nurse prac**oner educaon in developmental disabilies ... 4.pdf · nurse prac**oner educaon in...
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NeurologicComplica/onsinAdultswithI/DD
SethM.Keller,[email protected]
NursePrac**onerEduca*oninDevelopmentalDisabili*es
WebinarSeries
Outline� Epilepsy� MovementDisorders� GaitDysfunction� Spasticity� Dementia
EpilepsyinI/DD
STATISTICS� 3%ofthepopulationhasI/DD� 10-20%ofallI/DDindividualshaveepilepsy� 50%ofindividualswithI/DD&CPhaveepilepsy(Morecommonintetraorhemiplegicthandystonicordiplegic)
� 21%ofI/DDwithIQ>50haveepilepsy� 50%ofI/DDwithIQ<50haveepilepsy� 40%ofindividualslivinginlargeresidentialfacilitieshaveepilepsy
Pellock JM, Hunt PA. A decade of modern epilepsy therapy in institutionalized mentally retarded patients. Epilepsy Res.
Gates, Huf, et al. Epilepsy and Behavior,2001,2, 563-567.
Difficul*eswithSeizureCare� Refractoryepilepticsyndromes� Multipleseizuretypes� Frequentstatusepilepticusandclusters� LifelongAEDuse� Polypharmacy(epilepsyandmedical)� Side-effectsarefrequentbuthardtodetect� Side-effecttolerance;statusquo� Co-occurrencewithchallengingbehaviorsincludingAutism� Challengesinobtainingdataandcommunication� Transitioningofcare� Staffknowledge/training� Acuteseizurecare
IssuesinDevelopingOp*malPlansforSeizureCare
� Allparoxysmaleventsarenotseizures� Allseizuresarenotdangerous� Notallseizuresarerefractory� Multipledrugsareusuallynotnecessary� Side-effectsareveryimportant� Seizuresmaynotbelifelong� Datacollectionandcommunication
� Seizuretracker.com� Expectationsfrompatient/families/staff/providers
Differen*alDiagnosisofSeizures� Syncope� Behavior� Toxicity� Pseudoseizures� Panicattacks� Hypoglycemia� Vertigo
EpilepsyTreatmentsTreatment
AEDs
Ketogenic Diet Epilepsy Surgery VNS Therapy
Age
Children Adults
Primarily children Children Adults 12 and older
Indication
Specific AEDs for specific seizure types
All seizure types Pharmacoresistant or localization-related epilepsy Pharmacoresistant epilepsy, partial seizures
Efficacy �64% sz freedom1
54% pts >50% sz reduction at 3 months2
�70% in select patients sz freedom3
43% of pts >50% sz reduction at 3 years4
Side Effects Vary by AED, typically CNS- and endocrine-related
Lipid disorders, ketoacidosis
Cognitive effects, surgery-related risks Voice alteration, cough, pharyngitis, dyspnea
1Brodie MJ, Kwan P. Neurology. 2002;58(suppl 5):S2-S8. 2Vining EP, et al. Arch Neurol. 1998;55:1433-1437. 3Van Ness PC. Arch Neurol. 2002;59:732-735. 4Morris GL III, Mueller WM. Neurology. 1999;53:1731-1735. 5Renfroe JB, Wheless JW. Neurology. 2002;59(suppl 4):S26-S30.
Tonic Tonic-clonic Myoclonic Atonic Infantile
Spasms Absence
Pregabalin, Phenytoin,
Carbamazepine, Phenobarbital,
Gabapentin, Tiagabine,
Oxcarbazepine, Lacosamide
ACTH Vigabatrin Topiramate Zonisimide
Ethosuximide
Valproate, Lamotrigine, Topiramate, Zonisimide Levetiracetam, Felbamate, Rufinamide, Clobazam
Generalized Partial Simple
Complex Secondarily Generalized
An*epilep*cDrugOp*ons
Treatment/Evalua*onSequenceforPharmacoresistentEpilepsy
4%
13%47%
36%
S z - f re e with 1s t A E D
S z - f re e with 2 nd A E D
S z - f re e with 3 rdA E D /P o lythe ra pyP ha rm a c o re s is ta n t
1st Monotherapy AED Trial
2nd Monotherapy AED Trial
Epilepsy Surgery/VNS Therapy/ Neuropace Evaluation
Resective Surgery Stimulator Therapy
3rd Monotherapy/Polytherapy AED Trial
Polytherapy AED Trials
Kwan P, Brodie MJ. NEJM;342:314-319.
