Physical education for children with Prader-Willi syndrome.Robert C. Weber is assistant professor of physical education at Texas Tech University. He is also coordinator of Adapted Physical Education Adapted physical education is a sub-discipline of physical education. It is an individualized program created for students who require a specially designed program for more than 30 days. Programs and sponsor for the wheelchair athletic club. In addition, he has been active with Special Olympics.
According to Public Law 94-142 (1975) all handicapped children are entitled to a free public education that meets their individual and unique needs, including physical education. Benefits of physical education for children with disabilities are widely documented in the literature (Cratty, 1989; Dunn & Fait, 1989; French & Jansma, 1982; Kalakian & Eichstaedt, 1982; Seaman & DePauw, 1989; Sherrill, 1986). Children with Prader-Willi syndrome Prader-Willi Syndrome Definition
Prader-Willi syndrome (PWS) is a genetic condition caused by the absence of chromosomal material from chromosome 15. The genetic basis of PWS is complex. have a need and can benefit from participating in physical exercise and leisure activity programs (Page, Stanley, Richman, Deal, & Iwata, 1983). Due to obesity and behavioral problems, children with Prader-Willi syndrome may seem disinterested in participating in physical education. Because of this apparent disinterest dis·in·ter·est
1. Freedom from selfish bias or self-interest; impartiality.
2. Lack of interest; indifference.
To divest of interest.
Noun 1. in physical activity and need for increased physical activity to control their weight, it is very important that these children have positive experiences in physical activities with friends, family members, and in various school programs. From results of recent studies, children with Prader-Willi syndrome can benefit from instruction in physical education and learn to enjoy physical activity (Weber & Miller, 1990; Weber, Bobo, Wolfe, & Robert, 1990).
During the past several years, the author has had the opportunity to work directly with several children with Prader-Willi syndrome. Due to a previous lack of research findings and information involving instructional methods for these children in physical education, the author has become actively involved with colleagues in research studies involving various physical fitness parameters and biomechanical analyses of gross motor skill The term gross motor skills refers to the abilities usually acquired during infancy and early childhood as part of a child's motor development. By the time they reach two years of age, almost all children are able to stand up, walk and run, walk up stairs, etc. performances of these individuals (Weber & Miller, 1990; Weber et. al., 1990). From these studies some key ideas have been developed that can be helpful to physical educators who teach students with Prader-Willi syndrome. To provide meaningful physical education experiences, physical educators should be knowledgeable about etiology, characteristics, teaching considerations, and suggested activities for the child with Prader-Willi syndrome.
In 1956, Prader, Labhart, and Willi described a new syndrome which has characteristics of obesity, short growth, poor development of genitalia genitalia /gen·i·ta·lia/ (jen?i-tal´e-ah) [L.] the reproductive organs.
ambiguous genitalia , and mental retardation mental retardation, below average level of intellectual functioning, usually defined by an IQ of below 70 to 75, combined with limitations in the skills necessary for daily living. , with deficiency in muscle tone in the neonatal period (Juul & Dupont, 1967). Since then what is commonly referred to as Prader-Willi syndrome has been reported in the literature under various titles such as Syndrome of Willi Prader (Royer, 1963); of Willi, Prader, and Labhart (Larbre, 1965); of Prader, Labhart, and Willi (Dunn, 1968); of Prader, Labhart, Willi, and Fanconi (Hooft, Delire, & Casneuf, 1966); and HHHO (Zellweger & Schneider, 1968).
This condition is considered to be very rare with Crnic, Sulzbacher, Snow, & Holm (1980) estimating an incidence of between 1 in 5,000 and 1 in 10,000 births. Cause of the syndrome has been traced to an abnormality, resulting in a deletion of chromosome 15 contributed by the father (Bonuccelli, Stette, Levitt, Levin, & Pyeritz, 1982; Butler & Palmer, 1983; Elder, Nichols, Hood, & Harrison, 1985; Fear, Mutton mutton, flesh of mature sheep prepared as food (as opposed to the flesh of young sheep, which is known as lamb). Mutton is deep red with firm, white fat. In Middle Eastern countries it is a staple meat, but in the West, with the exception of Great Britain, Australia, , Berry, Heckmatt, & Dubowitz, 1985; Kousseff, 1980; Ledbetter, Riccardi, Airhart, Strobel, Keenan, & Crawford, 1981; Ledbetter, Mascarello, Riccardi, Harper, Airhart & Strobel, 1982).
