Spinal muscular atrophy (SMA) is a neuromuscular condition that can cause progressive muscle weakness. Some people with SMA can stand and walk with minimal assistance, but others cannot maintain a seated position on their own. Luckily, a wide variety of technology has been developed to help people with SMA be as upright as they are able to be. That is important because there are many health benefits to spending time upright.
Your care team, including occupational therapists, physical therapists, orthotists, assistive technology specialists, and other clinicians, can guide you toward the technology best suited to your — and your family’s — unique needs and abilities. They should also be involved with the fitting of new devices, and they should periodically check how your older devices fit. (This is because poorly fitting devices can cause discomfort, pain, or even permanent damage over time.)
It is also important to remember that if you have spent years without standing, you should ask your primary care provider for an osteoporosis check before attempting a new standing regimen. Why? Knowing may help you prevent fractures.
It’s also worth mentioning that all caretakers involved with someone who is physically dependent, like many people with SMA, should always ask for help before doing anything they are not entirely confident doing.
The human body has evolved over millennia to operate in an upright position for most of our waking hours. Whether sitting, standing, or walking, we are healthier when we are upright and can interact with the rest of the upright world.
Being upright — especially in a standing position for a dedicated amount of time daily — has been shown to:
Given the many benefits, it is therefore recommended to use all assistive technology available to help and encourage anyone living with SMA to spend as much time in an upright position as they are able to comfortably tolerate.
Upright standing devices can help maintain bone health by assisting people with SMA to bear weight. Standing and bearing your own weight uses many muscles simultaneously, something that people with SMA have varying degrees of ability maintaining.
While there are no set guidelines for how much time anyone should be encouraged to stand, expert opinions range from one to two hours per day, three to five days per week, up to the same for seven days per week. Of course, no one regimen suits all people, so any regimen you adopt should be uniquely tailored by your professional health care provider to match your abilities and personal situation. Because some people with SMA can tolerate standing for longer periods and others can tolerate shorter stints, the standing program that gives you the maximum quality of life and the most health benefits is the right one for you.
Parents of children with SMA are encouraged to use strategies to maximize their child’s enjoyment of standing. For example, pairing standing time with video games and other forms of play, eating meals, or watching movies can be enticing combinations.
The most simple kind of assistive device for standing is an upright (or vertical) stationary stander. As the name implies, this device is not meant for forward, backward, or sideways movement. A stationary stander simply helps you remain in a fixed position comfortably. Given that, this device is best suited for people with considerably limited or poorly controlled mobility.
Many stationary standers incorporate casters or wheels to better move the device from room to room, but caretakers should never attempt to move a stationary stander while someone is standing in it. A braking system, if available, should always be engaged while you are using a stationary stander.
To be in the supine position means you are lying on your back. A stander with a feature enabling a supine position allows its user to tilt backward into that position. This feature can relieve some of the weight from your legs, if you become fatigued or cannot support your full body weight. A stander with this feature is usually the easiest choice to get positioned in the device, because you can do so lying down.
To be in the prone position means you are lying on your stomach. A stander that supports the prone position allows you to tilt forward. This can aid children with SMA who are learning to support their head effectively, shift their weight from one leg to the other, or tackle activities at a desk or table. It may be difficult for a parent to position their child in this kind of device on their own, as it often requires the person with SMA to be positioned while standing.
Dynamic standers are propelled or self-driven. Sometimes referred to as “gliders,” these devices assist in walking. They allow you to flex, extend, stretch, and push yourself around a space by your own strength.
As such, gliders can provide a deep sense of ability, resourcefulness, independence, and self-esteem by allowing you to help with daily chores and activities at work or school.
Sue, parent of a child with SMA, told Quest Magazine about the boost to her daughter’s confidence that her dynamic stander gave her in 2008: “She’s on the same level as the kids when she’s standing. Nobody’s looking down at her, so her confidence level is up, too.”
Sit-to-stand assistive devices are generally used by children and adults who are relatively physically able. Some people with SMA can transfer themselves into these kinds of devices, and are effectively enabled to stand independently. Sit-to-stand devices allow you to be positioned and secured into a seated position, and then the device uses a hydraulic or powered system to lift you into a standing position.
This kind of device requires good torso and head control — many of them do not provide head and neck support — so it may not be appropriate for all.
Wheelchairs with an integrated standing assistive feature offer people the most independence, but these are also the most expensive assisting device. This kind of device is usually a power wheelchair with a built-in lift, which, when activated, raises you into an independent standing position.
Some people with this kind of assistive technology can do many, if not most, daily activities, including cooking, eating, cleaning, and even dancing.
Insurance coverage for this kind of medical equipment varies by country, state, carrier, and your individual policy. Your health care team will likely be advocating for you or your family’s needs with your insurance carrier, with a series of requests and appeals. They will likely be required to provide documentation outlining the specific benefits that you or your caretakers would experience with this device. You can make this documentation compelling by sharing concrete and specific examples of the ways in which the device would help. For example, examine whether the device would:
Families should also reach out to all local and national supportive agencies which may be able to help with the funding or loaning of a device. Some examples of agencies include:
On mySMAteam, the social network for people with SMA and their loved ones, more than 1,300 members come together to ask questions, give advice, and share their stories with others who understand life with SMA.
Do you use one of these devices? Have tips or tricks to share? Share your experience in the comments below, or start a conversation by posting on your Activities page.