Mobility

Mobility can be a challenge for people with SMA. Specialized equipment, such as adaptive strollers, wheelchairs, and even robotic devices, can help to optimize their care and maximize their independence. A practitioner experienced in working with people with SMA can help to choose the specialized equipment that is best suited to an individual’s need, as well as assist with incorporating them into daily life.

Adaptive Strollers

Also called medical strollers, adaptive strollers allow a child to be supported and positioned comfortably. They are usually lighter and more portable than wheelchairs and can easily be loaded in and out of a vehicle. Accessories can also help with comfortable positioning and posturing, or with carrying medical or other equipment. Adaptive strollers are usually used until a child is big enough for a wheelchair.

Wheelchairs

Wheelchairs are mobile chairs, either manual or powered, that can improve mobility for people with SMA. They come in many types, made by numerous brands. They can be off the shelf or customized to fit a patient’s specific needs.

Manual chairs can be manipulated by their user or pushed by a caregiver. Power chairs are electronically controlled with a joystick that is usually placed on the side of the dominant hand and are an alternative for people who are not able to manipulate a chair with their arms, allowing for greater independence. Children as young as 18 months old can learn how to use power chairs.

Robotic Arm

The JACO Robotic Arm is specifically designed for power wheelchair users who have little to no use of their arms and hands. The arm replicates the natural movements of a human arm, a six-movement axis, corresponding to shoulder, elbow, and wrist. The arm can be mounted to the any side of a power wheelchair and operated by the chair’s native control system.

The robotic arm is lightweight and easily mounted to a chair’s power supply and following the tilt movement of the seat. The positioning of the arm minimizes the chair’s width and maximizes the arm’s reach. When the chair needs to be charged, the arm can be removed by turning it off and unplugging it from its cords. It can then be placed back in its case while the wheelchair is charging.

Braces and Standers

Due to the difficulty of SMA patients in maintaining good body alignment, they may be at risk for some orthopedic problems related to bone, muscles, and ligaments. Devices such as braces and standers may be used to support different parts of the body. A therapist with experience in working with SMA may help with choosing adequate braces and how to incorporate them into daily life.

Braces

Braces can be of three different types:

Ankle-foot orthoses: externally applied and intended to control the position and motion of the ankle to compensate for weakness and correct deformities. They can be used to give support to weak limbs or to position a limb with contracted muscles into a normal position.

Knee-ankle-foot orthoses: designed to provide support and proper joint alignment to the knee, foot, and ankle. They are used to help or replace weak muscles and protect the foot and lower limb.

Thoraco-lumbo-sacral orthosis: back braces that wrap beneath the arms and around the thorax, lower back, and hips to help cast the spine in a straighter position. Thoraco-lumbo-sacral orthosis cannot prevent scoliosis, or abnormal curvature of the spine, but can help people with SMA achieve a more upright position, improving function, independence, comfort, and quality of life.

Standers

Weight-bearing activities are important for bone growth and development. People with SMA — even those who are unable to sit — may benefit from devices that help promote weight-bearing. These devices, commonly known as standers, come in a variety of configurations to meet the specific needs of each person with SMA.

For a patient who is primarily supine, a supine stander may be used. With this stander, the patient is held nearly upright but with the head slightly behind the feet. This stander also is the best choice for patients who have insufficient head control — gravity and padding can hold the head in place. These standers may also be referred to as standing frames, so called because although they may have wheels, these wheels are primarily used for positioning purposes. Standing frames may be used as part of a regular physical therapy program. 

Conversely, patients who are mostly prone may use prone standers, which are configured in much the same way as supine standers, except the head is slightly in front of the feet in the vertical direction. These standers are not solely for static standing; some have integrated wheel systems that can allow patients to move while in the standing position, i.e., a standing “wheelchair.”

Lastly, sit-to-stand standers allow patients to transition between a sitting “wheelchair” configuration and a standing configuration. In most cases, someone has to help the patient make this transition, but gaining independence in this task could be a useful goal for some people with SMA.

Travel

People with SMA may be intimidated by the thought of travel, but proper preparation can make traveling easier for them.

The key is to plan travel well in advance, with reservations for the flight, accommodations, transportation, and special equipment made a few months prior to the trip. It’s also a good idea to confirm reservations one or two weeks prior to the date of travel.

It is easier to travel with fewer suitcases, so patients should consider carrying only the essentials, such as breathing equipment, portable hoist, and shower chair.

In the Air

Patients should consider taking nonstop flights whenever possible to avoid transit hassles. Checking the airline’s website for any information on “traveling with special needs” can be helpful. The airline should be informed about specific requirements such as needing an aisle seat or assistance with boarding. A neck support or cushions can make the flight more comfortable.

On the day of travel, arrive early at the airport to avoid delays. Wheelchairs need to be checked in with the rest of the baggage, so batteries must be removed or disconnected. Individuals with special needs often receive priority boarding, so reaching the gate early is a good idea.

On the Road

Road travel can be made easier with a wheelchair-accessible car or van, which is especially designed to accommodate disabled people sitting in a wheelchair. These vehicles can be rented if the individual doesn’t own one. A disabled parking permit should be displayed to avoid parking fines.

The vehicle must be fitted with special equipment to fulfill the patient’s needs, including a wheelchair ramp, Hoyer lift, four-point tie-downs and raised roof to accommodate a wheelchair-bound person. An emergency medical kit, specific medications, and equipment also should be included. For babies, car beds should be used, since infant seats may pose breathing risks.