Receiving a diagnosis of Lambert-Eaton myasthenic syndrome can be overwhelming. For many, the journey likely entailed multiple visits to doctors and many years of waiting.
When we received our daughter Grace’s diagnosis, we learned there is no cure, but we were encouraged upon discovering that numerous treatments are available to improve her quality of life.
It took some time to find a treatment that gave Grace significant, life-altering results.
Ruzurgi (amifampridine) or Firdapse (amifampridine) in conjunction with Mestinon (pyridostigmine) is often the first line of defense in helping LEMS patients gain strength and manage symptoms. Ruzurgi has allowed my daughter to get out of bed in the morning. Without it, she would be hindered significantly.
But after these medications, what works best is very person-specific. Many seem to try intravenous immunoglobulin (IVIG) therapy next because it can positively affect the immune system by flooding it with antibodies.
Grace’s neurologist recommended IVIG. It definitely gave her a boost of energy and balance, but she was still hindered in her abilities.
I am grateful that Grace’s doctor has never been too proud to ask for his colleagues’ professional opinions. He first invited Mayo Clinic doctors to help diagnose Grace. He also referred her to a pediatric rheumatologist so he could consult with her about the next course of treatment.
Grace’s neurologist and rheumatologist mutually agreed that it was in Grace’s best interest to start a course of Rituxan (rituximab).
Rituxan is administered via infusion. It suppresses the growth of B-cells, decreasing the amount in the blood, which in turn hinders them from attacking the neuromuscular junctions.
The first round of Rituxan is given in two doses, two weeks apart. The morning of the infusion, nurses start Grace with oral Tylenol and Benadryl.
They then run her IV and give her a drip of Solu-Medrol (methylprednisolone), a corticosteroid that can lessen or prevent allergic reactions to Rituxan. This steroid leaves Grace with a metallic taste in her mouth, so we always have gum or lollipops on hand.
Once the steroid is administered, nurses begin the Rituxan drip. This is when we settle in for the day. Typically, our days at the hospital run about 10 hours long.
Grace has experienced impressive results from Rituxan, including:
She has more energy
Before starting Rituxan, Grace would express how tired she was throughout the day. She now has significantly more energy. Not only does she have more energy, but also it is more consistent, with fewer highs and lows throughout her day.
She no longer slurs her words
Grace usually wakes up in the morning with slurred speech until her Ruzurgi kicks in. Since her second dose of Rituxan, she has been able to speak clearly from the moment she wakes up.
Rituxan kicks in faster
It normally takes Grace about 35 minutes to feel the effects of Ruzurgi, but with Rituxan, she is able to get up after about 15 minutes.
She has a sturdier, faster walk
Most notably, Grace’s ability to walk unassisted has significantly increased. She is able to walk faster, farther, and more sturdily without becoming tired.
She is more productive throughout the day
Because of the increased energy, Grace is able to accomplish more throughout the day, which brings her much hope as she looks to her future.
Although the infusion is an extremely long day, the results of Rituxan have proven to be well worth the effort. We have yet to see any adverse side effects.
This treatment has given us hope for her future and encouragement for today.
Note: Lambert-Eaton News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Lambert-Eaton News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to Lambert-Eaton myasthenia.
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