcholine receptors causes weakness.
- Immunosuppressant agents – such as Imuran®, Cellcept®, or Cyclosporin® work similar to Prednisone without the same harsh side effects.
- Monoclonal antibodies – Monoclonal antibodies such as Soliris®, also known as Eculizumab or Rituximab, are infusible drugs that work as complement inhibitors, to reduce immune system attacks that may contribute to MG symptoms.
Other Treatment Strategies
- Intravenous immune globulins (IVIg) – During IVIG, a line is placed into a vein to receive delivery of immune globulins (IgG). The IgGs are thought to override your own antibody production
- IgG Sub-cue Hizentra– A new, less invasive method of IgG delivery, a series of 4-6 short needles are placed into the subcutaneous layer of skin across your abdomen. These needles are connected in a spider web fashion to the pump.
- Therapeutic Plasma Exchange, or Plasmapheresis – (also known as Plasma exchange or PLEX) is a filtration procedure whereby abnormal antibodies are removed from blood plasma via two IV line or a port.
- Thymectomy – This is the surgical removal of the thymus gland. The thymus gland is located in the middle of your upper chest and lies over your heart. Traditional thymectomies are open chest sternotomy but there are less invasive versions like the video-assisted or robot-assisted thymectomy.
One of the most primal concepts to understanding myasthenia gravis is that MG is not “curable” (yet) but it’s also not terminal. While existing treatments do not cure MG, most patients have improved muscle strength, and some even experience remission.
With proper management techniques, a person with MG can live a full life.
Understanding Myasthenia Gravis Living
Ok so now that you’ve come to realize MG isn’t a death sentence, how can you learn to flourish in your new normal. Daily tasks turn into amazing feats. Here are a few lifestyle strategies that may be helpful on your journey.
Tips and Hacks for Living with MG
This is probably the most important thing in this guide so pay close attention. You ready?
Learn your triggers and adjust.
Most things are still possible with MG. It takes getting to know your body and figuring out what works for you and what doesn’t.
If you know that heat is a trigger for you, take steps to make sure you’re prepared to stay cool no matter the situation. Once you master that, you’ll be great.
Here are a few helpful ideas to cope with MG symptoms and triggers.
Droopy Lids and Double Vision:
- Eye patch
- Prism lenses
Voice & Speech:
- Avoid talking when possible
- Use gestures
- Have friends and family ask yes/no questions
- Write/ type or text responses
- Break food into smaller pieces
- Eat softer foods
- Resting prior to meals
- Protein Shakes/Juices
- Smaller, more frequent meals
- Softer foods to reduce chewing
- Resting prior to meals
- Consuming cold foods and liquids
- Alternating solid food and liquid between bites
- Time meals around peak medication times; eating about an hour after taking your medication
- Crushing medication into soft foods if swallowing pills are difficult
- Neck brace
- Cane / Walker/ Scooter
- Handicap Placard to decrease walking distance while out
- Avoid Stairs
- Rest as needed
- Portable misting fan
- Cooltech towels
- Drinking cool drinks/ eating cool food
- Keep temperature at 70-72 degrees
Getting the Support you Need
Your primary focus should be making sure you have a top tier medical team on your side. Myasthenia Gravis is something you will have for the rest of your life. Your health care team is the first line of defense in helping you fight back.
MG is not a cheap illness. So reach out to your insurance company to see costs of doctors, tests, medications and procedures. You may want to change insurance companies or policies based on who provides the best coverage based on your new reality.
If you have multiple doctors (primary care physician, neurologist/specialist, obgyn, nutritionist etc), make sure that they are all on the same page.
Keep impeccable records. Take thorough notes during your visits. Ask for copies of your labs and write ups to keep everyone informed of updates and changes.
This is your health. Don’t be afraid to ask questions if something doesn’t make sense. And don’t be afraid to push back and advocate for yourself when something doesn’t feel right.
They are doctors but YOU are the one experiencing the illness so your thoughts, opinions and experiences matter.
Physical Support from Family and Friends
Chronic illnesses like Myasthenia Gravis don’t just affect the person diagnosed. Family and friends are impacted too.
