ATP Series

The Land of the Quarter-Inch

Clients with Duchenne md often resist change. Here's how to introduce successful drive control options.

• By Haley Samsel
• Jun 01, 2020

Ask clinicians or Assistive Technology Professionals (ATP) who have worked with boys with Duchenne muscular dystrophy (DMD), and they will likely tell you a similar story: This patient population is notoriously resistant to changes in their mobility equipment, including driving controls and seating.

Behind that opposition is a complex condition caused by the failure of the X chromosome to make a necessary protein called dystrophin. DMD primarily presents itself in boys, who have one less X chromosome to work with than girls.

While boys with Duchenne often show signs of normal development in their first few years of life, their shoulders, upper arms, hips and thighs are typically the first muscles that are weakened by DMD, according to the Muscular Dystrophy Association1. The progressive nature of the disease leads most Duchenne patients to die in their mid-20s, with some men living until their mid-30s. This is a significantly longer life expectancy compared to expectancies for prior generations of patients with Duchenne MD.

DMD patients will typically require a manual chair to travel long distances before moving to a power chair as they enter their teenage years. By the time they enter high school, Duchenne boys have been through years of assessments and are experiencing the realities of muscle fatigue and atrophy, as well as cardiac and respiratory issues.

During those teen years, many families have found mobility solutions that work for their kids. In turn, patients and parents alike are concerned that newer technological options and alternative driving controls may not give them the same results.

Lisa Rotelli, the Director of Adaptive Switch Laboratories, said that Duchenne
boys are often misunderstood because they do not want to speak up for themselves during an assessment, even if their current mobility system is uncomfortable or inefficient because they have been told what they need over and over again.

“This population is very near and dear to our hearts because I think they are misunderstood in many ways,” Rotelli said in an interview with Mobility Management. “When you make big changes on them, they are absolutely not going to like you, and sometimes when you make recommendations as well. Their first word is no, and I don’t blame them because they can’t trust the outcome. They have things set up that work for them, and when I make a change, it may mean that nothing will work for them.”

But the difficulties of convincing patients and their families to try out a new system does not mean that clinicians and ATPs should give up on providing new technology to this population, said Gabriel Romero, VP of Sales & Marketing for Stealth Products.

“The thing that I notice the most with Duchenne’s patients is that they want to move, they want to be independent,” Romero told Mobility Management. “What I appreciate the most is breaking down barriers with them and building that confidence and showing them that there is stuff they can do. They knew they could do it, but they just didn’t know the technology or needed insight on how to do it.”

Starting with a Standard Joystick

Approximately 15,000 boys in the U.S. are living with Duchenne, with about 300,000 cases worldwide, according to the research and patient community nonprofit CureDuchenne2. If those boys have access to healthcare and complex rehab technology (CRT) providers, they will usually be in a wheelchair full-time by their teens.

In most cases, boys will start with a standard joystick thanks to their cognitive and physical abilities. However, Romero said that he will not always start DMD patients on a standard joystick, particularly if it’s the first time they are being assessed for a power chair.

“Everyone wants to start with a standard joystick, but there’s a lot of complexity,” Romero said. “It doesn’t mean that I won’t get to a standard joystick very quickly.”

While Duchenne boys pick up the steering and speed mechanisms at a fast pace, Romero will sometimes allow the user to focus on steering and set up speed settings on the chair with a switch until the patient is ready to move on to the standard joystick.

“If I can see that they can already do left, right, forward, then they could pick up a joystick very quickly,” Romero said. “But
don’t sacrifice the process of having successful moments and putting something there that they could struggle with and start
to develop biases against just because it wasn’t set up properly.”

After starting most Duchenne patients with standard joysticks, Rotelli said it is crucial to remain cognizant of how to adjust the system to their changing needs. Mounting the joystick or alternative driving control in the right place is always important, but especially with a population that has limited hand dexterity.

Typically, she leans toward light-touch joysticks and fiber-optic options that require less force and range of motion since the boys live in the “land of a quarter inch,” as Rotelli puts it.

“A quarter-inch difference makes all the difference to them,” Rotelli said. “I have worked with many, many patients that can drive by just moving their joystick forward and maybe to the right or left. The funny thing is, if you ask them, if they are just going forward and making left turns and that’s all they can do, and somebody has to reposition their hands to do right or reverse, they’re totally okay with that.”

Do’s and Don’ts for Duchenne Tech

As the disease progresses, boys will begin to exhibit cardiac and respiratory issues on top of their muscular atrophy, making it even more difficult for CRT professionals to find a mobility solution that patients can accept and integrate into their daily lives.

