Nahal Derayeh
Exceptional Children 414-309 (Department of Educational Psychology & Counselling)
Winter 2001, McGill University
Printed with the permission of Nahal Derayeh

Down syndrome is a disability that was first described one hundred and thirty-five years ago. Evidence in paintings and sculptors from thousands of years ago have however supported the idea that Down syndrome was not new from the 19th century (Wishart 1998). It has nonetheless been a controversial disorder since Down's paper on it was first published. Since then, Down syndrome has been referred to by a variety of names and our understanding of the disorder has developed increasingly with the years. Initially referred to as Mongolism, followed by Down's syndrome and finally by either Trisomy 21 or Down syndrome, the disorder that I will refer to by the latter has had much light shed on it as a result of years of research and an understanding of what it is as well as what it not. The following research paper will describe some historical facts that date back to Down's description of the disorder, followed by current prevalence rates, it's known and assumed causes, characteristics, and finally teaching strategies for the inclusion of individuals with Down syndrome.

History

Down syndrome and its characteristics were first described by John Langdon Down in a paper entitled "Observations of an ethnic classification of idiots." It was in 1866, at a time when Charles Darwin's theory of evolution had gained quite some attention; the British scientist, Darwin had proposed the concept of natural selection as well as the concept of ancestral descent -(Encarta 2000). Down's observations on what he called "Mongolian type of idiocy" (Down 1866: 260) emphasized the disorder's source was the result of racial degeneration.

It is clear to see that this was a period when racist theories of the evolution of man were quite common. As outlined by Lane and Stratford in their book 'Current Approaches to Down's Syndrome', in 1844, theorist Robert Chambers stated the brain's stages went "from that of a fish's, to a reptile's, to a mammal's, and finally to a human's". This last category, the human's brain, also went through stages from the "Negro, Malay, American, and Mongolian nations, and finally [the] Caucasian" (1987:4). It is no doubt that this period's ignorance was due to a lack of understanding of the two main observable characteristics of the disorder: the intellectual challenge associated to it as well as the physical appearance of the individuals.

It was in the early 1950's that new scientific methods were discovered that could depict chromosomes in modern ways, and so in 1956, the 46 chromosomes within each human cell were discovered (Rynders 1987: 7-8). By 1959 however a group of geneticists headed by a scientist named Jérôme Lejeune found that a chromosomal disorder was present within individuals with Down syndrome. Lejeune and his colleagues found that people with Down syndrome had an additional 47th chromosome.

Prevalence

Down syndrome, the most common genetic condition, occurs in approximately one in every eight hundred to one thousand live births, and this number is said to increase in women over the age of thirty-five (Britannica.com 2000, Wishart 1998, NDSS 2001). It has also been stated that eighty percent of Down syndrome births are actually to women under the age of thirty-five (Wishart 1998, NDSS 2001). Although this information may seem contradictory, it may be explained by the decrease in fertility levels in older women and the increase in births in younger women. The risks of giving birth to a child with Down syndrome do therefore increase with age. In fact, the incidence of Down syndrome births in women forty-five years of age and over is of approximately one in thirty five (NDSS 2001, Encarta 2000, Britannica.com 2000).

Cause

Scientific advances from the 1950's have enabled us to get a more accurate understanding of the causes of Down syndrome. It has been found that a chromosomal disorder is rooted at the time of conception. Three forms of chromosomal disorders are present, all of which will be briefly discussed, as described by the National Down Syndrome Society (NDSS 2001).

Before doing so however it is important to define meiosis - the process of cell division in which the number of chromosomes is reduced to half. Cells of the human species are diploid - doubles of chromosomes, and it is upon fertilization that the cells are haploid - divided in halves. The haploid cells of both the sperm and the ovum then come together. A disjunction - failure to disjoin, may take place at the level of meiosis, resulting in chromosomal Trisomy, referring to an extra 21st chromosome in each cell. Trisomy 21 is the most common cause of Down syndrome and accounts for ninety-five percent of cases (NDSS 2001, Lane & Stratford 1987: 27).

