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As the population of ventilator-dependent children (VDC) with tracheostomies due to underlying severe bronchopulmonary dysplasia grows, there is an increasing need to shift the care of these children from hospital to home. Transitioning the ventilator-dependent child from the hospital to home is a complex process that requires coordination between the medical team and the family. One crucial step in the process is transitioning from an Intensive care unit (ICU) ventilator to a portable home ventilator (PHV). The Clinical team needs to understand the nuances in transitioning to PHV, including assessing readiness to transition and choosing the optimum settings on an available home ventilator. In recent years, various ventilator modes have been available in PHV that can help achieve synchronous breathing to allow for adequate gas exchange for the infant. This review details some approaches to asses readiness to transition and the process of Transition along with commonly used modes of support available in PHV, as well as the primary and secondary settings in which we should be mindful in supporting a child with chronic respiratory failure in the home setting.

Objective Severe bronchopulmonary dysplasia (sBPD) can lead to long term morbidity. We created a sBPD multidisciplinary team in 2011 to optimize care and improve outcomes. Study design Retrospective chart review of three groups between 2008 and 2016: patients with sBPD born before 2011, patients with sBPD born after 2011, and patients with moderate BPD born after 2011. Results Infants with sBPD after 2011 had a shorter NICU length of stay compared with children born before 2011 (mean 140 days vs 170 days p

Introduction: Guillain-Barre syndrome (GBS) is the most common form of acute flaccid paralysis in pediatric patients. Classically, an antecedent illness provokes an autoimmune response against the myelin sheath of peripheral nerves, leading to ascending paralysis that can cause respiratory failure. Although associated with significant morbidity and mortality, it is treatable. Here, we present a case of GBS that manifested as respiratory distress and was initially diagnosed as simple community acquired pneumonia (CAP). Case Description: A 16 year old male with a history of Sotos syndrome, stage III CKD, autism spectrum disorder, nonverbal status, seizure …

Different types of patient triggered ventilator modes have become the mainstay of ventilation in term and preterm newborn infants. Maintaining spontaneous breathing has allowed for earlier weaning and the additive effects of respiratory efforts combined with pre-set mechanical inflations have reduced mean airway pressures, both of which are important components in trying to avoid lung injury and promote normal lung development. New sophisticated modes of assisted ventilation have been developed during the last decades where the control of ventilator support is turned over to the patient. The ventilator detects the respiratory effort and adjusts ventilatory assistance proportionally to each phase of the respiratory cycle, thus enabling the patient to have full control of the start, the duration and the amount of ventilatory assistance. In this paper we will review the literature on the ventilatory modes of proportional assist ventilation and neurally adjusted ventilatory assistance, examine the different ways the signals are analyzed, propose future studies, and suggest ways to apply these modes in the clinical environment.

A 6-year-old girl presented with several months of intermittent “bad smells” and “bad thoughts” and one prior episode where she was found unresponsive, making strange noises, with urinary incontinence, and left Todd paralysis. At current presentation, she had her typical aura, followed by staring and lip smacking and this time by rhythmic right eye blinking and right hand automatisms (video 1). Postictal EEG showed focal right posterior quadrant slowing (figure). Unilateral blinking is a rare ictal phenomenon usually associated with an ipsilateral frontotemporal seizure focus,1,2 which in this case is right-hemispheric. The patient's MRI was normal and the seizures were controlled by oxcarbazepine.

With decreasing mortality in PICUs, a growing number of survivors experience long-lasting physical impairments. Early physical rehabilitation and mobilization during critical illness are safe and feasible, but little is known about the prevalence in PICUs. We aimed to evaluate the prevalence of rehabilitation for critically ill children and associated barriers.National 2-day point prevalence study.Eighty-two PICUs in 65 hospitals across the United States.All patients admitted to a participating PICU for greater than or equal to 72 hours on each point prevalence day.None.The primary outcome was prevalence of physical therapy- or occupational therapy-provided mobility on the study days. PICUs also prospectively collected timing of initial rehabilitation team consultation, clinical and patient mobility data, potential mobility-associated safety events, and barriers to mobility. The point prevalence of physical therapy- or occupational therapy-provided mobility during 1,769 patient-days was 35% and associated with older age (adjusted odds ratio for 13-17 vs3 yr, 2.1; 95% CI, 1.5-3.1) and male gender (adjusted odds ratio for females, 0.76; 95% CI, 0.61-0.95). Patients with higher baseline function (Pediatric Cerebral Performance Category, ≤ 2 vs2) less often had rehabilitation consultation within the first 72 hours (27% vs 38%; p0.001). Patients were completely immobile on 19% of patient-days. A potential safety event occurred in only 4% of 4,700 mobility sessions, most commonly a transient change in vital signs. Out-of-bed mobility was negatively associated with the presence of an endotracheal tube (adjusted odds ratio, 0.13; 95% CI, 0.1-0.2) and urinary catheter (adjusted odds ratio, 0.28; 95% CI, 0.1-0.6). Positive associations included family presence in children less than 3 years old (adjusted odds ratio, 4.55; 95% CI, 3.1-6.6).Younger children, females, and patients with higher baseline function less commonly receive rehabilitation in U.S. PICUs, and early rehabilitation consultation is infrequent. These findings highlight the need for systematic design of rehabilitation interventions for all critically ill children at risk of functional impairments.

