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Saliva control in Cerebral Palsy:

multidisciplinary management and research findings from the Australian and Dutch drooling

team

AACPDM 67th Annual meetingMilwaukee, October 16-19, 2013

Dinah ReddihoughLouise Baker, MB, BchSue Reid, PhDJan van der Burg, PhDKaren van Hulst, MSc

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Disclosure Information

AACPDM 67th Annual Meeting October 16-19, 2013

Speakers Names: Jan van der Burg

Karen van Hulst

Disclosure of Relevant Financial Relationships

We have no financial relationships to disclose.

Disclosure of Off-Label and/or investigative uses:We will not discuss off label use and/or investigational use in my presentation

Speakers Names: Louise Baker

Sue Reid

Disclosure of Relevant Financial RelationshipsWe have the following financial relationships to disclose:

• Grant/Research support from: Allergan Australia (Botox)

Disclosure of Off-Label and/or investigative uses:We will discuss the following off label use in my presentation:

• BoNT-A

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Amalia’s child Hospital

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The Multidisciplinairy Australian drooling team*

Paediatricians• Dinah Reddihough MD• Louise Baker MB Bch

Speech pathologists• Hilary Johnson PhD• Katherine Ong BAppSc

Dentist• Mala Desai BDSc

Surgeon• David Chong FRACP

Clinic co-ordinator• Christine Westbury RN

Researcher• Sue Reid PhD

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The multidisciplinary Nijmegen drooling team*

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From left to right:Rehabilitation Doctor : • Dr. P. Jongerius, PhD

Psychologist:• Dr. J. Van der Burg, PhD

Paediatric Neurologist:• Dr. C. Erasmus, PhD

Speech pathologists:• K. Van Hulst, MSc• S. de Groot

ENT specialist:• Dr. F. Van den Hoogen, PhD• A. Scheffer, Phd• S. Kok

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STREETS OF LONDON -Ralph McTellSTREETS OF LONDON - Ralph

McTell

Program

• Introduction Louise

• Role of the speech pathologist Karen

• Behavioral therapy Jan

• Intra-oral appliances and Botulinum toxin Sue

• Medications and Surgery Louise

• Discussion

• Case History

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Role of the Speech Pathologist

• Facts about swallowing problems in children with CP

• Anterior and Posterior drooling

• Assessement

• Objective drooling measures

• Subjective drooling measures

• Intervention

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Drooling is a swallowing problem

Dysphagia:

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Inability to swallowdrinks, food andmedication efficient andsafely

Inability to swallow reflux material efficient andsafely

Inability to swallow salivaefficient and safely

Direct aspiration

Indirect aspiration

Anterior and posterior drooling/saliva aspiration

Erasmus CE, van Hulst K, Rotteveel JJ, Willemsen MA, Jongerius PH.Clinical practice: swallowing problems in cerebral palsy.Eur J Pediatr. 2012 Mar;171(3):409-14

Prevalence and predictors of drooling in children w ith CP

(Weighted) Prevalence of drooling22% Parkes et al, 201039.6 % Reid et al, 2012

Significant association drooling and ……GMFCS levelTopografical patternHead posture stabilisationEpilepsyIntellectual disabilityType of schoolingOromotor functionEating/speech difficulties

Reid SM, McCutcheon J, Reddihough DS, Johnson H.Prevalence and predictors of drooling in 7- to 14-year-old children with cerebral palsy: a population study. Dev Med Child Neurol. 2012 Nov;54(11)

The role of saliva;

Saliva has seven main functions:

• Protect teeth and gums

• Prepares foods for chewing and swallowing

• Initiates carbohydrate digestion

• Lubricates tongue and lips for speech

• Assists with oral hygiene

• Regulates acidity

• Facilitates taste

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Sublingual glands

Submandibularglands

Parotid glandParotid gland

Salivary glands Saliva production

• Daily saliva secretion: 500 ml – 1.5 litres

• Most produced by two pair of glands:- Submandibular (seromukus saliva): 60 - 70% in rest- Parotid (sereus): 25% during eating, chewing

• Swallowing occurs:- in rest: 600 times a day (Lear 1965)

