Exploratory Study of Respiratory Bacterial Infections or Superinfections and Colonizations in Patients With Spinal Muscular Atrophy Under NIV

SMA (Spinal Muscular Atrophy) is a rare neuromuscular disease characterized by motoneuron damage. Symptoms consist of respiratory involvement with numerous respiratory infections and eventually respiratory failure, for which NIV (Non Invasive Ventilation) is often used. Ventilation machines are in close contact with the respiratory tract of patients. They contain heated water to humidify the circuit. These humid and warm environments are conducive to the development of bacteria such as Pseudomonas aeruginosa. In this context, it is interesting to look for the presence or absence of bacteria, in comparison with the respiratory ecology of the patients. The aim is to highlight the microbiological role of NIV on the occurrence of respiratory bacterial infections or secondary infections in patients with SMA. To do this, samples are taken from the machines, and ECBCs are performed on patients during respiratory physiotherapy sessions.

Inclusion Criteria:Diagnosis of AMS (previous diagnosis or new diagnosis during the inclusion period)Age from 0 to 12 years oldNusinersen treatmentBenefiting from a NIVCurrent monitoring at the University Hospital of AmiensExclusion Criteria:Invasive ventilation on tracheotomyNo ventilationOther treatment than Nusinersen or no treatmentParental refusal to participate in the study