Response to Kuhse.

In this short paper, we respond to critics of our original paper, The agony of agonal respiration: is the last gasp necessary?. A common thread in both Hawryluck's and Kuhse's responses is the difficulties encountered when using the agent's intentions to make moral distinctions between using neuromuscular blocking drugs to palliate versus using neuromuscular blocking drugs to kill. Although this difficulty does exist we maintain that the intentions of the physician must matter when providing end-of-life care.

The agony of agonal respiration: is the last gasp necessary?

Gasping respiration in the dying patient is the last respiratory pattern prior to terminal apnoea. The duration of the gasping respiration phase varies; it may be as brief as one or two breaths to a prolonged period of gasping lasting minutes or even hours. Gasping respiration is very abnormal, easy to recognise and distinguish from other respiratory patterns and, in the dying patient who has elected to not be resuscitated, will always result in terminal apnoea. Gasping respiration is also referred to as agonal respiration and the name is appropriate because the gasping breaths appear uncomfortable and raise concern that the patient is suffering and in agony. Enough uncertainty exists about the influence of gasping respiration on patient wellbeing, that it is appropriate to assume that the gasping breaths are burdensome to patients. Therefore, gasping respiration at the end of life should be treated. We propose that there is an ethical basis, in rare circumstances, for the use of neuromuscular blockade to suppress prolonged episodes of agonal respiration in the well-sedated patient in order to allow a peaceful and comfortable death.

Predictive value of proton magnetic resonance spectroscopy in pediatric closed head injury.

We studied 26 infants (1-18 months old) and 27 children (18 months or older) with acute nonaccidental (n = 21) or other forms (n = 32) of traumatic brain injury using clinical rating scales, a 15-point MRI scoring system, and occipital gray matter short-echo proton MRS. We compared the differences between the acutely determined variables (metabolite ratios and the presence of lactate) and 6- to 12-month outcomes. The metabolite ratios were abnormal (lower NAA/Cre or NAA/Cho; higher Cho/Cre) in patients with a poor outcome. Lactate was evident in 91% of infants and 80% of children with poor outcomes; none of the patients with a good outcome had lactate. At best, the clinical variables alone predicted the outcome in 77% of infants and 86% of children, and lactate alone predicted the outcome in 96% of infants and 96% of children. No further improvement in outcome prediction was observed when the lactate variable was combined with MRI ratios or clinical variables. The findings of spectral sampling in areas of brain not directly injured reflected the effects of global metabolic changes. Proton MRS provides objective data early after traumatic brain injury that can improve the ability to predict long-term neurologic outcome.

Nasal continuous positive airway pressure use in children with obstructive sleep apnea younger than 2 years of age.

STUDY OBJECTIVES: To assess the efficacy of continuous positive airway pressure (CPAP) in obstructive sleep apnea (OSA) patients who are < 2 years of age. DESIGN: A retrospective chart review of 18 patients from 1992 to 1999 who had OSA confirmed by polysomnography. All patients in this study also completed a separate night of CPAP polysomnography to determine the effectiveness of CPAP in the correction of OSA. Nasal CPAP compliance data were gathered via clinical follow-up examination, telephone interview, or mailed questionnaire. SETTING: All patients were studied in the Sleep Disorders Center at Loma Linda University Children's Hospital in Loma Linda, CA. PATIENTS: All patients were < 2 years old. INTERVENTION: After OSA was confirmed by the results of technician-attended nocturnal polysomnography, separate technician-attended nocturnal CPAP polysomnography was completed. On CPAP nights, CPAP pressure was titrated to ameliorate OSA and snoring. CPAP pressure was increased by 2-cm H(2)O or 1-cm H(2)O increments. RESULTS: Data were analyzed by dependent groups t test at p < 0.05 level of significance. CPAP statistically improved respiratory parameters significantly when compared to baseline polysomnography. The following four patient subgroups emerged from the analysis: group 1 consisted of six patients who had tracheostomies prior to the CPAP trial, with two patients using CPAP as an alternative to tracheostomy; group 2 consisted of two patients who had previous unsuccessful adenostonsillectomies and who used CPAP successfully, with both having OSA resolution over time; group 3 consisted of four patients who did not tolerate CPAP on the study night; and group 4 consisted of six patients who used CPAP nightly, had OSA resolution over time, and therefore, no longer needed CPAP therapy. Thus, 10 of 18 patients used CPAP either on an interim basis for corrective therapy or as a primary treatment modality for OSA. CONCLUSIONS: These data show that children < 2 years of age can tolerate and use CPAP effectively. In several cases, CPAP treatment could be discontinued as OSA resolved over time. The reasons for this are discussed in the text.

