Speech and language in PWS

Children with PWS may have the same range of disorders of speech and language development that can occur in any child. There is also the possibility of additional problems associated with the syndrome.

Note: Words in bold black type are explained in the glossary at the end of the leaflet.

These may be caused by:

• Learning disabilities e.g. retrieval of information and memory deficits.
• Physiological characteristics e.g. high arched palate causing inaccurate articulation.
• Low muscle tone making it difficult to make the fast accurate movements required for clear speech

Most people with the syndrome do have some difficulty with speech and/or language at some time during their lives.

 

Difficulties associated with PWS

 

Eating skills


In the early stages of the syndrome hypotonia is the most noticeable feature. This can make sucking and early feeding extremely effortful. Advice may be needed on appropriate stimulation of the baby’s sucking reflex, positioning the baby to enable him to swallow more easily, and external stimulation of the oral muscles. There can be delay in development of chewing and swallowing, sometimes causing choking. Professional assessment and advice from a suitably qualified speech and language therapist (SLT) is therefore advisable.

 

Articulation


Inaccuracy of movements and inability to change quickly from one tongue or lip position to another (sometimes described as dysarthria) are often features of PWS. This is caused by the combination of the characteristic high arched palate and the small lower jaw. Hypotonia in the oral muscles can also contribute. More rarely dyspraxia occurs, this condition leads to extremely poor intelligibility.

 

Fluency


Most non-PWS children experience a stage of non-fluency, usually between the ages of 3 and 5 but for the child with PWS it can be later occurring and last longer. Occasionally it can develop into a true stammer, but this would probably have been present if the child had not had PWS as there is a strong genetic component in the incidence of stammering within families. Some children with PWS are unusually fluent.

 

Language Delay

 
Language delay will often occur, with verbal comprehension being better than expressive language. This can lead to extreme frustration and exacerbate temper episodes if the child knows what they want but cannot put it into the right words.  Early stimulation at the appropriate level for the child’s understanding is essential for him to maximise skills in this area, and the use of signing to aid development of comprehension is often a valuable technique suggested by SLTs. The discrepancy between receptive and expressive language can cause frustration at school if the child has learnt something but cannot demonstrate their knowledge by answering questions.

 

Language Disorder


More rarely a specific language learning difficulty exists.  The child may not be able to understand language adequately, leading to confusion, frustration and difficulty learning. This may be mistaken for a learning disability, but only collaboration between an SLT and an educational psychologist can determine the origin of the problem.

Some children may experience difficulty with expressive language, with their verbal comprehension more intact, this is similar to a language delay, but more pervasive and persistent in nature, so that the ability to express ideas and comment on events does not develop along expected lines.

 

Pragmatic Disorder


Pragmatic skills concern the child’s ability to use language appropriately in social situations; this can cause a significant barrier to learning if not dealt with.

Problems in development of these skills are less easy to spot and to diagnose, and may be thought to be behavioural. The children will talk a lot, but the language is inappropriate and social interaction, conversation and turn-taking skills are affected, making it harder to form relationships and friendships. These children are often not referred to SLT service because they can speak, although SLTs are the appropriate professionals to give advice and support in this situation.

 

Repetitive use of language (Perseveration)

 
 This is the term used for the habitual discussion of one topic, often associated with an obsession with food. It may take the form of the constant repetition of a question, even after an answer has been given several times. This is extremely difficult for people who are not familiar with the child to understand, and has lead to situations where the child is punished or ignored because of it.

 

Speech and Language Therapy

 

Role of the Therapist


The Speech and Language Therapist's (SLT) role is to assess and diagnose which, if any, speech and language, communication or feeding difficulty the child is presenting with. This may not be because of PWS. If any disorder is found the SLT will advise on the best way to enable the child to reach their maximum communication potential. S/he will liaise with parents, teachers, carers, nursery staff and anyone else who is a regular part of the child’s environment.

Service Delivery may include:

• Consultation and advice about how people can support the child’s communication needs.
• Teaching parents/ carers to work with their child and giving specific work for them to do
• Advice to others involved with the child to maximise communication opportunities in different environments e.g. home, nursery, school.
• Assessment and explanation of the result, which includes the impact of any difficulty the child may have.
• Hands on therapy with the child, probably with backup practise for home (the therapy programme may be carried out by an SLT support worker under the guidance of a qualified SLT)
• Re-assessment of the impact for the child.
• Speech and language therapists work in episodes of care so the child may move in and out of the service as their needs change.

 

Difficulties of direct (hands on) therapy


The SLT supporting your child may advise that direct therapy is not the way forward. This will be decided on your child’s clinical needs. Children, who are resistant to therapy, as is the case with many children with PWS, will become distressed and not achieve as much as they could. There may be a need for constant and prolonged repetition of work for learning to take place; this cannot be achieved in weekly therapy sessions.

