Improving paediatric advance care planning: Results of a learning needs analysis and simulation-based education programme.

AIM: To assess clinicians' experience, attitudes and confidence with advance care planning (ACP) at a quaternary paediatric referral centre using a learning-needs survey, and then apply this information to develop and examine the feasibility of simulation-based education for this topic. METHODS: An electronic learning-needs survey was distributed to medical, nursing and allied health clinicians from departments who provide primary care for children with life-limiting conditions. Results were incorporated into the design of a simulation-based education session which was piloted with the Royal Children's Hospital Oncology Department. RESULTS: A total of 157 learning-needs surveys were included in analysis, and included quantitative and qualitative responses from nursing, allied health, senior and junior medical staff from intensive care, general and subspecialty medical teams. Most (75.0%) participants had participated in ACP discussions previously. Only 40.1% of participants felt confident to assess appropriate timing of, and 45.2% felt adequately prepared to participate in ACP discussions. Barriers identified were both clinician and patient/parent related, including clinicians not knowing when to address issues (43.9%) or what to say (21.0%). Participants indicated that ACP discussions are most frequently initiated when death is clearly imminent. Following a pilot of simulation-based education with the oncology department, 90% of participants expressed confidence to participate in ACP discussions. CONCLUSIONS: Barriers to paediatric ACP, including lack of clinician training, continue to contribute to delayed conversations. Simulation-based education is a valuable and feasible educational tool that can increase clinicians' understanding and confidence in this area.

Medical retrieval and needs of infants with bronchiolitis: an analysis by gestational age.

AIM: Viral bronchiolitis is the most common lower respiratory tract infection in children less than 12 months of age. Prematurity is an independent risk factor for disease severity. Many infected infants require hospitalisation and those living in regional centres frequently require transfer to metropolitan hospitals capable of providing assisted ventilation. METHOD: We reviewed infants with bronchiolitis transported by the Victorian Newborn Emergency Transport Service between January 2003 and June 2007. We compared the clinical presentation and treatment required by infants born preterm with those of their term counterparts. RESULTS: Of the 192 infants transported, 92 were born preterm. Preterm infants were younger at time of transport (mean post-menstrual age 41 weeks vs. 45 weeks) and were more likely to require invasive ventilation (60% vs. 32%, P < 0.001) and to receive a fluid bolus (47% vs. 34%, P = 0.04) when compared with infants who had been born at term. Apnoea, either as a presenting symptom or in combination with respiratory distress, was more common in the preterm group (70% vs. 36%, P < 0.001). CONCLUSION: Higher illness severity should be anticipated in ex-preterm infants who present with bronchiolitis. Preterm infants with bronchiolitis are more likely to require invasive ventilation and fluid resuscitation than term infants, suggesting the need for a lower threshold for referral and medical retrieval.

Use of continuous positive airway pressure during stabilisation and retrieval of infants with suspected bronchiolitis.

AIM: Infants with viral bronchiolitis are often hospitalised with a proportion requiring respiratory support. The aim of this review was to examine the use of nasal prong continuous positive airway pressure (CPAP) as a management strategy for infants with a diagnosis of bronchiolitis, who required stabilisation and transport to a tertiary centre. METHOD: A retrospective audit of infants with bronchiolitis requiring CPAP during transport between January 2003 and June 2007. RESULTS: Nasal CPAP was initiated in 54 infants with 51 of these (34 ex-preterm, 17 term) subsequently continuing on CPAP during retrieval. Mean CPAP pressure was 7 cmH(2)O. Oxygenation improved between stabilisation and the end of retrieval (P < 0.01). During retrieval, there was no significant increase in transcutaneous CO(2), no infant required endotracheal ventilation and no adverse events were noted. Five infants were intubated within the first 24 h of admission at the receiving hospital. CONCLUSION: This review demonstrated that use of nasal prong CPAP to transport infants with bronchiolitis was a safe management strategy in those with moderate to severe disease severity.

Impact of introducing binasal continuous positive airway pressure for acute respiratory distress in newborns during retrieval: Experience from Western Australia.

