Presentation on theme: "How to Provide a Safe and Stimulating Classroom Environment for Students with Down Syndrome-A Medical and Educational Perspective February 26, 2008 Toni."— Presentation transcript:
1 How to Provide a Safe and Stimulating Classroom Environment for Students with Down Syndrome-A Medical and Educational PerspectiveFebruary 26, 2008Toni Benton, M.D.Metro Area Regional Medical ConsultantContinuum of Care ProjectUNM HSC School of Medicine
2 Down Syndrome Dr. John Langdon Down described the syndrome in 1866. Diagnosis was made clinically until 1959The chromosome abnormality was discovered in 1959.Down syndrome is one of the first symptom complexes associated with mental retardation to be identified as a syndrome.
3 Down Syndrome Overview It is the most common pattern of human malformation with an incidence in the general population of 1 in live births.Over 350,000 people in the United States have Down syndrome.A high correlation exists between increasing maternal age and the presence of an extra chromosome in the offspring.Therefore, the expected rate of Down syndrome in a woman 20 years of age is 1 in 1,925 compared to an expected rate of Down syndrome in a woman 45 years of age of 1 in 20.
4 Down Syndrome Genetics All persons with Down syndrome have duplicated chromosome 21 materialThe origin of the duplicated genetic material can varyNondisjunction (about 96% of cases)The extra chromosome may arise by abnormal sorting to produce an extra, free-standing chromosome (trisomy 21)Translocation (3 % of cases)a joining of chromosomes to produce extra chromosome 21 material that is attached to another chromosomeMosaicism (1-2%)Patients with mixtures of normal and trisomic cells (mosaic Down syndrome) often have milder phenotypes.Percentages of normal cells within the blood sample used for chromosome studies may differ from the percentages of normal cells in other tissues like brain or heart.
6 Extensive mapping of genes on this "critical region" of chromosome 21 is under way as part of the human genome initiative.The mechanisms by which increased dosage of these genes leads to the Down syndrome phenotype are as yet unknown.
7 Clinical Features Extra and loose neck skin Single creases on the palmsClinodactyly (curved fifth finger)Broad space between the first and second toesA deep plantar creaseThe tongue often protrudes, more because of low muscle tone than true enlargement.
8 Clinical FeaturesAs with all syndromes, the pattern of minor and major defects in Down syndrome varies from individual to individual.None of the anomalies taken alone are specific or diagnostic for Down syndrome.When Down syndrome is suspected, a chromosome study (karyotype) will confirm or exclude the diagnosis.
9 Clinical Features Central hair whorl (cowlick) Flat occiput (back of the head)Upslanting eyesEpicanthal folds (folds around the corner of the eye)White spots in the iris of the eye (Brushfield spots)Upturned nose
11 Heart Defects40% of individuals with Down syndrome have some form of congenital heart defectAV (atrioventricular) Septal defectVentricular Septal Defect (VSD)Patent Ductus Arteriosus (PDA)Atrial Septal Defect (ASD)Tetralogy of FallotAberrant Subclavian ArteryPulmonary HypertensionAdolescents and young adults can develop heart valve dysfunction even when they have had no history of congenital heart problems.SBE prophylaxis may be necessary.The most common defects are Atrioventricular Septal Defect (formally called Endocardial Cushion Defect), Ventricular Septal Defect, Persistent Ductus Arteriosus and Tetralogy of Fallot.Atrioventricular Septal DefectAn Atrioventricular Septal Defect is caused by a failure of tissue to come together in the heart during embryonic life. This results in a large opening in the center of the heart, with usually a hole between the two pumping chambers (a Ventricular Septal Defect) and between the two collecting chambers (an Atrial Septal Defect) as well as abnormalities of the two atrioventricular valves, the mitral and tricuspid valves. This defect occurs in nearly 60% of the children with Down syndrome who are born with congenital heart disease. In less severe cases, Ventricular Septal Defects and Atrial Septal Defects can also occur separately.Persistent Ductus ArteriosusThe ductus arteriosus is a channel between the pulmonary artery and the aorta and during fetal life diverts blood away from the lungs. After birth this channel usually closes on the first day of life. If it does not close, it is termed "persistent" and results in an increased flow of blood into the lungs.Tetralogy of FallotThis is a term given to a heart condition in which there is both a Ventricular Septal Defect and a narrowing of the passage from the right ventricle to the lungs causing Pulmonary Stenosis. The Pulmonary Stenosis causes the blue blood in the right ventricle to cross the Ventricular Septal Defect into the aorta and produces what is commonly called a "blue baby."What are the effects of Atrioventricular Septal Defect, Ventricular Septal Defect and