- total (sleep, eating, rest): ca 1200 times a day (Rudney & Larson 1995)- 0.3 – 6.7 x per minute- At night: 0 - 7x per hour

• Drooling beyond the age of 4 is abnormal

• Drooling is a multifactorial problem

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Anterior - Posterior drooling

Anterior drooling:Saliva spilled from the mouth that is clearly visible

Posterior drooling:Saliva spilled into the pharynxpossibly creating a risk of aspiration

posterior anterior

Jongerius PH, van Hulst K, van den Hoogen FJ, Rotteveel JJ.The treatment of posterior drooling by botulinum toxin in a child with cerebral palsy.J Pediatr Gastroenterol Nutr. 2005 Sep;41(3):351-3

Consequences drooling

Anterior drooling

• Social rejection and isolation• Lack of self confidence• Stigmatizing, shame• Damp and soiled clothes• Irritated chapped skin• Damage (communication devices,

computers, furniture, books, etc)• Interference with speech• Unpleasant odour

Posterior drooling

Saliva contains bacteria and yeastwhich can cause:

• Recurrent respiratory symptoms• Wheeze• Chronic cough• Choking• Failure to thrive• Radiological signs of chronic lung

injury

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Drooling: direct causes

Disturbed oropharyngeal swallow phase:

• Poor saliva bolus formation

• Inadequate lip closure

• Reduced frequency of swallowing

• Absent oropharyngeal sensation

• Hypo/hypertonia tongue, lips, cheek

• Disorganised tongue movements

• Delayed coordination of swallowing/dysphagia

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Drooling: Indirect causes

• Inadequate posture (trunk/head)

• Retardation; cognitive level < 3 years

• Reduced awareness, not able to divideattention to double tasks

• Malocclusion

• Mouthing

• Medication

• Reflux (oesofageale saliva reflex)

• Dentition

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Hypersalivation is no cause!

Hypersalivation?normal salivation = mean 0.3 ml/min

Study Erasmus, van Hulst et al 2009: normal rest salivationof 0.34 ml/min in CPvs. 0.32 ml/min in Controls

Erasmus, C. E., Hulst K. van, L. J. Rotteveel, P. H. Jongerius, F. J. Van den Hoogen, N. Roeleveld, and J. J. Rotteveel, 2009, Drooling in cerebral palsy: hypersalivation or dysfunctional oral motor control?:Dev.Med.Child Neurol., v. 51, no. 6, p. 454-459.

Except in the subgroup children with dyskinetic CP…

SLT Assessment

History and patient’s goal for treatment

Posture and mobility

Orofacal examination and oral hygiene

Oral sensory motor functions and abilities

Communication and speech possibilities

Eating and drinking observation

Specific swallow and drooling measures

Self managment strategies

Specific swallow measures

Swallowing on demand

Swallow frequency

Quality and safety of the swallow act

Tongue action during swallowing

Oral control

Cough reaction

Composition of saliva (muceus, sereus, etc)

Specific drooling measures (clinical and research t ools)

Subjective reporting:• Visual Analog Scale (VAS)• Questionnaires/ Drooling Impact Scale Burg van der 2006, Reid 2010

• Drooling Severity and Frequency Scale Thomas Stonell & Greenberg 1988

Objective measures : • Collection devices bibs/cups Sochaniwiskyj 1982

• Drooling Quotient Rapp 1980, van Hulst 2012

Burg van der JJ, Jongerius P, van Limbeek J, van Hulst K, Rotteveel J. Drooling in children with cerebral palsy: a qualitative method to evaluate parental perceptions of its impact on daily life, social interaction, and self-esteem. Int J Rehabil Res 2006 Jun;29(2):179-82.

Reid SM, Johnson HM, Reddihough DS. The Drooling Impact Scale: a measure of the impact of drooling in children with developmental disabilities. Dev Med Child Neurol 2010 Feb;52(2):e23-e28.

