Chatting with a pillar of the community about life with LEMS

I am a mom to an adult daughter who has Lambert-Eaton myasthenic syndrome (LEMS). My daughter Grace was diagnosed at the age of 15, after nine months of falling, experiencing severe muscle weakness, and slurring her words. One of the most encouraging aspects of our LEMS journey has been meeting other people with LEMS.

This journey can be a lonely one. Meeting someone who knows firsthand what you go through is priceless. After Grace’s diagnosis, I had so many questions. What would her life look like going forward? What types of activities would she be denied because of limitations set by her own body?

So when our youngest daughter moved to Colorado for college, I was determined to meet Julianna Kustelski, the administrator of a LEMS Facebook group and a force within the LEMS community.

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Julianna, a great source of knowledge, experience, and encouragement, is often the first point of contact for many who are joining the LEMS community. She has lived with LEMS for a long time yet maintains a positive attitude that sets an example for us all.

Grace and I met her on a hot August day in Denver, Colorado, in her beautiful downtown apartment. Excerpts of our conversation follow.

LD: How long have you had LEMS, and what symptoms led you to seek medical help?

JK: I was diagnosed in April 1993 after eight months of symptoms. What led me to seek medical help were the constant falls.

What treatment has worked for you?

I’ve been on Firdapse (amifampridine) or 3,4-DAP [3,4‐diaminopyridine] since 1995. Up until then, it was plasmapheresis and IVIG [intravenous immunoglobulin]. I now have Rituxan [rituximab] infusions every six months, IVIG monthly, and Firdapse [amifampridine] and Mestinon [pyridostigmine]. I also take CellCept [mycophenolate mofetil] daily.

What does a typical day living with LEMS look like for you?

It starts with taking meds first thing in the morning to get going. I keep a pretty good schedule with my Firdapse during waking hours and Mestinon with my meals.

What adjustments have you made to continue doing the things you enjoy?

The most important adjustment is managing stress and keeping a positive attitude. Because I’m in a power chair, I’ve had to make a lot of adjustments as far as where I can go. My favorite thing to do is just ride in my chair outside and go for a long distance.

Finding accessible restaurants and other venues is always a challenge.

I keep in touch with family and friends mainly through FaceTime and social media, because I don’t travel well with my chair. One of my biggest challenges has been when I recently broke my right knee and lost my ability to do transfers. I’m now in and out of bed using a Hoyer lift.

What has surprised you the most about living with LEMS?

The waxing and waning. Some days, I feel almost normal in my mind until I try to do anything. There are good days and bad days, but LEMS never lets you forget you are sick.

If you could send a message to your newly diagnosed self, what would you say?

Three things: Faith, family, and friends will be your strength when you have none to give.

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I know Julianna has been an encouragement and support to so many members of our community. She serves as a founding board member of the Lambert-Eaton LEMS Family Association and works every day to better the lives of those living with LEMS.

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 myasthenic syndrome.