Communication is the most important thing. You have to be honest with your loved ones about what you’re going through and what you need from them.
While I love my independence like the next, Myasthenia Gravis is one of those illnesses that humbles you. There may come a time when you aren’t able to do the things that you used to be able to do.
Asking for help is not being needy or a burden. It’s a necessity of the new reality.
Emotional Support from Others with Chronic Illnesses
Now family and friends are great and all. But try as they might there are some things you’re going through that they can’t help you with.
I’ve learned that chronic illness life is a lonely life especially when you’re surrounded by “healthy” people.
Chronic illness is just one of those things that you don’t understand unless you have one. Who else can relate to needing to take a nap after a shower or from seeing your doctor more than you see your relatives?
So I encourage you to find others (even if it’s not someone with MG) who can relate to what you’re going through.
If there aren’t any support groups in your area, the internet is the perfect place to find new spoonie friends. I’ve listed a few in the Resources section.
Understanding Myasthenia Gravis and Pregnancy
If you have Myasthenia Gravis and are considering becoming pregnant, you should discuss your plans with your healthcare provider well in advance.
It is possible to have a great normal pregnancy with Myasthenia Gravis. In fact some women reported going into remission with their pregnancies. Other reported crises and bed rest. Like everything with MG it’s unique to the person.
The most important thing is to talk about medications with your doctor. Some treatments aren’t approved for pregnant women and there will need to be adjustments made.
Traveling with MG
Traveling with Myasthenia Gravis requires more planning and preparation than a regular trip. Here are some things to consider when planning for travel.
- How stable are you currently?
- What method of travel are you taking and how will that affect your MG?
- Will weather conditions at your destination exacerbate your symptoms?
- What activities are you planning to do that may exacerbate your symptoms?
- Is there a hospital nearby and are they familiar with MG?
- Is it peak flu season or infectious diseases concerns at my destination?
Prior to leaving for your trip, make sure you have a record of your medications and treatments and your doctors contact information on it. In the unfortunate case that a crisis occurs, it will come in handy in case you need assistance or medical care while travelling.
Other Helpful Tips for Travel
- Allow time for rest and reduced physical activity.
- Bring a list of medications that may worsen MG.
- Put medications in your carry-on bag so you don’t get separated from them.
- Make a list of medical terms in the language of the country you are visiting.
- Bring a medical alert ID bracelet or alert card in the event of an emergency.
- If on IVIG, have your treatment a few days before you leave for your trip
- Check the details of your medical travel insurance and what’s covered
- Travel with someone who knows about your MG and can be your advocate in the event of an emergency
- Ask about seating arrangements and travel accommodations for disabled passengers
Talking About MG
While you are coming to terms with your diagnosis you may be wondering who to divulge the information to and how. I kept my MG a “secret” for about a year after being diagnosed. Only those who saw me daily, knew about it. I could barely wrap my head around it let alone talk about it with others.
Talking About MG in the Workplace
The decision to tell your anyone including coworkers, supervisor etc about your MG is YOUR decision alone.
You are not legally required to do so. However, it may be a good idea.
Letting your workplace know about your MG, would allow them to explore “reasonable accommodation” to help you fulfill your job duties.
As with most things, communication is key. If you’re getting tired, say something. If you can’t physically do something, say something.
Stress can be a trigger for MG symptoms so if you have a stressful position, you may want to explore rotating to another position.
Explaining Myasthenia Gravis to Friends
When describing it to your friends or anyone who is unfamiliar with MG, I’ve found that it’s best to keep it short and sweet.
Don’t go into too much detail. Give the most generic version you’re comfortable with.
“ My muscles randomly get weak.”
“It’s a neuromuscular autoimmune disease.”
“My body attacks itself”
Explain to them how you constantly feel fatigued even though all you do is rest.
In addition to making your friends aware of MG, also let them know how they can help you, and if they are your friends, they are going to want to know how they can help their friend in need.
Many drugs have been associated with worsening myasthenia gravis (MG). However, that doesn’t mean that if you take one of these drugs it will for certain make your MG worse.
