treatment of dementia in people with intellectual and developmental disabilities

1. Cliff Singer, MD Chief, Geriatric Mental Health and Neuropsychiatry Acadia Hospital and Eastern Maine Medical Center Bangor, Maine treatment of dementiain people with intellectual and developmental disabilities

2. Once Dementia is Diagnosed……Know What You’re Treating: • Primary Dementia: gradual, progressive • Alzheimer’s disease • Multi-infarct vascular dementia • Dementia with Lewy Bodies • Parkinson’s Disease Dementia • Frontotemporal Dementia • Secondary dementia: acute or subacute: • Traumatic Brain Injury • CNS Infections • Alcohol-related (Korsakoff’s) • “Reversible” Causes • Medical and psychiatric causes

3. Rule Out Depression and Delirium* Memory Attention Depression Mood Delirium Dementia Motor Executive *Fact is, this may not be so easy; they often co-exist.

4. Clinical Features At Diagnosis

5. Tasks in Early Dementia • Clarify diagnosis • Discuss prognosis • Discuss safety issues • Encourage quality of life activities • Basic geriatric care to minimize incontinence, maximize mobility and stability, address hearing and vision impairments • Planning for smooth transitions of care

6. More Tasks in Early Dementia • Caregiver wellbeing addressed • Consider cognitive enhancing meds • Adapt daily activities to changing abilities • Address pain to enhance comfort and mobility • Minimize iatrogenic problems • Advanced directives established

7. The Edinburgh PrinciplesWilkinson H, Janicki M et al. J IntellDisabil Res 2002; 46:3:279-84 • 1. Focus on QOL as that person would define it • 2. Focus on a person’s capabilities • 3. Involve the family • 4. Ensure good diagnostic assessment and treatment • 5. Work to keep people with ID and dementia in their chosen home • 6. Ensure people with ID and dementia have access to the broad range of dementia care in general community • 7. Advocate for research and public policy to meet current and future needs

8. Goals in Severe Dementia • Maintain function and maximize comfort • Explore options for change of residence based on caregiver capabilities and needs • Consider small details that may enhance quality of life • Minimize transitions between home and ED and hospital

9. Tasks for Severe Dementia • Assess cognition, behavior, function, nutrition/hydration, pain/discomfort, caregiver wellbeing at frequent intervals • Make adjustments for decline in mobility • Make adjustments for change in diet and feeding strategies • Review advanced directives • Discuss transition to palliative care or hospice

10. Cognitive enhancing medicatinos: Cholinesterase Inhibitors • Many neurodegenerative diseases associated with reduced cholinergic function (↓Ach) • Inhibit acetylcholinesterase and ↑ Ach • Acetylcholine: promotes alertness, concentration, memory, visual perception

11. Cholinesterase Inhibitors 2 • Donepezil (Aricept: AD, Mild-Mod-Sev AD) • 5 mg daily for one month, then 10 mg daily. May go to 23 mg daily. • Galantamine (Razadyne ER: Mild-Mod AD) • 8 mg daily for one month, then 16 mg. May go to 24 mg daily. • Rivastigmine (Exelon Patch: Mild-Mod AD, PDD) • 4.6 mg/24 hrs. daily for one month, then 9.5 mg/24 hrs. May go to 13.3 mg/24 hrs.

12. Cholinesterase Inhibitors 3 • Alzheimer’s Disease • Start and maintain for at least 1 yr. • Expect improvement in some, slowed decline in most, mild psychotropic effect • Multiple small trials in DS w AD: generally positive results but evidence of efficacy not yet convincing (methodology?) • PDD/LBD • Expect better response and moderate psychotropic effect (VH, delusions) • No controlled data in DS or ID

13. Other Clinical Issues • When to stop? • Are they worth the money? • Relative contraindications: PUD, bradycardia, syncope, weight loss • Off label • Vascular dementia: possible benefit? • FTD, EtOH, TBI: No benefit • No controlled data in ID

14. Memantine (Namenda) • Effective in monotherapy but better as adjunctive therapy with ChEI • Improves neuronal function • Well tolerated • Approved for moderate to severe AD • Started at 5 mg daily, with weekly increases of 5 mg a day to 10 mg BID • Not proven to be effective in DS w AD

15. Case Example 1 • Psychiatric consult requested for 62 year old man with Down’s Syndrome • Admitted to med-surg unit • Yelling for 6 weeks. • Consult request: “Help with yelling. Dementia getting worse.” • Patient kept in room down hallway with two doors to reduce disruptions from loud patients.

