outcome of intensive oral motor and behavioural interventions for

7/23/2019 Outcome of Intensive Oral Motor and Behavioural Interventions For

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Outcome of intensive oral motor and behavioural interventions for

feeding difficulties in three children with Goldenhar Syndrome

E. P. CLAWSON1, K. S. PALINSKI2, & C. A. ELLIOTT1

1Childrens Hospital, Richmond, VA, USA and

2VCU Medical Center, Richmond, VA, USA

(Received 29 June 2004; accepted 18 February 2005)

AbstractObjective: The facial anomalies and surgeries associated with Goldenhar Syndrome often lead to feeding problems.The purpose of this study was to demonstrate the outcome of Goldenhar Syndrome children admitted to the day patientpaediatric feeding programme.Subjects: Three children with Goldenhar Syndrome and feeding difficulties participated, mean age 3 years. Average length

of stay was 8 weeks. Upon admission all were dependent on tube feedings.Methods: The children received four therapeutic sessions daily. The sessions included oral motor interventions, behaviouraltechniques and a structured meal. Data regarding childrens feeding were collected at admission and discharge.Results: The patients had 52% acceptance of food/drink at admission and 88.7% at discharge. Expels were 30% at admissionand 14% at discharge. Mouth clean was 9.3% at admission and 97% at discharge. Total inappropriate feeding behavioursaveraged 83% at admission and 27.3% at discharge. Children averaged 3.4 g per meal at admission and 105.4 g at discharge.Two children were completely weaned from tube feedings by discharge.Conclusions: Results support the hypothesis that combining oral motor and behavioural interventions offer effective treatmentfor children with Goldenhar Syndrome. Many children with Goldenhar Syndrome are reliant on tube feedings and thisregimen allows them to eat orally, thereby improving their nutrition and quality of life.

Keywords: Goldenhar, feeding, oral-motor, behaviour, OAVS, dysphagia

Objetivo: Las anomalas faciales y las cirugas asociadas al Sndrome de Goldenhar generalmente ocasionan problemas dealimentacion. El proposito de este estudio fue demostrar el resultado en nin os con Sndrome de Goldenhar admitidos alprograma diurno de alimentacion de pacientes pediatricos.Sujetos:Participaron tres ninos con Sndrome de Goldenhar y dificultad para la alimentacion, con un promedio de edad detres anos. El promedio de duracion de la estancia fue de ocho semanas. Antes de la admision todos eran dependientes dealimentacion por sonda.

Me todos: Los ninos recibieron cuatro sesiones terapeuticas diariamente. Las sesiones incluyeron intervenciones motorasorales, tecnicas de comportamiento y una comida estructurada. Los datos en relacion a la alimentacion de los ninos fueronrecolectados al ingreso y al egreso.Resultados: Los pacientes tenan al momento de la admision un 52% de aceptacion de la comida/bebida, y un 88.77% alegreso. Las expulsiones fueron de 30% a la admision y 14% al egreso. La boca limpia fue de 9.3% a la admision y de 97% alegreso. El total de los comportamientos de alimentacion inapropiados tuvo un promedio de 83% a la admision y un 27.3%al egreso. Los ninos tuvieron un promedio de 3.4 gramos por alimento en la admisio n y de 105.4 gramos al egreso. Dosninos fueron separados completamente de las sondas de alimentacion al egreso.Conclusiones: Los resultados apoyan la hipotesis de que la combinacion de las intervenciones motoras orales y decomportamiento, ofrecen un tratamiento efectivo para los ninos con Sndrome de Goldenhar. Muchos ninos con Sndromede Goldenhar dependen de sondas de alimentacion, y este regimen les permite alimentarse por va oral, y por lo tantomejorar su nutricion y su calidad de vida.

Introduction

Goldenhar Syndrome is a constellation of

deformities of the head and neck. The incidence

of this syndrome is 1/30001/5000 live births,

with a male-to-female predominance of 3 : 2 [15].

Facial anomalies associated with Goldenhar

Syndrome include ear abnormalities, periauricular

tags, anotia, mandibular hypoplasia, orbital

dystopia, cervical spine anomalies and ocular

Correspondence: Elizabeth P. Clawson, PhD, LCP, Pediatric Feeding Program, 2924 Brook Road, Childrens Hospital, Richmond, VA 23220, USA.

Tel: (804) 228-5878. Fax: (804) 228-5989. E-mail: [email protected]

Pediatric Rehabilitation, January 2006; 9(1): 6575

ISSN 13638491 print/ISSN 14645270 online/06/010065-75

2006 Taylor & FrancisDOI: 10.1080/13638490500144809


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dermoids. Cranial nerve palsies as well as multiple

central nervous system anomalies can also be

present. Up to 70% of these anomalies are asym-

metric and unilateral. During speech and swal-

lowing, the movement of the affected side of the

oropharynx and larynx is decreased in comparison

to the unaffected side. In children with bilateralpresentations, movement of both sides is decreased

or absent [1]. Children with Goldenhar Syndrome

often have associated cleft lip and palate, which,

along with their small mandibles, often require

surgery [6,7]. There can also be anomalies in the

structure and function of the tongue [4].

