Category Archives: Myasthenia Gravis

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

 

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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

 

 

 

 

 

 

 

 

 

 

 

 

 

Do Yellow Footies Really Prevent Falls? Really?–Days four and five in the hospital

By day four of my hospitalization, things were going smoothly.  Competent nurses were helping me.  I was receiving the medications and IVIG transfusions needed to treat my disease, Myasthenia Gravis.  I was looking forward to going home soon, following my last transfusion.

The evening nurse, who identified herself as the charge nurse, disconnected my finished transfusion.  I reached for my blue slippers. I brought these slippers from home and I have been wearing them daily when up.  I wanted to walk to the bathroom now that I was free from my IV pole for a while.

The nurse said that my slippers should be yellow.  I gave her a befuddled look.  She repeated that it was important for me to wear yellow slippers.  I responded with a weak giggle.  I mistakenly assumed that she was making a joke that I didn’t understand.  After I giggled, she said, “Didn’t THEY bring you a pair of yellow footies?”.  I shook my head in a “no” motion.  She elaborated that I was on fall precautions.  She explained that I was to wear yellow footies at all times, so all hospital staff would keep an eye on me to keep me from falling.  She left the room, saying that she would get me some yellow footies right away because THEY did not give me yellow footies.

I kept my giggles to myself as I read the board on my wall.  It said, “Up At Lib”.  I recalled other staff showing me how to unplug my IV when I wanted to get up.  When this nurse returned, I offered no resistance to accepting a new pair of yellow footies.  However she did not bring the yellow slippers.  She said that she told THEM that I was steady and alert, but that THEY insisted I be on ‘fall precautions’ because of my diagnosis and “many factors”.  I don’t know what the many factors were.  Several hours later, the nurse returned to my room.  She looked at my feet and said, “THEY didn’t bring your yellow footies yet ?”  She seemed quite annoyed with THEM.  I simply said, “No”.  Footies were never mentioned again and I did not receive any.

Day five arrived.  My care was excellent.  At 11pm I slumbered into sleep, knowing that I would be going home the next evening. Each night in the hospital, I had turned out my lights and closed my door because of the bright lights and noise in the hall.  I followed my routine on my last night here.  At midnight, someone opened my door widely and turned my lights on.  Of course, this woke me up.  I decided this person had forgotten their blood pressure cuff, or meds.  I waited for about an hour, unable to sleep because of the light and noise.  I decided to get up, turn off my lights, and close my door again.  As I put my feet over the side of the bed, piercing loud alarms began to ring repetitively .  I walked to the hall and asked the CNA why my room was making noises in the middle of the night.  The CNA said that the nursing supervisor had come through, opened my door, put my lights on, and activated a bed alarm because she wanted me to be on Fall Precautions.  As the CNA said, “nursing supervisor”, she seemed quite intimidated.  I stressed that I had been up without precautions all week.

I called for my nurse, who shook his head and said, “The supervisor did this”?  He turned off alarms and he apologized for the supervisor’s disruption.  I was so grateful for his common sense.  I again read the board on my wall that said,”Up At Lib”.  Following this commotion, it took quite a while to return to sleep.

The next day I was exhausted.  My doctor said he would be talking to the supervisor about  her behavior. This was not helpful to me, but perhaps a future patient will be spared sleep deprivation.  My discharge day had arrived.  I would be going home in the evening after my last transfusion was completed.  On this last day, yellow footies were not mentioned by anyone.

 

 

 

 

 

 

 

 

 

 

Hospitalization Stress

On day two in the hospital, I woke up feeling relieved that last night’s irresponsible nurse had said that she would not be here today.  Actually, she never returned.  Today’s nurse was wonderful.  She was efficient, kind, smart, and helpful.  Yet her hands were somewhat tied by mistakes that had been made he night before.  I was patient when my morning medicine was late.  I am aware that it can take some time to get things rolling along for a new patient.  When I finally put my call light on, my nurse looked baffled.  She stated that the night nurse had not put through my admitting orders.  This nurse did not know about my med order. In addition to my doctor’s orders,  I had arrived with a list of my meds, doses, and frequencies.

This day nurse worked hard to contact the pharmacy and to fix what the previous nurse had neglected.  However, this meant that I missed my 7pm, 8 am, & 12 pm doses of Mestinon.  This is an oral medication that has helped me for these past two years, as I cope with Myasthenia Gravis, an autoimmune disease that effects all my voluntary muscles.  On my own, I have managed not to miss any doses, prior to today.  This really good nurse got my Immunoglobulin transfusions flowing without further pain or bruises. The night nurse was also great.  She helped me feel safe and cared for while in the hospital, quite a contrast to the previous day.  I had no idea what was going to happen the next day.  My doctor was angry about my missed doses.  He talked with me about how he hated to raise his voice to staff, but it had been needed.  I appreciated his advocacy.

