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Cornelia de lange syndrome / maple syrup urine disease / Rubinstein-Taybi syndrome: behavior issues, activities, and conclusions.


Parts 1 and 2 of this series, addressed need to expand the body of knowledge with respect to providing appropriate physical education services to students with Cornelia de Lange syndrome Cornelia de Lange Syndrome aka CdLS is a little known genetic disorder that can lead to severe developmental anomalies. It affects both the physical and intellectual development of a child. Exact incidence is unknown, but is estimated at 1 in 10,000 to 30,000.  (CdLS), Maple Syrup Urine Disease ma·ple syr·up urine disease
n.
A hereditary metabolic disorder due to a deficiency of decarboxylase enzyme that leads to elevated concentrations of leucine, isoleucine, and valine in the blood and urine, characterized by the urine having an odor
 (MSUD MSUD maple syrup urine disease.
MSUD Maple sugar urine disease, see there
), and Rubinstein-Tayhi syndrome (RTS (Request To Send) An RS-232 signal sent from the transmitting station to the receiving station requesting permission to transmit. Contrast with CTS.

1. (operating system) RTS - run-time system.
2.
), so their individual and unique needs could be met. In Part 1, information was provided according to according to
prep.
1. As stated or indicated by; on the authority of: according to historians.

2. In keeping with: according to instructions.

3.
 etiologies of Cornelia de Lange syndrome, Maple Syrup Urine Disease, and Rubinstein-Taybi syndrome Rubinstein-Taybi syndrome (also known as Broad Thumb-Hallux syndrome) is a condition characterized by short stature, moderate to severe mental retardation, distinctive facial features, and broad thumbs and first toes. , and indicators of individuals with each of these conditions.

In Part 2, information was provided regarding general considerations and techniques for teaching physical education to individuals with any of these three genetic disorders The following is a list of genetic disorders and their origins. Beside most disorders is a code that indicates the type of fertilization and the chromosome involved.
  • P - Point mutation, or any insertion/deletion entirely inside one gene
, and then individual considerations and techniques pertinent to individual disorders so their unique needs can he met.

In this third and final plant of time series, information is provided regarding other physical factors, behavioral issues, suggested activities to he a part of time curriculum, and concluding information.

Other Physical Factors

For CdLS

Individuals with CdLS were reported to have a number of other physical problems requiring modifications in physical education programs.

Individuals with severe cases of CdLS may be born with missing fingers, hand, or arm; clubfoot clubfoot or talipes (tăl`əpēz'), deformity in which the foot is twisted out of position. Maldevelopment is usually congenital, although it can result from injury or disease (e.g., poliomyelitis) after birth.  or missing feet, and congenital dislocation of the hip (CdLS Foundation Inc., 1994). In milder cases, individuals often have small hands and feet with curving of the fifth finger, a short thumb placed closer than usual to the wrist, and some limitations of elbow movements (CdLS Foundations Inc., 1994; Halal ha·lal   Islam
n.
Meat that has been slaughtered in the manner prescribed by the shari'a.

adj.
1. Of or being meat slaughtered in the prescribed way: a halal butcher; a halal label.
, & Silver, 1992; Mosher A mosher is a person who is crossed between goth/punk/skater they have long hair and listen to music like slipknot and metal music. Some people call them headbangers. At certain music shows they have something called a mosh pit, basically its a fight pit with loads of people bashing each other.  et al., 1985; Wilson, Dasouki, & Barr, 1985). Webbing of one or more fingers, and dislocation of the upper end of the radius bone were also reported (CdLS Foundation Inc., 1994; Opitz, 1985). Opitz (1985) mentioned deep creases in the hands with many more distinct lines than normal. For those who are missing a limb, prosthetic pros·thet·ic
adj.
1. Serving as or relating to a prosthesis.

2. Of or relating to prosthetics.



prosthetic

serving as a substitute; pertaining to prostheses or to prosthetics.
 devices might assist in grasping and eye-hand activities. However, as reported, (CdLS Foundation Inc., 1994), children rarely utilized prosthetic devices with much success because of difficulty in maintaining a good fit due to rapid growth changes and difficulties in how to utilize the devices because of 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. . Individuals with more normal hands need to utilize smaller balls for throwing, and the physical educator needs to consider small hand sizes ill other activities.

Malformations in the legs and feet are reported to be less common and usually less severe. Individuals with CdLS have very small feet, often with webbing between second anti third toes (CdLS Foundation Inc., 1994; Filippi, 1989; Mosher et al., 1985; Opitz, 1985). This is usually not a problem in walking, but could become a factor in some physical education and sport activities requiring high levels of balance, such as walking a balance beam. Club-feet and congenital dislocations of the hip, if not corrected, necessitate major modifications in activities.