The facial anomalies associated with Goldenhar

Syndrome and the surgery these children often go

through lead to multiple feeding problems in chil-

dren with this disorder. Feeding difficulties related

to facial anomalies can include an under-developed

maxilla or mandile, hemifacial microsomia, high

arched palate, facial palsy, malocclusion, cleft lip

and/or cleft palate, crammed teeth and irregularly

developed pharynx, palate or tongue [8]. These

children often suffer from frequent regurgitation,

swallowing of air and aspiration, which can result in

failure to thrive and pneumonia [7].

Another symptom of Goldenhar Syndrome that

can lead to feeding problems is a decrease in

muscle tone. In a study of patients with Goldenhar

Syndrome, Cohen et al. [9] found 46% of patients

with decreased muscle tone in the oral-facial

musculature. Eighty-two per cent of these patients

had feeding disturbances, swallowing disruption orfailure to thrive. In this study, Cohen et al. suggested

a strong association between oral-facial hypotonia

and feeding difficulties. Yokochi et al. [7] stated that,

although some infants with this syndrome, especially

those having cleft palate, have been empirically

known to have feeding difficulty, this frequency had

not been systematically studied. This study also

concluded that treatment for refusal of oral intake

caused by prolonged tube feeding may also be

necessary in some patients.

Due to the array of facial malformations in

Goldenhar Syndrome, there are various dental and

surgical procedures that restructure the muscles and

bones of the face. These procedures can help

structurally enhance feeding potential. However,

children who undergo multiple craniofacial surgeries

can develop oral motor problems, feeding problemsand oral aversions. Once the physical problems are

resolved and the surgeries are completed, the oral

motor problems are often still present. These feeding

difficulties and learned cycles of oral aversion serve as

barriers to transitioning to oral feedings [6]. By using

a behavioural approach to decrease oral aversion, the

compliance with therapy increases and outcomes are

enhanced [10]. However, there is a lack of research

pertaining to specific feeding interventions for chil-

dren with Goldenhar Syndrome. More research is

needed to determine the effectiveness of oral and

behaviourally based feeding programmes with medi-

cally complex children. The purpose of this studyis to discuss the effectiveness of a comprehensive

behavioural based feeding programme to improve the

feeding abilities of three children with Goldenhar

Syndrome.

Methods

Participants and setting

Three children with Goldenhar Syndrome and

feeding difficulties participated in this study. Names

have been changed to provide patient confidentiality.

Patient characteristics are outlined in Table I.

The mean age of these children was 36 months

old (range1542 months). The average length of

stay was 37 treatment days (range2844 days).

Upon admission, all children were dependent on

feeding tubes (two gastrostomy and one jejunost-

omy) and had no significant oral intake. All children

had participated in ongoing outpatient therapy to

address feeding difficulties. Parents reported no

significant improvements with early intervention

services provided by general paediatric practitioners.

Table I. Characteristics of the three patients with Goldenhar Syndrome included in this study.

Patient characteristic Susan Kelly John

Age at admission to feeding programme 15 months 42 months 38 months

Gestation at birth 39 weeks 24 weeks 38 weeks

Type of tube feeding Gastrostomy Gastrostomy Jejunostomy

Gastroesophageal reflux Yes No Yes

Vision difficulties None Dwayne syndrome Blind

Hearing loss Yes, severe Yes, mild Yes, severe

History of cleft lip and palate No Yes Yes

Vertebral problems Sacral agenesis Clefting lower thoracic spine Scoliosis

Tracheostomy No No Yes

Recurrent otitis media Yes Yes Yes

66 E. P. Clawson et al.


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The medical issues of the children were treated as

optimally as possible prior to being admitted to this

programme.

Case 1. Susan was a 15-month-old girl with

gastrostomy tube (G-tube) dependency and whose

Goldenhar Syndrome manifestations include severe

hearing loss requiring bilateral hearing aids, sacral

agenesis, Arnold Chiari malformation, vesicourethral

reflux, laryngomalacia and multiple episodes of otitis

media. She was born near term (39 weeks gestation)

weighing 2.98 kg. Susans surgical history included

the placement of her G-tube and a Nissen fundopli-

cation. Susan primarily used sign language to

communicate. She was allergic to milk products.

