Childh d A i fChildhood Apraxia of SpeechSpeech

Cheryl D Tierney MD MPHCheryl D. Tierney, MD, MPHAssistant Professor Of Pediatrics

Penn State Hershey Children's HospitalDepartment of Pediatrics

Division of Human Genetics, Growth and Development

There are no disclosures related to today’s

t tipresentation

Objectives•Relate key speech and language concepts

Objectives

necessary to understand Childhood Apraxia of Speech (CAS)

•Discuss evidence available to guide treatment of Childhood Apraxia of SpeechChildhood Apraxia of Speech

•Review considerations of care in the child with Childhood Apraxia of Speech

Case of Henry

Henry is a 2 1/2 year old who presents today for a WCC. Hi f il j t d d i t bli hi ith

y

His family just moved and is establishing care with you.As recommended by the AAP Practice Guidelines (2006) you administer a developmental surveillance tool andyou administer a developmental surveillance tool and review it before you enter the room. You notice mom indicates a concern with Henry’s

i ti H i i ST th h EI f 18communication. He was receiving ST through EI from 18 months but progress was noted to be slow.Because of this, mom wonders if she should try and get EI , y gstarted here or just wait and see?

Conceptual Framework: What is speech and what is language?language?

Conceptual Framework: What is speech and what is language?

Dysfluency

language?

Dysfluency

Articulation Disorder / Phonological Disorder

Childhood Apraxia of Speech

DysfluencyDysfluency

Stuttering: Interruptions in the normal flow of speechStuttering: Interruptions in the normal flow of speech

Typical •Whole word or phrase repetitions

Less Typical •Repetition of sounds •Repetitions of syllablesp

•Interjections •Revisions•Hesitations

•Repetitions of syllables •Prolongation

Etiology unknown•Hesitations Etiology unknownAges affected: 2-5 yrsOften resolves on its own Exacerbating factors include Stress, Fatigue & Language complexity

Articulation/Phonological Di dDisorder

Articulation Disorder•A person cannot produce the sounds necessary for

Articulation Disorder

correct speech.

•Predictable: Unable to make: p, b, m by 3 years Unable to make: d, n, k by 4 years Unable to make sh and th by 6 years Unable to make sh and th by 6 years Unable to make s by age 7

Phonological DisorderPhonological Disorder

Poor use of certain speech sounds expected for age

o Predictable errors, consonants more commono M > F o 3% of preschool children and 2% of children 6 - 7yr o Prognosis is generally good for normal or near-o Prognosis is generally good for normal or near-

normal speech

Childhood Apraxia of S hSpeech

Childhood Apraxia of SpeechChildhood Apraxia of Speech

Neurological speech sound disorder That affects a child’s ability to coordinate the muscles of the tongue, lips, mouth and jaw to

make accurate and reproducible speech dsounds

GeneticsForkhead Box Promoter Gene7q31, at the SPCH1

locus Promoter Gene (FOXP2)

locus

Gene Mutation or deletionleads to decreased protein productionp p

FOXP2 Protein

*

Protein

Genetics

FOXP2 is required for proper brain and lung q p p gdevelopment.

-mice studies

FOXP2 may regulate genes involved in l ti itneuroplasticity: songbird studies bat studies

Haesler S, Wada K, Nshdejan A, Morrisey EE, Lints T, Jarvis ED, Scharff C (March 2004). "FoxP2 expression in avian vocal learners and non-learners". J. Neurosci. 24 (13): 3164–75. doi:10.1523/JNEUROSCI.4369-03.2004.

Teramitsu I White SA (July 2006) "FoxP2 regulation during undirected singing in adult songbirds" J Neurosci 26 (28): 7390 4Teramitsu I, White SA (July 2006). FoxP2 regulation during undirected singing in adult songbirds . J. Neurosci. 26 (28): 7390–4. doi:10.1523/JNEUROSCI.1662-06.2006.

