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MG & ME (SECTION FOUR, MY PERSONAL EXPERIENCE WITH MYASTHENIA GRAVIS, THE SNOWFLAKE DISEASE)

The first three sections of this Myasthenia Gravis article were based on facts and researched medical information.  This section is about me and my reactions to the losses caused by this disease.  I have been coping with MG for the past three years; actually many symptoms were here for a year before I actually collapsed.  I had been dealing with my symptoms with denial and rose colored glasses.  I insisted that I wasn’t feeling “that bad”.  Most of us tend to deal with big obstacles by using whatever coping tools have worked in the past. I tried forging through with a positive attitude.  I later learned that I was making myself sicker by pushing it with activity.  If you are an MG patient or if you are reading this with a patient in mind, your snowflake experience may be very different than mine, that is why MG is nicknamed the snow flake disease.  We are similar, yet different.

My eye muscles were the first muscles that I noticed weakening.  My eye problems began about four years ago. At that time, my eye difficulties were fleeting.  They would come and go. Sometimes it was hard to open my eyes.  One or both lids would droop (ptosis). I saw my long time family doctor about this.  There was no conclusive diagnosis or treatment at the time.  Double, triple, and overlapping vision (diplopia) began about four years ago.  It was also off and on.  At church, I would see several priests, crosses and statues. I would see several of the same characters and objects in movies. This began to happen in traffic, so I went to see my ophthalmologist. He examined me and referred me to a neuro opthalmologist. I am fortunate to live near an urban area with large medical centers because this is a rare specialty.  During my first appointment, I was fitted with stick on prisms.  These pieces of plastic clung to my glasses and immediately corrected by double vision. I was so thrilled and grateful to be able to see. fullsizerender-2 I went to work the next day rejoicing about my improved vision.  The neuro ophthalmologist assessed that my eyes were out of sinc. The left eye moved much more slowly than my right eye.  I had difficulty following his fingers.  This doctor ordered an MRI of my brain and a variety of blood tests. He was concerned about Myasthenia Gravis or a brain tumor. Test results were not conclusive. I did not freak out about the possibility of a serious diagnosis.  I remained in la la land and was thrilled with the benefits of my prisms.  I continued to use my cling on prisms until a few months ago because my eyes have changed so frequently and so drastically during this past four years. I kept my stick ons because I did not want to need new glasses every few months. My stick ons look goofy, but I really did not care. The improved vision out weighed my appearance. I now have glasses with ground in prisms. These glasses do not look unusual. Sometimes the prisms work. Sometimes they don’t. At times, I need to keep my left eye closed or covered. That’s the nature or being a snowflake.

As my vision decreased, I was also losing my stamina. I remember a specific day in my garden.  I could not use the muscles in my hands and I was terribly exhausted after fifteen minutes of weeding.  I used to love to spend entire days gardening.  I cried that day and said I was out of shape.

I had been in the same Zumba class with the same teacher and classmates, twice per week, for a few years.  I was unable to keep up.  I found myself slipping closer to a back corner of the gym.  I am someone who loved exercise. I used the elliptical machine or rode my bike daily. I live near beautiful path that I walked several times per week. I participated in lots of 5K fund raising walk/runs. One by one, these activities became more difficult. I replaced some of these workouts with easy exercise videos. I did not know yet that I was harming myself with exercise.  We live in a society that promotes exercise for everything.  Exercise is good for most conditions.  It is often not good for Myasthenia Gravis.  Exercise that weakens muscles exacerbates MG.  Sometimes the harmful antibodies rush toward the exerted muscles and attack.  I did not know yet that  I had MG. I kept trying to push my limits.  I had never been good at accepting limits.

I was simultaneously having cognitive problems. I was saying that I just wasn’t smart any more.  I am someone who has always loved to read. I was dealing with a problem that was very different than my vision difficulty.  I could see individual words, but I could not make sense out of sentences and paragraphs. This was frightening. I later learned, from my neurologist,  that this was due to low oxygen in my brain. I was unaware that I was having breathing and lung problems caused by MG.

I continued to go to work. After work. I would then collapse on my couch with my head tilted up to breathe. Sitting on the couch felt like I was running.  At work , I was very short of breath after walking a short distance.  I was embarrassed and tried to find places to be alone to catch my breath. I continued to call myself “out of shape”.  I made an appointment with my long time doctor.

