Annie Janvier

Apnea Is Associated with Neurodevelopmental Impairment in Very Low Birth Weight Infants

OBJECTIVE: To determine whether apnea in preterm infants is associated with abnormal neurodevelopmental outcome.METHODS: We determined the number of days that apnea and bradycardia spells were noted by the nursing staff, during the initial hospitalization of 175 preterm infants of less than <1250 g birth weight or <32 weeks gestation who had been enrolled in the follow-up program of the Royal Victoria Hospital. Multiple logistic and multiple linear regression models were constructed to determine the relationships between apnea days and neurodevelopmental impairment at 3 years of age, after correcting for gestation, sex, intrauterine growth restriction, intraventricular hemorrhage, periventricular leukomalacia, pre- and postnatal steroids, and maternal education.RESULTS: A total of 41 infants had neurodevelopmental impairment (Bayley MDI or PDI <70, cerebral palsy or blindness). By multiple logistic regression, an increasing number of days on which at least one apnea occurred, “total apnea days”, and male sex were significantly associated with increasing probability of neurodevelopmental impairment, p<0.01, the sum of days of assisted ventilation and apnea days occurring after extubation was also associated with impairment in a separate regression model, p<0.001. Lower MDI at 3 years was significantly associated with postnatal steroid use, p=0.004. Lower PDI was associated with increasing apnea days, male sex, and postnatal steroid use, p<0.001. Functional impairment (a score on any one of the four dimensions of the Vineland scale <70), found in 17% of the infants, was associated with increasing apnea days, p<0.05. Caffeine treatment was not independently associated with any outcome.CONCLUSION: An increasing number of days that apnea was recorded during hospitalization was associated with a worse outcome. Among the potential explanations for this finding is the possibility that multiple recurrent hypoxic and bradycardic spells may cause brain injury.

The Experience of Families With Children With Trisomy 13 and 18 in Social Networks

BACKGROUND: Children with trisomy 13 and trisomy 18 (T13-18) have low survival rates and survivors have significant disabilities. For these reasons, interventions are generally not recommended by providers. After a diagnosis, parents may turn to support groups for additional information. METHODS: We surveyed parents of children with T13-18 who belong to support groups to describe their experiences and perspectives. RESULTS: A total of 503 invitations to participate were sent and 332 questionnaires were completed (87% response rate based on site visits, 67% on invitations sent) by parents about 272 children. Parents reported being told that their child was incompatible with life (87%), would live a life of suffering (57%), would be a vegetable (50%), or would ruin their family (23%). They were also told by some providers that their child might have a short meaningful life (60%), however. Thirty percent of parents requested “full” intervention as a plan of treatment. Seventy-nine of these children with full T13-18 are still living, with a median age of 4 years. Half reported that taking care of a disabled child is/was harder than they expected. Despite their severe disabilities, 97% of parents described their child as a happy child. Parents reported these children enriched their family and their couple irrespective of the length of their lives. CONCLUSIONS: Parents who engage with parental support groups may discover an alternative positive description about children with T13-18. Disagreements about interventions may be the result of different interpretations between families and providers about the experiences of disabled children and their quality of life.