Drooling Severity and Frequency Scale (DSFS)

Severity Frequency

1. Dry Never drools 1. Never

2. Mild Wet lips only

3. Moderate Wet lips and chin 2. Occasionally Not every day

4. Severe Wet clothes 3. Frequently Part of every day

5. Profuse Wet clothing, hands, tray and objects

4. Constantly Constantly

Thomas-Stonell N, Greenberg J. Three treatment approaches and clinical factors in the reduction of drooling. Dysphagia 1988;3(2):73-8.

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Drooling Quotient; 5 minute version

A semi quantitative, direct observational method that evaluates drooling by measuring (new) leaked saliva from the lips

Conclusion:• DQ10 and DQ5 can be used interchangeably• DQ5 is reliable, accurate, and time efficient• The DQA during activity is most discriminative for drooling severity• A clear cut-off point can be used to support clinical decision

making

van Hulst K, Lindeboom R, van der Burg J, Jongerius P.Accurate assessment of drooling severity with the 5-minute drooling quotient in children with developmentaldisabilities. Dev Med Child Neurol. 2012 Dec;54(12):1121-6

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How to measure posterior drooling?

Mostly by clinical signs:

Cervical auscultation

Diagnostics• FEES (fiberoptic-endoscopic

evaluation of swallowing)

• Saliva is not visible duringvideofluoroscopic study

Interventions drooling

Anterior drooling

• Oro-motor, oro-sensory therapy• Behavioral therapy• Intra-oral appliances• Pharmacologic treatments• Surgery

Posterior drooling

• Physical and dysphagiatreatment

• Pharmacologic treatments• Surgery• Radiotherapy

Drooling interventions

Cochraine review (Walshe et. al. 2012)Lack of consensus regarding which interventions are most effective for children with CP.

Pharmacologic treatments (BoNT-A, anticholinergica)

Surgery

Behavioral therapyIntra-oral appliances

Conservative; oro-sensory motor therapy

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Oro-sensory motor therapy

• Take care of conditions (adequate posture, reflux treatment, no caries, no ENT problems, etc)

• Oro- Motor therapy to improve lip and jaw closure, increasing tongue andoral control, reduce tongue thrust, etc

• Improve oral perception, awareness (Is my chin dry?)

• Learn slurp-swallow on demand (“swallow factory”)

• Improve swallowing frequency

• Learn to stay dry (wiping, swallowing)

• Eating and drinking therapy

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Behavioral therapy for drooling

• Drooling defined as behavior problem: too little swallowing, too little wiping, etc.

• Intervention goal: increasing the frequency of swallowing and/or wiping

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Behavioral procedures for drooling

Five types of procedures have been proven effective:

1. instruction, prompting en positive social reinforcement

2. negative social reinforcement and other ‘decelarative’procedures

3. automatic cueing

4. automatic reinforcement (microswitch-based technology)

5. self-management procedures

Van der Burg, J., Didden, R. & Lancioni, G. (in press). Drooling and Tongue Protrusion. In: D.I. Mostofsky & F. Fortune (Eds). Behavioral Dentistry, 2nd Edition. Wiley-Blackwell.

Behavioral procedures for drooling

Intervention techniques:• Antecedent control

• Consequent control

• Combination (antecedent and consequent control)

• Self-management

• Instruction, prompts• Automatic cueing (devices)

• Positive feedback• Automatic reinforcement

(microswitch technologies)• Negative reinforcement• Decelarative procedures

Behavioral procedures for drooling:from external to internal control

Intervention techniques:• Antecedent control

• Consequent control

• Combination (antecedent and consequent control)

• Self-management

• Instruction, prompts• Automatic cueing (devices)

• Positive feedback• Automatic reinforcement

(microswitch technologies)• Negative reinforcement• Decelarative procedures

Examples of behavioral procedures

Instruction

Prompts

Automatic cueing

Positive feedback

Automatic reinforcement

Negative reinforcement

Decelarative procedures

• e.g. ‘Wipe your face when wet’

• Auditory/verbal, visual or tactile cues from a trainer, parent or teacher

• Auditory, visual or tactile cues at set time intervals from a portable device

• Positive remark, token (sticker)

• Music after activation of microswitch in bib

• Negative remark

• Overcorrection: wipe chin and mouth 10 times after being wet

Behavioral therapy for drooling

Two treatment protocols:

• Self-management

• Automatic tactile cueing

Self-management trainingfor drooling: case-series 1

•Inclusion criteria

•Setting

•Method- Dependent variable (latency and VAS) & design

•Treatment procedure- Instruction -> self-instruction- Positive/negative feedback -> self-evaluation and

self-reinforcement- Gradual increase of time interval

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Results case-series 1 (n=10)

Van der Burg, J., Didden, R., Engbers, N., Jongerius, P., & Rotteveel, J. (2009). Self-management treatment of drooling: A case series. Journal of Behavior Therapy and Experimental Psychiatry, 40, 106-119.

