Neurpsychlgical Assessment in Patients with Sickle Cell Disease Dr. Heather Rawle (Cnsultant Clinical/Health Psychlgist, GSTT - Adults) Gary Bridges (Cunselling Psychlgist, KCH - Adults) Dr. Natalie Ck (Clinical Psychlgist, KCH - Paediatrics) Thanks t Dr. Carline Jhnsn (GSTT) and Dr. Hatal Bhatt (GSTT) Why Assess? Individuals with SCD f all ages are at higher risk f cerebrvascular cmplicatins, such as acute ischaemic and haemrrhagic strke and silent cerebral infarcts (SCI) This can result in cgnitive deficits that impact upn their cmmunicatin with prviders, medical adherence, academic and ccupatinal achievement and verall quality f life. 1
Strkes in SCD Chances f having first strke by SS SC 20 years f age 11% 2% 30 years 15% 4% 45 years 24% 10% (CSSCD, Ohene-Frempng et al, 1998) 250 times mre cmmn in SCD than in ther children If untreated, risk f recurrence (ischaemic strke) = 50-92% Will ften damage bth grey and white matter Leading cause f mrbidity and mrtality in SCD. Cmmnly encuntered patterns f cgnitive impairment after strke Aphasias - impairments f language Apraxias - impairments that affect limb mvement and speech Visuperceptual and visuspatial disrders - disrders f visual recgnitin (agnsias), visuspatial abilities and visual neglect Memry impairments fr events prir t the strke (retrgrade memry) ability t lay dwn new memries (antergrade memry) the inability t retain and manipulate infrmatin fr a shrt time (wrking memry) 2
Cmmnly encuntered patterns f cgnitive impairment after strke Executive dysfunctin impairments in cnceptual reasning, cgnitive flexibility, planning, prblem slving, etc. Attentinal impairments and speed f infrmatin prcessing General intellectual functining (i.e. I.Q) Persnality / behaviur changes Silent Cerebral Infarcts (SCI) Silent cerebral infarcts ( silent strke ) Mst cmmn frm f neurlgical injury in children with SCD Prevalence increases during childhd: 10% in infants 28% by age 5 37% by age 15 Prevalence cntinues t increase thrughut adulthd Typically ccur within small vessels, generally cnfined t deep white matter, and invlve nn-mtr areas f the brain (esp. frntal crtex) Increased risk fr further vert and silent strkes. 3
Impact f SCI in SCD Cgnitive difficulties Glbal cgnitive dysfunctin, particularly nn-verbal IQ Prcessing speed Wrking memry Executive functin (planning, prblem slving, rganisatin, inhibitin, respnse mnitring, mental flexibility) Attentin, divided attentin / switching (Berkelhammer et al, 2007; Mackin et al, 2014; Rawle et al, 2010; Vichinsky et al, 2010) In children, difficulties becme mre apparent in later stages f primary educatin, when intellectual demands increase Pr schl/wrk perfrmance Deficits in measures f executive functining and attentin/cncentratin Difficulties with paying attentin, shrt-term memry, rganising and planning schl wrk, initiating tasks and staying fcused n them, regulating emtins, self-mnitring. Impact f SCI in SCD Cgnitive difficulties Cgnitive impairments tend t be mre severe when patients have abnrmal MRIs, but significant cgnitive impairment in sme patients with nrmal MRIs MRIs nt sphisticated enugh t detect sme brain changes; pr perfusin; effects f pain Level f anaemia is mre predictive (Vichinsky et al., 2010). 4
Standards f Care Sickle Cell Disease in Childhd: Standards and Guidelines (2006); Standards fr Management f Sickle Cell Disease in Childhd (2008) Regular neurpsychlgical screenings and mnitring f schl attainment shuld be carried ut n a regular basis Patients shuld have access t a neurpsychlgist within the MDT. Standards fr the Clinical Care f Adults with Sickle Cell Disease in the UK (draft 2017); Peer Review Standards fr Sickle Cell Disease (updated draft 2017) Patients shuld have access t neurpsychlgy via a defined pathway What is a neurpsychlgical/ cgnitive assessment Interview Medical, Educatinal, Emplyment, Family, Develpmental, Language, Migratin histry reasns and stressrs Cping, Views f prblems (memry diary) Md, Pain Infrmatin frm ther surces Health/Scial Educatinal recrds/feedback frm schl HCPs and family members Research literature 5
