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Summary of Clinical Practice Guideline

British Thoracic Society

British Thoracic Society Guideline for Respiratory Management of Children with Neuromuscular Weakness
Hull, J., Aniapravan, R., et al. (2012).
Thorax, 67 Suppl 1, i1-40.

AGREE Rating: Highly Recommended

This guideline provides recommendations for the assessment and treatment of children with neuromuscular weakness (NMW). The target audiences of this guideline are healthcare professions who care for these children. The guideline provides graded evidence statements and recommendations. Evidence statements are graded as:

  • Level 1 (1-, 1+, 1++) - Meta-analyses/systematic reviews of randomized controlled trials (RCTs) or RCTs with low (1++) to high (1-) risk of bias.

  • Level 2  (2-, 2+, 2++) - Case control or cohort studies or a systematic review of such studies with a minimal (2++) or high (2-) risk of bias.

  • Level 3 - Non-analytic studies

  • Level 4 - Expert opinion
The recommendations included in the guideline are classified based on the strength and quality of evidence.
  • Grade A - Based on 1++ or 1+ evidence directly applicable to the target population and overall consistency of results.

  • Grade B - Based on 2++ evidence directly applicable to the target population or extrapolated evidence from studies classified as 1++ or 1+.

  • Grade C - Based on 2+ evidence directly applicable to the target population or extrapolated evidence from studies classified as 2++.

  • Grade D - Evidence level 3 or 4 extrapolated evidence from level 2 + studies.

  • Good Practice Point - Important practice points for which there is no or likely to be no evidence.


Pediatric Dysphagia Map

  • Assessment/Diagnosis

    • Assessment Considerations

      • "Feeding and swallowing difficulties are common in children with neuromuscular disease and are frequently under-recognized (evidence level 2-)" (p. 26).

      • In boys with Duchenne muscular dystrophy, advancing age leads to increased oral and pharyngeal dysfunction (evidence level 3).

    • Instrumental Assessment

      • Videofluoroscopy

        • Children with NMW and a history of recurrent chest infections or swallowing difficulties should have "a feeding assessment by a speech and language therapist including a videofluoroscopy swallow assessment if the swallow is thought to be unsafe" (good practice point) (p. 1).

  • Treatment

    • General Findings

      • "A problem-oriented approach to nutrition should aim to minimize risk of aspiration, optimize nutritional status, promote comfort, and balance the positive social consequences of continued oral feeding" (good practice point) (p. 26).

    • Tube Feeding

      • "Gastrostomy feeding can improve and maintain adequate nutrition in children with NMW (evidence level 3)" (p. 27).

Neuromuscular Diseases

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