Children may not generalise what they learn in a specific situation, so that the trend now is for development activities to be devised which can be built into the child’s daily routine, or carried out within his school curriculum, rather than specific ‘therapy sessions’ taking place.

 Rewards for carrying out therapy activities must be carefully prepared according to the child’s interest, and must not be food- based. Suggestions could include playing with a favourite toy or a colouring sheet.


 
How to access SLT services

 

Every child with difficulties in the following skills should be assessed by an SLT

• Eating and drinking (if it is suspected there is a physical cause)
• Saliva control (excessive salivation for the child’s age)
• Speech (if not intelligible to the adults in the child’s environment)
• Language development (if not in line with the child’s other development, or to establish whether this is the case)
• Pragmatic skills or any other problem that is causing difficulty with communication

Most Health Board Trusts operate an open referral system, so that parents can request that an SLT give advice about their child by telephoning the local SLT department. If the child has a Statement of Need or co-ordinated support plan and SLT is specified, it is a legal requirement for the Education Authority to ensure that this is provided.

 

Adults


Some or all of the disorders mentioned above can persist into adult life. This may be because SLT was not available at the appropriate moment during the child’s development, or it may be that any learning disability meant that they could not learn the required skills.

• Speech may still not be intelligible, either due to lack of therapy, or because there are insurmountable physiological problems. Lack of intelligibility can lead to frustration and a lack of confidence in speaking to anyone outside those who are familiar with the persons’ speech patterns. Some young adults will appear to cope well when communicating with family and in school until they leave and the support of those familiar with them is withdrawn. If there is an impact on the person’s life then an SLT can provide advice on ways to support the person which may or may not include alternative or augmentative methods of communication.
• Immature or inappropriate use of language can be a source of embarrassment especially to teenagers with their desire to conform. What sounded ‘cute’ at 6 may not be so acceptable at 16.
• Puberphonia is a failure of the voice to break in young adult men. The voice remains high pitched and childish, and is a great disadvantage socially. Advice about this should be sought from a consultant endocrinologist, via the local hospital.

 

Accessing SLT for Adults

 

If the adult himself is concerned about any aspect of his ability to communicate, and therefore sufficiently motivated to change advice can be obtained from an SLT by self- referral.


This service may be available through a day Centre or college, or a Community learning disability team. If none of these are available contact the local SLT service via the adult department at your local hospital. If your child is about to leave schools and you wish to continue to receive support from SLT services, ask your current therapist, or the local department, to put you in touch with the appropriate adult department.

 

Service delivery


The type of support required would be decided on clinical need and might include:

• Assessment with advice
• Outpatient attendance and home practise
• Group therapy with others with a similar presentation
• Advice and training to allow the Patient and carers to deal with the problem themselves
• Support within the day to day environment e.g. college, care home or residential place.

 

Some self-help suggestions


Inhibition of the desire to communicate or inappropriate use of language can cause considerable embarrassment. The following ideas may be helpful.

 

• Attendance of a group where speaking is a natural part of the activity, eg drama group, self-advocacy, discussion group.
• Attendance at a course on assertiveness or confidence-building.
• Rehearsing. This involves practising aloud situations that might arise which require you to speak, eg a visit to the doctor. Practice what you want to ask, and the answers to the questions the doctor might ask. Also practice useful phrases, so that when someone speaks to you, you will know what to say. Do this with someone you know and trust and you should soon find you can build up more situations where you have something to say. Start with family and friends and progress to asking for something in a shop, then making a phone call, etc.

Note: Remember that not all children and adults with PWS will have all or any of these problems, and some of them might have been there if the individuals had not had PWS.

Fiona Whyte
Reg MRCSLT, Clinical lead SLT – advisor to the Royal College of speech and language therapist

 

Glossary

 

Articulation     Production of meaningful speech sounds by co-ordinated movement of tongue, lips, teeth palate and jaw.
Clinical          Relating to treatment of a medical condition.
Dysarthria    Inability to make accurate rapid speech movements. Generally such speech is slow, with indistinct consonants and long intervals between words. Overall effect is of slurred or indistinct speech.
Dyspraxia    Disability of motor programming of articulatory movements or lack of voluntary control over the muscles needed for speech, neurological in origin. Characterised by difficulty in imitating words or repeating them accurately and/or poor sequencing of sounds in words, and/or sentences.
Expression    The ability to use language in a way meaningful to the listener.
Fluency    The flow of speech.
Hypotonia    Lack of tension and strength in the muscles
Oral     Relating to the mouth
Physiological     Physical characteristics (e.g. shape of lips, tongue, palate).
Signing    The use of a sign language system e.g. Makaton.
Verbal comprehension     The ability to understand the spoken word

Copyright PWSA UK

 

Thank you

 

We are most grateful to the M&G Staff Charity Fund for their support for the revision and production of this document. 

 

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