My doctor’s appointment was scheduled.  I was at work when I picked up the phone and found that I could not speak.  My voice was a raspy whisper. I shrugged it off as laryngitis and decided that I was catching a cold.  Three years later, my voice is still a raspy whisper. When I kept my doctor’s appointment, I was sent for a chest x ray, chest ct scan, and a pulmonary function test. These tests showed severe lung impairment. I was also tested for the need for oxygen.  My saturation levels are very low.  I was prescribed oxygen.  I have an oxygen concentrator in my home and tanks  for when I go out.  This was three years ago.  I was recently retested and I am directed to continue my oxygen.fullsizerender-2

I began pulmonary rehab, which consisted of exercise machines.  I did quite well, but my oxygen levels dropped in response to activity.  My fellow rehabbers oxygen saturation went up, as it was supposed to. I was also prescribed additional pulmonary meds and treatments. Pills, nebulizers, and inhalers were part of my daily routine during that first year.  One by one, these lung treatments were eliminated as it became clear that my lung weakness was due to chest muscle weakness , rather than lung disease.

I continued to weaken.  A walker and wheelchair were needed. Needless to say, I could no longer work. At this point, I returned for an appointment with the neuro ophthalmologist. When he saw my breathing difficulty combined with double vision, he said that another assessment for Myasthenia Gravis was needed. I saw a neurologist who gave me a thorough neurological physical exam.  I felt that someone finally got it.  He connected all of these seemingly unrelated symptoms and said , “I think you have Myasthenia  Gravis”.  He directed me to stop the pulmonary rehab because the exercise was making me sicker. My breathing  difficulty was due to MG breathing muscle problems rather than lung disease. Of course, he needed more evidence than his exam. Brain and spinal MRI’s were completed, as well as more blood work. All of those tests were uncomfortable. The test that gave the absolute diagnosis of Myasthenia Gravis was the single fiber EMG.  It was a very painful and lengthy test.

I was sent to an ENT because of my difficulty speaking.  I saw him several times for testing. My vocal chords were fine. This confirmed that my difficulty speaking was caused by my breathing muscles being too weak to move my vocal chords.

Now that I had an absolute diagnosis, treatment began.  I was started on Mestinon, a common pill for this disease.  Over the months, my dosage increased. I felt an improvement of energy and strength. I now take a large dosage of Mestinon, four times per day.  A tolerance for Mestinon is diagnostic in itself.  If someone does not have MG, they will feel ill in response to the medicine. I soon felt almost normal for about one month. This crashed. All my muscles weakened. I could not grasp things. My legs lost their strength.  My breathing and vision worsened.  This lead to a ten day stay in ICU in the hospital.  I was told that I may need a respirator.  I was treated with with IVIg infusions.  By the 5th day, I was feeling stronger. On the tenth day, I was able to come home.fullsizerender-2

Until this point, I had strongly refused Prednisone, a steroid that is often used to treat MG. I did not want the side effects.  My stint in ICU had been so frightening that I followed my doctor’s advice and began prednisone. I started with small doses that were gradually increased.  One of my side effect fears happened: weight gain and fluid retention. I gained sixty pounds and developed the stereotypical moon face that happens with steroids. I needed the strength provided , in spite of these side effects.  For that last several months, my prednisone is being gradually reduced.  I now take no prednisone every other day.  The opposite day is a low dose.  I am eager to be free of steroids, but grateful for the strength this medicine has given me when I was unable to do things on my own.

Five months after my ICU hospitalization, I was sent to an MG specialist for a consultation.  He hospitalized me right away. This was a five day stay. This time my treatment was Plasmapheresis. An indwelling catheter was surgically placed in my clavical area. In this plasma exchange, my blood was removed, my harmful plasma was removed and replaced with artificial plasma.  This is an over simplified description, but it is basically what happened.  I was then started on a medicine called Imuran. I had a terrible reaction to Imuran that lasted for a week. This medicine was stopped.  I did not feel much benefit from Plasmapheresis.  I try to think that I would have been sicker without it. FullSizeRender (2)

Two more hospitalizations were needed for IVIg and IV steroids.  I am now on a new regime.  I am excited and hopeful.  Every three weeks, I go to an out patient infusion center for one day. It is a comfy room with a lounge chair and TV.  I have been there three times. The day of and the day after, I have felt ill. This has been due to my body being swarmed by the immune globulin of a thousand different donors. This has been followed by feeling pretty good for two weeks.  My voice has even been stronger.  At the end of the second week, I have started to fizzle out. By the time I have shown up for the next infusion, I have been weak and raspy. However each infusion has really helped.  If I continue to weaken at the two week point, My doctor will change my interval  to 2 1/2 weeks.  However, he says that each infusion will last a little longer.  The plan is for out patient IVIg to be ongoing.

My purpose in writing these articles has been to help anyone effected by Myasthenia Gravis.  This chapter has told the events of my journey with MG.  My next chapter will cover emotions.  I will share tips for making tasks easier. I will talk about mistakes that I have made and the lessons learned. Gratitude and hope  are also a part of this journey.  I’ll see you in Chapter Five.

 

 

 

 

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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