Prader-Will syndrome is characterized during infancy by hypotonia hypotonia /hy·po·to·nia/ (-ton´e-ah) diminished tone of the skeletal muscles.
1. Reduced tension or pressure, as of the intraocular fluid in the eyeball.
2. , weak cry, poor sucking, and failure to thrive Failure to Thrive Definition
Failure to thrive (FTT) is used to describe a delay in a child's growth or development. It is usually applied to infants and children up to two years of age who do not gain or maintain weight as they should. (Berry, Whittingham & Neville, 1981; Brissenden & Levy, 1973). These infants are considered behaviorally and psychomotorically to be retarded, especially in regard to developmental milestones of ability to hold the head erect, sitting, walking, and talking (Foster, 1971; France, Beemer, & Ippel, 1984). Obesity generally becomes prominent between one and four years of age, with face, lower trunk, and buttocks buttocks /but·tocks/ (but´oks) the two fleshy prominences formed by the gluteal muscles on the lower part of the back. more involved than the rest of the body (Cline, Williams, & Smith, 1970; Foster, 1984).
During childhood, the syndrome is characterized by abnormal increase in appetite, obesity, short stature, small hands and feet, malformed mal·formed
Abnormally or faultily formed. facial features, mental retardation, and motor delays (Crnic et. al., 1980; Zellweger & Schneider, 1968). Other symptoms can include hypogonadism Hypogonadism Definition
Hypogonadism is the condition more prevalent in males in which the production of sex hormones and germ cells are inadequate. , central nervous dysfunction, scoliosis Scoliosis Definition
Scoliosis is a side-to-side curvature of the spine.
When viewed from the rear, the spine usually appears perfectly straight. , abnormalities of the central visual pathways, and diabetes(Bray, Dahms, Swerdloff, Fiser, Atkinson,& Carrel Car·rel , Alexis 1873-1944.
French-born American surgeon and biologist. He won a 1912 Nobel Prize for his work on vascular ligature and grafting of blood vessels and organs. , 1983; Creel, Bendel, Wiesner, Wirtschafter, Arthur, & King, 1986; Hamilton, Scully, & Kliman, 1972; Hittner, King, Riccardi, Ledbetter, Borda, Ferrell, & Kretzer, 1982; Holm & Laurnen, 1981; Magana, 1984; Tamarkin, Abastillas, Chen, McNemar, & Sidbury, 1982).
According to Holm and Pipes (1976) and Otto, Sulzbacher, and Worthington-Roberts (1982), food-related behavior problems are common. Individuals with Prader-Willi syndrome have been known to steal and eat products such as dog food which typically are considered unappetizing (Holms, Moser, Halldorsson, Mack, Pont, & Matzilevich, 1972). Excessive appetitie exhibited by children with Prader-Willi syndrome is thought to be biologically based and results from a hypothalamic hypothalamic
pertaining to the hypothalamus.
hypothalamic-pituitary-adrenocortical axis disturbance which leads to obesity (Hall & Smith, 1972; Zellweger & Schneider, 1968).
Families of students with whom the author has worked have been forced to place locks on refrigerators, freezers, and kitchen cabinets. In addition, family members, teachers, and others working with or associated with these individuals must monitor and alter eating patterns.
Several studies have attempted to control obesity in children with Prader-Willi syndrome through dietary management with limited success (Bistrian, Blackburn, & Stanbury, 1977; Coplin, Hine, & Gormica, 1976; Page, et al., 1983; Pipes & Holm, 1973; Wodarski, 1985). This is particularly difficult according to Holm and Pipes (1976) since caloric caloric /ca·lo·ric/ (kah-lor´ik) pertaining to heat or to calories.
