pfs presentation...brain tumors ~40% to 50% of these are medulloblastoma posterior fossa tumors 3...

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2/14/2018 1 Posterior Fossa Syndrome: Rehabilitation Across the Continuum Lindsey Christoffersen, PT, DPT, C/NDT Sherry Lockett, PT, DPT The speakers have no conflicts of interest to disclose Any equipment or materials mentioned in the presentation are available to the speakers at their facility The speakers have no financial gain in mentioning any specific products Conflict of Interest Disclosure At the conclusion of this session, participants will be able to: 1. Discuss the pathophysiology and clinical characteristics of Posterior Fossa Syndrome (PFS). 2. Discuss the role of each member of the interdisciplinary team in the comprehensive management of patients with PFS. 3. Identify and discuss appropriate evaluation tools and treatment strategies to address the impairments and functional limitations associated with PFS across a variety of settings. Learning Objectives Near the base of the skull Contains the brainstem and cerebellum - Brainstem: breathing, heart rate, swallowing, alertness, digestion - Cerebellum: movement, posture, balance, coordination The Posterior Fossa 1,2 Image courtesy of University of Rochester Medical Center Account for 60-70% of all pediatric brain tumors ~40% to 50% of these are medulloblastoma Posterior Fossa Tumors 3 Reported incidence following PF tumor surgery ranges from 2%-50% Older studies often report lower numbers - Failure to recognize the syndrome? Gadgil et al. (2016) knowledge update - Occurs in 8-24% of children following resection of PF masses Robertson et al. (2006) - Reported PFS in 24% of their study population (N=450) Incidence of PFS 4-6

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  • 2/14/2018

    1

    Posterior Fossa Syndrome: Rehabilitation Across the Continuum

    Lindsey Christoffersen, PT, DPT, C/NDTSherry Lockett, PT, DPT

    • The speakers have no conflicts of interest to disclose

    • Any equipment or materials mentioned in the presentation are available to the speakers at their facility

    • The speakers have no financial gain in mentioning any specific products

    Conflict of Interest Disclosure

    At the conclusion of this session, participants will be able to:

    1. Discuss the pathophysiology and clinical characteristics of Posterior

    Fossa Syndrome (PFS).

    2. Discuss the role of each member of the interdisciplinary team in the

    comprehensive management of patients with PFS.

    3. Identify and discuss appropriate evaluation tools and treatment

    strategies to address the impairments and functional limitations

    associated with PFS across a variety of settings.

    Learning Objectives

    Near the base of the skull

    Contains the brainstem and

    cerebellum

    - Brainstem: breathing, heart rate, swallowing, alertness, digestion

    - Cerebellum: movement, posture, balance, coordination

    The Posterior Fossa1,2

    Image courtesy of University of Rochester Medical Center

    Account for 60-70% of all pediatric brain tumors

    ~40% to 50% of these are

    medulloblastoma

    Posterior Fossa Tumors3

    Reported incidence following PF tumor surgery ranges from 2%-50%

    Older studies often report lower numbers

    - Failure to recognize the syndrome?

    Gadgil et al. (2016) knowledge update

    - Occurs in 8-24% of children following resection of PF masses

    Robertson et al. (2006)

    - Reported PFS in 24% of their study population (N=450)

    Incidence of PFS4-6

  • 2/14/2018

    2

    Most common malignant brain tumor of childhood

    - Accounts for 20% of all childhood brain tumors

    - 250-500 children are diagnosed in the U.S. annually

    - Primarily found in children < 16 years old

    Medulloblastoma7

    Image courtesy of RM DeSouza and colleagues

    Risk Stratification8

    Standard RiskStandard Risk

    >3 years of age>3 years of age

    No detectable metastatic disease

    No detectable metastatic disease

    Near or total resectionNear or total resection

    High RiskHigh Risk

  • 2/14/2018

    3

    Motor

    • Ataxia• Dysmetria

    • Dysphagia• Dysarthria• Hypotonia• Hemiparesis• Nystagmus• Oculomotor problems• Urinary

    retention/incontinence

    Speech/Language

    • Cerebellar mutism• Naming problems

    • Verbal fluency• Dysarthria• Slowed speech• Decreased verbal

    output• Short phrases• Distorted vowels• Prolonged phonemes• Mono- pitch/loudness• Hypernasality• Vocal tremor

    Affect/Behavior

    • Depression• Irritability

    • Lability• Apathy• Anxiety• Forced crying/laughter• Transient eye closure• Impulsivity