Upper endoscopy demonstrated aphthus ulcera-

tions in her oesophagus and colonoscopy done on

the same day revealed lymphonodular hypoplasia,

indicative of milk/protein intolerance. A gastricemptying scan showed that Susans gastric emptying

was delayed by 120 minutes. Upon arrival to the

feeding programme, Susans medications included

Cefaclor for prophylactic treatment of otitis

media, Omeprazole, for gastroesophageal reflux

and retching, and liquid glycerin enemas daily

for chronic constipation. Susan had mild gross

motor skill delays. At the time of admission,

Susans feeding behaviours included food refusal,

expelling, gagging and retching. She typically pushed

her spoon away, would not open her mouth and

turned her head away when presented with food.

She was receiving 100% of her nutritional needsvia G-tube. A videofluoroscopic swallow study done

prior to admission to the day treatment programme

was within normal limits. Findings were unchanged

during a repeat study done during her admission.

Case 2. Kelly was a 42-month-old girl with G-tube

dependency and whose Goldenhar Syndrome

manifestations included an incomplete cleft lip and

nose, a cleft palate, facial abnormalities including

small mandible, small oral cavity, high palate,

abnormal tongue and periauricular skin tags. She also

had mild hearing loss, clefting of the lower thoracic

spine, Dwaynes Syndrome affecting her eyes, peri-

ventricular leukomalacia and lipoma of the brain.

Kelly was 8 months behind developmentally and

suffered from recurrent otitis media. Kelly was a twin

birth at 24 weeks, weighing 530 g. She had retino-

pathy of prematurity with vision loss and wore

corrective glasses. Kelly also had a small oral cavity,

with an abnormally shaped tongue that deviated to

the right. Kellys past surgical history included laser

surgery for retinopathy of prematurity, gastrostomy

with Nissen fundoplication, left arm z-plasty for

amniotic bands, small bowel lysis of adhesions,

costochodral rib graft to her jaw, jaw distraction

surgery, soft palate closure, cleft lip repair, tongue

clipping, tear duct probe/tube surgery, epibulbar

dermoid removal from left eye and periauricular tag

removal. Kellys only medication was Glycopyrrolat,

to decrease oral secretions. At the time of admission,Kelly was receiving 100% of her caloric needs via G-

tube. During the baseline sessions, Kelly was capable

of consuming 2% of her recommended calories by

mouth. She typically refused food and would pack

(holding food for greater than 30 seconds) and expel

when she did accept. She did not actively swallow

any bites at admission. Prior to admission,

a videofluoroscopic swallow study was performed

using a syringe that demonstrated adequate airway

protection; however, there was trace nasal regurgita-

tion, no bolus control, pooling in the anterior floor,

no lip closure and no true swallows were elicited.

Case 3. John was a 38-month-old boy with

tracheostomy tube, G-tube and jejunostomy tube

( J-tube) dependency. Johns Goldenhar Syndrome

characteristics included jaw deformity, unilateral

cleft lip and palate, bilateral microphthalmia with

blindness, unilateral atresia of the right ear and

abnormal cochlea on the left, resulting in profound

deafness, tethered tongue, hemifacial microsomia,

oral pharyngeal inco-ordination with aspiration and

scoliosis. Additional diagnoses included oral aver-

sion, oral dysphagia and gastroesophageal reflux.John suffered from the Prosanski 3 defect, which

resulted in an unstable airway, for which he received

immediate intubation at birth and a tracheostomy

at 1 week old. John was born via ceasarian section

secondary to pregnancy-induced hypertension at

38 weeks gestation; he weighed 2.52 kg. Johns

past surgical history included unilateral cleft lip

and palate repair, myringotomy tubes, right-

sided bone arachnoid hearing aid (BAHA), Nissen

fundoplication with gastrostomy, Rouxen-Y

jejunostomy and rib graft to jaw. He aspirated

thin liquids on his most recent videofluoroscopic

swallow study done prior to admission; however,he safely swallowed thickened liquids and puree.

John was on day 4 of 10 of Amoxicillin/Clavulanate

Potassium for a left ear infection and Ranitidine

for reflux prophylaxis when he was admitted to

the feeding programme. He had no known drug or

food allergies. Upon admission, John was receiving

100% of his nutrition through his J-tube; his G-tube

was used for venting. He refused foods presented

by mouth and had a history of vomiting when G-tube

feedings had been tried in the past.

Behavioural interventions for feeding difficulties 67


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General procedure including data collection

Admission. Children were referred to the feeding

programme by their general paediatrician secondary

to feeding difficulties. An inter-disciplinary team,

including a paediatric gastroenterologist, a nurse

practitioner, a behavioural psychologist and a nutri-

tionist evaluated the children. The initial evaluationincluded a general health assessment, nutritional

evaluation and feeding assessment. These three

children were all placed in the intensive day feeding

programme. The day treatment programme was

a regimen of four meals per day, 5 days per week,

designed for children with moderate-to-severe

feeding problems who were medically stable.