The events of the morning seemed routine on day three.  My oral meds were on time. Breakfast was tasty.  My  nurse was friendly.  Mid morning, I asked my nurse when she would be starting my IVIG.  She responded by saying that I could not have it until 10pm.  I explained that the only reason for this hospitalization was to receive these transfusions each day.  She shook computer print outs at me to prove she was right.  I explained the importance of my treatment.  I stated that her papers were mistaken.  Four times, she said, “Don’t cry”.  This baffled me because my emotions were nowhere near tears. I was assertive, as I asked for my treatment.  Without explanation, at 2 pm she started by transfusion, rather than holding it until 10pm, as she said that she would.

I was enjoying an afternoon visit with my son.  At 4:30pm, I put on my call light for my 4pm Mestinon.  C NA’s responded, but my nurse did not appear until 5:10pm.  She announced that she had to pass all her 5pm meds first.  She would then bring my pills.  My son said, “She will bring your 4pm medicine after she passes her 5pm meds to other patients”?   I continued to use my call light.  I told others what I needed.  One half hour later, she returned and announced that I could not have my 4pm dosage until 7pm. Again she waved her papers at me as proof that I could not have the medicine that my doctor ordered for me.  As I explained  the importance of my medicine,  she said “Don’t Cry”.  I explained that my voice is weak and raspy for the past two years because by breathing muscles are not strong enough to move my voice box, not because I am crying.  My son reminded the nurse that I had told her earlier that my impaired speech is a symptom, due to my disease, not due to crying.  My husband said to this nurse, “Look at her, do you see any tears?.  I asked my nurse to call the doctor.  She scolded me and said the doctor doesn’t make the decisions: the pharmacy and nurses do.  I felt that staff forgot that I was sick and weak.

At this point, we could hear my son speaking to my doctor’s answering service.  He asked to be called back because of the poor care I was receiving.  He asked the nurse if she would like her mother to be treated the way that I was being treated.  She looked sad and she said,”No”.  “Well, this is my Mother and I don’t like how you are treating her. Do you say ‘Don’t cry’ to all your patients just to placate and shut them up? She is not crying.”

The phone in my room rang.  It was my doctor,  He apologized to me for the mistakes of the pharmacy and nurse.  He stated that if there were any more late meds, I should call him directly  He then spoke firmly with the nurse.  Arrangements were  made for meds from home to be brought to me so that I would  self administer from this point on.

This nurse left and I never saw her again.  The night nurses for future shifts were good.  By this, I mean that they did their job and for that I was grateful.  I had the same day nurse for the remainder of my stay.  My IVIG transfusions were started promptly at 7 AM on days four, five, and six.  My medicines arrived on time.  I was discharged on the evening of day six.  I was grateful for the kind, accurate care that I received during my last few days. I felt exhausted and relieved to have survived the stress and mistakes of the hospital.

It is two weeks since my treatment.  I have been feeling quite sick. This is a common side effect of the IVIG transfusions. The benefits will be here soon and I will feel this experience was worth it.

All of these experiences have been reported to proper authorities.  I hope education will be provided to the staff who need it and that praise will be given to those who fixed mistakes.  I have been able to forgive and move on .  This does not mean that the mistreatment was O.K.  It does not mean that I was not angry and scared.   It means that I can let go of it in the healing environment that I have created at home.

There will be future articles about this hospital stay.  They will about less extreme situations. Actually,  one of the scenarios is quite funny.  I also plan to write about what I’ve learned and how I hope to use it to help others.

 

 

 

 

Unusual Hospital Admission (I hope so)

I am hoping this was an unusual hospital admission and not the norm.  I am trying to have some compassion for my admitting nurse’s behavior because I arrived at change of shift  time.  I know this can be difficult.  This nurse’s comments and dirty looks toward the previous shift nurse told me that she wanted the previous shift nurse to do the admission tasks.

My nurse was grumpy and did look at me.   With a scowl on her face, she stared at the computer screen and scrolled down.  She said, “Why are you here?  I responded, ”  I am here for five IVIG transfusions.  My diagnosis is Myasthenia Gravis.”    She continued to scroll and then said  that there is no such thing as IVIG; you mean IVP; you are a kidney transplant.”I am not here for a kidney transplant” was my quick response; “My diagnosis is Myasthenia Gravis”

My bed was also the scale.  It showed my weight as being 20 pounds less than my actual weight.  I made a joking comment about liking the lighter weight.  I emphasized that my doctor’s scale and my scale matched.  She said she needed the bed scale’s number (wrong weight) to calculate my IV.