    Cognitive

    • Attention• Memory

    • Executive functioning• Visuospatial skills• Processing speed• Verbal comprehension• Reading/writing

    difficulties

    Postoperative Deficits9,10

    Decreased speech or mutism

    Dysphagia

    Hypotonia

    Ataxia

    Mood changes

    Neurological deficits

    Most Common Deficits3,9,11

    Transient mutism

    - Delayed onset (0-15 days, mean 2 days)

    - Limited duration (1 day-2.5 years, mean 43 days)

    - Recovery period marked by dysarthria

    - Not uncommon for patient to speak a few words after surgery but unable to speak the next day

    Not all patients become completely mute

    - May be limited to single words or short sentences (not easily elicited)

    Hallmark Characteristic: Mutism9

    Duration of mutism varies

    - Gadgil et al. 2016 knowledge update

    • Average: 8 weeks

    • Range: 4 days to 5 months

    Correlation found between duration of mutism and severity of neurologic symptoms

    Speech Outcomes12,13

    Slow rate: 33 patients

    Short phrases: 13

    Hypophonia: 10

    When Speech Begins to Return13

    Latent onset of 1-6 days

    - Children have normal speech prior to onset

    Total absence of speech

    - Not of nonverbal utterances such as crying, screaming

    Worsening neurological symptoms

    - Ataxia, pyramidal paresis, oculomotor dysfunction, diminished facial expression

    - Loss of bladder and bowel control

    Behavioral disturbances

    - Apathy, depressed affect, agitation, irritability, emotional lability

    Clinical Characteristics: Acute Stage13

  • 2/14/2018

    4

    Kirk et al. (1995): Retrospective study of children after PF surgery

    Timeline of Onset3

    Postoperative Symptoms

    Post op in ICU

    Able to move purposefully, focus on an object, make some attempts to verbalize when intubated; similar to preoperative state with expected ataxia

    and dysmetria

    Within 24−107 hours

    Neurologic status changes without an identifiable factor (e.g., no change in vitals, intracranial pressure monitoring, or radiographic abnormality)

    Timeline of Onset3

    Postoperative Symptoms

    Within 24−27 hours

    Facial weakness and nystagmus

    Mean time 33 hours

    Weakness of upper extremities

    Mean time 40−58 hours

    Weakness of lower extremities, emotional lability, irritability, random nonpurposeful movements of extremities, difficulty verbalizing, and mutism

    Differs for each patient

    More severe symptoms early in recovery ���� symptoms that

    persist longer

    Onset, Severity, & Duration5,12

    Absence or reduction in

    speech 1-2 days post-op

    Mutism 2-7 days post-op;

    Profound axial hypotonia and

    ataxia

    Recovery of speech begins after 8 weeks (on average)

    Duration of Deficits6

    Kupeli et al (2011)

    - Pre and post-op neurologic and psychological evaluations of children with brain

    tumors having PF surgery (PFS developed in 25% of patients)

    Multiple theories, but no clear culprit

    - Damage to dentato-thalamo-cortical (DTC)

    pathway

    - Damage to the vermis

    - Bilateral dentate nuclei damage

    This damage may result from

    - Poor cerebellar perfusion due to vasospasm

    - Injury related to surgery

    - Postoperative edema

    Causes5,11,14

    Avula et al. 2016

    Prospective review of postoperative MRI

    - 10 patients who developed PFS

    - 13 randomly selected patients who did not develop PFS

    Preoperative Imaging

    - Rostral position of tumor within 4th ventricle associated with PFS (P=0.035)

    Causes: Morris et al15

  • 2/14/2018

    5

    Postoperative Imaging

    - PFS group had bilateral multi-focal postop

    injury (edema)

    • 90% of PFS group had damage to ≥ 3 of the following: Pons, dentate nuclei, superior cerebellar peduncles, midbrain

    – None of the unaffected patients had ≥

    3 structures involved

    - 80% of PFS group had bilateral involvement

    • Compared to 15% of patients without PFS

    Causes: Morris et al15 Risk Factors5,16

    Medulloblastoma diagnosis

    Midline tumors

    Tumors involving the vermis

    Tumors high in the 4th ventricle

    Brainstem invasion

    Retrospective study of 63 children with medulloblastoma; PFS developed in 18 patients (29%)

    Predictors found included:

    - Brainstem invasion

    - Midline tumor location

    - Younger age

    - Absence of radiographic residual tumor

    Conclusions:

    - Children with midline tumors exhibiting brain stem invasion are at increased risk for PFS

