Respiratory management
Respiratory failure in Duchenne may present without the usual signs of respiratory distress. Subtle signs could include early morning headaches, fatigue,daytime sleepiness, reduced appetite and weight loss. Consider underlying respiratory failure in case of a chest infection.
Respiratory infections need prompt treatment with antibiotics to prevent deterioration as well as regular physio to help clear secretions.
Most ambulant patients will have good respiratory function and cough, however once non-ambulant the risk of respiratory complications increases and some of them may need help with assisted coughing techniques, respiratory physiotherapy, and volume recruitment techniques including cough assist machines or Ambu-bag, therefore early intervention and close monitoring is paramount.
Once the FVC starts to deteriorate, boys are susceptible to chest infections and very vulnerable when FVC <50% or <I.5L. Ventilation may be required.
In Hospital
Assess Airway, Breathing and Circulation
Contact their specialist team who may have information that may be pertinent to their care.
Consider
In younger, ambulant boys:
- with mild/no fever and well, treat with oral antibiotics and review in 24 hours.
- with fever and unwell
admit
- Intravenous antibiotics and physio
- Monitor respiratory function (FVC & CO2)
- If 02 required, check CO2 level (do not treat with O2 indiscriminately) Assisted ventilation may be required (6)
Consider
In older +/- non-ambulant patients +/- ventilated:
Assess FVC – if baseline FVC when well is <I.5L or <50% they are very vulnerable.
- if FVC >2L or >50%, no/mild fever and well, treat with oral antibiotics and review in 24 hours.
- if FVC <2L or <50%, fever and unwell,
admit
- Intravenous antibiotics.
- Monitor respiratory function (CO2 and FVC).
- If 02 required, check CO2 (do not treat with 02 indiscriminately).
May require non invasive ventilation during admission.
In patients already ventilated at night and unable to perform own FVC -assess CO2
If ↑ – will need ventilation during the day with infection
Seek specialist help from home ventilation team. When contacting them have your ventilation details ready.
