Georgia State Chapter

Newsletter

Myasthenia Gravis Foundation of America

A protocol for emergency care for every myasthenic is a great idea, but remember, as we are all unique and react differently, our crisis parameters will differ as well. The best I can offer is a 'shell' and you will need to think about your own specific details and how they relate to these tips.

1. At the next appointment with your neurologist, tell her/him that you want to understand what mechanism is in place should you "go into crisis". Do not accept any unequivocal responses (like, "Oh don't worry about that') but come up with a plan TOGETHER. It won't help if you've made a plan that excludes your neurologist's input - he/she is the best reference point you have.

2. Along with your neuro or his/her nurse, write up a brief explanation of your illness and all of your meds and dosages. Include your insurance info, next-of-kin, neuro's phone numbers and address and phone of the hospital you use. This is also a good time to tell your neuro who your advocate is (your spouse, friend, adult child). Make several copies of this and update it whenever there is a change. Keep a copy of this history in your medical information folder (You do have one, right?); in the glove compartment of your family car; in your purse/briefcase/pocket and in a prominent place in your home.

3. Locate the EMT/Fire Squad closest to your home and go there. Ask to speak with the captain and tell him/her where you live and that IF you should ever need an ambulance, they will have your history on file. If it’s refused (not unusual, since they don't want to be responsible for out-of-date info) ask him where you should keep this info in your house for the EMT squad to find.

In general, it is a good idea to get in the habit of keeping a medication "diary". This is really just a list of what medications you've taken each day, in what strength and at what times. This is invaluable to any caretaker should you not be able to speak for yourself.

what way did it manifest (fever, open sore not healing, nausea, vomiting? Have you been exposed to anyone with a cold or other contagious illness? Did you remember to take ALLof your meds today? What time was your last MG medication and did it help? Did it make your symptoms worse? Are you having problems breathing? What is the highest number you can count to and has it changed over the course of an hour? Did it get better or worse with medication? Does it get better if you slow yourself down and make yourself relax so that you won't hyperventilate? WRITE ALL OF THIS DOWN ON A PIECE OF PAPER - believe it or not, not being able to swallow your meds or enough liquid to stay hydrated is a SERIOUS situation for an MGer and you should go to an ER, no questions asked.

2. Either you or your advocate call your neurologist, as per the plan you drew up with him/her. Relate the above answers. Follow his/her directions. ****lf you feel you must go to the ER, have someone call ahead and ask for the Triage Nurse or Doctor in charge and state that a person with MG will be arriving shortly and cannot swallow, breath, move or whatever the crisis situation is.

TAKE THE PIECE OF PAPER WITH YOU ON WHICH YOU OUTLINED YOUR PROBLEM AND WHAT YOU'VE TRIED TO DO ABOUT IT. Believe me when I tell you that you won't be in shape to answer their 9 million questions and they MUST know your history to treat you appropriately. Take with you ALL of your MG meds and whatever you need to swallow them (icing, yogurt, pudding) because you won't want to wait for the hospital pharmacy to order it - this can take several hours. HOWEVER, do not take any meds until you are seen by the doctor once you have reached the ER in case it's the medication level itself that turns out to be the problem. If the ER triage nurse does not understand MG or MG problems, you will have to make sure the person accompanying you makes sure the nurse READSthe paper! Likewise, your accompanying advocate will need to watch the clock, and YOU, to make sure you're not getting worse while you wait to be seen.

Finally, if you can possibly remember to do this - DO NOT PANIC - and try to make sure your appointed advocate is not panic-prone. Panic will only make your symptoms worse and cloud the underlying MG issue needing attention. Likewise, it will make you seem like you are over-reacting to your "invisible" problem and even medically trained personnel might miss the reason you have presented yourself to the ER.

The treatment of MG is similar to all other autoimmune diseases, namely, immune function must be suppressed. Over the years many immunosuppressive drugs and treatments have been developed to treat autoimmune diseases such as MG. One such treatment, intravenous immune globulin (1VIG) is the focus of recent interest for an expanding role in the management of MG.

IVIG is pooled human immunoglobulin type G (IgG) collected from blood donors. The IgG is purified from the plasma component of the blood, and it is estimated that tens of thousands of individual donors are represented in one batch of IVIG; therefore, the product contains a large variety of IgG, which is important to its function.

Immunoglobulins are the antibodies of the immune system. They are proteins that have one end that is highly variable between different antibodies so that they have varying affinity for different substances, and the other end that interacts with other components of the immune system. Antibodies with an affinity for a particular protein on an invading organism, such as a virus,

will bind to the protein with their variable end, while the other end activates the cellular components of the immune system, thereby targeting and destroying the invader. It also stimulates immune cells to produce more antibodies, thereby increasing the immune response against the invader.

