Use of Joint Commission International standards to evaluate and improve pediatric oncology nursing care in Guatemala.

BACKGROUND: Inadequate nursing care is a major impediment to development of effective programs for treatment of childhood cancer in low-income countries. When the International Outreach Program at St. Jude Children's Research Hospital established partner sites in low-income countries, few nurses had pediatric oncology skills or experience. A comprehensive nursing program was developed to promote the provision of quality nursing care, and in this manuscript we describe the program's impact on 20 selected Joint Commission International (JCI) quality standards at the National Pediatric Oncology Unit in Guatemala. We utilized JCI standards to focus the nursing evaluation and implementation of improvements. These standards were developed to assess public hospitals in low-income countries and are recognized as the gold standard of international quality evaluation. METHODS: We compared the number of JCI standards met before and after the nursing program was implemented using direct observation of nursing care; review of medical records, policies, procedures, and job descriptions; and interviews with staff. RESULTS: In 2006, only 1 of the 20 standards was met fully, 2 partially, and 17 not met. In 2009, 16 were met fully, 1 partially, and 3 not met. Several factors contributed to the improvement. The pre-program quality evaluation provided objective and credible findings and an organizational framework for implementing change. The medical, administrative, and nursing staff worked together to improve nursing standards. CONCLUSION: A systematic approach and involvement of all hospital disciplines led to significant improvement in nursing care that was reflected by fully meeting 16 of 20 standards.

Thrombolytic therapy for central venous catheter occlusion.

BACKGROUND: Long-term central venous catheters have improved the quality of care for patients with chronic illnesses, but are complicated by obstructions which can result in delay of treatment or catheter removal. DESIGN AND METHODS: This paper reviews thrombolytic treatment for catheter obstruction. Literature from Medline searches using the terms "central venous catheter", "central venous access device" OR "central venous line" associated with the terms "obstruction", "occlusion" OR "thrombolytic" was reviewed. Efficacy of thrombolytic therapy, central venous catheter clearance rates and time to clearance were assessed. RESULTS: Alteplase, one of the current therapies, clears 52% of obstructed catheters within 30 min with 86% overall clearance (after 2 doses, when necessary). However, newer medications may have higher efficacy or shorter time to clearance. Reteplase cleared 67-74% within 30-40 min and 95% of catheters overall. Occlusions were resolved in 70 and 83% of patients with one and 2 doses of tenecteplase, respectively. Recombinant urokinase cleared 60% of catheters at 30 min and 73% overall. Alfimeprase demonstrated rapid catheter clearance with resolution in 40% of subjects within 5 min, 60% within 30 min, and 80% within 2 h. Additionally, urokinase prophylaxis decreased the incidence of catheter occlusions from 16-68% in the control group to 4-23% in the treatment group; in some studies, rates of catheter infections were also decreased in the urokinase group. CONCLUSIONS: Thrombolytic agents successfully clear central venous catheter occlusions in most cases. Newer agents may act more rapidly and effectively than currently utilized therapies, but randomized studies with direct comparisons of these agents are needed to determine optimal management for catheter obstruction.

A sustainable model for pediatric oncology nursing education in low-income countries.

Effectiveness of a nurse educator in the pediatric oncology unit in Guatemala was assessed by measuring completion of an education course, chemotherapy and central line competency, continuing education, and cost. All newly hired nurses completed the education course. Of the nurses employed, 86% participated in the chemotherapy course, and 93% achieved competency; 57% participated in the central line course, and 79% achieved competency. The nurses completed a mean of 26 hours continuing education yearly. The annual direct cost of the educator ($244/nurse) was markedly less than other models. This is an effective and sustainable means to educate nurses in low-income countries.

Regression of a congenital mesoblastic nephroma.

Histologically, the cellular variant of congenital mesoblastic nephroma (CMN) is very similar to another rare tumor of infancy, infantile fibrosarcoma (IFS). In addition to the histologic similarities, these tumor types share cytogenetic abnormalities including translocation t(12;15)(p13;q25). We describe herein the case of a child who did not have immediate surgical resection of a CMN and whose tumor was untreated for 8 months. During that time, the tumor demonstrated a significant degree of regression. The shared translocation with IFS, a tumor with well-documented potential for spontaneous regression, suggests that this genetic abnormality may have contributed to the favorable clinical course.

Management of occlusion and thrombosis associated with long-term indwelling central venous catheters.

