Treatments

Myasthenia Gravis There is no cure for myasthenia gravis, but treatments are available to help control the symptoms.
In many cases, treatment for myasthenia gravis can significantly improve muscle weakness and a person with the condition is able to lead a relatively normal life.
If your symptoms are mild, you may find that getting plenty of rest helps improve your symptoms without the need for additional treatment.

Pyridostigmine (Mestinon)

What is pyridostigmine?

Myasthenia GravisPyridostigmine is an anticholinesterase agent used for the symptomatic treatment of myasthenia gravis (MG). In the United States, pyridostigmine is produced in a 60 milligram tablet, a 60 milligram/5 milliliter raspberryflavored syrup, and a 180 milligram sustained release (long-acting) tan-colored tablet under the trade name Mestinon. Regular-release pyridostigmine 60 milligram tablets are available in generic formulation also. Pyridostigmine injection 5 milligrams/milliliter is available under the trade name Regonol Solution for Injection.

How is pyridostigmine used in the treatment of myasthenia gravis?

Pyridostigmine prevents the breakdown of acetylcholine in the neuromuscular junction. Acetylcholine travels across the space to the muscle-fiber side of the neuromuscular junction, where it attaches to many receptor sites. The muscle contracts when enough of the receptor sites have been activated by the acetylcholine. Pyridostigmine allows more acetylcholine to remain at the neuromuscular junction longer than usual so that more receptor sites can be activated. More acetylcholine in the neuromuscular junction results in stronger muscle contractions and less MG weakness. Pyridostigmine does not cure myasthenia gravis but does help to improve the symptoms.

What are some important things to consider when taking pyridostigmine?

It is important to take pyridostigmine on time and exactly as it has been prescribed. If one dose is missed within an hour of the prescribed dose, the patient should take the missed dose and continue with the other doses as scheduled. If the dose is missed by more than one hour, the patient should take the dose immediately and then wait the required 3 to 4 hours before taking the next dose. Subsequent doses should be taken with the prescribed intervals as well. For example, if a dose of pyridostigmine missed at noon is taken at 2 p.m., the next dose would be taken at 5 or 6 p.m. or as directed by the neuro surgeon. Never take a double dose to make up for a missed one.

What safety measures should I follow when taking pyridostigmine?

  • Watch for possible adverse reactions and reportthem to your MG neuro surgeon.
  • Carefully follow the prescribed dose regimen. To help your neuro surgeon individualize the amount of medication you need, record the response after each dose for the initial period of taking pyridostigmine or in times of instability of the MG weakness.
  • Recognize that choosing an optimal dose of pyridostigmine for you can be difficult because the symptoms of overdose and underdose can be somewhat similar.
  • Seek immediate medical attention should breathing and swallowing become difficult.
  • Refill prescriptions early, in case there is difficulty in obtaining a supply of pyridostigmine.
  • Store pyridostigmine in a dry place, never in a moist climate like the bathroom or refrigerator.
  • Carry medications on your person when traveling, not in your luggage.

What are some possible adverse reactions of pyridostigmine?

  • Gastrointestinal upset, nausea, vomiting
  • Abdominal cramps and diarrhea
  • Increased salivation; drooling, and tearing
  • Increased bronchial secretions
  • Increased sweating
  • Muscle cramps
  • Muscle fasciculations (twitching)
  • Muscle weakness
  • Headache

All adverse reactions should be reported to your MG-treating neuro surgeon. Many of the adverse effects can be relieved by a change in the dose of pyridostigmine. If changing the dose does not solve the problem, the neuro surgeon may add another medication to help control the adverse reactions.

Are all pyridostigmine or Mestinon preparations equal?

  • Regular pyridostigmine or Mestinon comes in two forms: Mestinon 60 mg. tablets and Mestinon Syrup 60 mg./5 ml. The greatest effect usually occurs in 60 to 90 minutes and lasts for 3 to 4 hours.
  • Mestinon Timespan is an extended release form of pyridostigmine bromide that slowly releases its active ingredients over an approximately 12-hour period. When prescribed, Mestinon Timespan is usually given as a bedtime dose so that the patient does not need to wake up every 3 or 4 hours to take a dose of regular Mestinon.
  • Mestinon Timespan should never be substituted for regular Mestinon or generic pyridostigmine.
  • Mestinon Timespan should never be crushed.
  • The possible adverse effects of Mestinon Timespan are the same as those for regular Mestinon or generic pyridostigmine, but are possibly more likely to occur.
  • The absorption and effect of Mestinon Timespan are sometimes erratic. Some neuro surgeons and patients prefer to schedule nighttime doses of regular Mestinon or generic pyridostigmine.
  • Regonol Solution for Injection 5 mg./ml. is sometimes required when patients cannot take anything by mouth. The neuro surgeon will prescribe 1/30th of the usual oral dose by IM injection or very slow IV administration.
  • No single fixed dose schedule will suit all patients with MG, whose medication requirements vary from time to time, day to day, and in response to stress or infection.
  • Different muscles respond differently to a given dose of Mestinon or generic pyridostigmine. The neuro surgeon will select a dose that produces the best response in the most vitally affected muscles.

Prednisone

What is Prednisone?

Prednisone is a synthetic hormone commonly referred to as a “steroid.” Prednisone is very similar to the hormone cortisone, which your own body manufactures. Prednisone is used to treat many illnesses.

How does prednisone work?

In part, prednisone acts as an immunosuppressant. The immune system protects you against foreign bacteria and viruses. In some illnesses, the immune system produces antibodies that become overactive and cause undesirable effects. These illnesses are referred to as “autoimmune diseases.” Prednisone suppresses the production of antibodies. This suppression can make it slightly harder for you to fight off infection, but also stabilizes the immune system if it is overactive. Prednisone also works against inflammation to reduce heat, redness, swelling, and pain. When beginning prednisone, there is a small chance that it may cause serious increased weakness for a short period of time. Therefore, it may be necessary for you to be hospitalized for the first few days for observation.

What are the possible adverse effects of prednisone?

Adverse side effects do not occur in all patients and are usually related to the amount and length of time prednisone is used. Potential adverse effects will be monitored by your neuro surgeon and include:
  • Insomnia and mood changes.
  • Euphoria or depression may occur. The cause is uncertain. It is best to take prednisone in the morning to reduce the chances of insomnia at night.
  • Increased appetite and weight gain.
  • Prednisone increases your appetite. Follow a diet to maintain your weight. Develop an eating schedule and stick to it. If you eat when you feel hungry, you will be eating all the time.
  • Susceptibility to infections.
  • Prednisone slightly decreases your resistance to infection. Avoid individuals with infectious illnesses if possible. Notify your neuro surgeon if you develop persistent signs of an infection.
  • Stomach upset (indigestion, stomach burning or ulcer).
  • Prednisone may cause gastrointestinal irritation. Take prednisone with meals, milk or antacids. Do not take on an empty stomach.
  • Fluid retention.
  • Prednisone can cause you to retain fluid. Your neuro surgeon will monitor this process. Fluid retention can be caused by sodium retention and potassium depletion through frequent urination. A salt-restricted/potassiumrich diet may help reduce fluid retention.
  • Hypertension.
  • Prednisone may cause a rise in blood pressure. Your neuro surgeon can treat this, if necessary.
  • Skin changes.
  • Prednisone can cause a change in the condition of your skin. You may notice that your skin bruises more easily, or that wounds take longer to heal.
  • Change of physical appearance.
  • These changes may include swelling of the face or the back of the neck or ankles; acne; thinning of skin; or skin stretch-lines.
  • Osteoporosis.
  • Prednisone can make your bones become fragile by increasing calcium loss. This usually occurs after taking prednisone for a prolonged time. It may be recommended that you take a calcium and vitamin D supplement or increase the amount of calcium-rich foods in your diet.
  • Cataracts and worsening of glaucoma.
  • After prolonged use of prednisone, cataracts or glaucoma may develop. This condition can be monitored by periodic eye examinations.
  • Alterations in hair growth.
  • Prednisone can cause a darkening and/or increase in hair growth. This disappears when the dose of prednisone is decreased.
  • Hyperglycemia or diabetes (elevated blood sugar).
  • Prednisone may increase the amount of sugar (glucose) in your blood. With periodic blood work, your neuro surgeon can monitor this. If prednisone is stopped too quickly, serious adverse effects can occur (nausea, vomiting, pain, fever, and/or flare-up of disease). Rapid withdrawal or stopping use of prednisone may cause these adverse effects. Never stop or change your prednisone dose without your doctor’s consent. If you are planning a trip, always carry an extra supply.