Conclusions from case series 1

• Participants learn to apply the selfcare-routine and manage to staydry during daily activities for intervals of 30-60 minutes

• Parents and teachers report changes in selfcare and reduceddrooling at home and at school (VAS) after discharge

• Latency-scores FU 6 & 24 weeks: 3 participants effectsmaintained; 5 children decrease to baseline-level

• VAS-scores (parents & teachers) FU 6 & 24 weeks: positiveeffects at home and at school, compared to baseline

Generalization & maintenance should be improved!

Self-management trainingfor drooling: case-series 2

Procedural additions to promote generalization & mainten ance:• Personal motivation child made explicit

• Differential evaluation and reinforcement of (1) swallowing,

(2) controlling and (3) wiping the mouth/chin area

• Parent/teacher instruction and feedback

• 4 post-intervention sessions:

2x phone consults (3 & 12 weeks after discharge)

2x school visits (6 & 24 weeks after discharge)

Case series IIResults case series 2 (n=10)

(Results not yet published)

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Conclusions from case series 2

• 9 (from 10) participants learn to apply the selfcare-routine and manage to stay dry during daily activities for intervals of 30-45 minutes

• Parents and teachers report changes in selfcare and reduceddrooling at home and at school (VAS) after discharge

• Latency-scores FU 6 & 24 weeks: 3 (from 9) participants effectsmaintained; 2 children decrease to baseline-level

• VAS-scores (9 parents & teachers) FU 6 & 24 weeks: positiveeffects at home and at school, compared to baseline

Overall conclusions on self-management

• Self-management program appears effective for a selectedsubgroup of children

• Instructing parents and teachers makes them independent fromprofessional trainers in case of relapse/increase of drooling

• Maintenance appearsdependent on personal factors (e.g., motivation, degree of (oral)motor problems) and environmentalfactors (e.g., attitude, motivation)

• Latency scores are stern: not only a decrease in frequency and severity of drooling, but non-drooling is the target!

Behavioral therapy for drooling

Two treatment protocols:

• Self-management

• Automatic tactile cueing

Automatic tactile cueing: devices

Prototype 1

Prototype 2

Prototype 3

Automatic tactile cueing: procedure

1. Introduction of cueing device in training setting (outside classroom)

2a. Teach the child to swallow on the cue (instruction, modeling, positive social reinforcement)

If 2a is not successful :

2b. Teach the child to wipe the mouth/chin on the cue (instruction, modeling, most-to-least prompting, positive social reinforcement)

3. Implementation of cueing device in the classroom at set times

4. Implementation of cueing device in the classroom throughout the day

5. Implementation of cueing device at home

Some results from our N=1 studies

Results up to now are variable:

+ A boy with CP (CA 15y; DA 2-3 years) learned to wipe his mouth on the tactile cue, and remained dry at school and at home, even when he was not wearing his cueing device anymore.

- After 20 one-to-one trainingssessions, a girl with a non-classified syndrome (CA 8y; DA 20 months) appeared not to be able to wipe her mouth on the tactile cue.

+ A boy with CP (atactic type, bilateral spasticity) and mild intellectual disability (CA 8y; IQ 63) learned to swallow on the tactile cue, but after introduction of the cueing device in the classroom and at home, this effect slowly disappeared.