16. Case Example 2 • 82 year old woman in SNF with large right frontal CVA three years ago • Consult requested because of months of intense crying episodes unresponsive to multiple trials of antidepressants • Cried with ADL care but also loud sobs and long periods of wakefulness at night • Exam: L hemiparesis w/contractures, marked abulia and apathy, but could make eye contact and show gentle smile

17. Behavioral Symptoms ADLloyd et al. J. Geriatric Psychiatry Neuro 8:4:213-216, 1995

18. Agitation • Can be due to anything causing distress • R/O urinary retention, impaction, pain • Consider the environment/interpesonal • May be due to primary or secondary psychiatric disorders • But, dysphoric irritability is also a primary symptom of neurodegeneration

19. Other Sources of Agitation

20. Symptom Review • MOMS • mobility, output, memory, senses • AND • aches, neuro, delirium/delusions • DADS • depression, appetite, dermis, sleep

21. Analegesics:Percentage of NH Residents With Dementia Receiving Analgesics

22. Sources of Pain • Obvious: arthritis, spondylosis, GERD, known injury or infection, headache, neuropathy, pressure ulcers, skin tears, joint deformities, compression fractures, shingles, fibromyalgia • Subtle: contractures, pressure points, immobility, dental and periodontal, constipation, urinary retention, unknown injury, tight clothes, ear infection

23. AGS Guidelines in Mild Dementia • Generally able to reliably report pain but less reliable in people with low IQ • Pose questions in present tense • Use various terms for pain, discomfort, hurt, uncomfortable, etc. • Use frequent direct questioning • Multidimensional pain instrument may be helpful but not necessary

24. APS and AGS in Severe Dementia • Recommend using a validated pain scales for cognitive impaired or nonverbal patients • Scales are based on observation of behavior and expression • Scales have limitations (false + and -) • Verbal scales may be best in this group6 • In DS w AD: Note recent changes in vocalizations, facial expression, body posture and movement patterns, agitation with ADL care • Physiologic clues of distress may be only clue: increased breathingor heart rate, increased BP

25. Behavioral Clues • Facial expressions and affect • Verbalizations/vocalizations • Irritability and agitation • Postural guarding • Restlessness • Withdrawn • Anorexic • Insomnia

26. Suggested Scales Numbers are percentages.

27. Discriminant validityZwakhalen, S., Hamers, J. & Berger, M. (2007). Journal of Advanced Nursing, 58(5), 493-502.

29. PADEVillanueva M et al. JAMDA 2003; 4:1:9-15 • 24 items • Facial expression • ADLs • Caregiver’s judgment of pain • Good reliability and validity • 5-10 minutes to administer

30. Agitated Behavior and PainHusebo BS et al. Am J Ger Psych 2013; in Press • Controlled trial of pain intervention • 352 patients in Norwegian NHs • Dementia and moderate to severe pain • All patients in intervention group received scheduled analgesics in stepwise approach • CMAI factor analysis: • Verbal agitation showed greatest reduction • Aggressive behaviors declined

31. CMAI Scores: Cohen-Mansfield Agitation Scale scores for verbal agitation

32. Most Common Behaviors in Cohen-Mansfield Study: • General restlessness • Constant requests for attention • Pacing • Complaining • Repetitiveness • Cursing • Oppositional behavior

33. Scheduled Analgesic Trial for Agitation • Topical agents, lidocaine skin patch • Avoid NSAIDs, muscle relaxants • Acetaminophen 325-1000 mg TID or QID (max. 3000 mg/day) • Gabapentin 100-800 mg BID or TID • Tramadol 25-50 mg BID or TID • Opioid analgesics • Hydrocodone 2.5-5 mg Q4-6 hrs. • Oxycodone 2.5-5 mg Q 6 hrs. • Hydromorphone 1-2 mg Q 4-6 hrs. • Methadone 2.5 mg Q 8-12 hrs.