1. Of or relating to calories.
2. Of or relating to heat. requirements for weight maintenance in requirements for weight maintenance in Prader-Willi syndrome have been found to be 20-50% lower than in normal-weight individuals. Surgical procedures such as small intestine small intestine
Long, narrow, convoluted tube in which most digestion takes place. It extends 22–25 ft (6.7–7.6 m), from the stomach to the large intestine. bypass (Bristrian et. al., 1977) and gastric bypass gastric bypass
A surgical procedure used for treatment of morbid obesity, consisting of the severance of the upper stomach, anastomosis of the small upper pouch of the stomach to the jejunum, and closure of the distal part of the stomach. (Soper, Mason, Printer, & Zellweger, 1975) have been successful but are not recommended due to possible complications with dehydration, malnutrition, and liver damage.
After 15 years of age, individuals with Prader-Willi syndrome usually are very obese, have defiant scoliosis, and heart problems. Due to heart problems, which usually result in cardiorespiratory car·di·o·res·pi·ra·to·ry
Of or relating to the heart and the respiratory system.
Adj. 1. cardiorespiratory - of or pertaining to or affecting both the heart and the lungs and their functions; "cardiopulmonary failure, average age expectancy for these individuals is the late twenties. The oldest known individual having Prader-Willi syndrome lived to 42 years of age (Zellweger & Schneider, 1968). In addition, these individuals are also sexually underdeveloped and exhibit behavioral problems characterized by stubbornness and sudden outbursts of temper (Laurance, Brito, & Wilkinson, 1981).
Teaching Considerations and Techniques
The following teaching considerations, as well as techniques, are results of personal research completed and medical considerations. Children with Prader-Willi syndrome have so many different medical considerations that usually an individualized education plan taught by an adapted physical education specialist is needed. From past experiences of the author, the earlier the adapted physical educator and various other members of the motor development team, such as a physical therapist, can begin working with these children the better. Fortunately with passage of PL 99-457 (1986), this intervention can begin shortly after birth once Prader-Willi syndrome is diagnosed.
The adapted physical education specialist must collect as much information as possible about the student. Sources of information should include classroom teachers, aides, psychologists, parents, siblings, medical personnel, and others who have worked with the child. This sharing of pertinent information among physical educator, classroom teacher, and parents needs to be done daily so that food intake, medication, and behavior management behavior management Psychology Any nonpharmacologic maneuver–eg contingency reinforcement–that is intended to correct behavioral problems in a child with a mental disorder–eg, ADHD. See Attention-deficit-hyperactivity syndrome. can be monitored. In addition, it is important for the physical eductor to become well acquainted with the child so that a mutual trust and understanding can develop. This allows the physical educator to understand the child and to motivate him to participate in regular physical activity.
The teacher should assess abilities of each child on a regular basis and utilize teaching techniques that encourage the child to improve weaknesses. At the same time the child needs to use learning strengths with the teacher making necessary modifications and manually assisting if needed. Accurate records for each child on participation, skill acquisition, and behavioral management techniques utilized are important for monitoring progress and programs.
Dealing With Hypotonisity
Since these children at an early age are considered hypotonic hypotonic /hy·po·ton·ic/ (-ton´ik)
1. denoting decreased tone or tension.
2. denoting a solution having less osmotic pressure than one with which it is compared. (Berry, Whittengham, & Neville, 1981; Brissenden & Levy, 1973), and have early signs of motor delay (Stephenson, 1980), a program which encourages physical activity and motor patterning of various muscle groups is very important. Problems of hypotonisity and motor delay appear to be caused by ultrastructured abnormalities of the thick filaments of the sarcomere sarcomere /sar·co·mere/ (sahr´ko-mer) the contractile unit of a myofibril; sarcomeres are repeating units, delimited by the Z bands, along the length of the myofibril.
n. and increased osmiophilic density of Z lines (Afifi, 1969; Afifi & Zellweger, 1969). According to Afifi and Zellweger (1969), with muscle stimulation and patterning the hypotonic conditions of individuals with Prader-Willi syndrome at birth can be improved with age. Examples of early intervention ear·ly intervention
n. Abbr. EI
A process of assessment and therapy provided to children, especially those younger than age 6, to facilitate normal cognitive and emotional development and to prevent developmental disability or delay. techniques that have been successful involve sensory stimulation sensory stimulation,
n in acupuncture, the practice of inserting needles into skin and tissue to coax the body into using its energy to heal itself. , positioning and placement strategies, rhythmic sterotypes, utilization of objects to motivate actions, and good therapy hands (manual assistance) which have been recommended for hypotonic children by Cratty (1989).