Genetics: Human correlates

Apraxia in humans has been linked to mutations in the FOXP2 gene.

fMRI analysis of these individuals performing silent verb generation and spoken word repetition tasksand spoken word repetition tasks showed under-activation of Broca's area and in the putamen. p

Suggestive History

•Feeding problems

gg y

•Feeding problems•Prolonged drooling •Hard time blowing bubbles•Trouble drinking through a strawTrouble drinking through a straw•Weak kisses

Distinguishing characteristicsg gPhonological Disorders CAS

Consonant omissions and substitution errors

Consonant omissions and substitution errors

common uncommon

D l ti t ifi T i l d l tiDeletions not specific to position

Terminal deletionscommon

Accuracy unrelated to # syllables in words

Accuracy reduced with longer utterances# syllables in words with longer utterances

Features• Limited babbling as an infant

Featuresg

• Delayed onset of speechRestricted sound inventory• Restricted sound inventory

• lots of words.....all with the same sound• ‘word approximations’• word approximations

Features•Error (un)predictability

V l i t•Vowel errors prominent

•Effect of increased complexityEffect of increased complexity

•Dysdiadokokinesis effect/ / / / / / / / / / /k //pa/, /pa/, /pa/ versus /pa/ /ta/ /ka/

•Disturbances of prosody•Disturbances of prosody

•Groping (age effect)

Back to Our Case:You ask some more questions and you find that she has

few words, many of which are word approximations ex da for daddy, ma for mommy, ba for bottle

She is frustrated and gets angry easily when she is notShe is frustrated and gets angry easily when she is not understood.

She is wearing a bib around her neck that you remove when you do your physical exam

• History suggests a possible speech disorder, or maybe apraxiaapraxia...

An approach: My child isn’t t lki• Identify all domains of developmental delay first

talking…• Identify all domains of developmental delay first

Refer to Early Intervention Does the profile you see fit a speech disorder?

• Refer for confirmatory testing Tx needs to be geared to the dx in terms of

frequency intensity and approachfrequency, intensity and approach• Consider the co-morbidities possible

Ex. Apraxia in children with autism• Consider alternative and augmentative

communication devices (AAC) for voice and for keeping up with learning while speech developskeeping up with learning while speech develops

Once upon a time..p

EVALUATE AND TREATEVALUATE AND TREATDX SPEECH DELAY

Developmental DomainsMotor

fine motortgross motor

speech

Cognitivevisual motorplayadaptive skillsadaptive skills

Communicationnonverbal skillsreceptive languageexpressive languagespeech social interactions

Speech and Language p g gEvaluation•Possible speech disorder r/o apraxia of speech

The EvaluationExpressive & Receptive L

The Evaluation

Language

Speech

Phonemic awareness and early literacy (for ages 4-5 and older)literacy (for ages 4-5 and older)

How Many Treatment Sessions Are Required t I M Child' S h?

Phonological disorder

to Improve My Child's Speech?

ApraxiaPhonological disorder 21-42 sessions (x 29)

Apraxia144-168 sessions(x 151)

1/2 3/4

Children with apraxia of speech required 81% more individual treatment sessions than the children with severe phonological disorders in order to achieve a similar functional outcome.

Thomas F. Campbell, "Functional Treatment Outcomes in Young Children with Motor Speech Disorders," Clinical Management of Motor Speech Disorders in Children, edited by Anthony J. Caruso and Edythe A. Strand, Thieme Medical Publishers Inc., New York, 1999.

similar functional outcome.

Treatment Evidence Summary•Although there are differences in definitions of

Treatment Evidence Summary

intensive remediation for children with apraxia, there appears to be emerging consensus within the literature that therapy should be conducted at leastliterature that therapy should be conducted at least three to five times weekly, in sessions lasting between 30 and 60 minutes each, and that thebetween 30 and 60 minutes each, and that the intervention should be conducted on an individual basis (1:1).

Penelope K. Hall, Linda S. Jordan, Donald A. Robin, Developmental Apraxia of Speech: Theory and CClinical Practice , 2nd Edition, page 200, Pro-ed Publishers, Texas, 2007.

What evidence exists to guide treatment?

