Overview of Disabilities

1. Overview of Disabilities Considerations for the Dental Health Professional

2. Introduction to disabilities Why is it important to know about disabilities? More people living in the community More people living longer More people returning to the community Americans with Disabilities Act (1991) More people in the community seeking services, including dental services

3. Introduction (cont) Many myths and misconceptions regarding: People who have a disability Providing dental services for persons who have a disability The intent is to provide some basic information regarding common disabilities and reduce some barriers to oral health care

4. Do�s and Don�ts Offer assistance as you would anyone else Respect �no thank you� Ask pertinent and appropriate questions regarding the disability Always talk directly to a person with a disability rather than accompanying person

5. Do�s and Don�ts (cont) When talking to a person in a wheelchair, sit down to be at the same eye level Be flexible Be sensitive to architectural barriers that may exist in your office Use �people first� language; i.e., � a person with a disability� rather than a �disabled person�

6. Do�s and Don�ts (cont) You may unintentionally offend someone by use of a certain word or phrase; if so, apologize and move on � don�t dwell on it Let the patient guide you as to what they prefer regarding terms and phrases Be sensitive to and aware of cultural differences

7. Do�s and Don�ts (cont) People with disabilities are a broad and diverse minority so �politically correct� terms may be hard to find that please everyone View the person as an individual first with the disability as a component of that person, no more, no less

8. Format Definition Incidence Etiology Diagnosis Classification Findings Oral findings Medical management Dental management Resources Today: MR, DD, EP, and CP Next time: Spinal Cord Injury, ADHA, Hearing Impairments, Visual ImpairmentsToday: MR, DD, EP, and CP Next time: Spinal Cord Injury, ADHA, Hearing Impairments, Visual Impairments

9. DEVELOPMENTAL DISABILITY A severe, chronic disability: Attributable to mental and/or physical impairment Manifested before age 22 Likely to continue indefinitely

10. DEVELOPMENTAL DISABILITY Results in substantial functional limitations in 3 or more areas of major life activities Self care Receptive of expressive language Learning Mobility Self direction Independent living Economic self sufficiency

11. DEVELOPMENTAL DISABILITY Needs special interdisciplinary or generic care for an extended duration that is individually planned and coordinated

12. Mental Retardation Based on the following three criteria: Significant subaverage intellectual functioning (IQ) Deficits in 2 or more adaptive skill areas Manifests before the age of 18 (AAMR, 1992)

13. Incidence 2.5 - 3% of the general US population (1990 census) 6.2 - 7.5 million people 1 out of 10 American families directly affected by MR

14. Etiology Genetic conditions: e.g. Down Syndrome, Fragile X, Neurofibromatosis Inborn errors of metabolism: e.g. PKU Prenatal influence: Rubella, Drug abuse Perinatal: e.g. Anoxia, Toxemia Postnatal: e.g. Poverty, Lead ingestion, Trauma, Cultural deprivation

15. Diagnosis Standardized intelligence test and standard adaptive skills test Describe person�s strengths and weaknesses across four dimensions Interdisciplinary team determines needed supports across the four dimensions

16. Classification Normal IQ range 80-110 (Stanford Binet) Mild- 85% of MR population Moderate- 10-12% Severe- 2-3% Profound- 1-2%

18. Classification Mild IQ 52-67 (S-B) often undistinguishable from normal independent may need help under stress

19. Classification Moderate IQ 36�51 (S-B) bathes and dresses self simple chores or errands

20. Classification Severe IQ 20-35 (S-B) feeds with spoon/fork bathes with supervision often toilet trained

21. Classification Profound IQ 0 �19 (S-B) may self-feed with spilling needs assistance with all self help may be non-verbal

22. Adaptive skill areas Communication Self care Home living Social skills Community use Work Self direction Health and safety Functional academics Leisure

23. Medical management Variable, dependent on other or associated health/behavioral problems

24. Dental management Variable, but usually little different from general population Adapt patient management/communication to functional level Adapt patient education to cognitive level Involve caregiver