The next possible chromosomal cause is that of Mosaicism which occurs when there is a nondisjunction within the 21st chromosome. This results in the presence of 46-chromosome-cells as well as 47-chromosome-cells, the latter containing the extra 21st chromosome. Mosaicism accounts for 2% of cases.

It is important to note that in any case, the 21st chromosome may originate from either the ovum or the sperm although there is only five-percent occurrence traced to the sperm cell (NDSS 2001).

The last chromosomal cause is that of Translocation. It occurs when "a part of the number 21 chromosome breaks off during cell division and attaches to another chromosome" (NDSS 2001). The presence of a piece of the 21st chromosome results in Down syndrome. In the case of Translocation, it has been said that maternal age is not associated with this risk but that two-thirds of the chances are rather sporadic and the rest are inherited from a parent (NDSS 2001).

The effect of having an extra 21st chromosome cannot be ignored. The presence of abnormal "gene dosage" has been found to be disruptive in the development of the central nervous system from fertilization onwards (Wishart 1998).

Literature and scientific research have supported the latter genetic factors although the etiology remains unknown. Some theories on non-genetic causes do however exist including x-radiation, radar, oral contraceptives, cigarette use, and alcohol consumption to name but a few (Jagiello et al 1987:23-24, Lane & Stratford 1987: 38). Despite the fact that these hypotheses are interesting, they remain just that and are unsupported by scientific research.

Characteristics

The characteristics associated with Down syndrome are many and vary amongst individuals. There are widely held stereotypes of people with Down syndrome that have been present since John Langdon Down's paper was published in 1866. The same stereotypes that were then present may be read in today's modern literature. A few of these ideas include a universal love for music, constant happiness, feeble mindedness, an inability to become independent, good mimicry, etc. There has however been no scientific evidence of individuals with Down syndrome being similar in either their abilities or their personalities because of their disability (Wishart 1998). Stereotyping denies an important factor of Down syndrome, which is "just how much variability there can be in the development of children" (Wishart Online).

This is not to say that there are no shared characteristics within children with Down syndrome. In fact the characteristics that are most common may be divided into subsections including physical traits, health issues, and intellectual challenges.

The physical features that individuals with Down syndrome have been characterized by, are what once were independently used to classify them. Dr. Down had initially named the disorder "Mongolism" because of the physical appearance of the individuals and their resemblance to the Mongolian Asians (Down 1866: 261). It should be clear however that no matter the physical appearance of people with Down syndrome, each one remains a unique individual and he/she may not have all of the following traits that have been characterized as most common to individuals with Down syndrome. These traits include muscle hypotonia - low muscle tone, flat facial profile, depressed nasal bridge and small nose, oblique palpebral fissures - an upward slant to the eyes, epicanthal folds - small skin folds on the inner corner of the eyes, dysplastic ear - an abnormal shape of the ear, hyperflexibility - a profound ability to extend the joints, poor muscle tone, and an enlargement of the tongue in relation to size of mouth (NDSS 2001, Down 1866: 259-260).

These physical characteristics often result not only in a lowered self-esteem but also in some serious problems such as hearing difficulties and articulation problems.

The next subsection that is included in the characteristics of individuals with Down syndrome is the most common health issues. An alarming statistic from the National Down Syndrome Society is that only twenty to twenty-five percent of children conceived with Down Syndrome survive past birth (NDSS 2001). Children with Down syndrome are also at greater risk of developing health problems. Up to fifty percent of people with Down syndrome are born with a congenital heart disease. Also shocking is the fifteen to twenty percent higher chance of developing acute leukemia within the first three years of life (Encarta 2000, Wishart 1998: 343). As devastating as these statistics are, it is due to effective research that a variety of treatments and surgeries have been successful. The cure rates for most health problems have therefore increased dramatically (Lane & Stratford 1987: 39, Wishart 1998).

