Johnny's story: transfusing a Jehovah's Witness

Jehovah's Witnesses refuse blood transfusions for themselves and for their children. This action can be difficult for health professionals to understand and can lead to tensions between the staff and family. For one family, their refusal of blood for their child lead to a greater understanding of their religion and its beliefs for those who cared for them. Interspersed with their story are the medical reasons their son required blood, the reasons Jehovah's Witnesses refuse blood transfusions, and what the acceptable alternatives are to Jehovah's Witnesses. This article will share the thoughts and feelings of the family and the nursing staff who cared for the family during this crisis.     

Extended hemipelvectomy in a Jehovah's Witness with erythropoietin support

The care of patients refusing blood transfusion who require major ablative surgery for malignancy is a continuing challenge. The use of recombinant human erythropoietin is clearly efficacious in patients with renal disease and may be useful in anemic patients who refuse transfusion. Herein, we report a successful extended hemipelvectomy in a Jehovah's Witness using recombinant human erythropoietin support.     

Laparoscopic splenectomy in a Jehovah's Witness with profound anemia

Open surgery in a severely anemic patient may be complicated by a substantial blood loss from a large incision and subsequent poor wound healing secondary to the anemia. We report our success in performing a splenectomy laparoscopically in a profoundly anemic patient. A 50-year-old white male Jehovah's Witness who was HIV positive was referred for splenectomy after he developed profound, worsening anemia secondary to hypersplenism that was refractory to medical management. His preoperative hemoglobin and hematocrit levels were 2.7 g/dl and 8.8%, respectively, but his religious beliefs precluded transfusion. A laparoscopic splenectomy by the posterior gastric approach was performed. The patient tolerated the surgery well and experienced no additional morbidity. On postoperative day 7, his hemoglobin and hematocrit were 6.8 g/dl and 22%, respectively. We conclude that laparoscopic splenectomy is an attractive procedure in a severely anemic patient who requires splenectomy and refuses blood transfusion.     

Avoidance of homologous blood transfusion despite extreme blood loss

We report the case of a 22-year-old woman who underwent two-step scoliosis surgery without allogeneic transfusion, although the intraoperative blood loss (3500 ml) during the first procedure was higher than the calculated blood volume (3250 ml). Preoperatively the patient had donated four units of autologous blood. Intraoperatively blood-saving methods were combined. During the first operation acute normovolemic hemodilution (target hemoglobin 9.0 g/dl) was applied and during the second operation controlled hypotension (systolic blood pressure 80 mmHg). Intraoperative auto-transfusion was used in both procedures. During the first operation severe normovolemic anemia (minimal hemoglobin 3.5 g/dl) was accepted while the patient was ventilated with FiO2 1.0. The hemoglobin concentration was 8.6 g/dl after the first procedure and had increased to 11.6 g/dl 4 weeks after the second procedure. No severe complications occurred during the postoperative phase. This case report shows that also in surgical procedures with extreme blood loss any allogenic transfusion can be avoided by the combination of blood-saving methods, acceptance of low intraoperative transfusion trigger and ventilation with 100% oxygen.     

Clinical and ethical issues in the treatment of a Jehovah's Witness with acute myeloblastic leukemia

We report the first documented case of the use of peripheral blood stem cell autografting in the treatment of a Jehovah's Witness with acute myeloblastic leukemia. This case illustrates the complex ethical and clinical issues that arise in the treatment of such patients.     

The effect of intermittent pneumatic compression devices on intraoperative blood loss during radical prostatectomy and radical cystectomy

Intermittent pneumatic compression devices are a widely used, effective and presumed risk-free method of deep venous thrombosis prophylaxis, presumably by increasing peak venous blood velocity, and stimulating local and systemic fibrinolysis. We investigated whether intermittent pneumatic compression devices had any effect on intraoperative blood loss or transfusion during radical pelvic urological surgery. To our knowledge no previous study has addressed these issues. Records were reviewed for patients undergoing radical retropubic prostatectomy or radical cystectomy with diversion from 1985 to 1990. A total of 91 cases was reviewed: 38 radical retropubic prostatectomies and 53 radical cystectomies with diversion (34 male and 19 female patients). There were 59 patients with intermittent pneumatic compression devices (29 radical retropubic prostatectomies and 30 radical cystectomies with diversion) and 32 without intermittent pneumatic compression devices (9 radical retropubic prostatectomies and 23 radical cystectomies with diversion). Intraoperative blood loss and transfusions were calculated for each group with and without intermittent pneumatic compression devices. No clinically apparent lower extremity deep venous thrombosis or pulmonary embolus was diagnosed in any patient. For the group with intermittent pneumatic compression devices mean intraoperative blood loss was 2,541 ml. (range 700 to 8,850) versus 1,807 ml. (range 450 to 5,100) without a device, for a statistically significant difference of 734 ml. (p = 0.005). When 5 patients with excessive intraoperative blood loss (more than 5,000 ml.) were excluded the statistically significant difference was maintained. When comparing radical retropubic prostatectomy and radical cystectomy with diversion, with and without intermittent pneumatic compression devices, blood loss was greater for the group with a device for each procedure. Differences in intraoperative blood loss were independent of sex or tumor stage. Intraoperative transfusions were increased by approximately 0.6 units per patient with the device. Our study suggests that intermittent pneumatic compression devices may increase blood loss during a radical pelvic operation.     