25. Resources American Association of Mental Retardation www.aamr.org The Arc www.thearc.org

26. Down syndrome A chromosomal abnormality: 47 chromosomes instead of 46 An extra partial or complete 21st chromosome Also called Trisomy 21

27. Incidence 1 in 800-1000 5% of cases linked to paternal age Usually linked to maternal age 1 in 400 at age 35 1 in 110 at age 40 1 in 35 by age 45

29. Etiology Caused by an error in cell division Trisomy 21 - ~94% Translocation - 3-4% Mosaic - 2-4%

30. Characteristics hypotonia (low muscle tone) flat facial profile underdeveloped midface thick, furrowed tongue upward slant to eyes simian crease hyperflexibility heavy epicanthal folds short stature fifth finger has one flexion furrow instead of two atlanto axial instability round face saddle nose low set ears short, stubby fingers speckling of iris (Brushfield spots) wide gap between first and second toes

31. Facial Characteristics Underdeveloped midface saddle nose low set ears

32. Facial Characteristics Upward slant to eyes heavy epicanthal folds round face

36. Oral Characteristics

37. Hypotonia

38. Simian Crease

39. Diagnosis Prenatal tests/amniocentesis Chromosomal studies

40. Medical management Higher incidence of heart problems 35-40% incidence Ventricular septal defect (VSD) common Higher incidence of upper respiratory infections and pneumonias Higher incidence of acute lymphocytic leukemia Premature aging

41. Dental management Higher incidence of unresponsive periodontal disease Higher incidence of congenitally missing teeth Higher incidence of dental crowding Significantly delayed eruption Tooth size and shapes differ from norm Check need for antibiotic coverage for dental procedures (AHA guidelines)

42. Resources National Down Syndrome Society http://www.ndss.org/ National Down Syndrome Congress http://www.ndsccenter.org/ Down Syndrome Information Network http://www.down-syndrome.info/

43. Videos �Educating Peter� (30 min) A story of a child with Down syndrome and his classmates testing the limits of a law stating whenever possible, children with disabilities should be included in class with typically developing children. �Graduating Peter� The follow-up to �Educating Peter� Ambrose Video Publishing 1290 Avenue of the Americas, Suite 2245 New York, NY 10104

44. Epilepsy Comes from the Greek word for seizure Recurrent seizures are the major chronic recurrent symptoms Symptoms of the brain�s temporary bursts of abnormal electrical activity

45. Incidence 0.5% of the US population has been diagnosed with a recurrent seizure disorder 10% of the population will have at least one seizure in their lifetime highest prevalence in children between 2 and 5 and at puberty may still see incidence rates of 9-11% but this is due to the inclusion of febrile seizures which can occur in infants (these should not be included)

46. Etiology Symptomatic (identifiable cause) head trauma (most common) neoplasms metabolic disorders drug withdrawal infections e.g. meningitis, encephalitis Idiopathic

47. Diagnosis Computed tomography (CT) and magnetic resonance imaging (MRI) Electroencephalography (EEG) History

48. Seizure Type Determined by: Part of brain involved Number of brain cells involved Duration of electrical discharge Symptomatology

49. InternationalClassification Partial Complex partial Generalized tonic clonic absence myoclonic atonic

50. Medical management Anticonvulsants Neurosurgery Vagal nerve stimulation (electrical stimulation through implant) Ketogenic diet

51. Dental management Thorough history: What happens during the seizure? What do they look like? How long do they last? Is there an aura and what is it? Are there triggers that tend to set off the seizures? How frequent are the seizures? �Control� does NOT mean no seizures

52. Dental management Thorough history (cont) What happens after the seizure? How long has the person been on the current drug schedule? When was the last time the person was seen for an evaluation of the seizure activity? Has there been any change in frequency of seizures or alertness recently?

