Challenges in the Assessment and Rehabilitation of Prolonged Disorders of Consciousness (PDOC) Dr Andrew Harrison Director / Consultant Clinical Neuropsychologist Case Management Services Ltd. SHIF - City Chambers, Edinburgh - 30.03.17

What is ‘consciousness’ and what are ‘prolonged disorders of consciousness’? Evidence that an individual is aware of and responsive to their surroundings When an individual shows signs of wakefulness but evidence of awareness is absent or significantly reduced for a period of at least 4 weeks after brain injury Thereafter considered to be in either Vegetative State (VS) or Minimally Conscious State (MCS)

Vegetative State (VS) ‘Awake but not aware’ Involuntary, reflexive behaviours may be observed (e.g. very brief visual fixation; teeth grinding; limb/trunk movement; smiling; crying; grimacing; moaning; grunting) No evidence of purposeful or goal directed behaviour UK annual incidence estimated just under 1,000 at 1mth post injury and 350 at 6mths post injury UK estimates range from 4,000 - 16,000 living in the VS

Permanent VS 12 months after traumatic injury 6 months after non traumatic injury Considered the point after which recovery of consciousness is ‘highly improbable but not impossible’ (Giacino et al., 2002) Significant legal and prognostic implications

Minimally Conscious State (MCS) Wakefulness with some evidence of awareness (e.g. following verbal commands; yes/no responses; intelligible verbalisations) Re-emergence of visual tracking is a common but not universal early marker of the transition from VS → MCS Responses may not be consistent but are reproducible UK estimates range from 12,000 – 48,000 living in MCS May be considered permanent after 5 years in the absence of improved response level (RCP; 2013)

Emergence from the MCS Functional, interactive communication and/or functional use of two or more everyday objects (Giacino et al., 2002) Responses should be consistent and reliable 100% correct Yes/No answers to minimum of six, basic orientation or autobiographical questions on two consecutive occasions Appropriate use of two different objects on two consecutive occasions 100% correct choice from pairs of pictures or objects on two consecutive occasions (RCP; 2013)

Challenges in assessing evidence of awareness Communication may be limited, inconsistent or absent Limited range of voluntary physical movements Complications with seating, positioning and posture Fluctuating periods of wakefulness and marked fatigue High probability of sensory impairment Verbal/behavioural responses may be very subtle and easily missed Responses may vary considerably over time, in different settings and with different people

Challenges in assessing evidence of awareness Some patients considered to be in VS show evidence of awareness following detailed, specialist assessment Rates of misdiagnosis as high as 40% (Andrews et al., 1996; Childs et al., 1993; Schnakers et al., 2009) Many referred to specialist PDOC centre from generic neurorehabilitation services and/or residential placement Potentially wide ranging implications (e.g. access to specialist rehabilitation; considerations regarding preserving life) Emphasises the importance of providing specialist assessment and rehabilitation services for PDOC

How is awareness in PDOC assessed? Detailed, systematic observation and documentation of behavioural responses at rest and in context of specific forms of sensory stimulation Royal College of Physicians - National Clinical Guidelines (2013) recommend two from: Coma Recovery Scale – Revised (CRS-R) (Giacino et al., 2004) Sensory Modality Assessment Rehabilitation Technique (SMART) (Gill-Thwaites & Munday, 2004) Wessex Head Injury Matrix (WHIM) (Shiel et al., 2000) SIGN 130 – Brain Injury Rehabilitation in Adults (2013) CRS-R supported with ‘minor reservations’; SMART/WHIM with ‘moderate reservations’ (Seel et al., 2010)

Practical considerations Assessment location (e.g. hospital setting; residential/care setting) Availability of suitable facilities (e.g. quiet, distraction free assessment space) Cost of assessment materials (e.g. CRS-R = free; WHIM = £53; SMART = purchase of kit + assessor accreditation) Costs and availability of trained assessors: Two assessors required SMART accreditation: 5 day course + re-accreditation every 4yrs WHIM/CRS-R: training not mandatory but strongly advised Significant financial/resource implications

Neuroimaging and neurophysiological techniques Structural and functional brain imaging techniques to measure brain activity at rest and contingent on sensory stimulation Expensive, impractical and medically contraindicated for some patients (Di Perri et al., 2014) Only to be used in specialist research programmes and in conjunction with detailed clinical assessment (RCP, 2013) Further research required to investigate their prognostic value and relationship with the findings of behavioural assessments (RCP, 2013; SIGN, 2013)

Rehabilitation of PDOC Specialist nursing care (e.g. skin integrity; continence management; oral health care; nutrition and hydration) Prevention of secondary medical complications (e.g. chest infection; aspiration; contractures; pain management; seizure management) Multidisciplinary treatment (e.g. Speech and Language Therapy; Neurophysiotherapy; Occupational Therapy; Neuropsychology; Music Therapy) Maximise engagement, participation, quality of life and emotional well-being Goals determined by findings of assessment (e.g. Targeted sensory stimulation; communication system; specialist seating; environmental control systems; engage in meaningful activity; accessing local community)

PDOC care pathway guidelines (RCP; 2013) All patients in PDOC require assessment and treatment in a specialist rehabilitation centre Patients should move to slow stream rehabilitation after 3-4 months if not making significant gains Regular re-evaluation is required (i.e. 4wks post; 6mths post; annually thereafter) Relatives should be actively involved in the treatment process Findings should inform ‘best interest’ decision making on behalf of the patient, including input from all relevant parties

Concluding comments All patients in PDOC require access to assessment and treatment in a specialist rehabilitation centre Assessment and treatment must be conducted by appropriately trained staff Treatment resources for PDOC are limited (e.g. one pair of accredited SMART assessors in Scotland!) Assessment and rehabilitation of PDOC requires continued research to target resources most effectively A coordinated national database is required to collate long term outcome data in PDOC