Degenerative conditions Working with clients who have degenerative conditions requires specific considerations for seating interventions. Common degenerative conditions requiring wheelchair seating include MS; amyotrophic lateral sclerosis (ALS); muscular dystrophies (such as Duchenne); congenital myopathies; and spinal muscular atrophy (SMA). All of these clients may have progressive postural changes that seating cannot completely prevent, and thus the seating will have to be modified and adjusted over time. Growth capabilities of the frame and seating need to be considered when completing recommendations. Multiple sclerosis Multiple sclerosis (MS) is a progressive condition that may involve periods of remission and exacerbation. The client may experience loss of function in nearly any area of the body (National Multiple Sclerosis Society, 2019). Four basic MS disease courses have been defined: clinically isolated syndrome; relapsing remitting (RRMS); secondary progressive (SPMS); and primary progressive (PPMS). About 85% of people with MS are initially diagnosed with RRMS and experience periods of remissions and exacerbations. Most people with RRMS are eventually diagnosed with SPMS, which is characterized by a progressive worsening of function. PPMS is characterized by worsening function from the time of diagnosis (National Multiple Sclerosis Society, 2019). This loss of function can lead to an increased need for postural support and increased muscle tone and spasticity. Unlike other degenerative conditions characterized primarily by muscle weakness, postural support needs vary greatly among individuals with MS. The support required is dependent on which areas of the body are affected. Knowledge regarding the type and stage in disease process is critical for occupational therapy and physical therapy practitioners to have as they use their clinical reasoning to guide the wheelchair seating and positioning process. Muscle weakness Other conditions, such as ALS, muscular dystrophies, congenital myopathies, and SMA, lead to progressive loss of muscle strength. This muscle weakness leads to increasing need for postural support and inability to shift weight, resulting in an increased risk of pressure injury development. These conditions spare sensation, so the client can sense discomfort (ALS Association, 2019). Even slight seating changes may affect Previous sections have already addressed strategies to prevent pressure injury development. Most people with degenerative Postural care Proper positioning has long been recognized as an important consideration when evaluating and recommending seating systems for wheelchairs. However, clinicians are beginning to realize the importance of looking at a person’s posture throughout his or her 24-hour day. Twenty-four-hour postural management or care is an approach that looks at all of the different positions a person with severe motor impairment assumes and all of the supportive equipment he or she uses throughout the day and tries to optimize postural alignment as much as possible in all environments and times of day, including nighttime (Ágústsson & Jónsdóttir , 2018). A 24-hour positioning evaluation examines how a person is positioned in his or her wheelchair, in bed, and during alternative daytime positioning. A child with cerebral palsy, for example, may have an appropriate seating system in a manual wheelchair that helps to maintain the spine and hip joints in neutral alignment. However, if that same child spends only 6 hours a day in the wheelchair, he or she is spending 18 more hours in other positions – often in destructive postures that place the child at risk for orthopedic complications. function. Pressure
conditions can detect discomfort and the need to provide a change in position. These clients may require power seating to shift their weight, using tilt and/or recline combined with power elevating leg rests. The seating surface must distribute pressure well but may not need to provide significant postural support. Clients with significant weakness do not usually move out of position with the same forces as someone with increased muscle tone. Instead, the seating surface’s primary goal is to reduce peak pressures and provide comfort. Clients with muscle weakness typically experience atrophy, which increases risk of pressure injuries over bony prominences. Case study Jordan is 10 years old and has SMA type I. He has used a ventilator since age 1 and has been driving a power wheelchair since age 3. Initially, he was positioned in a linear seating system, which provided adequate postural support and easily grew to match his body growth. His orthopedist recommended that Jordan wear a TLSO to provide more postural support to the trunk. His seating system had to be modified to fit Jordan while he was wearing the TLSO. As he continued to grow and his spine and rib cage became more distorted, he stopped using the TLSO, and a molded orthotic seating system was recommended to accommodate his unique body shape, to distribute pressure along these contours, and to provide intimate contours that offered the significant postural support he required (Figure 16). Figure 16: Jordan in Custom-Molded Seating System
Sleep positioning Sleep positioning is the specific therapeutic positioning of a person’s body during sleep. Sleep positioning has three main goals: ● To improve the quality and duration of sleep. ● To promote health and maintain safety during sleep. ● To prevent or lessen the development of orthopedic distortions. (Ágústsson & Jónsdóttir, 2018) Many individuals with physical disabilities have a difficult time sleeping, due to an inability to change position, abnormal muscle tone and movement, discomfort or pain, or because of difficulties with breathing or swallowing. This leads to poor sleep quality and duration – essentially sleep deprivation – for both the disabled individual and for the individual’s caregiver. Restorative sleep is essential for people with physical disabilities in order to help repair soft tissue trauma that may have occurred during the day (from abnormal postures and spasticity; to optimize immune system functioning; to promote normal growth in children; and to maximize cognitive and physical performance during the daytime (Ágústsson & Jónsdóttir, 2018).
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Book Code: PTNC1023
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