53. Dental management Seizures are NOT an emergency, but: Be prepared to establish airway Be prepared to provide positive pressure oxygen/air No contraindication to local anesthetic If using sedation, be aware of potentiation of drugs and effects

54. First Aid

55. People with Epilepsy Socrates Martin Luther Napoleon Handel Lord Byron Gary Howatt Former hockey player Alexander the Great Julius Caesar William Pitt Alfred Nobel Van Gogh Tony Coelho Former US Rep Ca

56. First Aid Prolonged or clustered seizures sometimes develop into non-stop seizures, a condition called status epilepticus Status epilepticus IS an emergency

57. Incidence: status epilepticus 3-8% in people diagnosed with epilepsy Male:female ratio is equal 75% of cases in childhood occur before age 3

58. Status epilepticus first aid Brain equivalent to a heart in ventricular fibrillation Can occur with no previous history of seizure disorder First aid CPR/BLS prn Call 911

59. Resources Center for Disease Control www.cdc.gov American Epilepsy Society www.aesnet.org/ www.Epilepsy.com Epilepsy Foundation www.efa.org

60. Videos �How to Recognize and Classify Seizures� (25 min.) Demonstrates how seizures are diagnosed and gives classification of seizures types �The Comprehensive Clinical Management of the Epilepsies� (17 min.) Discusses the diagnosis, treatment and follow-up with regards to medical, psychosocial, educational, rehabilitative and prognostic components. Epilepsy Foundation of America

61. Cerebral Palsy Any disorder of movement and posture that results from a nonprogressive abnormality of the immature brain

62. Incidence ~.5-1% Incidence has been reduced due to improved neonatal intensive care units (NICU�s) Usually manifests by 1 year old

63. Diagnosis No specific diagnostic criteria Persistence of primitive reflexes Asymmetric tonic neck response, (ATNR) e.g., should not persist beyond 4 months Moro reflex (embrace response), e.g., should not persist beyond 6 months

64. ATNR Turn head to the side and opposite side arm and leg rise in �fencer�s� position. Will not release until head is moved back to the front.

65. MORO reflex Infant in semi upright position. Head allowed to fall backward with immediate resupport. Arms abduct and extend. Upper extremities flex and adduct.

66. Causative Factors 60% known cause; 40% unknown Numerous factors/causes Pre-term, low birthweight (most common) Fetal malformations, intrauterine infections, neonatal complications, anoxia, hypoxia, sepsis, meningitis More frequent in twins

67. Apgar scale Assessed at 1 and 5 minutes and may be repeated at 5 minute intervals for depressed infants If 20 minute Apgar is 0-3, likelihood of cerebral palsy is 57% 10 point scale 5 signs 0-2 score

68. Apgar Scale

69. Classification Classified by site of injury to brain and extremities or body parts effected Diplegia: legs effected more than arms Paraplegia: legs only (rare) Quadriplegia: arms and legs effected Hemiplegia: one side, arms more than legs are effected

71. Classification: site Spastic: motor cortex or pyramidal tract is the site of injury Athetoid: injury is extrapyramidal, usually at the basal ganglia Mixed

72. Spastic cerebral palsy Muscle tone increased with characteristic �clasped knife� quality As extremity is moved, much resistance initially, then it gives way abruptly like a closing pocket knife ~35% will develop seizure disorders

73. Athetoid cerebral palsy Major problem is controlling movement and maintaining posture Variable changes in muscle tone Facial muscles are more effected Speaking problems are more common Head and neck involvement common

74. Oral Manifestations Increased salivary flow; decreased ability to control it; leads to increased drooling May have increased or decreased gag reflex Higher incidence of bruxism Higher incidence of primitive bite reflex Class II Division I malocclusion common Anterior dental trauma common due to malocclusion and poor self-protective mechanisms

75. Medical Management Aim is to prevent secondary problems due to abnormal or asymmetrical tone Botox injections being used with success to reduce muscle tightness Neurosurgical procedures have had limited success

76. Dental Management Diazepam to decrease tone in spastic cerebral palsy Nitrous oxide can help reduce muscle tone in spastic cerebral palsy Positioning in dental chair Maintain bend in hips; avoid extension Good head/neck support; avoid extension Bite block or mouth prop if primitive bite reflex is active

