Case Report B7 Developing Independent Downhill Skiing for a Child with Ataxic Cerebral Palsy As Neuro-Developmental Treatment (NDT) therapists, we have used the International Classification of Functioning, Disability and Health (ICF) model over the last 2 decades in our clinical approach to enhancing the skills in functional and meaningful tasks for our patients.1,2 We have also attended to overall quality of life issues, including the social participation domain of the ICF model. Our literature demonstrates the social cost of disability for children.3,4,5 Parents and youths report isolation, loneliness, and lack of self-efficacy (a person’s belief in his or her ability to succeed in a particular situation).5 Differences in social participation (outside of home) between children with cerebral palsy (CP) and children without disability demonstrate a marked difference between the groups, with less participation and interaction in the population with CP.6 A study published by Palisano et al6 showed that social and community participation of children and youths with CP is associated with older age (13–21) and with children whose Gross Motor Function Measure (GMFM) scores were at level I as well as at levels IV and V. What does this mean for the group of children with CP at levels II and III? Being able to function socially in society and to have recreational activities have been delineated as core values in our public school systems. We, as physical, occupational, and speech therapists, have roles as consultants for accessibility, activity modifications, and assistive technology, and as advocates for inclusive environments.3 As an experienced physical therapist prior to becoming an adaptive ski instructor, I (KG) never would have considered putting a nonambulatory child with ataxic CP on skis. This personal adventure for me demonstrated that, as Neuro-Developmental Treatment (NDT)-educated therapists, we have a full understanding of our patient’s movement problems resulting from interaction of multiple systems leading to participation restrictions.2 Also, in sports, we can easily understand what the functional limitation is and problem-solve with persons in the sport of the child’s choice to help evaluate adaptations to facilitate sport participation. Skiing offers an element of moving through open space outdoors with speed, affecting the visual, vestibular, and postural systems in a beneficial and organizing manner. With the increasingly serious problem of childhood obesity and our knowledge that daily exercise has a positive impact on our quality of life, we have an important role both to help our children participate in sports and to encourage participation in a sport as a direction toward a long-term healthy lifestyle. In reviewing the literature for information on adaptive skiing and CP, an article published in 2006 demonstrated improvement in the GMFM score for ambulatory children with CP who had 10 weeks (one session per week) of adaptive downhill skiing (ADS).7 Other literature pertaining to CP, recreation, participation, or physical fitness was extremely limited.8 Sports commonly described were swimming and hippotherapy.9,10 Adaptive skiing became recognized by the Professional Ski Instructors of America (PSIA) in 1996 as a specialized area of ski instruction and certification for those individuals who were interested in teaching skiing to individual students with a vast range of physical and developmental challenges. When choosing adaptive skiing as a discipline in professional ski instruction, a person is required to take a series of courses in biomechanics, skiing, teaching, disability awareness, and knowledge of specialized ski equipment. There are three levels of certification; once completed, an individual can then become accredited as a trainer in adaptive skiing. The purpose of developing this body of knowledge for adaptive ski instructors is to provide education to the already accredited ski instructor on the specialized methods of teaching people with disabilities, including information on common medical diagnoses, visual impairment, cognitive impairment, biomechanics, and adaptive specific equipment, such as a mono ski for individuals who have limited mobility from the waist down (e.g., a spinal cord injury, multiple sclerosis, or amputations); a bi-ski for individuals that are considered more involved, such as a those with quadriplegia; or even a ski bra to help with abduction and ski tip stabilization. As an adaptive ski instructor (ASI), I (KG) functioned as an employee of a resort company under the direct supervision of RD. I served clients for the entire day and attended to all their daily needs (lunch, bathroom, emotional needs). A variety of tools, such as signing, flash cards, and gesturing, were used for children who were nonverbal. A phone or personal interview with the parents occurred prior to meeting the child. Initially, the children