Two other physical problems reported were tight heel cords (CdLS Foundation Inc., 1994), and brisk deep tendon reflexes deep tendon reflex
n.
Abbr. DTR Tonic contraction of the muscles in response to a stretching force, due to stimulation of muscle proprioceptors. Also called myotatic reflex.
 in the lower limbs (Halal & Silver, 1992). Correction and stretching tight heel cords is important to give an individual a stable base of support for walking, and other upright activities (CdLS Foundation Inc., 1994). The adapted physical educator should work with the physical therapist to implement stretching and flexibility activities to deter tight heel cords and enhance movements. Activities in the swimming pool are excellent for muscular strengthening when brisk deep tendon reflexes are involved. The adapted physical educator needs to have excellent control of the temperature of both the pool and the room. Weber and Weber (1995) developed a pool exercise program entitled Aquacise: Fish Do It, successfully utilized with individuals with various disabilities involving tight tendons and brisk tendon reflexes.

For MSUD

According to Jackson, Kline, Barr, and Kock (1993), congenital heart disease congenital heart disease, any defect in the heart present at birth. There is evidence that some congenital heart defects are inherited, but the cause of most cases is unknown.  was diagnosed in about 25% of children with CdLS. Heart murmurs Heart Murmurs Definition

A heart murmur is an abnormal, extra sound during the heartbeat cycle made by blood moving through the heart and its valves. It is detected by the physician's examination using a stethoscope.
 were also reported by Mosher et al. (1985). When heart or respiratory problems are involved, it is imperative the adapted physical educator work closely with the student's physician. Program considerations and intensities of activities need to follow recommendations of the student's physician. A graded exercise program, similar to that used by heart attack clients, may need to be followed. Minimally, an age-adjusted target heart rate range should be monitored, and exercise precluded if respiratory or heart problems develop.

Many individuals with CdLS have feeding difficulties. Cates n. pl. 1. Provisions; food; viands; especially, luxurious food; delicacies; dainties.
Cates for which Apicius could not pay.
- Shurchill.

Choicest cates and the fiagon's best spilth.
- R. Browning.
 et al. (1989) stated feeding difficulties had been recorded in 70 to 100% reported cases of CdLS. These difficulties included frequent vomiting, difficulties swallowing, and aspiration (CdLS Foundation Inc., 1994). Time of day physical education classes are conducted, and how soon after a meal a class is scheduled impacts planning of physical education activities. Food or candy, which some teachers use for rewards, is contraindicated, due to feeding difficulties and fitness considerations. If proper nutrition proper nutrition,
n in Tibetan medicine, a therapeutic concept that begins with a digestive formulation because it is believed that a medical condition is primarily the result of a nutritional dysfunction or disturbance in the process of delivering nutrients.
 can not be maintained, then a student's energy and strength can be adversely affected.

According to Mosher et al. (1985), key to success for having a child with MSUD who is normally active, and one who has a cardio-respiratory problem is proper diagnosis and treatment within the first five days. Most of these children treated during the first five days are capable of leading active lives. They may tire easily, but this is believed to be more a problem with diet, than a physiological problem. However, children not diagnosed within the first five days seem to have problems with irregular breathing, and appear to be more susceptible to respiratory infections (McMahon & MacDonnell, 1990)

Although control of diet is frequently easier as the child gets older, intermittent infections can cause acute cardiovascular deterioration. Much like working with a person who is diabetic, the adapted physical educator or regular physical educator should work closely with parents and other school personnel in monitoring rest, diet, and amount of physical activity.

Sometimes over-protection of a child becomes so pronounced the child doesn't develop normally. Activities such as swimming, jogging, biking, rowing, canoeing, cross-country skiing cross-country skiing

Skiing in open country over rolling, hilly terrain. It originated in Scandinavia as a means of travel as well as recreation. The skies used are longer, narrower, and lighter than those used in Alpine skiing, and bindings allow more heel movement.
, aerobic dance, and other forms of endurance activities are recommended.

However, one should not be surprised if a child with MSUD attempts to avoid some of these activities, by complaining to parents about being tired. A graded exercise program, may need to be followed for persons having irregular breathing problems. If the physical educator teaches the child to self-monitor activity for her/his age-adjusted target heart rate, and precludes exercise if problems develop, no problems should arise.

Dietary considerations are essential for life, health, and physical endurance of individuals with MSUD (Naughten, Jenkins, Francis, & Leonard, 1982). Dietary considerations are directed toward controlling intake of the three branched-chain amino acids The phrase branched-chain amino acids or BCAA is sometimes used to refer to the amino acids having aliphatic side-chains that are non-linear. These are leucine, isoleucine and valine.  (BCAA BCAA Branched Chain Amino Acid
BCAA British Columbia Automobile Association
BCAA Branched Chained Amino Acids
BCAA Boulder County Arts Alliance (Boulder, CO)
BCAA British Columbia Assessment Authority
). Diets should be formulated to meet specific needs of the child, based upon degree of enzyme impairment, age, nutritional requirements nutritional requirements,
n the food and liquids necessary for normal physiologic function.
, and clinical state (Synderman, Norton, Roitman, & Holt, 1964; Elsas & Acosta, 1987; Bell, Chao, & Milne, 1979). Because BCAAs are essential, they cannot be deleted totally from the diet without causing absence of growth or death.

