Category Archives: tests and treatment for MG

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 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