Health-related Quality of Life in Children With Sickle Cell Disease Undergoing Chronic Red Cell Transfusion Therapy.

Chronic red cell transfusion (CRCT) therapy is one of few disease-modifying treatments for sickle cell disease (SCD). This study evaluated health-related quality of life (HRQL) in children receiving CRCT relative to 2 comparison groups: children with similar, severe SCD and children with milder disease risk defined by SCD genotype. For this study, 67 children with SCD between the ages of 8 and 18 completed the self-report Pediatric Quality of Life Sickle Cell Disease module (PedsQL SCD) as part of a pilot clinical program during routine hematologic visits. A medical chart review was also performed. Linear regression suggested that children in the CRCT group had significantly higher self-reported HRQL ratings for domains related to pain, F2,64=4.07 (P=0.022) and pain-related functioning, F2,64=4.32 (P=0.017), compared with children with similar and milder disease risk. Exploratory analyses implied that children in the CRCT group also had fewer worries about SCD-related complications, F3,63=9.68 (P<0.001). These patient-perceived benefits of CRCT may have important implications for treatment decisions and for providing ancillary support for children with SCD and their families.

Red Blood Cell Transfusion in Palliative Care: A Survey of Palliative Care Physicians.

Background: Red blood cell transfusion is one therapeutic option for the treatment of anemia. Current transfusion practices and factors influencing the decision to prescribe this therapy are not well understood. Objective: To explore current transfusion practices, attitudes, and factors that influence the decision to transfuse among palliative care physicians. Design: Self-administered questionnaire addressing clinical experiences and decision making pertaining to blood transfusion. Setting/Subjects: Specialist palliative care physicians at two Canadian academic centers. Measurements: Descriptive, presented as the number/proportion of respondents indicating a specific answer. Results: Of 62 physicians surveyed, 29 (47%) responded to the study questionnaire. For patients with solid tumors and hematologic malignancies, respectively, 79% and 82% of respondents reported prescribing blood transfusion; 59% and 46% reported that they would seldom recommend its discontinuation. Factors influential in the decision to transfuse included symptoms of anemia (97%), bleeding (62%), low hemoglobin (52%, of whom 87% indicated a hemoglobin threshold <70 g/L), and pressure from patients/families (48%). Physicians routinely reassessed patients for symptomatic improvement following transfusion, but 72% did not use an objective symptom scale. Twenty-six (90%) respondents believed that transfusion provided symptomatic benefit; the majority had observed adverse reactions to transfusion. Most perceived a lack of evidence to guide transfusion therapy in palliative care, and 79% indicated willingness to enroll their patients in a trial aiming to address this question. Conclusions: Most palliative care specialists consider red blood cell transfusion to have a role in symptom management, but many clinical and nonclinical factors influence their decisions to provide or discontinue transfusions. Prospective clinical trials will likely be needed to inform transfusion practices in this population.

Behaviour modification interventions to optimise red blood cell transfusion practices: a systematic review and meta-analysis.

OBJECTIVE: To assess the impact of behaviour modification interventions to promote restrictive red blood cell (RBC) transfusion practices. DESIGN: Systematic review and meta-analysis. SETTING, PARTICIPANTS, INTERVENTIONS: Seven electronic databases were searched to January 2018. Published randomised controlled trials (RCTs) or non-randomised studies examining an intervention to modify healthcare providers' RBC transfusion practice in any healthcare setting were included. PRIMARY AND SECONDARY OUTCOMES: The primary outcome was the proportion of patients transfused. Secondary outcomes included the proportion of inappropriate transfusions, RBC units transfused per patient, in-hospital mortality, length of stay (LOS), pretransfusion haemoglobin and healthcare costs. Meta-analysis was conducted using a random-effects model and meta-regression was performed in cases of heterogeneity. Publication bias was assessed by Begg's funnel plot. RESULTS: Eighty-four low to moderate quality studies were included: 3 were RCTs and 81 were non-randomised studies. Thirty-one studies evaluated a single intervention, 44 examined a multimodal intervention. The comparator in all studies was standard of care or historical control. In 33 non-randomised studies, use of an intervention was associated with reduced odds of transfusion (OR 0.63 (95% CI 0.56 to 0.71)), odds of inappropriate transfusion (OR 0.46 (95% CI 0.36 to 0.59)), RBC units/patient weighted mean difference (WMD: -0.50 units (95% CI -0.85 to -0.16)), LOS (WMD: -1.14 days (95% CI -2.12 to -0.16)) and pretransfusion haemoglobin (-0.28 g/dL (95% CI -0.48 to -0.08)). There was no difference in odds of mortality (OR 0.90 (95% CI 0.80 to 1.02)). Protocol/algorithm and multimodal interventions were associated with the greatest decreases in the primary outcome. There was high heterogeneity among estimates and evidence for publication bias. CONCLUSIONS: The literature examining the impact of interventions on RBC transfusions is extensive, although most studies are non-randomised. Despite this, pooled analysis of 33 studies revealed improvement in the primary outcome. Future work needs to shift from asking, 'does it work?' to 'what works best and at what cost?' PROSPERO REGISTRATION NUMBER: CRD42015024757.

