Dr. Roizen has no conflicts of interest to report OBJECTIVES. Adults with Down Syndrome

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1 Dr. Roizen has no conflicts of interest to report Adults with Down Syndrome Nancy J. Roizen, MD Professor of Pediatrics Rainbow Babies and Children s Hospital Division Developmental-Behavioral Pediatrics & Psychology Department of Pediatrics Case Western Reserve University School of Medicine (HRSA-T77MC00004; Rainbow Babies and Children s Foundation) OBJECTIVES To describe the spectrum of cognitive, academic, and adaptive function in the adult with DS To predict the likely cognitive and academic function, employment, independence, and behavior problems in an adult with Down syndrome Jack who has DS and ADHD was born with feeding problems that resulted in failure to thrive necessitating tube feeding. Ventricular septal defect was corrected surgically at less than a year of age. His congenital glaucoma was corrected at 10 months of age. Recently, he had to be sedated for much needed ophthalmic and dental examinations. At 29 years of age, Jack works in a sheltered workshop that would like his father to give him medication for his ADHD.. He likes to listen to music and on weekends bowls with a friend with DS. 1

2 Sandy at 38 years of age has not had significant health problems. He attended early intervention and graduated from special education at 21 yrs of age. He uses public transportation to his full time job as a mail clerk. He is actively involved in his church, plays piano, and swims regularly. Disorders of Similar Frequency in Childhood & Adulthood Hearing problems 2 mo-3yr 66% 6-23 yr 90% Childhood (%) Adulthood (%) <50yr 64% > 50yr 93% Celiac disease 3-10% >3-10% Type 1 diabetes 0.38% >0.38% AAI 10-30% 18% Seizures 8% 8% Psychiatric disorders 18-23% 26% (Steingass et al. JDBP,2011, 32, ) Disorders of Increased Frequency in Adulthood Childhood % Adulthood % Vision problems 60-66% % Dental esp periodon 94% Thyroid disease % 12-37% Osteoporosis > 50% Obesity >50% of 3-4yr olds 89% Testicular CA NA SIR gen pop OSA 30% of 42 mo olds 94% Mitral valve prolapse 27-63% Depression Can start in adol % Dementia NA 50-70% in 7 th decade Study: 186 adults (16-74 years) Over 12 months Mental illness: DS 23.7% Other ID 40.9% (Mantry et al. J Intellect Disabil Res.2008;52: ) General Pop 26% (Kessler et al. Arch Gen Psych ;62:617-27) (Steingass et al. JDBP,2011, 32, ) 2

3 DS Compared to Adults with Other ID Higher rates depression Lower rates schizophrenia personality disorders internalizing behavior Same externalizing behavior Factors Complicate Evaluation & Diagnosis of Mental Problems Articulation problems 72% (unintelligible to strangers) Mental Age 5.6 years Medical Disorders: pain (dental, arthritis, constipation, menses), OSA diagnostic overshadowing (McGuire & Chicoine. Mental Wellness in Adults with Down Syndrome A Guide to Emotional and Behavioral Strengths and Challenges. 2006) Cognitive Function 186 with DS yrs Intellectual Disability Percentage Mild 41.4% Moderate 26.9% Severe 18.3% Profound 13.4% Longitudinal Cohort-Carr Age IQ 6 months 80 4 yrs yrs yrs yrs yrs 42.0 Carr J. J Child Psychol Psychiatry. 1988;29: Carr J. J Appl Res Intellect Disabil ;13 :1-16. Carr J. J Appl Res Intellect Disabil ;21 :

4 Longitudinal Cohort-Carr (cont) At 30 yrs mean mental age 5 yr 6 mo on Leiter Cognitive performance peak 50 yrs (vocabulary and comprehension beyond) 20 s-70 s with means in the 40 s Carr J. J Appl Res Intellect Disabil ;13 :1-16. Academic Achievement Reading, Writing, Numbers Skills in all three at level of 5 year old 75% Skills in some at 11 yr level 25% Turner S & Alborz A. Br J Edu Psychol. 2003;73: Adaptive Function Adults with DS vs adults similar levels of other ID DS better Fx home related activities: housekeeping skills, personal care, & meal-related activities Esbensen A et al. Am J Ment Retard ;113 : Seltzer MM, et al. Am J Ment Retard ;97 : Adaptive Function: 2 Group children & adolescents with mean age : 13 yr 8 mo Independent with: dressing 37% washing dishes 40% getting around neighborhood 55% Sloper P & Turner S. J Intellect Disabil Res. 1996;40:

5 Adaptive Function in Young Adults: 3 Independent in: feeding & toileting 2/3rds dressing 1/3-2/3rds bathing ½ simple cooking ¼-1/2 help with housework >50% left home alone for 2 hrs 50% Carr J. J Child Psychol Psychiat ;35 : Adaptive Behavior Pattern -general pattern of improvement into young adulthood -followed by period of stability -decline after yrs. Performance on social skills, communication, motor skills, independence relatively stable until mid-40s and then decline in raw scores at an accelerating rate Hawkins B et al. Ment Retard. 2003;41 : Cognitive Decline & Dementia General Population : IQ declines with age with performance IQ changing earlier and more rapidly than verbal DS Population: IQ decline with age begins earlier (50 yrs) with verbal ability declining relatively more and performance relatively less and aging shifted 10 yrs earlier Consider: depression, physical health problems, sensory impairments, distressing life events, premature aging, and dementia Carr J. J Intellect Disabil Res ;49 : Hawkins B et al. Ment Retard ;41 :7-28. Alzheimer s Dementia General population >65 years 13% DS population >45 years 16% Urv et al. AJIDD. 2010;115: Tyrell et al. Int J Geriatr Psychiatry. 2001;16: Increase dementia >50 yrs of age by 70th decade 50-75% Devenny et al., J Intellect Disabil Res ;44 : Coppus et al. J Intellect Disabil Res. 2006;50: Alzheimers Dement. 2010;6:

