Quality of investigations into unexpected deaths of infants and young children in England after implementation of national child death review procedures in 2008: a retrospective assessment.

OBJECTIVES: In 2008, new statutory national procedures for responding to unexpected child deaths were introduced throughout England. There has, to date, been no national audit of these procedures. STUDY DESIGN: Families bereaved by the unexpected death of a child under 4 years of age since 2008 were invited to participate. Factors contributing to the death and investigations after the death were explored. Telephone interviews were conducted, and coroners' documents were obtained. The nature and quality of investigations was compared with the required procedures; information on each case was reviewed by a multiagency panel; and the death was categorised using the Avon clinicopathological classification. RESULTS: Data were obtained from 91 bereaved families (64 infant deaths and 27 children aged 1-3 years); 85 remained unexplained after postmortem examination. Documentation of multiagency assessments was poorly recorded. Most (88%) families received a home visit from the police, but few (37%) received joint visits by police and healthcare professionals. Postmortem examinations closely followed national guidance; 94% involved paediatric pathologists; 61% of families had a final meeting with a paediatrician to explain the investigation outcome. There was no improvement in frequency of home visits by health professionals or final meetings with paediatricians between 2008-2013 and 2014-2017 and no improvement in parental satisfaction with the process. CONCLUSIONS: Statutory procedures need to be followed more closely. The implementation of a national child mortality database from 2019 will allow continuing audit of the quality of investigations after unexpected child deaths. An important area amenable to improvement is increased involvement by paediatricians.

Pulse oximetry screening for critical congenital heart disease: a comparative study of cohorts over 11 years.

Background: Pulse oximetry (POS) has been proposed as a screening tool for CCHD in newborn. The aim was to identify the effect of POS on the rate of diagnosis after discharge and survival to one year in cases with CCHD.Material and Methods: All cases of CCHD from three tertiary level hospitals in the Northern region of UK between 1st January 2001 and 31st December 2011 were identified from the Northern Congenital Abnormality Survey (NorCAS). A retrospective cohort study comparing screened and unscreened population for CCHD was undertaken. The main outcome was post discharge diagnosis rate and mortality at one year between the cohorts.Results: Total number of births during the 11 years was 138,176. A total of 147 cases had CCHD, 59 diagnosed postnatally. Five and eight cases were diagnosed after discharge in the screened and the unscreened cohort respectively. The rate of post-discharge diagnosis in the screened population was 7/100,000 and 13/100,000 in the unscreened population with a relative risk of 0.52 (CI 0.2 to 1.42). Mortality at one year in postnatally diagnosed cases was five and one in the screened and unscreened cohorts respectively.Conclusion: With good antenatal detection rates, POS did not have a statistically significant impact in identifying cases of CCHD, when added to the present screening process of antenatal ultrasound and postnatal examination. The same conclusion cannot be made for regions with lower antenatal detection rates; perhaps it may be more appropriate to consider pulse oximetry as a screening tool for hypoxemia of any cause.Brief rationaleThis is the first study evaluating the contemporaneous post-discharge diagnosis rate between screened and unscreened populations. The rate of post-discharge diagnosis was 7/100,000 in the screened and 13/100,000 in the unscreened populations. However, this did not achieve statistical significance and in a setting with high antenatal diagnosis a very large study would be required to demonstrate efficacy of POS.

Sudden infant death syndrome (SIDS) and the routine otoacoustic emission infant hearing screening test: an epidemiological retrospective case-control study.