would often display insecurity and fear at either the novelty of the ski environment or the instructor, and the fitting of boots and skis that would occur together with the parents provided us all with the opportunity to engage in a playful manner with the child, similarly to what occurs in a therapy setting to ease a transition. Because the ski environment is typically high energy, fun filled, and happy, it is often the case that the separation of the parent and child goes smoothly. Once the thrill of moving through space freely occurs (through the use of adaptive equipment), the child is engrossed in the new experience, and parental separation typically is no longer an issue. We learn early on to get going with skiing! The principles of assessment, equipment selection, and ski instruction that I was taught match my NDT practice. Evaluation of the family and child’s needs as an individual, as well as the child’s function, body structure, strength, and cognitive function with respect to her medical restrictions, are analyzed to put the client into the safest, least restrictive adaptive equipment for a maximum ski experience. As part of my training, I was required to role-play different disabilities and use various pieces of adaptive equipment while trusting other trainees to lead me down the slope. This reminded me of all the handling laboratories and adaptive equipment laboratories we had as NDT students. The child, Patty Grace, featured in this case report did not receive physical therapy services from this therapist outside of the adaptive skiing program. This case report is a retrospective study. The author, KG (ASI instructor), was an instructor under the supervision of the author RD (director of an adaptive ski school). RD instructed Patty Grace in her beginning ski experience and knew the student and family from both ski school and the community. Patty Grace was selected as a person to demonstrate how, in spite of her significant locomotion restrictions, she was able from the beginning of adaptive skiing to participate as a skier on Vail Mountain and to progress quickly in participating with less physical support. Further, the remarkable point of this case study is to highlight the similarities of our NDT philosophies, assessments, and viewpoints on inclusion of function and participation with the sport of adaptive skiing. Patty Grace is a 9-year-old girl who has a diagnosis of ataxic CP as a result of glucose transporter type 1 (Glut1) deficiency.11 This deficiency is associated with intellectual impairment, visual impairment, gross and fine motor delay, speech and language delay, and seizures. She is on seizure medication. An evaluation of Patty Grace’s posture and movement, body systems, and functional activities took place in a community playground setting 4 years after she began skiing. The selection of a playground setting was determined based on Patty Grace’s ability to ambulate independently in her school with the supervision of her aide for safety. The playground provided an age-appropriate setting to observe her gait, spontaneous function, postures, and movements as well as age-appropriate sensory perceptual skills, communication, and behavior. Using the NDT Practice Model with the ICF model guiding organization of domains of functioning, Patty Grace’s functional activities in the playground setting were found to be as follows. Patty Grace can control her body posture and movement in sitting, in standing, and when walking on an uneven surface. She can climb up a slide and ascend and descend stairs, leading with her right leg, descending with her left leg, while holding a railing. Patty Grace uses her arms to assist her in all movement transitions, and places her hands appropriately on playground equipment to adjust her balance or position. Patty Grace’s receptive communication is good, and it far exceeds her verbal abilities to communicate. She often responds with “yes” or “no,” or points. Cognitively, she is functioning at the first-grade level. She now uses an iPad with audio application. Patty Grace attends but does not always direct her face toward the speaker. Socially, she is appropriate for her age. Patty Grace demonstrated very little rotation in her trunk for all movement transitions and moved in the sagittal plane. Her gait was wide-based, feet pronated, trunk aligned posteriorly to her hips, and her head often tilted to the right side. This posture contributed to her slow movement and poor coordination. An example of her functional limitation was her inability to sit on the teeter-totter with her feet flat because she needed to keep her legs widely abducted, which hampered her ability to initiate moving the teeter-totter into the air. Patty Grace demonstrated a loss of anticipatory control in activation of postural musculature. There was a delay in her ability to direct her body to a new piece of equipment on the playground and walk toward