Strongly consider videoEEG Monitoring
Epilepsy
Psychogenic, migraine, syncope, sleep disorders, movement disorder’s, etc.
Non-epileptic
Seizure control at what cost?
� Toxicity� Cognitive� Physiologic� Behavioral
� Financialconsiderations
Psychiatric adverse events during levetiracetam therapy M. Mula, MR. Trimble, et al
Neurology. 2003 Sep 9;61(5):704-6
Topiramate and Psychiatric Adverse Events in Patients with Epilepsy
M. Mula, MR. Trimble, et al Epilepsia. 2003 May;44(5):659-63.
BehavioralOutcomeswithElimina*ngSeda*ngAgents
� Improvedalertnessandinteraction
� Improvedmaladaptivebehavior
� Reducedpsychotropicmedicationusage
Poindexter AR, et al. Am J Ment Retard. 1993;98:34-40. Coulter DL. AM J Ment Retard. 1988;93:320-327 Clancy RR et al. Ann Neurol. 1991;30:493.
AcuteSeizureCare� Recognitionoftheeventandappreciatewhensignificant� Clusters,StatusEpilepticus,Seizuretypes
� Firstaid� UsageofVNSmagnet� Diastatacudial� 9-1-1� Firstrespondercare� EDandhospitalcare� Documentdetailsoftheevent
SuddenUnexpectedDeathinEpilepsySUDEP
� Maybethecauseofdeathwhen:� Ahealthypersonwithepilepsydiessuddenlywithoutdrowningortrauma
� Thepersonmayormaynothavehadaseizurebeforedeath
� Nootherreasonfordeathisfounduponexamafterdeath� Personwasnotusingillegaldrugs(example:cocaine)
� Persondidnothaveaheartattack
� SomecommontheoriescausingSUDEPinclude:� Heartarrhythmias� Breathingtrouble� Brainshutdown
� 1outof1,000patientswithepilepsydieunexpectedlyeachyear
� Inthosewithuncontrolledepilepsy,riskincreasesto1outofevery150people
� RiskofSUDEPincreaseswhen:� Seizuresarenotwellcontrolled(treatmentresistantepilepsy)
� Treatmentresistantepilepsy=failureof2roundsofappropriateandtoleratedseizuremedication� Treatmentresistantepilepsyiscommoninpatientswithautism
� Apatientsuffersfromgeneralizedtonic-clonicseizures
� Seizureshappenatnightwhenthepersonissleeping
MovementDisordersinI/DD
MovementDisordersClassifica*on
� HyperkineticvsAkinetic� Bytypeofmovement;dyskinesia,myoclonic,tremor,dystonia,chorea
� Ageofonset� Acquiredvsgenetic� Behaviorvsorganic
ExtrapyramidalEffects� TardiveDyskinesia� Akathisias� Parkinson’s� DystonicRx
0.5%-56% TD in long term usage of Neuroleptics
JournalofIDD,June2008;33(2):171-176
Management� Stopoffendingagent� Switchagent� Reducedosage� AddBenztropine,Diphenhydramine� L-DopaTherapy
GaitDysfunc/onandSpas/cityinI/DD
NormalvsPathologicChangesofGaitinAdultswithIDD
� Whatisbaselineandwhydidpastdysfunctionoccur?
� Whatnormalagingchangesareexpected?� Howtodiscernpathologicchanges� Whataretherisksandcomplicationsofalteredgaitandspasticity?