OBJECTIVES: To investigate whether decreased otoacoustic emission (OAE) signal recordings in the right ear are associated with an increased risk of sudden infant death syndrome (SIDS) and to monitor any temporal changes in risk factors. DESIGN: Retrospective case-control study. SETTING: Telephone interviews with families recruited in England between July 2016 and October 2017 who experienced the unexpected death of a child <4 years old since 2008 and control families recruited from maternity wards in Bristol and Birmingham. PARTICIPANTS: We recruited 91 (89%) of the 102 bereaved families who made initial contact, 64 deaths were under 1 year (sudden unexpected death in infancy) of which 60 remained unexplained (SIDS). Of the 220 control families, 194 (88%) follow-up interviews were conducted. We had analysable hearing data for 24 SIDS infants (40%) and 98 controls (51%). RESULTS: OAE signals were marginally increased rather than decreased among SIDS infants for the right ear, especially at lower frequencies, but not significantly so. The strongest predictors of SIDS were bed-sharing in hazardous (infant sleeping next to a carer who smoked, drank alcohol or slept on a sofa) circumstances (35% vs 3% controls, p<0.0001), infants found prone (33% vs 3% controls, p<0.0001) and infants whose health in the final week was 'not good' (53% vs 9% controls, p<0.0001). The prevalence of maternal smoking during pregnancy among both SIDS mothers (20%) and controls (10%) was much lower than previous studies. CONCLUSIONS: Hearing data were difficult to obtain; larger numbers would be needed to determine if asymmetrical differences between the right and left ear were a marker for SIDS. A national prospective registry for monitoring and a renewed campaign to a new generation of parents needs to be considered underlining the initial message to place infants on their backs for sleep and the more recent message to avoid bed-sharing in hazardous circumstances.

"You have to keep your nerve on a DMC." Challenges for Data Monitoring Committees in neonatal intensive care trials: Qualitative accounts from the BRACELET Study.

BACKGROUND: Data Monitoring Committees (DMCs) are essential to the good conduct of many trials. Typically they comprise a small expert group which monitors safety, efficacy, progress and early outcome data as trials recruit. DMCs can recommend protocol revisions and early stopping of a trial. As DMC meetings usually consider unblinded interim data confidentially, their deliberations are seldom exposed to research scrutiny. Although there have been some case studies from trials from mixed specialties which offer insights into some of the common issues faced by DMCs, we have, however, little empirical information about the challenges faced within specific clinical settings. METHODS: In-depth interviews with participants in the BRACELET Study on death and bereavement in neonatal intensive care trials produced qualitative accounts of experiences and views of a subgroup of 18 DMC members. These interviews explored views of DMC members in relation to the clinical context of neonatal intensive care and the conduct of neonatal intensive care trials. RESULTS: Interviewees felt that an understanding of both the neonatal intensive care setting and population was crucial in a DMC. They considered the neonatal intensive care research population especially vulnerable, and that outcomes that included both death and severe disability raised particular challenges rarely faced in other settings. In exploring these key outcomes they were mindful of the need to meet high scientific standards and the needs of babies in the trials and their families. DMC members discussed particular difficulties around the composite outcome of death and severe disability, especially when mortality data were available long before data on longer term disability. While statistical stopping guidance is helpful, DMC members described decisions about stopping, revising or continuing a trial being informed by a wider set of considerations and discussions than a pre-set p value. These included potentially competing needs of current trial participants and future patients, and reflections on the nature of benefit and harm. Given their cognisance of the potential impact and consequences of the decisions made by DMCs in this setting of life, death, and disability, interviewees commonly used the imagery of bravery, and described DMCs either holding or losing their nerve. CONCLUSIONS: DMCs for trials in other fields may also face difficult ethical trade-offs in monitoring composite outcomes. The experience from this sample of DMC members suggest that for neonatal intensive care trials there are some very specific challenges seldom faced elsewhere. The vulnerability of the population, and the different timescales for essential data becoming available to inform decisions, presented particular challenges. We suggest that it is important to consider the challenges raised in other settings to better understand the complex work of these committees and to prepare future generations of DMC members.

Fifteen-minute consultation: Supporting colleagues through a serious incident or complaint.

Being involved in a complaint or serious incident can be a huge stress for a doctor, whether a trainee or consultant. When affected, it is important to be able to look for the support of a colleague or a more senior person, and just as importantly, those not directly involved need to know what to do to provide support. In this piece I consider what 'support' really means, I provide some tips on what to do and what not to do, and I consider how incidents and complaints can need different approaches.

Fifteen-minute consultation: Asking questions: the puzzles and problems model.