    - More aggressive surgeries may result in increased incidence of PFS

    Risk Factors16

    Many patients suffer from long-term sequelae

    - Mutism tends to be transient, but speech rarely returns to being completely

    normal

    - Increased risk for neurocognitive impairment

    - Neurological deficits, mainly ataxia

    - Decreased quality of life and environmental access

    Looking Long-Term13,17,19

    Palmer et al. 2010

    - 25 patients with PFS following medulloblastoma resection

    - 25 matched patients without PFS being treated on same protocol

    - Significant differences (p

  • 2/14/2018

    6

    Robertson et al. 2006

    - 1-year post-diagnosis

    Long-Term Speech & Motor Outcomes5

    • 92% had ataxia• 66% had speech and language dysfunction• 59% had some degree of global intellectual handicap

    Initially Rated Severe

    • 88% had abnormal coordination• 25% had speech and language dysfunction• 17% had some degree of global intellectual handicap

    Initially Rated Moderate

    Steinbock et al (2003)

    Long-Term Speech & Motor Outcomes18

    Brinkman et al. 2013

    - In-home evaluations of brain tumor survivors ≥ 18 years old (N=78) and

    matched population-based controls (N=78)

    • Environmental access

    • Health related quality of life

    • Social participation

    • Demographic and treatment information

    • Physical performance

    • Cognitive function

    • Psychological distress

    - Median age of survivors was 22 years (range 18-58)

    Long-Term Quality of Life19

    Brinkman et al. 2013

    BT survivors:

    Long-Term Quality of Life19

    Were more likely to avoid

    aspects of physical

    environment

    Were more likely to report

    they did not drive

    Were more likely to report

    decreased HRQOL

    Participated less in

    functional living and

    social activities in their

    community

    Treatment of PFS12,13,20

    Pharmacology

    Physical Therapy

    Speech Therapy

    Occupational Therapy

    Neuropsych

    & Psychology

    Child Life

    Physiatry

    Rehabilitation Management

  • 2/14/2018

    7

    Evidence is significantly lacking regarding rehabilitation for patients with PFS

    Primarily limited to case studies and case series

    This may come as a shock…

    Initial rapid gain of skills

    - During RT and during break before chemotherapy

    - Critical period for rehab

    - Inpatient rehab highly recommended during this time if appropriate

    Plateau during chemotherapy

    - Doing our best to “hang on” to skills gained

    - Typically an appropriate time to order a custom WC if indicated

    More progress after completion of chemotherapy, but much

    slower than initially seen

    - Remaining deficits are often long-lasting

    Rehab Trajectory: Our Experience

    � If severe PFS, patient may remain inpatient after surgery or be admitted directly inpatient if coming from an outside hospital

    � May remain inpatient throughout scans and during the beginning of

    radiation therapy (RT), with a break between RT and chemotherapy

    � Depending on physical function and burden on caregivers, patient may remain inpatient for duration of chemotherapy

    � Interdisciplinary collaboration is key

    Acute Care Overview

    History

    - Previous level of function, activity level, previous limitations

    - Home location, accessibility issues, plans to return home

    - Caregivers available for training, assisting patient, equipment management

    Communication & Behavior

    - Alertness and orientation

    - Ability to follow commands

    - Ability to communicate needs (yes/no, thumbs up/down, squeezing hand)

    - Mutism/ability to voice

    - Flat, emotional lability

    Initial Evaluation

    Vision/Facial/Voice Screen

    - Drooling, smiling (facial droop)

    - Wet voice

    - Ability to track objects, attend to objects

    - Strabismus and/or nystagmus

    ROM (AAROM, PROM) & Strength

    Neurological screen

    - Muscle tone

    - Clonus

    - Reflexes

    - Coordination and motor control (finger to nose/finger, ataxia)

    Initial Evaluation

    Functional Mobility

    - Bed mobility

    - Sitting balance

    - Sit stand

    - Standing

    - Transfers

    - Ambulation and stair negotiation

    - Gross motor abilities

    - Equipment needs

    Initial Evaluation

  • 2/14/2018

    8

    14-item instrument

    Examines functional balance

    - Steady state

    - Anticipatory

    Administration time < 20 minutes

    Validated for children with mild, moderate, and severe motor

    impairments

    - Athetosis, hemiplegia, hypotonia, spastic diplegia, s/p brain tumor resection

    Pediatric Balance Scale21,22 Pediatric Balance Scale21,22

    Pediatric Balance Scale21,22

    Cut off scores

    - Based on typical performance

    - Scoring below cut score may indicate decreased functional balance

    - Should be interpreted with findings from clinical examination

    Pediatric Balance Scale22

    Minimal Clinically Important Difference (MCID)