AbnormalGaitandIDD� Pain� ImpairedJointMobility(arthritis,contractures)� Muscleweakness(Spinabifida,lowtone)� Spasticity(stroke,cordlesion,CerebralPalsy)� Sensory/balancedeficit(neuropathy,stroke,vision,vestibular)� Impairedcentralprocessing(dementia,stroke,delirium,drugs)� CognitiveImpairment� Syndromespecific(Downsyndrome,FASD,FragileX)
ConsequencesofGaitDysfunc*on� Falls
� Injury,fracture,CHI,hospitalizations� Pain� Osteoporosis� Riskstoskinintegrity,cardiopulmonarysystem� DVT’s/PE’s� ADL’s� QOL/Independence� Impactuponcareteam
UpperMotorNeuronSyndromeAgroupofsymptomsthatmaybecausedbydamageorinjury
tomotorneuronpathwaysorbrainregionsthatcontrolmovement2,3
2 Katz RT, Rymer WZ. Spastic hypertonia: mechanisms and measurement. Arch Phys Med Rehabil 1989; 70:144-55 3 O'Brien CF, Seeberger LC, Smith DB. Spasticity after stroke. Epidemiology and optimal treatment. Drugs Aging 1996; 9:332-40 4 Young RR ,Wiegner AW. Spasticity.ClinOrthop Relat Res 1987; 50-62
PositiveSymptoms4 Negative Symptoms4
Characterization Muscleoveractivity Muscleunderactivity
Examples Spasticity,clonus,flexor/extensorspasm,hyper-reflexia,dystonia,andrigidity
Decreaseddexterity,weakness,paralysis,fatigability,andslownessofmovement
TreatmentGoals
1 Gormley ME, Jr., O'Brien CF, Yablon SA. A clinical overview of treatment decisions in the management of spasticity. Muscle Nerve Suppl 1997; 6:S14-20
2 Barnes MP. Spasticity: a rehabilitation challenge in the elderly. Gerontology 2001; 47:295-9
MajorClassesofTreatmentGoalswithExamplesofEach1,2
• Improveactivitiesofdailyliving(e.g.,dressing,hygiene)• Reducepain• Enhanceeaseofcare• Improvelimbposition• Improvegait
FunctionalObjectives
• Increaserangeofmotion• Reducetone• Reducespasm
TechnicalObjectives
• Preventcontracture• Preventskinmaceration• Preventskinulcers
PreventiveObjectives
Tradi*onalStep-LadderApproachtoManagementofSpas*city
NeurosurgicalproceduresOrthopedicproceduresNeurolysisOralmedicationsRehabilitationTherapyRemovenoxiousstimuli
Spas*cityTreatmentTeam
Rehabilitative Therapy -Physiatry
-Physical Therapy -Occupational Therapy
Neurologist
Patient
Primary Care Provider
Nursing
Family
Direct Care Staff
Orthopaedic Surgeon
Neurosurgeon
Anesthesiologist
Demen/ainI/DD
Func*onalDecline
� Aprocessinwhichapersonisunabletoperformatthesamelevelofactivityaspreviouslyperformed� Cognitive� Physical
� Whatisnormativeagingandwhatispathologic?� FunctionaldeclinehasanimpactupononesADL’s,QOL,andneedsforsupports
Functional Decline
Cognitive Neuromotor Psychiatric
General Medical
Dementia
Visual Impairment
Peripheral Neuropathy
Myelopathy
Radiculopathy
Depression
Psychotic Disorders
Cardiac
Endocrine
Musculoskeletal
ADR
Bipolar Dis
Stroke
Head Injury
Pulmonary
SIB Seizures
Nerve Comp
Spasticity
Anxiety
Sensory
Hearing Impairment
Vestibular
DomainsofCogni*onandDemen*a
� Memory� Shortandlongterm
� Attention� Executivefunction� Language� Visuospacial� Praxis
� Progressivedeclineincognitionandfunctionwithevolutionofsymptomsovertime
Classifica*onofDemen*asPotentiallyReversible Irreversible
� DrugToxicity� MetabolicDisturbance� NormalPressureHydrocephalus� MassLesion(Tumor,ChronicSubdural)� InfectiousProcess(Meningitis,Syphilis)� Collagen-VascularDisease(SLE,Sarcoid)� EndocrineDisorder(Thyroid,Parathyroid)
� NutritionalDisease(B12,thiamine,folate)� Mooddysfunction� Sleepdysfunction
