DOWN SYNDROMEQUARTERLY

VOLUME 6, NUMBER 2JUNE, 2001

ISSN 1087-1756

DOWN SYNDROME QUARTERLYDown Syndrome Quarterly (ISSN 1087-1756) isan interdisciplinary journal devoted to advancingthe state of knowledge on Down syndrome andwill cover all areas of medical, behavioral, andsocial scientific research. It is published in March,June, September, and December and is distributedby subscription to individuals, organizations, andlibraries. Copyright © 2001 by Down Syndrome Quarterly

CONTENTS• Original short papers describing current

research, including current or proposedprojects or the results of completed studies;

• Reviews of the literature in specialized areas;• Healthcare, including preventive medical

recommendations based on assessment of theliterature and considered opinions on whatconstitutes state-of-the-art practice;

• Editorial statements reflecting opinion on thestate of the field;

• Book reviews and longer book review articles;• Bibliographic compilations and/or lists of

current research studies in various specialties;• Abstracts of recently-published research

studies with critical commentary;• Letters to the editor;• Statements/suggestions regarding research

directions that may be most promising, or forwhich there appears a significant current need.

Subscription Rates: Individual: 1 Yr: $24; 2 Yr:$45; Library/Organization: 1 Yr: $48; 2 Yr: $90.Orders outside the US: in Canada/Mexico: Add$6/Year for postage; other foreign orders add $10per year. Single issues: $10. (US dollars Only.)Orders should be addressed to: Down SyndromeQuarterly, Samuel J. Thios, Ph.D., Editor,Denison University, Granville, OH 43023.Submission of Papers: Manuscripts should beaddressed to: Samuel J. Thios, Ph.D., Editor,Down Syndrome Quarterly, Denison University,Granville, OH 43023, 740-587-6338, Fax (740)587-6417. E-Mail: THIOS@DENISON.EDUPublication Policy and Author Information: Allmanuscripts are subject to anonymous peer review.Submit four copies of each manuscript (includingfour copies of illustrations, one of which should bean original). All copies should be clear, readable,and on paper of good quality. In addition tomailing address and telephone number, authorsshould supply their electronic mail address and faxnumber, if available. Manuscripts should be prepared according to theformat specified in the Publication Manual of theAmerican Psychological Association (1995, 4th ed.)accompanied by text on floppy diskette inWordPerfect (IBM compatible) or as an ASCII file.

PUBLICATION OF DOWN SYNDROMEQUARTERLY IS MADE POSSIBLE, IN PART,

BY THE SUPPORT OFTHE NISONGER CENTER

THE OHIO STATE UNIVERSITYAND

DENISON UNIVERSITY

VOLUME 6, NUMBER 2 • JUNE 2001

EDUCATIONJohn Rynders, Ph.D.

University of Minnesota

MEDICINEWilliam Cohen, M.D.

University of Pittsburgh

PSYCHOLOGYJohannes Rojahn, Ph.D.

The Ohio State University

THERAPIESLibby Kumin, Ph.D.

Loyola College in Maryland

ABSTRACTSDavid Smith, M.D.

Medical College of Wisconsin, andSt. Michael Hospital Milwaukee, WI

BOOK REVIEWSBarry M. Mitnick, Ph.D.

University of Pittsburgh

EDITOR IN CHIEF Samuel J. Thios, Ph.D.Denison University

EDITOR EMERITUS Mary Coleman, M.D.

EDITORS

Assistant to the EditorChristy Cox Trager

CONSULTING EDITORSAndrew Barclay, M.D.

University of KansasR. Dwain Blackston, M.D.

Emory UniversityGeorge T. Capone, M.D.

Kennedy Krieger InstituteBrian Chicoine, M.D.

Lutheran General HospitalRobert James Clayton, M.D.

Santa Rosa Medical CenterW. Carl Cooley, M.D.

Dartmouth-Hitchcock Medical Center

Allen C. Crocker, M.D.Children’s Hospital, Boston

Thomas E. Elkins, M.D.Louisiana State University

Charles J. Epstein, M.D.University of California,San Francisco

Terry Hassold, Ph.D.Case-Western Reserve University

Caryl Heaton, D.O. New Jersey Medical School

DeAnna Horstmeir, Ph.D.Ohio Department of Mental Retardation and Developmental Disabilities

Matthew P. Janicki, Ph.D. New York State Office of Mental Retardation and Developmental Disabilities

Connie Kasari, Ph.D.University of California, Los Angeles

Ira T. Lott, M.D.University of California, Irvine

Martin J. Lubetsky, M.D.University of Pittsburgh

Phillip Mattheis, M.D.University of Montana

Dennis McGuire, Ph.D.University of Illinois, Chicago

Robert J. Pary, M.D.Southern Illinois University

Bonnie Patterson, M.D.Cincinnati Center for Developmental Disorders

Siegfried M. Pueschel, M.D., Ph.D.,M.P.H.

Rhode Island HospitalNancy J. Roizen, M.D.

University of ChicagoWilliam Schwab, M.D.

University of WisconsinWayne Silverman, Ph.D.

Institute for Basic Research in Developmental Disabilities

David Smith, M.D.Medical College of Wisconsin, andSt. Michael Hospital Milwaukee, WI

Patricia C. Winders, BS, PTKennedy Krieger Institute

Down Syndrome Quarterly Internet Homepage Address: http://www.denison.edu/dsq

The mother of an infant with Downsyndrome recently asked aboutbeginning physical therapy for her child.She began the meeting by asking: “If westart physical therapy now, whatdifference will it make when my child is9 or 10 years old?” What a greatquestion! It is exactly how she shouldbe thinking about physical therapy, and,in fact, it is exactly how she should bethinking about all the services for herchild. She has focused on the long-termfunctional outcome for her child. Thatquestion and that focus have guided mywork for many years. This paper willanswer her question. What difference,indeed, will it make years from now,when a child is an adolescent or anadult, whether or not he or she hadphysical therapy as a child? This articlewill address the goal of physical therapyfor children with Down syndrome, andthen looking beyond that goal, willdiscuss an additional opportunity that isavailable to parents while their child isreceiving physical therapy.THE GOAL OF PHYSICALTHERAPY

Before discussing what the goal ofphysical therapy for children with Downsyndrome is, it is necessary first tounderstand what the goal is not. Thegoal of physical therapy is not toaccelerate the rate of gross motordevelopment. This statement is morecontroversial than it may initially seemto be. Many parents, many physicaltherapists and many insurance

The Goal and Opportunity of Physical Therapyfor Children with Down Syndrome

Patricia C. Winders1

North East, Maryland

DOWN SYNDROME QUARTERLYVOLUME 6, NUMBER 2, JUNE 2001

PAGES 1-5

companies assume that the value ofphysical therapy can be measured bywhether or not a child is achievingmotor skills more quickly. Sometherapeutic techniques promotethemselves by saying that children whoare treated with that technique developmotor skills earlier. If, however, onebegins with the premise that the goal ofphysical therapy is to accelerate the rateof gross motor development, then oneneeds to answer the question posed bythat mother. What difference will itmake in 9 or 10 years that a child withDown syndrome walked at 21 ratherthan 24 months of age? How will thatthree-month difference affect a child’slong-term functional outcome? I do notbelieve that it will make any differencewhatsoever, and, therefore, I do notbelieve that it is the appropriate goal forphysical therapy for children with Downsyndrome. The rate of gross motordevelopment in children with Downsyndrome is influenced by a number offactors, including:

• hypotonia • ligamentous laxity• decreased strength• short arms and legs.

These factors are determined bygenetics, and although some may beinfluenced by physical therapy, theycannot be fundamentally altered.

So then, what is the goal of physicaltherapy for children with Downsyndrome? Children with Downsyndrome attempt to compensate fortheir hypotonia, ligamentous laxity,

decreased strength and short arms andlegs by developing compensatorymovement patterns, which, if allowed topersist, often develop into orthopedicand functional problems. The goal ofphysical therapy is to minimize thedevelopment of the compensatorymovement patterns that children withDown syndrome are prone to develop.