Persistent Ductus Arteriosus?These defects result in increased blood flow going to the lungs as the blood goes through the septal defects or the ductus from the high pressure left heart into the low pressure right heart. This flooding of the lungs results in a buildup of pressure in the pulmonary circulation and this higher pressure and high flow leads to damage to the pulmonary circulation and pulmonary hypertension. In the first few months of life, signs of heart failure may be prominent with rapid breathing and failure to grow and gain weight. Later, progressive damage to the blood vessels and the lungs may result in reversal of the shunt and blueness of the skin (cyanosis).What is the relationship of heart defects to the respiratory system?The lungs of children with Down syndrome do not develop as fully as in the general population. Consequently, the growth of blood vessels throughout the lungs is limited. The narrowed arteries of the lungs hold potential for long-lasting damage in the face of increased pressure and flow of blood through the lungs.How are the defects diagnosed?Some children with Down syndrome and major heart defects will present with heart failure, difficulty breathing and failure to thrive in the newborn period; however, because in some children the defect may not be at first apparent, it is important that all children born with Down syndrome, even those who have no symptoms of heart disease, should have an echocardiogram in the first two or three months of life. The most common defects are Atrioventricular Septal Defect (formally called Endocardial Cushion Defect), Ventricular Septal Defect, Persistent Ductus Arteriosus and Tetralogy of Fallot.
12 Ophthalmologic Features Brushfield spots (speckling of the iris)Fine Lens OpacitiesNystagmusStrabismusKeratoconusPtosisCataractsAstigmatismHyperopia (far sightedness)Myopia (near sightedness)BlepharitisTear Duct Obstruction
13 Ophthalmologic Features KeratoconusBrushfield Spots
14 Hearing Loss Can be conductive, sensorineural or both Can be unilateral or bilateralOften undiagnosedChange in learning or behavior-consider hearing screenEtiology can be multifactorialNarrow posterior throat structuresSubtle immune deficiency resulting in recurrent ear infections
15 Gastrointestinal Disease Pyloric stenosisTracheo-esophageal fistulaEsophageal atresia (obstruction of the esophagus)Duodenal atresia (obstruction of the duodenum).Gastroesophageal Reflux (GERD)ConstipationHirschprung’s diseaseCeliac diseaseImperforate AnusNeonatal Liver Disease (rare)
17 Dental Diseases Microdontias Missing Teeth/Fused Teeth Delayed tooth eruption (1-2 years later than average)MalocclusionsPeriodontal diseaseGingivitis leading to loss of Alveolar boneDental Caries occur with lower prevalence than in the general populationPeriodontal disease in this population is early in onset and rapidly progressive.This is a manifestation of the general low resistance to infectionGingivitis leading to loss of Alveolar boneWe believe people with Down syndrome have genetic changes, or polymorphisms, that produce more inflammation than normal, leading to gum disease," said Dr. Ahmed Khocht, associate professor of periodontics and principal investigator of an approximately half-million-dollar National Institutes of Health study to test his theory. Periodontal disease is an inflammation of tissue surrounding and supporting the teeth, often caused by excess bacteria that left untreated can lead to tooth loss and systemic disease. The disease is relatively common in the general population but is highly treatable with twice-yearly visits to the dentist and regular brushing and flossing. Yet proper oral hygiene seems insufficient to protect those with Down syndrome from periodontal disease, Dr. Khocht said. "Eighty-five to 90 percent of people with Down syndrome have periodontal disease, and nothing else has explained the level of disease," he said. "We think perhaps there is something in their system that produces more inflammation than normal." He plans to take blood samples of 300 adults with Down syndrome during the three-year study, seeking genetic markers that trigger excess inflammation. If he and his colleagues detect those markers, the finding will help dentists develop a treatment protocol specifically for this population. The study should also provide critical information about the overall health of Down syndrome patients, Dr. Khocht said. "If our theory is correct, then people with Down syndrome are at higher risk for other inflammation-related diseases as well, including cardiac problems," he said. Targeting the faulty inflammatory response--for instance, by prescribing anti-inflammatory medication--may have wide-ranging benefits for Down syndrome patients, Dr. Khocht said. Adults with Down syndrome, with or without periodontal disease, are eligible to participate in Dr. Khocht's study. The patients are screened for eligibility and, if eligible, come in a for a second visit to have their gums examined. Blood is also drawn during the visit.