It’s better to be safe than sorry so keep a copy of this list of drugs somewhere handy. Consult your doctor for some alternatives when available.
Sometimes use of certain medications aren’t avoidable. You’ll just have to consider the pros and cons and monitor your symptoms for flare.
Here are a few common drugs that may worsen MG symptoms
- Telithromycin: antibiotic for community acquired pneumonia.
- Fluoroquinolones (e.g., ciprofloxacin, moxifloxacin and levofloxacin): commonly used antibiotics
- Botulinum toxin
- D-penicillamine: used for Wilson disease and rarely for rheumatoid arthritis.
- Quinine: occasionally used for leg cramps.
- Macrolide antibiotics (e.g., erythromycin, azithromycin, clarithromycin): commonly used antibiotics for gram-positive bacterial infections.
- Aminoglycoside antibiotics (e.g., gentamycin, neomycin, tobramycin):used for gram-negative bacterial infections.
- Corticosteroids: weird right since prednisone is commonly used to treat MG.
- Procainamide: used for irregular heart rhythm.
- Desferrioxamine: Chelating agent used for hemochromatosis
- Beta-blockers: commonly prescribed for hypertension, heart disease and migraine
- Statins (e.g., atorvastatin, pravastatin, rosuvastatin, simvastatin): used to reduce serum cholesterol.
- Iodinated radiologic contrast agents: used for contrast radiology (CT scans).
- **Chloroquine (Aralen): used for malaria and amoeba infections
- **Hydroxychloroquine (Plaquenil): used for malaria, rheumatoid arthritis, and lupus.
In general, it is believed that vaccinations (like the flu shot) are safe for patients with MG, however, there is an exception.
If you are taking immunosuppressive medication, such as prednisone, it is usually recommended that you avoid live, attenuated (alive) vaccines. Live, attenuated vaccines carry higher risk for those who are immunosuppressed.
Examples of live, attenuated vaccines include the shingles vaccine and the nasal spray form of the flu vaccine.
Common MG Terms
- Acetylcholine: a neurotransmitter
- Acetylcholine receptors (AChR): an integral membrane protein that responds to the binding of acetylcholine
- Autoimmune: the immune system mistakenly attacks and destroys the body’s own cells and tissues
- Corticosteroid: medication that suppresses the body’s production of antibodies
- Diplopia: double vision
- Dysphonia: voice disorder
- Dysarthria: slurred speech
- Dysphagia: difficulty swallowing
- Edrophonium: a drug that prevents the breakdown of acetylcholine, allowing the clinician to understand your muscle response
- Electromyography (EMG): Repetitive nerve stimulations (RNS) applying electrical shocks are often used to gauge muscle response
- Exacerbation: also known as flare-up, symptoms increase in frequency and/ or become more severe
- Immunosuppressant: medication that suppresses the body’s production of antibodies
- MuSK: stands for Muscle-Specific Kinase, a protein
- MuSK receptor: a protein receptor required for the formation and maintenance of the neuromuscular junction
- Myasthenic Crisis: worsening of muscle weakness resulting in respiratory failure
- Neuromuscular junction: site where nerves and muscle fibers communicate to facilitate voluntary movement
- Ptosis: drooping eyelids
- Refractory: people with MG who do not respond to conventional treatment
- Remission: symptoms disappear completely for some time even without treatment
- Seronegative myasthenia gravis: is a form of MG where autoantibodies (anti-AChR and anti-MuSK autoantibodies) are not detectable in the blood
- Single-fiber electromyography (SFEMG): Detects neuromuscular transmission defects by recording action potentials from individual muscle fibers using a small needle electrode
- Subcutaneous: under the skin
- Therapeutic Plasma Exchange (Plasmapheresis): also known as plasma exchange or PLEX, is a filtration procedure whereby abnormal antibodies are removed from blood
- Thymectomy: surgical removal of the thymus gland
Research and Information
Prescription Cost Assistance
Support Groups for Myasthenia Gravis
In person Support Group
FB Support Groups
I hope you found this information helpful in understanding myasthenia gravis. Don’t hesitate to reach out with questions or just to vent. I’m around!