77. Autism Now part of Autism Spectrum Disorder Classified under DSM IV as a Pervasive Developmental Disorder (PDD) Characterized by profound withdrawal from contact with people including parents Often obsessive; problems with change DSM: Diagnostic and Statistical Manual (for psychological/psychiatric diagnoses) IV: fourth version Revised version (IVR) available but few changesDSM: Diagnostic and Statistical Manual (for psychological/psychiatric diagnoses) IV: fourth version Revised version (IVR) available but few changes

78. DEFINITION � DSM IV Qualitative impairment in social interaction Qualitative impairment in communication Restricted, repetitive and stereotyped patterns of behavior Abnormal or impaired development prior to age 3 years

79. Pervasive Developmental Disorder Includes: Autistic disorder Rett�s disorder (only females) Childhood disintegrative disorder Asperger�s disorder (less language delay) PDD-NOS (�not otherwise specified�)

80. Autistic Disorder Also has been called: Early infantile autism Childhood autism Kanner�s autism Childhood schizophrenia

81. Incidence ~5/10,000 Third most common developmental disability in the US Seems to be increasingly diagnosed Boys:girls = 4-5:1 Girls more likely to exhibit more severe mental retardation

82. Etiology Essentially unknown, however� Some strong evidence for linkage to chromosomes 2q, 7q, 16p and 17q

83. Diagnosis Usually appears before 3 years old Exhibits a number of symptoms Poor social interaction and communication Deviant patterns of behavior/interest/activity No imaginative play Delay in attaining milestones Question hearing impairment

84. Diagnosis Symptoms (cont.) Stereotyped behavior e.g. rocking, hand waving, spinning Little to no spontaneous speech; echolalia May exhibit self-injurious behavior (SIB) Make little or no eye contact Don�t like to be touched Emotionally flat affect usually but can flare

85. MH? MR? Arguments have persisted whether autism is a psychiatric/emotional disorder or an intellectual deficit Has swung back and forth Difficult to test for IQ because of inconsistent responses Currently viewed as both emotional disorder and intellectual deficit

86. Coexisting conditions Seizure disorder Fragile X Tuberous sclerosis PKU

87. Medical management Many drugs and combinations and therapies have been used with inconsistent results Some advocate use of positive physical supports because there is some evidence that deep pressure has a calming effect Behavior can be very inconsistent

88. Dental management Keep directions simple - do not give multiple commands at the same time Many are �sound sensitive� and react strongly, especially to suction; however, if turned on BEFORE entering the treatment area so it is part of ambient sound, response is much less or absent

89. Dental management Use a calm, consistent voice when giving guidance/speaking May take multiple appointments before treatment is �accepted� Persistence and patience will prevail

90. Attention Deficit Hyperactivity Disorder ADHD is a behavioral disorder characterized by impulsivity, inattention, and motor restlessness

91. Incidence 3 � 5% of school-aged children Male predominance 3:1 It typically affects children and adolescents with an onset prior to age 7

92. Etiology Unknown Hereditary Some evidence linked to maternal alcohol consumption and cigarette smoking during pregnancy

93. Diagnosis Based on behavioral observations and assessments from multiple sources including family members, educational professionals and physicians Most frequently diagnosed at school age

94. Dental/Oral Considerations Shorter dental appointments Give patient clear rules or instructions to follow one at a time Make eye contact during each request Reinforce good behavior Stop unacceptable behavior before it escalates

95. Medical management Behavioral programs Medication (when required) Psychostimulants Ritalin Adderall Dexedrine Concerta Strattera (non-stimulant)

96. Resources Children and Adults with Attention Deficit Disorder http://www.chadd.org/ Attention Deficit Disorder Association http://www.add.org/ National Institute of Mental Health http://www.nimh.nih.gov/publicat/adhdmenu.cfm

97. Hearing Impairment 25-45 dB loss: mild 45-70 dB loss: moderate 70-90 dB loss: severe

98. Hearing Impairment When hearing is impaired to the extent it has no practical value for purpose of communication

99. Incidence ~10% of US Population (28 million) 30% people over 65 14% people between 45-64 8 million age 18-44 7 million children