6 Alzheimer s Dementia (cont) Compared to Other Disabilities: 55 subjects assessed 5 yrs apart Personality changes (emotional lability, lack of concern for others, and apathy/indifference) were 1.5 times more likely to progress to frontal lobe dementia Frontal lobe dementia were 1.5 times more likely to progress to AD Ball et al. Int J Geriatr Psychiatry. 2006;21: UNIQUE FAMILY LIFE Older siblings: caring for DS sibling & aging parents & DS advantage families w/ children with DS: cope better Hodapp. Ment Retard Dev Disabil Res Rev ;13 : Mothers of adults with DS: less family conflict, burden, stress Seltzer et al. Am J Ment Retard. 1993;97: LIVING ARRANGEMENTS Majority unable to live independently in community > institutional settings 20 s &30 s: often reside with family Older adults: often group homes SOCIAL + sociable & outgoing - social peer interactions Social networks often limited to family, community organizations w/ disabilities, religious groups Recreation often solitary: watching TV & puzzles Visit with friends & neighbors: >weekly about 50% <yearly 30% 6

7 SOCIAL (cont) Special vs mainstream school higher Vineland interpersonal relationship Special friend Boyfriend/girlfriend Mainstream vs special school higher Greater academic progress 3 yr ahead reading and writing 78% intelligible to strangers vs. 56% in special schools SOCIAL (cont) Adolescents interested in the opposite sex: majority Dating 1/5 Married/have had partner 1.6% (186 adults) Received sexual education 1/3 Pueschel & Scola. J Ment Defic Res ;1988 :32 : Carr. J Child Psychol Psychiat. 1994;35: Mantry et al. J Intellect Disabilit Res ;52 : Bononi et al. Int J Adolesc Med Health. 2009;21: Buckley et al. Downs Syndr Res Pract.2006;9:54-67 EMPLOYMENT: Study 70 adults (37.6 yrs) Competitive employment 13% Supported employment 19% Day habilitation programs 13% Volunteer work 15% Esbensen et al. Am J Intellect Dev Disabil. 2010;115: Informal Survey Young Adults 20 s-30 s: Shelia Hebein (2010) Most jobs part time Length employment 1-17 years Types of jobs: food service, supermarket bagger/stocker, mail clerk, custodian, retail stores. 7

8 Predictive Factors: Cognitive Function Most significant predictor functional outcomes & independence cognitive impairment Health status including physical & mental health & sensory impairments Childhood function predictive of adult functioning Female gender Shepperdson. J Intellect Disabil Res ;39 : Predictive Factors : Academic Achievement Cognitive function Higher SES Level of stimulation at home & parenting characteristics Mainstream schooling Female gender Carr. J Appl Res Intellect Disabil. 2000;13:1-16. Turner S & Alborz A. Br J Edu Psychol. 2003;73: Buckley et al. Downs Syndr Res Pract. 2006;9:54-67 Predictive Factors: Adaptive Function Cognitive ability Social support Maternal coping style in childhood Placement outside parental home: : housekeeping & meal-related activities & personal care poorer with physical and mental health and sensory impairments McGuire & Chicoine. Mental Wellness in Adults with Down Syndrome A Guide to Emotional and Behavioral Strengths and Challenges Predictive Factors: Employment Similar to others with ID: Higher IQs: 17.1% with IQ >50 vs 4.9% with IQ<50 Higher adaptive scores Fewer emotional & health problems Younger & Male McDermott et al. Am J Ment Retard. 99;104:

9 Predictive Factors: Independence Independence = degree of independent living + social contact with friends + vocational independence Services significant predictor: Speech therapy Recreational services Transportation services Vocational training & job placement assistance = Twice as likely to be employed Moore et al. Ment Retard. 2004;42: Predictive of Behavior Disorders in Adults with DS Impairments in: Adaptive functioning Severity of intellectual disability Childhood psychiatric disorders Esbensen et al. Am J Ment Retard. 2008;113: McCarthy. J Intellect Disabil Res. 2008;52: SUMMARY Life events (death of family member, changes in environment, losses/separations, & medical events) more common & may precipitate depression & functional decline Patti et al. J Policy Pract Intell Disab.2005;2: Esbensen et al. Am J Ment Retard. 2008;113: In general, adults with DS function in the mildmoderate range of intellectual disability and have academic skills and cognitive functioning at a 5 ½ year level Function is stable until yrs of age with decline and development of Alzheimer s dementia beginning yrs earlier that in the general population. Predictors of functional outcomes and independence include cognition, physical & mental health, higher SES, level of stimulation at home and parenting characteristics, mainstream vs special schooling, and childhood function. 9