In planning nutritional support nutritional support,
n the supply of foods and liquids necessary to advance healing and support health.
 for a child with MSUD, a formal prescription including recommended amounts of BCAAs, protein, energy, and fluid for the day should be written. Adjustments in the dietary prescription may be necessary based upon appetite resulting from energy expenditures, fluid needs caused by perspiration, and other needs, such as growth, development, and laboratory analyses of plasma BCAAs and branched chain Noun 1. branched chain - an open chain of atoms with one or more side chains attached to it
open chain - a chain of atoms in a molecule whose ends are not joined to form a ring
 aketo acids. Since diet control is extremely important to maintain health and energy levels, it is important to have good communication with the child's parents. Physical educators or coaches interested in understanding dietary needs of children with MSUD are encouraged to read the Danner and Elsas (1989) chapter (Disorders of branched chai and keto acid keto acid /ke·to ac·id/ (ke´to) a carboxylic acid containing a carbonyl group.

ke·to acid
n.
A compound containing a ketone and a carboxyl group.
 metabolism) in the text edited by Scriver, Beaudit, and Valle, D. titled The Metabolic Basis of Inherited Disease (6th ed.), New York New York, state, United States
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
: McGraw-Hill, 671-692.

For RTS

Diagnosis for individuals with RTS had been established based on broad short terminal phalanges phalanges

plural of phalanx.
 of the thumbs and big toes, with or without angulations deformity Deformity
See also Lameness.

Calmady, Sir Richard

born without lower legs. [Br. Lit.: Sir Richard Calmady, Walsh Modern, 84]

Carey, Philip

embittered young man with club foot seeks fulfillment. [Br. Lit.
 (Rubinstein, 1990). Because hands are small when compared with many mainstreamed students, throwing and catching skills appear to be adversely affected (Weber, 1997). Since the feet are also small, flat, and have overlapped toes, standing balance in some activities is affected. An example is the young man previously studied would not jump, or if he did, fell down. Balance appeared to be a problem not only because of small size of the feet, but also because these individuals tend to be in the fifth percentile for their age and gender for height (Rubinstein, 1990), and obesity, especially in females (Partington, 1990). Combination of excessive weight on a short frame, and small feet cause 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).
. Balance is further complicated by spinal deviations reported for individuals with RTS. According to Hennekam, Van Den Boogaard, Sibbles, and Van Spijker (1990), 62% of these individuals have cervical hyperkyphosis, 16% lumbar hyperlordosis, 38% scoliosis Scoliosis Definition

Scoliosis is a side-to-side curvature of the spine.
Description

When viewed from the rear, the spine usually appears perfectly straight.
; spinal bifida has also been reported. Sherrill (1993) suggests a number of exercises addressing kyphosis kyphosis (kīfō`səs): see hunchback. , lordosis lordosis /lor·do·sis/ (lor-do´sis)
1. the anterior concavity in the curvature of the lumbar and cervical spine as viewed from the side.

2. abnormal increase in this curvature.
, and scoliosis. Hennekam, Van Den Boogaard, Sibbles, and Van Spijker (1990) report high incidences of fractures, as a result of slender bones, hypotonia hypotonia /hy·po·to·nia/ (-ton´e-ah) diminished tone of the skeletal muscles.

hy·po·to·ni·a
n.
1. Reduced tension or pressure, as of the intraocular fluid in the eyeball.

2.
, and an unsteady, stiff gait.

Behavioral Issues

For CdLS

Many behavior disorders in individuals with CdLS are reactions to something happening within the individual's body or their environment. These behaviors include attention deficit, hyperactivity hyperactivity, excessive physical activity of emotional or physiological origin, usually seen in young children; one of the components of attention deficit hyperactivity disorder. , hypoactivity, crying, yelling, and loud noises at inappropriate times disturbing others. As more and more of these individuals are involved in inclusion, behavior problems due to others making fun of their abnormal hands, feet, facial appearances, and hirsutism Hirsutism Definition

Excessive growth of facial or body hair in women is called hirsutism.
Description

Hirsutism is not a disease. The condition usually develops during puberty and becomes more pronounced as the years go by.
 could be more common.