Gait is a primary example.Ligamentous laxity, hypotonia andweakness in the legs lead to lowerextremity posturing with hip abductionand external rotation, hyperextension ofthe knees, and pronation and eversion ofthe feet. (See Figure 1.) Children withDown syndrome typically learn to walkwith their feet wide apart, their kneesstiff, and their feet turned out. They doso because hypotonia, ligamentouslaxity and weakness make their legs lessstable. Locking their knees, wideningtheir base, and rotating their feetoutward are all strategies designed toincrease stability. The problem is,however, that this is an inefficient gaitpattern for walking. The weight is beingborne on the medial (inside) borders ofthe feet, and the feet are designed tohave the weight borne on the outsideborders. If this pattern is allowed topersist, problems will develop with boththe knees and the feet. Walking willbecome painful, and endurance will bedecreased. Physical therapy shouldbegin teaching the child with Downsyndrome the proper standing posture(i.e., feet positioned under the hips andpointing straight ahead with a slightbend in the knees) when he is still veryyoung. (See Figure 2.) With appropriatephysical therapy gait problems can beminimized or avoided. (See Figure 3.)

Trunk posture is another example.Ligamentous laxity, hypotonia, anddecreased strength in the trunkencourage the development of kyphosis,which is often first seen when the childis learning to sit. Children with Downsyndrome typically learn to sit with aposterior pelvic tilt, trunk rounded andthe head resting back on the shoulders.(See Figure 4.) They never learn toactively move their pelvis into a vertical(upright) position, and therefore, cannot

1 Correspondence: Patricia C. Winters, PT, P.O. Box 433, North East, MD 21901. Email: wind3829@dnet.net Phone: (410) 398-9193 Fax: (410) 398-2680.

The appropriate goal of physical therapy for children with Down syndrome is not to accelerate their rateof gross motor development as is commonly assumed. The goal is to minimize the development ofabnormal compensatory movement patterns that children with Down syndrome are prone to develop.Early physical therapy makes a decisive difference in the long-term functional outcome of the child withDown syndrome. Beyond this goal, there is an additional opportunity that physical therapy makesavailable to parents. Because gross motor development is the first learning task that the child with Downsyndrome encounters, it provides parents with the first opportunity to explore how their child learns.There is increasing evidence that children with Down syndrome have a unique learning style.Understanding how children with Down syndrome learn is crucial for parents who wish to facilitate thedevelopment of gross motor skills as well as facilitating success in other areas of life including language,education and the development of social skills.

PATRICIA C. WINDERS2

hold their head and trunk erect over it. Ifthis posture is allowed to persist, it willultimately result in impaired breathingand a decreased ability to rotate the trunk.Physical therapy must teach the child theproper sitting posture by providingsupport at the proper level even before thechild is able to sit independently. (SeeFigure 5.) First, the therapist providesupper trunk support, then middle trunksupport, then support between the scapulaand the waist, then support at the waistand finally pelvic support. The supportprovided at each level keeps the spine andpelvis in proper alignment until the childdevelops the strength to hold that segmentin alignment himself. Appropriatephysical therapy can minimize problemswith trunk posture. (See Figure 6.)

develops optimal movementpatterns;

• should focus on gait, posture andexercise.

So the answer to that mother’squestion is that physical therapy for theyoung child with Down syndrome willmake an enormous difference not onlywhen the child is 9 or 10 years of age, butalso when he or she is an adolescent andan adult. It can and should result in adultswho are healthier and more functional.

Physical therapy services:• should be concerned with the child’s

long-term functional outcome;• should seek to minimize the

development of compensatorymovement patterns;

• should be based on a thoroughunderstanding of the compensatorymovement patterns that children withDown syndrome are prone to develop;

• should be strategically designed toproactively build strength in theappropriate muscle groups so thatthe child with Down syndromeFigure 3

Figure 2

Figure 1

Figure 4

Figure 5

Figure 6

for Parents and Teachers, provides acomprehensive, step-by-step guide toteaching reading to children with Downsyndrome. All of this work points to howimportant it is for parents to have anunderstanding of how their child assimilatesinformation so that they can be successfulpartners in their child’s learning.

It has been my experience in 20 years ofproviding physical therapy to children withDown syndrome that they do indeed learndifferently and that it is necessary to modifymy approach if I wish to obtain the bestresult. I consider it an important opportunityof my work to help parents begin tounderstand how their child learns. Thefollowing “tips” are provided from manyyears of working with children with Downsyndrome. They are offered to provide astarting point for both parents and therapiststo begin to explore the unique learning styleof the child with Down syndrome.

1. Children with Down syndromehave a decreased ability togeneralize. This means that a skilllearned in one setting does notnecessarily transfer to anothersetting. For instance, a child maybe quite competent climbing thestairs at home, but whenconfronted with stairs at the clinic,he or she may regress to a muchmore primitive stair-climbingstrategy until he or she hasrelearned the skill in the newsetting.

2. Children with Down syndromeneed information to be deliveredin small bite-sized pieces. It hasbeen my experience that if a childappears to have plateaued, theproblem is most likely to be thatthe next piece of information istoo large and needs to be furtherbroken down.

3. The setup is crucial and needs to beas close to perfect as possible.Children with Down syndromeneed structure, consistency and afamiliar environment if you hopeto get their best performance. Donot try something new orchallenging when the child is tired,hungry or not at his best for somereason. The quality of the workyou do together is more important

THE GOAL AND OPPORTUNITY OF PHYSICAL THERAPY

THE OPPORTUNITY OFPHYSICAL THERAPY

If physical therapy has achieved thegoal of minimizing the development ofabnormal movement patterns, it will haveinfluenced the health of the child withDown syndrome throughout the course ofhis or her life. But there is actually anopportunity beyond the development ofmotor skills of which parents may wish totake advantage while their child isreceiving physical therapy.

There is mounting evidence thatchildren with Down syndrome do notlearn in the same manner that typicalchildren do. They have a different styleof assimilating information, and,therefore, the usual methods ofinstruction are less effective. Thedevelopment of gross motor skills is thefirst learning task that the child withDown syndrome and his parents willface together. There are many otherchallenges to come including language,education, and the development of socialskills, but learning gross motor skills isthe first developmental challenge. Theopportunity is for parents to use thearena of gross motor development tobegin to understand how their childlearns. Knowing how to facilitate theirchild’s learning will be critical to theirsuccess in collaborating with their childthroughout his or her lifetime.

Wishart (1991), a psychologist atthe University of Edinburgh in Scotland,has done leading edge work in studyinghow children with Down syndromelearn. She writes:

Despite the absence of an adequatedevelopmental database, theory andpractice in this area have nonethelesscontinued to assume that the process oflearning in children with DS isessentially a slowed-down version ofnormal cognitive development. Anincreasing number of recent studies aresuggesting that this ‘slow development’approach may be ill founded and thatlearning may differ significantly instructure and organization from thatfound in ordinary children…(p. 28-29).

Infants with DS consistentlyshowed evidence of underperforming,with avoidance routines being producedon many of the tasks presented,

3

regardless of whether these were aboveor below the infant’s currentdevelopmental level. New skills, evenonce mastered, proved to beinadequately consolidated, oftendisappearing from the infant’s repertoirein subsequent months. Follow-up studiesusing a wider range of tasks providedadditional evidence of this tendency to‘switch out’ of cognitive tasks, withmany children failing on items whichshould have been well within theircapabilities and which had been passedin earlier sessions…(p. 29).

Regardless of whether these irregularperformance profiles reflect genuinedevelopmental instability or are the result offluctuating motivation in assessment-typesituations, it remains that if test behaviour istypical of behaviour in other, everydaysituations, development itself must becompromised. (p.29).

Investigation into the learning style ofchildren with Down syndrome is in its earlystages. Kumin (2001) and Oelwein (1995)also have made important contributions inthis area. In her book, Classroom LanguageSkills for Children with Down Syndrome: AGuide for Parents and Teachers, Kumindiscusses how the insights of HowardGardner can be applied to children withDown syndrome. Gardner’s book, Frames ofMind, presents the theory of multipleintelligences, which postulates thatintelligence is multi-faceted. The theoryholds that besides linguistic andmathematical intelligences, there are alsospatial, interpersonal and musicalintelligences, to mention only a few. Kuminnotes that it has been her experience thatmany children with Down syndrome learnwell using music. She has also written aboutthe unique learning style of children withDown syndrome, and how it pertains tolearning speech and language in her book,Communication Skills in Children withDown Syndrome: A Guide for Parents(Kumin, 1994).