Comparison of intubation skills between interfacility transport team members.

OBJECTIVE: To compare intubation skill level and success rate between interfacility transport team members. DESIGN: Prospective collection of data. SETTING: University affiliated children's hospital interfacility transport team. PATIENTS: One hundred thirty-two pediatric patients (age range 4 days to 11 years) intubated prior to transport by a specialized team. INTERVENTIONS: None. METHODS: Prospective data was gathered from June 1992 November 1996. In 3616 transports reviewed, 132 intubations were performed by the team at the referring facility. Patient ages ranged from 4 days to 11 years with a mean age of 23 months. We compared resident physicians and respiratory care practitioners (RCPs) to a standard threshold of 1 attempt per successful intubation. An attempt was defined as passage of the endotracheal tube into the oropharynx in an effort to pass it through the vocal cords. Patients were sedated and paralyzed for the procedures. The physicians were 2nd and 3rd year pediatric or emergency medicine residents. They received intubation training in Pediatric Advanced Life Support (PALS), Neonatal Resuscitation Program (NRP), and during rotations through neonatal and pediatric intensive care units. RCPs had an average of 3.5 years of experience overall on the transport team. They received training primarily on mannequins and written tests while in school. They were certified in PALS and NRP and required to participate in annual skill laboratories, which consisted of mannequin intubations and a written examination. RESULTS: The results showed the RCPs to have greater overall success as well as greater success of intubation on first attempt compared to the resident physicians. CONCLUSION: In our experience, RCPs on the interfacility transport team were very successful in performing endotracheal intubations and were more successful than resident physicians. RCPs are established members of not only the transport team, but also the intensive care units and, therefore, should be considered qualified to routinely perform endotracheal intubations in those settings as well.

Sleep-disordered breathing in infants and children.

Sleep-disordered breathing (SDB) is underdiagnosed in infants and children. In addition to causing physical ailments that range from failure to thrive to cor pulmonale, SDB is often an unrecognized cause of failure in school or of behavioral disorders. Diagnosis of SDB requires a careful and detailed history and physical examination. Polysomnography is required to determine the nature of the problems, the magnitude of the physiologic disturbance, and, ultimately, the significance of the problem for the child. Earlier recognition, accurate diagnosis, and appropriate treatment should alleviate much of the childhood morbidity associated with these conditions.

Mild head injury in children: identification, clinical evaluation, neuroimaging, and disposition.

Pediatric head injury presents in various degrees of severity. Early intervention in the patient with a severe head injury is the key to preventing secondary central nervous system damage. Patients with a head injury are easily identified, often by clinical examination alone. However, patients with a mild head injury present a challenge to practitioners, particularly in identification, knowing what is important in the clinical evaluation, deciding whether to use neuroimaging, and knowing where to send the child for observation. Use of the Glasgow Coma Score, primary survey, and identification of historic and clinical features that are suggestive of severe head injury may guide pediatric nurse practitioners in providing appropriate medical care and disposition.

Intravenous immunoglobulin as therapy for pediatric Guillain-Barré syndrome.

Seven children with Guillain-Barré syndrome were treated with intravenous immunoglobulin. Median patient age was 5.8 years. A standard dosage of 0.4 g/kg/day for 5 days was administered. Clinical improvement occurred on average within 2.4 +/- 1.3 days of beginning intravenous immunoglobulin. One child required mechanical ventilation for 7 days. Eight comparable children with Guillain-Barré syndrome at our institution in a prior study treated with plasmapheresis alone had similar clinical results. However, the need for admission to the pediatric intensive care unit and duration of pediatric intensive care unit stay were lower in the intravenous immunoglobulin treated group (P < .05). There were no complications with intravenous immunoglobulin therapy except for a brief episode of hypotension in one patient. Review of the literature identified 74 additional children with Guillain-Barré syndrome successfully receiving intravenous immunoglobulin therapy. We suggest intravenous immunoglobulin as initial therapy for pediatric Guillain-Barré syndrome, because it appears equally as effective as plasmapheresis and is associated with fewer complications.