It is believed an underlying biochemical disorder, associated with the syndrome, makes self-injurious behavior more likely (CdLS Foundation Inc., 1994). These severe behavioral problems are sometimes associated with seizure activity, or could be triggered by commonly prescribed medications. Some of these behaviors can be very difficult to deal with, therefore, a team approach is recommended. Parents, teachers, and all personnel in contact with the individual must agree, understand, and utilize an established plan for managing behaviors in all situations.

By utilizing task variations with interspersed-maintenance tasks recommended by Weber (1989), most individuals with CdLS appear to have fewer attention deficits. If a teacher utilizes bright red, yellow, or orange equipment with hypoactive, and black, brown, or dark blue equipment with hyperactive hy·per·ac·tive
adj.
1. Highly or excessively active, as a gland.

2. Having behavior characterized by constant overactivity.

3. Afflicted with attention deficit disorder.
 individuals, inappropriate behavior appears to be reduced. With older individuals, the teacher may be able to utilize a reward system. Kline et al. (1993) suggested use of computers, with emphasis on visual memory to be of benefit over verbal instruction. Since in the more mildly affected cases environmental factors might be a cause, it is important the physical educator maintains excellent control of a class and pays close attention to colors, sounds, weather changes, and other possible causes. With hearing and communication problems these individuals have, it is no surprise problems dealing with attention span are often reported by teachers and others.

For individuals with more difficult behavioral problems, Jansma and French (1994) suggested time-out, over-correction, extinction, satiation sa·ti·a·tion
n.
The state produced by having had a specific need, such as hunger or thirst, fulfilled.



sa
, deprivation, response cost, and corporal punishment corporal punishment, physical chastisement of an offender. At one extreme it includes the death penalty (see capital punishment), but the term usually refers to punishments like flogging, mutilation, and branding. Until c.  (like spanking spanking Pediatrics Corporal punishment, usually of children, in which the buttocks, are pummeled, swatted, or otherwise struck. See Corporal punishment Sexology Slapping, usually of the buttocks as a part of sexuoerotic activity. Cf Sadomasochism. ) as techniques to eliminate or weaken behaviors. However, if an individual becomes physically abusive to self or others, the physical educator may need to resort to physical restraint Physical restraint refers to the practice of rendering people helpless or keeping them in captivity by means such as handcuffs, shackles, straitjackets, ropes, straps, or other forms of physical restraint. .

As an individual enters junior and senior high school, where showering is often expected, additional problems, due to incomplete or delayed descent of testes testes
 or testicles

Male reproductive organs (see reproductive system). Humans have two oval-shaped testes 1.5–2 in. (4–5 cm) long that produce sperm and androgens (mainly testosterone), contained in a sac (scrotum) behind the penis.
 in males (Halal & Silver, 1992), can be expected. As more and more of these individuals are involved in inclusion, behavior problems due to others making fun of their abnormal hands, feet, facial appearances, and hirsutism could be more common. It is important all teachers intervene to keep other individuals from teasing an individual with CdLS, and inform them that these individuals have feelings like theirs, and may be extremely sensitive. Being exposed to stressful environments leave them vulnerable to behavioral situations (CdLS Foundation Inc., 1994). Commonly used stress management programs can probably be adapted.

For MSUD

Brubacher (1993) reported, "It is hard for these children and young adults to develop close friendships because they are very manipulative and possessive of persons showing them much attention." This can be difficult to deal with when parents are overprotective o·ver·pro·tect  
tr.v. o·ver·pro·tect·ed, o·ver·pro·tect·ing, o·ver·pro·tects
To protect too much; coddle: overprotected their children.
. Parents, teachers, and all other personnel in contact with the child must agree, understand, and utilize the set plan of dealing with these situations for proper behavior modification behavior modification
n.
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.
 to take place. Jansma and French (1994) suggested time-out, over-correction, extinction, satiation, deprivation, response cost, and corporal punishment as techniques to eliminate or weaken behaviors.

For RTS

One major teaching consideration when working with individuals possessing RTS is use of various behavior modification programs. Because of a tendency toward obesity and diabetes, 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. Parents and school personnel must closely observe these individuals, and not permit them to eat, except at scheduled meals.

Excessive mood and behavior changes are quite common. Partington (1990) reported most individuals with RTS could function fairly well in groups in home situations, but behaviors of some individuals with RTS throughout life were socially unacceptable. Based upon past experiences, a combination of social reinforcement and token economy Noun 1. token economy - a form of behavior therapy that has been used in some mental institutions; patients are rewarded with tokens for appropriate behavior and the tokens may be cashed in for valued rewards  incentives have been successful in controlling and changing behaviors.

