Collagen‑VI Muscular Dystrophy – General Emergency Guidance
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Brief overview
Collagen‑VI muscular dystrophies are neuromuscular disorders with musculoskeletal and respiratory implications. In emergencies, airway management, gentle handling, and respiratory support require special attention.
General immediate measures
- Gentle handling and positioning; prevent pressure sores.
- Early airway assessment; difficult airway may occur.
- Consider respiratory insufficiency; monitor SpO₂/CO₂.
- Beware of fracture risk (possible reduced bone density).
- Early mobilisation/physiotherapy after stabilisation when feasible.
Contraindications / Caution
- Avoid unnecessary immobilisation (risk of contractures).
- Verify medications and interventions against up‑to‑date guidance.
- When uncertain, consult specialised centres early.
Notes for EMS and Emergency Department
- Have an airway plan including alternatives available.
- Use careful transfer and repositioning techniques.
- Initiate respiratory support as indicated.
Further reading
- Anaesthesia: OrphanAnesthesia – Rare Diseases Supplement 02‑2020
- General: Collagen VI CMD – Swiss Muscle Society
- Genetics/Overview: GeneReviews: Collagen VI‑Related Dystrophies