In this issue:

	A message from our Foundation chair
	A message from our Chief Executive
	Presentation summaries from the 2006 Annual Meeting
	2006 Annual Meeting Awards
	A poem
	An update from Ester Neurosciences
	XIth International Conference on Myasthenia Gravis and Related Disorders
	GoodSearch.com
	Update: Foundation directors
	A summary of “The Psychosocial Aspects of Myasthenia Gravis”
	Across the country, great things are happening!
	Calendar
	Associations
	Would you like to donate to MGFA?
	Subscription information

Enthusiasm and renewed commitment are the offspring of the 2006 Annual Meeting. This newsletter features highlights from the conference.

An energized Board of Directors shared with the membership its strategy and vision. A year of listening to members and chapters shapes our vision for the future as we implement new initiatives in communication and focus on long-term research programs.

Passage of the resolution to establish a research endowment fund is a visionary and monumental step forward to ensure ongoing research for improved treatment modalities and, ultimately, the cure of MG. A Chapter Challenge was presented to attendees to initiate this fund. In cooperation with the Medical/Scientific Advisory Board’s new Steering Committee for MG Research, additional implementation plans will be launched in the coming months.

Photo courtesy of Janet A. Myder

We are also delighted to announce the return of the Greater Kansas City Chapter (Myasthenia Gravis Association of Kansas City). After several independent years, the leadership recently sought chapter status with our Foundation. Marie Ronnlof, Chapter Liaison Officer, proudly welcomed them and presented the Chapter Charter to Abbie Whited, executive director, and Lisa Sackuvich, RN, Board President, during the Awards Banquet. We look forward to a mutually rewarding partnership with everyone at our newest chapter.

This coming year is also exciting as we plan for the prestigious XIth International Conference on Myasthenia Gravis and Related Disorders to be held May 14-16, 2007 in Chicago. Presentations from world-renowned researchers will confer the latest findings in the neuromuscular and immunology fields. (See article.)

Summer is here, and with it comes a flurry of activities: graduations, weddings, pool parties, barbecues, vacations, camp, family reunions, as well as all the stuff that we already do day after day! The song says “summertime, and the livin’ is easy,” but I know that, at my house, life is never busier than on a beautiful summer day.

Sometimes it’s not easy keeping up with all that’s going on around us. I often look at my children and wonder where they get all their energy, and I’m sure that, no matter how young or old you might be, or whether or not you have MG, there are times when you just have to sit down and relax. These are the times when you have to look after yourself and know your limitations. This is a lesson I learned at the recent Annual Meeting.

I learned that MG patients can know their limitations; they can know when they need to take a break and rest. I saw that MG patients can be in control of their illness and not allow it to control them. I also saw patients and their families looking for help, determining what their boundaries were, and I saw sharing of ideas and stories to provide support to those looking for guidance. MG patients and their families “know” MG and all that it means. They want answers, they want control over their treatment, and they want to have all available information—and they want it now! I have never met people so determined and focused on finding out more about their disease. This tells me that the MG community is active, strong and demanding for knowledge and action. The “Message of Hope in the Desert” was loud and clear for all to hear.

So, as we enjoy the summer, set your goals and expectations on what you can and cannot do. I know I will. Listen to that song and make the livin’ easy.

Disclaimer: The 2006 MGFA Annual Meeting presentation summaries represent the opinions and experiences of the individual presenters. The summaries are meant to give a sample of the content and complexity of the presentations and should not be construed as recommendations for treatment or diagnosis. Please refer to your personal physician should you have questions regarding any medication or treatment plan.

Health in a New Key
Roger A. Hughes, Ph.D.

When viewed in a “new key,” health is the harmonious integration of mind, body, and spirit within a responsive community. The central concept of health in a new key is resilience, which is a type of elasticity. The components of resilience include biological factors (temperament, genetic, and physical components), attachment, and control. Illnesses occur when you lack the above factors but, with mental capabilities, you can begin to change that outcome.

Resilient communities have the following components: diversity of species, redundancy in the sense of overlapping species, and feedback loops that create stable connectivity. Furthermore, resilient communities can be characterized by their setting of boundaries, sense of time, committed leadership, high degree of civic engagement, approach to development, focus on strengths, culture of active learning, and access to knowledge.

To develop resilience and begin thinking about health in a new key, we all need both strong and weak ties to really be happy.

Managing Myasthenia Gravis: for the Patient and Family
K. Sivakumar, MD

The three steps to managing MG are:

• Recognizing the symptoms.
• Confirming the diagnosis.
• Knowing the therapy.