Suggested Activities For Curriculum

Activities for CdLS, MSUD, and RTS

Like the student with CdLS entering junior and senior high school settings, similar problems can be expected; especially when showering because of incomplete or delayed descent of the testes in 87% of males with RTS (Rubinstein, 1990), and presence of hair in unusual places in 75% of individuals (Hennekam, Van Den Boogaard, Sibbles, & Van Spijker, 1990). Elementary school elementary school: see school.  students commented on stiff walking gait of the boy with RTS, used as a subject by the author. Some students said, "He walks like a robot." All teachers must intervene to prevent other individuals from teasing the individual with RTS, and inform them these individuals have feelings similar to theirs.

Like most youngsters, individuals with CdLS, MSUD, and RTS need and can benefit from activities involving muscular strength and endurance, flexibility, cardiovascular endurance, balance, agility, speed, coordination, and reaction time. These skills can be developed through much effort. Since most individuals with CdLS, MSUD, and RTS have learning difficulties, programming efforts should be predominately jogging, biking, dancing, and other forms of weight control activities that can be enjoyed with family members and peers in leisure time activities. Bowling, skating, angling, cross-country skiing, and swimming also have high priorities because they are lifetime activities the whole family can enjoy. Parts of the I CAN program (Wessel, 1976), or the newer achievement-based curriculum development in physical education (Wessel & Kelly, 1985) have been successfully utilized with individuals with all three of these genetic disorders.

The I Can program is excellent for working with these individuals. The program has eight boxes consisting of Aquatics, Fundamental Skills, Body Management Skills, Health/Fitness Skills, Dance Skills, Team Sports, Individual/Dual Sports, and Outdoor Activities. Each skill to be taught has a card providing information regarding what to do, what to say, materials needed to carry out successfully the activity, suggestions on how best to organize the class, and basic focal points for doing the task properly. The program also has an assessment tool, a checklist of all the focal points for each of the skills, which is very helpful in tracking the progress of each student. This checklist of focal points can become a rubric RUBRIC, civil law. The title or inscription of any law or statute, because the copyists formerly drew and painted the title of laws and statutes rubro colore, in red letters. Ayl. Pand. B. 1, t. 8; Diet. do Juris. h.t.  that is being required in many states around the country. These focal points can be put into your palm pilot to make assessment in the gym quick and easy. After marking down on my palm pilot in the gym what the student can do, I can then go to the office and download this information onto my computer to generate a ready-made progress note to share in IEP IEP

In currencies, this is the abbreviation for the Irish Punt.

Notes:
The currency market, also known as the Foreign Exchange market, is the largest financial market in the world, with a daily average volume of over US $1 trillion.
 meetings or with parents. I have also duplicated parts of skill progressions of various skills and sent them home as homework so parents, siblings, and friends can work with the student. Once the student has developed various skills, the I Can program also has a series of games that can be utilized for fun and to reinforce skills developed.

Summary of CdLS, MSUD, and RTS

Early diagnoses and treatments of various medical difficulties are important in attaining the highest functional levels of

individuals with CdLS, MSUD, and RTS. With a team approach and good communications between school and home, these individuals can be successfully involved in physical education. However, when teaching and developing programs to utilize with individuals having each of these genetic disorders, the physical educator needs to consider and address all their indicators, and not just the fact they are mentally retarded Noun 1. mentally retarded - people collectively who are mentally retarded; "he started a school for the retarded"
developmentally challenged, retarded
. In each of these genetic disorders there are various indicators, other than mental retardation that are just as significant, or more so, in impacting successful teaching of physical education, recreation, and sport skills.

General Information Available

Cornelia de Lange Syndrome: contact the Cornelia de Lange Syndrome Foundation, Inc., 60 Dyer Avenue Dyer Avenue:
  • Dyer Avenue, Tai Wan, Kowloon, Hong Kong http://www.centamap.com/gc/location.aspx?x=837379&y=818676&sx=837379.44062&sy=818676.53281&z=2
  • Dyer Avenue, Manhattan, New York City, New York, United States
 Collinsville, CT 06022-1273.

Maple Syrup Urine Disease: contact Joyce Brubacher, Newsletter Editor, Maple Syrup Urine Disease, 2406 SR 119, Goshen, IN 46526 or FAX (219) 862-2021.

Rubinstein-Taybi syndrome: contact Jack H. Rubinstein, MD, Director, University Affiliated Cincinnati Center for Developmental Disorders, Pavilion Building, Elland and Bethesda Avenues, Cincinnati, OH 45229; or Rubinstein-Taybi Parent Group, c/o Lorrie Baxter, P.O. Box 146, Smith Center, KS 66967; (913) 697-2984.