Variation in red cell transfusion decisions in the intensive care unit - a nationwide survey in the Netherlands.

BACKGROUND AND OBJECTIVES: Most guidelines recommend a restrictive transfusion trigger of 7 g/dl. It is unclear whether this resulted in more uniform transfusion practices. The primary objective was to uncover the extent of variation in transfusion decisions within four scenarios of critically ill patients among critical care physicians in the Netherlands. MATERIALS AND METHODS: An online survey comprising four different hypothetical clinical scenarios was sent to all members of the Dutch Society of Intensive Care. The scenarios represented patients with acute myocardial infarction (Hb 8·5 g/dl), abdominal sepsis (Hb 7·1 g/dl), traumatic brain injury (TBI) (Hb 7·9 g/dl) and post-surgical complications (Hb 7·3 g/dl). The questions explored the decision whether or not to transfuse and a ranking of clinical characteristics playing the most important role in the transfusion decision. RESULTS: A total of 224 members (22%) participated in the study of whom 188 (84%) completed all questions. The percentages of respondents that decided to transfuse ranged from 25·9% in the scenario with TBI to 81·6% in the scenario with post-surgical complications. Most controversy was seen in the scenario with sepsis for which 43·2% decided to transfuse, whereas 56·8% decided not to. Haemoglobin level, diagnosis and haemodynamics were most important for the transfusion decision in all scenarios. CONCLUSIONS: Physicians decided differently on red-blood-cell transfusion given the clinical scenarios and weighed clinical characteristics differently in their transfusion decisions. These findings suggest there still is substantial variation in critical care transfusion practice.

Use of the blood substitute HBOC-201 in critically ill patients during sickle crisis: a three-case series.

BACKGROUND: Red blood cell (RBC) transfusion is an important treatment modality during severe sickle cell crisis (SCC). SCC patients who refuse, or cannot accept, RBCs present a unique challenge. Acellular hemoglobin (Hb)-based oxygen carriers (HBOCs) might be an alternative for critically ill patients in SCC with multiorgan failure due to life-threatening anemia. HBOC-201 (HbO2 Therapeutics) has been administered to more than 800 anemic patients in 22 clinical trials, but use of any HBOCs in critically ill sickle cell patients with organ failure is exceedingly rare. In the United States, HBOC-201 is currently only available for expanded access. CASE REPORT: We report three cases of HBOC-201 administered to critically ill sickle cell disease patients in SCC with multiorgan failure, either who refused RBCs (Jehovah's Witnesses) or for whom compatible RBCs were not available. RESULTS: Two patients received more than 20 units of HBOC-201, while the other received 6. The 27 units used in the third case equals the largest volume a patient has successfully received to date. All three patients survived to hospital discharge. CONCLUSION: These reports suggest that blood substitutes such as HBOC-201 can provide an oxygen bridge in SCC with multiorgan failure, until corpuscular Hb levels recover to meet metabolic demand, and highlight the compelling biochemical properties that warrant further investigation.

[Effectiveness of a nursing intervention on patient anxiety before transfusion of packed red blood cells]. / La efectividad de una intervención enfermera sobre la ansiedad del paciente ante la transfusión de concentrado de hematíes.