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Behavioral procedures for drooling: analysis of stu dies

Conclusions from reviews (Van der Burg et al., 2007a,b, 2013):

• Instructions, prompts, feedback & reinforcement: systematic implementation

• Cueing interventions: children remain dependent on the device

• Microswitch technologies: severe-to-profound range of ID

• Negative reinforcement and decelarative procedures: avoid if possible

• Self-management: maximum independence; re-training/procedural changes necessary for generalization & maintenance; contraindicated severe/profound ID or DA below 6 yrs

Van der Burg, J., Didden, R., Jongerius, P., & Rotteveel, J. (2007a). Behavioural treatment of drooling: a methodological critique of the literature with clinical guidelines and suggestions for future research. BehaviorModification, 31, 573-594.Van der Burg, J. , Didden, R., Jongerius, P., & Rotteveel, J. (2007b). A descriptive analysis of studies on behavioural treatment of drooling (1970-2005). Developmental Medicine and Child Neurology, 49, 390-394.Van der Burg, J, Didden, R., & Lancioni, G. (in press: december 2013). Drooling and Tongue Protrusion. Chapter 12. Behavioral Dentistry 2nd Edition, Mostofsky DI & Fortune, F. (Eds). Wiley-Blackwell

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Drug therapy

Drug therapy

• Oral medication (anticholinergics)• Benzhexol hydrochloride (Artane) • Glycopyrrolate (Robinul)

Usage:• In young children where maturation of oral function

may still occur• In older children and adults with relatively mild saliva

control problems• As an alternative to surgery for those who prefer a

non-operative approach

Drug therapy• Individual dose quite variable

• Medication begins to act within an hour, peaks at 1-3 hours, and duration of action is 6-12 hours

• Best therefore taken with breakfast and lunch

• Tablets can be crushed and placed in food

Drug therapy

• Side effects• Sedation and irritability• Worsening of constipation

and urinary retention• Blurred vision• Flushed dry skin

Drug therapy - dosage

• Benzhexol hydrochloride• 1 mg twice daily for 1-2 weeks• 2 mg twice daily for 1-2 weeks

• 2 mg up to 3-4 times daily

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Drug therapy - dosage

• Glycopyrrolate• 0.01 – 0.04 mg / kg per dose• 10-15 kg

• 0.25 mg bd, 0.5 mg bd, 1 mg up to tds

• 15 kg – 25 kg• 0.5 mg bd, 1 mg bd, 1 mg tds

• ≥ 25 kg• 1 mg bd, 1 mg tds, 1.5 mg tds

Drug therapy – research evidence

• Benztropin• 27 patients (7 dropped out)• Positive effect on drooling(Camp-Bruno 1989)

• Benzhexol• 20 children aged 3 – 12 years

• 17 children showed improvement in drooling• Side effects were minimal(Reddihough 1990)

Drug therapy – research evidence

• Glycopyrrolate• RCT of 39 patients (4-19 years)• 12 children did not complete study• Overall improvement(Mier et al 2000)

• Open label study of 40 patients (4-27 years)• Drooling improved in most(Blasco & Stansbury 1996)

• Retrospective trial in 54 children• Most improved but 50% had adverse effects(Bachrach et al 1998)

Glycopyrrolate: recent papers• Zeller RS, Lee HM, Cavanaugh PF, Davidson J.

Randomized Phase III evaluation of the efficacy and safety of a novel glycopyrrolate oral solution for the management of chronic severe drooling in children with cerebral palsy or other neurologic conditions. Ther Clin Risk Manag. 2012;8:15-23.

• Eiland LS. Glycopyrrolate for chronic drooling in children. Clin Ther. 2012; 34(4):735-42.

• Reddihough DS, Reid SM, Plover C. Evaluation of glycopyrrolate in the treatment of chronic drooling. Degen Neuro Neuromusc Dis 2011; 1:3-7.

Intraoral appliances The ISMAR (Haberfellner)

• Innsbruck Sensory Motor Activator and Regulator

• Stabilizes jaw to facilitate lip and tongue movements

• Worn for short periods each day then overnight

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ISMAR insertion ISMAR study aims

To determine whether• the ISMAR is an effective intervention

to improve drooling in children with CP

• we could identify any factors to indicate which children were good candidates for this type of therapeutic approach

Johnson HM, Reid SM, Hazard CJ, et al. Effectiveness of the Innsbruck Sensorimotor Activator and Regulator in improving saliva control in children with cerebral palsy. Dev Med Child Neurol 2004; 46:39-45.