Day programme. The day feeding programme was

carried out by an inter-disciplinary team includ-

ing a paediatric gastroenterologist, paediatric nurse

practitioner, behavioural psychologist, occupationaltherapist, speech-language pathologist, feeding

technicians, registered dieticians, diet technician,

nursing staff, licensed clinical social worker and

case manager. Patients attended the programme

MondayFriday for 6 hours and were provided

four therapeutic meals each day. Each therapeutic

meal included oral motor exercises, followed by oral

feeding. Weekly inter-disciplinary team meetings

were held to review feeding data, calories consumed,

adjustments to tube feedings and the treatment

plan for the coming week.

Baseline sessions. Upon admission to the pro-

gramme, anthropometric data including height

and weight were collected along with calculation of

average calories consumed and ideal body weight.

Caregivers were instructed to continue the same

schedule of tube feeds. For the first 2 days of the

admission, the parent and the therapist fed the child

without using any therapeutic intervention strategies

to determine the childs skills and amount they

were able to consume during a meal. An oral motor

assessment determined what functional, emerging

and non-productive patterns were present. A rein-

forcer evaluation was conducted to identify toysand videos that could be used as motivators during

meals. Positioning was assessed and adjusted to

provide optimal postural stability during meals.

Intervention. The independent variable in this study

was a multi-component package that was made up

of four main components. These included oral

motor exercises, structured meals, behavioural inter-

ventions and caregiver training. Oral motor exercises

were performed for 1520 minutes prior to each

oral feeding. These interventions provide assisted

movement to stimulate muscle contraction and

facilitate movement against resistance to build

strength [11]. The goal of these interventions was

to increase functional response to pressure and

movement, specifically increasing the range,

strength, variety and control of movement for the

lips, cheeks, jaw and tongue.Meals were conducted by the same primary

therapist (speech or occupational therapist) and

feeding technician throughout the admission. All

feeders are trained to greater than 80% reliability

with all data measured prior to independently work-

ing with patients. Meal length was determined

according to skill level and endurance. An audible

timer was used to indicate the end of the meal

sessions. Behavioural interventions included differ-

ential attention and prolonged presentation of food

to the childs lips until the child opened and accepted

the bite into their mouth. Preferred toys selected

by the individual reinforcement assessments, videosand verbal praise were used to reward appropriate

feeding behaviours (e.g. accepting food, chewing

and swallowing). Reinforcement was withheld for

negative behaviours such as refusal to take the bite,

expelling food and packing. Toys were returned

and verbal praise given when appropriate behaviour

resumed. Other inappropriate behaviours such as

crying and gagging were treated via extinction and

removal of social attention. This feeding protocol

was carried out as part of each therapeutic meal.

Parent training. All caregivers observed the treat-

ment sessions via video monitor outside the treat-

ment room. Psychologists and social workers

accompanied parents at the viewing stations to

educate as well as address questions and concerns.

Once the child was tolerant of oral motor exercises

with the therapist, parental instruction began.

Parents were instructed using verbal, written and

hands-on demonstration in the implementation

of the prescribed exercises. Prior to initiating

home-based meals, training was also provided in

food preparation and calorie boosting (e.g. puree,

texture grading, food allergies) specific to the childsneeds. At 2 weeks prior to discharge, caregivers

began to be faded into the treatment sessions.

Caregivers received training in handling, facilitation

techniques, use of adaptive feeding utensils, as well

as the three behavioural components of the treatment

protocols: instructions, prompts and consequences

(IPC). They learned and practiced single compo-

nents of the treatment and did not progress to the

next level of training until they had mastered the skill

at or above 80% accuracy. Finally, the caregiver fed

the child independently while the therapist observed



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from a video monitor and provided instruction as

needed via a wireless communication system.

Measures

Outcome measures of anthropometric data (weight

and height) were collected at admission and dis-charge from the programme. Feeding data were also

collected based on percentage of bites accepted,

average length of meal, grams taken per meal,

percentage of bites expelled, total inappropriate

behaviours and IPC at baseline sessions and the

week prior to discharge from the programme.

Child feeding behaviours were recorded for every

feeding session. Positive feeding behaviours were as

follows: (a) an accept was recorded each time the

child opened his/her mouth and allowed the food

or liquid to be placed in the mouth when it was

presented or touched to the lips within 5 seconds of

the presentation; (b) mouth clean was scored by the

absence of food or drink, larger that 1/4 square

inch, in the childs mouth following the accep-

tance of a bite and before 30 seconds had elapsed.

Negative feeding behaviours were defined as follows:

(a) expels included any food/drink that was in the

mouth and came back out past the border of the lips,

(b) total inappropriate behaviours included the total

number of bites in which negative feeding behaviour

occurred (i.e. refusing to take a bite, expelling,

packing, gagging or emesis).

