Sheila S. Kun

Outcomes and causes of death in children on home mechanical ventilation via tracheostomy: an institutional and literature review.

OBJECTIVE To describe outcomes and causes of death in children on chronic positive-pressure ventilation via tracheostomy. STUDY DESIGN We conducted a retrospective observational cohort analysis of 228 children enrolled in an university-affiliated home mechanical ventilation (HMV) program over 22 years (990 person-years). Cumulative incidences of survival and liberation from HMV are presented. Time-to-events were compared by reason for chronic respiratory failure (CRF) and age and date of HMV initiation with Kaplan-Meier and Cox regression analyses. Circumstances of death are described. RESULTS Of our cohort, 47 of 228 children died, and 41 children were liberated from HMV. The 5-year cumulative incidences of survival and liberation were 80% and 24%, respectively. Being placed on HMV for chronic pulmonary disease was independently associated with liberation from HMV (hazard ratio, 7.38; 95% CI, 3.0-18.2; P < .001). Neither age nor reasons for CRF were associated with shortened survival. Progression of underlying condition accounted for only 34% of deaths; 49% of deaths were unexpected. CONCLUSION Most children on HMV survive or were weaned off. However, a sizable number of children in our cohort died, and many deaths were unexpected and from causes not directly related to their primary reason for CRF.

Hospital readmissions for newly discharged pediatric home mechanical ventilation patients

Ventilator‐dependent children have complex chronic conditions that put them at risk for acute illness and repeated hospitalizations.

How much do primary care givers know about tracheostomy and home ventilator emergency care

Children on home mechanical ventilation are a high‐risk population. How much do primary caregivers (PC) know about handling unexpected situations with tracheostomies or malfunction of their ventilators? To answer this, we prospectively studied the knowledge of 152 PC (108 parents and 44 nurses), using a 25‐question survey regarding emergency situations at home. Results: their mean score was 20.2 correct answers (81%). However, 96 PC (63%) did not know that the low pressure ventilator alarm would not sound if the tracheostomy tube decannulated while still connected to the ventilator. Seventy‐nine PC (52%) failed to understand high pressure alarms sound and mucous plugging. Sixty‐six PC (43%) did not know how much power a battery stored after the suction machine was fully charged. Sixty‐one PC (40%) did not know when the low pressure or low minute volume alarm sounds. Fifty‐six PC (37%) relied on the ventilator alarm to determine the presence of mucous plugs. There were no significant differences in the scores of PC who used continuous flow ventilators, nor in English‐speaking PC versus Spanish‐speaking PC. The experience of the PC did not make a difference in their knowledge of HMV emergency care. Having professional training did not pose an advantage in the score for nurses. We conclude that most PC had a good understanding of emergency care. We speculate that more in depth education on the technical aspects of ventilator alarms and tracheal mucous plugging may help to reduce or prevent emergencies of children on HMV. Pediatr Pulmonol. 2010; 45:270–274.

Children with corrected or palliated congenital heart disease on home mechanical ventilation

Infants and children with surgically corrected or palliated congenital heart disease (CHD) are at risk for chronic respiratory failure, necessitating home mechanical ventilation (HMV) via tracheostomy. However, very little data exists on this population or their outcomes. We conducted a retrospective chart review of all children with CHD enrolled in the Childrens Hospital Los Angeles HMV program between 1994 and 2009. Data were collected on type of heart lesion, surgeries performed, number of failed extubations, timing of tracheostomy, mortality, length of time on HMV, weaning status, associated co‐morbidities, and Risk Adjusted classification for Congenital Heart Surgery (RACHS‐1) category. Thirty‐five children were identified; six with single ventricle anatomy, who received palliative procedures. Twenty‐three (66%) patients are alive; 8 (23%) living patients have been weaned off HMV. Twelve (34%) patients are deceased. The incidence of mortality for single ventricle patients was 50%, and only one of the surviving children has received final palliation and weaned off HMV. Eight of nine patients (89%) with a RACHS score ≥4 died, and none have been weaned off of HMV. The 5‐year survival for all CHD HMV patients was 68%; 90% for patients with RACHS ≤3; and 12% for patients with score ≥4. Children with more complex lesions, as demonstrated by single ventricle physiology or greater RACHS scores, had higher mortality rates and less success weaning off HMV. This case series suggests that caregivers should give serious consideration to the type of heart defect as they advise families considering HMV in children with CHD. Pediatr Pulmonol. 2010; 45:645–649.

