Life with Myasthenia Gravis Myasthenia Gravis Survival Guide
Nutrition and Myasthenia Gravis Safety and Myasthenia Gravis
Myasthenia Gravis the ACh Receptor Emergency management of Myasthenia Gravis
Myasthenia Gravis Survival Guide
Safeguarding your health l Finding Neurologist l Support of family
Additional local support l Human Energy Conservation l Personal Hygiene
Visual Disturbance l Eating Difficulties l Therapies
General Medication Consideration l Drugs which may aggravate Myasthenia Gravis
Disability and the work place l Conclusion
l. Introduction
This guide is intended to help myasthenic to fully understand and cope with their disease process. The patient should be willing to become an active participant in achieving and maintaining a good quality of life, in conjunction with his/her family, his physician and other health professional.
The goal of this guide is to allow the myasthenic, through a better understanding of himself/herself, the health care system and other important factors, to participate in discussions with his health care team and make truly informed decisions about available treatment options.
One should note that not all myasthenics will experience severe symptoms and encounter emergency procedures. However, one needs to be informed of the possibilities and the intervention procedures.
II. Patient-Directed Health Management Outline
A. Safeguarding your health Top
1) Accept responsibility for your health; educate yourself with the available information about MG. Resources for pamphlets, articles, and current research information include:
a. Myasthenia Gravis Foundation,
b. Your local chapter:
c. Medical journals
d. Personal accounts and articles
2) Carry a personal written information sheet containing the following information:
a. Your medical diagnosis(es).
b. Symptoms you experience when your MG worsens - state your normal lung vitalcapacity.
c. All prescribed medications (for MG and other illnesses), and medication allergies. Also list any over-the-counter medications you take regularly.
d. If you take Myestin -list the symptoms of Myestin overdose.
e. List drugs or treatments which worsen your MG.
f. List therapies and/or medications that proved helpful in the past under similarcircumstances.
g. List the phone numbers of your private MD and neurologist and two friends orrelatives.
B. Finding a neurologist Top
1. Find a neurologist who is knowledgeable about MG.
2. Talk with your family doctor~ other myasthenics and friends about neurologists knownto them. Your type of health insurance or lack of insurance may also influence which
physician you choose.
3. On the first visit, take a complete set of your medical records. Have the doctorplace a copy of your records in your file.
4. If you have a difficult time remembering what the doctor tells you:
a) Bring a family member with you;
b) Write it down;
c) Ask him to write it down;
d) Ask to tape record your discussion.
5. Your medical care plan: a. Help develop a treatment plan choosing current therapieswhich incorporate your abilities and goals. b. Discuss a personal treatment plan if your condition should worsen. c. Give your neurologist the telephone number of your family doctor. d. If you have respiratory involvement, request an incentive spirometer for home breathing evaluation. With a spirometer a patient can give an objective evaluation of
their breathing capacity. Please note: measure your breathing capacity with one
maximum effort. (Also ask for instructions!) e. Meet your doctor as often as
necessary to discuss changes in your care plan as your condition changes.
6. Family Physician
a) Have a Physician's MG handbook sent to your doctor to provide him with a guide toMG treatment.
(Contact the National Myasthenia Gravis Foundation Office)
b) Discuss your treatment plan with your doctor.
c) Discuss the difficulties you face when your MG worsens; include helpful treatmentsbased on past experiences.
d) Provide family M.D. with the telephone number of your neurologist.
C. Support of family and friends Top
1. Educate family and friends about Myasthenia Gravis.
a) Obtain literature for family members about MG from the National or local chapter of the Myasthenia Gravis Foundation.
b) Invite family and friends to MG support group gatherings.
2. Develop a distress phone signal for use if you are unable to talk, i.e. 2 rings, then hanup. Use phone receiver clicks to answer yes or no questions; one click for yes and
two clicks for no. Write down some ''key" questions to ask; such as, "Are you having
trouble breathing?'' or "Do you choke when you try to swallow?"
3. Take literature from the MG Foundation to the hospital with you. You can also get Freecopy of "Coping with Myasthenia Gravis" from the office of VHB Pharmaceuticals Pvt
Ltd.
4. If necessary, attend group therapy sessions with others dealing with the realities ofliving with a chronic illness to ventilate feelings, and share coping mechanisms, and
ways to solve common problems.