Effect of sodium nitroprusside on postoperative blood loss in the cardiac surgical patient

Sodium nitroprusside (SNP) is known to inhibit platelet aggregation and has been implicated in postoperative hemorrhagic complications. Because it is a useful agent for treating postoperative hypertension and low cardiac output in the cardiac surgical patient, the authors retrospectively reviewed the course of 53 patients undergoing open heart procedures on cardiopulmonary bypass. Twenty-three patients received SNP and 30 did not. There were no differences in baseline hematological or clotting profiles, liver functions, bypass or cross-clamping times or heparin/protamine requirements between the two groups. Analysis revealed no significant differences between the groups in blood product requirements, actual mediastinal drainage, or postoperative measurements of routine clotting parameters. Although biochemical inhibition of platelet aggregation can be demonstrated, the use of SNP in the cardiac surgical patient has no apparent clinical effects which sould detract from its utility in treating hypertension or low cardiac output.     

Open heart surgery in a Jehovah's Witness boy--a case report of successful management of aortic regurgatation and aneurysm of sinus Valsalva due to infective endocarditis

Jehovah's Witness who require operation represent a challenge to the physician because of the patients' refusal to accept blood transfusion. We report an 8-year-old male of Jehovah's Witness who underwent a surgical treatment of infective endocarditis. He was transferred to our hospital because of high fever and heart murmur. Echocardiogram revealed a developing vegetation of aortic cusps and an aneurysmal change of the non-coronary sinus Valsalva. On admission he was complicated by anemia, purulent meningitis and suppurative arthritis of left knee. There were no signs of cardiac failure. Erythropoietin (6000 U thrice weekly) and iron (60 mg daily) were given for 11 weeks prior to surgery, raising the hemoglobin level from 9.2 g/dl to 18.4 g/dl. Aortic valve replacement and plasty of the sinus Valsalva were then performed. Intraoperatively hemoglobin concentration dropped to 10.3 g/dl and it raised to 15 g/dl postoperatively. We also used Cell-Saver to reduce blood loss. The patient made an uncomplicated recovery. Erythropoietin therapy contributed substantially to the successful outcome of this case.     

Penectomy: a technique to reduce blood loss

Twelve SHV-type extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli lac mutant isolates were recovered in October 1997 from 11 patients of the neonatal ward in a Warsaw hospital. The outbreak was clonal; however, some of the isolates expressed a much higher level of resistance to several beta-lactam antibiotics, including expanded-spectrum cephalosporins. This phenotype has been attributed to beta-lactamase hyperproduction correlating with the multiplication of ESBL gene copies, as was demonstrated for representative isolates.     

Managing hearing loss in a patient with Alzheimer disease

Advances in combined transcranial and transfacial (craniofacial) approaches for malignant tumors involving the anterior skull base have demonstrated improved survival. The technique allows adequate assessment of the intracranial extent of the tumor through an appropriate craniotomy. Vital structures, such as the dura, brain, and blood vessels, can be protected or resected and reconstructed safely. An en bloc excision can be accomplished. Dural defects and/or tears are satisfactorily repaired under direct vision, ensuring a watertight closure. Finally, adequate closure of the soft tissue defect is obtained, thus segregating the cranial cavity from the potentially infected nasal cavity and the nasopharynx with a resultant decrease in morbidity. Operative mortality is low, although complication rates are high. The technique is safe and continues to be improved to reduce morbidity. To evaluate the true impact of this surgical procedure on improvement in survival as well as quality of life, a multiinstitutional registry with uniform indications is indicated. With increasing experience and well-defined indications, improvement in survival (from 50% to 60%) and reduction in morbidity (from 30% to 40%) can be demonstrated through multiinstitutional, cooperative efforts.     

Correction of spurious blood cell counts in a patient with cryoglobulinemia reversed by the use of citrate-anticoagulated blood

Cryoglobulins may interfere with automated cell counting when proteins precipitate or blood cells aggregate. This results in spurious platelet or leukocyte counts, false values for red blood cell parameters and abnormal scattergrams. Usually it can be reversed by warming blood specimens to 37 degrees C. We describe the case of a 63-year-old woman with autoimmune disease in whom the diagnosis of cryoglobulinemia was suspected due to typical changes in blood cell counts. Artifacts were resistant to warming of the sample to 37 degrees C in both EDTA- and heparin-anticoagulated blood specimens. In contrast, the use of citrate as anticoagulant allowed correct measurements. This case underlines the role of assessment of blood counts in the detection of cryoglobulinemia. Correct measurements of automated blood counts in cryoglobulinemia may depend on the use of alternative anticoagulants.     

Prediction of hemorrhagic blood loss with a genetic algorithm neural network

There is no established method for accurately predicting how much blood loss has occurred during hemorrhage. In the present study, we examine whether a genetic algorithm neural network (GANN) can predict volume of hemorrhage in an experimental model in rats and we compare its accuracy to stepwise linear regression (SLR). Serial measurements of heart period; diastolic, systolic, and mean blood pressures; hemoglobin; pH; arterial PO2; arterial PCO2; bicarbonate; base deficit; and blood loss as percent of total estimated blood volume were made in 33 male Wistar rats during a stepwise hemorrhage. The GANN and SLR used a randomly assigned training set to predict actual volume of hemorrhage in a test set. Diastolic blood pressure, arterial PO2, and base deficit were selected by the GANN as the optimal predictors set. Root mean square error in prediction of estimated blood volume by GANN was significantly lower than by SLR (2.63%, SD 1.44, and 4.22%, SD 3.48, respectively; P < 0.001). A GANN can predict highly accurately and significantly better than SLR volume of hemorrhage without knowledge of prehemorrhage status, rate of blood loss, or trend in physiological variables.     

A series of self-measurements by the patient is a reliable alternative to ambulatory blood pressure measurement

BACKGROUND: Several studies have reported overdiagnosis and overtreatment of hypertensive patients, especially in borderline hypertensives. AIM: To find a blood pressure measurement procedure that reduces the risk of misclassification to an acceptable level. METHOD: Comparative, prospective study over seven months of primary care patients with elevated initial blood pressures. Blood pressure measurements made by general practitioners (GPs), practice nurses, and patients were compared with ambulatory blood pressure measurements. RESULTS: Ninety-nine patients completed the study. Mean differences (systolic blood pressure) between different measurement procedures and ambulatory measurement ranged from +10 mmHg (doctor) to -1 mmHg (patient), and (diastolic) from +4 mmHg (doctor) to -2 mmHg (patient). Standard deviations of mean differences ranged from 12 mmHg (doctor/systolic) to 10 mmHg (patient/systolic), and from 8 mmHg (doctor/diastolic) to 7 mmHg (patient/diastolic). CONCLUSION: Self-measurements by the patient appear to be a reliable alternative to ambulatory blood pressure measurement. In diagnosing and managing mild hypertension, we recommend the use of a valid self-measuring device.     

Müller cell-specific autoantibodies in a patient with progressive loss of vision

The cause of Ménétrier's disease is unknown, although various autoimmune, allergic, and infective causes have been postulated. This case report describes a 37-yr-old man with Ménétrier's disease associated with protein-losing enteropathy and Helicobacter pylori infection. Clinical, endoscopic, histological, and biochemical resolution occurred after treatment of Helicobacter pylori infection. The improvement observed in this case supports an etiological role for H. pylori infection in Ménétrier's disease.     

An analysis of the impact of the loss of a primary care physician on a patient population

The authors describe two cases of giant middle cerebral artery aneurysms presenting as mass lesions. Angiograms in each case revealed a distinctive serpentine vascular channel surrounded by an avascular area causing a "mass effect." Both lesions were resected in toto with excellent clinical results. Similar lesions in the literature are noted and the pathophysiology and origin of this group of aneurysms are discussed.     

Umbilical cord blood infusion in a patient for correction of Wiskott-Aldrich syndrome

A 2 3/4 year old male with thrombocytopenia secondary to Wiskott-Aldrich Syndrome (WAS) and a history of two intracranial hemorrhages as well as hemolytic anemia and neutropenia received a placental blood infusion from an HLA-identical female sibling born by caesarian section at 35 weeks gestation. The patient was prepared with Thiotepa and Cytoxan and received a nucleated cell dose of 3.0 x 10(7)/kg. Cyclosporin A and Methylprednisolone was given for graft versus host disease (GVHD) prophylaxis. An ANC of 0.5 x 10(9)/L and 1.0 x 10(9)/L were achieved on post-transplant days 18 and 28, respectively. Platelet recovery was rapid with a platelet count > or = 100 x 10(9)/L on day +39. On posttransplant day +11, the patient developed an erythematous rash consistent with grade I acute GVHD that resolved without therapy. He was discharged day on +60 and has remained free of infections with a normal platelet count off all immunosuppression therapy 30+ months post-transplantation. Chimerism studies performed on peripheral blood mononuclear cells by fluorescent in situ hybridization indicated that the percentage of donor cells ranged between 55 and 80%. The phenotype and function of peripheral blood lymphocytes are completely normal and the patient has responded in vivo with production of antibodies to both diphtheria and tetanus immunizations. This study demonstrates the feasibility of collecting placental blood after a multiple birth delivery and the ability of umbilical cord blood to provide complete hematopoietic and immunologic reconstitution in a patient with WAS.