AIM: We aimed to review the impact of introducing binasal continuous positive airway pressure (CPAP) for acute respiratory distress in newborns ≥32 weeks gestation during retrieval in Western Australia. METHODS: Retrospective review of newborns ≥32 weeks gestation with acute respiratory distress, transported by the Western Australian Neonatal Transport Service between February 2002 and December 2004. RESULTS: Respiratory management of 369 newborns was examined. CPAP use increased significantly during the study period from 33% in 2002 to 59% in 2004. Overall, endotracheal tube (ETT) ventilation was required in 108 (29%), nasal CPAP in 166 (45%) and cot oxygen in 95 (26%) patients. Twenty-two (13%) newborns in the CPAP group subsequently required ETT ventilation within 24 h: these babies had higher initial oxygen requirements than those successfully transported on CPAP alone. There was no significant morbidity or mortality in patients retrieved on CPAP. CONCLUSION: CPAP was increasingly utilised as an alternative to ETT ventilation for the management of most cases of less severe acute respiratory distress in near-term neonates on retrieval. This review demonstrated that newborns requiring more than 45-50% oxygen at the time of retrieval were more likely to require intubation at a later time, and hence may benefit from intubation at the time of retrieval. A prospective randomised trial would assist in ascertaining the true benefit of CPAP during retrieval in the newborn period.

Intracerebral blood and MRS in neonatal nonketotic hyperglycinemia.

Nonketotic hyperglycinemia is an inborn error of glycine metabolism leading to the accumulation of glycine in the brain. The neonatal form presents in the first days after birth with encephalopathy, seizures. and characteristic "hiccups." Rapid progression can lead to intractable seizures, coma, and respiratory failure. The outcome is invariably poor, and many die before age 1 year. The diagnosis of nonketotic hyperglycinemia is traditionally based on the finding of a raised cerebrospinal fluid-to-plasma-glycine ratio. An elevated glycine peak, using long echo time proton magnetic resonance spectroscopy, is also specific to nonketotic hyperglycinemia. The presence of blood in a cerebrospinal fluid sample is known to render the glycine level uninterpretable. However, there have been no reports of intracerebral blood confounding the cerebral glycine detected on long echo time proton magnetic resonance spectroscopy. We report on an infant who presented with neonatal encephalopathy and imaging findings consistent with hypoxic-ischemic encephalopathy, as well as intracerebral hemorrhage and an abnormal glycine peak on spectroscopy. The source of the abnormal glycine on long echo time proton magnetic resonance spectroscopy was intracerebral blood, highlighting an important confounder in the interpretation of long echo time proton magnetic resonance spectroscopy in suspected nonketotic hyperglycinemia.

Fetal pulmonary artery diameter measurements as a predictor of morbidity in antenatally diagnosed congenital diaphragmatic hernia: a prospective study.

OBJECTIVE: The objective of the study was to examine the size and growth of fetal pulmonary artery diameters in congenital diaphragmatic hernia and assess their correlation with postnatal survival and morbidity outcomes. STUDY DESIGN: Prospective antenatal echocardiographic examination of fetal branch pulmonary arteries were correlated with postnatal survival and respiratory morbidity in cases of congenital diaphragmatic hernia. Receiver operator curves were developed to assess their utilization as predictors of respiratory morbidity and survival. RESULTS: Twenty-one cases were diagnosed antenatally over 3 years. Fifteen subjects survived postnatally. The ipsilateral fetal branch pulmonary artery diameter was smaller throughout gestation in left-sided congenital diaphragmatic hernia (P = .008). The small left pulmonary diameter did not correlate with survival but correlated strongly with morbidity outcomes. Progressive ipsilateral fetal pulmonary artery hypoplasia was demonstrated throughout gestation in subjects who died or had severe morbidity. CONCLUSION: Fetal pulmonary artery diameter measurements correlate with respiratory morbidity in postnatal congenital diaphragmatic hernia and may assist with prediction of outcome. Survival has improved, possibly because of improved postnatal management of congenital diaphragmatic hernia, limiting this measurement in assessing survival.

Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia.

