Alisa Brownlee, ATP, has personally heard it multiple times during her long tenure at clinics specializing in amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease.

Already dealing with a devastating diagnosis, family members aren’t always prepared to hear that in addition to progressive loss of muscle control, ALS often also impacts a patient’s cognition — for instance, the decision-making processes, and how patients think about the people around them.

“They will tell us,” Brownlee said, “‘Okay, he’s got ALS, but you can’t tell me he’s got anything else going on. Because that’s enough.’ So they’ll bury the cognitive component.”

Nevertheless, researchers are finding that cognitive involvement for patients with ALS is much more common than previously thought. And healthcare professionals already know that cognition can be affected with such diagnoses as multiple sclerosis — though in very different ways.

Therefore, knowing what to expect from and how to best serve any seating & mobility client with dementia is a challenging task.

Defining Dementia

The mainstream public most commonly associates dementia with Alzheimer’s disease, which, despite early onset types, is itself most commonly associated with advancing age.

In reality, dementia simply refers to impact on a number of cognitive abilities and skills.

The Mayo Clinic says, “Dementia describes a group of symptoms affecting memory, thinking and social abilities severely enough to interfere with daily functioning. Dementia indicates problems with at least two brain functions, such as memory loss and impaired judgment or language, and the inability to perform some daily activities, such as paying bills or becoming lost while driving.”

From a seating & mobility perspective, clinicians are still learning which clients are impacted, how and why — and are also investigating why some, but not all, clients are affected.

Jay Doherty, OTR, ATP/SMS, senior clinical education manager, East Coast, Quantum Rehab, said of this ongoing research, “Interestingly, the presence of cognitive impact on those with ALS is a relatively newer discovery, with extensive research occurring as recently as the last two decades. While it was long thought that ALS only affected motor skills and respiratory function, researchers now know that there can be cognitive and behavioral components. The current research shows that 50 percent of those with ALS have no cognitive impairment. However, the other 50 percent have some level of cognitive and behavioral impairment, and 25 percent of that population experiences diagnosed dementia.”

Contrast that with how Doherty describes cognitive impact common in multiple sclerosis (MS) patients: “Cognitive impairment among some people who have MS can manifest in a variety of ways. It can involve memory, problems with planning or prioritizing, attention and concentration, processing sensory information, and other issues with everyday life. As the individual working with a person who has MS, you may need to help them develop strategies in order to overcome some of these cognitive limitations, but we also must look closely at this cognitive involvement and ensure that the person will be safe using their complex rehab technology (CRT). This process may include further training of the individual in order for them to use the CRT safely. As always, involving caregivers in this process to support a repetitive learning process is exceptionally helpful.”

Cognition & ALS

Cognitive involvement in ALS patients is healthcare research’s equivalent of breaking news: So much has been learned so recently, with new information still coming in all the time.

What we know now, Brownlee said, is that while about half of ALS clients do have cognitive impairment, it can be mild, moderate or severe in nature — and researchers aren’t sure why that’s the case, or which ALS patients are more liable to have involvement.

Researchers also know that in ALS, “the portion of the brain that is affected is the frontal lobe, the decision-making portion,” Brownlee said. “If you’re looking at that in terms of, for example, assistive technology, we have people who are impulsive. They have poor decision making or no decision making. They lack empathy. They lack the ability, when it comes to driving a wheelchair, to know safety precautions. So it would almost be like driving a car: You don’t want someone who first of all has problems with reactive time, or problems with judgment, driving any type of vehicle, whether that be a wheelchair or a car.”

Currently, Brownlee added that one of the challenges is the fact that not all ALS patients are undergoing the screenings that could help to identify cognitive issues.

“If somebody comes to an ALS clinic, we screen for cognitive impairment,” she said. “But there are only 46 clinics in the United States. So the significant number of PTs, OTs and ATPs are seeing people who haven’t been to an ALS clinic. And [the seating team] doesn’t know the cognitive impairment, so they’re not necessarily screening for it.”

Unlike the prototypical memory loss in Alzheimer’s patients that can become quickly obvious even to the casual observer, cognitive involvement in ALS patients can be more subtle and harder to detect.

“Even if you do a general cognitive screen, [symptoms might] not show up on your radar unless you ask more explicit questions,” Brownlee said. “So for example, because our folks have problems with executive function and decision making and impulsivity, the caregiver might say, ‘Well, yes, they’re a little impulsive when it comes to this.’ But then we see it come out as people who absolutely devastate a bank account. They go online and spend money crazily. But you wouldn’t know that unless you asked someone, ‘What do you mean they’re impulsive?’ Unfortunately, the other part of impulsivity is we have other people who act out sexually, who touch strangers in places they’re not supposed to be touched. But again, that’s not something you normally [ask about] during a seating eval.”