Oelwein (1995) also has written aboutthe learning style of children with Downsyndrome and how it impacts education. Shehas highlighted the need to consciously assistchildren with Down syndrome with howinformation can be effectively filed, storedand retrieved. Her book, Teaching Readingto Children with Down Syndrome: A Guide

PATRICIA C. WINDERS

than the quantity. Minimizedistractions in the environment.

4. Follow the child’s lead. The childmust be motivated to perform aparticular skill. Trying to imposeyour will on a child with Downsyndrome is a losing game. I oftentry to model my style of interactionafter the parent’s. It is familiar to thechild and most likely to besuccessful.

5. Be attentive to how the child reactswhen learning new gross motorskills. Some children are cautious,and others are risky. A cautious childprefers to stay in one position, whilethe risky child prefers to be inmotion. For example, when learningto walk, the cautious child will wantlots of support and will be upset if heor she falls. The risky child will likewalking because it involvesmovement and will not be concernedabout support or care how manytimes he or she falls.

6. Know when to quit. Some childrenwill only give you two repetitions ata particular skill and then insist onmoving on. Other children willgladly give you a dozen repetitions.Set up the game so that the child issuccessful and avoid frustration.

7. Be strategic in planning your session.Practice what the child is ready tolearn. Tackle the most difficult skillsfirst before the child becomes tired.Alternate difficult skills with easierones to give the child time to recoverhis strength.

8. Be strategic in providing support.Children with Down syndrome tendto become quickly dependent onsupport. Provide as little support aspossible while still allowing the childto succeed and remove the support assoon as possible.

9. Skills will be learned grossly at firstand then refined. For instance,children will initially learn to walkwith a wide base and their feetexternally rotated. This is not theoptimal gait pattern, but it needs to beallowed initially and then refinedthrough the post-walking skills.

10. Do not interfere with anestablished skill in which the child

4

has achieved independence. Youwill not be successful inintroducing change and the childwill only experience you asnagging. Changes will need to bemade at the next level of motordevelopment. For instance, somechildren, instead of learning tocreep on both knees, learn to creepon one knee and one foot. Oncethis pattern has been establishedand the child is proficient in itsuse, you will not be successful inaltering it and will succeed only inangering the child. Teach the childto use both knees in climbing upstairs rather than interfering withthis established pattern.

11. Children with Down syndromelearn best through a gradual process.

a. Introduction of the new skill isthe first step. The new skillneeds to be introduced slowlyand carefully with the goalbeing simply to have the childtolerate the movement.

b. Familiarity is the second step. Inthis step the child becomesaccustomed to the skill and howit feels physically. This is the “Iget it” phase in which the childunderstands the game and whatis being asked of him or her.

c. Collaboration is the third step.The child increases hiscollaboration and cooperation,and at the same time support isdecreased.

d. Independence is the final stepwhere the child has mastered theskill and can perform itindependently without support.

These tips are offered tentatively,knowing that they are far from definitiveanswers. Much more research is needed tobegin truly to understand the learning styleof children with Down syndrome. It iscrucial, however, that parents gain skill infacilitating the learning of their child.Otherwise, as Wishart (1995) says, we“could run the risk of changing slow butwilling learners into reluctant, avoidantlearners.” (p. 62).

Parents who are newly assuming theresponsibility of caring for a child with Downsyndrome are confronted with a confusing

array of treatment options and opportunities.It can be difficult to know where to focuslimited time and resources. It is hoped thisarticle will provide parents and caregiverswith a starting point and a framework formaking decisions about what is important.They should think about proposed therapiesjust like the mother described in the firstparagraph, from the perspective of the child’slong-term functional outcome. Physicaltherapy is a crucial service, not because it willaccelerate a child’s rate of development, butbecause it will improve a child’s long-termfunctional outcome by preventing thedevelopment of abnormal movement patternsthat are likely to become even more seriousproblems in adolescence and adulthood.Secondly, because gross motor developmentis the first learning task a child faces, itprovides parents and other caregivers withthe opportunity to learn how a given childlearns. The long-term functional outcomeshould be the guide in decisions about whatto work on, and understanding of a child’slearning style should be the guide in how towork on them.

References

Gardner, H. (1983). Frames of Mind: thetheory of multiple intelligences. NewYork: Basic. Books.

Kumin, L. (2001). Classroom Language Skillsfor Children with Down Syndrome: AGuide for Parents and Teachers.Bethesda, MD: Woodbine House.

Kumin, L. (1994). Communication Skills inChildren with Down Syndrome: AGuide for Parents. Rockville, MD:Woodbine House.

Oelwein, P. (1995). Teaching Reading toChildren with Down Syndrome: AGuide for Parents and Teachers.Bethesda, MD: Woodbine House.

Winders, P. (1997). Gross Motor Skills inChildren with Down Syndrome: AGuide for Parents and Professionals.Bethesda, MD: Woodbine House.

Wishart, J. G. (1995). Cognitive Abilities inChildren with Down Syndrome:Developmental Instability andMotivational Deficits. In: C. J.Epstein, T. Hassold, I. T. Lott, L.Nadel, & D. Patterson (Eds.), Etiologyand Pathogenesis of Down Syndrome.New York: Wiley-Liss, Inc.

Wishart, J. G. (1991). Taking the initiative inlearning: a developmental investigationof infants with Down syndrome.International Journal of Disability,Development and Education, 38, 27-44.

Descriptions of the integration ofchildren with Down syndrome into schoolenvironments often make the claim thatthe task of integration grows moreformidable with the age of the specialchild. Children with Down syndrome aresaid to blend very well into preschool andprimary classrooms, but to requireaggressive social interventions in order toensure appropriate integration by thesecondary grades.

Because of the wide range offactors that affect social integration, thisobservation may be taken as more of acommonplace requiring careful studythan an established research conclusion.It will of course be harder to integratechildren with special needs intoenvironments that have little experiencewith them, as was likely the case inyears past. In a world in which childrenwith Down syndrome are included withtheir peers from day one, the need forcreating new integration will be less.Still, there can be little doubt that thecomplexity of environmental socialeffects grows with age, and that issuesof how young people acquire and actupon views of their disabled (or, better,differently-abled) peers are likely to beimportant in building successfulintegrated communities.

Thus, understanding of how normalchildren learn about special needs peers,and how educational tools can bedesigned to promote such understanding,would be an important contributor tosuccessful integration. One probablysmall if still important component of thatoverall process of social learning consists

Book Review

Barry M. Mitnick, Ph.D.Book Review Editor

Explaining Down Syndrome to School-Age Children

Tocci, Salvatore. (2000) Down Syndrome. Franklin Watts, a division of GrolierPublishing, New York, NY. 144 pages. Hardbound, $25. ISBN 0-531-11589-5. Canbe ordered from such online sources as Barnes & Noble and Amazon, Inc.

REVIEWED BY: Barry M. Mitnick, Ph.D.University of Pittsburgh

of written materials about Downsyndrome. The list of publications aimedat older school-age children has beenexpanding (see, e.g., Bowman-Kruhm2000; Bryan 1999; Gordon 1999; for anexcellent source book on adolescents withDown syndrome, see Pueschel andSustrova 1997).

There appears to be a market nichefor such materials as school and publiclibraries fill out their collections withbooks on a variety of health and societalissues. The Franklin Watts seriesincludes books on Alzheimer’s disease,autoimmune diseases, H.I.V., the humangenome project, leukemia, Parkinson’sdisease, and the focus of this review,Down syndrome. Because libraries tendto purchase multiple books from suchseries, ordering them off the lists, Iwould expect that the book on Downsyndrome, offered by a major schoolpublisher, will pop up in public andschool libraries all over the country.

ContentsAlthough Salvatore Tocci’s Down

Syndrome has several attractivefeatures, it ultimately fails to adequatelyaddress its topic. In part, its deficitsillustrate some of the key concerns inexplaining Down syndrome to school-age children.