IVIG has two primary medical uses, quite opposite in effect. Some individuals have an immunodeficiency syndrome where they cannot produce their own antibodies in sufficient amounts to maintain an immune defense against infection. In these patients IVIG is given to supplement their immune systems and increase their defense against infection. In some autoimmune diseases, such as MG, one particular type of antibody is produced in large

numbers, attacking the patient's own healthy tissue. In these cases IVIG can be given to suppress the immune system.

1. The infused antibodies will, for a time, dilute the abnormal host antibodies that are causing the diseases.

2. The presence of large amounts of IgG will also suppress the production of host IgG (including, it is hoped, the disease causing antibodies).

3. The donated antibodies will also bind to the other components of the immune system, thereby using up resources, resulting in fewer immune resources to do damage.

4. The infused antibodies may bind to host antibodies, including those causing disease, and take them out of action.5. IVIG also increases the breakdown of host immunoglobulin and decreases its production.

1. As a rapidly acting but relatively short lasting treatment for a disease that is itself relatively short lasting or is currently flaring (eg: Guillain Barre Syndrome).

2. The second is for chronic autoimmune disease, such as MG. In this case 1VIG is given approximately once every three months over time for long term immunosuppression.

Side effects of IVIG usually are minimal, and may include headaches, local skin reaction at the infusion site and flu like symptoms. Less commonly patients may develop aseptic meningitis, although without long term consequence. Rare, but sever side effects, include thromboembolic events, such as pulmonary embolism, caused by increased blood viscosity.

Historically IVIG has been used in MG as an acute therapy for MG crisis. In this way it primarily has been an alternate therapy to plasmapheresis, which involves some hazards not found in IVIG. It also has been shown to be effective in treating patients prior to a thymectomy in order to improve their ability to handle the anesthesia and surgery.

Another use of IVIG is for severe refractory MG patients who have not been controlled with standard treatment. In these cases IVIG can be given over a short course to bring the patient under control, but traditional long term medications are still used to maintain control.

Recently there had been interest in using IVIG to chronically treat MG. The potential advantage is not that IVIG is likely to be more effective than current treatments, but the IVIG is likely to be

as, but not more, effective as plasmapheresis. The standard combination therapy of prednisone for short term treatment (usually around one year of treatment, tapering either to a very

low dose or completely off) with long term immunosuppressive therapy of azathioprine (Imuran), cyclosporine, or Cellcept has proven to be very effective in controlling MG in most cases, and it is unlikely the IVIG will prove to be more effective than these established therapies.

What is driving the interest in IVIG for chronic therapy is not improved efficacy but decreased side effects. Other treatments such as plasmapheresis and prednisone may have severe side effects. Infection and blood clots at the site of the plasmapheresis catheter would be avoided (IVIG also requires venous access, but the bore is much smaller and less prone to problems), and prednisone side effects, such as hiding many symptoms of infection, causing temporary diabetes, predisposing the patient to gastric ulcers, weight gain, osteoporosis, aseptic necrosis of the head of the femur (essentially, severe arthritis of the hips), and steroid induced myopathy (muscle damage) would not occur.

A major stumbling block for IVIG is cost. While treatment with prednisone, for example is very cheap - less than $50 annually - IVIG is very expensive, costing tens of thousands of dollars per year. IVIG payoff, however, is that after the treatment cost, there are no other costs. Prednisone has a host of hidden potential medical costs: medication to prevent gastric ulcers and osteoporosis, monitoring and treating ensuing diabetes, and hospitalization for treatment of opportunistic infections.

Plans for pilot study on using IVIG to reduce or eliminate the need for prednisone are underway. If the study shows that IVIG does this, it could lead to a significant shift in the standard management of MG, with more reliance upon intermittent

Conversations with your children about how myasthenia affects you are very important. Parents sometimes feel that they want to protect their children from upsetting information about family illness. Parents do this out of love and concern for their children, but it is far better to be open and honest about your illness.

Like adults, children can sense when something's wrong. They notice the way myasthenia physically affects you and they can pick up on the emotional distress that you feel about your illness. Without concrete information, children may begin to imagine that your illness is far worse than it actually is. Also, young children may believe that they have done something to cause you to become ill. Talking to your kids about myasthenia and their feelings will help to clear up misconceptions and provide much-needed reassurance.

Before you can explain myasthenia to your children, you must first educate yourself! Start your education by making the most of your medical visits.

• Ask your health care providers for information about myasthenia. Ask questions about things that you don't understand.

• Get information from MGFA and your local chapter. Go online to www.myasthenia.org to print out an array of useful patient brochures. (All of the medical information on the MGFA Web site has been reviewed and approved by our Medical/ Scientific and Nurses Advisory Boards.)

• Remember: each person with myasthenia is affected uniquely. Another's experience with the disease and its various therapies will be different than yours. If you have questions, always seek the advice of your physicians or other qualified health professionals before making any adjustments to your medical therapy.