So rooting out a cognitive component in an ALS situation can be very different than noticing forgetfulness in a patient with Alzheimer’s.

“Our folks don’t have a memory problem, they have a behavioral problem,” Brownlee said. “And that behavioral problem can cause them, when driving a wheelchair, to crash into the wall. Our folks don’t necessarily have issues remembering how to use the joystick or how to engage a scanner in a different mode. But they have problems when they’re in the mode to either put a stop to the wheelchair, to slow down, or not to hit someone. The other thing is our folks lack empathy. So their impulsivity literally might make them run over someone, and they can’t be sorry they did it.”

The possibility of making bad decisions — and being unable to learn from and correct them due to accompanying lack of empathy — can put seating & mobility clinicians and ATPs in a very difficult position as they decide on the assistive technology to recommend and provide.

Safe to Operate a Power Wheelchair?

While it can present in younger adults, ALS typically is diagnosed in middle age, among patients who are raising or have raised families, built careers and are active in their communities. Therefore, the potential loss of independence strikes them and their families very hard.

Often caught in the middle are the clinicians and ATPs who have to balance their clients’ continuing desires for autonomy for as long as possible with the very real possibility that operating a power wheelchair safely and responsibly may not be possible.

“I think the challenge with our clinicians is to determine whether someone is safe to drive,” Brownlee said. “And unfortunately, the crux of the problem is it can come to the PT or the OT to say to the caregiver or the patient, ‘You’re not safe to drive, and I’m not going to recommend this power wheelchair.’ Which of course leads to anger issues or people exploding at the PT or OT that won’t give them permission to drive the wheelchair.”

Even if an ALS client doesn’t act aggressively or impulsively toward others — and Brownlee pointed out that clients often “put on a better face for the clinician than what is happening in the home” — that client could still be endangering himself by, for instance, taking the power chair outside alone and driving it in unsafe locations or in bad weather.

“Instead of running over someone, maybe they would take themselves into the wall and hurt themselves or be out in the community,” Brownlee said. “That’s what is really disconcerting.”

While it’s not always possible to watch how an ALS client acts and reacts over a long period of time, Doherty said observation, when possible, can help seating team members detect potential problems.

“For example, if a client struggles with interpreting or remembering screen functions on a power chair, a simplified way of controlling the different functions of their power wheelchair may be needed or a different form of CRT may need to be considered — an example would be an attendant control,” he explained. “As ATPs and clinicians, we’re trained to have an awareness toward evaluating a client’s cognitive abilities toward using CRT, and such immediate signs as a client’s inability to follow or remember instructions are among key indicators that some level of cognitive impairment may be present.”

Brownlee acknowledged that a diligent clinician who declines to recommend a power chair for an ALS client unable to safely operate it runs the risk of not just alienating the client, but also motivating the client to go elsewhere — to a clinician or supplier willing to provide the equipment without questioning the cognitive component.

A Multi-Disciplinary Approach

In this quest to provide the best support for clients with ALS — particularly ones with tricky cognitive involvement — Brownlee suggested there can be strength in numbers.

That’s again where ALS specialty clinics can help.

“ALS clinics have an interdisciplinary team,” she said. “We have 10 or 12 professionals who make up the team, and the patient sits in the room, and the physicians go in one by one to talk with them. And then at the end of the day, we’ll do rounds. I might say, ‘He’s really saying that he wants to communicate, but he just doesn’t care to communicate.’ And then another person might chime in, ‘Well, you know he cleaned out their bank account.’ And our PT might chime in, ‘He fell three times because he doesn’t want to use the walker.’ So that kind of tells us there’s a pattern.”

Involving family members and caregivers from the start, Doherty said, is also critical.

“Progressed ALS always involves caregivers — whether family members or hired caregivers — and their needs must be considered as well in the CRT process,” he noted. “Even if the client possesses full cognitive abilities, his or her primary caregiver should be equally familiar with the CRT. In the event that cognitive abilities are affected — either in the present or future — the caregiver is then fully prepared to use the needed CRT. Therefore, in selecting and fitting CRT, both the client and primary caregivers should be included in the process.”

