Category Archives: Autoimmune Disease

Myasthenia Gravis, The Snowflake Disease(section three, diagnosis and treatment for Generalized Myasthenia Gravis)

Section two in my articles about Myasthenia Gravis focused on Ocular MG.  This section is about generalized Myasthenia Gravis.  However, the majority of patients with generalized MG also have ocular MG. I am hoping to help my fellow snowflakes and those who care about them.  My articles are also geared toward nurses and allied health care workers.  Unless a healthcare provider works in this specialty, they may only see this disease once or perhaps not at all.

Autoimmune MG is an acquired disorder.  Acetylcholine receptor antibodies attack the neuromuscular junction causing muscle weakness throughout the body.  The severity of the disease and the muscles effected vary from person to person and from day to day.  This is why the nickname for this illness is the Snowflake Disease. All voluntary muscle groups can be effected.  We do not know what causes MG.

With MG, muscle weakness worsens with activity and improves with rest.  When someone presents with the symptoms of MG, the doctor will probably order an anti acetylcholine receptor antibody blood test.  This test will show antibodies that attack acetylcholine receptor sites.

Another test is the Tensilon test.  This is given intravenously.. During he test, acetylcholine acts on the receptors.  If an individual has Myasthenia Gravis, they will show improved muscle strength in response to this test. A positive test leads to a diagnosis of MG

Through a repetitive nerve stimulation test, a neurologist can diagnosis MG by looking at patterns.  Another test, the single fiber EMG, is helpful for patients who have had negative results with other tests.

A CT scan of the thymus will probably be administered. 15% of people with Myasthenia Gravis have a thymoma, a tumor of the thymus gland.

I also discussed the above tests in Section Two when I focused on Occular MG.  The doctor will probably order consultations with a variety of specialists. A Neurologist will hopefully be leading the treatment team.  He will begin with a physical exam.  This exam will vary depending on which muscles are fatigued. The eyes will be observed.  The ability to open and close eyes will be tested.  Hand grips and the ability to raise arms will be measured.  Leg strength will be tested.  Shallowness of breath and speech difficulty will be observed.

A Pulmonologist will probably be needed for those times when the breathing  muscles are weakened. A Neuro opthamologist will be very helpful for eye and vision issues.  Neuro opthamology is a unique specialty that may not be available to all patients. An ENT may needed to assess speech problems.  Social services may be needed to assess what tasks a person needs help with.  Physical and Pulmonary therapy must be chosen with extreme care.  These specialists must be aware of what Myasthenia Gravis is.  If an MG patient is pushed to increase activity and exercise, they will usually get worse.  This is the opposite of what most other conditions need.  A psychotherapist who is familiar with chronic illness issues may be needed.  The inactivity and isolation caused by this disease can easily lead to depression.  There may also be consultations and diagnostic tests ordered to rule out other diseases.  This will vary from person to person.

Once the diagnosis has been completed, treatment begins.  Self care will be the first line of defense.  A healthy diet, high in potassium, will help decrease weakness.  Lots of rest is needed.  Over exertion causes weakness to increase. Weather effects MG.  Hot weather and cold weather make symptoms worse. A medical alert bracelet or an information card can be very helpful.

For those 15% of MG patients with a thymoma, a thymectomy (surgical removal of the thymus) may be chosen.

Most people will be started out with an Anticholinesterase drug.  Pyridostigmine bromide (Mestinon) is one of the drugs in this group.  This is not a cure for Myasthenia Gravis.  It can provide a short term boost in strength and energy. Tolerance for Mestinon is diagnostic in itself.  If someone does not have MG, they will have abdominal illness in response to taking Mestinon.  Dosages and frequency vary from person to person. Dosages will probably change for the same patient from time to time.

Many long term treatments may be tried in an attempt to get a remission. Immunosuppressive drugs are often used to treat Myasthenia Gravis.  Some of these drugs are Imuran, Cellcept, Cytoxin, Cyclosporine, and Prednisone.  These medicines are each unique.  The doctor will decide which medication is best for particular patients.

A temporary treatment is Plasmapheresis (Plasma Exchange).  In this procedure, both “good and bad” antibodies are removed. The cells are returned in artificial plasma. This is usually done once per day for five days in a row.  The patient usually feels better a few days after treatment. The benefit lasts for a few weeks.  This is very helpful for emergencies.  It is not recommended as repeated long term treatment.

