The Health Services Center

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S
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Syndrome Specific Somatic Conditions
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S
Syndrome Specific Somatic Conditions
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S
Cephalic
Syndrome Specific Somatic Conditions
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Fourth Essential Concept

• Microstomia and Macroglossia associated with Down
  syndrome

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S
Cephalic
Syndrome Specific Somatic Conditions
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S
Cephalic
Cardio Pulmonary
Syndrome Specific Somatic Conditions
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Cardiac Defects

• Down Syndrome 40-50 cardiac defect rate
• Fetal Alcohol Syndrome 29-41 cardiac defect
  rate
• Fragile X up to 52 cardiac defect rate
• Turner Syndrome up to 50 cardiac defect rate
• William Syndrome up to 75 cardiac defect rate

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Syndromes Associated with Structural Heart Defects
Townes-Brocks
Kearns-Sayre Syndrome
Ehler-Danlos Syndrome
Adams-Oliver
Treacher Collins Syndrome
Laurence-Moon-Biedle
Eisenmenger Syndrome
Allagile Syndrome
Tuberous Sclerosis
Leopard Syndrome
Ellis Van Crevald Syndrome
Apert Syndrome
Turner Syndrome
Marfan Syndrome
Emery-Dreifuss Dystrophy
Cantrell Syndrome
VACTERL Syndrome
Mucopolysaccharidosis
Fanconi Anemia
Carpenter Syndrome
VATER Syndrome
Muscular Dystrophy
Farber Syndrome
Cayler Syndrome
Velo-Cardio-Facial Syndrome
Osler-Weber-Rendu
Fetal Alcohol Syndrome
CHARGE Syndrome
Von Hippel Lindau Syndrome
Progeria
Fragile X Syndrome
Congenital Rubella
William-Beuren Syndrome
Scimitar Syndrome
Friedreich Ataxia
De Lange Syndrome
Williams Syndrome
Shones Syndrome
Hemorrhagic Telangiectasia
Dejerin-Soltas
Wolff-Parkinson-White
Shprintzen Syndrome
Heterotaxy Syndrome
DiGeorge Syndrome
Zellweger Syndrome
Smith Magenis
Holt-Oram Syndrome
Down Syndrome
Smith-Lemli-Opitiz
Dubowitz Syndrome
Ivemark Syndrome
TAR Syndrome
Edwards Syndrome
Kartagener Syndrome
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S
Cephalic
Cardio Pulmonary
Syndrome Specific Somatic Conditions
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S
Cephalic
Cardio Pulmonary
Genito Urinary
Syndrome Specific Somatic Conditions
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Genitourinary
Testicular Gynecological Kidney
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S
Cephalic
Cardio Pulmonary
Genito Urinary
Syndrome Specific Somatic Conditions
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S
Cephalic
Cardio Pulmonary
Genito Urinary
Syndrome Specific Somatic Conditions
Musclo Skeletal
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Musculoskeletal

• Limb Deformities
• Connective Tissue Disorders
• Osteoporosis
• Degenerative Joint Disease

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Trauma Risk Atlantoaxial Instability (AAI)

• Approximately 15 of people with Down syndrome
  will have positive x-ray findings indicative of
  AAI (x-ray findings are variable over time)
• 13-14 Asymptomatic
• 1-2 Symptomatic
• Symptomatic AAI is correlated with spinal cord
  injury at the C1-C2 level
• All athletes with Down syndrome currently must be
  screened for AAI prior to participation in some
  Special Olympics sports (see list)

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Trauma Risk Osteopenia/Osteoporosis
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• The percentage of
• Special Olympics athletes
• (average age 24)
• with low bone density

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S
Cephalic
Cardio Pulmonary
Genito Urinary
Syndrome Specific Somatic Conditions
Musclo Skeletal
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S
Cephalic
Cardio Pulmonary
Genito Urinary
Syndrome Specific Somatic Conditions
Musclo Skeletal
Endocrine - Metabolic
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Metabolic / Endocrine

• Obesity
• Hypothyroidism
• Hyperthyroidism
• Hormonal Changes

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Fifth Essential Concept
The Continuum of Quality Care
Each primary complication may be associate with
one or more secondary consequences.
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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S
Cephalic
Cardio Pulmonary
Genito Urinary
Syndrome Specific Somatic Conditions
Musclo Skeletal
Endocrine - Metabolic
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S
Cephalic
Cardio Pulmonary
Genito Urinary
Secondary Health Consequences
Syndrome Specific Somatic Conditions
Musclo Skeletal
Endocrine - Metabolic
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The Fifth Essential Concept

• Rampant dental decay secondary to the patients
  inability to perform self-care

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The Fifith Essential Concept

• Aspiration pneumonia

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Five Essential Concepts
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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Summary . . .

• Injury to the brain (genetic or acquired)

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Summary . . .

• Underlying neurodevelopmental disorder causes
  disability

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Summary . . .

• Primary cerebrogenic complications

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Summary . . .

• Syndrome-specific somatic conditions

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Summary . . .