Costs of hospitalized ventilator-dependent children: Differences between a ventilator ward and intensive care unit

Hospitalizing clinically stable patients in critical care settings results in unnecessary healthcare costs and thwarts timely patient throughput. Some pediatric hospitals care for their stable ventilator‐dependent children outside of pediatric intensive care units (PICUs). To date, no analysis of the costs of these pediatric ventilator units compared to PICUs has been performed. We conducted a retrospective comparison of PICU and ventilator ward costs of hospitalizations for 103 admissions in which ventilator‐dependent children served as their own matched controls between 2004 and 2007. For included admissions, patients were hospitalized in both units during the same admission and spent more than 1 day in their initial unit. Comparisons of costs were made using the last full PICU day and first full ward day. For the study period, the mean PICU cost of hospitalization per day was

Congenital hypoventilation syndromes.

3,565 (standard deviation [SD] ± 716.50). The mean ward cost was

Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients.

2,052 (SD ± 617). The mean PICU cost was significantly larger than the mean ward cost (paired t‐test, P < 0.0001). Ventilator ward total and variable costs were significantly less than those in the PICU, and such units represent a potential cost saving measure for hospitals that care for ventilator‐dependent children. Pediatr Pulmonol. 2011; 46:356–361.

Thoracoscopic placement of phrenic nerve pacers for diaphragm pacing in congenital central hypoventilation syndrome

Although rare, congenital hypoventilation syndromes profoundly impact affected patients and families. In some diseases, such as congenital central hypoventilation syndrome (CCHS), hypoventilation is a key presenting feature. Ventilatory abnormalities may not be immediately evident in other disease states. The clinical aspects of several pediatric hypoventilation syndromes, including CCHS, Chiari type II malformation, Prader-Willi syndrome, familial dysautonomia, and rapid onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation are presented.

Three-Generation Family With Congenital Central Hypoventilation Syndrome and Novel PHOX2B Gene Non-Polyalanine Repeat Mutation

Background: Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting central control of breathing. Thus, patients require lifelong assisted ventilation. Diaphragm pacing (DP) may permit decannulation in those who are ventilator dependent only during sleep. Objective: The purpose of this study is to determine if patients with CCHS can be successfully ventilated by DP without tracheostomy. Methods: We reviewed the records of 18 CCHS patients (mean age 19.5 ± 10.1 years; 44% female) who were ventilated by DP only during sleep. Results: Prior to diaphragm pacer implantation surgery, 14 CCHS patients had been using home portable positive pressure ventilation (PPV) via tracheostomy, 1 had been on PPV via endotracheal tube, and 3 had been using noninvasive PPV (NPPV). Of the patients with tracheostomy prior to DP (n = 15), 11 (73%) were decannulated and ventilated successfully by DP without tracheostomy. Of all the patients reviewed (n = 18), 13 (72%) were successfully ventilated by DP without tracheostomy. Obesity prevented successful DP without tracheostomy in 1 patient, and upper airway obstruction prevented success in another patient. Snoring and/or obstructive apneas were present in some patients, but they were improved by diaphragm pacer changes, adenotonsillectomy, and/or use of nasal steroids. Conclusions: DP without tracheostomy can be successfully achieved in patients with CCHS. Snoring and obstructive apneas, when present, can be managed by diaphragm pacer changes and medical therapies. Obesity can pose a challenge to successful DP.

Congenital central hypoventilation syndrome: diagnosis and management

PURPOSE Congenital central hypoventilation syndrome (CCHS), or Ondines curse, is a rare disorder affecting central respiratory drive. Patients with this disorder fail to ventilate adequately and require lifelong ventilatory support. Diaphragm pacing is a form of ventilatory support which can improve mobility and/or remove the tracheostomy from CCHS patients. Little is known about complications and long-term outcomes of this procedure. METHODS A single-center retrospective review was performed of CCHS patients undergoing placement of phrenic nerve electrodes for diaphragm pacing between 2000 and 2012. Data abstracted from the medical record included operation duration, ventilation method, number of trocars required, and postoperative and pacing outcomes. RESULTS Charts of eighteen patients were reviewed. Mean surgical time was 3.3±0.7 hours. In all cases except one, three trocars were utilized for each hemithorax, with no conversions to open procedures. Five patients (27.8%) experienced postoperative complications. The mean ICU stay was 4.3±0.5 days, and the mean hospital stay is 5.7±0.3days. Eleven patients (61.1%) achieved their daily goal pacing times within the follow-up period. CONCLUSIONS Thoracoscopic placement of phrenic nerve electrodes for diaphragmatic pacing is a safe and effective treatment modality for CCHS. Observed complications were temporary, and the majority of patients were able to achieve pacing goals.