The nutritional information collected included

calories consumed by mouth per treatment day andthe average grams consumed per meal. Food

was weighed prior to and following each meal;

total grams consumed per meal was the pre-

weight minus the post-weight of all the foods. The

difference scores of all foods were added together,

spilled food and emesis (post-meal bib weight

minus the initial bib weight) were then subtracted

from the total grams to obtain a measure of actual

grams of food consumed. The average calories per

treatment day was a calorie count based on the

gram weight of the foods consumed throughout the

treatment day. Percentage of calories by mouth was

the percentage of their recommended daily intakethat was consumed by mouth.

Weight was measured in kilograms using an infant

Health-O-Meter bucket scale. Height was measured

in centimetres. The patients weight and height were

taken at admission and discharge, as well as twice

per week while admitted to the programme.

Specific procedure

The basic behavioural components for each of the

treatment protocols used with the patients are

described below and are outlined in Table II.

Case 1. The focus of Susans therapy was for her

to learn to accept food readily with an open mouth,

increase her skills of lip closure and decrease expels.

Susans treatment began under Protocol 1, which

included continuous tangible reinforcement and

social praise for accepts, prolonged presentations

and extinction for all other inappropriate behaviours.

The therapist had difficulty offering immediate

reinforcement while providing hands on facilitation

for lip closure. As a result, on the 4th day of treat-

ment, Susans reinforcement schedule was switched

to Protocol 2, which included continuous access to

tangible and social reinforcement with cost response

for interrupts. She was fed using a squeeze bottle for

both formula and baby food. The squeeze bottle was

a soft plastic container with a flexible clear plastic

straw that, when pressure was applied, the liquid was

expressed. She began with a 1/8 inch diameter straw.

Significant emphasis was placed on supporting the

chin, inhibiting retraction and facilitating lip closure.Susans mother was trained in oral motor exercises

and she performed these on Susan prior to each meal

under the supervision of a therapist. During week 3,

skill level had improved to greater than 80% for

accepts and showed and intermittent ability to accept

without expelling. Protocol 3, which added response

cost for expels, was added with re-presentation of the

bite. During week 4, pureed table food presented on

a spoon was begun. Following an increased number

of interrupts, the reinforcement schedule was chan-

ged back to continuous reinforcement, while main-

taining cost response for expels and re-presentation

(Protocol 4). During week 5, the mother was trainedin the feeding protocol, preparation of the pureed

Table II. Basic behavioural components for each of the treatment protocols used with the patients in this study.

Treatment protocols Protocol description

Protocol 1 Continuous tangible reinforcement and social praise for accepts, prolonged presentations and extinction

for all other inappropriate behaviours.

Protocol 2 Continuous access to tangible and social reinforcement, cost response for interrupts, prolonged presentation

and extinction for all other inappropriate behaviours.

Protocol 3 Continuous access to reinforcement with cost response for interrupts and expels. Expels re-presented.

Protocol 4 Continuous reinforcement, cost response for expels. Expels re-presented.



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table food and was transitioned in as the primary

feeder. A large straw squeeze bottle (3/8 inch

diameter) was also introduced during this week.

During week 6, the mother fed all meals under direct

supervision of the therapist. Various cups were tested

with little success and the squeeze bottle was

continued for liquid intake. A larger bowl spoonwas introduced and tolerated. By the end of week 6,

Susan was consuming all of her caloric needs via

pureed table food and formula by mouth. During

week 7, Susans mother fed her for 1 day and then

her father was introduced as the feeder and fed for 3

days. Upon parent request, Susans treatment pro-

tocol was changed back to Protocol 3, continuous

access with response cost and re-presentation. The

parents felt this would allow for better generalization

to different settings and increased ease for feeding.

Susans parents were able to follow the protocol with

98% accuracy at the time of discharge.

Case 2. The focus of Kellys treatment was to

increase her acceptance of food by mouth and dev-

elop muscle control to form a bolus and co-ordinate

a swallow. During week 1, Kellys treatment began

using Protocol 1 and oral motor exercises. She was

presented with thickened milk in a squeeze bottle

with a 1/8 inch diameter straw. The liquid was

presented centrally with her head slightly extended to

keep the drink from running out of her mouth.

After accepting the drink, Kelly was cued to bring

her chin down in a slightly flexed position. She was

provided with physical and verbal cues to keep herlips closed and elevate her tongue within her mouth

to initiate a swallow. Her meals lasted 12 minutes at

the start of treatment due to fatigue. During week 2,

Kellys protocol was changed to provide continuous

reinforcement following three facilitated swallows,

because she was too distracted by the television to

be able to work on her swallowing while watching.

During week 3, she was introduced to smooth

solids off a spoon for two out of four meals per day.

