ATS GUIDELINES Bundle

3 Management ➤ Recommendation 3a: We recommend that an awake and attentive trained caregiver be in the home of a child requiring chronic invasive ventilation at all times. (strong recommendation; very low quality evidence). • Despite very low quality evidence supporting this recommendation, the Workgroup was confident that in this case the desirable consequences would clearly outweigh the undesirable consequences of following this recommendation. Lack of an awake and attentive trained caregiver would place the child in a life- threatening situation. Training of caregivers is irrelevant if one is not available to respond to an emergent situation. For most families this requires the support of a professional appropriately trained in-home caregiver to allow family caregivers time to sleep, work, and maintain a life balance. This recommendation places a high value on the safety of the patient, and low value is placed on avoiding the increased use of resources and the possible disruption to families who may need to accommodate a professional caregiver in their home. ➤ Recommendation 3b: For children requiring chronic invasive ventilation, we suggest that at least two specifically trained family caregivers are prepared to care for the child in the home. (conditional recommendation; very low quality evidence) • The experience of the Workgroup and available data indicate that a lone trained family caregiver would rarely be capable of shouldering the entire burden of care for a child using invasive ventilation in the home. This recommendation places high value on the safety of the patient and quality of life of caregivers and low value on increased resource use for training more than one caregiver. ➤ Recommendation 3c: We suggest that ongoing education to acquire, reinforce, and augment skills required for patient care be provided to both the family and professional caregivers of children requiring chronic home invasive ventilation. (conditional recommendation; very low quality evidence) • The Workgroup believed, based on clinical experience, that practitioners and professional personnel agencies must strive to provide ongoing education to family and professional caregivers. Continuing education would help reinforce learned skills and allow training on new technologies and protocols. This recommendation places a high value on safety and on the potential clinical benefits to the patient and a low value on increased cost and resource use. ➤ Recommendation 4a: For children requiring chronic home invasive ventilation, we suggest monitoring, especially when the child is asleep or unobserved, with a pulse oximeter rather than use of a cardiorespiratory monitor or sole use of the ventilator alarms. (conditional recommendation; very low quality evidence) • Small indirect studies and the experience of the Workgroup suggest that ventilator alarms may not always function correctly. Furthermore, hypoxemia is most likely to be the first indicator of a serious issue in a child with respiratory disease. The workgroup believes pulse oximetry is the preferred method for monitoring patients on home mechanical ventilation. This recommendation places high value on the safety of the child and low value on possible increase in caregiver burden secondary to false alarms.

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