Exchange versus simple transfusion for acute chest syndrome in sickle cell anemia adults

BACKGROUND: There are scant data regarding the relative efficacy of exchange transfusion (XC) versus simple transfusion (ST) for treatment of sickle cell anemia acute chest syndrome (ACS).
STUDY DESIGN AND METHODS: Twenty patients who received XC for ACS were compared with 20 ST patients. Hemoglobin (Hb) levels, platelet and white blood cell counts, lactate dehydrogenase (LDH), indirect bilirubin, and temperature were used to assess disease severity. Primary outcome was postprocedure length of hospital stay; secondary outcome was total length of stay.
RESULTS: Cohorts were similar with regard to age; sex; prior ACS episodes; echocardiogram results; and antibiotic, bronchodilator, and hydroxyurea use. Maximum temperature recorded was higher in the XC group (39.1°C vs. 38.4°C, p = 0.02), but LDH, WBCs, and indirect bilirubin were comparable. Admission Hb levels were higher for XC (XC 8.6 g/dL vs. ST 7.4 g/dL, p = 0.02) and XC had higher peak Hb levels during hospitalization (10.4 ± 1.4 g/dL vs. 9.3 ± 1.0 g/dL, p ≤ 0.01). No differences were demonstrable in postprocedure length of stay (XC 5.6 days vs. ST 5.9 days, p = 0.82) or total length of stay (XC 8.4 days vs. ST 8.0 days, p = 0.76). A total of 10.3 ± 3.0 units were transfused for XC compared to 2.4 ± 1.2 units for ST (p < 0.001).
CONCLUSIONS: Based on postprocedure length of stay or total length of stay, we could not detect a difference in the efficacy of XC compared to ST in populations despite red blood cell product usage fourfold higher in the XC group. We suggest that it is time for an adequately powered, randomized trial to examine the true risk:benefit ratio of XC in ACS.     

Acute chest syndrome of sickle cell disease

As patients with sickle cell anemia (SCA) are living longer, health care practitioners increasingly manage the chronic effects of the disease. Although significant strides in management of children with SCA over the past decade resulted in decreased mortality, less research exists to guide the care of the adult with complications from the disease. This case study reviews the care of a young woman with SCA admitted to the hospital for vaso-occlusive crisis with subsequent acute chest syndrome and acute respiratory distress syndrome. The pathophysiology and management of SCA, vaso-occlusive crisis, and pulmonary and neurologic complications of the disease are discussed.     

Acute chest syndrome in sickle cell disease. Crucial considerations in adolescents and adults

Rapid recognition of acute chest syndrome is essential in patients with sickle cell disease. The condition can be particularly severe in adolescents and adults and often leads to death. In this article, the authors review the challenges of evaluating the syndrome and outline current treatment and supportive care.     

Suboptimal vancomycin levels in critically ill children with sickle cell disease and acute chest syndrome

ObjectivesThis is a pilot retrospective study to assess the effect of glomerular hyper-filtration (GHF) related to sickle cell disease (SCD) on vancomycin clearance and ultimately on therapeutic drug levels in children admitted to the pediatric intensive care unit (PICU) with acute chest syndrome (ACS).MethodThe patients’ steady-state vancomycin trough levels (VTL) and the area under the curve (AUC) were compared with those of age- and gender-matched control group; matching was made at a 1:3 ratio.ResultsTwelve SCD patients with ACS and treated with vancomycin were compared with 36 non-SCD patients (control group). Compared with the control patients, the ACS patients had significantly lower initial serum VTL (median = 6.00 mcg/mL vs. 9.75 mcg/mL) (p = 0.007), and their average VTL were still lower (median = 6.65 mcg/mL vs. 10.00 mcg/mL) post vancomycin dose adjustment (p = 0.039). The time to achieve the therapeutic vancomycin level was significantly longer for the ACS patients (median = 4.75 days) than for the control group (median = 1 day) (p = 0.009). The AUC was also significantly lower in the ACS patients (median = 293 mg*h/L) than in the control group (median = 405.5 mg*h/L) (p = 0.007). The AUC was negatively associated with creatinine clearance (Beta Coefficient = ?0.366, p-value=<0.001) even when adjusted for receiving loading dose, standard dose per weight, and severity of critical illness.ConclusionThese findings support the attributed role of the GHF associated with SCD leading to lower vancomycin level in ACS cases. Therefore, the standard dosing approach for vancomycin in ACS patients may be ineffective. We thus advocate for individualized dosing with careful monitoring of drug levels to account for GHF.     

Bedside ultrasound as a predictive tool for acute chest syndrome in sickle cell patients

Background

Acute chest syndrome (ACS) is the leading cause of death for patients with sickle cell disease (SCD). Early recognition of ACS improves prognosis.