    - 5.83 points for PBS total

    - 2.92 points for static items

    - 2.92 points for dynamic items

    Pediatric Balance Scale22

    Initial Presentation

    Ataxia

    Axial hypotonia

    Max A –dependent transfers

    Non-ambulatory

    or max A for

    ambulation

    Mod –Max A for

    static sitting and standing

  • 2/14/2018

    9

    � Stay focused on discharge plan

    � Teaching parents/caregivers safety with mobility and transfers

    � Caregiver education on task carryover in home exercise program

    � Maximizing patient ability to perform assisted/independent mobility/transfers and desired activities

    � Standing and ambulation if possible

    � Order appropriate equipment/orthotics

    Goals for Acute Care

    � Meet with team to communicate progress, updated goals, and discharge planning

    � Interdisciplinary collaboration to incorporate behavior/speech goals into sessions � treating the whole person

    Goals for Acute Care (continued)

    Treatment Goals:1. Patient will be able to perform a stand pivot transfer from bed to chair with moderate

    assistance with an assistive device with verbal cueing for sequencing and increased time

    allowed for activity to assist caregivers with mobility tasks.

    2. Patient will be able to tolerate sitting at edge of bed with minimal assistance with bilateral

    LE support and use of bilateral UE on surface x 4 minutes with independent head control

    to assist with a dressing activity.

    Acute Care Interventions

    3-5 days/week

    30-60 minute sessions

    Individual or co-treats

    Bed mobility

    Transfers

    Standing & ambulation

    Caregiver Training

    Bed Mobility

    - Rolling to both sides (sequencing LEs, UEs, and head)

    - Supine sitting (propping on elbow, maintaining alignment)

    - Repositioning of self for comfort, pressure relief (bridging, scooting in bed)

    Sitting Activities

    - May require assistance of two people

    - Be aware of input being given and ability of patient to handle increased stimuli

    - Position of support (anterior vs posterior)/

    - Support surface (mat vs bed)

    - Use of mirrors/stools/auditory/manual cueing/weights/cervical collar

    - Consider ataxia and dysmetria present with movement

    Acute Care Interventions

    Static Sitting

    - Initial position of head/neck/shoulders/trunk/pelvis

    - Increasing length of time holding position

    - Having patient attempt postural corrections

    Dynamic Sitting

    - Weight shifting

    - Reaching inside and outside of base of support

    - Ability to return to midline

    - Scooting on surface

    Acute Care Interventions

    Transfers

    - Dependent � sliding board, patient lift

    - Use assistive devices as patient’s strength and balance improves

    - Sit stand

    Standing

    - Static (tilt table, standing frame)

    - Dynamic (parallel bars, walker)

    • Weight shifting

    • Postural control

    • Step-ups, weight bearing through weaker extremity

    Acute Care Interventions (continued)

  • 2/14/2018

    10

    Ambulation

    - Parallel bars (forwards, backwards, lateral stepping)

    - Walker (distance, weight shifting, step length control)

    - Hand held assistance

    Strength & Endurance

    - Gentle stretching of trunk and extremities

    - Strengthening of individual muscle groups

    - Recumbent bike

    - Sequencing of muscle groups to make exercises functional

    Acute Care Interventions (continued)

    Daily education to family and staff

    - Allow patient to initiate task if able

    - Stretching

    - Educate on muscle tone

    - Carryover of activities

    - Short bouts of activity, allowing for rest breaks

    Consider attention and ability to follow commands

    Use activities familiar to patient

    Be prepared to modify session based on patient presentation

    Use interdisciplinary team members

    Special Considerations

    Interdisciplinary team that meets biweekly

    - Neuropsychology & Psychology

    - Speech, Occupational, & Physical Therapy

    - School

    - Child Life Services

    - Clinical Nurse Specialists

    - Social Work

    Discuss patient status, goals, and potential barriers

    - Plan interdisciplinary goal for each team member to work on with patient

    - Goal example: Pt. will choose an activity in each session when presented with 2 options to increase initiation.