� Alzheimer’sDisease� FrontotemporalDementia� Parkinson’sDementia� LewybodyDisease� PrimaryProgressiveAphasia� Huntington'sChorea� KufsDisease� Multi-infarctDementia� Jacob-CruzefeldtDisease� Headinjuries� HIVDementia� MultipleSclerosis
Alzheimer’sDiseaseinDownSyndrome� WomenwithDown’ssyndromearemoreatriskof
developingAlzheimer’sdiseasethanmeninthe40to65agegroup
� PeoplewithDown’ssyndromewhodevelopAlzheimer’sdiseaselive,onaverage,9-10yearsfromfirstsymptoms
� Infrequentlyrapiddeclinecanoccur� Lateon-setseizures� Fromdiagnosistodeathisonaverage8.2years
PercentageofpeoplewithDownsyndromewhodevelopdementia
atdifferentages:
Agepercentagewithclinicalsignsof
dementia
30’s 2%40’s 10-15%
50’s 33%60’s 50-70%Source:Neil,M.(2007).Alzheimer'sdementia:Whatyouneedtoknow,whatyouneedtodo.Understandingintellectualdisabilityandhealth.Accessedfromhttp://www.intellectualdisability.info/mental-health/alzheimers-dementia-what-you-need-to-know-what-you-need-to-do.
PercentpersonswithDownsyndromeshowingevidenceofneurofibrillarytangles(NFT)andsenileplaques(SP)atautopsy
Source: Mann (1993) – [based on 39 published studies n=434]
PlaqueofAmyloidBeta-Protein.
Visibleasablackglobularmasswhenstained.Theplaqueissurroundedbyabnormalneuritesanddegenerating
neurons
NaturalhistoryofAlzheimer’sDisease
1 2 3 4 5 6 7 8 9
0
5
10
15
20
25
30
Time (years)
Symptoms
Diagnosis
Loss of functional independence
Behavioural problems
Nursing home placement
Death Min
i-Men
tal S
tate
Exa
min
atio
n (M
MSE
) Early diagnosis Mild-to-moderate Severe
Feldman and Gracon. The Natural History of Alzheimer’s Disease. London: Martin Dunitz, 1996
Updatedmodelintegra/ngAlzheimer'sdiseaseimmunohistologyandbiomarkersThethresholdforbiomarkerdetec/onofpathophysiologicalchangesisdenotedbytheblackhorizontallineJacketal.(2013).TheLancetNeurology,12,207-216.
AdultswithDownSyndrome:SpecialtyClinicPerspec*ves
Chicoine, B., McGuire, D., Rubin, S. Diagnosed Disorders for 148 Adults Who Presented with a Decline in Function
Disorder Frequency Percent of Diagnosed Disorders (%) Mood 76 31 Anxiety 31 13 Obsessive-Compulsive 29 12 Behavior 23 9 Hypothyroid 22 9 Adjustment 12 5 Alzheimer's 11 4 B12 Deficiency 7 3 Menopause 7 3 Attention Deficit / Hyperactive 6 2 Gastrointestinal or Urinary 6 2 Sensory Impairment 6 2 Psychotic 4 2 Other Medical Conditions* 4 2 Cardiac Conditions 3 1
TOTAL 247 100
Dementia, Aging and Intellectual Disabilities: A Handbook ed. by Janicki and Dalton (Taylor and Francis, 1999)
Challengestodiagnosisandcare� IndividualswithI/DDmaynotbeabletoreportsignsandsymptoms
� Subtlechangesmaynotbeobserved� CommonlyuseddementiaassessmenttoolsarenotrelevantforpeoplewithI/DD
� Difficultyofmeasuringchangefrompreviousleveloffunctioning
� ConditionsassociatedwithI/DDmaybemistakenforsymptomsofdementia
� Diagnosticovershadowing� Agingparentsandsiblings� Lackofresearch,education,andtraining
Early detection/screening ‘NTG-EarlyDetectionScreenforDementia’(NTG-EDSD)� Usablebysupportstaffandcaregiverstonotepresenceofkeybehaviorsassociatedwithdementia� Picksuponhealthstatus,ADLs,behaviorandfunction,memory,self-reportedproblems� AvailableinmultiplelanguagesUse:toprovideinformationtophysicianordiagnosticianonfunctionandtobegintheconversationleadingtopossibleassessment/diagnosis
http://aadmd.org/ntg/screening
