Lambert-Eaton Myasthenic Syndrome: Learning Self-Advocacy by Changing Treatment


Lori Dunham’s recent article for the Lambert-Eaton News is a message to parents that no matter what disease their children may have, teaching them to advocate for themselves is important.

Her daughter, Grace, was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) two years ago. So far, the family has been unable to adjust to the constant changes in symptoms. They are still trying to understand how LEMS is affecting their daughter.

About LEMS

LEMS is a condition whereby a person’s immune system disrupts the connections between the muscles and the nerves. Symptoms include difficulty walking, weak muscles, fatigue, and tingling sensations.

Lori explains that each time Grace seems to adjust to a routine they are understandably encouraged. Grace will appear to have more energy, an improvement in walking, strength, and balance. That “routine” may last for a week.

Typically the following week will be different. Grace will not have much strength and be unable to eat. She often has intestinal problems which appear to be a result of her medication. Her sleep on those nights is intermittent.

After two years of being unable to keep a routine because LEMS is so unpredictable, Lori decided, that with the doctor’s approval, they would make changes to Grace’s medication schedule.

Lori had an idea about which medication may be causing the problems and also what changes need to be made. That was the easy part.

Lori faced a more difficult problem when confronting Grace with suggestions about changing the medication schedule. Grace did not like change and had become accustomed to the current regimen.

As Lori said, her devotion and adherence to routine and “following the rules” is admirable. However, her rigidity prevented Grace from adjusting to the needs of her body. This was the dilemma that Lori faced.

Mestinon and Ruzurgi

Grace had been prescribed Ruzurgi (amifampridine) and Mestinon (pyridostigmine) which had recently been FDA approved for children with LEMS. Her parents felt strongly about the need to reconstruct Grace’s medication schedule (with the doctor’s approval).

Each medication was taken three times each day. Unless she took Ruzurgi before getting out of bed, Grace would remain confined to her bed.

Lori had always assumed that the two drugs must be taken together. She also did not know that Mestinon can cause intestinal problems.

Then to complicate the picture, Grace had several changes in her school schedule. She had to be kept active longer and that required a change in the schedule.

For example, taking the last dose of medicine at 5 p.m. was too early, but Grace made it clear that she would not take a lesser dose more often.

It was stressful trying to disrupt her routine, but Lori kept pressing the issue.

Lori was finally successful in getting Grace to make some changes. The idea worked. The main advantage was that her intestinal problems ended and her energy increased.

The changes, in general, amounted to taking Ruzurgi while she is in bed in the morning and taking Mestinon after each meal rather than before or during the meal. She then started taking a half dose of Ruzurgi at scheduled intervals throughout the day.

The Key is Flexibility

That is the message that Grace’s parents hope will help other parents. That they learned the importance of coordinating medication with circumstances. That the symptoms from most diseases are not consistent. That each day or each week may be different than the one before.

Lori calls this “looking for a sweet spot” that will provide optimal benefit all day long.

The Goal

The goal was to teach Grace to be her own advocate. To recognize when something is not working and to bring that issue to the attention of her doctor. Lori acknowledges that she may not always be there for Grace which in reality means having a DIY attitude.

There was no doubt that the change in Grace’s medication schedule improved her quality of life. Lori emphasizes that a patient must never make any changes without the doctor’s approval. She also emphasizes that Grace’s cooperation in trying a new routine has paid off significantly.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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