    Acute Neurological Injury (ANI) Rounds

    During break between RT and chemotherapy

    - 5-6 week break

    - Close to home when possible

    - If unable or unwilling to participate in inpatient rehab, encourage intensive outpatient program

    - Often shorter stays in inpatient rehab secondary to slow progress (FIM) and/or desire to be at home

    Transition to Inpatient Rehab

    Critical time to maximize return of function

    Transition to Inpatient Rehab

    Can completely

    focus on PT, OT, ST

    No other rigorous daily

    treatments

    Less fatigue (no radiation

    or chemotherapy)

    16-year-old female

    - Diagnosis of medulloblastoma, status post gross total resection

    - Presented to signs of PFS day +7 from surgery

    - Global ataxia, emotional lability, dysphagia, mutism

    - Patient underwent 6 weeks of RT and weekly vincristine

    - Main patient and family goal was to return home

    Discharge planning

    - 3 publicly funded options: adult rehab center, pediatric ABI program (far from home), community based rehab

    - Patient and family opted for community rehab to remain close to home

    Discharge Case Study23

  • 2/14/2018

    11

    Strengthening StretchingBalance

    Training & Coordination

    Gait Training

    EnduranceOrthotic

    PrescriptionEquipment HEP

    Outpatient Therapy Outpatient Therapy

    3 days per week

    30-45 minute sessions

    Primarily individual

    treats

    Occasional co-treats

    with challenging

    tasks

    � Gain and maintain as much function as possible during chemotherapy

    � Ongoing education regarding activity expectations and rehab trajectory

    � Monitor equipment needs, order custom WC if appropriate

    Goals for Outpatient Therapy

    Treatment Goals:1. Patient will ambulate 500’ with least restrictive assistive device without LOB,

    provided SBA for balance and verbal cueing 25% of the time to increase step

    length and to narrow BOS.

    2. Patient will reach outside of BOS anteriorly in standing to retrieve 5 objects with minimal A to achieve weight shift and to maintain balance.

    NuStep, Total Gym

    Emphasis on eccentric/graded control

    - Squats (don’t pop the ball), stoop and recover

    - TheraBand (control band back to starting position)

    - Step-ups/downs

    - Sit stand

    - Jumping, jumping down, hopping, descending stairs

    - Jump/hop and freeze

    Postural muscle strengthening

    - Glutes, abdominals

    - Emphasis on engaging abdominals/upright posture during functional tasks

    Strengthening

    Will sometimes perform in therapy sessions, but often have family/patient perform as part of HEP

    - Ankle dorsiflexion

    - Hamstrings

    - Trunk rotation, extension

    Stretching

    Control of dynamic movement

    - Weight shifting in sitting � standing

    - Activation of glutes and abdominals to perform true weight shift

    Movement in multiple planes

    Coordination

    - Timing, sequencing, amount of force, spatial awareness

    - Zip Ball, catching and throwing a ball, weighted therapy bar ball taps, squat

    stand, jumping (in place, to a target)

    Obstacle negotiation

    - Small obstacles (hurdles, River Stones, ramps), unstable surfaces (AirEx foam,

    DynaDisc), unpredictable surfaces (grass, gravel)

    Balance Training & Coordination

  • 2/14/2018

    12

    Narrowed base of support

    - Walking between balance beams

    - Romberg position (eyes open, eyes closed)

    - Tandem stance on line � balance beam � elevated balance beam

    - Single limb stance

    • Trapping soccer ball

    • Flamingo freeze

    Balance Training & Coordination

    Pre-gait activities

    - Foot to target

    • Make increasingly difficult: heel strike when bringing foot to target, weight shift onto limb once foot is on target, weight shift with step through to next target

    - Step up and over therapy bench for stair prep

    LiteGait on treadmill or over ground

    Ambulation with appropriate assistive device or in || bars

    - Anterior walker, posterior walker, hemi walker, cane

    - Ambulation through obstacle course

    Begin working on stairs, curb, and obstacle negotiation as skills

    progress

    Gait Training

    Transfers

    Standing

    Ambulation

    NuStep

    Recumbent or adaptive bike

    Advanced:

    - Floor hockey

    - Soccer

    - Gross motor bingo

    - Yoga

    Endurance

    Use of PRAFOs initially to prevent plantar flexion contractures, pressure wounds

    Fit for appropriate orthotics ASAP

    - AFOs (solid, hinged, carbon fiber)

    - SMOs

    - Arch Support

    Consider use of anti-hyperextension or hinged knee sleeve with

    instability

    Orthotic Prescription

    Often difficult to anticipate secondary to rapid improvements initially, followed by plateau