OBJECTIVES: To evaluate the effectiveness of an educational intervention regarding anxiety and satisfaction in patients requiring a red blood cell transfusion. METHOD: Randomised, controlled, single-blind clinical trial in patients requiring a packed red blood cell transfusion. SAMPLE SIZE: alpha=.05, beta=.10, to detect a 10% difference, 70 subjects in each group. The sampling recruitment was randomised to the intervention group (IG) and the control group (CG). INDEPENDENT VARIABLES: an intervention protocol with oral and written information using a published guide on the safety, risks and benefits of haemotherapy for the IG, and an equivalent one on general health topics for the CG. DEPENDENT VARIABLES: pre- and post-anxiety state; Spielberger's validated questionnaire: STAI. Satisfaction, by an ad hoc questionnaire. Sociodemographic and clinical variables: description, reason for transfusion, prescription knowledge, incidents, records. RESULTS: There was a total of 144 subjects, 73 (50.69%) in the IG, and 71 (49.31%) in the CG. The mean age was 55.80 years, with 56.94% males, and a first transfusion in 52.08%. Comparability between the IG and the CG was tested and confirmed. The decrease in anxiety after the intervention for the IG was 19.99, compared to 25.48 in CG. The difference was greater than the proposed 10%, and was statistically significant. The preference for information was 98.60% in IG, compared to 43.70% in CG. CONCLUSIONS: The hypothesis was confirmed; a protocolised nursing educational intervention protocol increased patient satisfaction with nursing care, and decreased patient anxiety, thus preventing complications and providing greater safety to the users.

Autonomous adrenocorticotropin reaction to stress stimuli in human fetus.

The aim of the study was to determine whether human fetuses show ACTH response to stress stimuli, to define the gestational age from which these reactions may be present and to analyze the relationship between hormone concentrations and their changes, both in fetuses and in pregnant women. The study included 81 intrauterine transfusions carried out in 19 pregnant women. 52 procedures were performed directly into the umbilical vein, which is not innervated, so neutral for the fetus (the PCI group) and 29 transfusions into the intrahepatic vein -which puncture is stressful for the fetus (the IHV group). ACTH and cortisol concentrations in fetal and maternal plasma obtained during the procedures were assayed. The initial mean plasma ACTH concentration in the PCI group equaled 18.94pg/mL, but in the IHV group it was significantly higher and amounted 75.17pg/mL (p<0.001). There was no significant change in the hormone concentration during the transfusion both in the IHV group (95.8pg/mL, p>0.05) and in the PCI group (22.36pg/mL, p>0.05). The observed hormonal response in the IHV group proves the existence of fetal pituitary reaction to stress. The initial fetal ACTH concentration in the IHV group correlated with the number of transfusions performed on a single fetus (R=0.41; p=0.04). No correlation with parity, gestational weeks or the volume of transfused packed red blood cells was found. There was also no correlation between fetal and maternal ACTH concentrations in any group. Presented data suggest that the human fetus shows autonomous ACTH reaction to stress stimulation.

Neurocognitive profiles of preterm infants randomly assigned to lower or higher hematocrit thresholds for transfusion.

OBJECTIVE: Preterm infants are frequently transfused with red blood cells based on standardized guidelines or clinical concerns that anemia taxes infants' physiological compensatory mechanisms and thereby threatens their health and well-being. The impact of various transfusion guidelines on long-term neurocognitive outcome is not known. The purpose of this study is to evaluate long-term neurocognitive outcome on children born prematurely and treated at birth with different transfusion guidelines. METHODS: Neurocognitive outcomes were examined at school age for 56 preterm infants randomly assigned to a liberal (n = 33) or restrictive (n = 23) transfusion strategy. Tests of intelligence, achievement, language, visual-spatial/motor, and memory skills were administered. Between-group differences were assessed. RESULTS: Those in the liberal transfusion group performed more poorly than those in the restrictive group on measures of associative verbal fluency, visual memory, and reading. CONCLUSIONS: Findings highlight possible long-term neurodevelopmental consequences of maintaining higher hematocrit levels.

[Paramedical staff knowledge and practice related to the blood transfusion safety]. / Connaissances et pratiques du personnel soignant en matière de sécurité transfusionnelle.

Blood transfusion is a complex activity, involving many actors. It is a high risk activity which couldn't be controlled without the use of specific methods. Health care workers beliefs and organisational factors are two major issues for the blood transfusion safety. Our study objectives were to describe the paramedical staff's knowledge and practice regarding blood transfusion safety and to identify factors that are related to them. We carried out a cross sectional study. The information was gathered by using a questionnaire. The latter was developed by foreign teams and adapted to the local context. Two outcome measures were used: the knowledge and practice score and the proportion of true answers. The study showed that only 15% of the interviewed persons have had a score less than 30 (scale range from 0 to 100), i.e. only 15% have had appropriate knowledge and practice with no negative consequences for the patient safety. 13.8% of the study population provided right answers related to the biologic exams required before red cells transfusion and 34% for the abnormal reaction circumstances. These results underscores the importance of strategies improving the quality and the safety of blood transfusion, i.e. the continuous medical education, implementing a blood transfusion information system and the use of transfusion practice guidelines.