Acknowledgments--The author would like to thank Ms. Lynne Berube, Dr. Phillip Conatser, Mr. Matt Hanna Matt Hanna (b January 29, 1980) is a lacrosse player for the Denver Outlaws of Major League Lacrosse. Statistics
MLL

    Statistics
Season Team GP G 2ptG A Pts GB
2006 Denver 6 5 0 7 12 11 ALIGN="center" bgcolor="#e0e0e0"

Persondata
, Mrs. Heidi Harris Heidi Harris is a conservative radio talk host and columnist based in Las Vegas, Nevada. The "Heidi Harris Show" airs weekday mornings on NewsTalk 720 KDWN 8:30AM-Noon (kdwn.com). , Mr. Phil Hisey, Mr. Joseph Rogers, Mr. Paul Springer for their assistance in working with the students in the treatment part of the biomechanic research studies and digitizing, the parents of Scott Broderson, Zoe Georgopoulos and the parents of our students with CdLS. Additional thanks to the teachers and administrators of the Lubbock Independent School District Lubbock Independent School District is a public school district based in Lubbock, Texas (USA). Schools
High Schools
  • Lubbock High School
  • Coronado High School
  • Monterey High School
  • Estacado High School
Middle Schools
.

Selected References

Bell, L., Chao, E., & Milne, J. (1979). Dietary management of Maple Syrup Urine Disease: Extension of equivalency system. Journal of the American Dietary Association, 74,357.

Brubacher, J. (1993). The big picnic. Maple Syrup Urine Disease Newsletter, 11, 4-5.

Cates, M., Billmire, D. F., Bull, M. J., & Grosfeld, J. L. (1989). Gastro esophageal CdLS Foundation (1993). Facing the challenges: A family's guide to Cornelia de Lange Syndrome. Collinsville, CT: CdLS Foundation, Inc.

Cornelia de Lange Syndrome Foundation. (1994). Album. (Available from Cornelia de Lange Syndrome Foundation, Inc. 60 Dyer Avenue, Collinsville, CT 06022-1273).

Danner, D.J., & Elsas, L.J. (1989). Disorders of branched chain amino and keto acid metabolism. In Scriver, C.R., Beaudet, A.L., Sly, W.S., & Valle, D. (Eds). The metabolic basis of inherited disease (6th ed.). New York: McGraw-Hill, 671692.

Delsas, L.J., & Acosta, P.B. (1987). Nutritional management of inherited metabolic disorders. In Shils, M.E., & Yound, V. (Eds). Modern nutrition in Health and Disease (7th ed.). Philadelphia: Lea and Febiger.

Filippi, G. (1989) The de Lange Syndrome. Report of 15 cases. Clinical Genetics clinical genetics
n.
The study of the possible genetic determinants affecting the occurrence of diseases and disorders.
, 35,343-363.

Halal, F., & Silver, K. (1992). Syndrome of microcephaly microcephaly /mi·cro·ceph·a·ly/ abnormal smallness of the head.microcephal´ic

mi·cro·ceph·a·ly
n.
Abnormal smallness of the head. Also called nanocephaly.
, Brachmann-de Lange-like facial changes severe metatarsus adductus metatarsus adductus Forefoot varus Orthopedics A foot deformity characterized by a sharp inward angle of the front half of the foot; flexible deformity; the foot can be straightened and poses little risk for the infant; most cases resolve voluntarily; the rest , and developmental delay developmental delay
n.
A chronological delay in the appearance of normal developmental milestones achieved during infancy and early childhood, caused by organic, psychological, or environmental factors.
: Mil Brachmann-de Lange Syndrome? American Journal of Medical Genetics medical genetics
n.
The study of the etiology, pathogenesis, and natural history of diseases and disorders that are at least partially genetic in origin.
, 42, 381- 386.

Hennekam, R., Van Den Boogaard, M. J. Sibbles, B. J., & Van Spijker, H. G. (1990). Rubinstein-Taybi syndrome in the Netherlands. American Journal of Medical Genetics Supplement, 6, 17-29.

Jackson, L. G., Kline, A. D., Barr, M. A., & Koch, S. (1993). De Lange Syndrome: A clinical review of 310 individuals. American Journal of Medical Genetics Supplement, 47(7), 940-946.

Jansma, P., & French, R. W. (1994). Special physical education: Physical activity, sports, and recreation. Englewood Cliffs, NJ: Prentice-Hall, Inc.

Kline, A. D., Stanley, C., Belevich, J., Brodsky, K., Barr, M., & Jackson, L. G. (1993). Developmental data on individuals with the de Lange Syndrome. American Journal of Medical Genetics, 47(7), 1053-1058.

McMahon, Y., & MacDonnell, R.C. (1990). Advances in peritoneal dialysis peritoneal dialysis
n.
The removal of soluble substances and water from the body by transfer across the peritoneum, utilizing a solution which is intermittently introduced into and removed from the peritoneal cavity.
. Peritoneal Dialysis Bulletin, 6, 31-34.

Mosher, G. A., Schulte, R. L., Kaplan, P. A., Buehler, B. A., & Sanger, W. G. (1985). Brief clinical report: Pregnancy in a woman with the Brachmann-de Lange Syndrome. American Journal of Medical Genetics, 22,103-107.

