Kristen Nelson McMillan

Pain is a common problem affecting clinical outcomes in adults with cystic fibrosis

BACKGROUND As the cystic fibrosis (CF) population has aged, many chronic health problems have emerged, including diabetes mellitus and osteoporosis. Previous studies have suggested that pain is common in patients with CF; however, little is known about the factors associated with it or its impact on clinical outcomes. We hypothesized that pain is common, is associated with psychologic distress, and adversely affects clinical outcomes. METHODS From February 1, 2008, to April 3, 2008, adults with CF from Johns Hopkins Hospital were surveyed about their pain. Outcomes were assessed for 12 months following survey completion. Bivariate analyses were performed using Wilcoxon log rank, Kruskal-Wallis tests, and Spearman correlations. Logistic regression models and Cox proportional hazard models were used to analyze clinical outcomes. RESULTS Eighty-three patients (61%) completed the survey. Eighty-two percent of patients reported pain within the past month, the most common sites being the head, sinuses, back, and chest. Pain frequently interfered with general activities (41.9%), mood (56.8%), and work (47.3%). Symptoms of depression and anxiety, as well as lower quality-of-life (QOL) scores, were associated with the presence of pain (P < .05 for each). The risk of pulmonary exacerbations was increased in patients with higher levels of pain, even after adjusting for FEV(1) and age (OR = 1.65; P = .038; 95% CI, 1.03-2.64). Additionally, the risk of death was higher in patients with higher average pain scores (HR = 2.28; P = .008; 95% CI = 1.2-4.2). CONCLUSIONS Pain is common in adults with CF, interferes with activities, and is associated with lower QOL and an increased risk of both exacerbations and death.

Inhaled Epoprostenol Therapy for Pulmonary Hypertension: Improves Oxygenation Index More Consistently in Neonates than in Older Children:

The purpose of this study was to determine the efficacy of inhaled epoprostenol for treatment of acute pulmonary hypertension (PH) in pediatric patients and to formulate a plan for a prospective, randomized study of pulmonary vasodilator therapy in this population. Inhaled epoprostenol is an effective treatment for pediatric PH. A retrospective chart review was conducted of all pediatric patients who received inhaled epoprostenol at a tertiary care hospital between October 2005 and August 2007. The study population was restricted to all patients under 18 years of age who received inhaled epoprostenol for greater than 1 hour and had available data for oxygenation index (OI) calculation. Arterial blood gas values and ventilator settings were collected immediately prior to epoprostenol initiation, and during epoprostenol therapy (as close to 12 hours after initiation as possible). Echocardiograms were reviewed during two time frames: Within 48 hours prior to therapy initiation and within 96 hours after initiation. Of the 20 patients in the study population, 13 were neonates, and the mean OI for these patients improved during epoprostenol administration (mean OI before and during therapy was 25.6±16.3 and 14.5±13.6, respectively, P=0.02). Mean OI for the seven patients greater than 30 days of age was not significantly different during treatment (mean OI before and during therapy was 29.6±15.0 and 25.6±17.8, P=0.56). Improvement in echocardiographic findings (evidence of decreased right-sided pressures or improved right ventricular function) was demonstrated in 20% of all patients. Inhaled epoprostenol is an effective therapy for the treatment of selected pediatric patients with acute PH. Neonates may benefit more consistently from this therapy than older infants and children. A randomized controlled trial is needed to discern the optimal role for inhaled prostanoids in the treatment of acute PH in childhood.

Tracheostomy in children with congenital heart disease: a national analysis of the Kids’ Inpatient Database

Background. While single-institution studies reported the indications and outcomes of tracheostomy in children with congenital heart disease (CHD), no national analyses have been performed. We sought to examine the indications, performance, outcomes, and resource utilization of tracheostomy in children with CHD using a nationally representative database. Methods. We identified all children undergoing tracheostomy in the Kids’ Inpatient Database 1997 through 2009, and we compared children with CHD to children without CHD. Within the CHD group, we compared children whose tracheostomy occurred in the same hospital admission as a cardiac operation to those whose tracheostomy occurred without a cardiac operation in the same admission. Results. Tracheostomy was performed in n = 2,495 children with CHD, which represents 9.6% of all tracheostomies performed in children (n = 25,928), and 3.5% of all admissions for children with CHD (n = 355,460). Over the study period, there was an increasing trend in the proportion of all tracheostomies that were done in children with CHD (p < 0.0001) and an increasing trend in the proportion of admissions for children with CHD that involved a tracheostomy (p < 0.0001). The population of children with CHD undergoing tracheostomy differed markedly in baseline characteristics, outcomes, and resource utilization. Similarly, the subgroup of children whose tracheostomy was performed in the same admission as a cardiac operation differed significantly from those whose tracheostomy was not. Conclusions. Tracheostomy is an increasingly common procedure in children with CHD despite being associated with significantly greater resource utilization and in-hospital mortality. The population of children with CHD who undergo tracheostomy differs markedly from that of children without CHD who undergo tracheostomy, and important differences are observed between children who undergo tracheostomy in the same admission as a cardiac surgical procedure and those who undergo tracheostomy in a nonsurgical admission, as well as between children with single-ventricle physiology and children with two-ventricle physiology.