IVIG is another temporary treatment. Gamma globulin is administered via an IV, over a period of sveral hours  This therapy may occur in a hospital, an infusion center, or a patient’s home.  IVIG is thought to suppress the immune system and increase muscle strength.

The above are common tests and treatments for people with Myasthenia Gravis.  My purpose in writing this series is to help. Perhaps on patient, caregiver, student, or healthcare worker will be helped by my information.  These first three sections have been objective and factual.  Section Four will be subjective. I will be writing about my personal  journey as a Myasthenia Gravis patient.

Sources: The Myasthenia Gravis Foundation of America, Myasthenia Gravis Foundation of California, Eye Wiki American Academy of Ophthamology,  Conquer MG of Il, Fellow Snowflakes, My treatment team members

 

 

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Myasthenia Gravis, The Snowflake Disease(section two, diagnosis and treatment for Ocular Myasthenia Gravis)

This article is second in my series about Myasthenia Gravis. I am targeting  patients and their caregivers, nurses, and allied health professionals. I hope this helps anyone with an interest in the topic. I am an RN and psychotherapist, as well as an MG patient.  I had planned to follow up on section one much more promptly, however I was hospitalized again with an exacerbation (flare up of breathing weakness) due to this disease.  This section will focus on diagnostic tests and treatments.  If you are a patient, your doctor will prescribe what is needed for you.  MG’s nickname is the snowflake disease because symptoms vary so much from person to person.  Also, a patient’s  symptoms can change quickly.  If I have had these diagnostic tests or treatments, I will share a personal experience comment.  Please remember that we patients are as diverse as snowflakes and our experiences vary.

Over two-thirds of Myasthenia Gravis patients experience ocular symptoms before they experience other MG symptoms.  Half the people with ocular symptoms will develop generalized muscle weakness in the first two years of their ocular symptoms. (This is true for me.  I had eye symptoms for one year prior to developing generalized MG). Fifteen per cent of people have only ocular problems.  If someone has only ocular MG for four years, they will probably not develop generalized MG.  Eye muscles are usually involved in Myasthenia Gravis, however a few patients do not have ocular symptoms.

These are tests that are often given to an individual with ocular MG symptoms. The anti-acetylcholine receptor antibody is a blood test that is usually given for both ocular and generalized MG. Your doctor will determine the need for some or all of these other lab tests: Anti-striated muscle antibody, Anti-Musk antibody, Anti-lipoprotein 4 antibody and Antistriational antibody (These lab tests are similar to other blood draws.  I did not find them difficult, but these tests would be traumatic for people who do not do well with needle sticks.)

These vision symptom may appear in other diseases, therefore tests to rule out other diagnoses may be administered. A brain MRI is helpful. (This is a noisy test and the MRI equipment is very close to the face.  Meditation gets me through this.) Claustrophobic patients may need an open MRI. Sometimes the doctor will prescribe an anti anxiety medicine to be taken prior to the test.

Patients with ocular Myasthenia Gravis e an abnormal single-fiber EMG.  In this test, a recording needle electrode is inserted into the neuromuscular junction.  This test is different than a regular EMG. This test should be administered by a doctor with extensive experience.(This test was very painful for me when done on my eye area nerves and muscles. In retrospect, it was worth it because it was conclusive in diagnosing my MG.)

Of course, the skills of the ophthalmologist  are very important in diagnosing and treating ocular MG.  A neuro ophthalmologist should be involved.  There are not many of these specialists.  (I live near a large city and I am fortunate to have this kind of specialist on my team.)

A variety of interventions can help the patient with ocular MG.  A patch on one eye helps  a person with double vision.  Prisms that cling to regular glasses are an inexpensive way to treat double vision.  Prisms can also be ground into glasses.  (Both types of prisms have helped me.) Eyelid crutches are sometimes attached to glasses to help lift drooping eyelids. A special tape can also be used to lift drooping eyelids. Dark glasses can be helpful.  As the day progresses, symptoms usually worsen. Rest may help eye symptoms.

MY REGULAR GLASSES AND SUNGLASSES WITH STICK ON PRISMSfullsizerender-2

Medicines that may be prescribed are drugs that alter the immune system such as prednisone (I have needed prednisone in varying doses.), Imuran (I had a severe negative reaction that lasted for about one week. but this drug does help many MG patients.), Cyclosporine and Cell Cept.  These drugs are quite a mouth full, but after a while they become common language for MG patients.  Mestinon is a different drug classification.  It improves neuromuscular transmission.  It helps relieve drooping eyelids. (I have been helped by varying doses of Mestinon throughout my illness.)