19 Endocrine Disorders Congenital hypothyroidism Thyroid Disease Diabetes Failure to Thrive- in infancy and early childhoodObesityShort StatureLipid abnormalitiesCongenital hypothyroidism occurs in persons w/ Down syndrome at a rate about 28 times the normal population30-50% of school age children manifest subclinical hypothyroidismDiabetes occurs about 1 in 250 children with Down syndrome (about twice the usual prevalence)The mechanism for obesity appears to be a lower resting metabolic rate, require fewer calories to gain weight.(despite similar activity and reduction of caloric intake to age matched peers, many continue to be overweight.)There is not sufficient data available at this point to know if there is increased risk for children with Down syndrome to develop type one diabetes as compared to the rate for their peers in the general population. However, research suggests that individuals who develop one type of endocrine autoimmune disorder, such as thyroiditis, are more likely to develop a second disorder, such as type one diabetes.What is hyperthyroidism?In this case, the thyroid gland is overactive. Symptoms are swelling in the neck, abnormal sweating and rapid pulse rate. No evidence exists as to whether hyperthyroidism is more prevalent in individuals with Down syndrome than in the general population.What is hypothyroidism?hypothyroidism results from a malfunctioning thyroid gland, which decreases the synthesis of the hormone thyroxin. Thyroxin is the hormone that promotes growth of the brain and other body tissue.How common is hypothyroidism?What treatment exists for hypothyroidism?The thyroid hormone, thyroxin, is readily replaced through medication.Are people with Down syndrome more prone to diabetes?What is the status of research on use of the growth hormone for children with Down syndrome?Use of growth hormone for children with Down syndrome is still in experimental stages. There are reports of increased rates of growth in children with Down syndrome who received the hormone for a brief period; however, these reports were not controlled studies, so there is no scientific evidence that long-term administration of the hormone would increase final height.Can any growth hormone be administered to any Down syndrome child?At present, doctors are licensed to prescribe growth hormone for individuals with Down syndrome only when there is a demonstrated deficiency of that hormone. It is strongly advised that such treatment be obtained only through a clinical trial until long-term benefits are demonstrated.Summary:Approximately 10 percent of children and between 13 to 50 percent of adults with Down syndrome have thyroid disease. Hypothyroidism, an under-functioning thyroid gland, is the most common condition.Hypothyroidism must be diagnosed by a standard blood test, beginning at infancy and continuing at regular intervals throughout the life span.Hypothyroidism is easily treated with replacement thyroxine hormone; treatment is particularly crucial for hypothyroid infants, as the hormone affects normal development of the brain. However, this thyroid disease can be developed at any time in the life span.Hyperthyroidism is an overactive thyroid gland. Diagnosis is also by blood test. The condition can be treated by medication, surgery or use of radioactive iodine to destroy the gland.Insufficient evidence exists on the issue of whether individuals with Down syndrome are more prone to type one diabetes. Research does suggest that individuals who develop one type of endocrine autoimmune disorder, such as thyroid disease, run a higher risk of developing other autoimmune disorders, such as type one diabetes.While some short-term uncontrolled studies on the use of growth hormone for children with down syndrome have demonstrated growth spurts, there is no long-term research to demonstrate an increase in final height. Growth hormone should be administered only as part of a clinical trial until long-term benefits are demonstrated.Hypothyroidism is the most common endocrine problem in children with Down syndrome. One study showed these individuals to be 28 times more prone to congenital hypothyroidism than the general population. It is estimated that approximately 10 percent of children with Down syndrome have congenital or acquired thyroid disease. Incidence of thyroid disease in adults with this genetic disorder varies from 13 to 50 percent. Hypothyroidism can occur at any time from infancy through adulthood.How is hypothyroidism diagnosed?All individuals with Down syndrome should be tested for hypothyroidism at birth and at periodic intervals (at least every two years) thereafter. The indicators of hypothyroidism -- enlarged tongue, constipation, poor circulation -- are also found in individuals who are not hypothyroid, so the blood test for thyroid function is an important diagnostic test. As the thyroid hormone affects normal development of the brain, testing of infants is particularly crucial.
