ALBERT

Description: Hurricane Katrina was one of the worst natural disasters to hit the United States. Pediatric hematology/oncology patients, having unique physical and mental health needs, are particularly vulnerable to social and mental effects of natural disasters. We conducted a study one year after Katrina to determine the impact of the hurricane on those under our care for sickle cell disease and cancer, conducting a two-step survey-based study at Children's Hospital of New Orleans. The first survey was conducted 1 year after Katrina, attempting to identify gaps in healthcare services for pediatric hematology oncology patients that could be remediated in the event of another disaster such as Katrina, e.g., what barriers to care they encountered; and their opinions as to how hurricane preparedness could be improved. Utilizing responses to this survey, we then implemented a "Hurricane Action Plan". Each year, at the beginning of hurricane season, families were given a questionnaire, which asked them about pertinent patient identification data; about their evacuation plan if a hurricane were to fall; about the presence of a hospital in the area to which they would evacuate; which pharmacy they would be using. They were assisted with updating their child's roadmap and were asked to have this updated copy of the roadmap (or other health information) with them in the event of an evacuation. Information given about the child/family's evacuation plan was then scanned to a flash drive by medical staff along with the child's health information. The families were also given information as to where the pediatric hematology oncology clinic would be held in case of evacuation and were instructed on how to contact the caregivers. A subsequent survey was performed 7-8 years later to evaluate the efficacy of those measures taken. In our first post-Katrina survey, more patients were found to be in lower socioeconomic and educational strata. In the first survey, a majority of our patients had evacuated before or immediately after the storm and some were even separated from their children. Only half of the families in our study had an evacuation plan in place for the hurricane and a third of the families did not know where they were going when they evacuated. This uncertainty made it quite difficult for the families to seek pediatric hematology/oncology care in the place of displacement. There was inadequate time for arrangements for referral to be made, for the child's medications to be procured, for an alternate provider to be identified, or even for the health records of the child to become available. A majority of our patient cohort was already at a higher risk of mental health disorders, and more than half the caregivers reported some kind of emotional disturbance in the child after the hurricane. With the second survey 7 years after the "Hurricane Action Plan" came into effect, we found that in subsequent hurricanes affecting New Orleans and the Gulf Coast, a significant number of families were still being displaced. However, a majority of families (78%) had a plan in place for evacuation and care of their child. This was a significantly better response than prior to Katrina. Families were now more likely to have a store of medications to last for at least 2 weeks. 92 % found the information given to them about alternative facilities and other possible care providers to be extremely helpful. We have found that the communication between families and those of us at CHNOLA who provide care for these children to be vital and hurricane preparedness to be a success. Disclosures No relevant conflicts of interest to declare.

Description: Introduction: Pulmonary Embolism (PE) is relatively rare in children but is associated with significant morbidity and many acute and chronic complications. Currently, based on ACCP guidelines, unfractionated heparin or low-molecular-weight heparin (LMWH) remains standard therapy for children with PE and systemic thrombolysis is reserved for life threatening thrombosis due to concerns with bleeding. Thrombolytic therapy has been shown to be more efficacious than anticoagulation alone in adults with PE but similar data in pediatric population is lacking. Specific guidelines for use of tissue plasminogen activator (TPA) in children with PE were created at Children's Hospital of New Orleans (CHNOLA) in 2008. We have used low dose systemic TPA (0.03 mg/kg/hr initially, increased to 0.05 mg/kg/hr if less than optimal response is seen within 24 hrs) together with unfractionated heparin (5 units/kg/hr) to achieve a goal PTT of 40-60 seconds. Thrombolytic therapy is continued until clot resolution is achieved, but not for a period longer than 72 hrs, at which point patients are transitioned to LMWH. The aim of our study was to determine the effectiveness of systemic low dose TPA therapy in treatment of PE in the pediatric population and also to determine if there are increased bleeding complications. Methods: After IRB approval, a retrospective chart review of patient incidences (PI) with PE and treated with systemic TPA at CHNOLA between June 2008 and June 2014 was performed. Underlying risk factors for thrombophilia were noted. Responses were classified as complete resolution of thrombus (CR), improvement but no complete resolution (PR) or no change in thrombus (NR) based on imaging at the end of TPA treatment. Data were analyzed to determine the efficacy of low dose TPA in treatment of PE. Results: During the specified time, 16 PI were identified for which low dose systemic TPA therapy was used in children with PE. Median age was 13 years. Mean duration of TPA treatment was 51 hrs (range 17-72 hrs). All patients received simultaneous therapy with unfractionated heparin. Seven PI needed an increase in the dose of TPA infusion to 0.05 mg/kg/hr after 24 hrs due to partial or no response to therapy. Nine (56%) PI had complete response, six (38%) were partial responders and one (6%) was classified as a non-responder. All PI were switched to LMWH at the end of systemic TPA. Duration of therapy with TPA did not affect response to treatment (p = 0.16) . There were no major bleeding problems. Furthermore, there were no complications that required TPA to be stopped. Table shows all the 16 PI, risk factors, duration/response to treatment and complications. Table Age (Year) Risk Factor Duration of TPA (Hours) Response Complication 12 Y MTHFR heterozygous + high Homocystein level 72 PR Fecal occult blood positive 21 Y Hip surgery 1 month prior 24 CR No 19 Y Leukemia, Bone marrow transplant 3 weeks prior, central line 24 CR Left hand/forearm hematoma 6 days Prematurity, maternal cocaine use 72 NR No 16 Y Unknown 48 CR No 14 Y H/o Deep vein thrombosis 72 CR No 9 Y Leukemia, Downs syndrome 72 PR No 29 Y History of Fontan procedure 24 PR No 13 Y Downs Syndrome, hypothyroidism 48 CR No 11 Y Diabetes mellitus 72 PR No 11Y Right hip fracture 72 CR Oozing from IV sites 16 Y Oral contraceptive pills 48 CR Oozing from IV sites 17 Y Oral contraceptive pills 72 CR No 9 Y Diabetes mellitus, high lipoprotein a 72 PR Epistaxis 11 Y Right femur surgery 17 CR No 11 Y Diabetes mellitus, high lipoprotein a 72 PR Epistaxis Conclusions: To our knowledge, this is the first clinical study of low dose TPA use for PE in pediatric patients. Our results show that low dose TPA therapy is effective and relatively safe in treatment of PE in children. Further large-scale prospective trials are needed to validate the results of our study. Disclosures No relevant conflicts of interest to declare.

Description: Temporal lobe epilepsy or limbic epilepsy lacks effective therapies due to a void in understanding the cellular and molecular mechanisms that set in motion aberrant neuronal network formations during the course of limbic epileptogenesis (LE). Here we show in in vivo rodent models of LE that the phospholipid mediator platelet-activating factor (PAF) increases in LE and that PAF receptor (PAF-r) ablation mitigates its progression. Synthetic PAF-r antagonists, when administered intraperitoneally in LE, re-establish hippocampal dendritic spine density and prevent formation of dysmorphic dendritic spines. Concomitantly, hippocampal interictal spikes, aberrant oscillations, and neuronal hyper-excitability, evaluated 15–16 weeks after LE using multi-array silicon probe electrodes implanted in the dorsal hippocampus, are reduced in PAF-r antagonist-treated mice. We suggest that over-activation of PAF-r signaling induces aberrant neuronal plasticity in LE and leads to chronic dysfunctional neuronal circuitry that mediates epilepsy.