Association of a Clinical Practice Guideline With Blood Culture Use in Critically Ill Children

Importance Sepsis and septic shock are common and, at times, fatal in pediatrics. Blood cultures are often obtained when clinicians suspect sepsis, yet are low-yield with a false-positive rate up to 50%. Objectives To determine whether a novel, 2-part, clinical practice guideline could decrease the rates of total blood cultures and cultures collected from central venous catheters in critically ill children and to examine the effect of the guideline on patient outcomes. Design, Setting, and Participants A retrospective cohort study was performed to determine the effect of a new clinical practice guideline on blood culture practices in a 36-bed, combined medical/surgical pediatric intensive care unit of an urban, academic, tertiary care center from April 1, 2013, to March 31, 2015. All patients admitted to the pediatric intensive care unit with length of stay of 4 hours or more were evaluated (4560 patient visits: 2204 preintervention, 2356 postintervention visits). Interventions Two documents were developed: (1) fever/sepsis screening checklist and (2) blood culture decision algorithm. Clinicians consulted these documents when considering ordering blood cultures and for guidance about the culture source. Main Outcomes and Measures Primary outcome was the total number of blood cultures collected per 100 patient-days. Results Of the 2204 children evaluated before the intervention, 1215 were male (55.1%); median (interquartile range) age was 5 (1-13) years. Postintervention analysis included 2356 children; 1262 were male (53.6%) and median (interquartile range) age was 6 (1-13) years. A total of 1807 blood cultures were drawn before the intervention during 11 196 patient-days; 984 cultures were drawn after the intervention during 11 204 patient-days (incidence rate, 16.1 vs 8.8 cultures per 100 patient-days). There was a 46.0% reduction after the intervention in the blood culture collection rate (incidence rate ratio, 0.54; 95% CI, 0.50-0.59). After the intervention, there was an immediate 25.0% reduction in the rate of cultures per 100 patient-days (95% CI, 4.2%-39.7%; P = .02) and a sustained 6.6% (95% CI, 4.7%-8.4%; P < .001) monthly decrease in the rate of cultures per 100 patient-days. Significantly fewer cultures were collected from central venous catheters after vs before the intervention (389 [39.5%] vs 1321 [73.1%]; P < .001). Rates of episodes defined as suspected infection and suspected septic shock decreased significantly after the intervention, but patients meeting these criteria underwent cultures at unchanged frequencies before vs after the intervention (52.1% vs 47.0%, P = .09, compared with 56.7% vs 55.0%, P = .75). In-hospital mortality (45 [2.0] vs 37 [1.6]; P = .23) and hospital readmissions (107 [4.9] vs 103 [4.4]; P = .42) were unchanged after the intervention. Conclusions and Relevance A systematic approach to blood cultures decreased the total number of cultures and central venous catheter cultures, without an increase in rates of mortality, readmission, or episodes of suspected infection and suspected septic shock.

A survey of perioperative management of sickle cell disease in North America

Background:  Children with sickle cell disease frequently undergo surgical procedures that are associated with acute exacerbations of the disease. Current perioperative management practices are unclear.

Unusual sudden cardiac death from an anomalous left coronary artery from the right sinus of Valsalva.

A left coronary artery arising from the right sinus of Valsalva is a rare congenital coronary anomaly. We report a case of a 5-year-old boy with an anomalous left coronary artery from the right sinus of Valsalva whose presenting sign was cardiac arrest. There is no reported instance of a child <9 years of age without other congenital cardiac defects having died suddenly with this coronary anomaly. The transthoracic echocardiogram demonstrated normal origins of the coronary arteries, but on autopsy, an anomalous origin of the left main coronary artery from the right sinus of Valsalva was found.