This last division includes the intellectual challenges often present in individuals with Down syndrome. It should be noted however that the degree of challenge will vary from one individual to the another. Researchers have stated that most people with Down syndrome do in fact some level of intellectual disability ranging from mild to severe, although the mean remains mild to moderate (NDSS 2001, Wishart Online, Wishart 1998). Much literature have stated that most individuals with Down syndrome do not progress beyond the intellectual capabilities of a normal developing six to eight year old (Kliewer 1993, Down 1867), and that Down syndrome may be the most common cause of intellectual disability (Wishart 1998). Needless to say, no disability should ever determine who an individual is. Assumptions about an individual's abilities could lead to limited expectation which in turn could lead to lack of achievement to one's full potential.

No matter the level of intellectual ability, early intervention and quality education have been proven to be major contributors in improving the lives and futures of individuals with Down syndrome (Gov. of Saskatchewan 1998).

The following pages will therefore focus on the education of children with Down syndrome and the teaching strategies that have been found to aid in their development.

Teaching strategies

Inclusion has gained much interest in the past years and the outcomes that are achieved through it have made it a priority in education. Acknowledging that all individuals with Down syndrome are different in their abilities and their interests, it is clear that teaching strategies will vary greatly from one child to the next. The overall goals remain that the children will develop new skills, they will acquire appropriate behaviors, and they will foster independence in their behavior and learning (Pueschel & Gieswein 1993). It is clear that these goals remain important regardless of whether the child has Down syndrome or not. Of course the varying abilities within the classroom should not be ignored and it is with the presence of an individualized education plan (IEP) that teachers can ensure the child is getting an appropriate education.

A team which includes the student, his/her parents, the classroom teacher, teacher assistant, special educator, as well as other professionals that are involved in the child's educational development puts together the IEP. The purpose of the IEP is to be attentive to the student's needs, abilities, strengths, and weaknesses as well as to include the child within the regular classroom. In doing so, the child's level and potential should be respected at all times (considering that the material used remains age appropriate at all times.)

As previously mentioned, the characteristics that are most common in individuals with Down syndrome are many and vary to different degrees. It is therefore important to note that of the following strategies, it is the individual needs of the particular child that will determine whether or not one strategy will be more relevant than another.

The characteristics that are most relevant to the learning and schooling of children with Down syndrome follow along with some common strategies that may enrich their experience in the regular classroom.

A common problem for children with Down syndrome is the inability to focus on specific stimuli - attention span problems (Kliewer 1993, NDSS 2001). This can have serious and very negative affects on children's learning and it is very important to use a variety of strategies to control it. A suggestion could include removing distracting stimuli such as excessive decorations as well as limiting the amount of information on handouts to prevent overflow. Cueing attention by using prompts such as a tap on a shoulder, a switch of the light, or even a 'secret sign', may be very effective and need not interrupt the flow of a lesson. Selecting meaningful and worthwhile activities and lessons also aid with attention problems for more focus may be expected when material being taught is of interest. As with any individual, when what is being learned has a distinct and significant purpose, the process becomes increasingly worthwhile and in turn encourages attentiveness.

Another prevailing characteristic is that of memory problems. Most literature on Down syndrome includes a section on the problems that memory loss may bring upon children and adults alike (Kliewer 1993). Memory deficits have a tremendous effect on learning; using past knowledge to link new knowledge is used everyday, as is the storing and retrieving of important information. Some strategies that may become routine in the classroom include teaching rehearsal strategies and promoting learning through repetition and practice.

Speech and language problems have been found to be quite current in individuals with Down syndrome (Gov. of Saskatchewan 1998, Kliewer 1993). The problems may be in expression -formal or informal social exchanges, articulation, fluency, etc. In order to cater to the needs of children with problems in these areas, it is important to adapt both the content (what is being taught) as well as the teaching style (methods used) of everyday lessons to the level of the child. Overall learning objectives may be made for the class as a whole but the child with the special need should have appropriate and adapted versions of the activities.