A diagnosis can be confirmed in several ways, including the following:

• Acetylcholine Antibody testing – Approximately 85% of MG patients have this antibody and, when detected, is a guaranteed diagnosis.
• Anti-Musk Antibody testing – For the remaining 15% of seronegative (SN) MG patients, those who have tested negative for the acetylcholine antibody, 40-70% test positive for the anti-MuSK antibody. The remaining patients have an unidentified antibody causing their MG.
• Other tests – Tensilon® test, Repetitive Nerve Stimulation to check for a post-synaptic defect, Single Fiber, or a muscle biopsy to look for antibodies may also be used.

MG treatment goals: To remove antibodies, reduce antibodies, and inhibit the binding of antibodies through the various medications and treatments available: Mestinon®, prednisone, Imuran®, cyclosporine, plasma exchange, thymectomy, IVIg, and CellCept®. Other medications that can be employed are tacrolimus, Cytoxan®, Enbrel®, and Rituximab®.

Please note: It is your neurologist who will make the determination on which medication you will likely benefit from.

To control MG symptoms: avoid fatiguing exercise, rest during the day, eat a high potassium diet (to help muscle function), reduce temperature, and always treat infections.

Myasthenic Autoimmunity and the Shrinking Seronegative Group
Tim Miller, MD

Most cases of MG can be traced to abnormal antibody production. Approximately 80-90% of MG patients have acetylcholine antibodies, which leaves 10-20% that can be classified as having “Seronegative” MG.

SN MG patients do not have acetylcholine antibodies, though clinically the disease is similar to those with AChR antibodies. Anti-Muscle Specific Kinase (MuSK) antibodies is another blood test that can be performed. Anti-MuSK antibody positive MG is more common in women, includes more bulbar and fewer ocular symptoms, and contains racial and ethnic differences.

Is this the whole story? Other areas to expand into include Anti-Striated Muscle Antibodies, Titin, and Ryanodine Receptors and, once this is discovered, the SN group will shrink even more.

The Blessings of Having MG
Dianne M. Pela, MAPC, LPC, CL

Dianna Pela is a myasthenia gravis patient who has overcome the traumatic first symptoms to become an inspiration to other patients as a therapist, motivational speaker, and theorist. For twenty years, she has helped change and challenged many lives who have participated in her seminars, workshops, and intensives to gain emotional and spiritual empowerment.

Her presentation focused on ways of getting rid of negative thinking and developing a personal strategy for letting go of negativity. As she was facing the challenges of the early days of diagnosis, she vowed, “MG won’t get my spirit!” She recorded tapes of self-affirmations of positive statements in the present tense.

MG is devastating—the not breathing, not swallowing, tongue thickness, helplessness of the patient and family. A person must find ways to cope with the many life changes. As she grew in going through the stages of accepting her life changes, she became aware of her humanity, was softened as a person, and realized her MG was a “blessing.” She became certain of why she is here today after being close to death. She tries to help others accept their circumstances. Now she says, “It is a wonderful feeling to be exactly where I should be. MG can never take my spirit.”

Thymectomy in Myasthenia Gravis
Michael T. Pulley, MD

The thymus is important for the development of the immune system and, as a person ages, it typically shrinks. This, however, does not necessarily occur, as 70% of MG patients have hyperplasia, which is a “revved up” or very active thymus, and about 10% of patients have a thymoma, or tumor, of the thymus gland. Thymic myoid cells express acetylcholine receptors and might play a role in triggering the development of MG.

Several issues occur when considering thymectomy:

• Patient selection: age, sex, severity of disease at time of surgery, thymic pathology, and antibody testing
• Timing: studies have suggested that earlier in the course of the disease is better but there is not a clear cut-off when it is no longer beneficial
• Surgical technique: depends on the anatomy of the thymus
• Preparation of the patient for surgery: could include use of IVIg or plasmapheresis if patient needs short-term improvement before surgery; low dose of steroids if possible
• Does thymectomy help: multiple studies suggest benefit with all surgery types but, because a controlled study has not been performed, success is challenging to record; a survey of MG experts indicated a majority have at least some hesitations about thymectomy

While many patients seem to benefit from thymectomy, the international thymectomy trial will help definitively prove its efficacy.

Myasthenic Crisis
Mark A. Ross, MD

A myasthenic crisis is characterized as severe weakness of the respiratory muscles and/or severe weakness of the bulbar muscles (used in speech and swallowing). Both situations would require artificial respiration to protect the airway. A crisis can be caused by a number of things, including rapid reduction of prednisone, infection, medication, surgery, initial presentation of MG, cholinergic crisis (too much Mestinon®), or the cause of the crisis may be unknown.

