Chronic respiratory failure is the leading cause of morbidity and mortality in children with multiple disabilities. Musculoskeletal disorders, swallowing disorders and ventilatory control abnormalities lead to progressive respiratory impairment, causing episodes of respiratory decompensation (RD). These are the leading cause of mortality (50 to 80%, average age of death: 21 years) and the cause of repeated hospitalizations.

Respiratory care for these children is therefore a major challenge. In respiratory physiotherapy, this can be achieved through manual techniques and/or instrumental techniques such as intrapulmonary percussive ventilation (IPV), which is particularly useful in this population because it does not require active cooperation.

The main objective of this study is to evaluate, in children with multiple disabilities, the effectiveness at 18 months of using an IPV at home and by carers, combined with standard respiratory physiotherapy, compared with manual respiratory physiotherapy alone.This is a multicenter, randomized controlled superiority trial with two parallel groups.

All centers will recruit children aged 1 to 16 with multiple disabilities, during hospitalization with respiratory decompensation (RD). After consent has been signed by the parents or legal guardians, the patient will be randomized into one of the two study groups:

• Control group: manual respiratory physiotherapy.
• Intervention group: intrapulmonary Percussive Ventilation (IPV)

IPV is used in France in different ways, depending on the center and service practices, as a complement to manual techniques.

At Armand Trousseau University Hospital, our experience over the past five years with more than 50 children suggests that the use of IPV by carers in their home environment. This is done as a complement to private manual physiotherapy sessions after training by a professional.

However, the benefits and impact of IPV on patients' respiratory development remain largely unstudied, particularly in patients with multiple disabilities. This multicenter randomized controlled study could provide recommendations for the respiratory management of these patients.

The main objective of this study is to evaluate, in children with multiple disabilities, the effectiveness at 18 months of using an IPV in the home and by carers, combined with standard respiratory physiotherapy, compared with manual respiratory physiotherapy alone. The primary endpoint is the number of rehospitalizations for RD at 18 months following the index hospitalization.

All centers will recruit children aged 1 to 16 with multiple disabilities, during hospitalization with Respiratory Decompensation (RD). the patient will be randomized into one of the two study groups:

• Control group:
• Intervention group: the use of IPV will be initiated during hospitalization. The total duration of the study is planned to be 36 months, Follow-up visits will be scheduled at 1, 3, 6, 12 and 18 months for both groups. In order to avoid adding too many trips for these families, who are often very busy, visits will be scheduled at the same time as routine medical and paramedical consultations whenever possible. Certain visits that cannot be synchronized with routine follow-up consultations will be conducted via videoconference or telephone calls by the study investigators.

An adjudication committee will validate respiratory decompensation following rehospitalization that may occur within 18 months of the child's inclusion/randomization in the PERKINE protocol. They will remain blinded to the child's randomization group until the end of the study.

Analysis will be performed at the end of the study after data review and before database lock

Inclusion Criteria:

• Polyhandicap defined by:

  • Causal brain injury or lesion occurring before the age of 3 years,
  • Severe or profound intellectual disability according to DSM IV definition
  • Motor disability: paraplegia/quadriplegia, hemiparesis, ataxia, and/or extrapyramidal motor disorders,
  • Gross Motor Function Classification System (GMFCS) score of IV or V,
  • Functional Independence Measure (FIM) score < 55,

• Age: 1 to 16 years,

• Hospitalization of the patient with respiratory decompensation (RD),

• Informed consent from parents/legal guardians

Exclusion Criteria:

• Associated neuromuscular disease

• Daily use of intrapulmonary percussive ventilation for more than 3 consecutive months within the 6 months prior to inclusion,

• Daily use of a cough assistance device such as an insufflator-exsufflator for more than 3 consecutive months within the 6 months prior to inclusion,

• Contraindications for the use of the medical device:

  • Pneumothorax,
  • Hypotension or hemodynamic instability,
  • Hemoptysis,

• Participation in another interventional study.

• Patients with tracheobronchial collapse during expiration