ISMAR study methods• Subjects: 18 children, 4-11y with mild-severe

CP, dysphagia, drooling (14 wheelchair dependent)

• Three phases of 6 months1. Control phase2. Stabilisation phase3. Mobilisation phase

• Assessments and dental impressions performed at start of each phase and completion of project

ISMAR study measures

• Frequency and severity of drooling measured• Thomas-Stonell & Greenberg

• Eating and drinking skills assessed using Functional Feeding Assessment (modified)• Gisel

• Compliance an issue • only 6/18 completed study

Severity of drooling

Dry

Mild

Moderate

Severe

Profuse

Control Stabilisation Mobilisation

Change in eating and drinking skills

0%

5%

10%

15%

Spoonfeeding

Biting Chewing Cup drinking

Straw drinking

Swallowing

20%ControlTreatment

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ConclusionsISMAR study

• Careful candidate selection is necessary

• Prolonged illness, surgery, seizures and intolerance of appliance led to many withdrawals

• Good cognitive function and motivation were key to successful outcome

Botulinum toxin (BoNT-A)

• 75% of salivation mediated by cholinergic neurotransmitters

• BoNT-A blocks release of acetylcholine

Background

• Within 1-3 days BoNT binds to nerve endings and reduces amount of saliva produced

Methods used

• In Melbourne, submandibular and parotid glands injected under ultrasonic guidance and G.A.• Dosage: 25 units per gland diluted to 1ml to a

maximum dose of 4 units per Kg (Total of 100 units in 4 ml in children >25kg)

• In The Netherlands submandibular glands injected initially • If good response, injection into submandibular glands

is repeated. • If poor response, combined submandibular and

parotid glands injections considered (or one of the other treatment options).

BoNT-A injections BoNT-A injections

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BoNT-A injections BoNT-A injections

BoNT-A injections RCT of BoNT-A injections

Aims:

• To assess effectiveness of BoNT-A injections into submandibular and parotid glands for anterior drooling in children with CPand other neurological disorders

• To ascertain timing of maximal response and duration of effect

Reid SM, Johnstone BR, Westbury C, Rawicki B, Reddihough DS. Randomized trial of botulinum toxin injections into the salivary glands to reduce drooling in children with neurological disorders. Dev Med Child Neurol 2008; 50:123-28.

Intervention

• 100u BoNT-A diluted into 4 mls normal saline

• Under short ga and ultrasonic guidance, 1 ml injected into each gland

• 25u per gland OR

• 4u per kg if child < 25kg

Parotid gland

Submandibulargland

Sublingual gland

Study methods

• Unblinded RCT, parallel groups • Sample size 28 per group• Follow-up for 6 months• Controls received no treatment but eligible for

treatment at end of follow-up

• Collected information on demographics, current medications, general health, side-effects

• Main outcome measure: Drooling Impact Scale (DrI Scale) at baseline and monthly for 6m

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Progress through study

Allocated to intervention n=26

Received intervention n=24Allocated no intervention n=24

Received no intervention n=24Allocation

LTFU n=0

Discontinued n=0

LTFU n=0

Discontinued n=0

Analysed n=24

Excluded n=0

Analysed n=23

Excluded n=1 (incomplete)

Follow-up

Analysis

Enrolment

Randomisation

Results: DrI Scale scores

0

10

20

30

40

50

60

70

Baseline 1 month

DrI

scal

e sc

ore

Control group

Treatment group

p<0.001

Results: Duration of response

0

5

10

15

20

25

Baseline 1 mth 2 mths 3 mths 4 mths 5 mths 6 mths 12 mths

Control

Treatment

Results: Summary

• 4/24 children had no response at 1m

• < 10 point reduction on DrI Scale

• 4/24 children had mediocre response • 10-20 point reduction on DrI Scale

• 67% response rate• Greatest response at 1m• At 6m difference between control and

treatment groups remained significant

Dutch BoNT study• Participants : 131 children with CP or other

neurological disorder with mod-severe drooling• Intervention : BoNT-A to submandibular glands

• 15 U per gland for children <15 kg• 20 U/gland for children weighing 15-25 kg• 25 U/gland for children weighing >25 kg

• Outcomes: Drooling Quotient and caretaker VAS

• Results : 47% response rate for median of 22w

Scheffer AR, Erasmus C, van Hulst K, van Limbeek J, Jongerius PH, van den Hoogen FJ. Efficacy and duration of botulinum toxin treatment for drooling in 131 children. Arch Otolaryngol Head Neck Surg. 2010;136(9):873-7.