To treat the crisis, the MG patient would likely be admitted to the intensive care unit. While there, the neurologist will address the crisis and attempt to determine its cause. The patient will be taken off anticholiesterase medications as other things will be used to treat the MG, evaluated for infection and treated if one is found, and intubated. An immunemodulating therapy will also be given and this could be either plasmapherisis or IVIg because of the rapid response a patient has to the treatments. Once a patient’s MG symptoms are controlled, the plasmapheresis or IVIg will help wean the patient off the ventilator and long-term treatments will be chosen.

Treatment of Myasthenia Gravis with IVIG
Todd D. Levine, MD

IVIg, or intravenous immune globulin, is used to treat an increasing number of immune mediated neurological disorders. It is a mix of antibodies that has been derived from a large pool of plasma donors. Potential uses of IVIg in MG include treating patients with myasthenic crisis, patients who are not doing well on their current therapy, or to avoid the use of corticosteroids in induction of remission. IVIg floods the patient’s system with two or three times the normal amount of antibodies in an attempt to block the bad antibodies causing the weakness and fatigue.

IVIg is a safe and effective adjunctive treatment for MG; however, it is not a cure, because you are not lowering the level of antibodies in the system. IVIg treatment has several advantages: the rapid onset of the effect, lack of long-term toxicities, and the possibility of reducing doses of the immunosuppressive drugs.

While IVIg is a valuable treatment, there are side effects that can occur. During the infusion, the patient may experience itching, fever, chills, headache, or hypotension. Following the infusion, the patient may develop aseptic meningitis or flu-like symptoms. The side effects can all be treated through changing the speed of infusion or use of medications.

An issue that is often encountered with IVIg is the cost associated with the treatment. A single monthly infusion can range in cost from $6,000 to $10,000, and insurance coverage varies by carrier, which is why it is important to discuss with your physician whether IVIg is the correct treatment for you.

The Bermeo Boys: It’s Child’s Play
Ashley and William Bermeo

Javier and Miguel Bermeo are twins who have been diagnosed with congenital myasthenia gravis—although they haven’t let this slow them down! Like other six-year-olds, the boys enjoy outside activities including soccer and swimming, as well as attending school.

Similar to what other MG patients experience, there was a period of worry when Javier first developed symptoms and the cause was unknown; and, soon after, Miguel began to show signs, too. After visits with many doctors, it was determined that they both had MG. Javier then had a thymectomy and they both were put on medication to treat their symptoms.

The 2006 Annual Meeting of the Myasthenia Gravis Foundation of America, Inc. held in Arizona featured an awards ceremony to confer national recognition for outstanding achievement in support of the conquest of myasthenia gravis. Six awards were given to volunteers who served their communities.

Several awards recognized achievement in medical and service areas.

Two other special awards were presented by the MGFA Board of Directors in honor of the recipients’ years of service to the MGFA.

Ester Neurosciences (www.esterneuro.com) is an Israeli start-up company that has developed a unique approach for the treatment of myasthenia gravis (MG) and other neurological disorders. MG is an acquired immunological disorder in which neuromuscular transmission is damaged as a result of destruction of functioning acetylcholine receptors (AChR) by the immune system. Since ACh action is regulated by acetylcholinesterase (AChE) hydrolyzing activity, and since MG is characterized by understimulation of the muscles, drugs blocking the activity of AChE have proven to have an effective palliative treatment for this disease. However, accumulated experience attests to the limited extent and duration of clinical effects achieved with pharmacological AChE inhibitors.

Ester’s understanding of the cholinergic molecular and cellular biology suggests a novel approach towards treatment of MG, which is now in clinical studies. The basic premise of Ester’s therapeutic paradigm is the inhibition of the overexpression of a variant of acetylcholinesterase (AChE-R). Ester has developed and used Monarsen (EN101), an oligonucleotide antisense that inhibits this enzyme at its molecular production inside the cell. Translation of the AChE protein from mRNA is inhibited by a short chain of 20 nucleic acids that interferes with the correct reading of the protein from the mRNA, making the code unreadable (hence antisense). Monarsen has already shown safety and efficacy results in Ib clinical trial for the treatment of myasthenia gravis (MG).

Following a successful phase Ib clinical trial, a phase IIa randomized double blind study has been designed to evaluate the safety and efficacy of three different doses of oral Monarsen (10, 20 and 40 mg) administered to MG patients in Israel and the UK. For each patient, the three blinded doses are administered for one week each (once daily), followed by one week of treatment with Mestinon® and a final follow-up period of four weeks.