Dealing with Obesity
According to Holm and Laurnen (1981), obesity generally becomes prominent at about two or three years of age, so parents and members of the motor team need to get these children into early patterns and routines of vigorous daily exercise. This is especially important in light of well documented food-related behavior problems and biological need for fewer calories (Holm & Pipes, 1976). Because of need for fewer calories, strict observation of calorie intake in conjunction with a minimum of two hours of vigorous daily activity are recommended (Weber et al., 1990). From observations made by the author and research by Weber et al. (1990), activities that can be engaged in on a regular basis with family members and/or peers tend to be most successful.
The extent of obesity that develops in these individuals is important because of other possible medical and mental considerations. Holm and Lauren (1981) reported that the more obese individuals with Prader-Willi syndrome become, the more prevalent and severe scoliosis becomes. Tamarkin et al. (1982) also reported that incidence and severity of diabetes increases with obesity.
However, one of the most interesting studies was that of Crnic et. al. (1980) regarding degree of obesity and intelligence. In this study it was reported that early intervention prevented obesity in one group of children with Prader-Willi syndrome, and that this group had significantly higher intelligence test scores than two other groups which did not have regular physical activity from an early age. These findings suggested that early factors of brain development (possibly related to myelination myelination /my·elin·a·tion/ (mi?e-lin-a´shun) myelinization.
my·e·li·na·tion or my·e·li·ni·za·tion
The acquisition, development, or formation of a myelin sheath around a nerve fiber. , neurodevelopment factors, and negative psychosocial factors) determine subsequent IQ in this syndrome.
Dealing with Mental Retardation
Individuals with Prader-Willi syndrome are mentally retarded, but degree of retardation varies. The adapted physical education specialist must be aware of degree of retardation of each student since this affects instructional techniques. Some teaching suggestions for individuals with mental retardation presented by Seaman and DePauw (1989) that have been found to be pertinent in trial and error experiences of the author with children possessing this syndrome include--
* Simplify and sequence instruction using short simple sentences.
* Praise attempt and reinforce performance with social praise (do not use food).
* Include structure and routine while stressing safety in the program.
* Use tactile and kinesthetic kin·es·the·sia
The sense that detects bodily position, weight, or movement of the muscles, tendons, and joints.
[Greek k cues and physical shaping if necessary.
* Provide opportunity for increasing emotional adaptability by systematically and slowly changing structure.
* Use demonstrations by teachers, aides, and other students.
While teaching new tasks, utilization of task variation with interspersed maintenance tasks has been highly successful. Examples of how to utilize the task variation teaching method with special populations in physical education are given by Weber (1987). Reduced student-teacher ratios are required to work effectively with these children due to the combination of physical, mental, and behavioral problems, so as to meet individual and unique needs.
Dealing with Visual Problems
Many different visual impairments have been associated with Prader-Willi syndrome, including convergent strabismus convergent strabismus
convergent strabismus (k , glaucoma, nystagmus Nystagmus Definition
Rhythmic, oscillating motions of the eyes are called nystagmus. The to-and-fro motion is generally involuntary. Vertical nystagmus occurs much less frequently than horizontal nystagmus and is often, but not necessarily, a sign of , cataracts, and ocular albinism albinism
Absence of the pigment melanin in the eyes, skin, hair, scales, or feathers. It arises from a genetic defect and occurs in humans and other vertebrates. Because they lack the pigments that normally provide protective coloration and screen against the sun's (Creel et. al., 1986; Futtenweit, Ritch, Teckhasaenee, & Nelson, 1986; Hittner et. al., 1982; Vukcevich, Adler, Hornblass, & Gombos, 1979).