People are full of useful advice, especially when you make the transition to the senior doctor grade. We present a very helpful model we have used over some time to aid us when we have issues.

Outcomes of infants with a birthweight less than or equal to 500 g in Northern England: 15 years experience.

AIM: We aimed to evaluate mortality and short-term neonatal morbidity of babies born ≤500 g cared for in the Northern Neonatal Network over a 15-year period. METHOD: Using regional databases, we identified all live-born babies ≥22 weeks gestation and ≤500 g, in North East England and North Cumbria from 1998 to 2012. We quantified major neonatal morbidities and survival to one year. RESULTS: We identified 104 live-born babies ≥22 weeks gestation and ≤500 g (birth prevalence 0.22/1000), of which 49 were admitted for intensive care. Overall one-year survival was 11%, but survival for those receiving intensive care was 22%. There was significant short-term neonatal morbidity in survivors, in particular retinopathy of prematurity and chronic lung disease. CONCLUSION: Survival of babies born weighing ≤500 g in this cohort remains poor despite advances in neonatal care, with considerable short-term neonatal morbidity in survivors. This could be due to a combination of attitudes and a rather conservative approach towards resuscitation and intensive care, and the intrinsic nature of these tiny babies.

Recent advances in the management of infants born <1000†g.

In this review, we survey some significant advances in the medical care of babies <1000 g and we highlight the development of care pathways that ensure optimal antenatal care, which is a prerequisite for good neonatal outcomes. We also suggest that the long overdue development of family integrated care will in the end prove at least as important as the recent medical advances.

Bed-sharing by breastfeeding mothers: who bed-shares and what is the relationship with breastfeeding duration?

AIM: To explore the link between breastfeeding duration and bed-sharing frequency among women reporting a prenatal intention to breastfeed. METHODS: About 870 participants in a randomised breastfeeding trial, recruited at mid-pregnancy, provided weekly snapshots of breastfeeding and bed-sharing behaviour for 26 weeks following birth. Strength of prenatal breastfeeding intent was recorded at recruitment using Likert-type scales. RESULTS: Outcomes were frequency of bed-sharing at home for at least one hour per week, and time to cessation of breastfeeding. There were insufficient data to classify bed-sharing pattern in 192/870 (22%) of mothers. Of the remainder, 44% (299/678) of participants 'rarely' or 'never' bed-shared, 28% (192/678) did so 'intermittently' and 28% (187/678) did so 'often'. These three groups did not differ significantly in marital status, income, infant gestational age, maternal age or delivery mode. Significantly, more participants who bed-shared 'often' reported strong prenatal breastfeeding intent (70% vs. 57% and 56% for 'intermittent' and 'rare' bed-share groups) and attached high prenatal importance to breastfeeding (95% vs. 87% and 82%). Significantly, more women who bed-shared frequently were breastfeeding at 6 months (p < 0.0001) than those who intermittently or rarely/never bed-shared. CONCLUSION: Women with strong motivation to breastfeed frequently bed-share. Given the complex relationship between bed-sharing and sudden infant death syndrome (SIDS) appropriate guidance balancing risk minimisation with support for breastfeeding mothers is crucial.

Patterns of child death in England and Wales.

In the past century, child mortality has fallen to very low rates in all developed countries. However, rates between and within countries vary widely, and factors can be identified that could be modified to reduce the risk of future deaths. An understanding of the nature and patterns of child death and of the factors contributing to child deaths is essential to drive preventive initiatives. We discuss the epidemiology of child deaths in England and Wales. We use available data, particularly that of death registration and other available datasets, and published literature to emphasise issues relevant to reduction of child deaths in developed countries. We examine the different patterns of mortality at different ages in five broad categories of death: perinatal causes, congenital abnormalities, acquired natural causes, external causes, and unexplained deaths. For each category, we explore what is known about the main causes of death and some of the contributory factors. We then explain how this knowledge might be used to help to drive prevention initiatives.

Death, bereavement and randomised controlled trials (BRACELET): a methodological study of policy and practice in neonatal and paediatric intensive care trials.