Prenatal ABO/RHD Genotyping: A New Paradigm to Allow for Fresh Whole Blood for Cardiopulmonary Bypass in the Immediate Newborn Period

Compared to standard component therapy, fresh whole blood (FWB) offers potential benefits to neonates undergoing cardiopulmonary bypass (CPB) in the context of open cardiac surgery: decreased blood loss and subsequent risk of volume overload, improved coagulation status, higher platelet counts during and following CPB, circumvention of limited vascular access, and significantly reduced donor exposures. Obtaining FWB, however, entails 2–5 days of preparation, which often precludes its availability for neonates requiring CPB in the immediate newborn period. Using a multidisciplinary approach and molecular ABO/RHD genotyping on amniotic fluid, we developed a protocol to allow procurement of FWB for timed delivery followed by open cardiac surgery. Eligible subjects include patients undergoing genetic amniocentesis following the diagnosis of a fetal cardiac anomaly likely to require open surgical repair in the initial days after birth. This protocol has been successfully implemented following prenatal diagnosis of severe fetal cardiac anomalies. Taking advantage of the prenatal time period and the ability to perform fetal blood typing prenatally using molecular genotyping makes possible a new paradigm for the availability of FWB for CPB to improve perioperative, short-term, and long-term outcomes in a population comprised of some of the smallest and sickest patients who will undergo CPB.

1302: CLINICIAN ULTRASOUND SCREENING FOR CATHETER-ASSOCIATED THROMBOSIS IN CRITICALLY ILL CHILDREN

www.ccmjournal.org Critical Care Medicine • Volume 46 • Number 1 (Supplement) Learning Objectives: Vascular catheter associated thrombosis (CAT) is an iatrogenic hazard to patient safety in the ICU as a source for ischemic and embolic vessel disease. This is particularly significant for the young patient, due to vessel obstruction from adult-sized catheters, sizedependent anticoagulant dosing, and projected life-years affected by CAT or its sequelae. Despite the crucial importance of durable vascular access in the critically ill child, an organized approach for screening for CAT in children and infants with central venous access is nonexistent. Methods: This is an IRB-approved pilot study examining the prospective use of ultrasound (US) by pediatric intensive care unit (PICU) practitioners in early identification of symptomatic CAT at a medical and surgical PICU. Patients < 18 years receiving central venous catheters in vessels, without previous thrombotic disease, were approached by the study team for consent. Enrolled patients received US examinations daily when safe. A simplified 4 image US protocol that could be completed in less than 15 min. was implemented, and thrombosis was defined as evidence of vessel noncompressibility in a region demonstrating a paucity of Doppler-detectable blood flow. Results: 25 catheters (14 CVC, 11 PICC) have been examined in 22 patients ranging from < 1 month to 16 years. 11 (44%) of the catheters demonstrated thrombosis confirmed by radiologist secondary read. 7 (28%) of the thrombi caused obstructive symptoms and the other four were asymptomatic. 10 of 11 thrombi were detected with CUS before other indicators of CAT were recognized. 2 patients identified with CAT by clinicians were negative according to a full diagnostic US (DUS). No patients with confirmed CAT were missed using CUS. This yielded a sensitivity of 1.00 and specificity of 0.86 (PPV 0.85, NPV 1.00) and suggests the modality is useful for screening. 3 patients identified with CAT using CUS did not demonstrate thrombosis using DUS, however the presence of CAT was confirmed by radiologist interpretation of the CUS images. The frequency of CAT in this population (44%) is higher than that reported in retrospective studies published to date (2–18%), and suggests that CAT can be readily missed in the ICU. Conclusions: CAT is detectable by clinicians with excellent screening accuracy using available PICU US equipment. Ongoing development and optimization of ICU US protocols for CAT screening is warranted to reduce the burden of thromboembolic disease in the critically ill patient.

Non-rheumatic streptococcal myocarditis mimicking acute myocardial infarction in an adolescent male

An adolescent male with a recent history of streptococcal pharyngitis presented with severe substernal chest pain, troponin leak, and ST-segment elevation, which are suggestive of acute inferolateral myocardial infarction. The coronary angiogram was normal. The patient was subsequently diagnosed with non-rheumatic streptococcal myocarditis. He was treated with amoxicillin and had excellent recovery. Non-rheumatic streptococcal myocarditis is an important mimic of acute myocardial infarction in young adults.