According to Hittner et. al (1982), oculocutaneous albinoidism (absence of pigmentation pigmentation, name for the coloring matter found in certain plant and animal cells and for the color produced thereby. Pigmentation occurs in nearly all living organisms. in eyes, skin, and hair) is a component of the chromosome 15 deletion which causes Prader-Willi syndrome. Because of this condition these individuals are wear sunglasses for most activities. In addition, Creel et al. (1986) indicated that individuals with Prader-Willi syndrome have misrouting of optic fibers which can result in strabismus strabismus (strəbĭz`məs), inability of the eyes to focus together because of an imbalance in the muscles that control eye movement; also called squint. and nystagmus. Either one of these conditions could adversely affect throwing, catching, and any other eye-hand or eye-foot coordination skills. The development of cataracts has also been associated with these individuals who have diabetes.
Dealing with Other Physical Factors
Individuals with Prader-Willi syndrome are reported to have small feet and hands and be significantly shorter than peers (Butler & Meaney, 1985; Cline et. al., 1970). Differences observed in hand size and structure of middle and distal phalanges phalanges
plural of phalanx. of the fifth finger have been suggested as possible diagnostic tools for physicians suspecting this syndrome (Butler, Kaler, Yu, & Meaney, 1982). Because the hands are so small compared to the average mainstreamed student, throwing and catching skills appear to be adversely affected (Weber & Miller, 1990). Since the feet are also small, it has been the experience of the author that balance in some activities is affected. This appears to be a problem not only because of small size of the feet, but because these children tend to be 3 standard deviations below average peers for height and 3 standard deviations above average peers for weight (Cline et. al., 1970). Combination of excessive weight on a short frame and small feet causes balance problems, especially during locomotion locomotion
Any of various animal movements that result in progression from one place to another. Locomotion is classified as either appendicular (accomplished by special appendages) or axial (achieved by changing the body shape). . These individuals, according to the research of Weber and Miller (1990) and photographs in various reports, are knock-kneed, which may also affect dynamic and static balance.
Since cardiorespiratory problems are common in older individuals with Prader-Willi syndrome, activities such as swimming, jogging, biking, rowing, canoeing, and cross-country skiing are excellent with moderation. However, it is advisable that teacher and/or student closely monitor heart rate of the student and duration of activity. Until the student has built up endurance over a period of time, frequent rest periods may be needed. In addition, according to Bye, Vines, and Fronzek (1983), obesity hyperventilation syndrome hyperventilation syndrome Psychiatry 1 Panic disorder, see there 2. Tachy-dyspnea 'syndrome' A clinical complex affecting neurotics with anxiety attacks; the hyperventilation, in addition to the characteristic EEG changes–bilateral synchronous theta wave may be present, which the physical educator must keep in mind.
Use of Behavior Modification behavior modification
1. The use of basic learning techniques, such as conditioning, biofeedback, reinforcement, or aversion therapy, to teach simple skills or alter undesirable behavior.
2. See behavior therapy.
One major teaching consideration when working with individuals possessing Prader-Willi syndrome is use of various behavior modification programs. Because of tendency toward obesity, food and drinks commonly used in special education as rewards for good behavior Orderly and lawful action; conduct that is deemed proper for a peaceful and law-abiding individual.
The definition of good behavior depends upon how the phrase is used. are contraindicated. In the physical education setting, as well as time between classes, food scavenging scavenging
of anesthetic. See anesthetic scavenging. can be a problem. It is important that all school personnel closely observe these individuals and not allow them to eat food except at scheduled meals. In addition, excessive mood and behavior changes are quite common. Individuals with Prader-Willi syndrome older than 4 years of age are noted for excessive stubborness and negativism negativism /neg·a·tiv·ism/ (neg´ah-ti-vizm?) opposition to suggestion or advice; behavior opposite to that appropriate to a specific situation or against the wishes of others, including direct resistance to efforts to be moved. leading at times to violent outbursts of rage (Zellweger & Schneider, 1968). Based on past experiences, a combination of social reinforcement and token economy incentives work well to control and change behaviors.
