Best practice recommendations for speech-language pathology in children with neuromuscular disorders: A Delphi-based consensus study

Abstract

Purpose

Speech-language pathology (SLP) is considered an essential intervention due to the high prevalence of dysphagia and dysarthria in paediatric neuromuscular disorders (pNMD). Evidence-based guidelines for SLP in pNMD are missing and children could be deprived the best of care. This study aimed to achieve consensus and present best practice recommendations on SLP intervention in pNMD.

Method

A modified Delphi technique was used with a panel of experienced Dutch speech-language pathologists. In two online survey rounds and a face-to-face consensus meeting, the SLP experts proposed intervention items for cases of four types of pNMD (congenital myopathy, Duchenne muscular dystrophy, myotonic dystrophy type 1, and spinal muscular atrophy type 2), covering symptoms of dysphagia, dysarthria, drooling, and oral hygiene problems. They rated the level of agreement.

Result

Intervention items that achieved consensus were incorporated into best practice recommendations. These recommendations cover six core intervention components (wait and see, explanation and advice, training and treatment, aids and adjustments, referral to other disciplines, and monitoring) suitable for the described symptoms.

Conclusion

Insight into treatment options is essential to facilitate speech-language pathologists in clinical decision-making. The current study led to best practice recommendations for speech-language pathologists working within the field of pNMD.

Keywords:

• Dysphagia
• dysarthria
• saliva control
• oral hygiene
• neuromuscular disorders
• best practice recommendations

Acknowledgments

The authors would like to thank the survey pilot testers, speech-language pathology experts and stakeholders for assistance with survey development and participation in this study.

Stakeholders

- Dutch Association for Speech Language Pathology and Phoniatrics (Nederlandse Vereniging voor Logopedie en Foniatrie) (NVLF), subject content guideline panel

- Dutch Neuromuscular Disease Association (Spierziekten Nederland) (congenital and metabolic muscle diseases guideline panel)

- Duchenne Parent Project (parent association to accelerate research into treatment or cure for Duchenne muscular dystrophy)

- Ms A.M.B. van der Heul, MSc, speech language pathologist and researcher SMA, UMC Utrecht

- Ms A. de Baaij-Daalmeyer, dietician, Radboudumc, Nijmegen

- Ms M.H. van Westen - de Boer, occupational therapist, Rehabilitation Centre Roessingh, Enschede

- Ms S. van de Kamp, paediatric physical therapist, Rehabilitation Centre Klimmendaal, Arnhem

- Dr M.J. Poelma, paediatric rehabilitation physician, Sint Maartenskliniek, Nijmegen

Declaration of interest

The authors report there are no competing interests to declare.

Supplementary material

Supplemental data for this article can be accessed at https://doi.org/10.1080/17549507.2023.2181224.

Additional information

Funding

This work was supported by the the Johanna KinderFonds under Grant 20170039; by Stichting Janivo under Grant 2018451; by the Damsté-Terpstra Fonds under Grant 200111/2; and the Christina Bader Stichting under Grant 619. The funding body had no influence on the conduct of the study.