Side effects noted

• Problems with swallowing (both studies)• 1 significant and 3 minor (17%) in Melbourne study• 3% minor in Dutch study + 6% deterioration in feeding

• 1 chest infection and 1 first seizure in Melb

• Changes in saliva consistency (both studies)• 41% had thickened saliva in Dutch study• 12% reported reduction in viscosity

• Improvements in handling of secretions, speech, feeding behaviour (both studies)

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Conclusions

• These studies support use of BoNT-A injections into submandibular +/- parotid glands in children with CP and other neurological disorders for management of drooling in approx. 50-67% cases

• Remained difficult to predict which children would respond and which children would experience unwanted side effects

• Response to repeat doses still unknown

Dutch study on saliva consistency

• 15 children: spastic or dyskinetic quadriplegic CP, GMFCS IV-V, mod-severe drooling

• Mucin concentration of saliva analysed pre and post BoNT-A.

• 9 children had thickened saliva. • 7 had swallowing and chewing problems

• 2 needed treatment with mucolytics due to pooling of thickened saliva in throat

Erasmus CE, Van Hulst K, Van Den Hoogen FJ, Van Limbeek J, Roeleveld N, Veerman EC, Rotteveel JJ, Jongerius PH. Thickened saliva after effective management of drooling with botulinum toxin A. Dev Med Child Neurol. 2010;52(6):e114-8.

Secondary effects of BoNT-AAims:

• To assess the secondary benefits and side effects of BoNT-A injections into parotid and submandibular glands in children with developmental disability

• To determine whether these effects are related to reduction in drooling

Study methods

Participants• 26 children were injected (14 males, 12

females, mean age 11y 3mo)

Pre and post assessments• Drooling Impact (DrI) Scale

• Secondary effects questionnaire• Eating, speech, saliva management, sleep

Results• Over 4 weeks, improvement seen for entire

group for drooling (p<0.001), eating (p=0.05), speech (p=0.04), and sleep (p=0.01)

• No improvementin ability tomanage saliva

• Graph shows decrease in drooling

Relationship b/t drooling response and changes in eating and sleep

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Conclusions

• Improvement in eating skills and sleep occurred in the children whose drooling also improved

• Deterioration in eating skills occurred in a minority whose drooling did not improve

• Hypothesised that may be related to accuracy in locating middle of gland

• Need to ensure child has adequate swallowing to allow for unforseen deterioration

Consensus statement

• Avoid BoNT-A if• Given in previous 3 mo• Patient has formed antibodies• Patient unfit for anaesthesia

• Limit adverse events by• Ultrasonic guidance• Observation for 2 hrs, regular contact first wk• Moist or pureed food over first wk• Be aware of swallowing/respiratory problems

Reddihough D, Erasmus CE, Johnson H, McKellar GM, Jongerius PH; Cerebral Palsy Institute. Botulinum toxin assessment, intervention and aftercare for paediatric and adult drooling: international consensus statement. Eur J Neurol. 2010 Aug;17 Suppl2:109-21

Saliva control surgery Saliva control surgery

Potential options:• Excision of the salivary glands• Ligation of salivary ducts• Relocation of ductsScheffer AR, Erasmus C, Van Hulst K, Van Limbeek J, Rotteveel JJ, Jongerius PH, van den Hoogen FJ. Botulinum toxin versus submandibular duct relocation for severe drooling. Dev Med Child Neurol. 2010; 52(11):1038-42.

Scheffer AR, Bosch KJ, van Hulst K, van den Hoogen FJ. Salivary duct ligation for anterior and posterior drooling: our experience in twenty one children. Clin Otolaryngol. 2013 Jul 3.