Down Syndrome takes us through anumber of the standard areas that needto be covered by a review of issues inDown syndrome. After introductorychapters that provide a vignette of anunusual child with Down syndrome anda description of the manifestations of the

syndrome as well as a discussion ofsocietal roles and public sectorresponses, the book presents a chapteron the “causes” of Down syndrome.This is an explanation of the geneticbasis for the syndrome and it is doneclearly and accessibly. The book thenoffers a series of chapters that treatissues at different age levels. Thus thebook cycles through from infancy toearly school years to adolescence toadulthood. Chapters on “the family,” on“the future,” and on “myths and truths”fill out the book. There is also a glossaryand lists of sources, further readings,and resources on Down syndrome.

The author appears to have ajournalistic knowledge of Downsyndrome based on a limited number ofsources. The book lists only foursources, all books, that “were used forfactual information and anecdotal storiesabout children with Down syndrome”(p. 130). Four sources were used for abook that is 144 pages in length. Thepublication dates of the books are 1986,1995, 1996, and 1997 for this book witha year 2000 copyright in an area thatsees new publication and new researchresults every year. Possibly as a result ofthe skimpy source list, there are glaringomissions and poor judgments aboutplacement and content of discussions.

Down syndrome and the issuessurrounding it are complex, even forthose with medical or other specialtieswho spend a good deal of time withindividuals with the syndrome. That iswhy the national network of clinicsmaintains health care guidelines (thepreventive medical checklist), publishedin Down Syndrome Quarterly andwidely re-published elsewhere. Theguidelines even address some importantissues that are not purely medical. Thisbook completely ignores the checklist,which has become an essential referencerepresenting best practices in the area.Books about Down syndrome need to beskillful in managing this complexity,without ignoring it. That applies toschool-age children as well as to parentsand professionals who seek tounderstand the condition. A book forteens that explains Down syndrome

DOWN SYNDROME QUARTERLYVOLUME 6, NUMBER 2, JUNE 2001

PAGES 5-7

BARRY MITNICK

should not be a checklist, but it shouldcertainly include the guidelines asreference and make sure its discussion isnot at odds with them.

Problematic Aspects of the BookAs I will describe below, this book

is sensitive to some hot-button issues,e.g., the need to see people with Downsyndrome as diverse individuals with thesame rights as others. But the book notesthat infants can have hearing problemsthat must be checked and nevermentions them again. It is as if certainissues are compartmentalized by lifestage, when in fact they remain things tobe concerned about in later life as well.Yes, “early school-age children” need tobe checked for thyroid problems, as thisbook notes, but that may be too late —the proper times to check are at birth,during infancy, and at regular intervalsthereafter. In addition, the checks shouldextend through adulthood. Thediscussion of atlanto-axial problems isalso flawed. The defense could beoffered that this is just a book to explainDown syndrome, not a manual of carefor it. The response is that you have toget the facts right in order to explain thesyndrome. If you present some issues asissues at a certain age, that’s the waythey will be understood.

The book’s description of programdesign issues fails both to recognizesome of the complexity and adaptabilityof current approaches as well as to takea stand on the relative desirability ofcertain designs, given the child’scapabilities and needs. The book placesits discussion in the chapter on earlyschool years, although these issues arerelevant throughout the school years.And, indeed, the designs most likely tobe used will change over the years.

The first design described isinclusion, but the author hastens to notethat it can be “traumatic” with a need forthe teacher and other students to be“sensitive to the child’s needs” and to“make every reasonable effort to includethe child in classroom activitieswhenever possible” (p.53). The authorthen notes that, because it may take thechild with Down syndrome longer to

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learn than his or her peers, inclusion canmean placing an older child with Downsydrome in a class of children who areyears younger. The author goes on that“if the child becomes frustrated, learningwill not take place” and that the teachermay “need to rely on certain strategiesto a greater extent” (p. 54).

I do not understand why a teacher’sneed to rely on “certain strategies” is atall problematic; teachers must beproperly trained — this is not an issue,and I do not know why it is being raised.I read the tone of this whole section asimproperly cautionary and negative.Yes, full, unsupported inclusion is notlikely to be the solution for all childrenwith Down syndrome throughout theirschool careers, but I fail to see why thebenefits of making settings asinclusionary as possible are not properlytrumpeted here.

The author identifies five “possibleeducational environments” (pp. 56-60),from regular classrooms through variouslevels of use of “additional services” anda resource room. In my view, these donot exhaust the possibilities and they donot clearly present, for example, howchildren may be included using a varietyof supports ranging from aides to peersupports. To offer one example thatwould not be apparent from thepresentation in this book, children withDown syndrome can do adapted work ina regular class, but in a way thatintegrates them. Thus, the special childcan present his speech in each area ofthe public speaking curriculum, just asthe other students do, only his will beadapted, e.g., shorter and less complex.

In Tocci’s world, IEP’s are justwritten and implemented (pp. 20, 54-55). So why do peers of students withDown syndrome see conflict betweenparents and teachers and administratorsin so many settings? Why are thosemeetings so long, and why do they occurso often? In other contexts, why dospecial education teachers seem to be sofrustrated so often, and so hard-working? Tocci asserts that the childwith Down syndrome can be frustrated,and the teacher has to use specialmethods, but is the frustration coming

from a deeper source as systems fail toprovide the services they must? Can’t hetell us about the real world and how itworks? Kids are perceptive — they cantell the difference between mere formand reality. The author should tell ushow and why parents often get upset atschool districts that cannot or will notprovide adequate services; at teacherswho are poorly trained or simply do notcare (and how grateful and supportivethey are when the reverse occurs); at thecruel things that happen in schools whensome children are not recognized aspeers and allowed to participate fully inthe life of a school.

In the chapter on adolescence, Tocciasserts in a section on personal hygienethat “because of their dry skin, youngerchildren with Down syndrome may notuse soap to wash. But, during adolescence,soap becomes a necessity.” (p.70) Not usesoap with younger children?!

The discussion of work in thechapter on adulthood seems overlylimited and pessimistic. People withDown syndrome hold jobs all the time,and they do not need to be one of thehighest-functioning individuals with thiscondition in order to do so. Yet theauthor says “near-normal intelligence” isnecessary and goes on to a descriptionof sheltered workshops for the rest(pp.87-88). This represents an olderview of what the vocational experienceof an individual with Down syndrome isand can be.

Evidence of spotty and oftencareless editing appears throughout thebook. A book that is meant as a clearexplanation should not be edited in away that creates questions or introducesconfusions, even little ones. Forexample, a caption on page 21 under apicture of a sign that points to a“handicapped route” reads “Somepeople with Down syndrome may not beable to walk due to a medical problem.”The text makes no reference to this.What medical problem? The booksometimes does not get names as well asfacts right. On page 128, Tocci refers tothe “American Academy of PediatricsAssociation.” On p.137, in the resourceslist, Tocci makes the common mistake of

BOOK REVIEW 7

describing the National Association forDown Syndrome, a Chicago-areaorganization, as “a national association.”

In some places, vague languageseems to be the author’s choice of how topresent potentially complex subjects orfact-filled topics to younger readers. Forexample, in the section on questionabletherapies for Down syndrome the authordoes not use the names most commonlyapplied to them, and does not mention thenames of the physicians or therapists thatadvance them. Some, like piracetam, arejust left out. I wondered if this representeda poor choice by the author, or even aneditorial decision by a press worried aboutliability. How is anyone supposed toidentify what the author is referring to?For example, if someone writes about Dr.Turkel’s vitamin regimen in another work,how will adolescent readers of this bookrecognize that the author has evencovered it and related approaches?

I could not check all the facts in thebook against the latest research, but Ifound myself questioning the author’sclaims and/or recommendations in severalplaces. Tocci does not use the commonnames for types of hearing loss or fortypes of disease, but does describe them.The author then says, incredibly, in regardto putting tubes in infants’ ears,“Implanting these tubes is a relativelysimple procedure that can be done in adoctor’s office” (page 44). For infantswith Down syndrome?! Certainly not.

Perhaps the author does not want tolitter the text with medical terms, names,and other details; perhaps he thinks hislargely younger readers will be put offby that. But there are ways of handlingthis. First, the language in the text mustbe absolutely correct and precise.Second, the medical terms and namescan be placed in boxes, footnotes, orappendices. Vagueness by itself is nevera satisfactory solution.