How long will the side effects last?

If adverse effects develop, they will usually persist as long as the medication is continued. As the dose decreases, so will the adverse effects. Some adverse effects are permanent and will not reverse after the medication is discontinued.

Are there any special dietary considerations?

Patients taking prednisone should be on high protein/ low salt/low carbohydrate diets and eat well-balanced meals.

What should I do if I miss a dose of prednisone?

You should try to avoid missing a dose of prednisone. If you do, follow these guidelines:

  • If you forget to take your prednisone at the usual time but remember later on the same day, take the missed dose immediately.
  • If you forgot to take yesterday’s dose, skip yesterday’s dose and take your usual dose for the day.
  • If you are on an alternate day schedule and forgot yesterday’s dose, take yesterday’s dose today. Tomorrow, resume alternate day schedule.

How are the dosages of prednisone determined?

Schedules are determined by the body’s response to the prednisone. Severity of myasthenia gravis, control of myasthenia gravis symptoms, and the development of adverse effects are all taken into consideration when your neuro surgeon determines your dosage of prednisone.
What is meant by the terms “Alternate Day,” “High Day,” “Low Day” and “Off Day”?
These are terms used to describe typical dosage patterns of prednisone.

  • Alternate Day is when you take prednisone every other day.
  • High Day and Low Day refer to the dosages you take on the alternate day schedule. High Day is the day when you take the higher dose of prednisone. Low Day is the day when you take the lower dose of prednisone.
  • Off Day describes a day when your prednisone dose is 0 mg (when you don’t take any prednisone).

Is it important for others to know that I am taking prednisone?

Yes. Any doctor or dentist who is taking care of you should know you are taking prednisone. In case of an emergency, your family or close friends should also know you are taking prednisone. You should carry an identification card stating that you are taking prednisone and any other medications, and including your doctor’s name and phone number. This is valuable information should an emergency occur.

Azathioprine

What is azathioprine?

Azathioprine is an immunosuppressive medication that decreases the actions of the body’s immune system. Drugs that suppress the immune system are used in patients with myasthenia gravis (MG) because MG is an autoimmune disorder that results from the production of abnormal antibodies. Azathioprine is available in a generic formulation or as the brand name, Imuran

How does azathioprine work?

Under normal circumstances, the immune system produces antibodies that protect the body against infection from invading bacteria and viruses. In autoimmune MG, the immune system produces abnormal acetylcholine receptor (AChR) antibodies. These AChR antibodies destroy or block certain receptor sites needed for neuromuscular transmission and strong movement of muscle groups. The result is the fluctuating and fatigable muscle weakness of MG. Azathioprine suppresses the immune system and reduces the production of AChR antibodies. This allows the receptors to regenerate and function more normally in neuromuscular transmission and results in a return of muscle strength. After a period of approximately 3 to 12 months, the
MG patient should notice a gradual improvement in muscle strength and a decrease in the severity of symptoms if azathioprine is working. This improvement may decrease the need for other MG treatments.

What are some special considerations when taking azathioprine?

Since azathioprine is a strong medicine, the neuro surgeon and patient must consider its risks and benefits. The neuro surgeon will want to perform a physical examination and gather a complete medical history and learn about any chronic or serious medical conditions and any medications that the patient has been taking, especially allopurinol (Zyloprim), ACE inhibitors such as Lotensin, Zestril or Altace, and the blood thinner Coumadin. Other medications may interact with azathioprine and the patient should always discuss any prescription or over the counter drugs used with the neuro surgeon.
Before taking azathioprine, the patient should tell the neuro surgeon if he/she has had an unusual or allergic reaction to this medicine previously. The neuro surgeon will want to know if the patient has any disease of the liver or pancreas or has a bacterial, viral or fungal infection. Azathioprine may cause some bone marrow suppression and elevation of liver enzymes. Therefore, the neuro surgeon will check blood tests regularly to monitor for significant changes. The neuro surgeon is prepared to discuss other possible, infrequent or theoretical complications, such as certain types of malignancies.
Myasthenia Gravis Female or male patients planning a pregnancy should discuss this with the MG treating neuro surgeon. Unless the neuro surgeon and patient determine that the benefits outweigh the risks, a woman should How does azathioprine work?
Under normal circumstances, the immune system produces antibodies that protect the body against infection from invading bacteria and viruses. In autoimmune MG, the immune system produces abnormal acetylcholine receptor (AChR) antibodies. These AChR antibodies destroy or block certain receptor sites needed for neuromuscular transmission and strong movement of muscle groups. The result is the fluctuating and fatigable muscle weakness of MG. Azathioprine suppresses the immune system and reduces the production of AChR antibodies. This allows the receptors to regenerate and function more normally in neuromuscular transmission and results in a return of muscle strength.
After a period of approximately 3 to 12 months, the MG patient should notice a gradual improvement in muscle strength and a decrease in the severity of symptoms if azathioprine is working. This improvement may decrease the need for other MG treatments.

What are some special considerations when taking azathioprine?

Since azathioprine is a strong medicine, the neuro surgeon and patient must consider its risks and benefits. The neuro surgeon will want to perform a physical examination and gather a complete medical history and learn about any chronic or serious medical conditions and any medications that the patient has been taking, especially allopurinol (Zyloprim), ACE inhibitors such as Lotensin, Zestril or Altace, and the blood thinner Coumadin. Other medications may interact with azathioprine and the patient should always discuss any prescription or over the counter drugs used with the neuro surgeon.
Before taking azathioprine, the patient should tell the neuro surgeon if he/she has had an unusual or allergic reaction to this medicine previously. The neuro surgeon will want to know if the patient has any disease of the liver or pancreas or has a bacterial, viral or fungal infection. Azathioprine may cause some bone marrow suppression and elevation of liver enzymes. Therefore, the neuro surgeon will check blood tests regularly to monitor for significant changes. The neuro surgeon is prepared to discuss other possible, infrequent or theoretical complications, such as certain types of malignancies.
Female or male patients planning a pregnancy should discuss this with the MG treating neuro surgeon. Unless the neuro surgeon and patient determine that the benefits outweigh the risks, a woman should not take azathioprine while pregnant or planning to become pregnant. Breastfeeding should be avoided if taking azathioprine.
How should azathioprine be taken? It is important to take azathioprine exactly as directed by the neuro surgeon. Never increase, decrease or stop taking azathioprine without checking with the hysician. Patients with MG may have to stay on this medication indefinitely because it is a long-term treatment. The main clinical improvement occurs during the first year of therapy. Improvement is gradual and it may take several months before a patient begins to notice a change in symptoms.
The dosage of azathioprine varies from 100 mg. to 200 mg. per day. If a patient misses a dose while on a once daily schedule, he/she should skip the dose that was missed and return to the regular schedule with the next dose. Do not take a double dose. If a patient misses a dose while on a several times a day dosing schedule, he/she should take the missed dose as soon as it is remembered. If it is time for the next dose, take both doses together, then resume the normal schedule. If more than one dose is missed, the patient should check with the prescribing neuro surgeon.
To prevent or lessen stomach upset, the patient should eat small meals frequently throughout the day, eat dried foods such as toast or crackers, and avoid fried or fatty foods. Store azathioprine at room temperature away from heat, direct light or moisture. While taking azathioprine, the patient should avoid immunizations without the neuro surgeon’s approval and take special precautions to avoid developing an infection. If a patient does become ill with fever, chills or infection, he/she should contact the neuro surgeon immediately. The patient should tell the neuro surgeon or dentist that they are taking azathioprine prior to having surgery.

What are the possible adverse effects of taking azathioprine?