20 Blood Disorders Immune Dysfunction Myeloid Proliferation Leukemia 1 in 150 in children with Down syndrome compared with 1 in 2800 in children at largePlatelet DisordersWhite blood cell impairmentErythrocytosis (secondary to chronic hypoxia)
21 Most related to Immune dysfunction/Inflammatory response Skin ConditionsMost related to Immune dysfunction/Inflammatory responseAtopic Dermatitis (eczema)Seborrheic DermatitisVitiligoChelitisIchthyosisXerosisAlopecia AreataSyringomasOnychomycosis
22 The Neurology of Down Syndrome The nervous system is always affected in Down syndrome. Developmental DelaysHypotoniaAtlantoaxial instabilitySymptomatic -vs- AsymptomaticSeizuresMental Health DisordersAlzheimer’s DiseaseFive to 10% of individuals with Down syndrome have a seizure disorder.ADHD and autism may occur in individuals with Down syndrome. Othermental health problems including depression, alcohol and substance abuse, psychosis, schizophrenia, OCD, anxiety disorders, and dementia of the Alzheimer type may be found among individuals with Down syndrome.It is important to rule out treatable causes of decline in mental functioning (thyroid problems, B vitamin deficiencies, vision and hearing problems, depression, sleep apnea, polypharmacy, etc.) before jumping to the conclusion that an individual has Alzheimer’s. What does the term "developmental disabilities" mean in relation to Down syndrome?This term refers to incomplete development of the brain, which leads to both mental retardation and to slowed and/or incomplete mastery of physical coordination. What is known about brain pathology in regard to developmental difficulties?Pathology in the Down syndrome brain includes a slightly smaller brain size for age, a shorter diameter for the anterior-posterior brain measurement, an unusually steep slope to the posterior portions of the brain and an insufficiently developed superior temporal gyrus. It is not known in what way these features contribute to the developmental disabilities of Down syndrome.Estimates vary, but a reasonable conclusion is that 25 percent or more of individuals with Down syndrome over age 35 show clinical signs and symptoms of Alzheimer's-type dementia. The percentage increases with age. In the general population, Alzheimer's disease does not usually develop before age 50, and the highest incidence (in people over age 65) is between five and 10 percent. The incidence of Alzheimer's disease in the Down syndrome population is estimated to be three to five times greater than in the general population.
23 Brain pathology Slightly smaller brain size for age. Shorter diameter for the anterior-posterior brain measurement.An unusually steep slope to the posterior portions of the brain.Insufficiently developed superior temporal gyrus.It is not known in what way these features contribute to the developmental disabilities of Down syndrome.The primary function of the Superior temporal gyrus is thought to be self awareness and laughter.The superior temporal gyrus includes an area (within the Sylvian fissure) where auditory signals from the cochlea (relayed via several subcortical nuclei) first reach the cerebral cortex. This part of the cortex (primary auditory cortex) is involved in hearing. Adjacent areas in the superior, posterior and lateral parts of the temporal lobes are involved in high-level auditory processing. In humans this includes speech, for which the left temporal lobe in particular seems to be specialized. Wernicke's area, which spans the region between temporal and parietal lobes, plays a key role (in tandem with Broca's area, which is in the frontal lobe). The functions of the left temporal lobe are not limited to low-level perception but extend to comprehension, naming, and other language functions. Sound processing is controlled by the temporal lobes- in the Broca’s area and Wernicke’s area.The underside (ventral) part of the temporal cortices appear to be involved in high-level visual processing of complex stimuli such as faces (fusiform gyrus) and scenes (parahippocampal gyrus). Anterior parts of this ventral stream for visual processing are involved in object perception and recognition.The medial temporal lobes (near the Sagittal plane that divides left and right cerebral hemispheres) are thought to be involved in episodic/declarative memory. Deep inside the medial temporal lobes, the hippocampi seem to be particularly important for memory function - particularly transference from short to long term memory and control of spatial memory and behavior. Additional images
24 Brain PathologyPost-mortem brain studies show that virtually all persons over 50 with Down syndrome have pathological plaques and neurofibrillary tangles- the hallmarks of Alzheimer’s disease, However only 15-20% of individuals with Down syndrome have clinical signs of the disorderThe prevalence of Alzheimer’s is much more prevalent than in the general population.It is important to rule out treatable causes of decline in mental functioning (thyroid problems, B vitamin deficiencies, vision and hearing problems, depression, sleep apnea, polypharmacy, etc.) before jumping to the conclusion that an individual has Alzheimer’s.
