Cognitive Considerations for Clients with ALS
Interview with Lois Brown, MPT, ATP
Amyotrophic lateral sclerosis (ALS) makes us think of loss of muscle control that leads to paralysis and respiratory difficulties. But research indicates that ALS also frequently impacts cognition — which can impact an ATP’s equipment decisions.
Cognitive Impact Is Common
In a presentation earlier this year, Lois Brown, MPT, ATP, rehab clinical education specialist for Invacare Corp., noted that cognition is affected in 49 percent of ALS patients. Memory, emotional responses and other behaviors can be impacted.
In an interview, Brown suggested that clinicians and providers should be aware of potential cognitive involvement while working with clients.
“I think that when therapists and the providers are working through a detailed process of evaluating and recommending the equipment, you can observe the patient for some of those behaviors,” Brown said. As an example, she cited difficulties “in following one-step, two-step or multistep commands.”
She explained, “If I’m considering a complex setup with switch access so that the patient can be independent in driving and other wheelchair functions such as power positioning and speech/computer access, but I’m finding that I’ve shown them the sequence two or three times and they’re not following the commands to access the functions independently, then maybe that is telling me I need to simplify how they access those features.”
Brown said she also pays attention to emotional reactions.
“If they become immediately frustrated, I might ask their caregiver or spouse: ‘Is that typical behavior, or is this something that’s different from what you might have noticed before?’”
ATPs can share their observances with the rest of the healthcare team.
“I’m not saying that any of us should presume to make any kind of a psychological or cognitive diagnosis,” Brown said. “But when noticing those kinds of symptoms, it’s a good time to alert the clinic for the followup and possibly have a speech or cognitive therapist or psychologist follow through with some testing.”
Seating & Mobility Customizations
As with any other client, Brown said she absolutely wants ALS patients to “have every option available to use, in any position or in any way that they can function.” But cognitive involvement can complicate the situation.
“Just because we can integrate all the features doesn’t mean we should if the cognitive demand is too high,” she said. “For instance, the individual may have one input type for driving and a separate toggle for power positioning or the communication device. Just because I can integrate them, it’s not necessarily the best thing for the individual. It complicates things, and we negatively impact their quality of life.”
The situation gets more complex for clients who have limited movement, but for clients still capable of hitting a separate switch and immediately activating tilt or recline, that option may be more manageable than having to first go through the driver control, Brown said.
While “a significant number” of ALS patients die within the first few years of being diagnosed, Brown pointed out, “the individual’s needs are constantly changing, and therefore we need to be able to continually respond to not only their physical changes, but their ability to utilize the features for as long as possible to maximize their quality of life.”
Editor’s note: Stay tuned for part 2 of this Clinically Speaking installment on ALS considerations for the ATP.
ALS “Common Cause” Is Discovered
Researchers at the Northwestern University Feinberg School of Medicine have identified what they say is the cause of all forms of amyotrophic lateral sclerosis (ALS).
An August report by the university describes the cause of ALS as “a broken-down protein recycling system in the neurons of the spinal cord and brain. Optimal functioning of the neurons relies on efficient recycling of the protein building blocks in the cells. In ALS, that recycling system is broken. The cell can’t repair or maintain itself and becomes severely damaged.”
Teepu Siddique, M.D., Northwestern Feinberg School of Medicine, said of the discovery, “This opens up a whole new field for finding an effective treatment for ALS. We can now test for drugs that would regulate this protein pathway or optimize it, so it functions as it should in a normal state.”
Siddique expressed hope that this discovery would also impact the understanding of other neurodegenerative diseases, including Alzheimer’s disease, frontotemporal dementia and Parkinson’s disease, “all of which are characterized by aggregations of proteins.”
The report added, “This breakdown occurs in all three forms of ALS: hereditary, which is called familial; ALS that is not hereditary, called sporadic; and ALS that targets the brain, ALS/dementia.”
Northwestern University researchers also found a new gene mutation present in both ALS and ALS/dementia, “linking these two forms of the disease.”
This article originally appeared in the October 2011 issue of Mobility Management.