Primary lateral sclerosis is a rare, debilitating neurodegenerative illness that is on the spectrum of motor neuron diseases. It is a disease without a cure and without disease modifying therapy. Current research in PLS has been sparse. Many of those with PLS find it difficult to overcome barriers to care and to receive appropriate symptomatic therapies. Here is the story of one patient. At Synapticure, we hope that opening our doors to those living with PLS will help change this narrative.
This is a story written by Sherry Quisenberry, who was diagnosed with PLS ~4 years ago. She lives in Atlanta with her family.
“With PLS, there are no drug trials for us, no treatment, little to no understanding of the disease from ALS specialists, and very few studies. Estimates suggest roughly 400 U.S. citizens have PLS. I am in contact with 10 of the 400 here in metro Atlanta alone. If this estimate is correct, the reality is that I and many others have upper motor neuron dominant ALS or HSP of which the causative gene is unknown at this time.
I began having symptoms 6-7 years ago. Slowly, I had to abandon running, long distance biking, and tennis. My “probable PLS or UMNd ALS” diagnosis from an Academic Medical center in Georgia didn’t come until 4-1/2+ years ago, much too late to be eligible for any drug trial. I go to an ALS clinic, am seen by an ALS multidisciplinary team, and receive ALSA’s mailings and emails. We’re treated as if we are classic ALS patients, and we are told that it is more than likely ALS. Once a person is diagnosed with probable PLS, there is a waiting period of 4 to 5 years before it is confirmed. The “waiting period” includes frequent rechecks to assess if the lower motor neurons have become involved, the rate of the disease progression, and if new symptoms have occurred. The average PLS patient is typically denied durable medical equipment, disability benefits, employment accommodations and much more. My multidisciplinary team finally gave me an ALS diagnosis on paper to eliminate the obstacles.
Unfortunately, many in the PLS community don’t have neurologists who will do this or who will prescribe drugs off label or make any accommodations whatsoever because on paper it says PLS. There are PLS patients who eliminate neurology visits entirely because they receive zero help. This is where Synapticure could be of great help to us.