Her mother was trained in oral motor exercises and

performed them, under the supervision of a thera-

pist, prior to each meal. During week 4, Kellys

endurance improved and the meal duration

increased to 19 minutes. Her mother was trained in

the feeding protocol and preparation of the pureed

table food. Her mother was then faded in as the

feeder on the last day of week 4 in order to begin

feedings at home over the weekend. During week 5,

Kelly was ill with an ear infection and, as a result,

began having difficulty managing large boluses and

textures. Throughout the week, Kelly demonstrated

increasing levels of packing, particularly with thick-

ened purees. As a result, yogurt was introduced with

success and, by week 7, became part of all meals.

Kellys local speech pathologist attended 1 day of

training during week 7 and was taught the oral motor

exercises and Kellys feeding protocol. Kelly began

producing more mucous this week and at times the

meal needed to be stopped to allow her to expel the

mucous and recover from related gagging. Milk

products were eliminated in an attempt to decreasemucous production. During week 8, Kellys treat-

ment protocol was changed due to a spike in negative

feeding behaviours as her mother was faded in as the

primary feeder. The change included continuous

reinforcement with the addition of pennies and a

musical timer to target packing and expels. Kellys

father was also faded in as a feeder and was trained

in all oral motor exercises and the feeding protocol

during week 9. Both parents had difficulty manip-

ulating the squeeze bottle to provide adequate

volume; therefore, instruction continued to achieve

the goal of 45 g of liquid per presentation. By

discharge, Kellys parents were able to feed herindependently with 89% IPC accuracy while the

therapist observed from outside the room. During

the last week, Kelly demonstrated significant

decrease in mucous production and improvement

in positive feeding behaviours.

Case 3. Johns goals were to learn to accept food

readily with an open mouth, decrease his gagging

and vomiting and improve his oral motor abilities.

At baseline, John refused all bites of food/drink

and he demonstrated severe defensiveness to the oral

motor exercises. John was started on Protocol 1, withcontinuous reinforcement utilized to complete

the oral motor exercises. Yogurt and Pediasure in

large-size straw squeeze bottles were added into

the sessions by the 3rd day of week 1. Placement

was determined to be best on the right side with

lip support. During week 2, Johns mother was

instructed on performing the oral motor exercises

resulting in a dramatic increase in Johns com-

pliance with the exercises. His mother performed

exercises before each meal with therapist supervision.

Johns liquids were thickened to a nectar consistency

to promote improved bolus control. John started

the negative behaviour of turning his head during

week 3. To decrease the refusals, he was given

a verbal prompt to keep his head still, face the feeder

and prolonged presentation was used until he did

not turn away. Following a noticeable increase in

secretions, John began to be regularly suctioned

before and after each meal as well as during the meal

if necessary. An empty spoon was introduced during

the oral motor exercises, but John did not tolerate

it well and demonstrated multiple negative behav-

iours. During week 4, Johns tolerance of the spoon

increased and yogurt was introduced on the spoon



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with a change in treatment to re-present expels

(Protocol 4). Johns parents were instructed in the

feeding protocol and began to give the instructions,

prompts and consequences as the therapist fed him.

During week 5, his parents began feeding during the

sessions. He was changed to Protocol 2 for increased

ease of caregivers feeding, which allowed John tokeep the toy and have it removed only when he did

not accept the bite. John had a repeat videofluoro-

scopic swallow study which indicated no aspiration

on thin liquids, thickened liquids or smooth puree

foods. His swallow was both safe and efficient to

continue increasing his oral intake. Caregivers were

trained in food preparation and calorie boosting.

During week 6, one of Johns primary nurses was

trained in all aspects of the feeding protocol and oral

motor exercises and fed him with direct supervision.

Throughout the week he had been demonstrating

symptoms of a respiratory virus as well as an increase

in negative feeding behaviours during meals.John was sent home on day 3 of week 6, after being

diagnosed with a severe ear infection. He returned

4 days later, at which time his family came in

for a half day to complete discharge training.

By discharge, Johns parents were able to feed him

independently with 98% IPC accuracy.

Results

All three children were 100% dependent on tube

feedings at admission. During baseline sessions,

they accepted food/drink an average of 52% of the

time. This improved to 88.7% at discharge. Expels

averaged 30% at admission and decreased to 14%

at discharge. Mouth clean (swallowing) improved

10-fold from an average of 9.3% at admission to

97% at discharge. Total inappropriate feeding behav-

iours averaged 83% at admission and were 27.3% at

discharge. Figures 14 demonstrate each individual

childs improvement from admission to discharge

in accepts (Figure 1), expels (Figure 2), mouth clean(Figure 3) and total inappropriate behaviour

(Figure 4).