Some Requisites for an AdequateIntroduction to Down Syndrome

for School-Age ReadersThe example of Tocci’s Down

Syndrome does suggest some featuresthat should be part of an adequateintroduction to Down synrome for

school-age children. If not part ofavailable readings, they should certainlybe in the relevant lesson plans as school-age children learn about their peers withspecial needs.

• Get the facts exactly right, and citethem. Give the readers more places toread, not only to expand the reader’sknowledge, but, especially for youngpeople and those new to the area, togive them additional explanations ofthe same things. In areas that changedue to new research and habilitativepractices that improve treatment, notethis and provide links so the readerscan get to the newest stuff. No bookpublished in 2000 or later with time-sensitive material should ignore theinternet. Yes, links drop. But the majorones should be there anyway, withcaveats and other ways of finding thesources (locations and phonenumbers, for example). The citationsshould include the health careguidelines and a URL to it (theguidelines are accessible fromhttp://www.denison.edu/dsq/) soreaders can get the latest version.

• Tell it like it is, names and all: justintroduce the abstruse parts in waysthat can be taken in steps, e.g., inboxes. Don’t talk down to yourreaders (or listeners) and don’tsimplify things in a patronizingway. It only generates morequestions, or confused ignorance.

• Answer tough questions about thingsthat young people observe directly.Why are the parents so upset whenthey talk to the special ed teachersand, especially, to theadministrators? How can life inschool be less than perfect for aspecial child? Don’t make believethat systems behave the way they aresupposed to. Adult systems don’t;why should those in schools andtreatment systems be any different?

• If the book aims at providing abetter understanding so that youngpeople can behave moreappropriately, give them explicitguidelines. What should I do ifthis person with Down syndrome

in my class comes over and triesto hug me? If the person has somebehaviors I find annoying, how doI approach that? Should I justignore them? How can I treat kidslike these as my peers? How can I“include” them? Special educationis not just a job for a specialeducation teacher. If we value ourpeers with special needs as wevalue all others, then the jobextends to us as well.

Although sections of this book areinnocuous and provide adequateintroductions to aspects of Downsyndrome, I would never risk putting itin a school library to serve as a primesource for someone’s essay for a biologyclass, or in a public library to serve as asource for a parent or relative of a childwith Down syndrome. Like some brandsof tires, this book should be recalled bythe publisher. Ultimately, a book forschool-age children about Downsyndrome must respect its readers asmuch as it says they should respect theirpeers with Down syndrome.

References

Bowman-Kruhm, Mary. (2000). Everything YouNeed to Know about Down Syndrome. TheRosen Publishing Group, Inc., 29 East 21stStreet, New York, NY 10010. Hardcover.ISBN 0-8239-2949-3.

Bryan, Jenny. (1999). Living with DownSyndrome. Raintree Steck-VaughnPublishers, P.O. Box 26015, Austin, TX78755. Hardcover. ISBN 0-8172-5569-9.

Gordon, Melanie Apel. (1999). Let’s Talk aboutDown Syndrome. The Rosen PublishingGroup,

Inc., 29 East 21st Street, New York, NY 10010.Hardcover. ISBN 0-8239-5197-9.

Pueschel, Siegfried M. and Sustrova, Maria, eds.(1997). Adolescents with Down Syndrome:Toward a More Fulfilling Life. Paul H.Brookes Publishing Co., P.O. Box 10624,Baltimore, MD 21285-0624. Paper. ISBN1-55766-281-9.

Mission The Down Syndrome Medical Interest Group (DSMIG) was founded in early 1994 with the expresspurpose of serving as a forum for professionals addressing aspects of medical care of persons with Down syndrome.DSMIG wishes to promote the highest quality care for children and adults with DS 1) by fostering and providingprofessional and community education; 2) by disseminating tools for clinical care and professional support; such asthe Health Guidelines for Individuals with Down Syndrome; 3) and by engaging in collaborative clinical researchregarding issues related to the care of individuals with Down syndrome.

For further information, contact either co-chair: Bonnie Patterson at 513-559-4691 or Bill Cohen at412-692-6546. If you are interested in being added to our mailing list, please send your name, professional title,agency, address, telephone number, fax number, and email address (if any) to William I Cohen MD, DownSyndrome Center, Children’s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213. (412-692-6546; fax412-692-5679; email: cohenb@chplink.chp.edu).

News from the Down Syndrome Medical Interest Group (DSMIG)

William I. Cohen, M.D. Down Syndrome Center, Children’s Hospital of Pittsburgh

Bonnie Patterson, M.D. Cincinnati Center for Developmental Disorders

Co-Chairs

It was a busy spring and summer forthe DSMIG.

A group of members were invited toparticipate in the II InternationalConference on Chromosome 21 andMedical Research on Down Syndromein Barcelona. This included Drs. DavidPatterson, Ira Lott, George Capone,Sally Shott, Bonnie Patterson and BillCohen. Our colleague, Dr. AugustinSeres-Santamaria, medical director ofthe Fundacio Catalana Sindrome deDown invited us to host DSMIGmeeting immediately preceding theconference. Over 35 individuals were inattendance, and we were delighted tohave an opportunity to meet with ourinternational colleagues from Europe,Asia and South America.

Many of us participated in theNational Down Syndrome Societyconference in San Diego, “One Vision,One Voice.” Close to 900 individualsattended this meeting, which wasdesigned for parents, professionals,individuals with DS and families.

Close to 60 individuals attended theDSMIG meeting on Sunday, July 8,2001. There were two main foci:discussion of Health Care Guidelines for

upcoming revision, and discussion of theother guidelines under development. PatWinders and Alice Shea presented theoverall framework for their guidelineson Gross Motor Development, as well asthe specifics for children from birth towalking. Pat and Alice will be workingon the other age groups, following thesame overall format.

Libby Kumin presentedSpeech/Language guidelines in detail,soliciting revisions and editorial changesto reflect the variability of development ofcommunication abilities of children withDown syndrome. In addition, Libbydescribed a project to develop norms forspeech/language development for childrenwith Down syndrome. Interestedindividuals should contact Libby at 410-617-7623 or LBKumin@aol.com

Copies of the Occupational Therapyguidelines, prepared by MaryanneBruni, OT(C), were distributed. Ms.Bruni, who was not in attendance, hadprepared these previously, and they werediscussed briefly.

Lastly, Bonnie Patterson distributed adraft of Behavioral Health Guidelines asprepared by her, George Capone and DavidSmith Ph.D. DSMIG members were asked

to review them and send comments to theauthors (pattb0@chmcc.org)

Dawn McKenna gave a briefupdate on the database project of DSRF(Vancouver, BC). David Rubenson ofthe RAND Corporation discussed theStanford project. Several DSMIGmembers, (David Patterson, BonniePatterson, Julie Korenberg, Ira Lott,Len Leshin, Bill Cohen) participated ina planning meeting at the end of May inPalo Alto to discuss a mechanism forintegrating basic and clinical science inthe area of Down syndrome studies.The planning process for that programis continuing, under the direction Dr.Bill Mobley, Chair of Neurology atStanford University.

The afternoon session included threepresentations: Peter Elliott of the DownSyndrome Research Foundation (UK)described a prospective study of anti-oxidants and/or folinic acid to “preventcomplications of Down syndrome.” Theprincipal investigators are xx, yy, zz, fromthe Institute of Child Health.

Dr. Kasuzo Iinuma (I.G. Clinic,Tokyo) presented a poster entitled“Accuracy of risk evaluation for apregnant woman and scientific attitudesof physicians.” Dr. Iinuma’s co-investigator was K. Shimomura.

Lastly, Bill Cohen described aproject in which DSMIG members andlocal community resources provide‘corrective educational experiences forgenetics counseling graduate students.

The next meeting of DSMIG will beheld in conjunction with the NDSS/DSRF(Vancouver, BC) scientific conference oncognition and behavior, scheduled to takeplace in the fall of 2002 in Denver, CO.Watch this column for more information

NDSS announces Charles J EpsteinDown Syndrome Research Award. Thisprogram replaces the NDSS ScienceScholar Award, and provides “seed moneyin grants of $5,000 to $35,000 to scientistsand clinicians who seek to gain a betterunderstanding of Down syndrome and toincrease the knowledge base about thisgenetic condition.” For an application,contact NDSS at 1-800-221-4602.

