**7.2 Participation in physical activities**

Weakness and difficulty in moving independently in patients with SMA contribute to physical inactivity and limited participation in exercise programs. The consequences of physical inactivity are particularly detrimental, could contribute to secondary impairments and may lead to additional decline in functional status. Recent evidence suggests that engagement in physical activities helps improve physical functioning in children with disability. Participation in physical activities may promote physical functioning, quality of life, health, and well-being.

Participation in physical activity may include participation in recreational and sports activities. Recreational programs that can be beneficial include swimming, cycling, and riding when appropriate.6 Activities should be selected carefully with the goal of improving functional performance and daily activities, promoting aerobic fitness and preventing complications of inactivity. Activities should be selected based on the age, developmental skills, and functional abilities.

### **7.3 Aquatic exercises**

Aquatic therapy or hydrotherapy is being used on an increasing basis and has been shown to be beneficial for children with SMA.26 The use of aquatic therapy in SMA may be related to the physical properties of water. The properties of water provide weight relief and postural support, facilitate antigravity movements allowing more freedom of movement, and provide an opportunity to perform activities that may be too difficult to accomplish on land.26 Aquatic exercises provide low-intensity strength training, walking and balance exercises, and aerobic training without the fear of fatigue or overwork.

#### **7.4 Feeding and nutrition**

Infants with type I SMA have poor oral motor control with sucking and chewing and tendency to get fatigued easily during feeding and swallowing. Lack of head control and head support may also affect swallowing. Those children may have difficulty getting enough nutrition and are at risk of aspiration. Some infants may require indwelling nasogastric tube to supplement oral feeding. Gastrostomy may be an option for some children to improve carer satisfaction and quality of life,23 and to avoid aspiration.

#### **7.5 Management of contractures**

Muscle contractures and orthopedic deformities are common complications among patients with SMA. Contractures and orthopedic deformities occur primarily in type II and type III

Spinal Muscular Atrophy 229

used to accommodate for weak neck and trunk muscles and lack of head and trunk control, they can be used to assist in positioning and to maintain upright head and trunk during

Non-ambulatory patients with SMA may benefit from a standing program using standing frames or swivel walkers. Standing programs are used for non-ambulatory patients to prevent or reduce secondary impairments by maintaining muscle extensibility, preventing muscle and soft tissue contracture, promoting optimal musculoskeletal development, and to

Because of the progressive weakness associated with SMA, many patients benefit from a wheeled mobility device as the primary means of locomotion. Wheelchair seating system deserves special considerations since many patients require a full-time use of the wheeled mobility device. The course and progression of the disease, presenting symptoms, degree of spinal deformity, and whether the patient is using mechanical ventilation should be taken into considerations when deciding on a mobility device. Manual wheelchairs allow the patients to maintain upper body strength and cardiovascular endurance. A power wheelchair should be considered when impairments prevent manual propulsion. Power wheelchairs enable patients to maintain a level of independence while moving within their environment and to compensate for mobility limitations.14 For young children who are not ambulatory, power mobility may be used to provide independent mobility at appropriate developmental

Progressive weakness and reduced mobility associated with SMA place the patients at risk of contractures and scoliosis. Scoliosis is the most serious orthopedic problem seen in patients with SMA. Scoliosis develops earlier and progresses faster in non-ambulatory children than ambulatory children, scoliosis is seen in almost all children with type II SMA and majority of patients with type III, with the severity is less in type III SMA as compared to type II SMA. The incidence and severity of scoliosis increase with age and severity of muscle weakness, with the severity and progression of scoliosis increase once patients lose ambulation and become dependent on wheelchair for ambulation. Reduced respiratory function is common in patients with scoliosis. As muscle weakness progresses, the degree of scoliosis increases causing more discomfort and difficulty in positioning and respiration. Presence and degree of spinal deformity should be monitored periodically by examination and routine radiography, particularly for the non-ambulatory patients or as patient loses ambulation. Spinal x-rays are indicated once there is clinically detected scoliosis.32 Range of motion program and spinal positioning are important to provide comfort and slow the progression of spinal deformities. Adequate trunk supports on a wheelchair, and wheelchair modifications such as custom molding, gel or air cushions may be needed to provide maximum support, and comfort and may minimize the progression of spinal deformity. As

age.25 Children as young as two years can independently propel wheelchair.14, 25

sitting.

**7.6.3 Standing devices** 

address the issue of reduced bone mineral density.27,29

**7.6.4 Wheelchairs and seating systems** 

**7.7 Management of scoliosis** 

patients who have longer periods of muscle weakness. Contractures develop secondary to muscle weakness, muscle imbalance, lack of mobility, and poor posture and positioning. Development and severity of contractures are related to the severity of muscle weakness, the duration of muscle weakness, and immobility. Muscle contractures are common in muscles that cross two joints or more. Classic contractures are seen in iliotibial band, hip flexors, knee flexors and plantar flexors.