As the student enters junior and senior high where showering is often expected, additional problems due to poor development of genitalia and secondary sex characteristics occur in individuals with Prader-Willi syndrome. This problem usually occurs because of taunting by other students, if the physical educator does not intervene to keep other students from teasing the individual (Hamilton et. al., 1972; Magana, 1984; Zellweger & Schneider, 1968).
Suggested Activities Physical and Motor Fitness
Like most youngsters, children with Prader-Willi syndrome need and can benefit from activities involving muscular strength, muscular endurance, flexibility, cardiovascular endurance, balance, agility, speed, coordination, and reaction time. These skills can be developed through a number of lead-up games and activities ranging from playing tag and parachute activities to weight training. Most physical and motor fitness activities utilized with mainstreamed children are appropriate; however, children with Prader-Willi syndrome, based on research of Weber et. al. (1990), may need to have more exercise compared to mainstreamed students due to physical and motor fitness problems. to physical and motor fitness problems.
Fundamental Motor Skills and Patterns
Since children with Prader-Willi syndrome have below average motor skills (Afifi & Zellweger, 1969; Stephenson, 1980; Zellweger & Schneider, 1968), it is important they be taught fundamental motor skills such as running, jumping, throwing, catching, sliding, hopping, galloping, skipping, and striking. These skills are important because they are building blocks for more advanced skills and games. Activities such as jogging, biking, and dancing are excellent weight control activities that can be enjoyed with family members and peers in leisure time activities.
In addition, the author has effectively utilized the Portage Guide to Early Education Curriculum (1975) and the I CAN program (Wessel, 1976) with these individuals.
Skills in Aquatics, Dance, Individual and Group Games, and Sports
Individual sports which children with Prader-Willi syndrome seem to enjoy include bowling, roller and ice skating, snow and water skiing, rowing, canoeing, swimming, and dancing. Most of these activities are excellent for cardiorespiratory endurance and weight control. These activities also have high priority because they are lifetime activities the whole family can enjoy. The I CAN program (Wessel, 1976) and Special Olympic Sports Skills Instructional Program have both been successfully utilized by the author.
The author has been successful, based on results of the study by Weber et al. (1990) and improvement on test results for the AAHPERD AAHPERD American Alliance for Health, Physical Education, Recreation, and Dance Fitness Test, in programming physical education activities for children with Prader-Willi syndrome. This has especially been true when a concentration of activities has been on basic skills, weight control, and lifetime activities. To be successful, however, it is important to intervene early, control weight through diet and exercise, and involve family, as well as friends of the student in regular cardiorespiratory endurance activities.
Those wishing general information regarding Prader-Willi syndrome are encouraged to write: Prader-Willi Syndrome Parents and Friends, Box 252, Long Lake, Minnesota Long Lake is a city in Hennepin County, Minnesota, United States. The population was 1,842 at the 2000 census. History
The first settlers arrived in Long Lake in early spring, 1855. 55356.
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Diminished pigmentation, especially of the skin.
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A condition, resulting from fertilization of a gamete containing a translocation chromosome by a normal gamete, in which a segment of the translocation chromosome is represented three times in each cell and a trisomic (15; 17) (q13; p 13.3) with apparent Prader-Willi syndrome but without Miller-Dicker syndrome. American Journal of Medical Genetics medical genetics
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New York, Middle Atlantic state of the United States. It is bordered by Vermont, Massachusetts, Connecticut, and the Atlantic Ocean (E), New Jersey and Pennsylvania (S), Lakes Erie and Ontario and the Canadian province of : McMillan.
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1. A piece composed for the development of a specific point of technique.
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Acknowledgements--The author would like to thank Dr. Maryln Miller for her assistance in the biomechanic research studies, Drs. Mike Bobo, Roy Wolfe, and Jacalyn Robert for their assistance in the exercise physiology exercise physiology
The study of the body's metabolic response to short-term and long-term physical activity. study recently completed, and the families of all the children who have allowed their sons or daughters to participate.