Highlights from the Consensus Statement in the Standard of Care in SMA
About Movement and Daily Activities
The Family Guide to the Consensus Statement for the Standard of Care in Spinal Muscular Atrophy* provides this helpful advice about movement and daily activities:
"Muscle weakness, the most obvious symptoms of SMA, varies from person to person, depending on disease severity. Care Plans for managing muscle weakness of the arms, legs, trunk and neck are important for helping your child achieve his or her highest level of function and independence"."Physical therapists, occupational therapists, speech therapists and /or rehabilitation specialists are the experts who can help you and the rest of the medical team design the best plan for your child. Their evaluations may include range-of-motion, strength and mobility tests. They can recommend exercises and tools or assistive devices to help your child maintain the best posture for lung function and eating, as well as tools for moving during work or play. These tools can also help manage and prevent muscle contractures, spinal deformity, pain and bone fractures that can make your child's disability worse".
"Assistive devices ranging from orthotics and braces to motorized wheelchairs are important for you child's health and daily activities. Consulting with a therapist who has experience in working with SMA patients can be helpful in determining which assistive devices are most appropriate for your child and how best to incorporate them into daily living. Children with SMA should be evaluated by an orthopedic doctor for spinal curvature; surgical stabilization of the spine may be recommended".
Work to develop a physical therapy plan to help your child achieve their highest personal level of function and independence
Consider use of assistive devices, tools and exercise to support breathing, eating, work and play
Consider use of assistive devices to help slow or prevent complications of SMA
Consult with your medical team about evaluation by an orthopedic specialist"
*This document was prepared by the Patient Advisory Group of the International Coordinating Committee for SMA Trials.
Highlights from the Consensus Statement for the Standard of Care in SMA
The Consensus Statement for Standard of Care in SMA, published in the Journal of Child Neurology, includes very well respected, expert opinion on SMA care. It is written for professionals and may be quite complicated, but is perhaps the best reference for you, your doctors and your therapists. The document includes a section on "Orthopedic Care and Rehabilitation," where you can find excellent information. Here are some highligths:
Overview of Orthopedic Care and Rehabilitation Strategies in Spinal Muscular Atrophy
Key problems: "Muscle weakness of varying severity limits motor function of trunk and upper and lower extremities, resulting in contracture formation, spinal deformity, limited mobility and activities of daily living, and increased risk of pain, osteopenia, and fractures.
Key Evaluation Procedures: "These include evaluating range of motion, strength, function, seating and mobility orthotics, with radiographs of the spine and other joints....The value of these procedures varies by degree of functional impairment".
"Traditional measurements of strength are not possible in severly affected infants and children; thus emphasis is on observation of function. Evaluation procedures that address rehabilitation/orthopedic concerns include the CHOP INTEND, the Hammersmith Functional Motor Scale for SMA, the Modified Hammersmith Functional Scale for SMA, the Gross Motor Function Measure, and the Motor Function Measurement Scale for Neuromuscular Disease".
Nonsitters: "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: "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: "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".