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

In today’s #apaperaday, Prof. Aartsma-Rus reads and comments on the paper titled: Best practice recommendations for speech-language pathology in children with neuromuscular disorders: A Delphi-based consensus study

Today’s pick is from the international journal of speech-language pathology by Kooi-van Es et al. DOI: 10.1080/17549507.2023.2181224. The paper describes a delphi process to come to recommendations for speech language pathology in children with neuromuscular diseases (NMD) in the Netherlands. NMD patients have muscle weakness, which can also influence speech, chewing, swallowing and saliva control.

Difficulties with chewing, swallowing and speech can have obvious impacts on quality of life. Authors stress the need for an individualized approach for speech-language pathology specialists for these individuals. However, often the specialists are not familiar with NMDs (rare).

To facilitate and align speech-language pathology care for patients with Duchenne, spinal muscular atrophy type 2, myotonic dystrophy type 1 and congenital myopathy authors started a delphi process, which consists on multiple steps to achieve consensus.

Case studies based on patients with the different NMDs were used to obtain consensus on a treatment approach. The process involved >12 NMD speech language pathology experts in each round (i.e. experts had hands on experience with NMDs).

During the process also a literature search was done to try and find support for the recommendations. Furthermore, the recommendations were provided to patient advocacy and support groups for input and feedback.

Authors outline care needs to be flexible & focused on clinical need. As individuals have a chronic condition, long term planning is needed. Due to rarity there is a lack of experience & quality evidence, authors hope the Dutch guidelines are a start for international versions.

Treatment/management aspects:

  1. Wait and see: monitoring the symptoms and only intervene if they are bothersome or pose a risk;
  2. Explain and advice: in every case patients and parents need to be made aware of the lifelong consequences and risks and possible interventions.
  3. Training and treatment: training should be functional rather than to improve strength, as this may worsen the condition for NMD patients
  4. Aids and adjustments: for this speech-language pathology expert should also discuss with other disciplines to align.Discussing with other disciplines is always a good idea of course. The patient is more than his/her speech-language pathology – for holistic approaches you need to work with other disciplines.
  5. Referral. This relates to the previous point: if you cannot provide the care / refer to experts in other disciplines (e.g. swallowing, dietician, occupational therapy etc), consider referring the patient to an expert center where all disciplines are under one roof.
  6. Monitoring: monitor how interventions/aids/treatments work and anticipate problems that may occur. Symptoms may be subtle and continuous monitoring (so regular follow ups with increased frequencies when there are concerns or problems) is crucial for optimal life quality.

Authors outline the limitations (only in the Netherlands and a limited field with little evidence as yet). However, we have to start somewhere. Authors end by saying that the care should always be person focused as each individual will present differently.