Stress and distress in pediatric nurses: lessons from Baby K.

BACKGROUND: Nurses experience stress and suffering when they care for critically ill and dying patients. Moral distress occurs when nurses are unable to translate their moral choices into moral action. In response to this stress, nurses may experience burnout. OBJECTIVE: To investigate the attitudes and perceptions of the nurses who were ordered by the court to provide long-term care for Baby K, a female child with anencephaly. METHOD: A questionnaire was developed to provide descriptive insight into the stress and distress the nurses experienced while caring for Baby K. RESULTS: Nurses caring for Baby K experienced stress and suffering. Although they felt supported and respected as nurses in their workplace, they felt conflict with the legal system. To compensate for feelings of powerlessness, the nurses simply put on a professional face and continued to provide care. They feared making mistakes, many felt guilty and angry, and all felt that they were not included in decision making. CONCLUSIONS: (1) Suffering among caregivers occurs and must be recognized, (2) measures must be taken to reduce the stress and distress of healthcare professionals as they provide care to patients who cannot recover, and (3) in addition to these measures, society must provide guidance to healthcare professionals, especially concerning the care of patients who are permanently unconscious.

Outcome and acute care hospital costs after warm water near drowning in children.

OBJECTIVE: Predictive efforts using individual factors or scoring systems do not adequately identify all intact survivors, and therefore all drowning victims are aggressively resuscitated in most emergency departments. More reliable outcome prediction is needed to guide early treatment decisions. METHODS: The charts of 274 near drowning patients admitted to Loma Linda University Children's Hospital were retrospectively reviewed. Patient outcome was categorized into good (near normal function), and poor (vegetative or dead) categories. Discriminant analysis was used to identify combinations of variables most able to predict outcome and a clinical classification system was constructed. The acute care hospital costs for each group were compared. RESULTS: Discriminant analysis classification achieved 95% accuracy, predicting death in 6 intact survivors. No combination of variables could accurately separate all intact survivors from the vegetative and dead groups. The clinical classification method achieved 93% overall accuracy, predicting death in 5 intact survivors. Of patients predicted to have a poor outcome, 5 (6.3%) survived intact. Children may experience an unpredictable, prolonged vegetative state followed by full recovery. Vegetative patients are the most expensive to care for (consuming 53% of total costs) while intact survivors are the least expensive. The majority of costs were spent on patients with poor outcome. CONCLUSIONS: Individual outcome cannot be reliably predicted in the emergency department; therefore, aggressive resuscitation of near drowning victims should be performed. Decisions to subsequently withdraw life support should be made based on integration of likelihood of survival, high (but not absolute) certainty, and parental/societal issues. The vegetative patients are the most expensive to care for, while intact survivors are least expensive. Reduction of expenditures on patients likely to have vegetative or dead outcome would result in substantial savings, but loss of normal survivors.

1H-magnetic resonance spectroscopy-determined cerebral lactate and poor neurological outcomes in children with central nervous system disease.