Nanghten, E.R., Jenkins, J., Francis, D.E.M. & Leonard, J.V. (1982). Outcome of Maple Syrup Urine Disease. Archives of Diseases of Childhood, 101,553.

Opitz, J. M. (1985). Editorial comment: The Brachmann-de Lange Syndrome. American Journal of Medical Genetics, 22, 89-102.

Partington, M. W. (1990). Rubinstein-Taybi syndrome: A follow-up study. American Journal of Medical Genetics Supplement, 6, 65-21.

Rubinstein, J. H. (1990). Broad thumb-hallux (Rubinstein-Taybi) syndrome 1957-1988. American Journal of Medical Genetics Supplement, 6, 3-16.

Sherrill, C. (1993). Adapted physical activity, recreation and sport: Cross disciplinary and lifespan (4th ed.). Dubuque, IA: Wm. C. Brown Company.

Snyderman, S .E., Norton, P.M., Roitman, E., & Holt, L.E. (1964). Maple Syrup Urine Disease with particular reference to dietotherapy. Pediatrics, 34,454.

Weber, R. C. (1989). Utilizing task variation in adapted PE to motivate and teach Disabled students. Journal of Physical Education, Recreation and Dance, 60(2), 85-87.

Weber, R.C. (1997). Kinematic kin·e·mat·ics  
n. (used with a sing. verb)
The branch of mechanics that studies the motion of a body or a system of bodies without consideration given to its mass or the forces acting on it.
 analysis of the throw for Cornelia de Lange Syndrome) Unpublished raw data.

Weber, R. C., & Weber, N.J. (1995, May). Aquacise: Fish do it. Session presented at 10th International Symposium on Adapted Physical Activity, Oslo, Norway.

Wessel, J. (1976). 1 CAN program. Northbrook, IL: Hubbard Scientific Company.

Wessel, J., & Kelly, L. (1985). Achievement-based curriculum development in physical education. Philadelphia: Lea & Febiger.

Wilson, G. N., Dasouki, M., & Barr, M. (1985). Further delineation of the dup (3q) syndrome. American Journal of Medical Genetics, 22, 117123.

Robert C. Weber is 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.  at the University of Wisconsin Oshkosh. The Adapted Physical Education Program at the University of Wisconsin Oshkosh is a full minor program based on APENS APENS Association of Professional Engineers of Nova Scotia  competencies and student engagement with individuals with disabilities in all of the courses. Dr. Weber has been conducting research involving individuals with genetic disorders for the past 25 years and encourages those in our field to visit the new UWO UWO University of Western Ontario
UWO Unit Watts Out
UWO University Wisconsin Oshkosh
UWO Unix, Windows, OS/2 (DB2)
UWO Undersea Warfare Office
UWO Underwater Ordnance
UWO Under Will Of (legal) 
 Adapted Physical Activity Web Site http://www.uwosh.edu/adaptedpe

While direct measures were not taken, the children, staff, and KIN 320 students all benefited from the OutBAC experience. In general, the staff and course instructor recognized that most of the children who otherwise were easily distracted when performing other activities during the camp, were more focused on the OutBAC activities. This was especially true for many of the children with autism autism (ô`tĭzəm), developmental disability resulting from a neurological disorder that affects the normal functioning of the brain. It is characterized by the abnormal development of communication skills, social skills, and reasoning. . One reason may have been the novelty of the equipment intrigued them and kept their attention. Another reason might have been enjoyment of the climbing and balance activities. For example, the Whale Watch activity included balance in a moving medium similar to stepping off a sidewalk curb, getting on a bus, or moving on an escalator.

Other program benefits observed and discussed by students and staff were promotion of the camper's self esteem, problem solving problem solving

Process involved in finding a solution to a problem. Many animals routinely solve problems of locomotion, food finding, and shelter through trial and error.
, and learning how to work together to accomplish a task. A good example of an initiative where children learned to problem solve was the Whale Watch (see Figure 3) where they communicated and shared ideas while negotiating moving the equipment. Moving through the color-coded Spider Web reinforced spatial awareness, understanding color concepts, communication, and problem solving (see Figure 1). For many children who climbed over the Wall, there was a sense of accomplishment for completing an activity they were not sure they could complete (see Figure 9).

For staff studying to be adapted physical education specialists and physical education majors enrolled in KIN 320, each learned new activities to incorporate into future teaching. Many had never experienced adventure education, so this not only served as a wonderful introduction to this medium of teaching, but to its use for children with disabilities. Also learned was that this medium can easily be incorporated into other physical activity settings. Overall, staff and university students learned with a few slight modifications, children with disabilities can have successful experiences on a low ropes course.