DOWN SYNDROME QUARTERLYVOLUME6, NUMBER 2, JUNE 2001

PAGES 8-8

CARDIOLOGYEidem, B. W., Jones, C., & Cetta, F. (2000).

Unusual Association of HypertrophicCardiomyopathy with CompleteAtrioventricular Canal Defect andDown Syndrome. Texas HeartInstitute Journal 27, 289-91.

Formigari, R., Gargiulo, G., & Picchio, F. M.(2001). Operation for PartialAtrioventricular Septal Defect: AForty-Year Review. Journal ofThoracic & Cardiovascular Surgery121, 398-9.

Kwiatkowska, J., Tomaszewski, M.,Bielinska, B., Potaz, P., & Erecinski, J.(2000). Atrioventricular Septal Defect:Clinical and Diagnostic Problems inChildren Hospitalised in 1993-1998.Medical Science Monitor 6, 1148-54.

Schmidt, V., Wolter, M., Lenschow, U., &Kienast, W. (2001). LactobacillusParacasei Endocarditis in an 18-Year-Old Patient with Trisomy 21,Atrioventricular Septal Defect andEisenmenger Complex: TherapeuticProblems. Klinische Padiatrie 213,35-8.

COMMENT: German.

COMMUNICATIONAbbeduto, L., Evans, J., & Dolan, T. (2001).

Theoretical Perspectives on Languageand Communication Problems inMental Retardation and DevelopmentalDisabilities. Mental Retardation &Developmental Disabilities ResearchReviews 7, 45-55.

COMMENT: ReviewCarlstedt, K., Henningsson, G., McAllister,

A., & Dahllof, G. (2001). Long-TermEffects of Palatal Plate Therapy onOral Motor Function in Children withDown Syndrome Evaluated by VideoRegistration. Acta OdontologicaScandinavica 59, 63-68.

COMMENT: Does a statistically significantlong-term effect on oral motorfunction translate into a clinicallysignificant effect on speechintelligibility and communication?

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DENTALAllison, P. J., Hennequin, M., & Faulks, D.

(2000). Dental Care Access amongIndividuals with Down Syndrome inFrance. Special Care in Dentistry 20,28-34.

Hanookai, D., Nowzari, H., Contreras, A.,Morrison, J. L., & Slots, J. (2000).Herpesviruses and PeriodontopathicBacteria in Trisomy 21 Periodontitis.Journal of Periodontology 71, 376-384.

DERMATOLOGYDourmishev, A., Miteva, L., Mitev, V.,

Pramatarov, K., & Schwartz, R. A.(2000). Cutaneous Aspects of DownSyndrome. Cutis 66, 420-4.

Schepis, C., Siragusa, M., & Alberti, A.(2000). Guess What! Milia-LikeIdiopathic Calcinosis Cutis. EuropeanJournal of Dermatology 10, 637-8.

EDUCATION & THERAPYPalisano, R. J., Walter, S. D., Russell, D. J.,

Rosenbaum, P. L., Gemus, M.,Galuppi, B. E., & Cunningham, L.(2001). Gross Motor Function ofChildren with Down Syndrome:Creation of Motor Growth Curves.Archives of Physical Medicine &Rehabilitation 82, 494-500.

ENDOCRINOLOGYAnneren, G., Tuvemo, T., & Gustafsson, J.

(2000). Growth Hormone Therapy inYoung Children with Down Syndromeand a Clinical Comparison of Downand Prader-Willi Syndromes. GrowthHormone & IGF Research 10, S87-91.

COMMENT: A review.Konings, C. H., van Trotsenburg, A. S., Ris-

Stalpers, C., Vulsma, T., Wiedijk, B.M., & de Vijlder, J. J. (2001). PlasmaThyrotropin Bioactivity in Down’sSyndrome Children with SubclinicalHypothyroidism. European Journal ofEndocrinology 144, 1-4.

COMMENT: TSH bioactivity is normal. Theyconclude that subclinical hypothyroidismis of thyroid (primary) origin.

EPIDEMIOLOGYTorfs, C. P., & Christianson, R. E. (2000).

Effect of Maternal Smoking and CoffeeConsumption on the Risk of Having aRecognized Down SyndromePregnancy. American Journal ofEpidemiology 152, 1185-91.

Verloes, A., Gillerot, Y., Van Maldergem, L.,Schoos, R., Herens, C., Jamar, M.,Dideberg, V., Lesenfants, S., &Koulischer, L. (2001). Major Decreasein the Incidence of Trisomy 21 atBirth in South Belgium: Mass Impactof Triple Test? European Journal ofHuman Genetics 9, 1-4.

COMMENT: There has been a shift in theincidence of trisomy 21 at birth from1/794 to 1/1606.

GASTROENTEROLGYBianca, S., & Ettore, G. (2000). Anorectal

Malformations and Down’sSyndrome. Paediatric & PerinatalEpidemiology 14, 372.

Csizmadia, C. G., Mearin, M. L., Oren, A.,Kromhout, A., Crusius, J. B., vonBlomberg, B. M., Pena, A. S.,Wiggers, M. N., & Vandenbroucke, J.P. (2000). Accuracy and Cost-Effectiveness of a New Strategy toScreen for Celiac Disease in Childrenwith Down Syndrome. Journal ofPediatrics 137, 756-61.

COMMENT: The authors suggest testingtwice for celiac disease. In their studythey tested 2 years apart. Prevalencewas 8% (11 individuals). I still onlyhave one person with celiac diseaseand he had symptoms.

Jennings, J. S. R., & Howdle, P. D. (2001).Celiac Disease. Current Opinion inGastroenterology 17, 118-126.

Salur, L., Uibo, O., Talvik, I., Justus, I.,Metskula, K., Talvik, T., & Uibo, R.(2000). The High Frequency ofCoeliac Disease among Children withNeurological Disorders. EuropeanJournal of Neurology 7, 707-711.

COMMENT: They had 3 cases of celiacdisease out of 206 children withneurological disorders and at least onehad Down syndrome. Is the increasedfrequency of celiac disease due to anyneurologic disorder or Downsyndrome?

Walker-Smith, J. A. (2000). Celiac Diseaseand Down Syndrome. Journal ofPediatrics 137, 743-4.

COMMENT: A letter.

GENETICSCapone, G. T. (2001). Down Syndrome:

Advances in Molecular Biology andthe Neurosciences. Journal ofDevelopmental & BehavioralPediatrics 22, 40-59.

Abstracts/References

David Smith, M.D.Abstracts Editor

DOWN SYNDROME QUARTERLYVOLUME 6, NUMBER 2, JUNE 2001

PAGES 9-12

DAVID SMITH

COMMENT: ReviewLazzaro, S. J., Speevak, M. D., & Farrell, S.

A. (2001). Recombinant DownSyndrome: A Case Report andLiterature Review. Clinical Genetics59, 128-130.

Nadal, M., Vigo, C. G., Melaragno, M. I.,Andrade, J. A. D., Alonso, L. G.,Brunini, D., Pritchard, M., & Estivill,X. (2001). Clinical and CytogeneticCharacterisation of a Patient withDown Syndrome Resulting from a21q22.1 -> qter Duplication. Journalof Medical Genetics 38, 73-76.

Petersen, M. B., & Mikkelsen, M. (2000).Nondisjunction in Trisomy 21: Originand Mechanisms. Cytogenetics & CellGenetics 91, 199-203.

COMMENT: A review.

GROWTH & DEVELOPMENTGlenn, S. M., & Cunningham, C. C. (2000).

Parents’ Reports of Young People withDown Syndrome Talking out Loud toThemselves. Mental Retardation 38,498-505.

COMMENT: Private speech, or self-talk,should be seen as adaptive, and not anindication of pathology.

Lobaugh, N. J., Karaskov, V., Rombough, V.,Rovet, J., Bryson, S., Greenbaum, R.,Haslam, R. H., & Koren, G. (2001).Piracetam Therapy Does Not EnhanceCognitive Functioning in Childrenwith Down Syndrome. Archives ofPediatrics & Adolescent Medicine155, 442-448.