We performed a point prevalence study on infants with severe BPD collecting data on type and settings of ventilatory support; 187 infants were included from 15 centers, 51% who were on invasive positive pressure ventilation. We found significant center-specific variation in ventilator modes.

Objective The aim of this study is to determine patterns of neurally adjusted ventilatory assist (NAVA) use in ventilator-dependent preterm infants with evolving or established severe bronchopulmonary dysplasia (sBPD) among centers of the BPD Collaborative, including indications for its initiation, discontinuation, and outcomes. Study Design Retrospective review of infants with developing or established sBPD who were placed on NAVA after ≥4 weeks of mechanical ventilation and were ≥ 30 weeks of postmenstrual age (PMA). Results Among the 13 sites of the BPD collaborative, only four centers (31%) used NAVA in the management of infants with evolving or established BPD. A total of 112 patients met inclusion criteria from these four centers. PMA, weight at the start of NAVA and median number of days on NAVA, were different among the four centers. The impact of NAVA therapy was assessed as being successful in 67% of infants, as defined by the ability to achieve respiratory stability at a lower level of ventilator support, including extubation to noninvasive positive pressure ventilation or support with a home ventilator. In total 87% (range: 78–100%) of patients survived until discharge. Conclusion We conclude that NAVA can be used safely and effectively in selective infants with sBPD. Indications and current strategies for the application of NAVA in infants with evolving or established BPD, however, are highly variable between centers. Although this pilot study suggests that NAVA may be successfully used for the management of infants with BPD, sufficient experience and well-designed clinical studies are needed to establish standards of care for defining the role of NAVA in the care of infants with sBPD.

Mechanical ventilation of infants with severe bronchopulmonary dysplasia (BPD) is guided by important differences in lung mechanics between acute neonatal respiratory conditions and established BPD that requires mechanical ventilation beyond the first few weeks of life. Severe BPD is characterized by increased airway resistance, alveolar simplification with reduced surface area for gas exchange, heterogeneity in regional time constants, and both large and small airway disease. Lower ventilator rates, higher tidal volumes, and higher end-expiratory pressures are needed to achieve adequate support, which is characterized not only by adequate gas exchange but also by an infant who is calm, breathing comfortably with the ventilator, growing well, and able to interact with caregivers. A chronic care model of must be adopted, with optimal support being prioritized over weaning of support. Tracheostomy should be considered in any infant approaching term gestation and still mechanically ventilated. Existing and novel approaches to ventilation need to be rigorously evaluated in clinical trials.

Bronchopulmonary dysplasia (BPD) is a major cause of morbidity and mortality in surviving extremely preterm infants, with long-term morbidity disproportionately affecting children with severe BPD (sBPD). Infants with sBPD experience multiple organ system dysfunction. To best treat these complicated patients, we created a multidisciplinary team in 2011 consisting of multiple pediatric subspecialists with a specific interest in sBPD. In the past six years, 150 patients have been referred to our multidisciplinary team, with 131 of the 150 patients discharged home, 65% on home oxygen. Twelve were transferred to the Pediatric Intensive Care Unit (PICU), 3 to a level 2 nursery and 4 died. The multidisciplinary BPD team has standardized the care of children with sBPD and complex medical problems and improved outpatient referral to subspecialists.

Administration of adenovirus (Ad) vectors to animals induces innate immune responses, typified by elevated interleukin-6 (IL-6). To assess innate responses to Ad vectors in humans, we evaluated serum IL-6 following administration of E1(-) E3(-) Ad vectors to different human hosts and the relationship among peak IL-6 and peak anti-Ad neutralizing antibodies. We administered: 1) Ad(GV)CFTR.10, a vector carrying the normal human CFTR cDNA (3 x 10(7) to 2 x 10(10) particle units (pu)) to airways of individuals with cystic fibrosis (CF); 2) Ad(GV)VEGF121.10, a vector carrying the normal human vascular endothelial growth factor (VEGF)121 cDNA, to the myocardium (4 x 10(8) to 4 x 10(10) pu) of individuals with coronary artery disease (CAD) and to lower extremity muscles (4 x 10(8) to 4 x 10(9.5) pu) of individuals with peripheral vascular disease (PVD); and 3) Ad(GV)CD.10, a vector carrying the Escherichia coli cytosine deaminase gene to skin (7 x 10(7) to 7 x 10(9) pu) and airways (7 x 10(8) to 7 x 10(10) pu) of normal individuals and to liver metastasis (4 x 10(8) to 4 x 10(9) pu) of individuals with colon carcinoma. IL-6 increased mildly (up to 220 pg/ml) following vector administration to skin and lung airways of normal individuals and of individuals with CF, and to muscle and liver metastasis of individuals with PVD and colon cancer, respectively. IL-6 responses were higher (up to 1100 pg/ml) following myocardial administration. Control individuals who had chest surgery and bronchoscopy, but no vector administration, had comparable IL-6 increases. Thus, both administration of Ad vectors of humans up to 10(10) pu and the procedures used to administer the vectors elicit systemic IL-6 responses. There was no correlation among peak IL-6 and peak anti-Ad antibodies. These observations indicate that the innate host responses following administration of Ad vectors to humans may result from the procedures used to administer the vector, and from the vector per se.