Systemic exposure to morphine and the risk of acute chest syndrome in sickle cell disease

BACKGROUND: The etiology of acute chest syndrome, the most severe complication of the sickle cell crisis, is unknown. OBJECTIVE: Our objective was to assess exposure to morphine as an etiologic factor for acute chest syndrome in sickle cell disease. METHODS: A post hoc analysis of a randomized controlled trial comparing oral with continuous infusion of morphine was performed. Children (aged 5-17 years) with sickle cell crisis were randomized to receive oral sustained-release morphine, 1.9 mg. kg(-1). 12 h(-1), or a continuous intravenous infusion of morphine at 0.04 mg. kg(-1). h(-1) by use of a double-blind, placebo-controlled design. In a subgroup of 15 patients, the pharmacokinetics of morphine and its active metabolite morphine-6-glucuronide were also studied. RESULTS: At baseline, demographic and physiologic characteristics were similar between groups. There were no differences in the number of previous rescue doses per day, painful sites per episode, physician contacts per year, and hospitalizations per year between treatment arms. There was a 2-fold higher morphine area under the concentration-time curve at steady state (AUC(ss)) and a 3-fold higher morphine-6-glucuronide AUC(ss) with oral morphine than with a continuous intravenous infusion of morphine (P <.001 and P <.006, respectively). New onset of acute chest syndrome was 3-fold more prevalent in the oral group (57%) versus the continuous intravenous infusion group (17%) (P <.001). CONCLUSIONS: The risk of acute chest syndrome is significantly associated with high systemic exposure to morphine and its active metabolite morphine-6-glucuronide after oral administration of slow-release morphine. Morphine may facilitate respiratory deterioration by eliciting a decrease in oxygen saturation, by inducing histamine release, or through an as-yet-unidentified mechanism. The safe systemic exposure to morphine in terms of area under the concentration-time curve should be further studied in children with sickle cell disease.     

Self-care in adults with sickle cell disease

This article summarizes the psychometric evaluation of the Chronic Illness Assessment Interview for Sickle Cell Disease (CIAI-SCD), an instrument based on a model of self-care for adult patients with chronic medical conditions. The CIAI-SCD was administered to 104 adults with sickle cell disease. A factor analysis identified three factors that reflected the psychological constructs of Personal Satisfaction and Perceived Control (Factor 1), Feeling Concerned and Worried (Factor 2), and Feeling Supported (Factor 3). Preliminary evidence for the internal consistency, test-retest reliability content validity, and construct validity of the CIAI-SCD was obtained. After further refinement and validation, the CIAI-SCD may be a useful tool for assessing factors related to self-care skills among adults with sickle cell disease.     

Unexpected hemoglobin electrophoresis results following red cell exchange in a sickle cell anemia patient with acute chest syndrome

Acute chest syndrome is a well described complication of sickle cell anemia. It is characterized by fever, pulmonary infiltrates, pleuritic chest pain and abnormal pulmonary auscultation. Transfusion therapy, either simple transfusion of red blood cells or a total red blood cell exchange, is a cornerstone therapy for these patients. Exchange transfusion is preferred when an acute reduction of the hemoglobin S (HbS) concentration is the therapeutic goal since it allows one to rapidly reduce the percent HbS without increasing blood viscosity or volume (Wayne, Kevy and Nathan, Blood 1993; 81:1109-1123). Hemoglobin electrophoresis may be used to monitor the effectiveness of the exchange in decreasing HbS. The post-exchange HbS electrophoresis results which were obtained in this case initially caused confusion. In this report we discuss the findings and the reasons why such results may be occasionally expected in future similar situations.     

Early and late failure of noninvasive ventilation in chronic obstructive pulmonary disease with acute exacerbation

BACKGROUND: Despite recent encouraging results, the use of noninvasive ventilation (NIV) in the management of acute exacerbations in chronic obstructive pulmonary disease (COPD), complicated by acute respiratory failure (ARF), is not always successful. Failure of NIV may require an immediate intubation after a few hours (usually 1-3) of ventilation ('early failure') or may result in clinical deterioration (one or more days later) after an initial improvement of blood gas tension and general conditions ('late failure'). MATERIALS AND METHODS: We enrolled 122 patients affected by COPD complicated by ARF, and treated with NIV. The schedule of NIV provided sessions of 2-6 h twice daily. RESULTS: Ninety-nine (81%) patients showed a progressive improvement of the clinical parameters and were discharged. Among the remaining 23 patients, 13 had an early failure and 10 had a late failure. In the 'success' group and 'late failure' groups we found after an increase of pH 2 h of NIV (from 7.31 +/- 0.05 to 7.38 +/- 0.04 P < 0.001 and from 7.29 +/- 0.03 to 7.36 +/- 0.02 P < 0.001, respectively) and a decrease of PaCO2 (from 80.93 +/- 9.79 to 66.48 +/- 5.95 P < 0.001 and from 85.96 +/- 10.77 to 76.41 +/- 11.02 P < 0.001, respectively). After 2 h of NIV in the 'late failure' group there were no significant changes in terms of pH (from 7.20 +/- 0.10 to 7.28 +/- 0.06) nor PaCO2 (from 92.86 +/- 35.49 to 93.68 +/- 23.68). The 'early failure' group had different characteristics and, owing to more severe conditions, the value of pH, of Glasgow Coma Score, and Apache II Score were the best predictors of the failure; while, among the complications on admission, metabolic alterations were the only independently significant predictor. CONCLUSIONS: Our study confirms that NIV may be useful to avoid intubation in approximately 80% of patients with COPD complicated by moderate-severe hypercapnic respiratory failure.     