BACKGROUND: Researchers have seldom included bereaved parents in studies of participants' views of randomised controlled trials (RCTs); hence our understanding of the impact of trials is based on skewed and incomplete samples. Little is known about parental experiences of the death of a child subsequent to their enrolment in a trial or of provision made for this experience by clinicians and trial teams. The Bereavement and RAndomised ControlLEd Trials (BRACELET) study was funded to consider bereavement in the context of paediatric intensive care (PIC) and neonatal intensive care (NIC) trials. DESIGN AND METHODS: The study comprised three interlinked components: a quantitative survey of RCT activity in UK paediatric intensive care units (PICUs) and neonatal intensive care units (NICUs), UK RCT recruitment and mortality rates, and provision for bereavement during 2002-6; a qualitative interview study involving 51 bereaved parents and 59 clinicians and trial team members associated with five neonatal trials; and a methodological study to inform future research. RESULTS: Fifty RCTs were identified as having enrolled babies or children from 2002 to 2006. Approximately 50% of UK NICUs and PICUs (54 NICUs, six PICUs) participated in at least one of these trials. Collectively they enrolled over 3000 children. Most enrolled small numbers, the majority of participants being enrolled by a small group of academic medical units. The proportion of deaths following trial enrolment was 17% in NIC trials and 6% in PIC trials. The qualitative study showed that trial-related decisions were made in a range of circumstances, some after extremely preterm births, others after complicated term deliveries, often under time pressures and in escalating crises. Parents' interest in trials appeared to recede initially but could re-emerge over time. They often valued opportunities to engage with a trial and were interested in more contact and information than they actually received. Clinicians often saw NICU bereavement policies as meeting parental needs, and trial participation as being of relatively minor significance in bereavement. This view may result from the positioning of clinicians' encounters with parents only in the initial stages of grief when trials were not a priority. Trial teams used a range of bereavement strategies, from no further contact to a pioneering multipart follow-up package. Communication with bereaved parents was complicated by limited contact opportunities. Trial teams were obliged to work without knowing whether their communications were appreciated, were problematic, or even whether they were received by parents. The methodological component highlighted strategies for recruitment and data collection in this sensitive setting. Recruitment by unsupported postal contact generally failed and a more personal approach via clinicians was more effective, supplemented by publicity material distributed via trusted organisations. CONCLUSIONS: A co-ordinated response to bereavement is as much a part of the running of trials as recruitment, and needs to be considered at trial inception. BRACELET has demonstrated the value and feasibility of research with bereaved parents involved in NIC trials. In order to respond to bereavement in a fair and sensitive way, as well as to better inform the design of RCTs, it is crucial that we listen to bereaved parents and evaluate new methods for so doing. More research is therefore needed into the experiences of bereavement subsequent to trial enrolment, with study of bereavement strategies in NIC trials as they are introduced. In addition, future studies should determine whether parents and triallists in PIC trials (and trials in adults) face the same issues as in NIC trials. Careful studies are necessary to explore how feedback of trial results are received and understood by bereaved and non-bereaved parents, and how individual trial teams manage this situation. An additional research area for exploring experiences of parenting twins and higher-order births in trials arose from BRACELET. Developmental research should continue to explore means of involving a wider range of parents in future research, including via publicity and specialist websites. Finally, methodological research is needed to ensure that we have the tools to explore, with parents and other relatives, as partners in research, a range of trial-related topics, which might be challenging, as the information is complex or the focus is sensitive. FUNDING: Funding for this study was provided by the Health Technology Assessment programme of the National Institute for Health Research.

The changing profile of infant mortality from bacterial, viral and fungal infection over two decades.