MoranJA,etal"ThenationaltaskgrouponintellectualdisabilitiesanddementiapracticesconsensusrecommendationsfortheevaluationandmanagementofdementiainadultsWithintellectualdisabilities"MayoClinProc2013;88(8):831-840.http://www.medpagetoday.com/TheGuptaGuide/Neurology/41094
� Takingthoroughhistory,withparticularattentionto"redflags"thatpotentiallyindicateprematuredementiasuchashistoryofcerebrovasculardiseaseorheadinjury,sleepdisorders,orvitaminB12deficiency
� Documentingahistoricalbaselineoffunctionfromfamilymembersofcaregivers
� Comparingcurrentfunctionallevelwithbaseline� Notingdysfunctionsthatarecommonwithageandalsowithpossibleemerging
dementia� Reviewingmedicationsandnotingthosethatcouldimpaircognition� Obtainingfamilyhistory,withparticularattentiontoahistoryofdementiain
first-degreerelative� Notingotherdestabilizinginfluencesinpatient'slifesuchasleavingfamily,
deathofalovedone,orconstantturnoverofcaregivers,whichcouldtriggermooddisorders
� Reviewingthelevelofpatientsafetygleanedfromsocialhistory,livingenvironment,andoutsidesupport
� Continually"cross-referencingtheinformationwiththecriteriaforadementiadiagnosis"
TheNTG’srecommendednine-stepapproachforassessinghealthandfunc*on.
Demen*aandGoalsofCare
� MaintainingQOL� Prolonginglife� Preventfunctionaldecline
� Slowprogression� Decreasepsychiatric/behavioralproblems
� Fallreductionprogram� Reducehospitalization
� Watchforsignsofabuse,neglect,andcaregiverburnout
� CholinesteraseInhibitionandMemantine
� Pharmacologicandbehavioralinterventions
� PalliativeCare� EndofLifeCare
BehavioralandPsychologicalSymptomsofDemen*a(BPSD)
� 90%ofpeoplewithdementiawillhaveatleastonesymptom
� Depression—40%� Delusions—63%� Hallucinations—4-41%� Aggression—31-42%� Apathy
� Associatedwithworseprognosis
� Morerapidcognitivedecline� Increasedcaregiverburden� Leadstoearlieradmissiontoinstitutionalcare
� IncreasedhealthcarecostFinkelSI,BurnsA,CohenG(2000)OverviewofBPSD,aclinicalandresearchupdate.IntPsychogeriatrics12(suppl1):13–18
CommonTriggers� Physical
� Acuteillness/infection,medications,pain,poorvision,hearing,poorsleep
� Cognitive� Inabilitytounderstand,expressoneself,lackofinsight,misinterpretationofenvironment,difficulttoproblemsolve
� Emotional� Fear,anxiety,depression,frustration,apathy,boredom
� Environmental§ Changesincaregiver,confrontationalapproach,tasksthatexceedabilities,changeinroutine,over/understimulation,lackofvisualcues
NonpharmacologicalApproaches
� Familiarenvironment—avoidfrequentmoves
� Softlighting� Calmcolors� Placestowalk� Accesstooutdoorspaces� Home-likeenvironment� Lowstimuli—minimizebackgroundnoise
� Timeoutspace
� IndividualizedCarePlanning
� Carefulanalysisofcareinteractions
� Meaningfulactivity� MusicTherapy� Exercise� Snoezelen(multisensorystimulationprogram)
� Aromatherapy� Yoga
Donot:� Argue–itwillmakethesituationworse� Tellthepersonwhattheycan’tdo–tellthemwhattheycando� Talkdowntothepersonasiftheyareayoungchild� Askalotofquestions� Talkaboutapersonwithdementiaasiftheyarenotpresent,evenifyouthinkthattheycannotunderstandyou
� Clearindication,potentialbenefitsandrisks� FDABlackBoxWarningforAntipsychoticsinusageinpatientswithdementia.Studieshaveshownanincreasedrateofmortalitysecondarytovascularcomplicationsincludingstrokesandcardiacevents1
� Identifytargetsymptoms� Expectedtimetoresponse� RisksassociatedwithandwithoutRx� Appropriatedoserange� Monitoringforsideeffectsandresponse� Whentoconsiderdosereduction,discontinuation.