Azathioprine is generally tolerated very well without serious adverse effects. Some adverse effects are very serious and need prompt attention; others may go away as your body adjusts to the medication. The patient should stop taking azathioprine and contact the neuro surgeon immediately if any of the following occurs: hives; swelling of face, lips, or tongue; difficulty breathing. The patient should also contact the neuro surgeon immediately if he/she has severe nausea and vomiting, diarrhea, fever or chills, loss of appetite, abdominal pain, skin rash (especially chicken pox or shingles), cough, cold sores in the mouth or on the lips, sore throat, blood in the urine or stool, unusual bruising, pale stools or darkened urine, yellowing of skin and eyes, darkening of the skin and fingernails, muscle or joint pain, hair loss, fatigue or a missed menstrual period.

Mycophenolate Mofetil (CellCept)

What is mycophenolate mofetil (CellCept)?

Mycophenolate mofetil is a relatively new immunosuppressive drug approved for use in certain organ transplant patients to prevent organ rejection. It has not yet been proven to be effective for myasthenia gravis (MG). Studies to determine the safety and effectiveness of mycophenolate mofetil in MG are necessary. Researchers are actively studying this promising new therapy in certain patients with MG by conducting clinical trials. Clinical trials are the safest and quickest way to find new treatments that work.

How does mycophenolate mofetil work?

People with MG may benefit from therapy with mycophenolate mofetil when other immunosuppressive treatments have been ineffective or contraindicated. Mycophenolate mofetil suppresses the immune system and reduces the production of antibodies attacking the receptor sites of the neuromuscular junction.

What are some special considerations to discuss with your health care provider before starting mycophenolate mofetil?

Before prescribing mycophenolate mofetil, your MG neuro surgeon will ask you if you have anemia or any blood conditions, unusual bleeding or bruising, or any viral or bacterial infections. If you are a woman, your neuro surgeon will want to know if you are pregnant, planning on getting pregnant, or breast feeding. In laboratory studies, mycophenolate mofetil has been reported to cause birth defects in animals. Although the effects are not known in humans, women of childbearing age should avoid becoming pregnant while taking the drug. Women who are planning a pregnancy or who become pregnant while taking mycophenolate mofetil should discuss potential risks and options with their neuro surgeon.

How should mycophenolate mofetilbe taken?

Mycophenolate mofetil is an oral medication that should be taken exactly as prescribed by your MG neuro surgeon. Swallow the tablet or capsule with water. Do not crush the tablet or open the capsule. If your skin comes in contact with the contents of the capsule or a broken tablet, rinse thoroughly with water. Be certain to take this medication exactly as prescribed at regular intervals. If you miss a dose of mycophenolate mofetil, take it as soon as you can. If it is almost time for the next dose, take only that dose. Never take extra medicine or double doses.

Does mycophenolate mofetil interact with other medicines?

Mycophenolate mofetil can interact with other drugs. For this reason, it is important to tell your neuro surgeon about all other medicines that you are taking. Be certain to mention all over-the-counter drugs, nutritional supplements or any herbal products that you are using. Antacids, vaccines, cholestyramine (a cholesterol reducing drug) and drugs that suppress your immune system can interact with mycophenolate mofetil.

What are the possible adverse effects of mycophenolate mofetil?

Your neuro surgeon will carefully monitor your situation for potential adverse effects. People with MG take much smaller doses of mycophenolate mofetil than those using it to avoid transplant rejection. For this reason, common adverse effects are limited and mainly related to gastrointestinal problems including nausea and diarrhea, low white blood counts, anemia and skin rash. Mycophenolate mofetil slightly reduces resistance to infection. It is important to avoid individuals with infectious illnesses and to notify your neuro surgeon if you develop persistent signs of infection.

How will a neuro surgeon monitor a patient taking mycophenolate mofetil?

Appointments will be scheduled at regular intervals to monitor patient progress. At these appointments, your neuro surgeon will ask you a series of questions as well as perform physical examinations and laboratory tests that provide important information to evaluate the safety and effectiveness of mycophenolate mofetil in MG. To watch for anemia or a decrease in the white cell count, blood samples will be examined frequently during the first few months of this therapy and then less often.

Plasmapheresis

What is plasmapheresis?

Plasmapheresis is a procedure in which blood is separated into cells and plasma (liquid). The plasma is removed and replaced with fresh frozen plasma, a blood product called albumin and/or a plasma substitute. The procedure is often referred to as plasma exchange.

Why should I have plasmapheresis?

Plasmapheresis may be recommended for a few reasons:
  • To stabilize a rapid decrease in muscle strength.
  • To reduce moderate to severe muscle weakness before surgery.
  • To add to present treatment if current forms of therapy are providing insufficient control of the disease.

How many plasma exchanges will I need and where will it be done?

The number of plasmapheresis treatments needed depends on the protocol the neuro surgeon has determined is best for the patient. Some patients are treated on a Monday/Wednesday/Friday schedule. Others are treated on a daily schedule or on a weekly or monthly schedule. Hospitalization may be necessary for treatment. Sometimes plasmapheresis can be done on an outpatient basis.

What should I expect to happen?

A needle will be placed in each arm. In some instances, only one arm may be used. If the arm veins are too small to use, the neuro surgeon may place a special long duration indwelling catheter into a large vein in the shoulder or groin. Unless the neuro surgeon has instructed otherwise, it is important to eat before the plasma exchange and not skip any meals. During plasma exchange, the patient may drink fluids. The patient should empty the bladder prior to the procedure and ask the nurse for a bedpan or urinal if needed during the plasma exchange procedure. The patient will need to keep both arms still unless the catheter is placed into a different spot. Wearing comfortable clothing with loose fitting sleeves that pull easily above the elbows will make it easier to place the needles in each arm. Bringing something to read or to do will help pass the time (e.g., a radio or tape/CD player with headphones). If the procedure is being performed with an indwelling catheter, it may be necessary to wear a hospital gown.

How long does plasma exchange take?

The time spent on the machine may be one to three hours. This depends upon the patient’s weight, height and the amount of plasma to be exchanged.

How soon will the treatment work?

Plasmapheresis works quickly to increase strength. Most patients begin to improve within the first few days of the treatment.

How long will the improvement last?

A typical patient who has received three to five exchanges over a week or two would commonly remain stronger for one to two months. After a month or two, the acetylcholine receptor antibodies usually return to pre-treatment levels in the bloodstream.

What are the possible adverse effects?

Common adverse effects may include a drop in blood pressure, feelings of faintness, dizziness, blurred vision, coldness, sweating or abdominal cramps. Rare adverse effects may include bleeding as a result of medications used to keep the blood from clotting, tingling associated with the mouth, eyes, fingers or toes and a possible allergic reaction to the solutions, which may result in itching, wheezing or rash.

How do I reduce my fears about the plasmapheresis procedure?

Often, anxiety about plasmapheresis—especially during the first few treatments—can produce some unusual sensations that will go away once the patient becomes more comfortable with the procedure. Helpful ways to alleviate fears may include speaking to someone who has been treated with plasmapheresis, discussing concerns with the neuro surgeon.

Immunoglobulin therapy (IVIG AKA PolyGam)

What is IVIg?

IVIg is intravenous immune globulin. It is also known as pooled human gamma globulin or simply gamma globulin. IVIg has been used for decades in modern medicine in the treatment of a variety of infectious or inflammatory diseases. In patients who are lacking in the antibodies necessary to fight infection, IVIg replaces those lost antibodies. IVIg is used also in the treatment of a variety of autoimmune disorders. Over the past two decades, IVIg has been used extensively in the treatment of some autoimmune neurological disorders, including myasthenia gravis. There are indications that IVIg is an effective treatment for some patients with autoimmune myasthenia gravis.

Why am I being treated with IVIG?

IVIg might be prescribed for an individual with MG for one of several reasons. The first indication for use might be for a hospitalized patient who is extremely ill and might not be responding adequately to other treatments. Some patients with MG who are being treated with IVIg have improved quickly, thus allowing time for other treatments to begin working. Patients at home who are having significant symptoms in spite of aggressive treatment with other drugs and treatments may be considered for treatment with IVIg also.

What are some important things to consider when taking pyridostigmine?