HEMATOLOGY/ONCOLOGYHongeng, S., Pakakasama, S., Hathirat, P.,

Phuapradid, P., & Worapongpaiboon, S.(2000). Diffuse Hepatic Fibrosis withTransient Myeloproliferative Disordersin Down Syndrome. Journal ofPediatric Hematology/Oncology 22,543-4.

Jindal, N., Ghoshal, N., & Kabra, S. K.(2000). Down’s Syndrome withTransient Abnormal Myelofibrosis.Indian Pediatrics 37, 808-9.

Ma, S. K., Wan, T. S., Chan, G. C., Ha, S. Y.,Fung, L. F., & Chan, L. C. (2001).Relationship between TransientAbnormal Myelopoiesis and AcuteMegakaryoblastic Leukaemia inDown’s Syndrome. British Journal ofHaematology 112, 824-5.

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Satge, D., Monteil, P., Sasco, A. J., Vital, A.,Ohgaki, H., Geneix, A., Malet, P.,Vekemans, M., & Rethore, M. O.(2001). Aspects of Intracranial andSpinal Tumors in Patients with DownSyndrome and Report of a RapidlyProgressing Grade 2 Astrocytoma.Cancer 91, 1458-1466.

Taub, J. W. (2001). Relationship ofChromosome 21 and Acute Leukemiain Children with Down Syndrome.Journal of Pediatric HematologyOncology 23, 175-178.

Villanueva, M. J., Navarro, F., Sanchez, A.,Provencio, M., Bonilla, F., & Espana, P.(2000). Testicular Germ Cell Tumor andDown Syndrome. Tumori 86, 431-3.

COMMENT: A review.

MISCELLANEOUSAbu-Saad, H. H. (2000). Challenge of Pain

in the Cognitively Impaired. Lancet356, 1867-8.

COMMENT: A letter.Anonymous. (2001). JAMA Patient Page.

Down Syndrome. JAMA 285, 1112.Brandt, B. R. (2001). Pain in Down’s

Syndrome. Lancet 357, 1041-2.COMMENT: This is a letter in response to a

previous article by Hennequin (sebelow) about pain perception inpeople with Down syndrome thatsuggested the decreased response topain was due to cognitive delays. Theletter points out that there are nerveconduction delays and loweramplitudes that explain this. Brandt,also, points out that other people withcognitive delays do not have adiminished pain response.

COMMENT: I have found it interesting thatmost of the people I see with Downsyndrome do not complain of chronicpain. They usually have a behaviorchange. Chronic pain is different thanacute pain and always is subject tointerpretation with a psychosocialeffect, whether the person has Downsyndrome or not. I think thispsychosocial interpretation is what isdifferent in Down syndrome. In thiscase actions do speak louder thanwords. As a family doctor working withpeople with Down syndrome hasreminded me that the words of mypatients without Down syndrome canmake confusing a diagnosis that is moreevident, if instead I study their behavior.

Cunniff, C., Frias, J. L., Kaye, C., Moeschler,J. B., Panny, S. R., & Trotter, T. L.(2001). Health Supervision forChildren with Down Syndrome.Pediatrics 107, 442-449.

Hedov, G., Anneren, G., & Wikblad, K.(2000). Self-Perceived Health inSwedish Parents of Children withDown’s Syndrome. Quality of LifeResearch 9, 415-22.

COMMENT: Mothers of children withDown syndrome showed poorer healththan their spouses and the controlmothers. N=165.

Hennequin, M., Morin, C., & Feine, J. S.(2000). Pain Expression and StimulusLocalisation in Individuals with Down’sSyndrome. Lancet 356, 1882-7.

Kmietowicz, Z. (2001). Down’s ChildrenReceived “Less Favourable” HospitalTreatment. BMJ 322, 815.

Langenbeck, U., Herzberger, G., &Kummerle, S. (2000). Parent-Offspring Resemblance of Palmer andPlanter Dermatoglyphic Patterns inDown Syndrome. Cytogenetics & CellGenetics 91, 157-159.

Norris, F. H., Jr. (1977). Terminology forDown Syndrome. JAMA 237, 2381.Oakley, G. P., Jr. (1978). NaturalSelection, Selection Bias and thePrevalence of Down’s Syndrome. NewEngland Journal of Medicine 299,1068-9.

Roizen, N. J. (2001). Down Syndrome:Progress in Research. MentalRetardation & DevelopmentalDisabilities Research Reviews 7, 38-44.

COMMENT: A review.

NEPHROLOGYFiller, G., Kotecha, S., Milanska, J., &

Lawson, M. L. (2001). Trisomy 21with Hypercalcemia, Hypercalciuria,Medullary Calcinosis and RenalFailure—a Syndrome? PediatricNephrology 16, 99-100.

Kim, B. S., Lee, S. H., Lee, J. E., Chung, S.W., Kim, Y. O., Choi, K. B., Choi, E.J., & Bang, B. K. (2001). PosteriorLeukoencephalopathy SyndromeDuring Steroid Therapy in a DownSyndrome Patient with NephroticSyndrome. Nephron 87, 289-290.

ABSTRACTS/REFERENCES

NEUROLOGYDel-Rio Camacho, G., Orozco, A. L., Perez-

Higueras, A., Camino Lopez, M., Al-Assir, I., & Ruiz-Moreno, M. (2001).Moyamoya Disease and Sagittal SinusThrombosis in a Child with Down’sSyndrome. Pediatric Radiology 31,125-8.

Devenny, D. A., Krinsky-McHale, S. J.,Sersen, G., & Silverman, W. P. (2000).Sequence of Cognitive Decline inDementia in Adults with Down’sSyndrome. Journal of IntellectualDisability Research 44, 654-65.

COMMENT: Twenty-two individuals withvarying degrees of cognitive declinewere compared to 44 adults with DSwho remained healthy. The duration ofthe longitudinal study was not evidentfrom the abstract. They used theWISC-R to test cognitive function.

Gulesserian, T., Engidawork, E., Cairns, N.,& Lubec, G. (2000). Increased ProteinLevels of Serotonin Transporter inFrontal Cortex of Patients with DownSyndrome. Neuroscience Letters 296,53-7.

Gulesserian, T., Seidl, R., Hardmeier, R.,Cairns, N., & Lubec, G. (2001).Superoxide Dismutase Sod1, Encodedon Chromosome 21, but Not Sod2 IsOverexpressed in Brains of Patientswith Down Syndrome. Journal ofInvestigative Medicine 49, 41-6.

Gyure, K. A., Durham, R., Stewart, W. F.,Smialek, J. E., & Troncoso, J. C.(2001). Intraneuronal a Beta-AmyloidPrecedes Development of AmyloidPlaques in Down Syndrome. Archivesof Pathology & Laboratory Medicine125, 489-492.

Huxley, A., Prasher, V. P., & Haque, M. S.(2000). The Dementia Scale forDown’s Syndrome. Journal ofIntellectual Disability Research 44,697-8.

Kim, S. H., Cairns, N., Fountoulakisc, M., &Lubec, G. (2001). Decreased BrainHistamine-Releasing Factor Protein inPatients with Down Syndrome andAlzheimer’s Disease. NeuroscienceLetters 300, 41-44.

Kishnani, P. S., Spiridigliozzi, G. A., Heller, J.H., Sullivan, J. A., Doraiswamy, P. M.,& Krishnan, K. R. (2001). Donepezilfor Down’s Syndrome. AmericanJournal of Psychiatry 158, 143.

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Lee, M., Hyun, D., Jenner, P., & Halliwell,B. (2001). Effect of Overexpression ofWild-Type and Mutant Cu/Zn-Superoxide Dismutases on OxidativeDamage and Antioxidant Defences:Relevance to Down’s Syndrome andFamilial Amyotrophic LateralSclerosis. Journal of Neurochemistry76, 957-65.

Pinter, J. D., Brown, W. E., Eliez, S.,Schmitt, J. E., Capone, G. T., & Reiss,A. L. (2001). Amygdala andHippocampal Volumes in Childrenwith Down Syndrome: A High-Resolution MRI Study. Neurology 56,972-974.