Enhancing self-management in children with sickle cell disease through playing a CD-ROM educational game: a pilot study

PURPOSE: To determine whether playing a simple CD-ROM educational game (developed specifically for children with sickle cell disease), improved children's knowledge and confidence in selected symptom management and practice. METHOD: Twenty-two eligible children completed a pretest to determine knowledge and confidence levels, played the Sickle Cell Slime-O-Rama Game, then completed an identical posttest. FINDINGS: Significant increases in knowledge (t = 2.828, p =.010) and confidence (t = 3.759, p =.001) levels between pre- and posttests were identified. CONCLUSIONS: It is promising that a simple, interacting CD-ROM game allowed children with sickle cell disease to quickly acquire knowledge about the disease and symptom management, and increased their confidence to apply this new knowledge. Results suggest the high utility of this tailored game to foster active self-management behaviors in this population.     

Rescue treatment for noninvasive ventilation failure due to interface intolerance with remifentanil analgosedation: a pilot study

Purpose  

To assess the feasibility of remifentanil-based sedation in hypoxemic acute respiratory failure (HARF) patients refusing to continue noninvasive ventilation (NPPV) for intolerance to two different interfaces—helmet and total face mask.     

Opioid doses and acute care utilization outcomes for adults with sickle cell disease: ED versus acute care unit

Background

Acute care units (ACUs) with focused sickle cell disease (SCD) care have been shown to effectively address pain and limit hospitalizations compared to emergency departments (ED), the reason for differences in admission rates is understudied. Our aim was compare effects of usual care for adult SCD pain in ACU and ED on opioid doses and discharge pain ratings, hospital admission rates and lengths of stay.

无创正压通气治疗创伤性湿肺的临床研究

目的 观察无创正压通气（NIPPV）治疗创伤性湿肺的疗效。方法 选择30例行无创正压通气治疗的创伤性湿肺患者为治疗组，24例未用NIPPV治疗的创伤性湿肺患者为对照组，通过对2组患者进行心电监测、血氧饱和度、呼吸频率、血气分析等指标的监测，比较2组患者呼吸频率和动脉血气参数、肺部病变吸收时间、气管插管率、ICU住院时间及预后。结果 30例创伤性湿肺患者经NIPPV治疗后动脉血气指标与临床表现明显改善（P〈0．01）；与对照组相比，NIPPV治疗组患者临床症状改善时间、肺部病变吸收时间、ICU住院时间明显缩短（P〈0．01），肺实变率显著减低（P〈0．05），但2组患者气管插管率和病死率差异却无显著性。结论 NIPPV通过调节压力支持与呼吸末正压水平，早期应用可明显改善创伤性湿肺患者病情及缩短病程，但并不能改善其预后。     

Extracellular hemin crisis triggers acute chest syndrome in sickle mice

The prevention and treatment of acute chest syndrome (ACS) is a major clinical concern in sickle cell disease (SCD). However, the mechanism underlying the pathogenesis of ACS remains elusive. We tested the hypothesis that the hemolysis byproduct hemin elicits events that induce ACS. Infusion of a low dose of hemin caused acute intravascular hemolysis and autoamplification of extracellular hemin in transgenic sickle mice, but not in sickle-trait littermates. The sickle mice developed multiple symptoms typical of ACS and succumbed rapidly. Pharmacologic inhibition of TLR4 and hemopexin replacement therapy prior to hemin infusion protected sickle mice from developing ACS. Replication of the ACS-like phenotype in nonsickle mice revealed that the mechanism of lung injury due to extracellular hemin is independent of SCD. Using genetic and bone marrow chimeric tools, we confirmed that TLR4 expressed in nonhematopoietic vascular tissues mediated this lethal type of acute lung injury. Respiratory failure was averted after the onset of ACS-like symptoms in sickle mice by treating them with recombinant hemopexin. Our results reveal a mechanism that helps to explain the pathogenesis of ACS, and we provide proof of principle for therapeutic strategies to prevent and treat this condition in mice.     

Acute chest syndrome in sickle cell disease/HBE patient,A case report

The presented case will shed some light on one of the rarest complications of HBSE disease, which is acute chest syndrome, and will highlight the management of that complication.