Spinal muscular atrophy (SMA) presents daily challenges to those living with the condition. The condition can make it difficult to sit, stand, walk, eat, prepare meals, and even communicate. Sometimes, the progressive muscle weakness that comes with SMA makes these tasks impossible. Other times, people are too fatigued to finish a task they started.
The good news is, a wide variety of adaptive equipment is available to help make these activities of daily living (ADL) easier. Your health care team — which may include your primary care provider, physical therapists, occupational therapists, orthotists (orthotic specialists), speech-language pathologists, respiratory therapists, and other assistive technology specialists — will help guide you or (your family member) toward the equipment best suited to your needs.
Adaptive strollers help families with children experiencing SMA. These strollers can include hooks, bars, or trays that can hold or stabilize medical equipment. They also have systems such as safety belts and shaped foam padding to help stabilize the child in the best position for their needs. Adaptive strollers can be lighter and easier to transport than a wheelchair. However, if a child can self-propel a manual or electric wheelchair, helping them try it on their own can boost their self-esteem and encourage independence.
A car bed can be useful for children who have difficulties breathing and cannot tolerate a traditional car seat. Allowing for a more reclined position during car rides, a car bed makes long trips more comfortable while helping a child maintain proper posture and alignment for breathing.
Soft foam seats and back supports can help families keep someone positioned comfortably at mealtime. These products can be used for people of all ages. Sometimes, these seats can be more comfortable than a wheelchair during meals.
Many products are available to help people of all ages with bathing. These items include:
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Orthopedic braces are commonly custom-designed to match a user’s unique body type and needs. A prescription from a medical provider is often required for insurance to cover the costs of this equipment. The individual then will be referred to a specialty office or clinic where orthotists will create a custom bracing system, often out of rigid plastic or carbon fiber.
Ankle-foot orthoses and knee-ankle-foot orthoses are often rigid plastic or carbon fiber frames that cradle the feet and legs, secured with a system of velcro straps. These braces support weakened ankles and knees, preventing injuries such as twists and sprains. They can also help correct the alignment of limbs with contracted muscles.
Many people experiencing SMA also develop scoliosis — a curvature of the spine that can cause pain and difficulty breathing. In these cases, a thoraco-lumbo-sacral orthosis (TLSO) may be helpful. A TLSO is a back brace that wraps around the ribs and abdomen, supporting a back with weakened muscles. For some people, these devices are crucial to support breathing.
All people need to maintain the physical abilities they have for as long as they can. Sometimes, we instinctively want to help someone who we think needs assistance by removing, completing, or making tasks easier for them. However, in many cases, it is much better to support and enable people to carry out ADLs on their own. This is true for those living with SMA.
Supporting and encouraging people to use mobility devices to propel themselves, if they are able, is valuable in that it can help them maintain their strength and mobility. If a person can walk, a walker may be all the support they need. A walker can help relieve some of the burden on the hips or knees, enabling people to use upper-body strength for support and stability.
Adapting a home for the use of mobility devices can improve safety and increase independence. This includes:
For those with upper body strength and mobility, a manual wheelchair can be the best choice. The user can propel a manual wheelchair with their arms and hands or can be pushed by someone else. Self-propelling a manual wheelchair can provide essential exercise for users and prevent muscle contracture (shortening or stiffening) caused by disuse.
Some people can be completely independent when using a manual wheelchair — they can transfer themselves in and out, put it into a vehicle, and drive themselves. For others, a manual wheelchair is the right choice when they lack the required dexterity to operate an electric wheelchair independently and thus require a caregiver to push the manual wheelchair for them.
Electric wheelchairs, or power wheelchairs, are a great choice for those with limited strength and mobility, but who have the dexterity to use the controls that propel them. These controls usually include a joystick that controls forward and backward movement and turning. Some controls can adjust speed, elevate or lower the chair, or recline the chair. Electric wheelchairs can provide independence that greatly improves a person’s quality of life.
For some people, an adaptive tricycle can provide a great way to exercise and participate in activities. These machines come in styles suited to all ages. They are best suited for those with leg strength but perhaps less endurance or stability. These tricycles can come with safety straps that stabilize the user in the machine.
Many stander devices are available to help people achieve and maintain a standing position. This is valuable for those who can tolerate being upright, as it can aid in digestion, circulation, breathing, and it can prevent pressure injury. It also allows people to participate more naturally in activities with peers, which can be essential for self-esteem and learning.
These devices come in a variety of forms, adapted for those who cannot stand independently, for those who can stand with some support, and for those who just need some assistance with rising. Some electric wheelchairs even feature integrated standing technology, which allows the user to propel themselves into the kitchen, for example, and then rise to a standing position in order to reach an item on a shelf or to use the counter for food preparation.
People with SMA sometimes experience difficulty with speech. This is often due to the weakened or dysfunctional throat and tongue muscles, but it can also be related to weakened breathing muscles. These differences can mean that people with SMA have trouble with the clarity or loudness of their speech, or simply become fatigued while talking.
People experiencing communication difficulties can oftentimes benefit from augmented and assistive communication (AAC) devices. These can include software that can enable people with limited dexterity to form written sentences and even translate these sentences into audible speech via speech-generating devices.
Read more about assistive technology for communication.
SMA can weaken all of a person's muscles over time, including those used for breathing. Therefore, some people with SMA require support in breathing and coughing. They can benefit from machines such as a cough assist machine, a bilevel positive array pressure (BiPAP) machine, or a continuous positive airway pressure (CPAP) machine. These are noninvasive technologies — they do not involve elements that go inside someone’s body. Rather, they provide breathing and coughing support via a mask worn over the nose or the nose and mouth together.
Other times, people require invasive technology, like ventilation technology that is inserted via a small incision in the throat at the trachea (tracheostomy). Keeping the tracheostomy site clean and free of mucus is essential to prevent infection and irritation.
Some people experiencing SMA may develop dysphagia (difficulties with swallowing). This condition can put people at risk for choking, poor nutrition, and impaired growth. Sometimes, dysphagia can be managed by eating soft or pureed foods. Other times, feeding tubes may be necessary to make ensure someone is getting the necessary nutrition.
Feeding tubes deliver nutrition directly into the stomach or small intestine. They can be inserted through the nose and throat or by a small incision in the abdomen. Liquid nutrition can then be delivered by a small bag hanging nearby, which looks similar to intravenous medication. A large plastic syringe can be used to deliver a meal more quickly. Care must be taken to keep the tubing and its insertion site clean. Flush the tubing with clean drinking water after every meal — or as otherwise instructed by your health care provider.
Insurance coverage for these kinds of medical equipment varies by country, state, carrier, and policy. Your health care team should be able to help guide you to options that your insurance covers and submit appeals to denials, if necessary. Families can also reach out to local and national support agencies who may be able to help with funding or even the loan of a device, including:
On mySMAteam, the social network for people with spinal muscular atrophy, more than 1,300 members come together to ask questions, give advice, and share their stories with others who understand life with SMA.
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