100. Etiology Heredity Pre /peri /post natal influences Prematurity

101. Hearing impairment Determine ability to read lips Maintain eye contact Use face shield rather than mask so patient can see lips Speak clearly (no need to shout)

102. Hearing aids Use gestures and facial movements and written materials to communicate Sometimes, it is best to remove or turn off the aids due to �feedback� and/or the amplified sounds of the dental office, e.g., suction, handpiece

103. Hearing impairment Patient is NOT a good lip reader: If it is a basic procedure and the patient can read and write, develop 3x5 cards with what you want to say Interpreter�?

104. Interpreters Required under Americans with Disabilities Act? NO Under Title III, places of public accommodation, it says there should be �effective communication� Patient requests, are you required? NO Act calls for �reasonable accommodation�

105. Interpreter services If negotiated and determined that interpreter would be helpful, cost is borne by the office Patient cannot be billed Insurance cannot be billed Average cost is ~$40/hr

106. Interpreter services Address patient and not interpreter Interpreter cannot clarify message Interpreter is merely translating the message into another format Speak a little slower so interpreter can keep up; use natural pauses

107. Relay service Exists throughout the US Enables hearing impaired person to send and receive messages to/from anyone Non hearing impaired person does not need TDD/TTY to communicate Available through the local phone company Ohio: 1-800-750-0750

108. Muscular Dystrophies Muscular dystrophy (MD) refers to a group of genetic diseases characterized by progressive weakness and degeneration of the skeletal or voluntary muscles which control movement

109. Muscular Dystrophies The muscles of the heart and some other involuntary muscles are also affected in some forms of MD, and a few forms involve other organs as well The major forms of MD include myotonic, Duchenne, Becker, limb-girdle, facioscapulohumeral, congenital, oculopharyngeal, distal and Emery Dreifuss

110. Incidence 0.14 per 1,000 children MD can affect people of all ages Although some forms first become apparent in infancy or childhood, others may not appear until middle age or later Duchenne is the most common form of MD affecting children Myotonic MD is the most common form affecting adults

111. Etiology Unknown Inherited

112. Diagnosis Enlargement of certain muscles Progressive weakness of muscles Lordosis (forward/inward curvature of the spine with abdominal protuberance) Gait � waddling Progressive muscle wasting

113. Dental/Oral Manifestations Facial musculature may be affected Gaping lips Eyes difficult to close completely (protective eye wear during treatment)

114. Spinal Cord Injury Impairment of the spinal cord function resulting from the application of external traumatic force The effect is partial or complete paralysis to a degree related to spinal cord level and extent of injury

115. Incidence 32 injuries per million population 7,800 injuries in the US each year 250,000 - 400,000 living with SCI 82% male vs. 18% female Avg. age at injury - 33.4 Most frequent age of injury - 19

116. Etiology Motor vehicle accidents (44%) Acts of violence (24%) Falls (22%) Sports (8%) (2/3 from diving) Other (2%)

117. Classification Dependent on level of spinal cord injury

118. Levels of the Spinal Cord

119. Levels of the Spinal Cord

120. Dental/Oral Considerations Adaptive techniques if hands are affected Wheelchair considerations Oral hygiene instructions to caregivers

121. Spina Bifida A neural tube defect in which the spine fails to close properly during the first month of pregnancy In severe cases, the spinal cord protrudes through the back and may be covered by skin or a thin membrane

122. 3 Types of Spina Bifida

123. 3 Types of Spina Bifida

124. Incidence 1 out of every 1,000 newborns in the US Women who have a child with spina bifida, who have spina bifida themselves or have already had a pregnancy effected by any neural tube defect, are at higher risk

125. Etiology Unknown

126. Diagnosis Ultrasound Amniocentesis

127. Physical Characteristics Bony deformities Hydrocephalus Loss of sensation Bladder and bowel paralysis Paralysis below the lesion