    Begin with loaner equipment when possible

    - Tilt in space/Convaid Rodeo � Standard or Semi-custom upright � Custom upright

    - Typically appropriate to purchase a custom wheelchair during chemotherapy

    Equipment

    Daily practice is emphasized

    Primarily functional mobility and gross motor tasks

    Train families to practice with assistive devices outside of

    therapy

    Home Exercise Program

  • 2/14/2018

    13

    Difficulty following commands

    - Often due to motor deficits

    Decreased/absent volitional speech

    - May be able to voice when laughing or crying but not on command

    Poor oral motor skills

    Receptive skills often relatively intact

    - May be difficult to detect due to motor and speech deficits

    - Speak TO them, not at or in front of them

    Role of Speech Therapy: Speech Outcomes24

    Initial goals and activities

    - Alternative means of

    communication

    • Gestures

    • GoTalk

    • Alphabet board

    • Picture communication board

    - Vocalizing on command

    - Identifying pictures

    - Following bodily commands

    Role of Speech Therapy: Speech Outcomes24

    As speech-language skills progress

    - Formal testing is initiated

    - Address dysarthria, initiation, and

    executive functioning

    Retrospective medical record review of 19 patients with PFS

    Role of Speech Therapy: Swallowing Outcomes3

    Symptom Number of Children

    Dysphagia 7 (37%)

    Impaired gag reflex 3 (16%)

    Facial weakness 4 (21%)

    Institution of enteral/parenteral feeds 10 (53%)

    Ability to swallow and mood typically improved before recovery of speech

    - May not indicate normal swallow

    - Mood changes may persist if patient is unable to communicate wants/needs

    effectively

    - Some patients made full recovery; swallowing difficulties persisted in others (sometimes long term

    Role of Speech Therapy: Swallowing Outcomes3,11,24

    Difficulty with oral

    motor movements

    Decreased strength

    Difficulty managing secretions

    Typically require

    NG- or G-tube

    Initial goals and activities

    - Following oral motor commands

    - Oral motor exercises

    - Swallowing exercises

    - Postural/compensatory strategies

    - Provision of adaptive equipment

    - Instrumental exam (if ready)

    Role of Speech Therapy: Swallowing Outcomes24

    As swallowing skills progress

    - Instrumental exam (when ready)

    - Advance PO diet as appropriate

    - Continue exercises, compensatory strategies

    Significant UE ataxia

    - Typically worse unilaterally

    Visual deficits

    - Nystagmus, diplopia

    Decreased level of functional performance

    Decreased participation in ADL & IADLs

    Difficulty with motor planning

    Sensory concerns, emotional lability

    Difficulty following commands

    Role of Occupational Therapy24

  • 2/14/2018

    14

    Initial goals and activities

    - Increasing independence with

    ADLs• Specifically dressing, toileting, and

    hygiene

    - Increasing mobility to participate in ADLs

    - Increasing visual skills

    - Functional reaching and strengthening

    - Provide toileting and bathing equipment

    Role of Occupational Therapy24

    As speech-language skills progress

    - Weight bearing activities (side sitting, quadruped)

    - Fine motor activities to improve coordination and strength

    - School based skills

    - Handwriting

    - Independence in higher level self

    care (tying shoes, buttons)

    - Functional cognition

    X Rigorous RT schedule, often with sedation

    X Side effects from chemotherapy

    - Nausea/emesis

    - Anemia

    - Thrombocytopenia

    X Infections

    X Slow progress due to nature of PFS

    X Cancer-related fatigue

    X Communication challenges

    X Psychosocial considerations

    Barriers to Achieving Goals

    Myelosuppression is a major side effect of chemotherapy

    - Potential for adverse events with stressful conditions (exercise)

    • Thrombocytopenia � bleeds

    • Severe anemia � cardiac arrhythmias

    • Neutropenia � sepsis

    Guidelines for Safe Exercise25

    Retrospective chart review (Gilchrist and Tanner, 2017)

    - Adverse events during or after 37 of 406 PT sessions

    • Most common event was tachycardia not requiring medical intervention

    - No serious adverse events occurred

    Guidelines for Safe Exercise25

    Cancer-Related Fatigue26

    “A distressing, persistent subjective sense of physical, emotional, and/or

    cognitive tiredness or exhaustion related to cancer or cancer treatment that is not proportional to recent activity and interferes with usual functioning.”

