Acquired haemophilia A in a patient who is a Jehovah's Witness.

We detail the case of a man in his 80s who was a Jehovah's Witness, presenting to hospital for the second time in 1 week with atraumatic, severe bruising affecting his right thigh and flank. He was subsequently diagnosed with idiopathic acquired haemophilia A (AHA) and was urgently treated with recombinant factor concentrate and immunosuppressive therapy. Management of his bleeding disorder and resultant severe anaemia was adapted in line with his religious beliefs. AHA is a rare bleeding disorder which should be considered in patients with an isolated prolonged activated partial thromboplastin time and a history of recent or acute bleeding. Prompt diagnosis and management are essential as delays may result in increased mortality. Given that this patient declined blood transfusion, management of his bleeding disorder presented a unique challenge.

Optimising bloodless cardiovascular surgery for Jehovah's Witnesses and beyond.

Emerging evidence suggests surgical outcomes of patients undergoing cardiovascular surgery that refuse autologous transfusion is comparable to those who accept whole blood product transfusions. There are several methods that can be used to minimize blood loss during cardiovascular surgery. These methods can be categorised into pharmacological measures, including the use of erythropoietin, iron and tranexamic acid, surgical techniques, like the use of polysaccharide haemostat, and devices such as those used in acute normovolaemic haemodilution. More prospective studies with stricter protocols are required to assess surgical outcomes in bloodless cardiac surgery as well as further research into the long-term outcomes of bloodless cardiovascular surgery patients. This review summarizes current evidence on the use of pre-, intra-, and post-operative strategies aimed at the subset of patients who refuse blood transfusion, for example Jehovah's Witnesses.

Is There Sufficient Evidence Justifying Limited Access of Jehovah's Witness Patients to Kidney Transplantation?

BACKGROUND: Jehovah's Witnesses (JWs) refuse blood transfusions due to religious issues. This situation may impact kidney transplantation (KT) outcomes in case of hemorrhagic complications. We evaluated demographic characteristics of this population, hematologic safety, and graft outcomes. METHODS: This was a retrospective, single-center study comparing KT outcomes in JW patients versus a non-JW control group. Hematologic endpoints included clinical indication for blood transfusion (hemoglobin <7 g/dL), decrease of hemoglobin >2 g/dL or hematocrit >5% in the first week after KT, hemorrhagic complications requiring surgery, and de novo prescription of erythropoiesis-stimulating agents. Secondary endpoints included delayed graft function, treated biopsy-proven acute rejection, renal function, mortality, and graft survival at 12 months. RESULTS: From January 1989 to September 2018, we identified 143 JW (10 pediatric) and selected 142 matched control (non-JW) patients. There were no differences in the incidence of clinical indication for transfusion (13.3% versus 11.3%, P = 0.640), but a higher proportion of non-JW patients received transfusions (2.1% versus 9.2%, P = 0.010). There were no differences in the proportion of patients with decreased hemoglobin concentration, in reinterventions due to hemorrhagic complications, in the use of erythropoiesis-stimulating agents at hospital discharge, in the incidence of acute rejection, in renal function, and in mortality or graft survival rate at 12 months. CONCLUSIONS: In summary, this matched control cohort study suggests that, when clinically indicated, blood transfusions can be safely avoided in the majority of JW kidney transplant, who achieve and maintain comparable hemoglobin concentrations during the first year after transplantation compared with non-JW patients.

Long-term survival and quality of life in Jehovah's witnesses after cardiac surgery: a case control study.

BACKGROUND: We previously analyzed morbidity and mortality in Jehovah's Witnesses patients after cardiac surgery compared to control population patients. Patients who were Jehovah's Witnesses were operated in accordance with their philosophical convictions and in respect of their refusal of transfusions. We propose to assess long-term survival and quality of life in the patients of this preliminary study. METHODS: We contacted 31 adult Jehovah's Witnesses patients who underwent heart surgery at the Brugmann hospital between 1991 and 2012 and compared them to a control population of 62 patients that had no transfusion restriction, and matched them for sex, age at the time of intervention and the type of surgery performed. We compared long-term quality of life in both populations through the MacNew software, a validated instrument to assess quality of life of patients with cardiovascular disease. The long-term survival of patients was analyzed by Kaplan Meier curves. RESULTS: Long-term quality of life and survival do not appear different between the two groups. Patient evaluation by MacNew software shows comparable physical (p = 0.54), emotional (p = 0.12), social (p = 0.21) and global (p = 0.25) scores between the two populations. The analysis of the actuarial survival curves shows no differences in terms of long-term survival of these patients (p = 0.37). CONCLUSIONS: Cardiac surgery in Jehovah's Witnesses can be performed with identical long-term quality of life and survival compared to surgery without blood transfusion restriction, if one follows rigorous blood conserving strategies. TRIAL REGISTRATION: NCT03348072 . Retrospectively registered 16 November 2017.

Patterns of care in Jehovah's Witnesses patients with solid tumours and lymphoma.

BACKGROUND: Supportive care of Jehovah's Witnesses (JWs) diagnosed with cancer can be challenging, as they do not accept red blood cell (RBC) transfusions. AIM: The study was designed to determine treatment preferences and pattern of care offered to JWs diagnosed with cancer and its impact on cancer management. METHODS AND RESULTS: A retrospective cohort study of JWs with solid malignancies or lymphoma in our institution between 2005 and 2015 was conducted. Survival statistics were estimated using Kaplan Meier survival curves and Cox proportional regression model. A total of 63 JWs were identified with a median age of 70 years. At diagnosis, 34% (n = 22) had anaemia. All 63 declined RBC transfusion, including 19 patients who later developed transfusion threshold during anti-cancer treatment. Forty-three percent (n = 27) JWs had advanced (stage 4) disease, and 76% (n = 48) had Eastern Cooperative Oncology Group of 0 to 1. JWs were willing to accept surgery and radiation rather than chemotherapy. Treatment was deemed to be suboptimal in 22% (n = 14) JWs due to early treatment discontinuation, administration of non-standard chemotherapy regimen, or dose reduction due to anaemia and denial of blood transfusion. Twenty-seven percent (n = 17) received hematopoietic growth factors (erythropoiesis-stimulating agents and pegfilgrastim). There was no mortality directly attributed to anaemia or refusal of blood transfusion in the entire cohort. CONCLUSION: Jehovah's Witnesses declined RBC transfusion at diagnosis and during cancer therapy even if medically indicated. Management pathways need to be prospectively defined for this group of patients.

Investigational use of PEGylated carboxyhemoglobin bovine in a Jehovah's Witness with hemorrhagic shock.

BACKGROUND: Jehovah's Witnesses pose a clinical challenge in the setting of critical anemia. Most do not accept transfusions, but some accept hemoglobin-based oxygen carriers on a compassionate-use basis. PEGylated carboxyhemoglobin bovine (PCHB) is an acellular dual-action carbon monoxide (CO)-releasing and oxygen transfer agent currently being investigated in Phase II clinical trials. CASE REPORT: We present the case of a 42-year-old Jehovah's Witness with an acute upper gastrointestinal bleed and hemorrhagic shock who required emergent PCHB for stabilization during lifesaving interventions. After PCHB infusion, the patient's shock and encephalopathy improved with decreased vasopressor requirement. Through gastroenterology and interventional radiology procedures, the patient's bleeding stabilized. While receiving five additional doses of PCHB and other supportive therapies (iron, folate, vitamin B12, darbepoetin alfa), the patient was extubated and weaned off vasopressors. CONCLUSIONS: PCHB was used to stabilize (bridge) a critically ill anemic patient for lifesaving interventions without adverse effects. Additional studies are warranted to explore the drug's safety profile and efficacy in patients declining blood products.

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.

Safety and feasibility of elective liver resection in adult Jehovah's Witnesses: the Henri Mondor Hospital experience.

BACKGROUND: Elective liver resection (LR) in Jehovah's Witness (JW) patients, for whom transfusion is not an option, involves complex ethical and medical issues and surgical difficulties. METHODS: Consecutive data from a LR program for liver tumors in JWs performed between 2014 and 2017 were retrospectively reviewed. A systematic review of the literature with a pooled analysis was performed. RESULTS: Ten patients were included (median age = 61 years). None needed preoperative erythropoietin. Tumor biopsy was not performed. Major hepatectomy was performed in 4 patients. The median estimated blood loss was 200 mL. A cell-saver was installed in 2 patients, none received saved blood. The median hemoglobin values before and at the end of surgery were 13.4 g/dL and 12.6 g/dL, respectively (p = 0.04). Nine complications occurred in 4 patients, but no postoperative hemorrhage occurred. In-hospital mortality was nil. Nine studies including 35 patients were identified in the literature; there was reported no mortality and low morbidity. None of the patients were transfused. CONCLUSIONS: By using a variety of blood conservation techniques, the risk/benefit ratio of elective liver resection for liver was maintained in selected adult JW patients. JW faith should not constitute an absolute exclusion from hepatectomy.

Treatment of individuals who cannot receive blood products for religious or other reasons.

By virtue of their religious principles, Jehovah's Witnesses (JWs) generally object to receiving blood products, raising numerous ethical, legal, and medical challenges for providers who care for these patients, especially in the emergent setting. In this review, we discuss several areas relevant to the care of JWs, including the current literature on "bloodless" medical care in the setting of perioperative and intraoperative management, acute blood loss, trauma, pregnancy, and malignancy. We have found that medical and administrative efforts in the form of bloodless medicine and surgery programs can be instrumental in helping to reduce risks of morbidity and mortality in these patients. Planning prior to an anticipated event associated with blood loss or anemia (such as elective surgery, pregnancy, and chemotherapy) is critical. Specifically, bloodless medicine programs should prioritize vigilant early screening and management of anemias, early establishment of patient wishes regarding transfusion, and the incorporation of those wishes into multidisciplinary medical and surgical care. Although there are now a variety of human-based and nonhuman-based products available as transfusion alternatives, the degree and quality of evidence to support their use varies significantly between products and is also largely dependent on the clinical setting.

Transfusion Refusal and the Shifting Limits of Multicultural Accommodation.

The refusal of blood products by Jehovah's Witness patients has provoked court proceedings, social science research, and contemporary fiction, all of which emphasize a seemingly intractable conflict between religious and secular ways of being. This article takes a different approach, focusing instead on the space that Witness patients have carved out for their accommodation in a major pediatric research hospital. Using discourse analysis and interview data, I map the way moralizing discourses surrounding Witness families have shifted over the past 70 years alongside advancements in bloodless medicine. I argue that Witnesses have helped to enable their present accommodation and recognition by marshaling particular forms of economic, human, and social capital, and consider whether their success might be attainable by other treatment-resisting patient groups. Thus, this article explores the shifting limits of multicultural accommodation and the conditions that make understanding, collaboration, and compromise possible.

"Bloodless" Neurosurgery Among Jehovah's Witnesses: A Comparison with Matched Concurrent Controls.

BACKGROUND: Jehovah's Witnesses (JW) are a Christian faith with >1 million members in the United States who do not accept autologous blood transfusions. The optimal management of these patients undergoing neurosurgical procedures is not well defined. Here, we examined the feasibility and safety of JW undergoing neurosurgery in a blood management program. STUDY DESIGN AND METHODS: Sixty-eight JW patients including 23 men and 45 women (mean age 53 ± 12 years) who underwent a variety of cranial (n = 19) and spinal (n = 49) neurosurgical procedures during a 5-year period were identified retrospectively and their hospital charts, anesthetic records, and operative reports reviewed. A concurrent cohort of sex-, age-, and procedure-matched non-JW controls also was identified. RESULTS: Among JW patients, a cell-saving system was used in 27 cases, with blood retransfused in 13 cases. Lactated Ringers solution was used extensively intraoperatively; albumin was given to 15 patients. The median decrease in hemoglobin was 2.1 g/dL. One patient had a postoperative hemoglobin value <7 g/dL. One patient returned to the operating room to revise a lumbar pedicle screw, and one patient had postoperative seizures. No cardiopulmonary complications, sepsis, pneumonia, or wound infection were observed. Compared with the matched control group, similar outcome results were observed. Blood loss and operative time also were similar in JW patients and controls. CONCLUSIONS: Neurosurgical procedures in Jehovah's Witnesses are feasible, safe, and have similar outcomes to patients willing to accept transfusion when managed within a multidisciplinary blood-management program.

Health care reform and Diagnosis Related Groups in Germany: The mediating role of Hospital Liaison Committees for Jehovah's Witnesses.

Resulting from health care reform in Germany that was implemented in 2003-2004, a new medical classification system called the "Diagnosis Related Groups" (DRGs) was introduced in hospitals. According to the media, social scientists, and a few physicians interviewed in this study the policy negatively transformed the German health care system by allowing the privatization of the hospital sector consistent with the neoliberal health care model. Allegedly, this privileged economic values over the quality of health care and introduced competition between hospitals. Nevertheless, members of the Hospital Liaison Committees (HLCs) of Jehovah's Witnesses argued that the DRGs system could be used to the advantage of Jehovah's Witness (JW) patients. HLCs often assist in the patient's search by providing names of physicians that would be willing to refrain from blood transfusions. This article draws from nine months of ethnographic research with Jehovah's Witnesses, including members of the HLCs, carried out primarily in Berlin between 2010 and 2012. By focusing on JWs, whose refusal of blood transfusions is often exemplified as particularly difficult for the biomedical profession, it addresses the "unintended" consequences of the introduction of DRGs into the German health care system that remain unexplored by health and social science scholarship. It argues that although JWs have long been associated with the judicialization of religious freedom globally, they do not equally engage in the judicialization of health in countries such as Germany. The reason for this is embedded not only in health care policy that favors mediation over medical malpractice litigation. It also results from the synergy of health care reforms that prioritize standardizing and economizing measures such as DRGs as well as practices implemented by Patient Blood Management programs that JW institutions, such as HLCs, have tapped into.

Jehovah's Witness patients within the German medical landscape.

Blood transfusions belong to standard and commonly utilised biomedical procedures. Jehovah's Witnesses' transfusion refusals are often referred to in bioethical and medical textbooks. Members of this globally active religious organisation do not, however, challenge biomedical diagnosis and treatment as such. A result of both their trust in and their interpretation of the Bible, they question only this medical treatment. In spite of the global presence of this religious community and its uniformly practised teachings, including those pertaining to blood, experiences and choices of Jehovah's Witness patients have been understudied. Drawing on a nine-month fieldwork with Jehovah's Witnesses and physicians in Germany (mainly in Berlin) between 2010 and 2012, the paper addresses treatment choices made by Witness patients and their relationship with physicians. In light of the long tradition of 'medical heterodoxy' established in German culture and society, Germany constitutes an ideal point of departure for such a study. By utilising the concept of 'medical landscape' it is argued that Jehovah's Witnesses in my field site find themselves at the intersection of different medical landscapes: in the 'immediate' surroundings of the German healthcare system that is open to different 'treatment modalities', and that of the United States, which favours biomedicine. The paper also argues that Jehovah's Witnesses' position towards blood transfusions can further be used as a lens to shed light on the German (bio)medical landscape itself.

Avoiding allogenic blood transfusions in endoscopic angiofibroma surgery.

BACKGROUND: Surgical approaches for many tumours are often limited by blood loss, exposure and risk to vital anatomical structures. Therefore, the standard of care for certain skull base tumours has become endoscopic transnasal resection. Other surgical disciplines often use cell salvage techniques, but review of the otolaryngology literature revealed very few case reports. This study investigated the value and safety of salvage-type autologous blood transfusion during the endoscopic resection of juvenile nasopharyngeal angiofibromas (JNA). METHODS: JNA is a rare vascular nasal tumour and the study extended over a 3-year period to obtain adequate patient numbers. All patients undergoing endoscopic resection during this period were included in the population sample. Ten patients with JNA were identified and underwent embolization prior to the endoscopic resection. In all cases the intraoperative blood salvage apparatus was used. Close post-operative monitoring was performed. RESULTS: Homologous blood transfusion could be avoided in all cases. Postoperative monitoring revealed transient bacteraemia in two cases where the leukocyte filter was not used, but no evidence of septicaemia. CONCLUSIONS: Perioperative cell saver and autologous blood transfusion in endonasal JNA surgery is safe. Homologous blood transfusion can be avoided by using this technique. The use of cell salvage allows for single stage surgery without the need to abandon surgery due to excessive blood loss and its future use is promising.

Refusal of Medical Blood Transfusions Among Jehovah's Witnesses: Emotion Regulation of the Dissonance of Saving and Sacrificing Life.

This study focuses on the requirement of JWs to refuse medical blood transfusions. We identified a life-death cognitive dissonance among JWs, with the opposing cognitions of being willing to sacrifice life by religious standards, while being unwilling to do so. Using a theory that connects cognitive dissonance with the need to regulate difficult emotions to analyze our qualitative data material, we identified two sets of dissonance reduction strategies among the JWs. Set 1 was tied to the individual-group: selective focus on eternal life, a non-blood support and control system, and increased individualization of treatment choices. Set 2 was in the religion versus medicine intersection: denial of risk combined with optimism, perception of blood as dangerous, and use of medical language to underscore religious doctrine.