Moral distress in the neonatal intensive care unit: caregiver's experience

Background:The Neonatal Intensive Care Unit (NICU) can be ethically charged, which can create challenges for health-care workers.Objective:To determine the frequency with which nurses and residents have experienced ethical confrontations and what factors are associated with increased frequency.Design/Methods:An anonymous questionnaire was distributed to nurses in a university center, a high-risk obstetric service, a maternity hospital NICU with 85% in-born patients and an outborn NICU, most of whose preterm admissions are those with surgical complications. Obstetric and pediatric residents in the four universities of the province also received the questionnaire, which included demographics, opinions regarding the gestational age threshold at which resuscitation of a premature infant with bradycardia was appropriate, knowledge of cerebral palsy (CP) outcomes (as an indicator of knowledge about long-term sequelae of prematurity) and questions about ethical confrontation in the NICU.Results:Two hundred and seventy-nine caregivers participated (115 full time nurses and 164 residents). All the distributed questionnaires were completed. Frequent ethical confrontation was reported by 35% of the nurses and 19% of the residents. Among the nurses, moral distress differed significantly between work environments. Nurses working in an out-born NICU and obstetric nurses were more likely to overestimate CP prevalence (P<0.05). Nurses who overestimated CP rates had higher thresholds for resuscitation and were more likely to experience ethical confrontations. Of the residents, 60% were pediatric and 40% obstetric. All groups of residents frequently overestimated the prevalence of CP, and knowledge differed significantly by residency program (P<0.05). The residents who overestimated CP rates had higher thresholds for resuscitation, had more incorrect answers regarding prematurity outcomes and were less likely to have ethical confrontations.Conclusions:A large proportion of nurses and residents report frequent ethical confrontations. Many residents and nurses have limited knowledge of outcomes and high threshold for resuscitation. Ethical confrontation is more common among nurses with poor knowledge about outcomes, and less common in residents with poor knowledge about outcomes.

Antenatal counselling for parents facing an extremely preterm birth: limitations of the medical evidence.

When physicians are asked for a consult for women in premature labour, they face a complex set of challenges. Policy statements recommend that women be given detailed information about the risks of various outcomes, including death, long‐term disability and various specific neonatal problems. Both personal narratives and studies suggest that parents also base their decisions on factors other than the probabilistic facts about expected outcomes. Statistics are difficult to understand at any time. Rational decision‐making may be difficult when taking life‐and‐death decisions. Furthermore, the role of emotions is not discussed in peri‐viability guidelines.

Nobody likes premies: the relative value of patients’ lives

Objective:The objective of this study was to examine whether patient selection or triage requires placing a relative value on human lives and whether the values placed on these lives are consistent with current ethical theories.Study Design:An anonymous questionnaire was administered to groups of physicians and students in Montreal. It presented eight currently incompetent patients with potential neurological sequelae requiring emergency care. Predicted outcomes were explicitly described. Four patients had a predicted 50% survival and a 50% chance of impairment; they were a preterm and a term neonate, a 2-month-old and a 50-year-old. Two already disabled patients, a 7-year-old and an 80-year-old, had 50% predicted survival. A 14-year-old and a 35-year-old had 5% survival, but differing impairment.Respondents were asked if they would resuscitate and in what order they would resuscitate if all needed intervention simultaneously.Result:Eighty-five percent response rate, n=524. The proportion stating they would always resuscitate was smallest for the 80-year-old (18% P<0.001 compared to other patients), then the preterm (35%, P<0.001), then the term and the 50-year-old (53 and 58%, P<0.01). The 2-month-old and the 7-year-old would be resuscitated most frequently (74 and 77%, P<0.01), followed by the patients with 5% survival (64 and 68%, P<0.001). The median order of triage was first the 2-month-old, followed by the 7-year-old, the 14-year-old, the term newborn, the 50-year-old, the 35-year-old, the premature newborn and the 80-year-old.Conclusion:Order of resuscitation was not closely related to the predicted survival, impairment or potential life years gained. Age appeared to have a strong influence, with childrens lives being valued more than the adults’. This tendency was reversed for the newborn infants who were undervalued compared with older children, and most particularly for the premature. The value placed on the life of newborns, in particular the premature, is less than that expected by any objective medical data and was not consistent with any ethical theory that we tested.

The OHRP and SUPPORT

A group of medical ethicists and pediatricians asks for reconsideration of the recent Office for Human Research Protections decision about informed consent in SUPPORT.

LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency

Background The natural history of all known patients with French-Canadian Leigh disease (Saguenay-Lac-St-Jean cytochrome c oxidase deficiency, MIM220111, SLSJ-COX), the largest known cohort of patients with a genetically homogeneous, nuclear encoded congenital lactic acidosis, was studied. Results 55 of 56 patients were homozygous for the A354V mutation in LRPPRC. One was a genetic compound (A354V/C1277Xdel8). Clinical features included developmental delay, failure to thrive, characteristic facial appearance and, in 90% of patients, acute crises that have not previously been detailed, either metabolic (fulminant lactic acidosis) and/or neurological (Leigh syndrome and/or stroke-like episodes). Survival ranged from 5 days to >30 years. 46/56 patients (82%) died, at a median age of 1.6 years. Of 73 crises, 38 (52%) were fatal. The immediate causes of death were multiple organ failure and/or Leigh disease. Major predictors of mortality during crises (p<0.005) were hyperglycaemia, hepatic cytolysis, and altered consciousness at admission. Compared to a group of SURF1-deficient Leigh syndrome patients assembled from the literature, SLSJ-COX is distinct by the occurrence of metabolic crises, leading to earlier and higher mortality (p=0.001). Conclusion SLSJ-COX is clinically distinct, with acute fatal acidotic crises on a backdrop of chronic moderate developmental delay and hyperlactataemia. Leigh syndrome is common. Stroke-like episodes can occur. The Leigh syndrome of SLSJ-COX differs from that of SURF1-related COX deficiency. SLSJ-COX has a different spectrum of associated abnormalities, acidotic crises being particularly suggestive of LRPPRC related Leigh syndrome. Even among A354V homozygotes, pronounced differences in survival and severity occur, showing that other genetic and/or environmental factors can influence outcome.

Whom are we comforting? An analysis of comfort medications delivered to dying neonates.

OBJECTIVES To clarify the use of end-of-life comfort medications or neuromuscular blockers (NMBs) in culturally different neonatal intensive care units (NICUs). STUDY DESIGN Review of medical files of newborns > 22 weeks gestation who died in the delivery room or the NICU during 12 months in four NICUs (Chicago, Milwaukee, Montreal, and Groningen). We compared use of end-of-life comfort medications and NMBs. RESULTS None of the babies who died in the delivery room received comfort medications. The use of opiods (77%) or benzodiazepines (41%) around death was similar in all NICUs. Increasing this medication around extubation occurred most often in Montreal, rarely in Milwaukee and Groningen, and never in Chicago. Comfort medications use had no significant impact on the time between extubation and death. NMBs were never used around death in Chicago, once in Montreal, and more frequently in Milwaukee and Groningen. Initiation of NMB after extubation occurred only in Groningen. CONCLUSION Comfort medications were administered to almost all dying infants in each NICU. Some, but not all, centers were comfortable increasing these medications around or after extubation. In three centers, NMBs were at times present at the time of death. However, only in Holland were NMBs initiated after extubation.

The Epidemic of Multiple Gestations and Neonatal Intensive Care Unit Use: The Cost of Irresponsibility

OBJECTIVES To determine the proportion of infants admitted to our neonatal intensive care unit (NICU) from multiple gestations resulting from artificial reproductive technology (ART), the complications experienced and interventions required by these infants, and the estimated effect of a mandatory policy of single embryo transfer on admissions and complication rates in our hospital and across Canada. STUDY DESIGN We conducted a review of a prospectively maintained database and of hospital records and calculated excess complications compared with either universal single embryo transfer or a policy allowing transfer of two embryos in as many as 33% of women. RESULTS Of our NICU admissions, 17% are infants from multiple gestations after ART, a significant increase in 10 years. In a 2-year period, the excess NICU use that would have been saved by mandatory single embryo transfer included 3082 patient days and 270 patient ventilator days. Extrapolated across Canada, a policy of single embryo transfer would prevent 30 to 40 deaths, 34 to 46 severe intracranial haemorrhages, and 13 to 19 retinal surgeries annually. Savings in NICU resources would be 5424 to 7299 patient-days of assisted ventilation and 35 219 to 42 488 patient-days of NICU care. CONCLUSIONS A mandatory policy of single embryo transfer would be of substantial benefit to the health of Canadian babies while still benefiting infertile couples.

Ethics ain't easy: do we need simple rules for complicated ethical decisions?

Background: Recommendations from national bodies regarding extremely preterm infants have focussed almost exclusively on thresholds for intervention based upon estimated gestational age (GA) alone.