Of the more than 9.4 million children in the United States with special health care needs, few present more challenges than those requiring chronic invasive ventilation at home. The shift from care in the pediatric ICU to intensive care at home requires invested families, multiple trained in-home caregivers, sophisticated technology, and ready access to primary and subspecialty care. Yet there’s little data in the literature and few clinical practice guidelines for health professionals and parents on providing this care at home.

“The quality of evidence provided by the scant research literature in this area is poor, and there are no randomized or controlled observational studies,” says pediatric pulmonologist Laura Sterni of the Johns Hopkins Children’s Center. “Standards and recommendations for the safe discharge and management of these complex children in the home were not available, making it difficult for medical teams and families to access needed support and resources.”

Until now. An American Thoracic Society (ATS) workgroup led by Sterni created recommendations for the care of these technology-dependent children. Recognizing that children dependent on long-term mechanical ventilation are at risk for several complications, including pulmonary hypertension, recurrent pulmonary infections, poor growth and developmental delay, the work group recommended as the centerpiece of the guidelines a medical home model—co-managed by a generalist and a respiratory subspecialist—to improve care coordination.

“It is our hope the recommendations will reduce family stress and burden by helping families navigate the complex health care and community systems involved in their child’s care and obtain the support they need,” explains Sterni.

Among the other recommendations under the guideline—

• The use of standardized discharge criteria to assess readiness for care in the home
• An awake and attentive trained caregiver in the home, and for children requiring chronic invasive ventilation, at least two trained family caregivers in the home
• Ongoing education to acquire, reinforce and augment skills required for patient care
• Monitoring, especially when the child is asleep or unobserved, with a pulse oximeter rather than a cardiorespiratory monitor
• Regular maintenance of home ventilators

Sterni stresses that the recommendations are important because children dependent on long-term ventilation have longer lengths of stay in the hospital, greater total costs of care and a higher risk of death. Home mechanical ventilation, she says, offers some of these children the chance to grow up with their families, experience a relatively normal life and maximize their rehabilitative potential.

The ATS recommendations are based on uncontrolled studies and the expert opinion of members of the workgroup. Physicians and respiratory therapists at the Pediatric Sleep Center at the Johns Hopkins Children’s Center, Sterni notes, have deep experience and expertise in conducting oxygen studies on infants and children and assessing their respiratory needs and requirements for home ventilator support.  

“Our lab is special not only because we can diagnose sleep apnea, but because we can do these complex studies that most laboratories cannot do and actually care for all these kids on ventilator and oxygen support,” says Sterni. “This is an important guideline, and our pulmonary team and sleep center can help parents manage these complex care issues.”