D. Additional local resources for your support Top
1. Support groups and local auxiliaries.
2. Organize an "MG emotional support wheel" of people to call in your area during difficult times.
3. Individual or group therapy sessions if needed.
lll . Human Energy Conservation Top
Kitchen Tips l Shopping Tips
These general suggestions for human energy conservation may apply to the work place or home and to men, women and children.
General Suggestions
Don't stand when you can sit.
· Schedule regular rest periods during each day.
· Reorganize daily tasks and reschedule some 3 to 4 times a week to allow for adequaterest each day.
· Delegate tasks to other family members or keep a servant.
· Plan all activities and eliminate extra steps, assemble all the necessary equipmentbefore beginning.
· Move frequently used devices or objects to low, easily accessible shelves for yourconvenience.
· To ease difficulty getting up from a sitting position try leg extenders to elevate chairs.
· Use power tools, and electrical appliances when possible to conserve energy, i.e.,electric mixer, electric screwdriver.
· Use walking aids if necessary for stabilization and support. Install grab bars in yourhome, or use a cane, walker or wheelchair as needed.
· Try a shoulder pad or intercom phone to completely free your hands when talking onthe telephone.
· Avoid hot and cold weather extremes; they exaggerate weakness.
· Avoid aerosol pesticides and cleaners, since they may trigger or aggravate symptoms.(Many pesticides contain neuromuscular paralyzing agents and may adversely affect
the MG patient.)
· Avoid excessive alcoholic drinks, tonic water (contains quinine) and smoking.
· Avoid any medications or activities that you know make you weaker.
· Avoid stressful situations
Kitchen Tips
When preparing foods chopping - dicing, etc., use a food processor.
· Look for various kitchen gadgets and aids in catalogs to help you.
· Transfer heavier bulk items, such as sugar and flour, into smaller containers easier tolift and handle.
Shopping Tips
Shop at peak medication times; avoid shopping during peak store or traffic hours.
· Shop by yourself only when you need a few items, saving big shopping trips for otherfamily members; or shop with a friend or family member who can assist carrying
groceries.
· Shop by telephone or mail when possible.
· Obtain a handicapped parking sticker if necessary.
lV.Personal Hygiene and Grooming Helps Top
Allow ample time to do grooming in "'phases", with rest periods in between; use electric appliances to conserve energy, i.e. electric shaver.
· Place a stool in the bathroom to sit on while shaving or applying make-up. Prop yourelbows on the counter top if necessary.
· Take short showers or baths using warm, not hot water.
· Install grab bars in the shower or next to the commode if needed.
Visual Disturbances (Blurred or Double Vision) Top
If double vision is a problem, an eye patch may be helpful while reading or watching TV. Alternate the eye patch from one eye to the other to avoid eye strain.
· Have your eyes checked to make sure you do not also need glasses.
· Rest for 30 minutes with your eyes closed before going out. Also take along an eyepatch if you develop double vision.
· Ptosis - eyelid crutches, tape eyelids open.
Eating Difficulties Top
Grind meats to make them easier to swallow
· Between meals with cold beverages; hot foods tend to increase muscle weakness andswallowing difficulties.
· Try semi-solid foods. They may be easier to handle than solids or liquids, (i.e. milkshakes, yoghurt, pudding or milk-kheer).
· Avoid foods that crumble and may be aspirated, (i.e. toast, cake, and potato chips.)
· Enlist the help of a registered dietitian if you need help in meal planning.
· If you take steroids, avoid salty foods and notify your physician of sudden weight gain(may indicate fluid retention) or gradual clouding or narrowing of vision (may be due to
the development of cataracts or glaucoma.)
· Keep nutritious snacks handy. Try milk shakes, Diluted fruit juice or pudding forsnacks.
lV. Current Therapies: making Informed Decisions Top
Any combination of therapies may be employed in the treatment of Myasthenia Gravis. There is no "standard" treatment plan for all myasthenics. No two patients' courses are the same; therapies which work for one patient may not help another. Choosing which therapies to use should be the decision of your "team": you, your family and your physician.
