Patient Story - Debbie Krause

I welcome you to my story just as the BEBRF welcomed me☺. They continue to inform and support me in my newer lifestyle. I am an analytical people person. I’m hoping my journaling is helpful and inclusive. In 2018, my eyes seemed to feel dry and irritated. I was working as a pediatric Respiratory Therapist at University of Wisconsin Hospital in Madison. I started trying saline drops, then preservative free eye drops 4-10 times/day, and eye gel at night. (At times, I used Refresh® eye drops whose main active ingredient is carboxymethylcellulose sodium. Currently, I use Systane® products whose main active ingredient is polyethylene glycol. This became one of my first frustrations with doctors saying just try products to see what works for you. This became a foreshadowing of my understanding that not everything works the same for everyone, and I have a rare condition.) In November 2018, I saw my eye doctor and was started on Restasis®, and oral fish oil. In May 2019, with severe eye blinking, I changed to Xiidra® and had eye (punctal) plugs placed. In August/September of that year, I had bigger punctal plugs placed since the last ones fell out, and I retried Xiidra.

Later in September, I took a vacation, not realizing I was never going to return to work (where I had started wearing sunglasses), and I was happy to try scleral lenses on a trial basis in my ophthalmologist’s office for my diagnosis of extreme dry eye. I had stopped driving since I was functionally blind. I walked into the office on my husband’s arm, using his guidance so I wouldn’t walk into walls. And my photophobia was bad. When I didn’t get quick relief from the trial, the doctor said she thought I had blepharospasm and in 3 days I saw an oculofacial doctor on September 23, 2019, and he scheduled me for Botox® one week later. My symptoms were really progressing, and before I saw him next, I started having facial grimacing. I called his office to report changes and to try and get Botox sooner, and even considered going to the ER. I finally got my first Botox to my eyes September 30th, and it greatly improved my spasming. I could see again! Once the doctor saw my grimacing, he told me he thought I had Meige syndrome, and he referred me to a neurologist in Movement Disorders.

After many tests to rule out other conditions, and several appointments with my Madison neurologist, she said my condition was progressing quickly. I now was also having more jaw and neck movements where my head wanted to tip down and to the left. I continued Googling on the computer as much as my eyes allowed. My husband, family, and friends were all very concerned and supportive. Getting other specialists involved was like pulling teeth. I found I had to be persistent and be my own advocate, and I found getting beyond the nurses to the doctors can be difficult.

In autumn 2019, I had shortness of breath (I’m still wondering if this was/is diaphragm spasming) for which I went to the ER three times. I lost 10 pounds (due to spasming and need for increased calories). Eating became more difficult. I begged and finally saw a speech therapist specializing in swallow problems and an Ear, Nose, and Throat specialist (ENT). Something wasn’t right/diagnosed, but it felt good to talk with an experienced speech person who did my swallow study. She understood how my inabilities linked with my study. Someone heard, confirmed, and empathized with me! An ENT physician’s assistant shared my appointment notes and speech therapy scoped video with the otolaryngologist, who said I had larynx tremor that he treated with Xeomin®. He said my symptoms and physical manifestations were consistent with Meige syndrome. I now had two doctors who said and wrote Meige syndrome in their notes. My neurologist was clinging to a diagnosis of functional movement disorder due to my quick progression. She also said people with anxiety can be prone to this. My quiet husband blurted out she is not an anxious person! (I was the extraverted person that friends often confided with since I was so chill.) I was able to get a referral to the Mayo Clinic in Rochester, MN, and they saw me Jan 2020.

January 6, 2020, my Mayo neurologist, said “You have Meige Syndrome hands down”. Notable findings: cranial/cervical dystonia including blepharospasm, oral facial (oromandibular) dystonia, cervical dystonia (with 40 degree antecollis and 30 degree left rotational torticollis). “There is no evidence of a functional neurologic disorder.” An EMG also gave definitive evidence of spasming/contractions.

I continue to go to Mayo Rochester which is about 3 hours from Madison WI every three months for Xeomin injections. My eyes have done well in response, which I am extremely grateful for. Eye doc says my eyes are no longer dry (but I continue with Systane eye drops/ goggles at night, as needed drops/warm eye mask during the day). We continue to tweak Xeomin amounts and locations. I still have some head pull to left (and this unpredictability especially keeps me from driving), left trapezius hypertrophy, some head bob, newer left arm movement, lip pursing, uncoordinated/minimal