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A Clinician's Perspective on ALS: An 80/20 Strategy

A clinician's perspective on ALSSeating and wheeled mobility clients are as unique as snowflakes. Each client has unique goals, activities, and priorities… and their functional presentations vary widely, even if formal diagnoses are the same.

That individuality impacts their seating and wheeled mobility. It’s a common Complex Rehab saying that no two wheelchairs are exactly alike. Even when the power wheelchair base is the same, how various components are positioned and dialed in makes every system unique unto itself.

The need for unique power wheelchairs would seem to be a given for clients with amyotrophic lateral sclerosis (ALS). The progressive degeneration of motor neurons that enable voluntary muscle movement eventually robs clients of the ability to walk, talk, swallow and breathe. ALS would seem the perfect example of a condition that needs a “snowflake” seating and mobility approach.

But what if you found enough commonality among ALS clients to home in on the options they most frequently require on their power chairs? If you could anticipate their needs, could you simplify the building and provision process — and get the optimal equipment to these clients faster?

It’s a process that Steve Mitchell, OTR/L, ATP, refers to as creating ice cubes instead of snowflakes.

A Presumption of Service Connection at the VA

Mitchell is an occupational therapist who works as a Clinical Specialist in seating/wheeled mobility and assistive technology for the Spinal Cord Injury & Disorders Service at the Cleveland Veterans Affairs (VA) Medical Center.

As one of “just a handful,” he said, of VA therapists who are fulltime seating specialists, Mitchell focuses exclusively on veterans with spinal cord injury/disorders (SCI/D) and veterans with ALS. In 2016, he wrote a series of Mobility Management articles on the concept of “Open CRT” — explaining that he’d found enough similarities among his ALS clients to see patterns not just in the seating and mobility they needed immediately, but what they were likely to need as the ALS progressed.

In talking with Mobility Management this time, Mitchell expanded on his “80/20” approach.

His specialization significantly narrows the range of clients he sees. “The veteran I’m working with is typically going to be a male,” Mitchell said. “They’re going to be roughly 6'0" tall, give or take 3" or 4".” Their weight, he added, usually falls within the same range, “give or take 20 or 30 pounds. We see some female veterans, but we don’t see that many.”

Because most of his clients fall into a relatively narrow size range, “I don’t have to worry about the size of the seat, and there’s enough modularity in today’s designs where you can make those adjustments. So I can really home in on the details. That’s where the big-picture success is, in execution of the details. And there are so many characteristics that my populations have in common that I can really home in on those and address those.”

One fact is well known: On its Web site, the ALS Association states, “Military veterans, regardless of the branch of service, the era in which they served, or whether they served during a time of peace or a time of war, are at a greater risk of dying from ALS than if they had not served in the military. For reasons as yet unknown, veterans are twice as likely to be diagnosed with ALS as the general population.” Multiple studies affirming the higher ALS risk for veterans led the VA to implement, in 2008, a “presumption of service connection” for ALS. That declaration “presumes that ALS was incurred or aggravated by a veteran’s service in the military.”

At that time, Mitchell was predominantly working with veterans with SCI or advanced multiple sclerosis. “But once [the ALS presumptive service connection] became part of the equation, we started our multi-disciplinary ALS clinic. We were the first VA to become a Certified Clinical Center of Excellence by the ALS Association.”

Seeing the Patterns

In focusing on this relatively narrow band of ALS clients, Mitchell saw patterns emerging.

“Somebody with a spinal cord injury is going to be relatively stable,” he explained. “If I accomplish my goals as an occupational therapist and dial in the [wheelchair and seating] configuration for optimal function — it inherently requires a lot of attention to detail. It’s a snowflake, of course. But once I had it dialed in, it was pretty stable.”

That wasn’t the case with ALS clients.

“What I began noticing is that veterans with certain functional presentations of ALS tended to experience many of the same problems,” he said. “I also realized that many of these problems weren’t going to die with the first user who had experienced them. I would see them again and again.” Many of these problems were directly related to gravity. Finding ways to effectively mitigate the effects of gravity on a user’s posture, positioning, and function is a continual challenge for any seating clinician who works with this population.

“As I came up with effective solutions to these problems in my clinic, it didn’t make sense to have to re-create the same configuration from scratch for every veteran who shared a given presentation.” Instead of delegating the job to the DME supplier he worked with, Mitchell looked for a more efficient process. “I began sending pictures to the manufacturers to see if they could replicate a given solution as a custom option,” he said. “And that’s evolved nicely over the years. Our ALS power chairs now come with a modular, adaptable infrastructure that allows us to really dial in the configuration so that it effectively meets their clinical and functional needs. As those needs change, the same options will allow us to reconfigure a chair so that the veteran can continue using it for as long as possible.”

The 80/20 Strategy

Although Mitchell initially developed the custom options to address the most frequent issues he saw in his clinic, he also encountered more challenging issues in subsets of veterans who shared specific functional presentations of the disease. This led to what he refers to as his “80/20” approach to ALS power chair configuration.

