Muscle weakness can cause several complications for individuals with spinal muscular atrophy (SMA). These complications are referred to as orthopedic or musculoskeletal issues.
Individuals with SMA can be classified by function including “non-sitters,” “sitters,” “standers,” or “walkers” based on the highest physical milestone achieved. This classification helps create a proactive plan to identify which complications pose the greatest risk to the functional well-being of an individual, choose the right preventative measures, or determine the best course of treatment if complications do occur.
Common Musculoskeletal Issues
Musculoskeletal issues can cause pain as well as difficulty sitting, standing, or performing normal daily activities.
These issues may include:
- Contractures, which limit the range of motion in a joint
- Bone fractures
- Hip dislocation
- Spinal deformities, such as scoliosis and kyphosis, or a combination of the two
Spinal deformities can lead to excessive pressure on hips and buttocks, causing skin breakdown. Scoliosis can also impair lung function by restricting motion of the chest and diaphragm during breathing. Spinal deformity can be treated through modification of the wheelchair seating system and use of braces. If severe, doctors may recommend surgery to help straighten the spine and chest.
Musculoskeletal Care Team
As with all medical issues in SMA, decisions on treatment of musculoskeletal problems should be made with an interdisciplinary healthcare team, who can evaluate all aspects of the situation. Often, the musculoskeletal team for an individual with SMA will include:
- Occupational Therapists (OT) work primarily with the arms and hands, focusing on activities like feeding, dressing, and other skills. They may recommend or make splints and teach exercises to maintain range of motion and improve function. They may recommend equipment to help with skills and activities
- Physical Therapists (PT) work primarily with the legs and feet. They focus on mobility. They may recommend orthotics (or braces) and teach exercises to maintain range of motion and improve function. They may recommend equipment such as special seats, strollers, and wheelchairs
- Orthotists focus on making and adjusting orthotics (braces)
- Orthopedic Surgeons For conditions like hip instability or spinal deformity that don’t respond to non-surgical treatment, an orthopedic surgeon may be brought in to evaluate and consider surgery
- Physical Medicine and Rehabilitation (PMR) Also known as physiatry or rehabilitation medicine, PMR aims to enhance and restore functional ability and quality of life
At-Home Musculoskeletal Care
The musculoskeletal team may prescribe activities that can be done at the therapist’s office, at home, or both. This includes:
- Exercises to improve or maintain joint range of motion
- Use of equipment for SMA, such as braces or a stander
- Supplemental calcium and vitamin D as part of nutrition plan to help with bone density
Individuals with SMA often require a range of specialized medical equipment. This equipment can be used to increase mobility, provide support and strength, or to assist with activities of daily living that individuals with SMA may not be able to do alone because of their limited strength and mobility. The following is a list of equipment that has proven beneficial for people with SMA.
Please note, this is not a comprehensive list, and you or your child may not benefit from all of the items described here. In addition, this list is not a substitute for professional, personalized advice from a physician or other qualified healthcare provider. Be sure to speak with your healthcare team and insurance company before making any decisions on medical equipment.
Also called medical strollers, an adaptive stroller is built for an individual who needs additional support. These strollers usually come with accessories, allowing the child to be supported and positioned comfortably. Some strollers also have trays to carry medical equipment.
Specialized bath chairs provide comfortable and secure bathing for individuals who need additional support. Bath chairs are adjustable, with multiple seat and back angles.
AFOs are externally applied and intended to control the position and motion of the ankle, compensate for weakness, or correct deformities. AFOs can be used to support weak limbs or to position a limb with contracted muscles into a more normal position.
A car bed is a car seat that lies flat, allowing the child to lie safely and comfortably in the prone position. For babies who do not tolerate sitting upright in a car seat such as infants who cannot sit , these are vital.
An E-Z-ON modified vest is used for transporting children with SMA type 1 once they outgrow the car bed. This modified vest buckles into the back seat seatbelts of a car and allows the child to lay flat across the back seat during transport.
These soft foam positioning seats are designed for all ages. They are simple to use and don’t require complicated adjustments. They are a great alternative to a wheelchair or stander.
KAFOs are designed to provide support and proper joint alignment to the knee, foot, and ankle. They assist or support for weak muscles and protect the foot and lower limb.
Standers provide several therapeutic and physiological benefits for individuals with SMA. They help with digestion, circulation, and breathing. Standers also promote bone strength as users bear weight on their legs.
Powered or manual wheelchairs allow individuals with SMA to be more mobile and independent. Wheelchairs are usually customized to an individual based on their unique needs.
A TLSO is a back brace that wraps under the arms and around the rib cage, lower back, and hips in order to support the spine while in an upright position against gravity.
Please visit our equipment pool page to find out which of these items may be available through Cure SMA and to submit an equipment pool request.