In cases of limited expression, it has often been recommended to accept both verbal and non-verbal forms of communication. Many authors and much of the literature on teaching strategies (Kliewer 1993, Gov. of Saskatchewan 1998) have advocated using an augmentative communication system with children to express and to complement their oral speech. Augmentative strategies include the use of gestures (signing), pictures and symbols (communication boards), as well as technological devices (computers). An interesting quote, stated in Chris Kliewer's article from the Facilitated Communications Institute, said that "signing does not interfere with spoken language, but rather seems to facilitate its onset and development." In this same article, Kliewer adds that much research has shown cases of children with poor speech quickly developing competency in their area of difficulty once they learned signing (1993.)

Other general strategies to complement communication for children with Down syndrome include patience, modeling and teaching listening techniques.

When a child has trouble communicating or articulating, being patient and modeling more appropriate interactions and speech can be very telling. Providing aid upon request (and waiting for the child to ask for the help rather than assuming that he/she needs it) has also been recommended. Giving the choice and the opportunity to ask for assistance produces a sense of independence that should be encouraged at all times.

An effective strategy that encourages interaction and speech could be creating situations where expression is essential. Such situations include circumstances where the child may need to ask for something (not giving sufficient materials for an activity), circumstances encouraging commenting (asking whether or not homework on Fridays is fair), as well as circumstances necessitating negotiations (discuss between classmates who will take what role in a presentation) (Gov. of Saskatchewan 1998).

Of course, communication entails an ability to express and speak, but listening skills should not be ignored. Listening is a lifelong skill that children should acquire. The child with Down syndrome should learn to develop his/her listening ability, and this may be done through cooperative learning situations or activities such as debates on topics of interest. An important aspect that should not be left out is that of respecting the child's pace and level (as well as his/her possible hearing difficulty); referring to speaking clearly and being considerate of the speed of our speech in circumstances of peer or student-teacher interactions.

In the case of a hearing impairment, some basic physical considerations should be taken. These considerations include seating the child in an appropriate area, near the teacher and away from noisy areas such the door and hallway. The use of visual aids in teaching are also worthwhile in keeping the child with a hearing difficulty on track or in giving the child an opportunity to follow along by reading or viewing. Literature has suggested that visual aids are very effective in educating children with difficulties in hearing, abstract thinking, social cognition, communication, and attention (Gov. of Saskatchewan 1998), all of which are common difficulties for children with Down syndrome. Using visual aids to complement teaching would nonetheless advantage not only the special needs child but also the mainstream student. Visual aids include overhead projections, posters, handouts with key information, or simply pictures. The visual representations play the part of getting a special attention or a point of focus during a regular classroom lesson.

There are also some very general classroom strategies that can be used in including a child with Down syndrome in a regular education classroom. These strategies do not focus on specific difficulties or disabilities but rather on having a successful overall inclusion. From enhancing contact between peers to environmental considerations, these strategies will lead to fulfilling experiences.

A strategy that has merit in inclusion involves cooperative learning situations in which students with different abilities work together with a specific goal in mind. A child with Down syndrome can not only enlighten others with his/her perspectives, but also learn a lot from peers. The child may also be given the opportunity to be a role model by helping younger students and teaching them a mastered subject area. Encouraging a child with special needs to share particular knowledge and serve as a role model may be very encouraging and meaningful.

Having regular and expected routines in teaching are often found to have very positive effects on all children. Having a structured and expectable environment often results in students feeling more at ease. Knowing what to expect and being assured that no major surprises are commonly called for may be reassuring for many students. Also, taking into account that children process information in small chunks, new material should be taught step by step.

As for the effectiveness of strategies considering environmental characteristics, aspects such as lighting, noise level, distracting windows, excessive or bombarding furniture or decorations, as well as unorganized classroom areas are but a few of the many that should not be ignored. All students will benefit from an orderly environment.