My next chapter in this MG series will address diagnostic tests  and treatments  for generalized Myasthenia Gravis.

Sources: The Myasthenia Gravis Foundation of America, University of Cincinnati Net Wellness, Medscape News and Perspective, Conquer MG of Il, My awesome treatment team, and Fellow Snowflakes

 

Myasthenia Gravis, The Snowflake Disease (section one, a description)

This is the first article in a series that will provide information about Myasthenia Gravis.  My target audience is patients, nurses and allied health providers.  Anyone with an interest in learning more about the topic may find this helpful. I am a retired nurse and psychotherapist.  I am a patient with the diagnosis of Myasthenia Gravis. Throughout my treatment, I have met many medical personnel who have not seen MG before.  In my years working in healthcare and social services, I had not seen this disease.  I am hoping that this article will help providers treat MG patients. I  also intend to encourage patients to know their bodies and learn how to manage their own symptoms  This is an effort to advocate for MG patients and the people in their lives. This is my way of flipping a negative experience around .  Perhaps I can provide a service to someone who needs it. Here goes:

The name, Myasthenia Gravis, literally means “Grave Muscle Weakness”. Myasthenia Gravis is a chronic autoimmune neuromuscular disease. Immune systems normally protect our bodies. When someone has MG, the immune system mistakenly attacks the patient’s own body. Antibodies destroy receptors for acetylcholine at the neuromuscular junction.  This prevents muscle contraction.  Muscles do not receive the messages being sent.  When antibodies attack and destroy communication between nerves and muscles, weakness in the skeletal muscles occurs.

MG is a rare disease. Most of the written sources that I have researched report that twenty per every one hundred thousand  people acquire this disease. However, a provider recently told me that only three  per million people acquire MG, worldwide.  The nickname for this condition is “Snowflake Disease” because the symptoms vary from person to person.  A patient with this disease may vary from day to day, sometimes from hour to hour. Myasthenia gravis can effect any of the voluntary muscles. Individuals may have one or many symptoms. The muscle weakness can occur on both sides of the body. The symptoms may come and go. The following are some symptoms of MG:

The eyes may be effected.  Diplopia means double vision.  The patient sees two or three images rather than one.  Sometimes the images are overlapping and blurry.  Ptosis means that one or both eyelids are drooping.  Vision may become obstructed. Ocular symptoms are often the first signs of this disease.  15% of people with ocular MG have only ocular symptoms.  Most people move on to experience weakness in other muscles.

About 15% of MG patients report their early symptoms as being face and throat muscle difficulties.  Weak muscles in the mouth and throat can cause difficulties with speech, chewing, swallowing, and choking. Facial expression may be limited due to muscle weakness.  An MG patient may have difficulty holding their head up.

Weakness in hands, arms and shoulders can make it difficult for a patient to lift their arms, wash their hair, shower, and brush their teeth.  Arm and hand weakness can make it hard to lift items from cabinet and refrigerator shelves.  Jars will become more difficult to open.  Gripping items with fingers may be challenging.

Leg muscle weakness may make it difficult to stand  after being in a sitting position.  Walking may become difficult.  Balance may be effected and may cause a wobbling gait.

When the diaphragm muscles are weak, breathing is difficult.  The voice may become weak and raspy when these breathing muscles are not strong enough to move the larynx. .

Extreme fatigue often occurs. Activity worsens MG weakness.  Rest improves symptoms.  Remissions are possible. Exacerbations (flare ups) may occur.  Myasthenia Gravis is not life shortening in the way that some illnesses predict that a person will live for a certain amount of time. Most MG patients will experience a normal life span.  However, MG may be life threatening  when the respiratory muscles are weakened.

Section One has been a description of Myasthenia Gravis.  Future articles will describe diagnostic tests. Common treatments and medicines that may be prescribed by the Doctor will be listed. Myasthenia Gravis psycho socials needs will be addressed.  My final section will be the story of my personal journey with this disease.

 

Sources: Genetics Home Reference @ U.S. National Library of Medicine,  Myasthenia Gravis Foundation of America,  Conquer MG (Myasthenia Gravis Association of Il),  John Hopkins Medical Health Library, and The National Institute of Neurological Disorders and Stroke

Snowflake Art is provided by James Aiello, painter