It is important to take pyridostigmine on time and exactly as it has been prescribed. If one dose is missed within an hour of the prescribed dose, the patient should take the missed dose and continue with the other doses as scheduled. If the dose is missed by more than one hour, the patient should take the dose immediately and then wait the required 3 to 4 hours before taking the next dose. Subsequent doses should be taken with the prescribed intervals as well. For example, if a dose of pyridostigmine missed at noon is taken at 2 p.m., the next dose would be taken at 5 or 6 p.m. or as directed by the neuro surgeon. Never take a double dose to make up for a missed one.

How does IVIg work?

IVIg seems to effect the function or the production of antibodies in the immune system. The exact mechanism of how IVIg works in successfully treating myasthenia gravis and other autoimmune disorders is not entirely understood.
Since IVIg is a blood product, is it safe? IVIg is felt to be very safe with regard to exposure to infection or viruses. Donors are screened. The processing of IVIg inactivates such infections as HIV and Hepatitis B and C. Nonetheless, it is a human blood product that comes from multiple donors.
Are there adverse effects that occur with IVIg treatments? For the most part, IVIg is considered quite safe. However, there are several adverse effects that may occur on occasion. Some patients develop headache, chills and aches during the infusion. Slowing the infusion and using medicines to relieve these symptoms may be helpful.
Some patients will experience fatigue, fever or nausea that may persist up to 24 hours after the infusion. Some patients may have migraine headaches, aseptic meningitis, skin reactions or a more severe allergic type reaction. Because IVIg is a highly concentrated protein, it can sometimes interfere with blood flow and clotting.

Myasthenia Gravis

How is IVIg administered?

IVIg treatments are administered intravenously. The medication is infused slowly over a number of hours. The dose is based on the individual’s weight. Many patients are treated with a series of infusions over a period of three to five days. Other patients may have infusion intervals even farther apart. Patients who are treated with long-term maintenance therapy have a single infusion every few weeks.
For some patients, the infusion is given in a neuro surgeon’s office, while others may have it in the home using a home health agency. Patients who are severely weakened are treated in the hospital.
A week or two may be required before the patient feels the onset of improvement, although this varies from patient to patient. The duration of improvement from IVIg is usually limited to a few weeks to a few months.

What are other concerns about using IVIg?

IVIg is expensive and this can be a major issue in management. It is important to discuss the cost issues with one’s health insurance provider in order to prevent an unforeseen financial dilemma.

Cyclophosphamide

What is Cyclophosphamide

Cyclophosphamide is used alone or in combination with other medications to treat Hodgkin's lymphoma (Hodgkin's disease) and non-Hodgkin's lymphoma (types of cancer that begin in a type of white blood cells that normally fights infection); cutaneous T-cell lymphoma (CTCL, a group of cancers of the immune system that first appear as skin rashes); multiple myeloma (a type of cancer of the bone marrow); and certain types of leukemia (cancer of the white blood cells), including chronic lymphocytic leukemia (CLL),

chronic myelogenous leukemia (CML), acute myeloid leukemia (AML, ANLL), and acute lymphoblastic leukemia (ALL). It is also used to treat retinoblastoma (cancer in the eye), neuroblastoma (a cancer that begins in nerve cells and occurs mainly in children), ovarian cancer (cancer that begins in the female reproductive organs where eggs are formed), and breast cancer. Cyclophosphamide is also used to treat nephrotic syndrome (a disease that is caused by damage to the kidneys) in children whose disease has not improved, has gotten worse, or has come back after taking other medications or in children who experienced intolerable side effects with other medications. Cyclophosphamide is in a class of medications called alkylating agents. When cyclophosphamide is used to treat cancer, it works by slowing or stopping the growth of cancer cells in your body. When cyclophosphamide is used to treat nephrotic syndrome, it works by suppressing your body's immune system.

How should this medicine be used?

Cyclophosphamide injection comes as a powder to be added to fluid and injected intravenously (into a vein) by a doctor or nurse in a medical office or hospital outpatient clinic. It may also be injected intramuscularly (into a muscle), intraperitoneally (into the abdominal cavity), or intrapleurally (into the chest cavity). The length of treatment depends on the types of drugs you are taking, how well your body responds to them, and the type of cancer or condition you have.
Your doctor may need to delay your treatment or adjust your dose if you experience certain side effects. It is important for you to tell your doctor how you are feeling during your treatment with cyclophosphamide injection.

Other uses for this medicine

Cyclophosphamide injection is also sometimes used to treat a certain type of lung cancer (small cell lung cancer; SCLC). It is also used to treat rhabdomyosarcoma (a type of cancer of the muscles) and Ewing's sarcoma (a type of bone cancer) in children and Myasthenia Gravis. Talk to your doctor about the risks of using this medication for your condition.
This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

What special precautions should I follow?

Before receiving cyclophosphamide injection,

  • tell your doctor and pharmacist if you are allergic to cyclophosphamide, other alkylating agents such as bendamustine (Treanda®), busulfan (Myerlan®), Busulfex®), carumustine (BiCNU®, Gliadel® Wafer), chlorambucil (Leukeran®), ifosfamide (Ifex®), lomustine (CeeNU®), melphalan (Alkeran®), procarbazine (Mutalane®), or temozolomide (Temodar®), any other medications, or any of the ingredients in cyclophosphamide injection. Ask your pharmacist for a list of the ingredients.
  • tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take. Be sure to mention any of the following: allopurinol (Zyloprim®), cortisone acetate, doxorubicin (Adriamycin®, Doxil®), hydrocortisone (Cortef®), or phenobarbital (Luminal® Sodium). Your doctor may need to change the doses of your medications or monitor you carefully for side effects. Many other medications may also interact with cyclophosphamide, so be sure to tell your doctor about all the medications you are taking, even those that do not appear on this list.
  • tell your doctor if you have previously received treatment with other chemotherapy medications or if you have had x-rays recently. Also tell your doctor if you have or have ever had kidney or liver disease.
  • you should know that cyclophosphamide may interfere with the normal menstrual cycle (period) in women and may stop sperm production in men. Cyclophosphamide may cause permanent infertility (difficulty becoming pregnant); however, you should not assume that you cannot get pregnant or that you cannot get someone else pregnant. Women who are pregnant or breast-feeding should tell their doctors before they begin receiving this drug. You should not plan to have children while receiving chemotherapy or for a while after treatments. (Talk to your doctor for further details.) Use a reliable method of birth control to prevent pregnancy. Cyclophosphamide may harm the fetus.
  • if you are having surgery, including dental surgery, tell the doctor or dentist that you are receiving cyclophosphamide injection.

What side effects can this medication cause?

  • nausea, Vomiting
  • Loss of appetite or weight
  • abdominal pain
  • diarrhea
  • hair loss
  • sores on the mouth or tongue
  • changes in skin color
  • changes in color or growth of finger or toe nails
  • sore throat, fever, chills, or other signs of infection
  • poor or slow wound healing
  • unusual bruising or bleeding
  • black, tarry stools
  • painful urination or red urine
  • rash
  • hives
  • itching
  • difficulty breathing or swallowing
  • shortness of breath
  • cough
  • swelling in the legs, ankles, or feet
  • chest pain
  • yellowing of the skin or eyes
Cyclophosphamide may increase the risk that you will develop other cancers. Talk to your doctor about the risks of receiving cyclophosphamide injection.
Cyclophosphamide may cause other side effects. Call your doctor if you have any unusual problems while receiving this medication.

Rituximab

What is Rituximab

Rituximab injection (Rituxan) and rituximab and hyaluronidase human injection (Rituxin Hycela) are used alone or with other medications to treat certain types of non-Hodgkin's lymphoma (NHL; a type of cancer that begins in a type of white blood cells that normally fights infection) and chronic lymphocytic leukemia (CLL; a type of cancer that begins in the white blood cells). Rituximab injection is also used with another medication to treat the symptoms of rheumatoid arthritis (RA; a condition in which the body attacks its own joints, causing pain, swelling, and loss of function) in people who have already been treated with a certain type of medication called a tumor necrosis factor (TNF) inhibitor. Rituximab injection is also used with other medications to treat granulomatosis with polyangiitis (Wegener's Granulomatosis) and microscopic polyangiitis which are conditions in which the body attacks its own veins and other blood vessels, which causes damage to organs, such as the heart and lungs. Rituximab is in a class of medications called monoclonal antibodies. It treats NHL and CLL by killing cancer cells. It treats rheumatoid arthritis, granulomatosis with polyangiitis, and microscopic polyangiitis by blocking the activity of the part of the immune system that may damage the joints, veins, and other blood vessels.