By using proton magnetic resonance spectroscopy ((1)H-MRS), cerebral lactate has been shown to be elevated in a wide variety of pediatric and adult neurological diseases. In this study we compared 36 newborns, infants, and children with elevated lactate peaks on (1)H-MRS with 61 patients without an identifiable lactate signal. (1)H-MRS was acquired from the occipital gray and parietal white matter (8 cm3 volume, STEAM sequence with echo time = 20 msec, repetition time = 3.0 seconds) and data were expressed as ratios of different metabolite peak areas (N-acetylaspartate [NA]/creatine [Cr], NA/choline [Ch], and Ch/Cr) and the presence of a characteristic lactate doublet peak at 1.3 ppm. Outcomes (Pediatric Cerebral Performance Category Scale score; PCPCS) were assigned 6 to 12 months after injury. Patients with lactate peaks were more likely to have suffered a cardiac arrest, were more often hyperglycemic, and had lower Glasgow Coma Scale scores on admission. They were also more likely to have abnormal metabolite ratios when compared with age-matched controls or with patients without detectable lactate. Of prognostic importance, patients with increased lactate were more likely to be severely disabled (39% vs 10%), survive in a persistent vegetative state (13% vs 2%), or have died (39% vs 7%). In contrast, patients with similar conditions without increased lactate were more likely to have had a good outcome (23% vs 3%) or recovered to a mild (38% vs 6%) or moderate disability (20% vs 0%). Our data suggest that (1)H-MRS is useful in the prediction of long-term outcomes in children with neurological disorders. Patients with elevated cerebral lactate are more likely to die acutely or are at greater risk for serious long-term disability.

Proton MR spectroscopy after acute central nervous system injury: outcome prediction in neonates, infants, and children.

PURPOSE: To evaluate the usefulness of proton magnetic resonance (MR) spectroscopy in predicting 6-12-month neurologic outcome in children after central nervous system injuries. MATERIALS AND METHODS: Localized single-voxel, 20-msec-echo-time MR spectra (including N-acetylaspartate [NAA], choline [Ch], creatine and phosphocreatine [Cr]) were obtained in the occipital gray matter in 82 patients and 24 control patients. Patient age groups were defined as neonates (< or = 1 month [n = 23]), infants (1-18 months [n = 31]), and children (> or = 18 months [n = 28]). Metabolite ratios and the presence of lactate were determined. Linear discriminant analysis-with admission clinical data, proton MR spectroscopy findings, and MR imaging score (three-point scale based on severity of structural neuroimaging changes)-was performed to help predict outcome in each patient. Findings were then compared with the actual 6-12-month outcome assigned by a pediatric neurologist. RESULTS: Outcome on the basis of proton MR spectroscopy findings combined with clinical data and MR imaging score was predicted correctly in 91% of neonates and in 100% of infants and children. Outcome on the basis of clinical data and MR imaging score alone was 83% in neonates, 84% in infants, and 93% in children. The presence of lactate was significantly higher in patients with poor outcome than in patients with good-moderate outcomes in all three age groups (neonates, 38% vs 5%; infants, 87% vs 5%; children, 64% vs 10% [chi 2 test, P < .02]). In children with poor outcomes, NAA/Cr ratios were significantly lower in infants (P = .006) and children (P < .001), and NAA/Ch ratios were significantly lower in infants (P = .001) and neonates (P = .05). CONCLUSION: Findings at proton MR spectroscopy helped predict long-term neurologic outcomes in children after central nervous system injury.

Long-term ventilation in children with severe central nervous system impairment.

Absent from the list of indications for long-term ventilation (LTV) is its use for children with severe central nervous system impairment, including those with severe mental retardation or in a permanent vegetative state. Over a two year period, we evaluated eight children with severe CNS dysfunction for whom long-term ventilation was being contemplated. Of these eight patients, three were in a permanent vegetative state and the remainder were severely neurologically impaired, with minimal cognition. The following recommendations were developed: (1) LTV for patients in a permanent vegetative state is inappropriate. (2) In a patient with severe neurologic disease, the process of informed consent must be viewed as dynamic; once the patient's condition is diagnosed, discussion should begin about the likely course of the disease (upper airway obstruction, respiratory failure, or both) and available treatment options. (3) Continued efforts must be made to resolve conflicts between healthcare professionals and surrogates concerning aggressive support of children with severe CNS dysfunction. Discussions should continue even after a decision to provide long-term ventilation is made. (4) Currently, requests by surrogates for LTV in patients with severe neurologic impairment are usually honored because of respect for family values. (5) Physicians and other healthcare professionals should develop an open and fair process for determining inappropriate care. (6) Once LTV is initiated, efforts to transfer the child to home or a long-term care facility should be made. Further life-saving support should be discouraged. (7) Irremediable patient suffering is reason to refuse a surrogate request for LTV. A patient's preservable existence might be so torturous, painful, or filled with suffering that continued medical intervention would be inhumane or abusive.