34. Opioid Concerns • Real • Opioid naïve patients may have strong reactions: over-sedation or delirium • Constipation • Tolerance • Diversion • Mythological • Addiction • Dementia: Opioids may increase confusion initially, but cognitive tolerance develops quickly and correlates with sedation

35. Case Example 1 • Quick exam revealed tender, distended abdomen • Abdominal X-ray confirmed obstipation • A wonderful nurse volunteered, did the work and the yelling stopped

36. Case Example 2 • Crying not due to depression but stroke-related affect dysregulation • But crying likely due to distress: • Pain from contractures and immobility • Anxiety during the night • Interventions: • Scheduled analgesia • Nighttime medication for anxiety and sleep • Environmental and comfort measures

37. Aggression • If due to agitation or delusions, treat with appropriate medications • If episodic, requires root cause analysis to identify triggers • Stimulus and response need to be modified • Medications do not work well for episodic aggression

38. Pacing and Wandering • Consider trials of meds for anxiety, akathisia, pain, RLS • May be “agenda-driven”, such as looking for something • May be frontal hyperactivity (ADHD) • May be tardive akathisia • Need exercise and safe and secure surroundings • May be a terminal sign

39. Sexual Disinhibition • Consider mania (especially in women) • Consider legitimate need for intimacy • If stimulus bound (eg breast grabbing), isolate from stimulus • If driven by libido, medroxyprgesterone can be tried (case reports and small case series)

40. Apathy • Majority of patients with dementia • Not depression • Impoverished thinking • Patients are quiet, placid, withdrawn • No initiative, reluctant to shower • “Ghosts”….spouses feel lonely • Occasionally responds to stimulants, ChEI and memantine

41. Hallucinations • Visual: optimize vision, keep nightlights on, cholinesterase inhibitors and low dose quetiapine if Lewy Body or PDD • Auditory: optimize hearing, mask with “white noise”

42. Sundowning • Circadian delirium with no effective treatment • Suggestions: • Midday nap? • Music? • Structured activities? • Enhance exposure to bright light in AM? • Cholinesterase inhibitor? • Decrease stimulation? • Trials of low dose antipsychotic midday?

43. Sleep Disorders • Nighttime insomnia: • Modest effect: daytime activity, enhanced light exposure, melatonin augmentation • More effective: trazodone, quetiapine, analgesia • Daytime sleepiness: R/O obstructive apnea, reduce sedating meds, increase daytime activity • REM Behavior Disorder: clonazepam, melatonin, cholinesterase inhibitor

44. Psychotropics for Agitation • Antipsychotics • Use when delusions present • Most evidence for efficacy in general dementia population • No data in ID dementia (fair quality data in younger ID people with aggressive behaviors) • Antidepressants (esp. SSRIs) • Use for irritability, anxiety, dysphoria • Antiepileptics (caution) • Benzodiazepines (caution) All psychotropics nearly double mortality risk. Sedation and lethargy = falls, aspiration and death

45. Targeting Psychotropics Trazodone, Sed/Hyp: insomnia Mood Stabilizer Antidepressant irritability anxiety dysphoria impulsivity hyperactivity Clonazepam: REM sleep behavior agitation Analgesic: restless calling out grimacing combative physical aggression ChI: apathy hallucinations misperceptions confusion inattention delusions hallucinations Antipsychotic Stimulants: apathy sleepiness

46. Comfort Measures • Music • Snacks and drinks • Scheduled toileting • Low stimulation • 1:1 activities: reading, singing, hand massage, games, etc.

47. Resources • National Task Group on Intellectual Disabilities and Dementia Practices • www.aadmd.org/ntg • Alzheimer’s Disease Education and Referral Center • www.nia.nih.gov/Alzheimer’s • Family Caregiver Alliance • www.caregiver.org • Alzheimer’s Foundation of America • http://www.alzfdn.org • NamesteEnd of Life Dementia Care • http://namastecare.com

48. References • Herrman N and Gauthier S. Diagnosis and Management of Dementia: Management of Severe Dementia. CMAJ 2008; 179:2:1279-87 • Hogan DB et al. Diagnosis and Management of Dementia: Nonpharmacologic and pharmacologic therapy for mild to moderate dementia. CMAJ 2008; 179:10:1019-26 • Hogan DB et al. Diagnosis and treatment of dementia: Approach to management of mild to moderate dementia. CMAJ 2008; 179:8: 787-93

49. References • Sadowsky CH and Galvin JE. Guidelines for the management of cognitive and behavioral problems in Dementia. JABFM 2012; 25:3:350-366 • Stanton LR and Coetzee RH. Down’s Syndrome and Dementia. Adv Psychiatric Treatment 2004; 10:50-8 • Steinberg M and Lyketsos. Atypical antipsychotic use in patients with dementia: Managing safety concerns. Am J Psychiatry 2012; 169:9:900-906