AIM: Infection is an important cause of neonatal and infant mortality. We evaluated changes in infant deaths from infections from 1988 to 2008 in the North of England. METHODS: We interrogated a population-based survey and reviewed infant deaths from infection. Proportional contribution to deaths, pathogens identified and risk factors were analysed. RESULTS: Thirteen percentage of 4366 infant deaths from a population of 704 536 livebirths were infectious. The absolute numbers of infant deaths from infection fell over time but the proportion of deaths from infection increased (12.1%, 13.6% and 14.9%). Significantly preterm infants were increasingly represented in successive epochs (14%, 24% and 38%). Infant mortality rate (IMR) from meningococcus and Group B Streptococcus (GBS) fell in the latest epoch, but there was a corresponding increase from Escherichia coli and candida. DISCUSSION: This large study shows that infections have become proportionately more important causes of death especially in very preterm infants. Recent significant reductions in death from meningococcus and GBS are likely to represent successful achievements of vaccination and antibiotic prophylactic policies. Increases in IMR from E. coli may relate to GBS prophylaxis and increases in candida to the increase from preterm populations. Further efforts to understand these changing patterns and develop additional prevention and treatment strategies and vaccines remain an urgent priority.

Viral infections: contributions to late fetal death, stillbirth, and infant death.

OBJECTIVE: To determine the role of viral infections in causing fetal and infant death. STUDY DESIGN: We assessed a well-validated population database of fetal (≥20 weeks gestation) and infant death for infective deaths and deaths from viruses over a 21-year period (1988-2008). We analyzed by specific viral cause, timing (late fetal loss [20-23 weeks], stillbirth [≥24 weeks], neonatal death [0-27 days], and post-neonatal infant death [28-364 days]) and across time. RESULTS: Of the 989 total infective deaths, 108 were attributable to viral causes (6.5% of late fetal losses, 14.5% of stillbirths, 6.5% of neonatal deaths, and 19.4% of postneonatal infant deaths). Global loss (combined fetal and infant losses per 100,000 registerable births) was 139.6 (95% CI, 130.9-148.3) for any infective cause and 15.2 (95% CI, 12.3-18.1) for viral infections. More than one-third (37%) of viral-attributed deaths were before live birth, from parvovirus (63%) or cytomegalovirus (33%). Parvovirus accounted for 26% (28 of 108) of all viral deaths. Cytomegalovirus was associated with a global loss rate of 3.1 (95% CI, 1.8-4.4) and an infant mortality rate of 1.3 (95% CI, 0.4-2.1) per 100,000 live births; 91% of cases were congenital infections. Herpes simplex virus caused death only after live births (infant mortality rate, 1.4; 95% CI, 0.5-2.3). No changes in rates were seen over time. CONCLUSION: We have identified a substantial contribution of viral infections to global fetal and infant losses. More than one-third of these losses occurred before live births. Considering our methodology, our estimates represent the minimum contribution of viral illness. Strategies to reduce this burden are needed.

Utility of genetic testing in suspected familial cranial diabetes insipidus.

AIM: Differentiating familial cranial diabetes insipidus (CDI) from primary polydipsia can be difficult. We report the diagnostic utility of genetic testing as a means of confirming or excluding this diagnosis. PATIENT AND METHODS: The index case presented at 3 months with polydipsia. He was diagnosed with familial CDI based on a positive family history combined with what was considered to be suspicious symptomatology and biochemistry. He was treated with desmopressin (DDAVP) but re-presented at 5 months of age with hyponatraemia and the DDAVP was stopped. Gene sequencing of the vasopressin gene in father and his offspring was undertaken to establish the underlying molecular defect. RESULTS: Both father and daughter were found to have the pathogenic mutation c.242T>C (p.Leu81Pro) in exon 2 of the AVP gene consistent with a diagnosis of familial diabetes insipidus. The index case did not have the pathogenic mutation and the family could be reassured that he would not require intervention with DDAVP. CONCLUSIONS: Gene sequencing of AVP gene can have a valuable role in predicting whether or not a child is at risk of developing CDI in future. This can help to prevent family uncertainty and unnecessary treatment with its associated risks. LEARNING POINTS: Differentiating patients with familial cranial diabetes insipidus from those with primary polydipsia is not always straightforward.Molecular genetic analysis of the vasopressin gene is a valuable way of confirming or refuting a diagnosis of familial CDI in difficult cases and is a valuable way of identifying individuals who will develop CDI in later childhood. This information can be of great value to families.