• First, consider your child's developmental stage and level of understanding. What you will say to a five-year-old will be far different than what you will need to say to a teenager!

• Talk to your health care provider, counselor or clergy about the best way to approach the topic with your individual children.

• Discuss the issue in a local MG chapter support group and leam from the experiences of others. Talk to your children's teachers to provide information about your illness and ask for their support. Communicating with your children's teachers will also help you identify early any behavior changes in your children that may be a reaction to your illness. When you talk to your children, be prepared to offer lots of reassurance.

• Anticipate the questions that your children may ask and be prepared to answer them honestly with information that they can understand based on their age and maturity. Anticipated questions may include: - What is myasthenia gravis (MG)? - Can I catch it? - How will your illness affect me and my activities? - What made you sick? - Will you die from myasthenia gravis? -Will you have to go to the hospital? If so, who will take care of me? - What will happen to you?

• Remember that children may have difficulty expressing their worries. You should be prepared to possibly lead the conversation by asking them if they are worried about some of the above issues.

• If you don't know the answer to one of their questions, say so. It's okay to share your own frustrations with having myasthenia gravis. This is a normal reaction, which children should understand.

• For your own personal support, ask your spouse, another adult family member, or other trusted person to join you in the conversation with your children.

Choosing the time to tell your children about your diagnosis of myasthenia gravis is best determined by you, because you know them best. However, it is generally better to talk to them soon after diagnosis because your kids are noticing your physical symptoms, too. If you don't talk to them about myasthenia, they'll resort to their fears and imagination to explain what they're observing about your symptoms. They may believe that your symptoms are punishment for something they did. If your child blames him- or herself for making you ill, encourage him or her to express his fears and provide reassurance. Tell him or her directly that he or she is not responsible for your illness.

Explain to your children that the symptoms of myasthenia are variable, fluctuating, and somewhat unpredictable. (This is hard even for adults to understand.) For example, your children may wonder why your right eyelid is droopy one day and your left eyelid is droopy another day. They may wonder why they can understand your speech at the beginning of reading their favorite story but not at the end. They may wonder why you look sad and can't smile anymore. There are no easy answers to explain this without going through difficult-to-understand pathophysiology. For children, you should just explain that "this is the way myasthenia is." You can reassure them that adjustments to your treatments may help to control the symptoms.

Finally, reassure your children that you will do everything possible to get and stay healthy.

• Explain to them that you have skilled doctors and nurses who are helping you.

• Reassure your children that the current treatments for myasthenia make most people significantly better.

Since the variability of myasthenia symptoms can affect the family, it's important to continue talking to your children about MG. A good forum for doing this is through your participation in MGFA chapter activities, which will better educate you and your family about your illness. It will also teach your kids that there's a lot of value in providing service to others.

To preserve their power, medications should be stored in a cool, dry place. That rules out the medicine cabinet; bathrooms are among the moistest rooms in a house. A kitchen cabinet is better, but your best bet is a dresser drawer. Just use the childproof cap if the grandkids are around: According to the U.S. Consumer Product Safety Commission, one-third of the accidental prescription drug poisonings in children involve a grandparent’s pills.

Savvy consumers dispose of outdated medications by flushing them down the toilet.

It’s a good idea to get rid of old medicines because prescription drugs are chemical compounds that break down over time. But flushing them is not the best option. Researchers are finding traces of prescription drugs in some public water supplies. The amounts are tiny, but the consequences could be huge. A better option: Take your old pills back to the pharmacy - most pharmacies accept the return of outdated drugs.

Prescription drugs are tested on people of all ages before they receive FDA approval.

Few research trials include older people. As a result, doctors often have little information on how older people may respond to a particular medication. It’s a critical issue because older people metabolize medications differently from healthy young adults. When prescribing medication to an older patient, my philosophy is “start low and go slow.” If you start taking a new medicine, ask to begin at a reduced dose and make changes gradually. Doing so can protect you from unpleasant and dangerous side effects.

After a medication is prescribed for a serious illness, it’s dangerous to ever decrease the dosage or stop taking it.

When I speak to doctors, I often ask if anyone remembers attending a med school lecture on the art of tapering medications. Typically no one raises a hand. Modern medical education pays very little attention to how to safely discontinue drugs. This is a tragic oversight, because continuing medications past their effectiveness is expensive and dangerous. Ask your physician, “Are the medications I am taking still necessary?” Help your doctor provide good care by letting him or her know you understand that more is not always better.

It is with great sadness that I inform you of the death of Dianne’s mom, Dot George. Dot passed away unexpectedly on August 28^th. Dot was Dianne’s biggest supporter since she was diagnosed with Myasthenia Gravis in 1986. Until the last two meetings Dot never missed a meeting. Everyone that knew Dot loved her and will miss her terribly.