Following the completion of patient recruitment in Israel, an interim safety and efficacy analysis was conducted, without un-blinding the data. Efficacy was measured by the QMG score—a score that is used commonly in MG studies and measures the strength of 13 different muscle groups. The QMG scored was measured at different time points: without any treatment, during Monarsen treatment, and during Mestinon® treatment. The percent improvement was defined as difference between QMG score measured without any treatment (12-18 hours without Mestinon®) and after treatment with Mestinon® or Monarsen. (In order not to un-blind the data, the QMG score of the three Monarsen doses was averaged.) Safety parameters included blood, urine, and ECG tests and reporting of adverse reactions.

The interim efficacy results showed that the treated group with Monarsen exhibited an improvement in the mean QMG score when compared to treatment with Mestinon®. The Monarsen groups showed a 20 to 25% improvement as compared to treatment with Mestinon®. The difference was statistically significant in all the groups and showed a dose-dependent efficacy. These results are very encouraging, especially due to the small number of patients.

Safety analysis showed Monarsen to be safe.

Under the direction of co-organizers Richard J. Barohn, MD and Henry J. Kaminski, MD, and in cooperation with the New York Academy of Sciences, May 14-16, 2007 are the dates set for this internationally esteemed symposium.

The conference provides a forum for basic and clinical investigators to explore new findings in MG research, as well as provide expertise from investigators outside MG to enhance investigative approaches to MG and related disorders. Additionally, junior and senior members of the research groups learn from and share with others, thus encouraging future project collaboration as the insights gained are effectively disseminated to other scientists from the diverse disciplines.

Held every five years since the mid-1950s, the conference draws presenters from around the world based on their expertise and how the topic fits with the thematic program. Since the 2002 conference, there have been a dramatic number of significant reports regarding the structure and function of the neuromuscular junction (NMJ) and corresponding studies in the understanding of disorders of the NMJ. Over the past few symposia, attendance has ranged from 130 to 160, with 2002 participants coming from 21 countries.

GoodSearch.com designated the Myasthenia Gravis Foundation of America, Inc. as the “Charity of the Day” on June 22, 2006.

An easy way for everyone to help MGFA a few pennies at a time is by using the search engine Good Search at www.goodsearch.com. When a person uses Good Search and designates the Myasthenia Gravis Foundation of America, Inc. as the charity to receive the proceeds, MGFA will be getting valuable dollars.

The Myasthenia Gravis Foundation of America, Inc. has partnered with GoodSearch.com to raise money for MGFA. GoodSearch.com is a search engine and site powered by Yahoo!. What’s unique about this search engine is that it has developed a way to direct money to MGFA with every click. The more people who use this site for their Internet searching, the more money will go to needed MG research and other programs.

If 1,500 people search only twice a day, MGFA will receive over $10,000 a year. This is something MG patients and their families can do—just searching the Internet with GoodSearch.com. There’s no limit; the more searches, the larger the donation.

So please spread the word to your friends and family. It doesn’t matter where you live; anyone can help! Go to www.goodsearch.com and put the Myasthenia Gravis Foundation of America, Inc. St. Paul, MN in the “I’m supporting” box and go searching!

You can easily spread the word of how MGFA can receive needed research funds from GoodSearch.com by using the “Tell a Friend” link on the left side of the GoodSearch opening page. In addition, by adding a line about GoodSearch to the signature line of your e-mails, you can spread the word with each e-mail you send. See www.goodsearch.com/EmailSignature.aspx for instructions on how to do this in various e-mail programs.

Marcia S. Lorimer, RN, MSN, CPNP, Durham, NC – Marcia continues on the Board after serving as Nurse Advisory Board (NAB) Immediate Past Chair, a non-voting position. By profession, she is Director of the Pediatric Nurse Practitioner Specialty at Duke University School of Nursing. A myasthenia gravis patient since age 10, Marcia is very much aware of the challenges that face MG patients and their caregivers. Prior to her service as NAB representative, she served on the Board from 1991-2001 in several capacities, on a variety of committees, and as Chairperson. Marcia brings valuable perspective and vision to the Foundation.

Robert L. Ruff, MD, PhD, Cleveland, OH – Dr. Ruff returns to our Board, having previously served from 1994-2003. He is a professor of neurology at Case Western Reserve University School of Medicine, National Director for Neurology and acting Director of the Rehabilitation Research and Development Service in the Department of Veterans Affairs, principal investigator at the Functional Electrical Stimulation Center, and chief, SCI Unit, at Cleveland VA Medical Center. Dr. Ruff has authored and co-authored many research papers on the neuromuscular junction. He has been a member of MGFA’s Medical/Scientific Advisory Board since 1988. He is a medical advisor to the Ohio Chapter and on their Board.