    - National Comprehensive Cancer Network, 2017

    Cancer-Related Fatigue27,28

    Cancer-Related Fatigue

    Cancer Treatment

    Burden

    Disease Burden

    Co-Morbid Conditions

    Psychosocial Burden

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    15

    Cancer-Related Fatigue29

    • Feeling tired, weary, exhausted even after a good night’s sleep

    • Lack of energy/prolonged fatigue after activity

    • Weakness, heaviness in arms/legs

    • Listlessness or irritability

    • Trouble starting or finishing tasks due to fatigue

    • Needing to sleep during the day

    • Unable or requiring help to do usual or desired activities

    • Being too tired to eat

    • Difficulty with concentration and memory

    • Limiting social activities due to fatigue

    Consistent therapist when possible

    - May have to introduce self and role multiple times

    One voice at a time

    1-2 step commands

    - Consider automatic movements

    - This then that statements

    Allow increased time for processing

    Collaborate with speech and psychology regarding

    communication and cognitive abilities

    Strategies for Communication

    7-year-old male

    - GTR medulloblastoma, 4th ventricle

    - Post-op: inability to speak, left-sided weakness, irritability

    - Transferred to inpatient rehabilitation hospital

    • PT, OT, ST; slow progress

    • Mute for a few days, then slow speech improvements but remained dysarthricwith “robotic speech”

    • Gait unsteady, but able to walk without assistance; unable to negotiate stairs

    • Challenging mood/behavior requiring risperidone during RT

    Two years after completion of treatment

    - Speech fluent, continuing to work with PT to address ongoing balance issues

    - Behaviorally and emotionally at baseline

    Published Case Studies20

    13-year-old male

    - GTR medulloblastoma

    - Post-op: paucity of speech, left-sided weakness, ataxia, VI CN palsy, emotional outbursts

    - Transferred to inpatient rehabilitation hospital x 6 weeks

    • Made significant progress with speech and ambulation

    • Mood remained flat over course of RT

    At 5-year follow-up

    - Performing well academically, attending college

    - Continued social and emotional struggles

    Published Case Studies20

    3-year-old female

    - Resection of posterior fossa ependymoma

    - Post-op: dysarthria, right-sided weakness, unable to ambulate independently, emotional outbursts

    - Began low dose risperidone to improve behavior

    • Allowed for safe participation in PT and ST and completion of RT

    At 5-year follow-up

    - Attending school full-time

    - Behavior and speech issues fully resolved

    - Ongoing challenges with balance � continuing to work with PT and OT regarding these issues

    Published Case Studies20

    Harbourne et al. 2014

    - 14-year-old female and 6-year-old female with PFS

    - Completed inpatient rehabilitation and outpatient programs, discharged due to lack of progress

    Neuromodulation devices on the tongue

    - Static and dynamic balance activities utilizing devices

    - 3 days of intensive training, followed by HEP consisting of activities performed 5 days/week for 8 weeks

    Clinical improvement noted in both participants

    - Improved balance beyond MCID on BBT-P

    - Increase in time standing on dynamic surface

    - Increased gait speed and step length

    Published Case Studies30

  • 2/14/2018

    16

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    - PFS occurs in up to 24% of patients who undergo posterior fossa tumor resection

    - Symptoms are often severe and last years beyond cancer treatment

    - The literature strongly advises participation in rehab, but specific strategies for

    management of this syndrome are lacking

    - More research is needed to determine the efficacy of specific rehab interventions for this population

    Summary

    Questions?

    1. Rehab for posterior fossa syndrome. St. Jude Children's Research Hospital. Available at: https://www.stjude.org/treatment/patient-resources/caregiver-resources/patient-family-education-sheets/rehabilitation/rehab-for-posterior-fossa-syndrome.html. Accessed October 25, 2017.

    2. Adler L, Dozier T, eds. Anatomy of a Child's Brain. Anatomy of a Child's Brain - Health Encyclopedia - University of Rochester Medical Center. https://www.urmc.rochester.edu/encyclopedia/content.aspx?ContentTypeID=90&ContentID=P02588. Accessed October 25, 2017.

    3. Kirk E, Howard V, Scott C. Description of posterior fossa syndrome in children after posterior fossa brain tumor surgery. Journal of Pediatric Oncology Nursing. 1995;12(4):181-187.

    4. Reed-Berendt R, Phillips B, Picton S, et al. Cause and outcome of cerebellar mutism: evidence from a systematic review. Child’s Nervous System. 2014;30(3):375-385.

    5. Robertson P, Muraszko K, Holmes E, et al. Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children’s Oncology Group. Journal of Neurosurgery: Pediatrics. 2006;105:444-451.

    6. Kupeli S, Yalcin B, Bilginer B, et al. Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatric Blood and Cancer. 2011;56(2):206-210.

    7. Medulloblastoma. St. Jude Children’s Research Hospital. Available at: https://www.stjude.org/disease/medulloblastoma.html. Accessed November 21, 2017.

    References

    8. Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)–Health Professional Version. 2017. Available at: https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq#link/_307_toc. Accessed November 28, 2017.