Some important considerations when making your choice may be: your current condition and level of ability/disability; effect of therapy on your ability to work; effect of therapy on your ability to carry out your daily activities; loss of income if extended absence from work is necessary; side effects of the therapy; expected change of life-style as a result of the therapy; and the cosmetic effects of certain therapies (specifically thymectomy and prednisone therapy).
Factors which are very important to one individual may be insignificant to another. Carefully evaluate all the available information - because the impact of these factors (plus others) will greatly affect your positive self image, your acceptance and ability to cope with your illness, and ultimately, your journey toward wellness. Your choices of therapy should follow a discussion with your doctor and family about the advantages and disadvantages of each medical or surgical treatment offered, plus thorough consideration of the factors listed.
Current therapies for MG include:
1. Anticholinesterase therapy - pyridostigmine bromide (Myestin); neostigmine (Prostigmin)
2. Prednisone therapy - daily therapy and alternate-day therapy
3. Immunosuppressant therapy - azathioprine (Azoprin); cyclophosphamide (Cycrame);cyclosporine (Sandimmune)
4. Thymectomy
5. Plasmapheresis
6. Other helpful therapies - atropine; pro-banthine; ephedrine
A brief discussion of each therapy follows. Dosages are not listed as they are very individualized and must be carefully adjusted to adequately control your symptoms. Obtain more detailed information on all medications from your doctor and pharmacist before starting the therapy.
Anticholinesterase Therapy Top
Anticholinesterase therapy prevents the breakdown of the neurotransmitter acetylcholine, allowing more acetycholine to accumulate. With more acetycholine, there is more control of voluntary functions such as eye movements, limited strength, swallowing and breathing.
Pyridostigmine bromide (Myestin) is the most common drug used in the treatment of MG. These medications increase muscle strength by blocking the enzyme which breaks down acetylcholine - a chemical substance (neuro-transmitter) necessary to transmit nerve impulses to the muscle tissue. An increase in muscle strength is usually noticeable within 20 to 40 minutes after taking the medication. Weakness returns as the drug wears off in approximately 3 to 4 hours.
Side effects:Stomach cramps, diarrhea, increased perspiration, increased salivation, muscle twitching, palpitations, increased urinary frequency.
Instructions: Medication is taken in the prescribed dosage every 3 to 4 hours, before meals or with a snack. The medication must be taken at regular intervals to maintain muscle strength throughout the day. A "brittle myasthenic" may be adequately medicated during one 3 to 4 hour period and over-dosed with the same dose in the next 3 to 4 hour period. Medication dosage may require frequent adjustment according to your response to the drug and your hourly/daily activity level. When making Myestin adjustments do not alter the dose more than 1/2 tablet (30 mg.) at a time. Myestin extended-release tablets act for 6 to 12 hours and are best when taken at bedtime. This allows for comfortable, uninterrupted sleep and reasonable strength in the morning.
Precautions:Simultaneous use with high dose anticholinergics (atropine), hydrozyzine (Vistaril) may decrease the effects of Myestin . Warning-If increasing shortness of breath or difficulty swallowing occurs within 20 to 40 minutes after a Myestin dose, these are symptoms of overdose! Call your physician or go to his office or the hospital immediately. Myestin extended-release is not recommended for daytime use because of the unpredictable release and variation in myasthenic symptoms.
Prednisone acts in part as an immunosuppressant. "Immune" refers to the immune system. The immune system protects you against foreign bacteria, viruses, etc. In some illnesses, the immune system produces antibodies which become overactive and cause undesirable effects. Prednisone suppresses the production of antibodies. This can make it slightly harder for you to fight off infection, but also stabilizes the immune system if it is overactive.
Prednisone, a synthetic glucosteroid, is the steroid of choice in the treatment of Myasthenia Gravis. It has both anti-inflammatory and immunosuppressant properties. It often proves helpful in a wide variety of other autoimmune diseases including rheumatoid arthritis and systemic lupus erythematosus. Several options are available when starting steroid therapy, daily or alternate-day therapy. Daily therapy is usually started when the patient's condition is critical or otherwise unstable, and quick relief of symptoms is desired. Once the patient is stable, a gradual change over to alternate-day therapy may be attempted. When patients begin alternate-day therapy, it takes longer to feel the positive effect of the drug, weeks to months.
