Key points
- Exercises should be tailored to the person — helpful, but not to the point of exhaustion.
- Daily stretching, splints and standing help prevent contractures and protect bones.
- Power wheelchairs can be used from as young as 18 months.
- Scoliosis and hip problems are common; an orthopaedic doctor should check them regularly.
Most care for people with SMA focuses on controlling symptoms and preventive rehabilitation. Physiotherapy and occupational therapy play a critical role in maximising physical function. They use exercises to improve flexibility and overall function, independence and quality of life.
Therapeutic exercises
Therapeutic exercises help children maintain and improve their mobility. They are tailored to the child’s needs and the severity of their SMA, and can include any movement or position that supports daily activities — rolling, reaching, sitting and perhaps walking.
What is appropriate depends on age, how much the muscles are affected, and developmental stage. Exercise is important, but too much can cause fatigue and damage.
Range of motion and contractures
Range of motion (ROM) is the amount of movement in our joints. We maintain it through stretching and movement. If joints are not moved through their full range, muscles and soft tissues get “tight”, and this tightness can become permanent — a contracture.
People with SMA may be too weak to move their joints fully, which puts them at risk of contractures that cause discomfort, reduce mobility and limit flexibility. Splints, braces, standers and assisted stretching help keep joints aligned and prevent tightness. Ankle-foot orthoses worn at night provide a long, gentle stretch to stop ankle contractures getting worse.
Standing and weight-bearing
Putting weight through the bones — for example by standing — can improve bone strength, breathing and bowel function. This is true even for children who cannot bear weight on their own; they can be helped into standing with a standing frame.
Orthotics: splints and braces
Orthotics support and align the body. Devices vary with the amount of support needed:
- SMO (supramalleolar orthosis) and AFO (ankle-foot orthosis) — lightweight plastic braces that fit inside a shoe and keep the foot aligned when standing or walking.
- KAFO (knee-ankle-foot), HKAFO (hip-knee-ankle-foot) and RGO (reciprocating gait orthosis) — for positioning and alignment when standing.
Orthotics are usually custom-made and adjusted as the child grows.
Wheelchairs and mobility
Wheelchairs may be manual or powered. Manual chairs are pushed by the user or a carer. Power chairs are controlled electronically, usually with a joystick placed near the stronger hand — its position can be adapted if strength or control is limited.
Power chairs give children who cannot push a chair themselves the freedom to move without help. Power mobility can begin as early as 18 months of age.
Aqua therapy
In water, the body feels lighter and easier to move. Buoyancy relieves pressure on the joints and makes exercise easier, so children with SMA can often move their arms and legs further and more often in a pool.
Orthopaedic care
Scoliosis
Weakness of the muscles that support the spine can cause scoliosis, a sideways curve of the spine in a “C” or “S” shape, especially in children who use a wheelchair. It affects comfort, balance, use of the arms and — most importantly — breathing.
Scoliosis in SMA progresses slowly and needs regular checks. It can be managed by:
- Positional support — wheelchair seating and positioning devices, or a spinal brace, to help maintain a straight sitting posture.
- Surgery — as the curve progresses, spinal surgery is generally recommended to protect breathing, ease care, and improve comfort and quality of life.
Regular consultation with an orthopaedic doctor is recommended.
Hips
Hip subluxation and dislocation are common in non-sitters and sitters, and occasionally in walkers. They can make sitting balance difficult, affect breathing and cause chronic pain. Hip surgery is usually not needed in people with SMA who do not walk.
Equipment your child may need
Depending on age, type of SMA and age of onset, equipment can range from a standing frame and walker to a manual or power wheelchair, spinal brace, splints, a cough-assist machine and BiPAP. Our team can advise, and our financial aid programme can help families who cannot afford assistive devices.
For breathing support and chest physiotherapy, see Breathing.