Greensmith AL, Johnstone BR, Reid SM, et al. Prospective analysis of the outcome of surgical management of drooling in the paediatric population: a 10 year experience. Plast Reconstr Surg 2005; 116:1233-42.

Submandibular Duct Relocation

• First reported by Ekedahl (1974).• Crysdale’s (1989) 194 patients

20% excellent47% good22% fair11% poor

• 8% developed ranulae

• 2% need for submandibular gland excisiondue to obstruction/cyst formation.

Current practice

• In Melbourne we perform a combination of bilateral submandibular duct transposition (BSMDT) and bilateral sublingual gland excision (BSLGE)• Additional parotid duct ligation performed

occasionally

• In The Netherlands standard surgery is submandibular duct relocation +/-sublingual excision, or duct ligations

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BSMDT and BSLGE BSMDT and BSLGE

BSMDT and BSLGE Melbourne surgical study

• Studied 72 children who had BSMDT and BSLGE between 1993 and 2001• 53% CP; 38% ID; mean age 10.4y (4 -19)

• Children assessed pre-op then at 1m, 6m, 12m, 2y, 5y post-op

• Outcome measures• Drooling Severity and Frequency Scale• No. bib/clothing changes

Greensmith AL, Johnstone BR, Reid SM, et al. Prospective analysis of the outcome of surgical management of drooling in the paediatric population: a 10 year experience. Plast Reconstr Surg 2005; 116:1233-42.

Frequency of drooling @ 2 yrs

Pre-op 2 yrs

Never

Occasionally

Frequently

Constantly

Severity of drooling @ 2 yrs

Dry

Mild

Moderate

Severe

Profuse

Pre-op 2 yrs

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18

Other results

1

2

3

4

2 yrs 5 yrs

� Bib/clothing changes fell from 4 to 0

� Nearly half had 2 point drop on severity scale

� Frequency and severity maintained at 5y

� 5 had further surgery

� 13 complications� 7 major bleeding/swelling

1

2

3

4

5

2 yrs 5 yrs

Severity

Frequency

Surgery compared to BoNT-A

• Children with severe drooling where surgery performed after BoNT-A

• DQ reduced to a greater extent after BSMGT and BSLGE than after BoNT-A (p=0.001) • DQ equivalent at baseline• DQ 10 v 18 at 8 wks• DQ 4 v 22 at 32 wks

Scheffer AR, Erasmus C, Van Hulst K, Van Limbeek J, Rotteveel JJ, Jongerius PH, van den Hoogen FJ. Botulinum toxin versus submandibular duct relocation for severe drooling. Dev Med Child Neurol. 2010; 52(11):1038-42.

Duct ligation alone

• 21 Dutch children underwent either 2 (SMx2), 3 (SMx2+Px1), or 4 (SMx2+Px2) duct ligation

• Less invasive• Good results

short-term• Long term results

not established

Scheffer AR, Bosch KJ, van Hulst K, van den Hoogen FJ. Salivary duct ligation for anterior and posterior drooling: our experience in twenty one children. Clin Otolaryngol. 2013 Jul 3.

Case history “Rose”

Case description

Assessment

Treatment history

Current situation

Discussion

Final conclusion

Take home message

106

Case history “Sarah”

• First presented at 4 years

• Cerebral palsy GMFCS V• Poor feeding skills• No expressive language

• Good understanding• Initial treatment???

Case history “Sarah”• Re-presented at 6 years

• Some improvement in feeding skills, still drooling ++

• Ongoing speech pathology

• Being teased at school

• What next??

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19

Case history “Sarah”• Reviewed at 9 years

• Medication trialled only for a short time

• Developed adverse effects

• Still some ongoing speech pathology

• Now has communication device

• Becoming more aware of problem herself and wants it “fixed”

• Intervention??

Case history “Sarah”

• Reviewed at 17 years prior to transition

• Had surgery at 12 years

• Good result

• Dentist monitoring dental status

• Participating well in home and community life

Comprehensive Resource Book

• Saliva Control in Children

• Website:

http//:www.rch.org.au/emplibrary/plastic/salivabook

Thank you