What is a neurpsychlgical/ cgnitive assessment Assessment f cgnitive dmains Memry, Attentin, Prcessing Speed, Language, Executive Functin, Visual-Spatial/Perceptin, Intellectual Functining, Wrd Reading, Reading Cmprehensin, Mathematics, Listening Cmprehensin, Spelling Interpretatin and recmmendatin Feedback and liaisn Patient/Family/Carers HCPs Emplyers/Schl/Cllege/SENC Can prvide supprt fr Educatin Health Care Plan (EHCP) Onward referrals Current Service Mdel in Paediatric Sickle Cell & Thalassaemia Service (KCL) Referral t Clinical Psychlgy Psychlgy Assessment Semi-structured interview (90 mins) Liaisn with educatinal services Psychmetric assessment Neurpsychlgical Assessment Cgnitive Ability (2-3 hurs) Schlastic Achievement (1-2 hurs) Further assessment if required Fllw-up F/U appintment ffered t all families t discuss assessment findings Recmmendatins f apprpriate educatinal, psychlgical r medical interventins Liaisn with schl/educatinal services (EHCP) Sign-psting/referral t ther services where apprpriate (e.g. SALT, OT) Discharge 6
Tests used with Adults IQ: WAIS-IV UK; WAIS-III UK; shrtened versins Premrbid IQ TOPF; WTAR Memry WMS-IV UK; WMS-III UK; RBMT Executive Functining: Hayling and Brixtn; Verbal & Categry Fluency; BADs key search, z map; Trail Making Test (TMT A&B): DKEFS Trails Visuspatial: VOSP Attentin: WAIS subtests; Test f Everyday Attentin Tests f Effrt: WAIS subtests Tests used with Children & Adlescents Cgnitive ability/iq: WPPSI (age range: 2:6 7:7) WISC-V; WISC-IV (age range: 6:0 16:11) WAIS-IV UK (age range: 17:0+) Schlastic Achievement WIAT-II (age range: 4:0 16:11) WIAT-III (age range: 4:0 25:11) Further assessment NEPSY-II (age range: 3:0 16:11) Attentin and executive functining; Language; Memry and Learning; Sensrimtr; Scial Perceptin; Visuspatial Prcessing Children s Memry Scale (age range: 5:0 16:11) D-KEFS (age range: 8:0 89:00) Psychmetric assessment: Cnnrs 3 rd Editin (Selfreprt/Parent/Teacher versins) Behaviur Rating f Executive Functin (BRIEF) (Parent/Teacher versins) Strengths and difficulties questinnaire (SDQ) (Selfreprt/Parent/Teacher versins) Revised Children's Anxiety and Depressin Scale (RCADS) (Selfreprt/Parent) 7
Cmplexities SCD and strke Duble time fr interview: Language, culture, educatin Hw SCD affects persn pain, fatigue, expectatins Strkes: Hemiparesis/plegia, sensry, arusal, dysphasia, dysarthria, apraxia, ataxia, fatigue, sleep, epilepsy, pain, cgnitive impairments SCD: Cultural, educatinal backgrund, language Multiple strkes/silent strkes ver time Pain, medicatin, depressin, anxiety Lack f inf frm thers as ften islated Premrbid IQ? (lack f inf) Impact f SCD n schl ach; expectatins f self Csts and time Neurpsychlgical testing is a scarce resurce Nt widely available and time cnsuming Therefre has nt regularly been integrated int rutine clinical care fr patients with sickle cell disease The Vichinsky et al (2010) study invlved a 6-hur neurpsychlgical battery, administered by a trained neurpsychlgist Future? Cmputerised testing NIH Tlbx - Cgnitin Battery (NIHTB-CB) (www.healthmeasures.net) Need t ensure this cntributes t a meaningful assessment when using it in clinical setting Q-Interactive testing (http://www.hellq.c.uk/hme.html) using ipads Create unique, client-centric batteries at bth the instrument and subtest levels Imprves administratin accuracy and speed, prvides real time scring, and allws fr flexibility in just a few simple taps. 8
T screen r nt t screen If strke histry and reprting cncerns: Indicates cmprehensive assessment (s screen nt required) SCD patients tend t be yunger strke screening measures culd still lack sensitivity (risk false negatives) If silent strkes/n strke histry: Lack f sensitivity (risk false negatives) Nt in cntext f cgnitive assessment meaningless Dn t have enugh infrmatin t frmulate why patient is presenting as they are cannt make meaningful recmmendatins Ethical dilemma What happens if they have a pr scre, but n service fr a cmprehensive assessment? Self fulfilling prphecy Lw scre anxiety perfrm wrse Factrs affecting screening scres e.g. why may the patient present with a lw prcessing speed scre? Fatigue Lw md Anxiety Trauma Pain Analgesia Other medicatins Effects n brain f strke/silent strke Part f glbal picture f lwer scres e.g. Learning disability Malingering 9
Cnclusins Clinicians shuld be aware f the risk f cgnitive impairment in patients with SCD, even amng thse with nrmal MRI scans this may impact n patient s understanding, decisin-making, and adherence t treatments Neurpsychlgical assessments fr patients with SCD are useful t highlight cgnitive impairments that may therwise be unnticed by clinicians, and be a useful way f identifying thse wh require supprt (e.g. at schl, university, wrk) Simple screening tls are nt apprpriate fr clinical use in this ppulatin. Questins? 10