Key Interventions for SMA Types 1, 2 & 3
Key Interventions:
Nonsitters: Type 1
"In nonsitters, nutritional support, posture management, seating, contracture and pain management, therapy for activities of daily living and assistive equipment, wheelchairs for mobility, limb orthotics, and developmental therapies are important. A multi-disciplinary approach to evaluation and management includes a strong partnership between therapists, patients, families, and physicians. Assessments include physical and occupational therapy and speech therapy if swallowing is impaired or if speech production is affected by jaw contractures and inadequate ventilatory support of voice. Play and occupational support should include lightweight toys and assistive technology with variable controls and a myriad of (many) activation systems (ways to activate). Consideration of the patient's primary posture should direct choice of equipment and devices that support function".
Sitters: Type 2
"In sitters, wheelchair mobilty, contracture management, physical therapy, and occupational therapy are of the highest value, with strong consideration for spine and limb orthotics and spine surgery. Evaluations by physical therapists, occupational therapists, and orthopedic surgeons include measurement of contractures and strength by goniomety (instrument for measuring the angle of one bone to another and range of motion in a joint), manual muscle testing, or myometry (a way to measure strength), with judicious (wise, cautious) use of spine and hip radiographs. Equipment evaluation includes seating and mobility, positioning, and equipment for self-care. Evaluations for manual and power mobility may be conducted as early as 18 to 24 months of age. Contracture management and exercise are a major focus of treatment, with a regular stretching and bracing program to preserve flexibility. Regular exercise should be encouraged to maintain fitness and endurance and might include swimming and adaptive sports".
Walkers: Type 3
"In walkers, the highest emphasis is on provision of physical therapy, occupational therapy, and wheelchair/mobility, although orthotics, scoliosis surgery and pain management figure prominently" (may be very important). The combination of proximal weakness (near the central part of the body) and impaired balance results in frequent falls. Limitations are found in transitions between the floor, sitting, and standing, distance ambulation, changes in terrain, and stair climbing. Physical and occupational therapy assessments to determine appropriate mobility aids, adaptive equipment, assistive technology, and environmental access will allow patients to maintain independence and mobility and to conserve energy. Treatment and interventions should consider goals of the family and /or caretakers and should be problem-driven. Physical therapy consultation helps to maximize safety, endurance, and independence or to prolong ambulation. Contracture management and education to maximize joint protection should be a part of any treatment program. Maximum functional activity includes access to leisure, adaptive sport, and play activities. Regular exercise to maintain fitness and stamina should be encouraged and may include swimming, aquatic therapy, horseback riding, and adaptive sports".
Nonsitters: Type 1
"In nonsitters, nutritional support, posture management, seating, contracture and pain management, therapy for activities of daily living and assistive equipment, wheelchairs for mobility, limb orthotics, and developmental therapies are important. A multi-disciplinary approach to evaluation and management includes a strong partnership between therapists, patients, families, and physicians. Assessments include physical and occupational therapy and speech therapy if swallowing is impaired or if speech production is affected by jaw contractures and inadequate ventilatory support of voice. Play and occupational support should include lightweight toys and assistive technology with variable controls and a myriad of (many) activation systems (ways to activate). Consideration of the patient's primary posture should direct choice of equipment and devices that support function".
Sitters: Type 2
"In sitters, wheelchair mobilty, contracture management, physical therapy, and occupational therapy are of the highest value, with strong consideration for spine and limb orthotics and spine surgery. Evaluations by physical therapists, occupational therapists, and orthopedic surgeons include measurement of contractures and strength by goniomety (instrument for measuring the angle of one bone to another and range of motion in a joint), manual muscle testing, or myometry (a way to measure strength), with judicious (wise, cautious) use of spine and hip radiographs. Equipment evaluation includes seating and mobility, positioning, and equipment for self-care. Evaluations for manual and power mobility may be conducted as early as 18 to 24 months of age. Contracture management and exercise are a major focus of treatment, with a regular stretching and bracing program to preserve flexibility. Regular exercise should be encouraged to maintain fitness and endurance and might include swimming and adaptive sports".
Walkers: Type 3
"In walkers, the highest emphasis is on provision of physical therapy, occupational therapy, and wheelchair/mobility, although orthotics, scoliosis surgery and pain management figure prominently" (may be very important). The combination of proximal weakness (near the central part of the body) and impaired balance results in frequent falls. Limitations are found in transitions between the floor, sitting, and standing, distance ambulation, changes in terrain, and stair climbing. Physical and occupational therapy assessments to determine appropriate mobility aids, adaptive equipment, assistive technology, and environmental access will allow patients to maintain independence and mobility and to conserve energy. Treatment and interventions should consider goals of the family and /or caretakers and should be problem-driven. Physical therapy consultation helps to maximize safety, endurance, and independence or to prolong ambulation. Contracture management and education to maximize joint protection should be a part of any treatment program. Maximum functional activity includes access to leisure, adaptive sport, and play activities. Regular exercise to maintain fitness and stamina should be encouraged and may include swimming, aquatic therapy, horseback riding, and adaptive sports".