As she continued to try to argue with me, I suggested that she search for IV immunoglobulin.  She found it and with a click, she moved on.  IVIG is one of the few treatments for Myasthenia Gravis.  It was becoming very clear to me that she did not know anything  about my disease or it’s treatment.  Sadly, it was more clear that she was not interested in learning about it.

Four times, she asked if I was diabetic.  Each time , I said “No”.  She seemed to be obsessed with her diabetes question.  She seemed to forget my answer each time.  Several times, I told her that I need oxygen  and that I  have used 2 liters of oxygen for the past two years.  I looked at her blank face and wondered if she noticed I was short of breath.  I also  did not receive my Mestinon, the oral medicine that I have taken for the past two years.

This admitting nurse attempted to start my IV four  times  My arm was quickly purple and swollen.  She said , “Look at how puffed up your arm is..that proves I am in your vein.”  Her logic was so bizarre.  She then said she would not try any more and that the IV would be started the next day.  She answered her phone and she argued with someone who was reprimanding her for not having given morning meds to a different patient.

Our relationship began when she brought me a pill at about 7:30pm.  She announced that the pill would relax me and make me sleepy.  I asked if she was still planning to do my admission procedure and start my IV.  She said “yes” and I said that I would decline her 7:30pm sleeping pill. Her admission interactions concluded about midnight.

I asked for some hot water to use for the herbal tea bag that I brought from home.  She told me that patients were not allowed to drink hot drinks, in effort to prevent burns. This was not true. Hot soups, tea, and coffee were brought regularly to patients by others.  Another nurse saw me running tap water, as I tried to make my tea.  She immediately brought me a cup of microwaved water while telling me I should have asked my nurse for hot water. Yikes????

Needless to say, I was sick and weak during this episode. I would like to say that things got better during the next six days.  It did not get better.  I will be telling stories of my experiences in upcoming blogs.

 

 

 

Health Care Visit Day

IMG_4193 (2)The day began with a reminder call that my portable oxygen tanks would be delivered between ten and twelve o’clock. “O.K.” is my standard answer.  I have learned that the delivery may happen at any time.  Sometimes the delivery time may be a different day.  These tanks are not an emergency.  They will be available for times when I leave the house.  I have a large concentrator that is to be used  around the clock at home.  I also have a large portable tank that will be available in case of a power outage.

I have needed these tanks for the past 1 1/2 years.  I smile as I remember the beginning of all this.  I cleaned the house to the best of my ability.  I wore clothes that would have been reserved for dress up.  Now days, the oxygen delivery finds me and the house “as is”.  What was once a big deal is now a casual occurrence.

The need for this delivery has not yet become a casual occurrence.  A huge part of me wants to say that this delivery person must have the wrong house.  This part of me finds it hard to believe that I am considered this ill.

For many years, when I worked for a family service agency, a large part of my job was to serve people via home visits.  I was the one who rang people’s bell and entered their homes.   I was the healthy one who climbed over drifting snow to get to the door.  They put the Christmas tree out for me.  Sometimes I would be the only visitor that season.

I still feel like that provider of services.  As I open the door and accept the oxygen, I tell the provider how I am doing.  The other part of me is also present.  That part says that the person must have the wrong house.  They better leave now because I have to go to work.

 

 

 

Introversion and Chronic Illness

IMG_0587 (2)The traits of introversion are an asset when someone is burdened with a chronic illness.  It has been two years since my diagnosis of Myasthenia Gravis.  All of my muscles are weakened.  My arms, hands and legs have lost strength.  I have double/triple vision.  It is difficult to speak because my chest muscles are not strong enough to move my vocal chords. Over exerting makes all of this worse.  Rest has been emphasized as a key part of treatment.  When this began, I was still working.  I expected the illness to be followed by recovery.  I grieved my busy life.

Prior to this illness, I developed the ability to go inside myself for answers.  I love to read.  I love to make art.  I find myself in the zone, when creating.  Seclusion doesn’t scare me; it delights me.  Silence fills me up.

I know people who need commotion and drama to fill themselves up.  They need outside structure and conversation.  A person with those needs would have a much more difficult time coping with the isolation  of chronic illness.

I am not accepting my situation.  I do miss my activities.  However, I believe that I am having an easier time because I can find joy in solitude.