Myasthenia gravis (MG) is a chronic autoimmune condition that impacts how your nerves and muscles work together. It usually causes muscle weakness and fatigue and can make it hard for you to swallow, breathe, talk, and sometimes see.
MG symptoms can be triggered by things like stress, heat, infections, and lack of sleep. That can make it hard for your doctor to manage your symptoms. The good news is there are lots of options to try, especially if you know your triggers and track your symptoms.
MG Treatments
Here are some treatments for MG your doctor may recommend, depending on your symptoms and the type of MG you have.
Acetylcholinesterase inhibitors. Pyridostigmine (Mestinon, Regonol) is usually the most common option for controlling MG symptoms. It treats them by improving the communication between your nerves and muscles by slowing the breakdown of the neurotransmitter acetylcholine, which is the chemical messenger that helps control muscle movements. It’s safe to take long-term when you have mild MG. It’s often given with immunotherapy when your MG is severe.
Immunosuppressants. Immunosuppressive drugs, including prednisone and others, may help your muscles get stronger by suppressing your immune system from creating abnormal antibodies. You might have to take them for several months, though, to see results. You’ll need to be monitored by your doctor when taking them because they can have serious side effects.
Biologics. If you don’t see any improvement with pyridostigmine or immunosuppressants, your doctor may recommend biologic therapies. These are more personalized than other treatments because they target your immune system. Drugs such as monoclonal antibodies or drugs that target your complement system can be used early in treatment, especially to help avoid side effects of long-term steroids.
Rapid immunotherapies. These include intravenous immunoglobulins (IVIG) and plasma exchange (PLEX). They can help control your symptoms during life-threatening or severe flares. IVIG gives you highly concentrated antibodies to replace the ones interfering with your nerve-muscle communication. PLEX filters your blood to remove harmful antibodies that interrupt that communication. Rapid immunotherapies may be used when you have life-threatening breathing or swallowing problems, or before surgery if you have refractory disease.
Thymectomy. Between 10% and 15% of people with MG have a tumor in their thymus gland, and removing it is usually recommended. Even if you don’t have a tumor, surgery could help improve your symptoms. About 70% of people with MG who had their thymus gland removed got better, and some even went into remission. Using this treatment in the first two years after being diagnosed may give the best results.
How Do I Know When My MG Treatment Is Working?
The Myasthenia Gravis Foundation of America established guidelines for treating MG and defined what it means when those treatments work.
Minimal manifestation status (MMS). The goal for treating MG is to manage or ease symptoms that restrict your muscle functions, even if you still have mild muscle weakness. The Myasthenia Gravis Foundation calls it minimal manifestation status (MMS). It’s when treatment stops your symptoms without giving you any side effects. MMS is different from minimal symptom expression, which is when you score between a 0 and 1 on the MG Activities of Daily Living scale (MG-ADL).
Remission. If you have no symptoms or signs of MG, you’re considered to be in remission. As many as 40% of people with MG can be symptom-free, though sometimes it may be temporary.
There are other ways you can help your health care team know when your treatment is working.
Know your triggers
Symptoms of MG are different for everybody, and they can come and go. Flare-ups can be triggered by things like infection, fatigue, stress, heat, and hormonal changes. If you know what causes your flare-ups, you can help avoid them. You can also let your doctor know if your treatments are working to prevent them.
Track your symptoms
Keeping track of your triggers is a great start, but you also need to regularly record your symptoms. That lets you know what’s normal for you, so you can let your doctor know right away what causes your flare-ups.
The best way to do that is with the MG-ADL. It includes eight questions you answer with a score of 0-3, based on how severe your symptoms are. The score 3 is the most severe. Studies have shown that the MG-ADL is good for measuring MG symptoms and how they respond to treatment.
Once you have a baseline score, your doctor may want you to fill out the MG-AGL scale a few times annually to determine how well your treatments are managing your symptoms. Even if your score goes down by 2 points, your doctor will consider your treatment successful.
There are also smartphone apps that let you track your daily symptoms and share them with your doctor electronically.
How Do I Know When My MG Treatment Isn’t Working?
There are signs to watch for. The most obvious is that your symptoms get worse, even when you’re taking your medications and following your treatment plan.
Signs your symptoms are worsening
- Your muscles get weaker, especially later in the day.
- You notice more drooping of your eyelids.
- You have more blurry or double vision.
- Your jaw muscles tire easily.
- You have trouble swallowing.
- Your speech becomes slurred.
- You have shortness of breath.
Your medications stop working
Even if your symptoms aren’t getting worse but you’re not improving, it’s probably a good sign it’s time to try something else. For example, if you’ve been taking pyridostigmine without good results, your doctor might want to try immunosuppressive therapy, which is a common second-line treatment.
You have a myasthenic crisis
About 15%-20% percent of people with MG will have what’s called a myasthenic crisis, which is when your respiratory muscles get so weak you can’t breathe. It can be caused by fever, respiratory infection, traumatic injury, or stress and is life-threatening. You usually need help breathing with a ventilator until your lungs regain their strength.
What Is Refractory MG?
About 10% to 20% of people with MG don’t get better with treatment. This is called refractory MG. It’s more common if you’re a woman or if you have a tumor on your thymus gland.
Doctors are still trying to understand refractory MG, and there’s not one definition. But most experts agree if you have one or more of the following, you could have refractory MG:
- MG symptoms don’t improve with common drug treatments.
- You have relapses when going off of treatments.
- You need regular therapy with intravenous immunoglobulin (IVIG) or plasma exchange.
- You have severe side effects from MG drugs, making treatment impossible.
- You have repeated MG crises.
New therapies are being developed to help treat refractory MG, including a T-cell therapy, which is showing success in Phase II studies.
What Can I Do When My Treatments Stop Working?
If you notice your treatments aren’t working, tell your doctor. They’ll probably want to consider other options, like new medications, or surgery, that might better help control your symptoms.
Call your doctor right away if you have any of these symptoms:
- Drooping eyelid or blurry vision
- Trouble breathing
- Difficulty swallowing
Your doctor might also recommend you to a specialist for a second opinion.
Lifestyle changes may help improve your quality of life. They may include:
- Exercise to help improve your balance.
- Eat small meals during the day to reserve your energy.
- Install safety rails and grab bars around the house to help you get around.
- Run errands when you have the most energy.
- Rest and nap when you need to.
These might sound minor, but they can have big results when other treatments don’t.
Just remember, MG can progress over several years. It may affect your vision first and then progress to your face and neck muscles, then to your arms, shoulders, and legs. MG is considered severe when it starts to affect your breathing.
Show Sources
Photo Credit: DigitalVision/Getty Images
SOURCES:
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Rare Disease Advisor: “Symptoms, Causes, and Treatments.”
Cleveland Clinic: “Myasthenia Gravis: What It Is, Causes, Symptoms & Treatment,” “Myasthenia Gravis Outcomes,” “What are the treatment options for myasthenia gravis if first-line agents fail?”
Johns Hopkins Medicine: “Myasthenia Gravis.”
Muscle and Nerve: “Utilization of MG‐ADL in myasthenia gravis clinical research and care,” “Myasthenic crisis.”
Harvard Health: “Myasthenia Gravis.”
Neurology: “International consensus guidance for management of myasthenia gravis,” “Design of KYSA-6, a Phase 2, Open-label, Multicenter Study of KYV-101, a Novel Fully Human Anti-CD19 Chimeric Antigen Receptor T-Cell Therapy in Refractory Generalized Myasthenia Gravis (P7-11.034).”
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