The care of a child/adolescent with cancer or a blood disorder is complex and often long term, involving many interdisciplinary team members across services and geographical boundaries. This experience can be overwhelming for patients and their families, highlighting the need for a family care coordinator (FCC) to help them navigate their care path. The purpose of this article is to illustrate the concept of family care coordination as experienced by the IWK Health Center in Nova Scotia, Canada, with the intent of sharing a valuable model of care with other pediatric hematology/oncology services. Key components of the role are ongoing assessment, education, partnerships, communication, support, and advocacy. Essential resources and pathways are required to implement the role and optimize patient/family outcomes, facilitating consistent and accessible care, enhancing quality and safety, building trust, and gleaning efficiencies. Inherent FCC challenges are identified as time constraints, replacement issues, maintaining professional boundaries, and emotional burnout. A FCC can enable seamless, individualized care for children/adolescents and their families with pediatric oncological and hematological disorders, optimizing the outcomes for all involved.
Blood transfusion is a well-documented route of transmission of hepatitis C virus (HCV). However, a persisting high frequency of HCV infections was recorded in our haematology ward even after screening of blood donors had been introduced. We investigated the viral strains in 37 patients with haematological malignant diseases who had developed hepatitis C when treated in the ward during 1990-93. 17 of the patients acquired hepatitis C despite being transfused only with blood components screened by second-generation anti-HCV tests. The viral strains were characterised by PCR genotyping and nucleotide sequencing of the hypervariable region of the E2 gene. Five clusters of closely related or identical viruses were found involving 2, 3, 4, 6, and 15 patients, respectively. Blood components could be ruled out as the common source of infection because no donor had given blood to all patients sharing a specific strain, and even donors whose blood had been given to several patients were negative for HCV RNA. All patients in each cluster had been treated in the ward during overlapping periods. These findings suggest that despite strict hygienic control, HCV transmission occurred between patients treated in the same hospital setting, as has previously been reported in a smaller group of haemodialysis patients.
Since 1982 the Canadian Apheresis Study Group (CASG) has collected data on plasma exchange activities in Canada. In 1987, 5907 such procedures were carried out on 700 patients for more than 22 different diseases; this represented an increase of 28% over the figure for 1982. A shift in activity has occurred over the years; originally hematologic disorders accounted for most of the procedures; however, in 1987, 60% of the exchanges were done to treat neurologic disorders, mainly myasthenia gravis and acute and chronic Guillain-Barré syndrome. Several prospective randomized clinical trials have recently been completed by the CASG in the hope of determining the optimal application of plasma exchange. These studies, currently under review, include 168 patients with multiple sclerosis, 100 with thrombotic thrombocytopenic purpura and 43 with rapidly progressive glomerulonephritis. Reactions occur in 12% of cases; they are usually minor and are limited to circumoral paresthesia, mild hypertension or hypotension and hives.
Blood product transfusions can be life saving and must be considered in the supportive care of children of any age with underlying oncological or haematological problems, as well as after major surgery or after serious trauma. Paediatric transfusions are particularly challenging because life-long effects of transfusion complications are more durable and serious in children than in adults, in whom the median age at transfusion is >70 years (Tynell E, Norda R, Shanwell A, Björkman A. Long-term survival in transfusion recipients in Sweden, 1993. Transfusion 2001, 41, 251-255). While the general indications for transfusions in paediatric patients are similar to adults, the threshold, volumes and infusion rates for transfusions vary with age. In this Update, we discuss current blood products, then suggest transfusion "triggers" in major surgery and haematological and oncologic practice. Finally, future developments and new possibilities are considered.
The Norwegian Society of Haematology has worked out guidelines for the use of granulocyte-colony stimulating factor and granulocyte-monocyte colony stimulating factor and interferon alpha in clinical haematological practice. We recommend not using growth factors as a routine to prevent or to treat fever in patients with granulocytopenia induced by cytostatics, or patients with myelodysplastic syndromes. At present such treatment should be restricted to clinical trials. The same conclusion was reached in regard to use of erythropoietin in the case of myelodysplastic syndromes. Harvesting of stem cells from peripheral blood is a well documented indication for administration of growth factors. Interferon alpha as maintenance treatment for cases of multiple myeloma and low grade malignant lymphoma delays progression of the disease but does not improve chance of survival. There is no documentation of improved quality of life. Use of interferon alpha is not justified as a routine treatment for multiple myeloma. In chronic myelogenous leukemia, interferon alpha seems to be equal to or better than hydroxyurea, and may be considered for patients who cannot undergo allogeneic bone marrow transplantation.
Comment In: Tidsskr Nor Laegeforen. 1996 Aug 10;116(18):22158801671
Comment In: Tidsskr Nor Laegeforen. 1996 Aug 20;116(19):2352-38804216