Behavior management

Adequate classroom management and behavior management techniques used for mainstream students were found to not differ from the management techniques successful with children having Down syndrome (Pueschel & Gieswein 1993). The obvious use of meaningful reinforcers (finding out what the child enjoys most) and the opting of positive reinforcement above punishment (using the least intrusive model of behavior modification) are supported by research as being especially beneficial for students with characteristics associated to Down syndrome (Gov. of Saskatchewan 1998). Another particular method that should be stressed is teaching of self-management. Encouraging students to adopt self-management techniques results in increased independence and monitoring of own behavior, both of which should be essential goals in the development of any child.

Conclusion

The characteristics that are associated with individuals having Down syndrome may cause many to fear the inclusion of a child with the disability into the regular classroom. Many will underestimate the abilities of these children and assume that only certain achievements can be accomplished. It is sad but true that many of today's teachers will have a stereotyped image of the abilities of a child with Down syndrome and in turn either block any possible blossoming or go as far as to cause regression.

It is important for all to acknowledge that individuals with Down syndrome do not resemble each other in any other way than in some common physical traits. Individuals with Down syndrome have different strengths and weaknesses as well as different likes and dislikes.

Most individuals with Down syndrome are not only able to go to school and this in an inclusive setting, but they are all also blessed with talents that make them unique. By developing these talent areas, we are providing opportunities for development that may lead to a life of independence and a sense of accomplishment and well being.

The strategies that have been offered in this research paper are not independently effective for children with Down syndrome. In fact, most of the strategies will be very beneficial for all students in the inclusive classroom. Considering that some curricular changes will surely be called for, there should be no other difference in the treatment offered to the individual with Down syndrome or the individual without. Treating people with Down syndrome with respect for the individuals that they truly are should not be underestimated but rather regarded as being one of the most important strategies in teaching students in an inclusive setting.

References

Internet sites:

Down Syndrome (2000) Britannica.com. [Online]. http://www.britannica.com/search?query=down%20syndrome&ct= [Accessed February 01]

Down Syndrome (2000) Encarta: Resources for interactive Learning. [Online]. [Accessed February 01]

Government of Saskatchewan (1998) Special Education Unit. Teaching Students with Autism: A Guide for Educators. [Online article]. http://www.sasked.gov.sk.ca/curr_inst/speced/hero.html [Accessed: February 01]

National Down Syndrome Society (2001) About Down Syndrome. [Online]. http://www.ndss.org/ [Accessed February 01]

Pueschel & Gieswein (1993) Ocular disorders in children with Down syndrome. Down Syndrome: Research and Practice [Online], 1(3), 129-132. Available: Riverbend Down Syndrome Association. http://www.riverbendds.org/ocular.html [Accessed: February 01]

Wishart J.G. Cognitive Development in Young Children with Down Syndrome: Developmental Strengths, Developmental Weaknesses. Research Foundation & Research Center. [Online article]. http://dsrf.org/index.cfm?FUSEACTION=inform.article&TitleID=32&StyleType=1 [Accessed February 01]

Journal articles:

Down, J.L.H. Observations on an Ethnic Classification of Idiots. London Hospital, Clinical Lecturer and Reports, vol. 3. 1866. 259-262

Jagiello G.M., Fang J.S., Ducayeh M.B., Kong W. Etiology of Human Trisomy 21. New Perspectives on Down Syndrome, 1987. 23-38

Kliewer C. The Communication Portfolio. Facilitated Communication Digest, vol. 2, no. 1. Nov. 1993

Rynders J.C. History of Down Syndrome: The Need for a New Perspective. New Perspectives on Down Syndrome, 1987. 1-20

Wishart J.G. Development in Children With Down Syndrome: Facts, Findings, the Future. International Journal of Disability, Development and Education, vol. 45, no. 3. 1998

Books:

Lane D. & Stratford B. (1987). Current Approaches to Down's Syndrome. Cassell Educational Limited. London.

Oelwein P.L. (1995). Teaching Reading to Children with Down Syndrome: A Guide for Parents and Teachers. Woodbine House. USA.