FDA Public Health Advisory: Deaths with antipsychotics in elderly patients with behavioral disturbances. Accessed January 16, 2006, at www.fda.gov/cder/drug/advisory/antipsychotics.htm
Medications Specifically for Behavioral Psychological
and Symptoms in Dementia (BPSD)
1
Target Symptoms Medication
Delusions Hallucination Aggression “Agitation”
Atypical Antipsychotics: • risperidone • olanzapine • quetiapine
Sadness Irritability Anxiety Insomnia
Antidepressants • citalopram • sertraline • venlafaxine • mirtazapine • trazodone
Target symptoms Medication
Mood swings Euphoria Impulsivity
Mood stabilizers: • valproic acid • carbamazepine
Agitation Apathy Irritability
Cholinesterase Inhibitors. Memantine
Anxiety (short term use in predictable situations)
Anxiolytics: • lorazepam • oxazepam
Sink KM et al. Pharmacological treatment of neuropsychiatric symptoms of dementia: a review of the evidence. JAMA. 2005;293:596-608
Medica*onsSpecificallyforAlzheimer’sSymptoms:
BehavioralPsychologicalandSymptomsinDemen*a(BPSD)
An*psycho*cs� Classofmedsusedtotreatpsychosisandothermentaloremotionalconditions;delusions,hallucinations,agitation,paranoia
� Blockreleaseofdopamineinthebrain� Typical(conventional)oratypical� Typicalarenotselectiveandalsoblockreceptorsinotherareasofthebrainwhichmayproduceunwantedsideeffects
� Atypicalcausefeweracuteorchronicextra-pyramidalsymptoms(EPS)
� Atypicalantipsychoticsresultinimprovementinmoodandcognitioncomparedtotypicalantipsychotics
Sideeffectsofan*psycho*cs� Parkinsonism� Dystonia-abnormalfaceandbody
movements� Akathisia(restlessness)� Tardivedyskinesia(longterm)� Exacerbatedbydrugholidayregime� Morecommoninfemales� Worsenedinresponsetoreducingdrug� Irreversible(denervationsupersensitivity)
Manyundesirablesideeffects(e.g.,constipation,metabolicsyndrome,lactation,andretrogradeejaculation)
Cogni*veEnhancers� CholinesteraseInhibitors;Aricept,Exelon,Razadyne
� Heller,J.AmericanJournalofMedicalGenetics,Oct.15,2004;vol130:pp324-326� LottITetal.ArchNeurol.2002;59:1133-1136� KishnaniPSetal.(1999)Lancet353:1064
� NMDA(N-methyl-D-aspartate)receptorantagonist;Namenda� Hanney,Prasher,TheLancet,Volume379,Issue9815,Pages528-536,11February2012
� HerbalSupplements/Vitamins� GinkgoBiloba� VitESano,Metal.(1997)Acontrolledtrialofselegiline,alpha-tocopherol,orbothastreatment
forAlzheimer‘sdisease.NEJM336:1216-22
� Research� Anticholinergics� Nicotine� Homocysteine� HuperzineA� NSAIDS� BetaAmyloidandTauproteinantagonists� Vaccinationtrials
ProgressionofDisease;An*cipatoryGuidance
� CognitiveSkillswilldecline� Supportneedswillincrease� Increaserisksoffalls,injuries� Swallowingdysfunction,clots,pneumonia,bladderinfections
� Seizures� Watchforsignsofabuseandneglect� Watchforsignsofcaregiverburnout� Endoflifedecisions
Pallia*veandEndofLifeCare� TherealizationthatAlzheimer’sdiseaseprogresseswithincreasingrisksofhealthcomplicationsimpactingonesQOL/ADL’s
� Respectingoneswishesforlevelofcareandqualityoflife� Defining,anticipating,andpreparingforendoflife