Roberta (Robyn) E. Spearot, Audubon, PA – Robyn joins our Board after being involved in leadership roles with the Connecticut “Nutmeg” State Chapter since her diagnosis if MG in 1996. Her professional career included 20 years of service to people with disabilities, during which she developed and coordinated staff training for a large social services agency prior to retirement. Robyn has a passion for helping others with MG and is excited about serving as a national Board member with the MGFA.

The Board also gratefully acknowledges outgoing directors Raina M. Ernstoff, MD and Cora E. Scarlet, RN, MS, who both faithfully served for the past nine and eight years respectively.

Additionally, the membership elected Marie Ronnlof as the incoming Chapter Liaison Officer and subsequently Stanley Way was elected Board Secretary.

Very little is known about the connection between mental and emotional stressors and MG. The limited research that has been conducted is outdated and often either overemphasizes psychological dysfunction or excuses this dysfunction as a result of MG. This study’s aim was to obtain current and accurate information about the psychosocial aspects of the myasthenia gravis population.

Sixty-seven people with MG, ages 21-81, completed the Millon Behavioral Medicine Diagnostic (MBMD) and a demographic questionnaire. The MBMD is a paper-and-pencil test that assesses the participant’s mental status and coping skills in relation to his/her chronic illness. The data gathered provided an MBMD profile for each participant and an MBMD profile for the MG sample population.

The MBMD is unique from past research because it compared the MG sample population to populations of other chronic illnesses such as cancer, diabetes, and pain. The research showed the MBMD profile for the MG sample was comparable to the norm sample of the MBMD, indicating that people with MG react to their illness similarly to how people with other illnesses respond. This refutes the past literature’s conclusion that people with MG have more psychosocial issues. The only area in which the MG differed from the MBMD norm group was in Adjustment Difficulty. This indicates that people with MG may have a more difficult time adjusting to their illness than people with other diseases. This may possibly be due to the obscurity of the disease or the fact that people with MG must always be adjusting to their illness due to the fluctuating nature of the disease.

Further analyses were done to evaluate the MG participants based on gender, age, length of time since diagnosis, thymectomy status, and self-reported severity of illness. MG respondents showed significant differences between genders in many areas, revealing that men and women react differently to the same diagnosis. Participants that have had MG for less than five years had more problems with functional deficits, ability to conduct activities of daily living, and adjustment difficulties than those who had MG longer than five years. Younger individuals, ages 18 to 49, had more anxiety/tension and a greater chance of poor psychological response to medical treatments than those who were older. Participants who rated their illness as severe had elevations in the following areas: Illness Apprehension, Functional Deficits, Future Pessimism, and Adjustment Difficulties. There were no significant statistical differences based on thymectomy status of the MG sample. These findings indicate that factors such as gender,
time since diagnosis, age, and self-reported severity of illness influence how people with MG react to the illness, while thymectomy does not have significant influence.

This study shows that there is concern about how people with MG are mentally and emotionally dealing with their disease. Out of the 67 respondents, only 23 people had sought counseling. The number one reason people did not seek out counseling was because they did not know about it or their doctor did not suggest it. The psychosocial barriers for people with MG that this research uncovered can be alleviated through the help of counseling. Counseling referrals can be made through physicians or insurance companies at the request of the patient. This study has provided a clinical picture of psychosocial stressors and coping styles of MG for patients and mental health professionals to better understand the psychosocial aspects of MG.

As (hopefully) many of you are aware, June is Myasthenia Gravis Awareness Month. Throughout the month of June, MGFA, our chapters—and many others involved in MG—work diligently to get the word out about the disease. June 2006 was no exception.

Across the country, many, many volunteers made special efforts to reach out to their communities about myasthenia gravis. I would like to share with you a few examples of what was accomplished. And remember: you don’t have to wait for next June to increase awareness of MG. Every day gives us the opportunity to teach others about the disease!

Our Massachusetts/New Hampshire Chapter was busy this year. Not only were they able to get several municipalities to officially declare and recognize MG Awareness Month as early as April, but managed to get Massachusetts Governor Mitt Romney to sign a proclamation recognizing June as Awareness Month throughout the entire state. Our Massachusetts/New Hampshire Chapter was honored at a State House ceremony on Wednesday, June 14, where the proclamation was presented.

In May, our Garden State Chapter had several great outreach successes leading up to June. On May 7th, WABC-TV, ABC’s New York affiliate, featured the chapter’s Walk-a-Thon during the morning show. About one week later, the Chapter also managed to get articles into two major local newspapers: The Herald News and The Record.

Associations

MGFA is proud to be a member of the following associations: