Appropriateness of the EQ-5D-5L in capturing health-related quality of life in individuals with transfusion-dependent ß-thalassemia: a mixed methods study.

BACKGROUND: Individuals with transfusion-dependent ß-thalassemia (TDT) experience symptoms and functional impacts that reduce their health-related quality of life. However, EQ-5D-derived health utility index scores in TDT often indicate good HRQoL, suggesting the EQ-5D may not adequately capture the impact of TDT. This study explored the disease and treatment burden of TDT and examined the appropriateness of the EQ-5D-5L descriptive system (DS) in measuring HRQoL in TDT. METHODS: Adults with TDT in the United Kingdom, United States, and France completed a background questionnaire and EQ-5D-5L DS, followed by 60-minute semi-structured interviews on symptoms and HRQoL impacts of TDT (concept elicitation) and appropriateness of EQ-5D-5L DS (cognitive debrief). Transcribed interviews were analyzed using thematic and content analyses. The relationship between TDT symptoms and impacts were summarized in a conceptual model. EQ-5D-5L DS was mapped to concepts identified in the qualitative data to assess its capture of HRQoL concepts. Participants' EQ-5D-5L DS scores were compared to their qualitative descriptions for each dimension to assess their concordance. RESULTS: Thirty participants in the United States (n = 14 [46.7%]), United Kingdom. (n = 12 [40.0%]), and France (n = 4 [13.3%]) completed the study (73.3% female; mean age = 28.4 years [standard deviation (SD) = 5.1]; mean annual red blood cell transfusion [RBCT] frequency = 18.4 [SD = 7.6]). Participants reported TDT symptoms and impacts on HRQoL, all fluctuating across the RBCT cycle. EQ-5D-5L DS did not fully capture 11 of 16 (68.8%) HRQoL concepts reported. Most participants (n = 20/27 [74.1%]) reported that EQ-5D-5L DS did not capture important aspects of living with TDT, and 42.9% (n = 12/28) reported negative/neutral overall impressions of EQ-5D-5L DS. The highest degree of discordance between participants' qualitative data and EQ-5D-5L DS dimension scores was observed with mobility (42.3%) and self-care (34.6%), where the qualitative descriptions relating to these dimensions were worse than their quantitative scores. CONCLUSION: Current findings suggest that EQ-5D-5L DS lacks content validity and the derived health utility index score may not fully represent the burden of disease in TDT.

Health-Related Quality-of-Life Impacts Associated with Transfusion-Dependent ß-Thalassemia in the USA and UK: A Qualitative Assessment.

BACKGROUND: Individuals living with transfusion-dependent ß-thalassemia (TDT) experience reduced health-related quality of life due to fatigue and chronic pain, which cause disruptions to daily life. Currently, limited qualitative data exist that describe these impacts. OBJECTIVE: This study aimed to examine the ways in which symptoms and current treatments of TDT impact health-related quality of life, to holistically describe the humanistic burden of TDT, and to identify the unmet needs of individuals living with TDT. METHODS: Adults (aged ≥ 18 years) with TDT and caregivers of adolescents (aged 12‒17 years) with TDT participated in semi-structured one-on-one virtual interviews and focus group discussions. Interviews were conducted in the USA and UK and lasted approximately 60 minutes. After transcription, the interviews were analyzed thematically using a framework approach. RESULTS: A total of ten interviews/focus group discussions (six interviews and four focus group discussions) were conducted with 14 adults with TDT and two caregivers of adolescents with TDT. A framework analysis revealed five themes describing health-related quality of life (negative impacts on daily activities, social life, family life, work and education, and psychological well-being) and three themes describing the lived experience of TDT (impact of red blood cell transfusions and iron chelation therapy, treatment, and stigma). Physical, psychological, and treatment-related factors contributed to negative impacts on daily activities, social and family life, and work and education. Concerns about reduced lifespan, relationships and family planning, and financial independence were detrimental to participants' mental well-being. Participants reported having high resilience to the many physical and psychological challenges of living with TDT. A lack of TDT-specific knowledge among healthcare professionals, particularly regarding chronic pain associated with the disease, left some participants feeling ignored or undermined. Additionally, many participants experienced stigma and were reluctant to disclose their disease to others. CONCLUSIONS: Individuals living with TDT experience substantial negative impacts on health-related quality of life that disrupt their daily lives, disruptions that are intensified by inadequate healthcare interactions, demanding treatment schedules, and stigma. Our study highlights the unmet needs of individuals living with TDT, especially for alternative treatments that reduce or eliminate the need for red blood cell transfusions and iron chelation therapy.

The advance decision in the limelight again: Re PW (Jehovah's Witness: Validity of Advance Decision) [2021] EWCOP 52SEF.

This paper discusses the advance decision, which was the subject-matter of Re PW (Jehovah's Witness: Validity of Advance Decision) [2021] EWCOP 52. An advance decision is basically a decision, made by a person with mental capacity, that they should not be given a particular medical treatment if they lack such capacity later. This paper considers and comments on some of the terms used to describe the advance decision, some principles relating to it, some arguments for and against it, whether it is revocable, and its standing in relation to the Mental Health Act 1983, as amended. Two limits of an advance decision (it is revocable and can be overridden by the Mental Health Act 1983, s 63) are exposed by the paper.

Increased risk of suicide attempt among patients receiving blood transfusion: A propensity matched analysis.

ABSTRACT: To examine whether the association between blood transfusion and suicide attempt exists.Utilizing the national insurance database from Taiwan and propensity score matching analysis, the incidence of suicide attempt in a cohort with blood transfusion versus controls was compared.The key finding is that higher incidence of suicide attempt in blood transfusion than control group (with an adjusted hazard ratio of 1.79 with 95% confidence interval, 1.72-1.88) after adjusted for the covariates.Patients receiving blood transfusion are an increased risk of subsequent suicide attempt.

EQ-5D-3L health state utility values in transfusion-dependent thalassemia patients in Malaysia: a cross-sectional assessment.

PURPOSE: There is a gap of information describing the health state utility values (HSUVs) of transfusion-dependent thalassemia (TDT) patients in Malaysia. These values are useful in the assessment of health-related quality of life (HRQoL), economic evaluations and provide guidance to disease management decisions. The objective of this study was to estimate and derive HSUVs associated with the treatment and complications of TDT patients in Malaysia using the EQ-5D-3L instrument. METHODS: A cross-sectional survey using the EQ-5D-3L instrument was conducted between May to September 2018 across various public hospitals in Malaysia. Using a multi-stage sampling, patients diagnosed with TDT and receiving iron chelating therapy were sampled. The findings on the EQ-5D-3L survey were converted into utility values using local tariff values. A two-part model was used to examine and derive the HSUVs associated with the treatment and complications of iron overload in TDT. RESULTS: A total of 585 patients were surveyed. The unadjusted mean (SD) EQ-5D-3L utility value for TDT patients were 0.893 (0.167) while mean (SD) EQ VAS score was 81.22 (16.92). Patients who had more than two iron overload complications had a significant decline in HRQoL. Patients who were on oral monotherapy had a higher utility value of 0.9180 compared to other regimen combinations. CONCLUSION: Lower EQ-5D-3L utility values were associated with patients who developed iron overload complications and were on multiple iron chelating agents. Emphasizing compliance to iron chelating therapy to prevent the development of complications is crucial in the effort to preserve the HRQoL of TDT patients.

Perceptions on consumer information in transfusion. A qualitative study of consumers and prescribers.

BACKGROUND AND OBJECTIVES: Fresh blood product transfusion requires patient education for fully informed consent, and written consumer information is frequently used. Few studies have examined consumer preferences regarding written and verbal transfusion information provided. As a qualitative study, this research was designed to explore participant understanding and by analysing and integrating themes, generate a model to understand how transfusion information should be developed and used in practice. MATERIALS AND METHODS: Semi-structured interviews were conducted with healthcare consumers of transfusion information from various hospital clinical departments. Transcripts were coded to qualitatively compare nature/extent of content and opinions regarding transfusion information through thematic analysis. RESULTS: Analysis identified themes relating to healthcare engagement, purpose of information, mode of delivery and content delivered. Differences were identified between perceived purpose of information provided to consumers between 13 transfusion prescribers and consumers. Prescribers viewed information as a tool for obtaining informed consent, whereas consumers desired reassurance and knowledge. Consumers described both the specialized nature and volume of information as limiting their ability to question professionals on whom they were dependent. Information provided should be tailored to consumers and utilize simple, succinct explanations. CONCLUSION: Both groups were satisfied with written information adjunctive to verbal information. These findings will be used to redesign transfusion information and may be employed at the bedside when discussing transfusion. They may have implications for consumer information in other settings.

Assessment of blood supply and usage pre- and during COVID-19 pandemic: A lesson from non-voluntary donation.

BACKGROUND: Ensuring steady stream of safe blood is the ultimate goal of blood transfusion practice. The current COVID-19 pandemic has affected almost every part of life and economy. Consequently, this study sets off to assess the effect of the pandemic on blood supply and blood transfusion in the University of Calabar Teaching Hospital. METHODS: Data from the Donor Clinic and Blood Group Serology Unit of the University of Calabar Teaching Hospital were retrospectively extracted to evaluate supply and use of blood before and during COVID-19 pandemic. RESULT: A total of 1638 donors were recorded within the study period. Age range 15-29 and 30-44 years constituted majority of the subjects (58.9% and 33.4%, respectively). The donor pool were male-dominated. Commercial donors (61.7%) and family replacement donors (30.6%) constituted majority of the donor pool. Most of the donor pool were students (37.1%), public servants (22.8%) and artisans (18.6%). A concomitant decrease of 26.1% and 18.9% were recorded in blood donation and request during the COVID-19 pandemic. CONCLUSION: Blood supply was not significantly affected in our study center as both requests and donations decreased. Consideration for improving family replacement donation was advised.

Patient and physician perceptions about blood transfusions in the myelodysplastic syndromes.

BACKGROUND: Little is known about the shared decision-making between patients with transfusion-dependent (TD) myelodysplastic syndromes (MDS) and their physicians about the benefits, risks, and alternatives to reduce the need for blood transfusions. METHODS AND MATERIALS: We conducted interviews and two cross-sectional surveys of MDS patients and MDS physicians in the US about the use of blood transfusions and disease-modifying therapies (DMTs). Responses from 157 MDS patients and 109 MDS physicians were analyzed. RESULTS: The TD-MDS patient cohort had a median age of 69 years and a greater proportion of lower IPSS risk. The MDS physicians primarily practiced in large centers, evenly distributed between academic and community hospitals. There was a high level of independence and generally positive quality of life among patients, who were mostly concerned about effectiveness of blood transfusions and iron overload. MDS patients with shorter duration of disease (less than 5 years) were primarily concerned with transfusion reaction, while MDS patients with longer duration of disease were primarily concerned with iron overload. Approximately half of TD-MDS patients stated they had not discussed alternatives to reduce the need for blood transfusions with their physician. Patients with longer duration of disease were more likely to have a discussion with their physician about alternatives to blood transfusions. Physicians stated that they administered blood transfusions as primary therapy for MDS when it was patient preference, advanced age of patient, frailty, lower risk MDS, significant comorbidities, or failed prior treatments. CONCLUSIONS: While quality of life seemed generally positive in TD-MDS patients, there were differing perceptions about blood transfusions between patients and physicians. In the future, appraisal and optimization of the informed consent process between MDS patients and physicians are needed.

Lower hemoglobin concentration decreases time to death in severely anemic patients for whom blood transfusion is not an option.

BACKGROUND: Anemia in patients who decline transfusion has been associated with increased morbidity and mortality. We hypothesized that the time to death decreases with increasing severity of anemia in patients for whom transfusion is not an option. METHODS: With institutional review board approval, a retrospective review of registered adult blood refusal patients with at least one hemoglobin (Hb) value of 12.0 g/dL or less during hospital admission at a single institution from January 2004 to September 2015 was performed. The association of nadir Hb category and time to death (all-cause 30-day mortality) was determined using Kaplan-Meier plots, log rank tests, and Cox proportional hazard models. We investigated if there was a nadir Hb level between the values of 5.0 and 6.0 g/dL at which mortality risk significantly increased and then categorized nadir Hb by the traditional cut points and the newly identified "critical" cut point. RESULTS: The study population included 1,011 patients. The Cox proportional hazard models showed a more than 50% increase in hazard of death per 1 g/dL decrease in Hb (adjusted hazard ratio [confidence interval], 1.55 [1.40-1.72]; p < 0.001). A Hb value of 5.0 g/dL was identified as defining "critical anemia." We found a strong association between anemia severity level and mortality (p < 0.001). Time to death was shorter (median, 2 days) in patients with critical anemia than in those having higher Hb (median time to death of 4 or 6 days, in severe or moderate anemia). CONCLUSION: In anemic patients unable to be transfused, critical anemia was associated with a significantly and clinically important reduced time to death. LEVEL OF EVIDENCE: Prognostic, level III.

Evaluation of postoperative clinical outcomes in Jehovah's Witness patients who receive prothrombin complex concentrate during cardiac surgery.

BACKGROUND: Patients who refuse allogeneic blood transfusions (alloBT) on the basis of religious doctrine, such as Jehovah's Witnesses (JWs), can pose a challenge when undergoing surgical procedures. During cardiac surgery, special considerations regarding surgical techniques and blood loss minimization strategies can lead to improved outcomes. Limited literature exists to guide the use of four-factor prothrombin complex concentrate (4PCC) in this patient population undergoing cardiac surgery. STUDY DESIGN AND METHODS: This retrospective, single-center study evaluated the impact of 4PCC on hemoglobin (Hgb) change from baseline to postoperative nadir within a 7-day period among patients who refused alloBT during cardiac surgery. This study identified patients who refused alloBT from January 2011 to June 2017. Multivariable linear regression was used to control for confounding variables to evaluate the effectiveness of 4PCC. RESULTS: During the study timeframe, 79 patients met inclusion criteria, all of whom identified as JWs, and underwent cardiac surgery. Of these, 19 received intraoperative 4PCC. Multivariable linear regression found no difference in Hgb change in patients who received 4PCC vs those who did not. No significant differences were found in mortality, thromboembolic complications, or in-hospital postoperative events. CONCLUSIONS: In JWs undergoing cardiac surgery who refuse alloBT, intraoperative use of 4PCC was not associated with a difference in Hgb change within 7 days postoperatively when adjusting for confounding variables. In the event of excessive blood loss, the utilization of 4PCC may provide a viable option in JW patients who undergo cardiac surgery where few options exist to mitigate blood loss.

Transfusion support and alternatives for Jehovah's Witness patients.

PURPOSE OF REVIEW: Jehovah's Witness patients with critical anemia or undergoing major surgery are challenging for healthcare providers to manage, as most will decline transfusion of whole blood and its main components. Recent advances in our understanding of hemostatic agents, alternative hemoglobin-based oxygen carriers, and patient blood management have culminated in a complex array of options to manage critical anemia and bleeding in this patient population. RECENT FINDINGS: Refusal of blood products in the setting of critical anemia is associated with significant risk of morbidity and mortality. With implementation of patient blood management measures, targeted treatment of anemia and coagulopathy has reduced the need for transfusions. Likewise, increased clinical experience with hemoglobin-based oxygen carriers in Jehovah's Witnesses with critical anemia has provided new insights into their potential benefits and pitfalls. SUMMARY: Options and alternatives to manage the Jehovah's Witness patient in the perioperative setting or in the setting of critical anemia will be reviewed.

Psychosocial and cognitive engineering contributions to blood transfusion.

Among the research developed in social psychology, there is a field of study questioning the mechanisms that explain prosocial behaviors. In 1956, the first links between transfusion medicine and social psychology were formed, with the study of underlying motivations to blood donation behavior. Sixty years later, the number of "fundamental" and "applied" research has widely increased, and we now have a theoretical understanding of blood donation engagement's levers, as well as experimental demonstrations of methods to activate these levers. Thus, the literature offers several strategies experimentally verified to improve the recruitment and retaining of blood donors. These methods can go from specific speeches when soliciting donors, to changes in the blood donation environment, etc. They aim either at impacting the donation experience, at obtaining a change in the donor's behavior, or at changing the determinants of blood donation. Yet, it is clear that the knowledge from the research in social psychology is not really deployed on the field by the organizations that could benefit from it. In this article, we will try to develop the difficulty regarding the application of fundamental and experimental knowledge, and to underline the implications for the blood transfusion establishments. We will then present the psychosocial and cognitive engineering method, and argue its relevance to answer this problematic.

Withholding Treatment From the Dying Patient: The Influence of Medical School on Students' Attitudes.

PURPOSE: To determine motives and attitudes towards life-sustaining treatments (LSTs) by clinical and preclinical medical students. METHODS: This was a scenario-based questionnaire that presented patients with a limited life expectancy. The survey was distributed among 455 medical students in preclinical and clinical years. Students were asked to rate their willingness to perform LSTs and rank the motives for doing so. The effect of medical education was then investigated after adjustment for age, gender, religion, religiosity, country of origin, and marital status. RESULTS: Preclinical students had a significantly higher willingness to perform LSTs in all cases. This was observed in all treatments offered in cases of a metastatic oncologic patient and an otherwise healthy man after a traumatic brain injury (TBI). In the case of an elderly woman on long-term care, preclinical students had higher willingness to supply vasopressors but not perform an intubation, feed with a nasogastric tube, or treat with a continuous positive air-pressure ventilator. Both preclinical and clinical students had high willingness to perform resuscitation on a twelve-year-old boy with a TBI. Differences in motivation factors were also seen. DISCUSSION: Preclinical students had a greater willingness to treat compared to clinical students in all cases and with most medical treatments offered. This is attributed mainly to changes along the medical curriculum. Changes in reasons for supplying LSTs were also documented.

Hepatitis C test uptake among historic blood transfusion recipients following media coverage of the Penrose Inquiry and an awareness-raising campaign.

The final report of the Penrose Inquiry into historic transmission of HIV and hepatitis C (HCV) through blood transfusion/products in Scotland was published in March 2015 and recommended "everyone who had received a blood transfusion prior to 1991 and who had not had a test for HCV should be offered one." A targeted awareness-raising campaign to encourage such individuals to be tested was launched in October 2016. We examined HCV testing undertaken in 2015-2016 in three NHS boards in Scotland to evaluate impact of these events. Statistical process control was used to monitor trends in individuals tested and those mentioning transfusion. HCV positivity was calculated and multivariate logistic regression was used to examine factors associated with mention of transfusion. A total of 22 842 individuals received an HCV test in 2015-2016 and 3% of those with clinical information mentioned transfusion. The total number of HCV tests was significantly higher in the week following the Penrose Report and the number mentioning transfusion was significantly higher for three weeks. There was no significant increase following the awareness-raising campaign. Women and those aged over 50 years were the most likely to have mentioned transfusion. Overall HCV positivity was 3.7% and <1% for the transfusion group. The impact of both intense media coverage and the government-funded awareness-raising campaigns in terms of HCV test uptake was modest and short-lived. Our findings highlight the challenges of case-finding for HCV and the limited impact of awareness-raising. This can be used by other countries aiming to identify those infected through historic blood transfusion.

Delirium following total joint replacement surgery.

Postoperative delirium (POD) is a common complication associated with increased resource utilization, morbidity and mortality. Our institution screens all postsurgical patients for postoperative delirium. The study aim was to perform an automated interrogation of the electronic health records to estimate the incidence of and identify associated risk factors for POD following total joint arthroplasty (TJA). Adult patients who underwent TJA with a multimodal analgesia protocol, including peripheral nerve blockade, from 2008 through 2012, underwent automated chart review. POD was identified by routine nursing assessment and administrative billing codes. Of 11,970 patients, 181 (1.5%) were identified to have POD. Older age (odds ratio, 95% CI 2.20, 1.80-2.71 per decade, p < 0.001), dementia (7.44, 3.54-14.60, p < 0.001), diabetes mellitus (1.70, 1.1.5-2.47, p = 0.009), renal disease (1.68, 1.03-2.65, p = 0.039), blood transfusions (2.04, 1.14-3.52, p = 0.017), and sedation during anesthesia recovery (1.76, 1.23-2.51, p = 0.002) were associated with POD. Anesthetic management was not associated with POD risk. Patients who developed POD required greater healthcare resources. Dementia is strongly associated with POD. The association between POD and transfusions may reflect higher acuity patients or detrimental effect of blood. Postoperative sedation should be recognized as a warning sign of increased risk.

Hemato-oncology and palliative care teams: is it time for an integrated approach to patient care?

PURPOSE OF REVIEW: Integrated palliative care for those with advanced solid tumors yields significant benefits in patient and caregiver outcomes. However, most palliative care clinical trials have excluded patients with hematologic malignancies. There is growing interest in whether integrated palliative care may yield similar benefits in hematologic malignancy patients and caregivers, but there has been little direct evidence of benefit in this population. This review summarizes new data on palliative care issues in hematologic malignancies, published in the preceding 12 months. RECENT FINDINGS: Most newly published evidence on this topic from the last year is descriptive of unmet needs, poor end-of-life care outcomes or unique differences and issues posed by hematologic malignancies as compared to solid tumors. A few articles describe models of collaborative care in hematologic malignancies, and just one describes the impact of an integrated palliative care intervention on patient and caregiver outcomes. Several studies point to transfusions as a unique and problematic barrier to high-quality end-of-life care in hematologic malignancies. SUMMARY: Recent evidence confirms that hematologic malignancy patients have unique and often unmet palliative care needs, and also have worse end-of-life outcomes. More work is needed to develop and test integrated palliative care interventions in this population.

Management of anemia in patients who decline blood transfusion.

Declining a treatment modality should not be considered the same as refusal of medical care as illustrated by the management of Jehovah's Witness patients who do not accept transfusions. Over the years, a comprehensive set of strategies have been developed to meet the specific needs of these patients and these strategies are collectively called "Bloodless Medicine and Surgery" (BMS). The focus in BMS is to optimize the patients' hematopoietic capacity to increase hemoglobin (Hgb) level, minimize blood loss, improve hemostasis, and provide supportive strategies to minimize oxygen consumption and maximize oxygen utilization. We present 3 case reports that illustrate some of the challenges faced and measures available to effectively treat these patients. Under BMS programs, patients with extremely low hemoglobin levels, not conducive to survival under ordinary conditions, have survived and recovered without receiving allogeneic transfusions. Additionally, the valuable experience gained from caring for these patients has paved the way to develop the concept of Patient Blood Management as a standard care to benefit all patients, and not only those for whom blood is not an option.