OBJECTIVES: There have been many recent reports of improved survival rates for congenital diaphragmatic hernia (CDH), largely derived from institution-based data. These are often flawed by case selection bias. The objectives of this study were to document the true incidence, management, and outcomes of CDH in a geographically defined population over a 12-year period and to determine the changing trends in these over time. We also sought to ascertain the prenatal and postnatal factors associated with morbidity and death among these infants. METHODS: A retrospective study of all cases of CDH in Western Australia from 1991 to 2002 was conducted. Cases were identified from 5 independent databases within the Western Australian health network, including the Western Australian Birth Defects Registry. All fetuses and neonates diagnosed with CDH in Western Australia during this period were identified, including miscarriages, stillbirths, and terminations of pregnancies in which a diagnosis of fetal CDH had been made, as well as those diagnosed postnatally. Cases not known to involve CDH until diagnosis at autopsy were also included. Infants with diaphragmatic eventration were excluded from the study. Detailed information was obtained from review of maternal and infant medical records. RESULTS: One hundred sixteen cases of CDH were identified. Of these, 71 (61%) infants were born alive and 37 survived beyond 1 year of age (52% of live-born infants, 32% of all cases of CDH). Pregnancies involving 38 (33%) fetuses were terminated electively, 4 (3%) fetuses were aborted spontaneously, and 3 (3%) fetuses were stillborn. Another major congenital anomaly was present in 54 (47%) cases. Twenty-one (18%) cases had other anomalies that were likely to be fatal. Of all cases with an additional major anomaly, 42 (78%) died. Twenty-seven (71%) of 38 fetuses for whom the pregnancy was terminated had another major anomaly. Twenty-three (32%) live-born infants had another major anomaly (4 of which were considered fatal conditions); however, this did not affect their survival rates. Fifty-three percent of cases were diagnosed prenatally, and 49% of these pregnancies were then terminated. Of live-born infants with prenatally diagnosed CDH, 10 (33%) survived beyond 1 year of age. The gestational age at diagnosis did not affect the survival rate for live-born infants. Postnatal diagnosis occurred in 55 (47%) cases. Of these, 41 (74%) case subjects were born alive and diagnosed on clinical grounds after birth. In the remaining 14 cases, the diagnosis was made in postmortem examinations of fetuses from pregnancies that were terminated for other reasons (8 cases) or after spontaneous abortion or stillbirth (5 cases). Significant differences were found between prenatally and postnatally diagnosed live-born infants. Among live-born infants, prenatal diagnosis was associated with a significantly reduced survival rate (33%, compared with 66% for postnatally diagnosed infants). Prenatally diagnosed live-born infants were of lower birth weight and were born at an earlier gestational age. There was no statistically significant difference between the 2 groups in the onset of labor (spontaneous or induced) or in the rate of elective cesarean sections. Prenatally diagnosed live-born infants were more likely to be delivered in a tertiary perinatal center and were intubated more commonly at delivery. No difference was found in the Apgar scores at either 1 or 5 minutes between the groups. Of 71 live-born infants, 37 (52%) survived to 1 year of age. The majority of deaths occurred within the first 7 days of life (44%). Preoperative air leaks occurred for 16 (22%) infants, of whom 14 (88%) died. Factors found to predict death of live-born infants included prenatal diagnosis, right-sided hernia, major air leak, earlier gestational age at birth, lower birth weight, and lower Apgar scores at 1 and 5 minutes. Over the course of the decade, there were significant increases in the proportion of cases in which the diagnosis of CDH was made with prenatal ultrasonography and in the number of live-born infants born at the tertiary perinatal center. The mortality rate for all cases, the mortality rate for live-born infants, and the proportion of pregnancies involving prenatally diagnosed cases that were terminated electively were all greater in the later epoch but not significantly so. CONCLUSIONS: This was a comprehensive, population-based study of CDH, with full case ascertainment, large sample size, and complete outcome data for all cases. The majority of published studies of CDH examined specific patient populations, such as neonates referred to tertiary pediatric surgical centers. Invariably, those studies failed to detect the demise of cases with CDH before arrival at the referral center, whether through termination of pregnancy, in utero fetal demise, or postnatal death occurring before transfer. Exclusion of these cases from calculations of mortality rates results in significant case selection bias. In our study, 35% of live-born infants died before referral or transport. The population of infants reaching the tertiary surgical center represented only 40% of the total cases of CDH. Wide variations in reported survival rates occur throughout the literature. These differences reflect the influence of this case selection bias, as well as variable referral policies and management practices. For our study population, survival rates differed vastly depending on the subgroup analyzed. Ninety-two percent of postoperative infants survived beyond 1 year of age, as did 80% of infants who reached the surgical referral center. However, only 52% of live-born infants, 32% of all cases, and 16% of all prenatally diagnosed cases survived. Therefore, the overall mortality rate for this condition remains high, despite increased prenatal detection, transfer to tertiary institutions for delivery, and advances in neonatal care, and is influenced significantly by the rate of prenatal termination. In our study, 33% of all cases of CDH and 49% of prenatally diagnosed fetuses underwent elective termination of pregnancy. This large number of fetal terminations confounds the accurate assessment of the true outcomes of this condition.