Prevention and treatment of contractures are important issues in the management of patients with SMA. Management of contractures should begin before the contractures exist. Management of contractures includes combination of consistent program of range of motion exercises, positioning, regular stretching, and splinting. Muscle groups that are at risk of developing contractures should be targeted for stretching. Range of motion and stretching exercises can be used to preserve and increase flexibility. Active range of motion and stretching exercises can be used to maintain flexibility and prevent contractures in the ambulatory patients. In the non-ambulatory patients, regular range of motion program and passive stretching are used to prevent development and slow progression of contractures. Ankle foot orthoses and night splints can be used to maintain flexibility and range of motion. Positioning devices and custom fitted equipment can be used for positioning to provide low-intensity prolonged stretching. A tilt in space or recliner chairs can be used to allow easy positioning changes. Standing program provides low-intensity prolonged stretch that can be used for the non-ambulatory children.

#### **7.6 Adaptive equipment and assistive devices**

Patients with SMA frequently benefit from use of assistive and adaptive devices, with changing needs as their condition progresses. Adaptive equipment and assistive devices can be used to provide positioning, control contractures and deformities, and support function. The choice of assistive devices for patients with SMA is based on individual clinical decisions due to lack of definite intervention trials. The decision to use an assistive device should be a collaborative decision between the patient, family, orthopedic surgeon, physiatrist, and therapist.

#### **7.6.1 Orthotics**

Ankle foot orthoses (AFO) or night splint can be used to provide prolonged stretch to control the progression of plantar flexion contractures. Knee splints may be used to control hamstring flexibility and knee flexion contractures. Thigh binders can be used to control iliotibial band contractures. Assistive devices including braces, taping, AFO, knee-ankle-foot orthoses (KAFO), and hip-knee-ankle-foot orthoses (HKAFO) can be used to provide support and maintain joints alignments. Assistive devices may be used to facilitate stability, weight bearing and upright posture during standing and ambulation.

#### **7.6.2 Positioning devices**

Positioning devices can be used to provide support, and control contractures and deformities. Positioning devices can be custom fitted, special foam, or cushions. They allow easy positioning and stretching, and provide support. Head and trunk lateral support can be

patients who have longer periods of muscle weakness. Contractures develop secondary to muscle weakness, muscle imbalance, lack of mobility, and poor posture and positioning. Development and severity of contractures are related to the severity of muscle weakness, the duration of muscle weakness, and immobility. Muscle contractures are common in muscles that cross two joints or more. Classic contractures are seen in iliotibial band, hip

Prevention and treatment of contractures are important issues in the management of patients with SMA. Management of contractures should begin before the contractures exist. Management of contractures includes combination of consistent program of range of motion exercises, positioning, regular stretching, and splinting. Muscle groups that are at risk of developing contractures should be targeted for stretching. Range of motion and stretching exercises can be used to preserve and increase flexibility. Active range of motion and stretching exercises can be used to maintain flexibility and prevent contractures in the ambulatory patients. In the non-ambulatory patients, regular range of motion program and passive stretching are used to prevent development and slow progression of contractures. Ankle foot orthoses and night splints can be used to maintain flexibility and range of motion. Positioning devices and custom fitted equipment can be used for positioning to provide low-intensity prolonged stretching. A tilt in space or recliner chairs can be used to allow easy positioning changes. Standing program provides low-intensity prolonged stretch

Patients with SMA frequently benefit from use of assistive and adaptive devices, with changing needs as their condition progresses. Adaptive equipment and assistive devices can be used to provide positioning, control contractures and deformities, and support function. The choice of assistive devices for patients with SMA is based on individual clinical decisions due to lack of definite intervention trials. The decision to use an assistive device should be a collaborative decision between the patient, family, orthopedic surgeon,

Ankle foot orthoses (AFO) or night splint can be used to provide prolonged stretch to control the progression of plantar flexion contractures. Knee splints may be used to control hamstring flexibility and knee flexion contractures. Thigh binders can be used to control iliotibial band contractures. Assistive devices including braces, taping, AFO, knee-ankle-foot orthoses (KAFO), and hip-knee-ankle-foot orthoses (HKAFO) can be used to provide support and maintain joints alignments. Assistive devices may be used to facilitate stability,

Positioning devices can be used to provide support, and control contractures and deformities. Positioning devices can be custom fitted, special foam, or cushions. They allow easy positioning and stretching, and provide support. Head and trunk lateral support can be

weight bearing and upright posture during standing and ambulation.

flexors, knee flexors and plantar flexors.

that can be used for the non-ambulatory children.

**7.6 Adaptive equipment and assistive devices** 

physiatrist, and therapist.

**7.6.2 Positioning devices** 

**7.6.1 Orthotics** 

used to accommodate for weak neck and trunk muscles and lack of head and trunk control, they can be used to assist in positioning and to maintain upright head and trunk during sitting.
