SMA is a degenerative illness that results in muscular atrophy and weakening. Since there is no known treatment for SMA, symptomatic management and preventative rehabilitation comprise the majority of the patient’s therapy. Maximizing the physical functioning of individuals with SMA is greatly aided by the services of physical and occupational therapists. Exercises designed to enhance flexibility, general function, independence, and quality of life are the core focus of physiotherapy.

The following is the main emphasis of the treatments:

Therapeutic exercises

Children can preserve and enhance their mobility with the aid of therapeutic exercises. Depending on how severe the child’s SMA is, these will be customized to meet their needs.
They include any posture or movement that a person wants to work on strengthening to enhance their everyday living tasks, such as sitting, rolling, reaching, and possibly even walking. Age, the degree of neuromuscular involvement, and the developmental stage all influence these actions. While exercise is vital, doing too much of it can be harmful and exhausting.

Range of motion

The range of motion in our joints is known as range of motion (ROM). Stretching and exercising help us to maintain our range of motion. Muscles and other soft tissues may become “tight” if we do not move our joints through their entire range of motion. This constriction may become irreversible and impede mobility. This is known as a contracture.
It’s possible that people with SMA are too weak to fully extend their joints. They may be more susceptible to contractures as a result, which could become irreversible and limit their ability to move. They may result in pain, restricted movement, and weakened flexibility in the afflicted body portion.

It may be necessary to employ braces, splints, standers, and active-assisted stretching in order to preserve joint alignment and avoid contractures and tightness. Ankle plantar flexion contractures can be prevented from getting worse by using ankle-foot orthotics at night. These orthotics can help provide sustained, passive stretching.

Weightbearing

Carrying a weight can be beneficial to the condition of our muscles and bones. Putting weight through our bones by standing, for example, can strengthen our bones and enhance our ability to breathe and urinate. Even for kids who can’t support their own weight, this is true. A standing gadget can assist these kids in getting to their feet.

Orthotics: Splints/Braces

Devices called orthotics help to correct and support the foot. The kind of device used depends on how much assistance a youngster requires.

An AFO (ankle-foot orthosis) or SMO (supramalleolarorthosis) may be used by children with SMA. When your child is standing or walking, these lightweight plastic braces can help preserve foot alignment by fitting inside their shoes. Orthotics are usually made to order and can be adjusted as the youngster develops or grows. To assist with ideal placement and alignment while standing, orthoses such as KAFO (knee-ankle-foot), HKAFO (hip-knee-ankle-foot orthosis), and RGO (reciprocating gait orthosis) can be utilized.

MOBILITY: Wheelchair

A wheelchair can be defined as a moveable chair in its most basic form. Wheelchairs come in manual and powered versions. Manual chairs can be pushed by a caregiver or adjusted by the person using it. Power chairs are electronic wheelchairs that are usually operated using a joystick. This joystick/control panel is usually placed close to the child’s dominant hand, although it can be moved if the youngster has trouble with muscular control or strength. Children who are unable to maneuver their chair with their arms may be able to do it with the assistance of a powerchair. It is possible to start power mobility training as early as 18 months of age.

Water therapy/Aqua therapy

Your body feels lighter and more flexible in the water. This is caused by the buoyancy of the water. It may be easier to complete exercises and reduce joint pressure when in a swimming pool. For kids with SMA, water therapy is especially advantageous because of this. When submerged in water, your child might be able to move their arms and legs more often and throughout larger ranges of action.

Orthopedic care

The condition of our bones and the corresponding muscles, ligaments, and other soft tissues are referred to as orthopedic disorders. Children with SMA may be more susceptible to some orthopedic problems because they may not be able to maintain proper alignment and/or may have trouble moving.

Children who are confined to wheelchairs may develop scoliosis as a result of weakening of the muscles that support the spinal column. Instead of having a straight spine, a person with scoliosis may have a “C” or “S” shaped spine. Comfort, arm function, and—most importantly—our ability to maintain our balance when sitting and standing are all impacted by a bent spine.

In SMA, scoliosis gradually worsens and requires routine monitoring. There are two methods to assist. Positional curve control comes first. This includes using any method to improve the alignment of the spine. To help the kid maintain a straight sitting posture, bracing with a spinal orthosis or wheelchair adaptations (positioning devices) can be used. In addition to stabilizing the spine, these postural curve adjustments may enhance function, independence, comfort, and quality of life. Surgery is typically advised as scoliosis worsens in order to maintain lung function, enhance overall quality of life, comfort, nursing care, and appearance. It is advised to get a consultation with an orthopedic physician.

Sitting balance can be challenging due to hip subluxation and dislocation, which are typical issues for non-sitters, sitters, and occasionally walkers. It could cause persistent pain and impede respiratory function, which is already weakened. Generally speaking, non-ambulatory SMA patients do not require hip surgery.

Respiratory care

One of the main goals of medical management for SMA is frequently respiratory care. If the muscles that support the chest wall, known as the intercostals, are weak, the diaphragm takes over as the main breathing muscle. Reduced respiratory function, including underdeveloped lungs and trouble clearing secretions through coughing, may result from this. Chest infections that come back could result from this.

Children older than five years old can have their pulmonary function tested to determine how well they breathe. The procedure involves having them breathe into a tube to gauge their lung capacity.

The child may benefit from chest physical therapy to help with secretion clearance. It frequently involves shifting positions so that gravity can help mobilize secretions and maintain clear lungs. The patient can be positioned in various ways to let gravity empty each lung lobe and compartment. Postural drainage is encouraged by this methodical positional shifting. It is frequently possible to carry it out at home under a therapist’s supervision.

Children can also benefit from using a cough assist gadget at home to help them cough out mucus. For breathing, children with SMA rely on their diaphragms. It is more difficult to take deep breaths while people are lying down at night since the contents of their stomach press against the diaphragm and gravity is removed. This may result in an accumulation of carbon dioxide and insufficient oxygen reaching our lungs. This may cause headaches or daytime sleepiness in children. Physicians may prescribe a BIPAP machine, which provides the youngster with extra air bursts to aid with breathing (intermittent positive inspiratory pressure). The child’s respiratory muscles can relax thanks to this help.

All of the aforementioned supportive care measures can be implemented as soon as a chest infection manifests. Inhalation therapy and antibiotics might also be required. In some cases, hospitalization is necessary to provide the youngster with the best possible care.