it. Toward the end of the playtime, her gait became slower, possibly due to postural control weakness and fatigue, which her mother reported as a contributing factor. Examination of her feet on the playground bench was conclusive for bilateral ankle pronation and heel cord tightness, with the right ankle at just less than 0° dorsiflexion and the left at 0° dorsiflexion. For her gross visual assessment, having Patty Grace track a small red ball from side to side across midline and in a circle demonstrated saccades of her right eye. She moved her eyes and head simultaneously. Thus, each time she directed her eyes, her head moved toward the target, which challenged her to sustain her postural control. From this cursory evaluation, it became apparent that Patty Grace’s movement patterns showed the following: • Limitations in movement planes—she moved in the sagittal plane only. • Force production difficulty of her postural muscles, especially her right hip flexor, and bilateral hip extensors. • Spatiotemporal impairments with a lack of adaptability, quickness, and reliability. • Visual function delays. Patty Grace also required monitoring for safety on the playground on unfamiliar equipment and for sudden seizure activity. Patty Grace was placed in an early intervention program at 4 years of age when the family moved to the Vail Valley in Colorado. She was nonverbal, could nod for yes or no, and was dependent on her caregivers. Patty Grace’s parents were avid skiers and worked as part-time ski instructors for Beaver Creek Resort. It was there that her parents learned of adaptive skiing and the Small Champions program for children with disabilities taught by adaptive ski instructors at the Vail Snow Sport School. Her parents were very apprehensive given that Patty Grace used a wheelchair and was nonambulatory, but they shared Patty Grace’s need to participate in what everyone was doing in the mountains and her love of movement and speed. Student assessment is the most important part of getting someone with a disability set up for skiing. It is very similar to what a physical therapist does before sessions, assessing and evaluating what will be worked on for that day. For the ski instructor to teach a successful lesson, the instructor must have a complete picture of the student’s abilities, movements, and goals. Gathering this information from the parents ahead of time at the initial phone reservation assists the instructor and program director to create a lesson plan and determine what type of adaptive equipment may be needed. Once the student arrives on site, the physical assessment and equipment fitting will be completed. The instructor must take into consideration the cognitive, affective, and physical aspects of the student. The instructor must look at the student’s stance and gait; balance fore, aft, and laterally; strength; and mobility to determine if a student will stand and ski or sit and ski. Then the medications that the student is taking and any associated side effects are assessed. Each adaptive program has a personalized assessment sheet or student information form and follow-up notes for future lessons, similar to a physical therapist’s evaluation and intervention plan. The assessment is guided by specific questions and discussion, and then moves into the physical aspects, such as muscular function and strength, to determine which muscle groups a student can use or cannot use or if there is a weakness from side to side, and how long they can remain active. Skills needed to ski must be assessed, and each person is assessed on how he or she is able to use the body for these skills. • Edging movements—movements that increase or decrease the edge angle of a ski are achieved through tipping parts of the body on a central axis—tipping the ankles, knees, spine and/or head (lateral weight shift). Any kind of tipping of a body part relative to the slope angle is inclination. A skier can incline the entire body into the slope or tip different parts to different degrees. • The next skill is turning movements or rotary skill—movements that increase, limit, or decrease rotation of the ski. These skills determine if a student can move the femurs in the acetabulum through closed chain lower extremity movements to create a circular motion about an axis—similar to bilateral integration. An atypical muscle synergy pattern, which will interfere with the sequencing of movements required in skiing, may be detected. This is common in the beginning lessons with a person with mild CP or hemiplegia or a student who is unable to maintain equilibrium. • The next skill is pressure control in movements—the pressure applied on the skis. This control or manipulation is achieved through leverage, flexion and extension, redistribution of weight from foot to foot, increasing and decreasing edge angle, and muscle tension, to allow changing a turn’s shape and size. To maintain the desired