26 Seizures Seizures occur in 6% of individuals with Down syndrome Most common seizure type is generalized tonic clonicAge of onset typically bimodal distributionOnset before age 3Onset after age 13
27 Neurologic Language Delays Central Hypotonia Oral motor dysfunction Receptive language tends to be better than expressiveCentral HypotoniaGross motor DelaysOral motor dysfunctionPoor oral motor coordinationDecreased sensory awarenessWhile most people with Down syndrome have some degree of mental retardation, recent research shows that not all parts of the brain are affected similarly. Professionals once thought that people with Down syndrome were slow to learn everything, and could not master some material. Now we know that this is not true; people with Down syndrome have some real strengths in their learning styles, as well as real disabilities.Just like with the typical population, no one generalization will apply to every person with Down syndrome. However, there are significant trends that teachers can use to help their students learn and become a part of the classroom.
28 The level usually falls into the mild to moderate range. Most people with Down syndrome have some level of intellectual disability.The level usually falls into the mild to moderate range.Not indicative of the many strengths and talents that each individual possesses.Children with Down syndrome learn to sit, walk, talk, play, toilet train and do most other activities only somewhat later than their peers without down syndrome.Down syndrome is the most frequently occurring chromosomal abnormality, occurring once in approximately every 800 to 1,000 live births. Over 350,000 people in the United States have Down syndrome. Down syndrome is usually caused by an error in cell division called non-disjunction. People with Down syndrome have an extra, critical portion of the number 21 chromosome present in all, or some, of their cells. This additional genetic material alters the course of development and causes the characteristics associated with the syndrome. Most people with Down syndrome have some level of mental retardation; however, the level usually falls into the mild to moderate range and is not indicative of the many strengths and talents that each individual possesses. Children with Down syndrome learn to sit, walk, talk, play, toilet train and do most other activities only somewhat later than their peers without down syndrome.Early intervention services, which begin shortly after birth, help children with Down syndrome develop to their full potential. Quality educational programs, along with a stimulating home environment and good medical care enable people with Down syndrome to become contributing members of their families and communities
29 Early intervention services, which begin shortly after birth, help children with Down syndrome develop to their full potential.Quality educational programs, along with a stimulating home environment and good medical care enable people with Down syndrome to become contributing members of their families and communities.
30 Early Intervention Works! Just a decade ago, the prognosis for these childrenwas not as bright as it is today.Early Intervention programs from birth to age 3have shown impressive results.Families receive training in how to help their children learn tomaximize the development that occurs in the early years.In addition, many children receive:PTOTSpeech TherapyAqua therapyHippotherapyMusic therapyInfant education individually and in groups
31 InclusionStudies have demonstrated social, academic, and behavioral benefits for students with disabilities who are placed in inclusive settings, without negatively impacting the educational experience of the other students. (Becker, Dumas, and Roberts ,2000)Many children with Down syndrome, if they were in the special education or self-contained classroom, would not have the same amount of language development as their non-disabled peers."The learning characteristics of students with Down Syndrome are more similar to their regular education peers than they are different.However, language and motivational deficiencies may necessitate more highly structured, sequenced activities, with smaller bits of information presented at a time, and lots of rewards and praise built into the design of the lesson" (Wolpert, 2001).