Side Effects:Usually related to dose, frequency and condition of the patient. Generally, the higher the dosage, and the more often you take the drug, the more prone to side effects you become. However, alternate-day therapy produces fewer side effects than daily steroid use. Also, the longer you take steroid therapy, the more apt you are to develop these side effects. Gastrointestinal nausea, vomiting, increased appetite, gastric irritation, peptic ulcer. Generalized skin thinning, acne, increased sweating, increased hair growth, facial roundness, easy bruising, delayed skin healing, insomnia, nervousness, irritability, headache, mood changes, high blood pressure, osteoporosis, increased susceptibility to diabetes, menstrual irregularities, and lowered resistance to infection.
Instructions:Taking the medication with meals may decrease stomach upset; however some physicians recommend you take steroids on an empty stomach. Avoid taking with antacids; antacids decrease the absorption of prednisone from the stomach. Take in the morning, around 6 a.m., to correspond with the body's normal release of steroids from the adrenal glands. Prednisone causes the body to lose potassium, so eat potassium-rich foods (bananas, orange juice, citrus fruits, and melons). Restrict salt intake.
Precautions:Prolonged use may cause cataracts or glaucoma; notify your doctor if your vision becomes gradually impaired. Severe hypokalemia (low potassium) can occur with simultaneous use of diuretics ("water pills"). Simultaneous use with other drugs which cause stomach upset may increase gastric discomfort. Prednisone interferes with the effectiveness of oral anticoagulants. Simultaneous use with antidiabetic agents may require higher doses of the antidiabetic drugs. Do not stop medication abruptly; it must be slowly tapered off over a lengthy period of time under the close supervision of your physician.
Immunosuppressant Therapy Top
Since MG is a disorder of the immune system, treatments that suppress the immune system have been used in MG with some success. Because an MG patient has an overactive immune system, the goal of their therapy is to reduce a myasthenic's symptoms by suppressing the overactive immune system.
Azathioprine (Imuran) may be chosen when use of an anticholinesterase or prednisone therapy is not effective. Often it is given in conjunction with plasmapheresis. The effect of this drug is not immediately felt, unlike the dramatic improvement following a Myestin dose. There may be slow, gradual, improvement over a 3 to 12 month period.
Side Effects:Low white blood cell count, anemia, low platelet count and bleeding. Also nausea, oral lesions, and liver toxicity.
Precautions:Live vaccine vaccinations are contraindicated. Frequent blood counts should be done
when therapy is started with less frequent blood counts for the duration of therapy. Periodic liver function tests should also be performed.
Cyclophosphamide (Cycrame)
Sometimes used in patients who fail to respond to other available therapies. It is commonly used as a "chemotherapy" agent in cancer treatment. It is not one of the "first line" drugs used to treat MG because of its harsh side effects and potent immunosuppression.
Side effects:Nausea and vomiting; mouth ulcers; colon hemorrhage; low white blood cell, red blood cell or platelet counts; interruption of menses or sperm production; hair loss; lung fibrosis; bladder hemorrhage.
Precautions:Simultaneous use with steroids will increase immunosuppressant activity. Simultaneous use with barbiturates may increase drug toxicity. Certain types of cancers, especially urinary and bladder cancers, have been known to develop with the use of this drug.
Cyclosporine, a potent immunosuppressive agent, is currently being researched as a possible new drug for the treatment of MG. Some notable side effects include nausea, vomiting, hypertension and kidney toxicity. Although it is felt to be a promising new drug, the risks and side effects are not yet known. Additional information will become available as research and clinical trials progress.
Thymectomy Top
Although the role of the thymus gland in Myasthenia Gravis is not fully understood, published 1983 study findings found as high as 85% of patients who underwent thymectomy showed clinical improvement. The decision whether or not to have this surgery should be made on an individual basis.
Plasmapheresis Top
Plasmapheresis is a short-term treatment in which several liters of blood are removed, spun in a centrifuge, and the red blood cells are returned in artificial plasma (aluminum and saline solution). During plasma-exchange the amount of antibodies located in the blood is reduced.
Plasmapheresis is generally reserved for patients who fail to respond to other therapies or for those who need an acute temporary boost. The patient undergoes a series of plasma exchanges. Temporary relief of symptoms may occur after 7 to 8 exchanges. This short-term treatment is sometimes used to strengthen a patient in preparation for a thymectomy. Prednisone or azathioprine (Imuran) are commonly given to patients undergoing plasmapheresis. Immune human serum globulin (gamma globulin) injection may also be given during this therapy to restore valuable serum antibodies removed by the plasmapheresis.