Upon admission, the children were taking an

average of 3.4 g per meal, which increased to

105.4 g per meal at discharge (Figure 5). Similarly,

calories averaged 12.1 kcal per treatment day at

admission and improved to 601.3 kcal per day at

discharge. All children were 100% dependent on

tube feedings for nutrition at admission. This

decreased as eating by mouth improved and upon

discharge tube feeding was only required for 25%

of their nutrition. Two patients, Susan and Kelly,

progressed to eating 100% by mouth and did notrequire any nutritional supplementation via gastro-

stomy tube at discharge.

Susan and Kelly continued to be in contact with

the programme staff following discharge; however,

Johns family did not maintain communication.

Susan and Kelly did not have any hospitalizations

for aspiration or recurrent respiratory infection

following discharge. Susan continues to consume

all of her calories by mouth and her gastrostomy

tube was removed. Kelly underwent additional

craniofacial surgery and consumes the majority of

her calories by mouth; however, requires occasional

gastrostomy supplementation depending on her oral

intake during the day.

62%

94%

30%

74%

66%

98%

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Admission Discharge

Percen

tA

ccep

ts

Susan

John

Kelly

Figure 1. Average percent accepts at admission and discharge.



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Case 1

Accepts, expels and mouth clean were the primary

targets for Susans treatment. Susans accepts

(Figure 1) improved to 100% after 1 week of

treatment. Accepts averaged at 98% throughout

her admission, however this dropped slightly to

94% at discharge when her parents were faded in

as the feeders. After accepts had improved, expels

(Figure 2) and mouth clean (Figure 3) were the

next targeted behaviours. With the initiation of

Protocol 3, swallowing increased from 57% to

greater than 95%. At discharge, Susan swallowed

100% of bites within 5 seconds of presentation.

Expels were addressed following the marked

improvement in mouth clean. The change in

reinforcement schedule associated with Protocol 4

brought expels down to 33% by week 4 and

ultimately to 6% upon discharge. Susans total

inappropriate behaviours (Figure 4) remained

greater than 80% until expels and mouth cleans

18%

100%

7%

96%

3%

95%

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Admission Discharge

Percen

tMou

thClea

n(Swa

llow

ing

)

Susan

John

Kelly

Figure 3. Average percent mouth clean at admission and discharge.

45%

6%

13% 15%

32%

21%

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Admission Discharge

Percen

tExpe

ls

Susan

John

Kelly

Figure 2. Average percent expels at admission and discharge.



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were more directly addressed with Protocol 3 and

4. With the introduction of these two protocols,

inappropriate behaviours decreased to 42%. As skills

continued to be refined, inappropriate behaviours

continued to fade and were noted at only 13% by the

time of discharge. Volume and calories consumed(Figure 5) increased steadily throughout the course

of treatment. This allowed for less frequent tube

feedings and resulted in Susan taking 100% of her

nutritional needs by mouth at discharge.

Case 2

The use of behavioural techniques to teach oral

motor skill was the primary focus of Kellys

admission. With the initiation of oral motor

exercises, facilitation techniques, verbal cueing and

reinforcing skill development, Kelly demonstrated an

improvement in co-ordination of oral motor muscu-

lature. This was shown in a repeat videofluoroscopic

swallow study toward the end of admission, which

demonstrated that Kelly was able to obtain lip

closure, collect the bolus with her tongue and

propel it posteriorly, triggering a normal pharyngealswallow in a timely manner. No nasal regurgitation

was noted in this repeat study.

Packing and expels were the primary targeted

behaviours of Kellys treatment. Kellys accepts

(Figure 1) improved immediately from 66% to

99% and stayed high (98% at discharge) with just

the initiation of a reinforcement schedule and the

structured meal. Packing developed once Kelly

began accepting food into her mouth. Initially,

Kelly did not have adequate oral motor skills to

manipulate the bolus and was only able to clean her

1.5

154

0.7

47.7

8

114.4

0

20

40

60

80

100

120

140

160

180

200

Admission

TotalGramsPerMeal

Susan

John

Kelly

Discharge

Figure 5. Average total grams per meal at admission and discharge.

91%

13%

65%

41%

93%

28%

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Admission Discharge

Percen

tInapprop

ria

teBe

hav

ior

Susan

JohnKelly

Figure 4. Average percent inappropriate behavior at admission and discharge.



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mouth 3% of the time (Figure 3). Mouth clean

steadily improved as oral motor skills developed. A

final significant improvement was noted during

weeks 89, with a shift in protocol that targeted

swallowing latency and reinforcement for clean

mouth. This resulted in mouth clean at 95% upon

discharge. Expels (Figure 2) dropped from 32%at baseline to 10% by week 8; however, this rose

to 21% at discharge due to parents learning

how to manipulate the squeeze bottle correctly.

Inappropriate behaviours (Figure 4) were 93% at

admission and steadily declined as skills improved

and were noted at 28% by discharge. Volume and

calories consumed by mouth steadily improved

throughout the course of treatment (Figure 5). At

discharge, Kelly met 100% of her nutritional needs

by mouth.

