Chronic Pancytopenia due to Centrally Mediated Hypothermia in Two Children with Severe Neurological Impairment.

We report on recurrent pancytopenia over five years in two children with severe impairment of the central nervous system. Assessment by hematology did not identify an etiology, including bone marrow biopsy in one. Both patients had sustained normalized blood cell counts following interventions to maintain or return to a temperature above 33 °C. Acute cytopenias following medically induced and environmental hypothermia have been reported. Recurrent pancytopenia due to centrally mediated hypothermia in patients with severe neurological impairment is often not recognized, putting such children at risk for unnecessary testing and transfusions. We provide a practical approach to management that is feasible for caregivers in the home setting with suggestions for monitoring.

Gabapentin Use in the Neonatal Intensive Care Unit.

Gabapentin was used for the treatment of term and preterm infants with suspected visceral hyperalgesia caused by a variety of neurologic and gastrointestinal morbidities. Improved feeding tolerance and decreased irritability were seen, as well as decreased usage of opioids and benzodiazepines. Adverse events occurred with abrupt discontinuation of this medication.

Gabapentin for management of recurrent pain in 22 nonverbal children with severe neurological impairment: a retrospective analysis.

BACKGROUND: Children with severe impairment of the central nervous system (CNS) have a high incidence of distressing symptoms, with many experiencing frequent recurrent pain episodes. OBJECTIVE: The study objective was to describe presenting pain behaviors, daily dose, and response to gabapentin for the management of frequent recurrent pain in this population. METHODS: A retrospective analysis was performed with data from 22 children with severe impairment of the CNS residing at a long-term care facility, treated with gabapentin for recurrent pain behaviors. Response was considered significant if the frequency and severity of symptoms decreased by more than 50% as assessed by nursing staff. RESULTS: Pain behaviors commonly reported included facial grimacing, crying, or moaning. Intermittent increase in muscle tone was identified in 86% (n=19). Gastrointestinal (GI) symptoms occurred in 64% (n=14), including pain localized to the GI tract and vomiting. All were assessed for nociceptive pain sources, many with repeated testing. Most were on medications for spasticity (n=20, 91%) and gastroesophageal reflux disease (GERD) (n=22, 100%) prior to gabapentin use. Of the 22 treated with gabapentin, 21 (91%) had a significant decrease in symptoms. No serious adverse events occurred. The mean gabapentin dose for children five years of age or less (n=11) was 50 mg/kg/day (95% CI 45-56) compared to children older than 11 years (n=11) with a mean dose of 36 mg/kg/day (95% CI 34-38). CONCLUSIONS: Gabapentin appears to be an effective treatment for children with severe impairment of the CNS and recurrent pain behaviors, including intermittent changes in muscle tone. Dosing information can guide treatment trials and future prospective studies.

Treating dyspnea with morphine sulfate in nonverbal children with neurological impairment.

Children with severe neurological impairment (NI) are at risk for recurrent respiratory illness with risk for associated distressing respiratory symptoms as respiratory exacerbations become more frequent. Evidence for treating dyspnea in adults with severe pulmonary disease offers interventions for consideration, including morphine sulfate. This case series of four individuals with severe NI reviews the benefit from morphine for respiratory distress. Information includes descriptors of distress, starting dose, and dose increases. This is the first report to review the use of morphine for the treatment of dyspnea in nonverbal children with severe impairment of the central nervous system.

Supportive and palliative care of children with metabolic and neurological diseases.

PURPOSE OF REVIEW: To review the role of pediatric palliative care (PPC) for children with metabolic and neurological diseases. RECENT FINDINGS: There is a growing body of literature in PPC, though it remains limited for children with metabolic and neurological diseases. Evidence indicates the benefit of PPC. Utilization of PPC programmes can facilitate communication, ensure that families are better informed, improve certainty with decisions, enhance positive emotions, result in fewer invasive interventions at the end of life, and have an impact on location of death. Barriers to utilization of PPC include concern about taking away hope and uncertainty about prognosis. Challenging areas for children with metabolic and neurological diseases include the identification of distressing symptoms and prognostic uncertainty. This article aims to review literature relevant to this group of children, as well as provide a framework when considering specific palliative care needs. SUMMARY: PPC for children with metabolic and neurological diseases can lessen a child's physical discomfort and enhance parental certainty with decision-making. These areas along with other needs throughout the illness trajectory and bereavement are being increasingly met by the growing availability of PPC programmes.

Central hypothermia as a cause of acute pancreatitis in children with neurodevelopmental impairment.

Children with severe neurodevelopmental impairment are at risk for recurrent hypothermia, defined as a temperature of less than 35 degrees C, as a result of hypothalamic dysfunction. Acute pancreatitis following hypothermia from environmental exposure or induced as medical therapy has been reported in adults. In this case series of 10 children (six males, four females) with severe neurodevelopmental impairment and associated hypothermia, five had an episode of acute pancreatitis. These five patients had documented hypothermia, an elevated lipase of greater than 1000U/L, and presenting symptoms of irritability or lethargy along with gastrointestinal symptoms such as feeding intolerance. Four of these five children had no other explanation for pancreatitis; the fifth had multiple gallstones. This case series identifies the risk of acute pancreatitis in children with central hypothermia. Monitoring for resolution upon establishment of euthermia can minimize unnecessary testing and cost.

Gabapentin successfully manages chronic unexplained irritability in children with severe neurologic impairment.

Neurologically impaired children have an increased frequency of recurrent pain and irritability that persist in some despite comprehensive evaluation and management of possible pain sources. We hypothesized that visceral hyperalgesia was a source of chronic unexplained irritability and report the outcome of gabapentin treatment in 9 severely neurologically impaired children. Caregivers reported marked improvement after treatment ranging from 3 months to 3 years. Nystagmus in 1 child was the only noted adverse effect. Visceral hyperalgesia may be a source of unexplained irritability in the neurologically impaired child. Symptoms may improve with gabapentin treatment.