How should this medicine be used?

Rituximab injection comes as a solution (liquid) to be injected into a vein. Rituximab and hyaluronidase human injection comes as a solution (liquid) to be injected under the skin, in the stomach area. Rituximab is administered by a doctor or nurse in a medical office or infusion center. Your dosing schedule will depend on the condition that you have, the other medications you are using, and how well your body responds to treatment.
Rituximab injection must be given slowly into a vein. It may take several hours or longer to receive your first dose of rituximab injection, so you should plan to spend most of the day at the medical office or infusion center. You must receive your first dose as rituximab injection into a vein. After the first dose, you may receive rituximab injection more quickly or your doctor may want you to receive rituximab and hyaluronidase human injection under the skin, depending on how you respond to treatment.
You may experience symptoms such as fever, shaking chills, tiredness, headache, or nausea while you are receiving a dose of rituximab, especially the first dose. Tell your doctor or other healthcare provider if you experience these symptoms while you are receiving your medication. Your doctor may prescribe other medications to help prevent or relieve these symptoms. Your doctor will tell you to take these medications before you receive each dose of rituximab.
Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.

Other uses for this medicine

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

What special precautions should I follow?

Before using rituximab,

  • tell your doctor and pharmacist if you are allergic to rituximab, any other medications, or any of the ingredients in rituximab injection or rituximab and hyaluronidase human injection . Ask your pharmacist or check the Medication Guide for a list of the ingredients.
  • tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take. Be sure to mention the medication in the IMPORTANT WARNING section and either of the following: adalimumab (Humira); certolizumab (Cimzia); cisplatin (Platinol); etanercept (Enbrel); golimumab (Simponi); infliximab (Remicade); medications for high blood pressure; other medications for rheumatoid arthritis; and medications that suppress the immune system such as azathioprine (Imuran), cyclosporine (Neoral, Sandimmune), sirolimus (Rapamune), and tacrolimus (Prograf). Your doctor may need to change the doses of your medications or monitor you carefully for side effects.
  • tell your doctor if you have any of the conditions mentioned in the IMPORTANT WARNING section and if you have or have ever had hepatitis C or other viruses such as chicken pox, herpes (a virus that may cause cold sores or outbreaks of blisters in the genital area), shingles, West Nile virus (a virus that is spread through mosquito bites and may cause serious symptoms), parvovirus B19 (fifth disease; a common virus in children that usually only causes serious problems in some adults), or cytomegalovirus (a common virus that usually only causes serious symptoms in people who have weakened immune systems or who are infected at birth), or kidney problems. Also tell your doctor if you have any type of infection now or if you have or have ever had an infection that would not go away or an infection that comes and goes.
  • tell your doctor if you are pregnant or if you plan to become pregnant. Rituximab may harm the fetus. You should use birth control to prevent pregnancy during your treatment with rituximab and for up to 12 months after your treatment. Talk to your doctor about types of birth control that will work for you. If you become pregnant while using rituximab, call your doctor.
  • tell your doctor if you are breastfeeding. You should not breastfeed during your treatment with rituximab and hyaluronidase human injection and for 6 month after your final dose.
  • ask your doctor whether you should receive any vaccinations before you begin your treatment with rituximab. Do not have any vaccinations during your treatment without talking to your doctor.

What side effects can this medication cause?

  • diarrhea
  • constipation
  • back or joint pain
  • flushing
  • night sweats
  • feeling unusually anxious or worried
  • runny nose
  • hair loss
  • pain, irritation, swelling, redness or itching in the place where the injection was given (rituximab and hyaluronidase human injection)
  • unusual bruising or bleeding
  • sore throat, fever, chills, or other signs of infection
  • earache
  • painful urination
  • redness, tenderness, swelling or warmth of area of skin
  • chest tightness
Rituximab may cause other side effects. Call your doctor if you have any unusual problems while using this medication.

Methotrexate

What is Methotrexate

Methotrexate is used to treat severe psoriasis (a skin disease in which red, scaly patches form on some areas of the body) that cannot be controlled by other treatments. Methotrexate is also used along with rest, physical therapy, and sometimes other medications to treat severe active rheumatoid arthritis (RA; a condition in which the body attacks its own joints, causing pain, swelling, and loss of function) that cannot be controlled by certain other medications. Methotrexate is also used to treat certain types of cancer including cancers that begin in the tissues that form around a fertilized egg in the uterus, breast cancer, lung cancer, certain cancers of the head and neck, certain types of lymphoma, and leukemia (cancer that begins in the white blood cells). Methotrexate is in a class of medications called antimetabolites. Methotrexate treats cancer by slowing the growth of cancer cells. Methotrexate treats psoriasis by slowing the growth of skin cells to stop scales from forming. Methotrexate may treat rheumatoid arthritis by decreasing the activity of the immune system.

How should this medicine be used?

Methotrexate comes as a tablet to take by mouth. Your doctor will tell you how often you should take methotrexate. The schedule depends on the condition you have and on how your body responds to the medication.
Your doctor may tell you to take methotrexate on a rotating schedule that alternates several days when you take methotrexate with several days or weeks when you do not take the medication. Follow these directions carefully and ask your doctor or pharmacist if you do not know when to take your medication.
If you are taking methotrexate to treat psoriasis or rheumatoid arthritis, your doctor may tell you to take the medication once a week. Pay close attention to your doctor's directions. Some people who mistakenly took methotrexate once daily instead of once weekly experienced very severe side effects or died.
Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take methotrexate exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.
If you are taking methotrexate to treat psoriasis or rheumatoid arthritis, your doctor may start you on a low dose of the medication and gradually increase your dose. Follow these directions carefully.
If you are taking methotrexate to treat rheumatoid arthritis, it may take 3 to 6 weeks for your symptoms to begin to improve, and 12 weeks or longer for you to feel the full benefit of methotrexate. Continue to take methotrexate even if you feel well. Do not stop taking methotrexate without talking to your doctor.

Other uses for this medicine

Cyclophosphamide injection is also sometimes used to treat a certain type of lung cancer (small cell lung cancer; SCLC). It is also used to treat rhabdomyosarcoma (a type of cancer of the muscles) and Ewing's sarcoma (a type of bone cancer) in children and Myasthenia Gravis. Talk to your doctor about the risks of using this medication for your condition.
This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

What special precautions should I follow?

Before receiving cyclophosphamide injection,

  • tell your doctor and pharmacist if you are allergic to methotrexate, any other medications, or any of the ingredients in methotrexate tablets. Ask your doctor or pharmacist for a list of the ingredients.
  • tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take. Be sure to mention the medications listed in the IMPORTANT WARNING section and any of the following: certain antibiotics such as chloramphenicol (chloromycetin), penicillins, and tetracyclines; folic acid (available alone or as an ingredient in some multivitamins); other medications for rheumatoid arthritis; phenytoin (Dilantin); probenecid (Benemid); sulfonamides such as co-trimoxazole (Bactrim, Septra), sulfadiazine, sulfamethizole (Urobiotic), and sulfisoxazole (Gantrisin); and theophylline (Theochron, Theolair). Your doctor may need to change the doses of your medications or monitor you carefully for side effects.
  • tell your doctor if you have or have ever had any of the conditions mentioned in the IMPORTANT WARNING section or a low level of folate in your blood.
  • do not breast-feed while you are taking methotrexate.
  • if you are having surgery, including dental surgery, tell the doctor or dentist that you are taking methotrexate.
  • plan to avoid unnecessary or prolonged exposure to sunlight or ultraviolet light (tanning beds and sunlamps) and to wear protective clothing, sunglasses, and sunscreen. Methotrexate may make your skin sensitive to sunlight or ultraviolet light. If you have psoriasis, your sores may get worse if you expose your skin to sunlight while you are taking methotrexate.
  • do not have any vaccinations during your treatment with methotrexate without talking to your doctor.