Saito, Y., Oka, A., Mizuguchi, M., Motonaga,K., Mori, Y., Becker, L. E., Arima, K.,Miyauchi, J., & Takashima, S. (2000).The Developmental and AgingChanges of Down’s Syndrome CellAdhesion Molecule Expression inNormal and Down’s SyndromeBrains. Acta Neuropathologica 100,654-64.

Schupf, N., Patel, B., Silverman, W.,Zigman, W. B., Zhong, N., Tycko, B.,Mehta, P. D., & Mayeux, R. (2001).Elevated Plasma Amyloid Beta-Peptide 1-42 and Onset of Dementiain Adults with Down Syndrome.Neuroscience Letters 301, 199-203.

Seidl, R., Cairns, N., Singewald, N., Kaehler,S. T., & Lubec, G. (2001). Differencesbetween Gaba Levels in Alzheimer’sDisease and Down Syndrome withAlzheimer-Like Neuropathology.Naunyn-Schmiedebergs Archives ofPharmacology 363, 139-145.

Temple, V., Jozsvai, E., Konstantareas, M.M., & Hewitt, T. A. (2001). AlzheimerDementia in Down’s Syndrome: TheRelevance of Cognitive Ability.Journal of Intellectual DisabilityResearch 45, 47-55.

OPHTHALMOLOGYCregg, M., Woodhouse, J. M., Pakeman, V.

H., Saunders, K. J., Gunter, H. L.,Parker, M., Fraser, W. I., & Sastry, P.(2001). Accommodation andRefractive Error in Children withDown Syndrome: Cross-Sectional andLongitudinal Studies. InvestigativeOphthalmology & Visual Science 42,55-63.

COMMENT: Accommodation was poor,regardless of the refractive error.Glasses do not remedy the problem.Therefore near vision is consistentlyout of focus. Not good for reading.N=69. Age 4-85 months. Why do somany kids with DS sit so close to theTV or a book? How close is near in“near vision”? One referencementioned using 40 cm as a testdistance but also said near is anindividual thing.

Haugen, O. H., & Hovding, G. (2001).Strabismus and Binocular Function inChildren with Down Syndrome. APopulation-Based, Longitudinal Study.Acta Ophthalmologica Scandinavica79, 133-139.

COMMENT: N=60.

ORTHOPEDICSBeguiristain, J. L., Barriga, A., & Gent, R. A.

(2001). Femoral AnteversionOsteotomy for the Treatment of HipDislocation in Down Syndrome:Long-Term Evolution. Journal ofPediatric Orthopaedics-Part B 10, 85-88.

Tyler, C. V., Jr., Snyder, C. W., & Zyzanski,S. (2000). Screening for Osteoporosisin Community-Dwelling Adults withMental Retardation. MentalRetardation 38, 316-21.

OTOLARTNGOLOGYBell, R. B., & Turvey, T. A. (2001). Skeletal

Advancement for the Treatment ofObstructive Sleep Apnea in Children.Cleft Palate-Craniofacial Journal 38,147-154.

Boseley, M. E., Link, D. T., Shott, S. R.,Fitton, C. M., Myer, C. M., & Cotton,R. T. (2001). Laryngotracheoplasty forSubglottic Stenosis in Down SyndromeChildren: The Cincinnati Experience.International Journal of PediatricOtorhinolaryngology 57, 11-15.

Kanamori, G., Witter, M., Brown, J., &Williams-Smith, L. (2000).Otolaryngologic Manifestations ofDown Syndrome. OtolaryngologicClinics of North America 33, 1285-+.

Pirsig, W., & Verse, T. (2000). Long-TermResults in the Treatment ofObstructive Sleep Apnea. EuropeanArchives of Oto-Rhino-Laryngology257, 570-577.

COMMENT: Review

DAVID SMITH

Uong, E. C., McDonough, J. M., Tayag-Kier,C. E., Zhao, H. Q., Haselgrove, J.,Mahboubi, S., Schwab, R. J., Pack, A.I., & Arens, R. (2001). MagneticResonance Imaging of the UpperAirway in Children with DownSyndrome. American Journal ofRespiratory & Critical Care Medicine163, 731-736.

PRENATAL DIAGNOSISChilaka, V. N., Konje, J. C., Stewart, C. R.,

Narayan, H., & Taylor, D. J. (2001).Knowledge of Down Syndrome inPregnant Women from DifferentEthnic Groups. Prenatal Diagnosis21, 159-164.

Delzell, J. E., Jr. (2000). What Can We Do toPrepare Patients for Test ResultsDuring Pregnancy? Western Journalof Medicine 173, 183-4.

COMMENT: A letter.Garne, E., Berghold, A., Johnson, Z., &

Stoll, C. (2001). Different Policies onPrenatal Ultrasound ScreeningProgrammes and Induced AbortionsExplain Regional Variations in InfantMortality with CongenitalMalformations. Fetal Diagnosis &Therapy 16, 153-157.

Hewison, J., Cuckle, H., Baillie, C., Sehmi,I., Lindow, S., Jackson, F., & Batty, J.(2001). Use of Videotapes for Viewingat Home to Inform Choice in DownSyndrome Screening: A RandomisedControlled Trial. Prenatal Diagnosis21, 146-149.

Hulten, M. (2001). Non-Invasive PrenatalDiagnosis of Down’s Syndrome.Lancet 357, 963-4.

Malhotra, B., & Deka, D. (2001). Can DownSyndrome Cause Persistent Non-Reactive Non-Stress Test?International Journal of Gynecology& Obstetrics 72, 261-262.

Nyberg, D. A., & Souter, V. L. (2000).Sonographic Markers of FetalAneuploidy. Clinics in Perinatology27, 761-+.

COMMENT: ReviewPetrou, S., Henderson, J., Roberts, T., &

Martin, M. A. (2000). RecentEconomic Evaluations of AntenatalScreening: A Systematic Review andCritique. Journal of MedicalScreening 7, 59-73.

COMMENT: A review.

12

Smith-Bindman, R., Hosmer, W., Feldstein,V. A., Deeks, J. J., & Goldberg, J. D.(2001). Second-Trimester Ultrasoundto Detect Fetuses with DownSyndrome - a Meta-Analysis. JAMA285, 1044-1055.

COMMENT: A review.Smith-Bindman, R., Hosmer, W. D.,

Caponigro, M., & Cunningham, G.(2001). The Variability in theInterpretation of Prenatal DiagnosticUltrasound. Ultrasound in Obstetrics& Gynecology 17, 326-332.

Toth, A., & Szabo, J. (2000). Ethical Aspectsof Prenatal Screening for Down’sSyndrome. Orvosi Hetilap 141, 2293-8.

COMMENT: A review. Hungarian.

PSYCHIATRYTsiouris, J. A., Mehta, P. D., Patti, P. J.,

Madrid, R. E., Raguthu, S., Barshatzky,M. R., Cohen, I. L., & Sersen, E.(2000). Alpha2 MacroglobulinElevation without an Acute PhaseResponse in Depressed Adults withDown’s Syndrome: Implications.Journal of Intellectual DisabilityResearch 44, 644-53.

RHEUMATOLOGYSuwa, A., Hirakata, M., Satoh, S., Ezaki, T.,

Mimori, T., & Inada, S. (2000).Systemic Lupus ErythematosusAssociated with Down Syndrome.Clinical & Experimental Rheumatology18, 650-1.

COMMENT: A review.

15

DOWN SYNDROME QUARTERLYVOLUME 6, NUMBER 2 • JUNE 2001

DOWN SYNDROME QUARTERLYDenison University

Granville, OH 43023

First Class PresortedU.S. Postage

PAIDGranville, OHPermit No. 22

CONTENTS

The Goal and Opportunity of Physical Therapy for Childrenwith Down Syndrome Patricia C.Winders . . . . . . . . . . . . . . . . . . . . . . . . .1

Book Review: Explaining Down Syndrome to School-Age ChildrenBarry M. Mitnick . . . . . . . . . . . . . . . . . . . . . . . . . . 5

News from the Down Syndrome Medical Interest Group(DSMIG)William I. Cohen and Bonnie Patterson . . . . . . . 8

References/AbstractsDavid Smith . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9