    9. Gudrunardottir T, De Smet H, Bartha-Doering L et al. Posterior Fossa Syndrome (PFS) and Cerebellar Mutism. In: Marien P, Manto M, ed. 1st ed. London: Elsevier;2015:257-281. Available at: https://books.google.com/books?hl=en&lr=&id=aQGdBAAAQBAJ&oi=fnd&pg=PA257&dq=posterior+fossa+syndrome+children&ots=yfPkzyVgI8&sig=z5I8lDm4Km4BoWa63htcnc2GVdk#v=onepage&q&f=false. Accessed November 14, 2017.

    10. Law N, Greenberg M, Bouffet E, et al. Clinical and neuroanatomical predictors of cerebellar mutism syndrome. Neuro-oncology. 2012;14(10):1294-1303.

    11. Steinbok P, Cochrane D, Perrin R, et al. Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatric Neurosurgery. 2003;39(4):179-183.

    12. Gadgil N, Hansen D, Barry J, et al. Posterior fossa syndrome in children following tumor resection: knowledge update. Surgical Neurology International. 2016;7(Suppl 6):S179-S183.

    13. Catsman-Berrevoets C. Cerebellar mutism syndrome: cause and rehabilitation. Current Opinion in Neurology. 2017;30(00).

    14. Avula S, Mallucci C, Kumar R, Pizer B. Posterior fossa syndrome following brain tumor resection: review of pathophysiology and a new hypothesis on its pathogenesis. Child’s Nervous System. 2015;31:1859-1867.

    15. Morris E, Phillips N, Laningham F, et al. Proximal dentatothalamocortical tract involvement in posterior fossa syndrome. Brain. 2009;132:3087-3095.

    References

    16. Korah MP, Esiashvili N, Mazewski CM, et al. Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. International Journal of Radiation Oncology* Biology* Physics. 2010;77(1):106-112.

    17. Palmer S, Hassall T, Evankovich K, et al. Neurocognitive outcome 12 months following cerebellar mutism syndrome in pediatric patients with medulloblastoma. Neuro-oncology. 2010;12(12):1311-1317.

    18. Steinbok P, Cochrane D, Perrin R et al. Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatric Neurosurgery. 2003;39:179-183.

    19. Brinkman T, Li Z, Neglia J, et al. Restricted access to the environment and quality of life in adult survivors of childhood brain tumors. Journal of Neurooncology. 2013;111(2):195-203.

    20. Lanier J, Abrams A. Posterior fossa syndrome: review of the behavioral and emotional aspects in pediatric cancer patients.

    Cancer. 2017;123:551-559.

    21. Franjoine M, Darr N, Held S, et al. The performance of children developing typically on the pediatric balance scale. Pediatric Physical Therapy. 2010;22(4):350-359.

    22. Pediatric Balance Scale. Shirley Ryan Ability Lab. Available at: https://www.sralab.org/rehabilitation-measures/pediatric-balance-scale. Accessed January 31, 2018.

    23. Pearlman L, McVittie A, Hunter K. Discharge management of an adolescent female with posterior fossa syndrome: A case report. Canadian Journal of Neuroscience Nursing. 2008;30(3).

    References

    24. Lyons K, Christoffersen L, Harris A. Posterior Fossa Syndrome. Marianjoy Rehabilitation Hospital. 2017.

    25. Gilchrist L, Tanner L. Safety of symptom-based modification of physical therapy interventions in pediatric oncology patients with and without low blood counts. Rehabilitation Oncology. 2017;35:3-8.

    26. “NCCN Clinical Practice Guidelines in Oncology: Cancer-Related Fatigue.” National Comprehensive Cancer Network. Version 2.2017 ed. Published April 10, 2017. Accessed July 11, 2017.

    27. Cleveland Clinic. “Fatigue & Cancer Fatigue.” Available at: https://my.clevelandclinic.org/health/articles/cancer-related-fatigue. Accessed July 11, 2017.

    28. Hill A, Hughes J. Empowering Cancer Survivors to Become the CEO (Chief Energy Officer) of Everyday Life. 2017.

    29. Calvet M, Curran J, Yamada K. Cancer-Related Fatigue. Oncology Section, APTA; 2015:1-3. Available at:

    http://oncologypt.org/pdfs/fact-sheets/Cancer-related-Fatigue-rev-4-10-16.pdf. Accessed July 13, 2017

    30. Harbourne R, Becker K, Arpin D et al. Improving the motor skill of children with posterior fossa syndrome: a case series. Pediatric Physical Therapy. 2014;26:462-468.

    References