128. Oral/Dental Considerations No specific oral manifestations 18 � 73% of children and adolescents with spina bifida are sensitive to latex If a shunt is present (hydrocephaly), antibiotic coverage will be necessary (AHA guidelines)

129. Visual Impairment Limitations of sight include the following: Partially sighted Low vision Legally blind Totally blind

130. Incidence 12.2 per 1,000 under the age of 18 Severe visual impairments (legally or totally blind) occur at a rate of .06 per 1,000 10 million persons in the U.S. (American Federation for the Blind)

131. Etiology Pre-natal influences Glaucoma Diabetic retinopathy Cataracts Macular degeneration

132. Dental management Move equipment out of client�s path Guide to chair Notify if leaving room and re-entering Describe each step/procedure during appointment Demonstrate OHI in client�s mouth Inform before perform Turning on suction, turning on sound

133. Resources American Federation for the Blind http://www.afb.org �Blind Walk� Simulation students are blindfolded and guided by classmates/faculty both inside and outside. Dependence on others, even classmates, is difficult. Likewise, giving unlimited trust is very challenging. The message for dentistry is that we place people in dependent/trust situations all the time and expect them to trust us, even when they have just met us. What is most interesting is that we are successful as often as we are.

134. Adjuncts to Treatment Patient positioning devices Bite blocks Mouth props

135. Vac-Pac Vacuum activated positioning aid (Olympic Medical, Seattle, WA)

136. Vac-Pac Hoses

137. Vac-Pac

138. Vac-Pac

139. Vac-Pac

140. Vac-Pac

141. Mouth Props

142. Mouth Props Be sure the mouth prop is resting on teeth distal to the canine Once in place, it must be stabilized to prevent dislodging

143. Bite Blocks

144. Bite Block with Saliva Ejector

145. Disposable Bite Block

146. Wheelchair Transfer

147. Wheelchair Transfers Unassisted One person transfer Two person transfer

148. Components of a Wheelchair

149. Armrest

150. Foot Pedal

151. Brakes

152. Unassisted Transfer Unassisted transfers are a patient-directed process Ask the patient how you can best assist There will be individual variation in transfer technique

153. Unassisted Transfer Block wheels to prevent pivoting of chair as person is making transfer

154. Unassisted Transfer Raise arm of dental chair

155. Unassisted Transfer Raise dental chair to approximately the level of the wheel

156. One Person Transfer Position your feet outside of the patient�s feet for balance and stability

157. One Person Transfer Block the knees of the patient with your knees Coordinate lift with patient There will be individual variation in transfer technique

158. One Person Transfer Position patient for comfort in the dental chair

159. Two Person Transfer

160. Two Person Transfer Gently grasp left wrist with left hand; right wrist with right hand to stabilize shoulders

161. Two Person Transfer Position hands above the knees Lift with legs, not arms or back

162. Two Person Transfer Coordinate lift verbally e.g.: 1,2,3 or ready, set, go

163. Two Person Transfer To return patient to wheelchair, you may need to lean them forward in the dental chair for proper lift position

164. Communication Video: �The Ten Commandments of Communication with People with Disabilities� (25 min) Training that uses humorous vignettes to deliver its disability awareness message. Program Development Associates 1-800-543-2119

165. Disability Awareness Video: �Look Who�s Laughing� (60 min) A funny and compelling documentary about the lives, experiences and humor of six working comedians who have various types of disabilities. Video: �Without Pity: A Film about Abilities� (56 min) A documentary celebrating the efforts of people with disabilities who live full productive lives. Program Development Associates 1-800-543-2119

166. Disability Awareness (con�t) Video: �Finding a Way� (28 min) The history of disabilities is discussed including portrayals of institutions and insane asylums. It shows how much progress has been made, but how much further there is to go toward equality for all. Video: �A Video Guide to (Dis)Ability Awareness (26 min) An orientation to the human side of the Americans with Disabilities Act. Program Development Associates 1-800-543-2119

167. Additional Web Resources

168. Additional Web Resources