What side effects can this medication cause?

  • nausea, Vomiting
  • Loss of appetite or weight
  • abdominal pain
  • diarrhea
  • hair loss
  • sores on the mouth or tongue
  • changes in skin color
  • changes in color or growth of finger or toe nails
  • sore throat, fever, chills, or other signs of infection
  • poor or slow wound healing
  • unusual bruising or bleeding
  • black, tarry stools
  • painful urination or red urine
  • rash
  • hives
  • itching
  • difficulty breathing or swallowing
  • shortness of breath
  • cough
  • swelling in the legs, ankles, or feet
  • chest pain
  • yellowing of the skin or eyes
Cyclophosphamide may increase the risk that you will develop other cancers. Talk to your doctor about the risks of receiving cyclophosphamide injection.
Cyclophosphamide may cause other side effects. Call your doctor if you have any unusual problems while receiving this medication.

Cyclosporine

What is Cyclosporine

Cyclosporine and cyclosporine (modified) are used with other medications to prevent transplant rejection (attack of the transplanted organ by the immune system of the person who received the organ) in people who have received kidney, liver, and heart transplants. Cyclosporine (modified) is also used alone or with methotrexate (Rheumatrex) to treat the symptoms of rheumatoid arthritis (arthritis caused by swelling of the lining of the joints) in patients whose symptoms were not relieved by methotrexate alone. Cyclosporine (modified) is also used to treat psoriasis (a skin disease in which red, scaly patches form on some areas of the body) in certain patients who have not been helped by other treatments. Cyclosporine and cyclosporine (modified) are in a class of medications called immunosuppressants. They work by decreasing the activity of the immune system.

How should this medicine be used?

Cyclosporine and cyclosporine (modified) both come as a capsule and a solution (liquid) to take by mouth. Cyclosporine is usually taken once a day. Cyclosporine (modified) is usually taken twice a day. It is important to take both types of cyclosporine on a regular schedule. Take cyclosporine or cyclosporine (modified) at the same time(s) each day, and allow the same amount of time between doses and meals every day. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take cyclosporine or cyclosporine (modified) exactly as directed. Do not take more or less of the medication or take it more often than prescribed by your doctor.
Your doctor will probably adjust your dose of cyclosporine or cyclosporine (modified) during your treatment. If you are taking either type of cyclosporine to prevent transplant rejection, your doctor will probably start you on a high dose of the medication and gradually decrease your dose. If you are taking cyclosporine (modified) to treat rheumatoid arthritis or psoriasis, your doctor will probably start you on a low dose of the medication and gradually increase your dose. Your doctor may also decrease your dose if you experience side effects of the medication. Tell your doctor how you are feeling during your treatment.
Cyclosporine (modified) helps control the symptoms of psoriasis and rheumatoid arthritis, but does not cure these conditions. If you are taking cyclosporine (modified) to treat psoriasis, it may take 2 weeks or longer for your symptoms to begin to improve, and 12 to 16 weeks for you to feel the full benefit of the medication. If you are taking cyclosporine (modified) to treat rheumatoid arthritis, it may take 4 to 8 weeks for your symptoms to improve. Continue to take cyclosporine (modified) even if you feel well. Do not stop taking cyclosporine (modified) without talking to your doctor. Your doctor may decrease your dose gradually.
You may notice an unusual smell when you open a blister card of cyclosporine capsules. This is normal and does not mean that the medication is damaged or unsafe to use.
Cyclosporine (modified) oral solution may gel or become lumpy if it is exposed to temperatures below 68 °F (20 °C). You can use the solution even if it has gelled, or you can turn the solution back to a liquid by allowing it to warm to room temperature (77 °F [25 °C]).
Cyclosporine and cyclosporine (modified) oral solution must be mixed with a liquid before use. Cyclosporine (modified) oral solution may be mixed with orange juice or apple juice but should not be mixed with milk. Cyclosporine oral solution may be mixed with milk, chocolate milk, or orange juice. You should choose one drink from the appropriate list and always mix your medication with that drink.

To take either type of oral solution, follow these steps:

  • Fill a glass (not plastic) cup with the drink you have chosen.
  • Remove the protective cover from the top of the dosing syringe that came with your medication.
  • Place the tip of the syringe into the bottle of solution and pull back on the plunger to fill the syringe with the amount of solution your doctor has prescribed.
  • Hold the syringe over the liquid in your glass and press down on the plunger to place the medication in the glass.
  • Stir the mixture well.
  • Drink all of the liquid in the glass right away.
  • Pour a little more of the drink you have chosen into the glass, swirl the glass around to rinse, and drink the liquid.
  • Dry the outside of the syringe with a clean towel and replace the protective cover. Do not wash the syringe with water. If you do need to wash the syringe, be sure that it is completely dry before you use it to measure another dose.

What special precautions should I follow?

Before taking cyclosporine or cyclosporine (modified),

  • tell your doctor and pharmacist if you are allergic to cyclosporine, cyclosporine (modified), any other medications, or any of the inactive ingredients in cyclosporine or cyclosporine (modified) capsules or solution. Ask your pharmacist for a list of the inactive ingredients.
  • tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, and nutritional supplements you are taking, or plan to take. Be sure to mention the medications listed in the IMPORTANT WARNING section and any of the following: acyclovir (Zovirax); allopurinol (Zyloprim); amiodarone (Cordarone); angiotensin-converting enzyme (ACE) inhibitors such as benazepril (Lotensin), captopril (Capoten), enalapril (Vasotec), fosinopril (Monopril), lisinopril (Prinivil, Zestril), moexipril (Univasc), perindopril (Aceon), quinapril (Accupril), ramipril (Altace), and trandolapril (Mavik); angiotensin II receptor antagonists such as candesartan (Atacand), eprosartan (Teveten), irbesartan (Avapro), losartan (Cozaar), olmesartan (Benicar), telmisartan (Micardis), and valsartan (Diovan); certain antifungal medications such as fluconazole (Diflucan), and itraconazole (Sporanox); azithromycin (Zithromax); bromocriptine (Parlodel); calcium channel blockers such as diltiazem (Cardizem), nicardipine (Cardene), and verapamil (Calan); carbamazepine (Tegretol); cholesterol-lowering medications (statins) such as atorvastatin (Lipitor), fluvastatin (Lescol), lovastatin (Mevacor), pravastatin (Pravachol), and simvastatin (Zocor); clarithromycin (Biaxin); dalfopristin and quinupristin combination (Synercid); danazol; digoxin (Lanoxin); certain diuretics ('water pills') including amiloride (Midamor), spironolactone (Aldactone), and triamterene (Dyazide); erythromycin; HIV protease inhibitors such as indinavir (Crixivan), nelfinavir (Viracept), ritonavir (Norvir, in Kaletra), and saquinavir (Fortovase); imatinib (Gleevec); metoclopramide (Reglan); methylprednisolone (Medrol); nafcillin; octreotide (Sandostatin); oral contraceptives (birth control pills); orlistat (Xenical); phenobarbital; phenytoin (Dilantin); potassium supplements; prednisolone (Pediapred); repaglinide (Prandin); rifabutin (Mycobutin); rifampin (Rifadin, Rimactane); sulfinpyrazone (Anturane); terbinafine (Lamisil); and ticlopidine (Ticlid). Your doctor may need to change the doses of your medications or monitor you more carefully for side effects
  • if you are taking sirolimus (Rapamune), take it 4 hours after you take cyclosporine or cyclosporine (modified).
  • Tell your doctor what herbal products you are taking, especially St. John's wort.
  • tell your doctor if you have or have ever had any of the conditions mentioned in the IMPORTANT WARNING section or any of the following: low cholesterol, low levels of magnesium in your blood, any condition that makes it difficult for your body to absorb nutrients, or liver disease.
  • tell your doctor if you are pregnant or plan to become pregnant. If you become pregnant while taking either type of cyclosporine, call your doctor. Both types of cyclosporine may increase the risk that your baby will be born too early.
  • tell your doctor if you are breast-feeding or planning to breast-feed.
  • do not have vaccinations without talking to your doctor.
  • you should know that cyclosporine may cause growth of extra tissue in your gums. Be sure to brush your teeth carefully and see a dentist regularly during your treatment to decrease the risk that you will develop this side effect.

