Introduction

All babies and children are individuals and will develop at their own pace, developing their own abilities, skills, and personality over time. Children living with PWS may experience similar learning challenges in speech and language development that can occur in any child.

There is also the possibility of additional learning needs associated with the syndrome. These may be caused by:

• Learning challenges, 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 are likely to experience challenges with speech and/or language at some time during their lives.

Suckling and 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 your baby’s sucking reflex, positioning your baby to enable him to swallow more easily, and external stimulation of the oral muscles. For more information see “Feeding and Weaning your Child”. [Add link when available]

There can be delay in development of chewing and swallowing, sometimes causing choking (aspiration). Professional assessment and advice from a suitably qualified lactation consultant, breastfeeding counsellor or speech and language therapist is suggested.

NOTE: Words in orange are explained in the glossary at the end of this brochure.

The role of the speech pathologist

The Speech Therapist’s role is to assess and diagnose which, if any, speech and language, communication or feeding difficulty the child is presenting with.

This may or may not be due to PWS. If any disorder is found the therapist will advise on the best way to enable your child to reach their maximum communication potential. They will liaise with parents, teachers, carers, kindergarten, creche staff and anyone else who is a regular part of your child’s community.

Language Delay

Language delay can often occur, with verbal comprehension being better than expressive language. This may lead to extreme frustration and exacerbate temper episodes if your child knows what they want but cannot put it into words.

Early stimulation at the appropriate level for the child’s understanding is essential for them to maximise skills in this area, and the use of signing such as Key Word Signing Australia to aid development of comprehension is often a valuable technique suggested by therapists as an interim solution. Signing can also reduce frustration if the child’s needs can be met more quickly.

‘I remember with gratitude how quickly my son picked up 5 or 6 Makaton signs (now known as Key Word Signing Australia) and began using them in his daily speech. Our daughter, my partner and I all integrated the signing into our conversation, and he soon copied. It was a great learning moment for him, and his communication skills flourished from that moment.’ Father, Melbourne.

Some people are concerned that signing will stop the child using speech, but research shows that signs are dropped once the child can say the word. At this stage, the most important thing is to undertake the same language stimulation activities you would with any child.

Articulation

Inaccuracy of movements and inability to change quickly from one tongue or lip position to another (sometimes described as dysarthria) are often experienced by people living with PWS. This is caused by the combination of a high arched palate and the small lower jaw. Hypotonia in the oral muscles can also contribute. More rarely dyspraxia occurs, this condition can lead to reduced intelligibility. Both are physical problems and speech, and language therapy may not solve them, requiring augmentative methods of communication, e.g., communication books or voice output devices.

Language challenges

More rarely a specific language learning difficulty exists. Your 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 a speech therapist and an educational psychologist can determine the origin of the problem.

Some children may have trouble with expressive language, with their verbal comprehension more intact, this is similar to a language delay, but can be more pervasive and persistent in nature, so that the ability to communicate is further delayed.

Fluency

Most children experience a stage of non-fluency, usually between the ages of 3 and 5 but for the child living 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 sometimes a genetic risk factor in the incidence of stammering within families.

Pragmatic Disorder

Pragmatic skills concern the child’s ability to use language appropriately in social situations; this can sometimes 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 speech therapy because they can speak, although therapists are the appropriate specialists to consult.

Autistic Spectrum Disorder

A small number of children living with PWS may also have an Autistic Spectrum Disorder (ASD). It is easy to miss this because of the difficulty or delay in social skills development. We have also heard of a few children with an ASD diagnosis where it was not realised that their difficulties with social skills were because of a delay in this area of development due to PWS, not ASD.

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 led to situations where the child is frustrated by being unable to get their message across.

Speech therapy

Service delivery may include:

• Consultation and advice about how people can support your child’s communication needs or how to develop eating, drinking and saliva control skills.
• Teaching parents/carers to work with your child and giving specific activities for them to practice.
• Advice to others involved with your child to maximise communication opportunities in different environments, e.g., home, creche, kinder, school.
• Assessment and explanation of the result, which includes the impact of any difficulty the child may be experiencing.
• Hands on therapy with your child, probably with backup practice for home. This therapy programme may be carried out by a therapy support worker under the guidance of a qualified Speech Pathologist.
• Re-assessment of the impact for each individual.
• Speech and language Pathologists work in episodes of care so each child may move in and out of the service as their needs change.
• They also write reports as evidence for funding and requests for other support types

Challenges of direct (hands on) therapy

The pathologist supporting your child may advise that direct therapy is not the way forward. This will be decided on your child’s clinical needs. Some children who are resistant to therapy 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 alone.

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 each child’s interest and must not be food-based. Suggestions could be playing favourite games, viewing favourite TV shows, listening to music, getting a new book, etc.

How to access Speech Therapy services

Every child with difficulties in the following skills should be assessed by a speech therapist:

• 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 providers operate an open referral system, so that parents can request that a speech therapist give advice about their child by telephoning the local speech department. There is likely to be a different cost if therapy is through a public or private provider. If your child has been accepted as a Participant under the National Disability Insurance Scheme (NDIS) you may be successful in securing funding for a speech therapist in their NDIS Plan.

Remember!

Each person living with PWS is an individual. Some may experience none of the above challenges and others perhaps more. Others might have been there even if the individuals are not living with PWS.

You can find more resources or a therapist at Speech Pathology Australia or by contacting PWSA at social@pws.org.au

Thank you ….

Thanks to PWSA UK, and Fiona Whyte, Reg MRCSLT, Clinical lead SLT, advisor to the Royal College of Speech and Language Therapists for sharing the original resource material.

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.

Hypotonia – Lack of tension and strength in the muscles.