Hospital to home transport at end of life: Survey of clinician experience.

BACKGROUND: Palliative transport is transport home of patients requiring critical care transport support with expectation of imminent death. Many parents prefer their child's death at home; evidence suggests death in the preferred location improves bereavement outcomes. Little is known about the clinical and demographic diversity of patients receiving palliative transport or the perspectives of participating staff. AIM: The objectives of the present study were to (1) characterize demographic and clinical factors involved in palliative transport, (2) identify challenges encountered, and and (3) ascertain staff perspectives. DESIGN: Ten-year retrospective chart review and cross-sectional staff survey using study-specific questionnaire. SETTING/PARTICIPANTS: Twenty-three patients had palliative transport from a tertiary pediatric hospital from 2004 to 2013, of which 12 met inclusion criteria. Survey responses from 22 participating staff were received. RESULTS: The cohort of 12 patients was 58% female, with a mean (range) age of 5.5 (0.01-22) years; racial composition was not significantly different than the palliative care clinical census over the same time period. Distances under 30 miles accounted for 50% of palliative transports. The majority of patients (75%) died within 2 days of palliative transport. Six unanticipated events are described. Staff reported palliative transport as a positive experience, regarding it as an important job component. However, 63% were dissatisfied or undecided about the plan should the patient die enroute, and 48% experienced some level of dissatisfaction with communication. CONCLUSION: Palliative transport is a feasible option for some patients. Staff experienced palliative transport as valuable, although process concerns were noted. This study underscores the importance of preparedness, training, and education for palliative transports.

End of life care in a level IV outborn neonatal intensive care unit.

OBJECTIVE: Describe care surrounding the end of life (EOL) in the neonatal intensive care unit (NICU). STUDY DESIGN: Retrospective chart review of 208 infants who died in a level IV referral-only NICU over 5 years. RESULTS: A goals of care (GOC) conversation was documented before the day of death for 63% of infants. 73% died following withdrawal of life-sustaining treatment (WD); 13% died in a code. The median age at death was 17.5 days. 72% were held by a parent at EOL. 94% of families desired formal memory-making. We identified associations with mode of death and parental holding at death, including: WD was associated with palliative care consultation, early GOC conversations, and increased unit-specific length of stay. Holding was associated with chaplain visits, memory-making, and increased home-to-hospital distance. CONCLUSION: We present a detailed description of EOL care in an outborn NICU, including novel data on parental holding and memory-making.

Efficacy of intravenous lidocaine to reduce pain and distress associated with propofol infusion in pediatric patients during procedural sedation.

BACKGROUND: Research suggests that young children experience an increased incidence and severity of discomfort during propofol infusion. Evaluations of varied interventions to reduce or eliminate this discomfort with adult subjects suggest that premedication with intravenously administered lidocaine (0.5 mg/kg) offers the best overall effectiveness. OBJECTIVE: Because this regimen's efficacy in a pediatric population is undocumented, we conducted a randomized, double-blind, placebo-controlled study to determine the effectiveness of intravenous lidocaine pretreatment to alleviate pain in pediatric subjects before propofol infusion. METHODS: Subjects (aged 2-7 years) scheduled for painless diagnostic procedures received either a saline placebo or 1 of 2 lidocaine doses before administering propofol. To capture the patient's baseline behavioral state, a trained observer administered the validated face, legs, activity, cry, consolability pain assessment scale before propofol infusion. During deep sedation induction, the sedating physician, a trained research assistant, and the patient's parent documented maximum distress using a 100-mm visual analog scale (VAS). RESULTS: Ninety-one subjects participated. We found no difference in VAS pain scores between groups pretreated with lidocaine 0.25 mg/kg, lidocaine 0.5 mg/kg, and placebo. Statistical analysis also found no interrater differences between parents, physician, or observer VAS scores. CONCLUSIONS: Our data do not support using lidocaine pretreatment to alleviate pain/discomfort in pediatric patients during propofol infusion.

O-fucosylation of thrombospondin type 1 repeats in ADAMTS-like-1/punctin-1 regulates secretion: implications for the ADAMTS superfamily.

The ADAMTS superfamily contains several metalloproteases (ADAMTS proteases) as well as ADAMTS-like molecules that lack proteolytic activity. Their common feature is the presence of one or more thrombospondin type-1 repeats (TSRs) within a characteristic modular organization. ADAMTS like-1/punctin-1 has four TSRs. Previously, O-fucosylation on Ser or Thr mediated by the endoplasmic reticulum-localized enzyme protein-O-fucosyltransferase 2 (POFUT2) was described for TSRs of thrombospondin-1, properdin, and F-spondin within the sequence Cys-Xaa(1)-Xaa(2)-(Ser/Thr)-Cys-Xaa-Xaa-Gly (where the fucosylated residue is underlined). On mass spectrometric analysis of tryptic peptides from recombinant secreted human punctin-1, the appropriate peptides from TSR2, TSR3, and TSR4 were found to bear either a fucose monosaccharide (TSR3, TSR4) or a fucose-glucose disaccharide (TSR2, TSR3, TSR4). Although mass spectral analysis did not unambiguously identify the relevant peptide from TSR1, metabolic labeling of cells expressing TSR1 and the cysteine-rich module led to incorporation of [(3)H]fucose into this construct. Mutation of the putative modified Ser/Thr residues in TSR2, TSR3, and TSR4 led to significantly decreased levels of secreted punctin-1. Similarly, expression of punctin-1 in Lec-13 cells that are deficient in conversion of GDP-mannose to GDP-fucose substantially decreased the levels of secreted protein, which were restored upon culture in the presence of exogenous l-fucose. In addition, mutation of the single N-linked oligosaccharide in punctin-1 led to decreased levels of secreted punctin-1. Taken together, the data define a critical role for N-glycosylation and O-fucosylation in the biosynthesis of punctin-1. From a broad perspective, these data suggest that O-fucosylation may be a widespread post-translational modification in members of the ADAMTS superfamily with possible regulatory consequences.