The issue is not a fear of change, but how young men develop habits to make their lives run more smoothly, Rotelli said. In addition, more advanced Duchenne patients who are headed to college and building careers feel psychologically opposed to adopting digital systems because it can serve as a confirmation that their condition is getting worse, Rotelli said.

“I try hard to show and say: Look at all these things you can do with these switches or look at all the things you can do with these different joysticks,” Rotelli said. “We have got to understand that whatever they are using to control their chair, they’re also going to be using to control technology — computers, laptops, phones, games, everything.”

Installing a system of sensors, fiber optics or electronic switches that do not require sustained pressure is the next step in the patient’s journey, according to Romero. Stealth offers precision mini proportional joysticks, including an i-Drive version that can be programmed through the PWC programmer or separately through i-Drive software on a computer or tablet.

“We have to understand during the assessment process: Can they use the joystick to the full capacity?” Romero said. “Is this just the reality of their usage? Or can we give them something more simple, something easier for them, that they could use more abilities and have more independence?”

In addition to introducing the technology, it’s crucial that parents understand what the technology can do and that they feel that they have some control over their child’s safety and mobility, he said.

“The moment parents feel out of control, that demo chair is back in the garage until they can get back to the clinic,” Romero said. “They’re not trialing it anymore, and the child is not getting to practice with it.”

For Rotelli, working effectively with Duchenne patients is also a matter of determining what technology will not work for their condition. She is not a fan of using head switches because they can quickly lead to weakness and fatigue in the necks of some boys. In the past, Rotelli has also heard suggestions that younger Duchenne boys should consider molded seating systems.

“That is 100 percent the wrong thing to do,” Rotelli said. “Number one, it’s harder to breathe, and number two, it’s also harder for access even for a joystick. I lose function the more that something touches my body and pulls me in one single place. It’s harder for me to use my hand.”

At the same time, she has also fielded clinician suggestions to not install tilt and recline on a patient’s chair — a recommendation she typically rejects because of the importance of “micromovements” for Duchenne patients.

“If I have tilt and recline on a chair and possibly some anterior [forward] tilt, you can show them how they can make those micromovements themselves,” Rotelli said. “Just bring the back up a little bit, slide their hand forward, and then they can use it.”

Several patients she knows have used tilt and recline to move their heads off their headrests and sit upright as well as to rest their heads back onto the headrest. These seat features mimic muscle control and help with energy conservation, particularly for boys who spend long lengths of time in their chairs to play video games, use their phones and move around independently.

Listen, Trust, Repeat

It’s the quality that makes great ATPs great3: the ability to listen and gather a patient’s history, allowing them to air grievances with previous seating systems or explain why they are anxious about using a different driving control. Both Romero and Rotelli said it was especially important to hear out Duchenne patients who would benefit from greater independence, but do not want to change what’s already working for them.

“They’ve seen so many people, from hospital visits to clinical rehab to outpatient visits to normal school,” Romero said. “They don’t know you from anything, and so you’re building that confidence and that trust with everyone. You don’t know the experiences they’ve had — I could be the guy coming in with a joystick when the last time they did something with a joystick, they ran into a wall.”

Romero has had better luck convincing families to switch to a new system when he explains how the patient is only able to functionally use their current system 80 percent of the time due to muscle fatigue or other issues.

“As a parent, you need to understand that there are fatiguing things that are happening, and they need to rest and relax,” Romero said. “But if I can install something that they can use 100 percent of the time because it’s allowing them to use two parts of their body instead of using one and only one, there are major differences in their independence.”

Almost every Duchenne patient Rotelli has worked with since her first in 1986 has been opposed to any change. Some have been very adamant about it, requiring extra time and patience to improve their mobility through a drive control or seating system they could accept.

Focusing on what the boy wants to do — change a TV channel, play Xbox, use their phone — and how a system can get him to that point is the most effective way of overcoming their initial frustration.

“If I can do this and you have four directions instead of two, then we have just quadrupled your output to play games and use computers and text on a phone,” Rotelli said. “That’s usually easier to get them to buy into than changing their mobility. Then I will say: I also believe this is an easier way for you to drive your chair.”

Reaching this patient population and transitioning them into the next phase of mobility solutions requires a willingness to listen and resist categorizing patients by diagnosis alone, Rotelli added.

“It’s their chair. It’s something they have to use and live in, and any change is going to be very, very hard,” Rotelli said. “You have to build a relationship with them, and once you do and have that trust, then they can tell you what they do and don’t like.”

This article originally appeared in the Jun/Jul 2020 issue of Mobility Management.