• Secondary health consequences

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Preventive Health
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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DSP / Patient Relationship

• Role Model
• Trust / Insipration
• Live by example

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Sleep
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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Sleep
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Sleep

• Lack of Sleep Leads to
• Increases anger, sadness, irritability
• Increased cardiovascular disease
• Increased obesity
• Increased hypertension
• Decreased immune function

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Sleep

• Duration
• Quality
• Avoid caffeine and nicotine close to bedtime.
• Avoid alcohol.
• Exercise, but finish 3 hours before bedtime.
• Establish a regular, relaxing bedtime routine
• Watch the effect of medications
• Avoid late night television
• Avoid Obesity

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Nutrition
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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Obesity

• Hypertension (high blood pressure)
• Osteoarthritis
• Dyslipidema
• Type 2 diabetes
• Coronary heart disease
• Stroke
• Gallbladder disease
• Sleep apnea and respiratory problems
• Some cancers (endometrial, breast, and colon)

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Obesity
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Portion Size
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Portion Size
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The Wrong Foods

• Reflux
• Constipation
• Sleep Disturbances
• Cavities
• Osteoporosis

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Nutrition
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Hygiene
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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Hygiene

• Ears
• Teeth
• Body
• Feet

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Aural Hygiene

• 53 need regular ear canal hygiene
• 38 failed hearing exam

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Aural Hygiene

• 1) Do not use Q-tips in ear
• Consider using Debrox drops
• Update hearing prescription frequently
• Avoid in-ear headphones

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Oral Hygiene

• Estimate
• IQ
• Employment Potential
• Life Expectancy
• Friendship Ability

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Oral Hygiene

• Estimate
• IQ
• Employment Potential
• Life Expectancy
• Friendship Ability

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Oral Hygiene

• Poor oral health leads to
• Periodontal disease
• Heart attack, Stroke
• Endocarditis, Ulcers
• Tooth Pain
• Behaviors
• Aspiration
• Tooth Loss
• Weight gain

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Oral Hygiene

• Proper oral hygiene includes
• Brush regularly
• Floss regularly
• Avoid soft drinks!
• Avoid chew tobacco
• Regular dental visits

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Body Hygiene

• Decubitus Ulcers
• Moisture
• Pressure
• Circulation
• Shear

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Body Hygiene

• Preventing decubitus ulcers includes
• Periodic repositioning lt 2 hours
• Padding prone areas
• Cleaning prone areas
• Drying prone areas
• Proper Clothing
• Proper hydration / nutrition

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Foot Hygiene

• Poor foot hygiene can lead to
• Fungal or bacterial infections
• Painful walking
• Ulcers
• Digit loss

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Foot Hygiene

• Proper foot hygiene includes
• Regular washing
• Regular moisturizing
• Not cutting nails too short
• Fresh socks
• Clean shoes
• Letting the feet breathe

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Physical Activity
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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Physical Activity

• Stretch regularly, increase flexibility
• Do muscle strengthening exercises
• Maintain aerobic conditioning
• Moderate- 30 min., 5 or more days per week
• Vigorous- 20 min., 3 or more days per week
• Special Olympics
• Special Olympics Unified Sports

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The Doctor
The Continuum of Quality Care
Improving Health Outcomes and Employment
Opportunities for Individuals with
Neurodevelopmental Disorders and Intellectual
Disabilities
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Surgeon General Satcher, 2002

• Health Disparities for People with ND/ID
• Lack of training of health care providers
• Lack of access to health care providers
• Medicaid reimbursement

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• A recent survey of the following groups was
  conducted in order to assess the general state
  of medical and dental education in the United
  States
• U.S. Medical School Deans
• U.S. Dental School Deans
• U.S. Medical Residency Directors
• U.S. Dental Residency Directors
• U.S. Medical Students
• U.S. Advocacy and Patient Care Groups

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• 81 of medical students will graduate without
  ever having ANY clinical training in how to care
  for a person with ND/ID
• 90 of primary care residency programs offer no
  formal training in ND/ID

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What You Can Document

• What time does the behavior occur?
• Regular or Irregular intervals
• Time ranges and duration ranges
• Negative space
• What happens before the behavior?
• What personal interactions is the person having
• What activities are they engaged in
• What environment are they in
• Negative space

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What You Can Document

• What triggers the behavior?
• Is a trigger identifiable
• Is there more than one trigger
• Negative space
• What happens during the behavior?
• What does the person do
• How intense is the behavior
• Negative space

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What You Can Document

• What triggers the end of the behavior?
• Is a trigger identifiable
• Is there more than one trigger
• Negative space
• What happens after the behavior?
• What does the person do
• What is their reward or punishment
• What is their secondary gain
• Negative space

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What You Can Document

• What is your role in the behavior?
• Are you a trigger
• Are you a reward or a punishment
• Negative space
• What modifies the frequency or severity of
  behavior?
• 1) What limits or extends the time of the
  behavior
• 2) What increases or decrease the frequency of
  the behavior
• 3) What increases or decreases the severity of
  the behavior
• 4) Negative space

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THANK YOU!
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