Case 3

At baseline, John refused all bites of food or drink

and he demonstrated severe defensiveness to the oral

motor exercises. Johns accepts (Figure 1) reached

92% within the first week of admission and remained

high through the course of his treatment until his

discharge. As a result of illness, accepts dropped

from 91% to 74% during his last week of admission.

Mouth clean was addressed after a favourable

response in accepts was noted. By week 2, mouth

clean (Figure 3) improved to 88% and gradually rose

to 96% at discharge. Expels did not require direct

intervention until the spoon was introduced in week

4 and expels rose to 25%. With a change in protocol

and development of skills to eat from a spoon, the

incident of expels decreased and remained at 15%

upon discharge (Figure 2). Johns total inappropriate

(Figure 4) behaviours went down to 25% immedi-

ately following the introduction of a structured

reinforcement strategy for accepts. He had a flare

up at week 4 with the introduction of the spoon.

However, with the implementation of Protocol 4,

total inappropriate behaviours went down to 27%.

During the last week of admission, John was ill and

his inappropriate behaviours increased to 41% due toa rise in refusals and a decrease in accepts. At

baseline, John ate less than 1 g per meal (Figure 5).

By week 2, he was up to 36 g per meal by mouth,

which steadily increased to 67 g by week 5 with the

change in reinforcement schedule. With the onset of

illness, including vomiting, total grams consumed at

discharge decreased to 48 g per meal. Johns tube

feeds were adjusted with the increase in oral feeds

to 25% by mouth at week 5. Johns tube feeds had

to be increased to 99% of his daily intake at discharge

due to his illness.

Discussion

The facial anomalies that most children with

Goldenhar Syndrome demonstrate often interfere

with the ability to consume adequate nutrition

by mouth. Subsequently, many of these children

are reliant upon tube feedings for nutrition. The

results of this study demonstrate that a consistent,structured model with increased frequency

and intensity of interventions was effective.

Simultaneous use of therapeutic handling/facilitation

techniques, appropriate positioning, consistent appli-

cation of oral motor exercises and behavioural

interventions to enhance skill development enabled

these children to progress when all other treatment

interventions had failed.

The data for all three children demonstrated

significant improvements in accepting and swallow-

ing food. Improved skill for oral management of the

food/drink bolus led to significant decreases inexpels, packing and total inappropriate behaviours.

Children with this level of medical complexity

require the use of behavioural strategies to overcome

their fears and negative behaviours as well as

reinforce them as they develop appropriate skills.

Therapy that provides repetitive, simultaneous use of

therapeutic tools is essential for measurable progress.

Great success was achieved with increased strength,

range of motion and co-ordination of oral motor

musculature as a result of the consistent oral motor

exercises.

Children with craniofacial anomalies can suffer

from multiple health and psychological issues thatnegatively affect their quality of life and that of their

parents and families [12]. Eating with peers is a

social norm. Not being able to eat food by mouth

not only affects nutrition, but also makes these

children different from their peers in yet another

way. These results show that it is possible to help

children with Goldenhar Syndrome eat a significant

part of their daily nutrition by mouth. This ability

to eat with peers is likely to improve their quality

of life and independence in both child-care and

school environments. Parents felt that oral feeding

improved their childs quality of life. They were nowable to participate in the family mealtime as well as

other social settings. While the food preparation and

20 minute structured meals 35 times per day can be

time-consuming for families, parents reported post-

discharge that the benefits of watching their child

eat by mouth far outweighed the caregiver burden.

Following discharge, caregivers modified the meal

length and frequency to minimize the impact on the

family schedule. Further research could be done

in this area to assess quality of life in oral feeders

vs tube-fed children in the school environment.



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There were a few limitations to this study. This

is a case study, which by definition cannot be as

strong as a double-blinded study or a study with

a considerably larger cohort. There were only

three patients in this study and one of the children

became ill prior to concluding the programme.

These children all travelled a long distance tothe institution for treatment and, therefore, received

follow-up care from their local physicians, giving

minimal follow-up data. Further analysis of more

children with Goldenhar Syndrome would indeed

be advantageous. While a double blind study is often

desirable, this is not possible in a situation where oral

motor exercises are extremely important.

In conclusion, many of these children require

tube feedings to achieve their daily nutritional goals.

The three children in this study were all 100%

dependent upon tube feedings for nutrition upon

admission to the day programme. Two of them were

able to consume 100% of their daily nutritionalrequirements by mouth by the time they were

discharged from the programme, with no supple-

mentation through their tubes. The ability of this

combined treatment regimen to have such success

in these two children should be a source of great

hope and further research for the oral feeding of

many children with facial abnormalities and tube

dependency.

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outcome of intensive oral motor and behavioural interventions for

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