Application to Physical Education Settings

Physical activity professionals can incorporate initiatives into existing physical activity programs without using expensive equipment or facilities. Professionals can use existing facilities, equipment, and/or purchase inexpensive supplies from a local hardware store. Examples follow as to how the professional can accomplish this.

Create a Spider Web from yarn or string, hang it from a volleyball stand, between two trees, or two poles (i.e., tetherball poles). With this apparatus, the professional can facilitate all of the previously mentioned activities. A Nitro Crossing can be a rope hung from a swing set, basketball goal, or any other sturdy horizontal support system. The rope needs to be soft and at least 3/4 "thick so it is gentle on the hands of participants.

A Plutonium Tub is a plastic tub, approximately 12X6" in size with holes drilled in it for strings to pass through. The Water Half Pipes are inexpensive 3" plastic drain pipes purchased from a hardware store and cut in half lengthwise length·wise  
adv. & adj.
Of, along, or in reference to the direction of the length; longitudinally.

Adj. 1. lengthwise
. Place the Water Half Pipes initiative on the floor, use 8' long 2X4" boards, or balance beams similar to the Plutonium Tub initiative.

Construct an All Aboard Platform from lumber found at any local hardware store. Exercise caution when building this piece of equipment since up to 13 participants could be on this initiative at the same time, and with adults it can be well over 2000 lb of body weight. A safer alternative is to use a carpet square or a folded tarp placed directly on the ground. Not only is it impossible to break, but unlike the traditional All Aboard Platform, use of a carpet square or folded tarp also reduces risk of injury from falling off a platform.

Professionals can create an obstacle course from existing equipment found in a physical activity program. For example, use balance boards, balance beams, tires, hula-hoops, crash mats, cheese wedges, mats, and noodles noo·dle 1  
n.
A narrow, ribbonlike strip of dried dough, usually made of flour, eggs, and water.



[German Nudel.
 to build a course. Most facilities do not have horizontal telephone poles on which students can balance, but low balance beams can serve as excellent alternatives. If those are unavailable, use opposing 8' 2X4" boards placed on the ground or floor. During construction, it is important for professionals to consider participant safety. The only limitation to developing an initiative is the creativity of professionals and participants.

Summary

Outdoor adventure courses can offer a variety of exciting and developmentally appropriate initiatives designed to meet each participant's physical, cognitive, and social needs (James, 1995). Creative modifications to initiatives assure that all participants, including individuals with disabilities, can successfully participate. With creativity, these initiatives can be incorporated into existing physical activity programs without using expensive equipment or facilities. For the professional who is new to outdoor adventure education, and those who have previously used it, an excellent source for information is Project Adventure (http:// www.pa.org). Practitioner and author Karl Rohnke has written several books on this topic including hundreds of warm-up activities, inclusive games, initiatives, and trust activities. Two good resources to start with are Quicksilver quicksilver: see mercury.


(1) (QuickSilver Technology, Inc., San Jose, CA, www.qstech.com) A mobile communications company that specializes in a reconfigurable logic chip for cellphones and PDAs. See adaptive computing.
 (Rohnke and Butler, 1995) and Silverbullets (Rohnke, 1984).

Selected References

James, T. (1995). Sketch of a moving spirit. In K. Warren, M. Sakofs, & J. Hunt (Eds.), The theory of experiential education. (pp. 85-96). Dubuque, IA: Kendal/Hunt.

Lavay, B. (2003). A university-based physical activity program for children with special needs: A unique blend of teacher training, instruction, and community service. PALAESTRA, 19 (2), 42-46.

Rohnke, K., Wall, J.B., Tait, C.M., & Rogers, D. (2003). The complete ropes course manual. Dubuque, IA: Kendall/Hunt.

Rohnke, K., & Butler, S. (1995). Quicksilver: Adventure games, initiative problems, trust activities and a guide to effective leadership. Dubuque, IA: Kendall/Hunt.

Rohnke, K. (1984). Silverbullets. Dubuque, IA: Kendal/Hunt. Project Adventure. (2004). Glossary of terms. Retrieved from http://www.pa.org on November 27, 2004.

Jeff Kress is an assistant professor in the Department of Kinesiology (KIN) at California State University Enrollment
, Long Beach, where his primary responsibility is to train students to teach secondary physical education. His duties in the KIN Department, where he has taught since 2000, include supervising student teachers and directing the Outdoor Beach Adventure Course. Barry Lavay is a professor in the Department of Kinesiology (KIN) at California State University, Long Beach, where his primary responsibility is to train students to teach physical education to individuals with disabilities. His duties in the KIN department, where he has taught since 1988, include coordinating the State Adapted Physical Education Teaching Credential Program and directing afterschool af·ter·school  
adj. often after-school
1. Taking place immediately following school classes: afterschool activities.

2.
 and summer physical activity programs for children with disabilities.
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