pressure, several movements are required from the body or accomplished through the aid of adaptive equipment. • The last skill to assess is dynamic balance—it is important for the body to be skeletally aligned. In skiing, alignment refers to the positioning of the body so that the forces derived from the interaction of the skis on the snow pass through the core or center of mass to produce the intended movement action or reaction. The slippery surface of the snow can cause issues for an individual, or it can enhance a more fluid motion. This motion is optimized when the skis, boots, or adaptive equipment is selected or modified to complement and/or to correct body movements to enhance strength and predictability of body movements. For example, if a student has severe alignment issues, these will need to be addressed before the student goes on the snow to create a flat ski from the start. The final assessment is a cognitive assessment. Can the student hear, understand, and answer questions? Is the student’s level of cognition age appropriate? Once a full student assessment is completed, the instructor can start to formulate a plan for adaptive equipment that may be needed to assist this person in skiing. Adaptive instructors are constantly assessing and reassessing throughout the day. Once on the slope, we use a process called movement analysis to assess a student’s ability, the movement patterns and skill blending to identify the cause-and-effect relationships of ski–snow contact. The instructor analyzes the separate components of the student’s movements to determine the skill and movements and identifies the steps and changes needed to produce the desired result, such as symmetrical turns or varying turn shape from medium to small. Patty Grace was ~6 years old, with very low postural tone, when she arrived for ski instruction. She had spent most of her time being transported in a wheelchair, and she was using ankle-foot orthoses (AFOs) for ankle stability to stand for short periods of time. She was unable to walk without the assistance of a walker. She had been told she would never walk independently. Cognitively she was delayed due to seizures. For Patty Grace, the bi-ski was the most suitable piece of equipment, with fixed outriggers on the side.12 The biski is an adaptive ski device that allows a skier to be seated and ski (Fig. B7.1). This device has articulating swing bow skis that allow for easy edge-to-edge movements. The fixed outriggers are placed on the side of the ski to facilitate the side-to-side movements but inhibit it from falling over. The design of the bi-ski and the abilities of the student and partnership with the instructor allow a person with a high level of disability to ski almost everywhere on the mountain. The bi-ski allows a student to participate in the ski lesson by leaning from side-to-side to engage the edges of the bi-ski. The more Patty Grace participated in skiing and following stand-up skiers, the more she understood the concept of skiing. This was the ski experience that was a catalyst for Patty Grace to want to stand and be more independent. Her goal was to stand and ski just like her brother and sister and everyone else (Fig. B7.2). As Patty Grace developed in muscle strength and in coordination, she was able to start to stand and ski with a piece of equipment called the Slider (Fig. B7.3).12 This piece of equipment is designed by Freedom Factory and is similar to a walker, but it has arm troughs and can be manipulated into 16 different positions to accommodate multiple disabilities. The concept for this piece of equipment was adapted from a hospital walker drilled onto a set of skis and has evolved to allow the student with a disability a wider range of movement and support, accommodating a larger population of individuals with disabilities. When a person transitions from adaptive equipment, it is important to redo an assessment. For her stand-up skiing assessment, Patty Grace used regular ski boots without her AFOs, so it was important to check the canting—was she standing on a flat foot or pronating or supinating? Could she plantar-flex or dorsiflex? Again, the assessment took into consideration the cognitive, affective, and physical aspects of her disability.
B7.1 Introduction
B7.2 Literature Pertaining to Sports with Children with Cerebral Palsy
B7.3 Professional Ski Instructors of America
B7.4 The Adaptive Skiing Experience
B7.5 Patty Grace’s Therapy Assessment
B7.5.1 Gross Motor Functional Activities, Participation—Social Functional Domain
B7.5.2 Communication Skills
B7.5.3 Social/Emotional Skills
B7.5.4 Postures, Movements, and Functional Limitations—Gross Motor
B7.5.5 Body Function and Structure
Musculoskeletal Control
Sensory Systems Tested—Vision
B7.5.6 Assessment
B7.5.7 Historical Information
B7.6 Skiing Assessment and Instruction
B7.7 Patty Grace Learns to Ski
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