“If I take away anything from our caregivers and knowing this cognitive component,” Brownlee said, “it’s just acknowledging to the caregiver that something is wrong. I can’t stress how important that is. So many caregivers don’t understand it: This is not the man I married, this is not the woman I married, it’s not their typical behavior. So we acknowledge it. Maybe they won’t process it, but at least we’re acknowledging it — and we’re saying maybe they’re not safe to drive right now. It doesn’t mean they can’t be. There’s always that hope.”

The assistive technology provider is also in a vital position to observe cognitive difficulties.

“If the suppliers are getting calls all the time — ‘We need to reduce the speed,’ or ‘We need to do this or that’ — they also have to be cognizant that maybe this is not a problem with the wheelchair. It’s a problem with the personality. The PT or OT who does the eval might see [the ALS client] one or two times, but the supplier is going to take over from there, and they’re going to be the ones getting calls for tweaks or modifications to the wheelchair, and they should also know about the issues. Granted, they’re not the people who are going to establish whether [clients are] safe to drive, but they’re going to be ones on the front lines that the families are calling, saying, ‘But I want him to drive.’ So they need to be able to stand up for themselves and say, ‘No, you need to go back to the person who prescribed the wheelchair.’ They need to be able to be comfortable saying, ‘I think you need another evaluation. We can’t do this until you see [the clinician], or I need to talk to the doctor.’”

Equipment such as head arrays or attendant controls for power chairs can sound like perfect solutions for ALS clients, and indeed do help many such clients to preserve independent function. But if clients have cognitive impairment, the situation can get murkier.

“We get a lot of calls about people who, because this is a progressive disease, say ‘Oh, I need a head array,’” Brownlee said. “Our standard protocol [at our ALS clinic] is no, you can’t get a head array until you go back to the person who’s evaluated you. But I’ve also heard other clinics ordering it without them ever being tested. The patient will say, ‘I can’t get back [to the clinic].’ Then the supplier says, ‘I want to do what’s best for them,’ and I get that. It’s a vicious cycle. That puts suppliers in a really awkward predicament. It puts a tremendous pressure on our suppliers.”

As for attendant controls that allow a caregiver to do the power wheelchair driving, Brownlee said funding is a common dilemma. “Because of the medical model we’re under, I’ll say, ‘She can drive the wheelchair with her hands, but she’s not cognitively able to do that.’ Medicare will come back and say, ‘But she can drive the wheelchair.’” And there goes the argument for attendant controls.

The Cognitive Take-Aways

Despite the many challenges of working with ALS patients who have cognitive involvement — or any wheelchair user who does — Brownlee said there are valuable take-aways to be had.

For example, thanks to ongoing research along a number of paths, the cognitive components of ALS are getting more attention, and the healthcare community is learning more all the time.

At December’s International Alliance of ALS/MND (Motor Neurone Disease) Associations event in Orlando, Fla., Brownlee said discussions included the physiology of cognitive involvement — why certain patients are affected while others aren’t, and what makes some people more susceptible. Attendees discussed “ALS-Plus Syndrome,” whose simple definition is ALS plus non-motor symptoms. An April 30, 2014, story on ALS-Plus Syndrome in Neurology, the journal of the American Academy of Neurology, described ALS-Plus symptoms as including such diverse conditions as gaze abnormalities, excessive sweating, loss of taste and smell, apraxia and dystonia.

“Why will some people get ALS-Plus, and some people don’t?” Brownlee asked. “There’s a whole bunch of genetic dispositions. We can’t say, ‘Of Joe, who was diagnosed today, and Ron, who was also diagnosed, which one of you will have a genetic component, if either of you.’ So they’re really trying to figure out why, and there’s a lot of studies going on across the United States and the world to figure this out.”

Attendees also discussed sharing information and the different cognitive screenings being used on ALS clients, including the Edinburgh cognitive screen.

“Not that we all have to get in line, but we’re all trying to share information so we know who’s doing what and getting the best results,” Brownlee said. “That’s where we are at this point, eight or nine years into the cognitive involvement. We’re still all figuring it out.”

At this still early stage, the greatest take-away may simply be to be aware of how common cognitive involvement is, so clinicians and ATPs know what to look for. When cognitive impact makes independently driving a power chair impossible, the seating team can be ready to offer options, such as manual mobility that will still enable repositioning for comfort and weight shifting. But for the client’s best interests, seating professionals should also be ready to say no when needed.

“Call your local ALS chapter,” Brownlee said of pulling in other resources. “Call the neurologist, ask the questions when something doesn’t feel right here.”