“Without getting too technical,” he explained, “I have found that nearly every veteran who could use a conventional joystick during their initial evaluation continued to be able to use a joystick for as long as they had their chair. Only 20 percent of the veterans we followed with ALS needed to use alternative controls. What’s more, we were fairly successful at predicting which veterans would need these systems at the time of their initial evaluation. While there have been some exceptions, having this knowledge has allowed me to develop two different types of ALS custom configurations: one to effectively meet the needs of the majority of veterans who have ALS, and another which has been optimized for the important subset of veterans who needed alternative driving controls.”

Mitchell has discovered a simple test has been a fairly accurate predictor of which configuration is likely to work better.

“I have them fully tilt back in the chair. If they have enough function against gravity to easily reach the end of the joystick, they’ll probably belong to the 80-percent of veterans who will continue using a joystick. If not, they would be a better candidate for my Hybrid Alternative Driving System (HADS) configuration.”

On his “80-percent” chairs, proper joystick positioning is key. “As a rule, you want to provide a ‘low, level and inline’ joystick configuration,” Mitchell said. “These users tend to do best when the joystick is located directly in front of the armrest — no higher than 2" to 3" above the surface of the arm pad. The armrest itself should be parallel to the seat surface at the back angle they need to breathe comfortably and have postural stability. Clinically, the objective is to minimize the need to use anti-gravity movement by providing uniform support along the entire length of the forearm. The armrest and joystick are basically configured so that if someone has full range against gravity, but can’t tolerate resistance, they will still be able to operate the joystick and power seating functions.”

For the remaining 20 percent of veterans, Mitchell said, “Their ALS typically affects both upper extremities first. Most will have very good lower-extremity function. They may also have fairly good head control or oral motor function that can be taken advantage of. These individuals tend to progress a little more slowly, which is good because their chairs typically take a bit longer to configure.

“Oftentimes, these users will start out using a compact joystick with the same low, level, and inline configuration. Some may continue using the compact joystick for some time. A few may never go on to use a hybrid system, but should we need to make that transition, that infrastructure is already there.”

Instead of using head arrays or other typical alternative controls, Mitchell has combined his clinical understanding of ALS with his years of providing conventional systems to develop a somewhat unconventional approach to providing alternative driving controls.

“To receive a diagnosis of ALS, there must be signs of upper and motor neuron dysfunction in three out of four segments of the nervous system,” he explained. “There’s probably not a single body part that’s left unaffected by the time they come to my clinic.

“Conversely, conventional alternative controls generally require very good use of a single body part. To effectively meet the needs of these users, we need to be able to customize systems to take advantage of whatever function they have. Implementing such systems can be done most easily by using a simple switched driving interface, a power seating switch interface, and a simple network of color-coded extension cables. If this infrastructure is already on a power chair, it becomes possible to implement a Hybrid Alternative Driving System with far less time, labor, and technical expertise than would otherwise be the case. It’s simply a matter of finding the right combination of switches.”

And regarding mini joysticks: “I have had a few people with ALS [for whom] I’ve used mini joysticks, but I think those tend to be the most beneficial for people who have muscular dystrophy and other conditions that affect distal movement. If the muscles can’t overcome the weight of the arm against gravity, taking a couple of ounces off the amount of effort required to move the joystick isn’t going to make a world of difference. So we normally prescribe a compact joystick with the same effort that the [standard] joystick has. It generally works pretty well.”

What Ice Cubes Can Accomplish

There are big upsides to building adaptable wheelchairs.

“One of the benefits is it’s making an ice cube instead of a snowflake, so you can adapt it as the illness progresses,” Mitchell said. “It’s one of the things that’s been really helpful and allows us to serve the needs of veterans better.

“Anytime we provide equipment to a veteran, it’s theirs to keep. But if it’s in good condition and we do get it back, those options that made it easier to configure for their needs also make it easier to reconfigure for another veteran. So we tell our veterans that up front, and if we do get a chair back that’s in good condition, sometimes we can quickly reconfigure and reissue it, and save the person the time that’s usually required to order something new.”

Keeping It Simple

Mitchell acknowledged that the 80/20 approach doesn’t fit every ALS client. “They’re not all the Cleveland VA custom configuration, these ice cubes that I do,” he said. “We do get people that are going to be exceptions. And then we might go more along the traditional Complex Rehab route, where it’s more like a spinal cord [injury] chair, especially if we know that their condition’s progressing pretty slowly and they’re younger.

“Others who unfortunately progress more rapidly are more urgent as far as giving them something. That’s where reconfigured chairs are helpful. Odds are [ALS is] probably not going to all of a sudden plateau to where their condition’s not going to change.”

While Mitchell’s approach is different from what you might expect for ALS clients, he said his approach is actually simple.

“Even for the 20-percent chairs: Those are going to take a little bit more work, but even then, I’m using mechanical switches,” he said. “I’m not using sensors. I haven’t done a head array in probably 10 years, and I’m getting good outcomes.

“These are really easy solutions. It’s custom, but not that complicated. It’s simple. It’s been so rewarding.”

Editor’s note: Steve Mitchell’s opinions do not constitute an endorsement of any specific product, nor do they represent the official policy or positions of the Department of Veterans Affairs.

This article originally appeared in the May/Jun 2022 issue of Mobility Management.

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