What side effects can this medication cause?

  • headache
  • diarrhea
  • heartburn
  • gas
  • increased hair growth on the face, arms, or back
  • growth of extra tissue on the gums
  • acne
  • flushing
  • uncontrollable shaking of a part of your body
  • burning or tingling in the hands, arms, feet, or legs
  • muscle or joint pain
  • cramps
  • pain or pressure in the face
  • ear problems
  • breast enlargement in men
  • depression
  • difficulty falling asleep or staying asleep
  • unusual bleeding or bruising
  • pale skin
  • yellowing of the skin or eyes
  • seizures
  • loss of consciousness
  • changes in behavior or mood
  • difficulty controlling body movements
  • changes in vision
  • confusion
  • rash
  • purple blotches on the skin
  • swelling of the hands, arms, feet, ankles, or lower legs
Cyclosporine and cyclosporine (modified) may cause other side effects. Talk to your doctor if you experience unusual problems while taking either medication.

Thymectomy

What is a thymectomy and why is it performed?

A thymectomy is the surgical removal of the thymus gland.
The thymus has been demonstrated to play a role in the development of MG. It is removed in an effort to improve the weakness caused by MG, and to remove a thymoma if present. About 10% of MG patients have a tumor of the thymus called a thymoma. Most of these tumors are benign and tend to grow very slowly; on occasion they are malignant (“cancerous”).

What is the function of the thymus? Is its removal harmful?

The thymus plays a major role in the development of the body’s immune system. This function appears virtually complete by birth. Removal of the thymus in the treatment of MG does not affect the immune system thereafter.

Exactly where is the thymus located?

The thymus is located in the front portion of the chest (anterior mediastinum) with “finger-like” extensions into the neck and consists of multiple lobes (two to five or more). In addition, varying amounts of thymic tissue may be present in the fat surrounding the lobes, both in the neck and chest.

Who should have a thymectomy?

Although it is not definitely established which patients should have a thymectomy or what type of operation should be performed, a thymectomy is frequently recommended for patients under the age of 60 (occasionally older) with moderate to severe MG weakness.
It is sometimes recommended for patients with relatively mild weakness, especially if there is weakness of the respiratory (breathing) or oropharyngeal (swallowing) muscles. It is also recommended for all patients with a thymoma. A thymectomy is usually not recommended for patients with weakness limited to the eye muscles (ocular myasthenia gravis).

What should I expect as I consider a thymectomy?

When a thymectomy is being considered, the patient is referred to a surgeon. It is important to choose a surgeon experienced in performing thymectomies for patients with MG. The surgeon will review the clinical records, examine the patient, discuss the surgical choices with the patient and make a recommendation. The surgeon also explains the anticipated pre- and post-operative courses, possible complications, and the anticipated results. The patient, in consultation with the neurologist and surgeon, then makes a decision whether to proceed with a thymectomy and the type of surgery to be used.

What are the goals of a thymectomy?

The neurological goals of a thymectomy are significant improvement in the patient’s weakness, reduction in the medications being employed, and ideally eventually a permanent remission (complete elimination of all weakness off all medications).
A thymectomy is not used to treat active disease but rather it is believed to improve long-term outcome. This may not be seen for one-two years or more. A research trial is currently underway (partially funded by MGFA) to examine the effects of thymectomy on MG patients.
These operations are now performed with minimal risk.

How is the surgery performed?

Here are three basic surgical approaches, each with several variations. Regardless of the technique employed, the surgical goal is to remove the entire thymus. Many believe this should include removal of the adjacent fat; others are less sure.
Transsternal Thymectomy
Incision: Vertical (lengthwise) on the anterior chest; the sternum (breastbone) is “split” vertically.
Thymus Removal: The chest and neck portion of the thymus are removed through this incision.
Extended Form: The fat located in the front part of the chest next to the thymus, as well as the thymus, is removed.
Complete removal of all tissue containing thymus is believed ensured.
Combined Chest and Neck: A few MG Centers add a formal neck dissection to the sternal technique to also ensure the removal of all the thymus in the neck.
Thymoma: Most recommend the transsternal approach for removal of a thymoma. Transcervical Thymectomy
Incision: Transverse (horizontal) across the lower neck.
Thymus Removal: The chest portion of the thymus is removed through this incision.
Extended Form: The “extended” form allows improved exposure of the thymus in the chest with more complete removal of the thymus. Although the adjacent fat is also removed, less is removed than in the extended Videoscopic (VATS) Thymectomy
Incision: Several small incisions on the right or left side of the chest.
Thymus Removal: Fiber-optic instruments are used. These are small flexible tubes with a light at the end through which small instruments can be passed. The amount of thymus and fat removed is variable.
Extended Form: In the “VATET” form, incisions are made on both sides of the chest, as well as in the neck, for “more complete” removal of the thymus.

What are the results of a thymectomy?

Myasthenia Gravis Many neurologists experienced in the treatment of MG are convinced that a thymectomy plays an important role in the therapy of MG, although the benefit is variable, hard to define and remains unproven by today’s strict standards. In general, most patients begin to improve within one year following a thymectomy and a variable number eventually develop a permanent remission (no weakness and no medication). Some physicians believe the remission rates after surgery are in the 20-40% range regardless of the type of thymectomy performed. Others believe that the remission rates following the more extensive procedures are in the 40-60% range five or more years after the surgery. It is important to note that rigorous scientific studies are needed to resolve the debate concerning the role of and best method of performing a thymectomy in the treatment of MG patients.

What type of thymectomy should I have?

Since there is no universal agreement, or unequivocal proof, as to which type of thymectomy is best, it is difficult for patients to decide what is best for them. There is, however, general agreement that the entire thymus should be removed and that the patient should select the procedure that ensures as much as possible that this is accomplished. Some surgeons believe that all the surrounding fat should be removed as well, because it frequently contains microscopic (very small) amounts of thymus; others believe this may not be necessary.
The most frequently used procedure is the extended form of the transsternal thymectomy. Its proponents believe that it gives the best assurance that, in most instances, the entire thymus is being removed and performed safely, and that it produces the best long-term results.
Those advocating the transcervical or the videoscopic thymectomy do not share the above observations. They believe the “minimally invasive” procedures (transcervical and videoscopic) are as effective. Since there is no absolute proof as to which type of thymectomy is the procedure of choice, patients need to be fully informed, review the evidence presented by the neurologist and surgeon caring for them, and perhaps obtain additional consultation.

What can the MG patient expect in the pre-operative, anesthesia and post-operative periods?

In general, MG Centers have developed protocols for the care of MG patients and have a team of neurologists, surgeons, pulmonologists, intensive care and respiratory care specialists, nurses and anesthesiologists caring for MG patients undergoing a thymectomy. Patients should discuss all aspects of the pre-and post-operative care and anesthesia with the surgeon, anesthesiologist and neurologist
To reduce the risks of post-operative respiratory complications or the post-operative need for prolonged respiratory support with a ventilator (breathing machine), many patients require pre-operative plasma exchange or intravenous immunoglobulin (IVIg), and some require immunosuppressive therapy as well. Pyridostigmine, if being administered, may or may not be discontinued the day of surgery, and may or may not be restarted immediately post-operatively.
The anesthesia for patients with MG is similar to the anesthesia given to other patients. An endotracheal tube (tube in the windpipe) is inserted after the patient is asleep. Muscle relaxing drugs, however, are usually avoided. The patient may or may not be extubated (removal of the endotracheal tube) upon awakening, depending on the patient’s strength. If the endotracheal tube is not removed on awakening, the tube will be attached to a ventilator.
Ordinarily after the surgery, the patient will go to a Recovery Room, Respiratory Care Unit or Intensive Care Unit depending on each hospital’s method of taking care of MG patients following surgery. A ventilator may be required depending on the type of operation and the severity of the patient’s weakness. As soon as the breathing tube has been removed, the patient will be asked and helped to deep breath and cough frequently to keep the lungs clear of secretions. One or two chest tubes (small tubes exiting the chest and attached to drainage bottles) are usually used after the transsternal and videoscopic operations, and removed soon after surgery.
Medications used to manage MG before surgery is usually continued after surgery. The neurologist will decide how to taper your medications after surgery in subsequent follow-up appointments. Pain is minimal following transcervical thymectomy and usually mild following videoscopic thymectomy, although some patients have reported late pain. The pain associated with transsternal thymectomy is temporary, well controlled with medication and gradually resolved within 3-5 days.
Patients typically require minimal pain medication on hospital discharge. The length of time in the hospital will vary epending on the type of surgery and the patient’s overall weakness. In most cases the patient will be ready to go home in a few days to a week. The patient’s preoperative medications, immune suppression and other forms of therapy are usually resumed after surgery for variable periods of time depending on the MG symptoms and the neurologist’s recommendations.

