Diaphragm Pacers in Pediatric Patients with Cervical Spinal Cord Injury: a Review and Implications for Inpatient Rehabilitation

Abstract

Pediatric spinal cord injury (SCI) at C3 level and above results in tetraplegia and profound respiratory insufficiency requiring long-term positive pressure ventilation (PPV) and is associated with significant complications and morbidity. Diaphragm pacing (DP), a form of functional electrical stimulation (FES) of the diaphragm, provides natural negative pressure breathing which can replace or decrease PPV need. There are numerous reported benefits of DP including improved speech, reduced caregiver burden, and decrease in secretions. The purposes of this paper are to review the limited literature on the safety and effectiveness of DP in pediatric SCI and describe collaboration between two unaffiliated, geographically separated institutions to facilitate implantation of DP for children with ventilator-assisted tetraplegia during inpatient rehabilitation. We further describe the process for ventilator weaning/diaphragm conditioning during inpatient rehabilitation. Two multicenter studies in adult patients with SCI demonstrate safety of the laparoscopic implantation procedure and effectiveness of the DPS system. The first pediatric patient was implanted in 2009, and since then, a total of 22 children with SCI had the system implanted. Among them, six children have now benefited from medically supervised multidisciplinary ventilator weaning/diaphragm conditioning supported by intensive activity-based restorative therapies (ABRT). For the pediatric patients reviewed, implantation of DP was a safe and effective alternative to long-term PPV. Using DP in lieu of PPV provided freedom from ventilator tubing and fear of disconnection, simplifying and facilitating ABRT. We have found that the benefits of DP far surpass the significant effort needed to facilitate implantation during inpatient rehabilitation.