Specialized Equipment for Spinal Muscular Atrophy
Adaptive Strollers and Wheelchairs
Mobility can be a challenge for people with SMA. Specialized equipment such as adaptive strollers and wheelchairs can help to optimize their care and maximize their independence. Adaptive strollers, which are also known as medical strollers, help a child sit more comfortably. Some have trays to carry medical equipment. Wheelchairs can help SMA patients get around, whether operated manually or motor-driven. Wheelchairs also can be off-the-shelf or customized to fit particular patients’ needs.
Bath Chairs and Feeder Seats
Families can do a number of things to improve the well-being and comfort of a child with SMA. These range from home modifications to specialized equipment. Bath chairs offer secure bathing to people with special needs. The seats and back angles are usually adjustable. Feeder seats are soft-foam positioning seats designed for all ages. An alternative to a stander or wheelchair, they are simple to use and don’t require many adjustments.
A bi-level positive airway pressure (BiPAP) machine can sense a person’s normal breathing cycle and work with it. The device provides a user with higher pressure and an increased volume of air while inhaling, and automatically lowers the pressure when the person is exhaling. It also helps users who are not breathing properly because they are sleeping. The airway pressure is delivered through a mask that’s either over the nose or over both the nose and mouth, or through a tube running from under the nose into the nostrils.
Braces and Standers
Due to the difficulty maintaining good body alignment for SMA patients, 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. Standers promote bone strength by forcing users to bear their body’s weight on their legs. They also help with digestion, circulation, and breathing.
Cough Assist Machine
Coughing plays a very important role in clearing the airways of mucus and other secretions, for better overall respiration. Infants and children with SMA types 1 and 2 often have weakened muscles, which may lead to chronic hypoventilation and low peak cough flows, resulting in impaired chest clearance. A cough assist machine forces air into the lungs at a predetermined pressure, then sucks the air out at another predetermined pressure. It helps maintain airway clearance.
Depending on the type and severity of the condition, children with SMA experience feeding problems such as difficulties swallowing or chewing due to limited range of jaw movement, trouble moving food around the mouth with the tongue, or decreased bite force and fatigue in the muscles involved in chewing. Poor head control can also cause problems and put patients at risk. Feeding tubes are an option for children with SMA who have poor nutrition or are at risk of developing aspiration pneumonia.
A pulse oximeter is a portable device used to check blood-oxygen levels, by measuring the amount of oxygen in the blood (pulse oximetry). It consists of a light small clamp or a Band Aid-like device that is wrapped on a finger or toe. The equipment alerts to problems (e.g., low oxygen levels) or if help is needed with coughing. Children with SMA need extra breathing support when oxygen levels drop too low.