• CAS over-rely on their own imprecise auditory feedback . Slowing down the rate of articulation can help correct this over

treatment?

o Slowing down the rate of articulation can help correct this over-reliance and may be a new angle for intervention [1].

• There is a lack of dissociation and decreased coordination amongThere is a lack of dissociation and decreased coordination among tongue tip, lower lip and jaw movements compared with normal age-matched peers [2]

• Therapeutic emphasis on training more difficult speech targets yields generalization to simpler sound productions[3].

[1]Ballard KJ, Robin DA, McCabe P, McDonald J. A treatment for dysprosody in childhood apraxia of speech. J Speech Lang Hear Res 2010; 53:1227-45[2]Terband H, Maassen B, van Lieshout P, Nijland L. Stability and composition of functional synergies for speech movements in children with developmental speech disorders. J Commun Disord 2010; 44:59-74.[3]Iuzzini J, Forrest K. Evaluation of a combined treatment approach for childhood apraxia of speech. Clin Linguist Phon 2009; 24:335-45..

Treatment programs availableg

•Multisensory programs work bestMultisensory programs work best.

PROMPT•PROMPT•Moving Across SyllablesW d Fli•Word Flips

•Kaufman Praxis

Apraxia's link to Literacy

Phonological Awareness is knowing that sentences are d f d d d f ll bl d

y

made up of words, words are made up of syllables and syllables are made up of sounds.

Phonemic awareness is required foro Rhymingo Manipulating sounds

"take the c in cat and put a b --- What do you get?"

Phonetics is how we learn to read

Vocabulary size is strongly related to phonological awarenessphonological awareness

*Velleman SL. Lexical and phonological development in children with childhood apraxia of speech--a commentary on Stoel-Gammon's 'Relationships between lexical and phonological development in young children' J Child Lang; 38:82 6children . J Child Lang; 38:82-6.

Lexicon phonological Lexicon p gawareness

decreased production of words and fewer vocalizations reduced

1

vocalizationspractice and feedback

decreased skill and

2

decreased skill and motivation

3

higher order literac

decreased vocabulary learning

4

literacy

What are the long term outcomes f hild ith CAS?for children with CAS?

Children with apraxia, even if they are completely remediated by school entry, are at increased risk of language based learning problems (dyslexia)language based learning problems (dyslexia)

R l ti i t d til i t dRe-evaluation is suggested until age appropriate and reading

• every 6 months under age 3 yearsevery 6 months under age 3 years• yearly from 3 onward• through early literacy years

So........

Identifying children with a speech / language delay in

So........

practice is important but only part of the story

Speech and language development are different and p g g punderstanding the differences helps to formulate an informed differential diagnosis

Diagnosis (working or confirmed) is very helpful in guiding treatment and advocating for such will only improve ultimate developmental potential for the children we serve

Back to our case….

We suspected a speech disorder. We requested an evaluation. Henry’s speech pathologist completed the PLS5 and the Kaufman to confirm normal receptive language, moderately delayed expressive language and g g , y y p g gfindings consistent with childhood apraxia of speech.

Back to our case….

He was treated 3 times per week with an evidence based treatment approach utilizing PROMPT methods by a certified and experienced therapist. He had a combination of school based and private therapy so that p pythe family could learn how to be involved and carry over the goals of therapy at home.

Henry loved using an Ipad with apraxia applications toHenry loved using an Ipad with apraxia applications to practice at home.

Back to our case…

3 ½ years later Henry returns for his kindergarten physical exam and mother delights in telling you that he is doing fabulously In fact he is already reading simpleis doing fabulously. In fact, he is already reading simple books and loves rhyming games on his mother’s Ipad.

Conclusions

Childhood apraxia of speech is a significant and not

Conclusions

uncommon speech sound disorder in children

Diagnosis can be suspected by the pediatrician and g p y pconfirmation with testing is needed by an SLP experienced in apraxia to confirm an ultimate di idiagnosis

Once diagnosed, specialized intervention, education and monitoring will be needed to help children with CAS reach their ultimate communication potential.

Thank you!!y