32 Strong short-term visual memory High social/interpersonal intelligence Learning strengthsStrong short-term visual memoryHigh social/interpersonal intelligenceLearning weaknessesPoor short-term auditory memoryDifficulty with basic math skillsAlthough these children generally have poor short term audiitory memory skills, their visual motor skills are relatively strong this cognitive pattern is consistent with functional neuroimaging studies that reveal impairments within and between the frontal and pareital lobes including the superior and inferior frontal gyrus and includes Broca’s speech area.
33 Short Term Auditory Memory “Working Memory”“Verbal Memory”It relates to the speed with which we can hold process understand and assimilate languageIt relates directly with the speed with which we can articulate words, influences the speed at which children learn new words and learn to read.
34 Short-term auditory processing impairment occurs almost universally in people with Down syndrome of all ages.Visual tools that support the short-term processing of concepts are amongst the most useful resources any school can implement to support students with Down syndrome and their teachers.Essential basics every classroom teacher needsShort-term auditory processing impairment occurs almost universally in people with Down syndrome of all ages. Visual tools that support the short-term processing of concepts are amongst the most useful resources any school can implement to support students with Down syndrome and their teachers..Please take a moment to read these key pieces of information. Your understanding of the impact of short-term auditory processing impairment, and how visual tools can support students with Down syndrome in managing every aspect of school life, will enable your students with Down syndrome to be more successful learners, and will make you a more effective teacher.These resources are readily available, and highly recommended - and most of them are free:Short term memory - an information sheet prepared by Sandy Alton, Education Adviser, DSA UK. Explains how short-term auditory processing impairment impacts across the curriculum and school environment, and gives practical strategies for support. This could be the single most important piece of information you put into practice in your classroom and playground.I See What You Mean, Pts 1 and 2 - published online, in Disability Solutions magazine. An excellent introduction to and explanation of using visual tools to support students with Down syndrome.Visual Aids for LearningReady-for-use packages of visual tools (images) for home and school. NSW based company.Living with Down SyndromePublished by the Down Syndrome Educational Trust is one of the best introductory surveys of the potential impacts of Down syndrome on both individuals and families. It is available to read online or to download (34 pages).Teaching Students with Down Syndrome, by Carol Johnson, Educational Psychologist. A very useful 4 page summary of the most important factors to consider for your students with Down syndrome to be successful learners.
35 Make Learning VisualAccompany keywords and checklists with pictures or symbols.Use keywords icons or diagrams rather than full text.Underline and highlight key words.
36 Communication through sign language,picture cards andassistive communication devicespromotes cognitive and language development even when children lack the oral-motor skills to speak.
37 Teaching StrategiesShort attention spans are also prevalent among students with Down's. Direct instruction in short periods of time along with smaller chunks of activities will help to support learning. Introducing new material slowly, sequentially and in a step by step fashion will help to ensure maximum learning occurs.Distractibility: Down's students are often easily distracted. You'll need to employ strategies that work to minimize distractions such as keeping the student away from the window, using a slightly more structured environment, keeping the noise level down and having an orderly classroom where students are free from surprises and know what your expectations, routines and rules are.Speech and Language: Down's students all suffer from serious problems such as hearing difficulties and articulation problems. Sometimes they will require speech/language intervention and a great deal of direct instruction. In some cases, augmentative or facilitated communication will be a good alternative for communication. Use patience and model appropriate interactions at all times.Behavior management techniques used for other students should not differ for the student with Down's Syndrome. Again, positive reinforcement is a much better method than anything punitive. Reinforcers need to be meaningful.Social Skills Training -Appropriate Boundaries and Sexuality training should start in Elementary grades
38 Things to keep in mindWhen behavior changes, escalates or new behaviors develop, think about pain or undiagnosed/untreated medical conditions first.Make sure the routine is consistent and predictable.Make sure accommodations for special communication needs are being addressed.
39 Services Available Continuum of Care Project 925-2350 NM SAFE ProgramLINC-Library Information Network CenterCDD-Center for Development and DisabilityPRO-Parents Reaching OutDDSD-DOH