When can I return to my usual activities?

Patients should discuss this with the surgeon in advance and let their employer or school know the anticipated time they will miss.
The recovery period and the time away from regular activities, like work or school, will vary depending on the patient’s weakness, type of surgery and type of the patient’s activities. A patient who does heavy lifting or construction work will be off work longer than someone who has a desk type job. In general, three to six weeks of limited activities is a common length of time for recovery.

Will my insurance pay for the surgery?

Health insurance companies do pay for thymectomies. Since there may be questions concerning insurance coverage with specific surgeons and specific institutions, it is a good idea to check with your insurance company as soon as a thymectomy is being considered to make sure that the coverage is in order. Some insurance companies may require additional consultation and some type of prior authorization. In some instances letters from the neurologist and surgeon will be required to defend the procedure selected.
These are just a few of the many questions patients with MG may have while considering a thymectomy. Physicians and nurses want their patients to do well, and part of that consists of patients being as informed and involved with their own care as possible.
It is encouraging to note that most patients have very few problems after the surgery and are able to return to their usual pre-operative activities relatively soon.

Biperiden – Akineton
Menrium, PMB
Decamethonium – Syncurine
Orphenadrine – Disipal
Dimethyltubocurarine – Metubine
Citrate – Norflex, Banflex, Marflex
Gallamine – Flaxadil
ASA & phenacetin – Norgesic, Norgesic Forte
Hexafluorenium – Mylaxen
Procyclidine HC1 – Kemadrin
Pancuronium – Pavulon
Chlorphenesin carbamate – Maolate
Succinylcholine – Qualicin, Sucostrin, Anectine
Chlorzoxazone – Paraflex
Tubocurarine – Syncurine
acetaminophen – Somacort
Phenyramidol salicylate – Analexin

Carisoprodol – Rela, Soma
Mephenesin – Dioloxol, Medigesic, Tolseram
ASA & codeine – Soma Compound
butabarbital – Halabar
prednisolone – Somacort
butabarbital & acetaminophen - Bancaps
Methocarbamol
Meprobamate – Equanil, Miltown, Neuromate
Aspirin – Marbaxin, Delaxin
Tranmep, Meprospan, Milpath
Phenephen – Robaxisal
Benactyzine – Deprol
Stramate – Sinaxar
Eptoheptazine – Equagesic
Metaxalone – Skelaxin
Pentaerythritol – Equanitrate, Miltrate
Chlormezanone – Trancopal
Conjugated estrogens – Milprem

Diuretics

Carbonic Annhydrase Inhibitors – Diamox
Hydro-t, various generics
Diamox Sequels, Ak-zol, Dazamide
Bendroflumethiazide – Naturetin
Chlorthalidone – Hygroton, Hylidone, Thalitone
Methyclothiazide – Enduron, Ethon, Aquatensen
Ethacrynic acid – Edecrin

Benzthiazide – Exna, Proaqua, Hydrex, Aquatag
Furosemide – Lasix, various generics
Hydroflumethiazide – Diucardin, Saluron
Metolazone – Zaroxolyn, Diulo
Trichlormethiazide – Naqua, Metahydrin, Diurese
Quinethazone – Hydromox
Polythiazide – Renese
Hydrochlorthiazide – Oretic, Hydrdiuril, Esidrix

Morphine and Narcotic Analgesics

Morphine – MS Contin, Roxanol, generics
Oxycodone w/ Aspirin – Percodan
Hydromorphone – Dilaudid
Acetaminophen – Percocet
Levorphanol – Levo-Dromoran

Tylox
Methadone – Dolophine
Propoxyphene – Darbon, Darvocet, Darvon-CPD
Meperidine – Demerol, Pethodol
Pentazocine – Talwin
Codeine – numerous generics

Thyroid Preparations

Thyroid USP – Westhroid, generics
Levo-Thyroxine – Levothroid, Synthroid, Noroxine
Dessicated Thyroid – Thyroid Strong

Liothyronine – Cytomel
Thyroglobulin – Proploid
Liotrix – Euthroid, Thyroloar

Tranquilizers

Diazepam – Valium, Valrelease

Chlordiazepoxide – Librium, Libritabs, Lipoxide, SK-Lygen, A-Poxide

Acth and Corticosteroids

Corticotropin inj. Acthar, ACTH
Methylprednisolone – Medrol
Repository inj. – Corticotropin Gel, Cortigel
Dexamethasone – Decadron, numerous generics
Cotropic Gel, H.P. Acthar Gel
Betamethasone – Celestone

Cosyntropin – Cortrosyn
Prednisone – many generic brands, *Deltasone
Fudrocortisone Acetate – Florinef
Prednisolone – many generics
Cortisone Acetate – various, UpJohn, MSD
Triamcinolone – Aristocort, numerous generics
Hydrocortisone – various, UpJohn, MSD

Anesthetics

Chloroform – numerous generics
Ether – various generics

Halothane – Fluothane
Procaine IV – Novocaine, Andurocaine

Anti-Arrhythmics

Procainamide – numerous generics, Pronestyl, Procan
Cardioquin

Quinedine – various generics, Cin-Quin, Quinidex
Propranolol – Inderal, Inderal-LA, generics soon
Extentabs, Quinaglute Duratabs

Antibiotics

Bacitracin – numerous generics
Polymixin B – Aerosporin, generics
Colistimethate – Coly-Mycin M
Sterptomycin – Various generics
Colistin – Polymixin E, Coly-Mycin S
Vancomycin – Vancocin, some generics
Gentamicin – Garamycin, generics

Tobramycin – Nebcin, some generics
Kanamycin – Kantrex
Tetracyclines - Achromycin, Cycline, Cyclopar
Neomycin – Mycifradin, Neobiotic
Sumycin, Tetralan, Robitet
Novobiocin – Albamycin
numerous generics

Anti-Convulsants

Magnesium Sulfate – numerous generics

Phynytoin – Dilantin, Ditan

Anti-Malarials

Quinine – Quine, Strema, QM-260, Quinite

Quiphile, coco-quinine

Anti-Psychotics

Chlorpromazine – Thorazine, Promapar
Lithium – Eskalith, Lithane, Lithotabs

Thor-Prom, numerous generics
Cibalith-S, Lithobid

Anti-Rheumatics

Chloroquine – Aralen, Plaquenil

Barbiturates and Other Sedative Hypnotics

Phenobarbital – Barbita, PBR 12, numerous generics
Glutethamide – Doriden, numerous generics
Mephobarbital – Mebaral
Methaprylon – Noludar
Amorbarbital – Amytal
Ethchlorvynol – Placidyl
Butabarbital – Butisol, Butaian

Flurazepam – Dalmane
Secobarbital – Seconal
Lorazepam – Ativan
Pentobarbital – Nembutal
Temazepam – Restoril
other combinations – Tuinal, Plexonal, Tri-barbs
Triazolam – Halcion
Chloral Hydrate – Noctec, Oradrate

Cathartics

Citrate of Magnesia – Citroma, Citro-nesia, generics

Epsom Salts – Magnesium sulfate, generics