Other Helpful Therapies Top
Low dose atropine is sometimes helpful to relieve the cramping and diarrhea often caused by Myestin. Propantheline bromide (ProBanthine), a synthetic drug similar in action to atropine, may also be helpful in treating gastrointestinal discomfort. Caution must be taken not to take too much atropine since it antagonizes the effects of the anticholinesterase drugs and may result in weakness.
Ephedrine is sometimes used with anticholinesterase therapy. The drug gives a "boost" in muscle strength in some myasthenics. Possible side effects include. headache, high blood pressure, nervousness, palpitations and insomnia.
VII. General Medication Considerations Top
Know your medication allergies and keep this record on your written information sheet.
· If you are very weak in the morning, keep one dose of medication and water at yourbedside to take when you awaken.
· Use a watch with a beeper alarm to remind you to take your next dose reset after eachdose.
· Use a dosage record chart (Provided with Myestin) to keep a track of your dosageschedule.
· If swallowing is not a problems take medications with food to decrease stomach upsetand diarrheoa.
· If swallowing is a problem, you may crush tablets and take with milk.
· Keep several doses of your medication in your car, and your work place, as well asyour wallet or purse.
· Do not take new medications, especially "over-the-counter" medications withoutasking your doctor first.
· For sinus and allergy sufferers, there is a new antihistamine which is safe formyasthenics (does not exaggerate weakness or cause drowsiness). It is terfenadine
(Seldane) 60 mg. tablets. If you occasionally need an antihistamine, ask your doctor
about this drug.
Drugs which may aggravate Myasthenia Gravis Top
(Webmaster Note: This is not a complete list and new drugs have been found to aggravate weakness since the publication of this pamphlet. Consult with your physician).
Heart medications - quinidine and propranolol, procainamide .
· Rheumatoid arthritis and gout medication - Colchicine, d-penicillamine
· Antidepressant medications - amitriptyline
· Antihistamines - diphenhydramine (Benadryl), plus others. (May be tolerated in very lowdoses).
· Antibiotics - Gentamicin, Neomycin, Kanamycin, erythromycin, tetracycline, somepenicillin derivatives.
· Narcotic medications - pain relievers like Morphine.
· Sedatives - Barbiturates
· Tranquilizers - Atarax, Valium, ("nerve pills"). (If used, the dose should be25-50% of the normal dose).
This is not intended to be a complete list of all the drugs that affect MG. So many new drugs are approved each year that it is impossible to predict what effect their use will have on the myasthenic. Be particularly cautious about drug interactions when medications for more than one medical problem are prescribed. Periodically sit down with your neurologist and family physician and review all your medications, prescribed and over-the-counter. Keep a medication journal and note medications which aggravate your MG. Keep your doctors up-to-date with your "medications to avoid" list. Add these jmedications to your medical information sheet next to your medication allergies.
VIII. Disability and the Work Place Top
Individuals with Myasthenia Gravis who have significant physical limitations or visual impairment may find it necessary to "creatively" alter their job schedule in order to continue working. For those who enjoy work, the threat of losing your job can a be devastating blow to your self-esteem and confidence. For those who want or need to continue working, consider working part-time, flexible work hours, or light duty assignments if available.
People with ocular Myasthenia Gravis should tap resources available to the visually impaired. Severe ocular Myasthenia Gravis may be regarded as functional blindness and retraining for job skills not requiring sight may offer new job avenues.
Depending on your abilities and limitations, many other resources may be available to meet your needs. If you want to work, do not hesitate to explore all possible pathways back into the work place.
IX. Conclusion Top
It is the intent of the VHB Pharmaceuticals Pvt Ltd that every myasthenic be well informed and have current available information. By being well informed the myasthenic patient will be better able to understand MG. the management of MG, and actively participate in his or her own wellness.
This pamphlet, of course, is designed as merely a guide. As with all such materials, the reader is cautioned to consult with his/her physician prior to reaching any conclusions or taking any advice from this or any other periodical.
DISCLAIMER:
All information is for educational purposes only. Contact your physician to make medical decisions