Paediatrics: Management of neuromuscular junction and muscular disorders

Management of neuromuscular junction and muscular disorders

These disorders are rare, severe and complicated. They need to be man-aged by a good local service with on going advice from a specialist centre. Key components of care include:

·  Assessment for power, joint ranges, and contractures. With appropriate advice from physio- and occupational therapists.

·  Access to speech therapy and dietary assessment.

·  Consideration on appropriate management of respiratory, cardiac and other systems’ complications.

·  Liaison with allied services, particularly education/social services.

·  Genetic counseling for the child and other family members.

·  Psychological support for the child and family.

It should be noted that, although a specialist centre will need to be involved, they will fail, if they do not liaise and empower a good local service, and the child/family, with the above key parts. Their advice on the projected trajectory and likely complications for the specific disorder should inform the exact local management plan.

Caution There is a particular risk of malignant hyperthermia during general anaesthesia for most neuromuscular disorders, so make sure child, parents and all relevant professionals are alerted, particularly fam-ily practitioners.