Long-term central venous catheters (CVCs) are important instruments in the care of patients with chronic illnesses, but catheter occlusions and catheter-related thromboses are common complications that can result from their use. In this Review, we summarise management of these complications. Mechanical CVC occlusions need cause-specific treatment, whereas thrombotic occlusions usually resolve with thrombolytic treatment, such as alteplase. Prophylaxis with thrombolytic flushes might prevent CVC infections and catheter-related thromboses, but confirmatory studies and cost-effectiveness analysis of this approach are needed. Risk factors for catheter-related thromboses include previous catheter infections, malposition of the catheter tip, and prothrombotic states. Catheter-related thromboses can lead to catheter infection, pulmonary embolism, and post-thrombotic syndrome. Catheter-related thromboses are usually diagnosed by Doppler ultrasonography or venography and treated with anticoagulation therapy for 6 weeks to a year, dependent on the extent of the thrombus, response to initial therapy, and whether thrombophilic factors persist. Prevention of catheter-related thromboses includes proper positioning of the CVC and prevention of infections; anticoagulation prophylaxis is not currently recommended.

Heparin induced thrombocytopenia and re-thrombosis associated with warfarin and fondaparinux in a child.

An 11-year-old female developed heparin induced thrombocytopenia (HIT) with thrombosis during therapy for lower extremity deep vein thrombosis and pulmonary embolism. Transition from bivalirudin, a direct thrombin inhibitor (DTI), to warfarin resulted in extensive re-thrombosis, and fondaparinux therapy similarly failed. She was then treated with argatroban, and transitioned successfully to warfarin after 9 weeks. The risk of re-thrombosis was ultimately reduced by allowing time for the thrombogenic potential to abate. The argatroban/warfarin transition was monitored with chromogenic factor X levels. This case highlights several difficult problems in pediatric thrombosis.

Development of retinoblastoma programs in Central America.

BACKGROUND: Retinoblastoma, a curable eye tumor, is associated with poor survival in Central America (CA). To develop a retinoblastoma program in El Salvador, Guatemala, and Honduras, twinning initiatives were undertaken between local pediatric oncology centers, nonprofit foundations, St. Jude Children's Research Hospital, and the University of Tennessee Hamilton Eye Institute. PROCEDURE: The retinoblastoma program focused on developing early diagnosis programs in Honduras with national vaccination campaigns, developing treatment protocols suited to local conditions, building local networks of oncologists and ophthalmologists, training local healthcare providers, using modern donated equipment for diagnosis and treatment, and the ORBIS Cybersight consultation program and Internet meetings to further education and share expertise. Pediatric ophthalmologists and oncologists worked with foundations to treat patients locally with donated equipment and Internet consultations, or at the center in Guatemala. RESULTS: Number of patients successfully treated increased after the program was introduced. For 2000-2003 and 2004-2007, patients abandoning/refusing treatment decreased in Guatemala from 20 of 95 (21%) to 14 of 123 (11%) and in Honduras from 13 of 37 (35%) to 7 of 37 (19%). Survival in El Salvador was good and abandonment/refusal low for both periods. Of 18 patients receiving focal therapy for advanced disease, 14 have single remaining eyes. CONCLUSION: Development of the program in CA has decreased abandonment/refusal and enabled ophthalmologists at local centers to use modern equipment to provide better treatment. This approach might serve as a guide for developing other multispecialty programs.

Renal function after ifosfamide, carboplatin and etoposide (ICE) chemotherapy, nephrectomy and radiotherapy in children with Wilms tumour.

We prospectively evaluated tumour response and renal function in 12 newly diagnosed children with high-risk Wilms tumour receiving ifosfamide, carboplatin and etoposide (ICE) chemotherapy. Two cycles of ICE were followed by 5 weeks of vincristine, dactinomycin and doxorubicin (Adriamycin) (VDA), and nephrectomy, radiotherapy, additional VDA, and a third ICE cycle. Carboplatin dosage was based on glomerular filtration rate (GFR) to achieve targeted systemic exposure (6mg/ml min). Mean GFR (measured by technetium 99m-DTPA clearance) declined by 7% after 2 cycles of ICE and by 38% after nephrectomy; the mean carboplatin dose was reduced 32% after nephrectomy. Mean GFR remained stable after the third ICE cycle. Although urinary beta(2)-microglobulin excretion increased during therapy, no patient had clinically significant renal tubular dysfunction at the end of treatment. Treatment with ICE, nephrectomy and radiotherapy significantly reduces GFR, largely as the result of nephrectomy. Adjustment of carboplatin dosage on the basis of GFR and careful monitoring of renal function may alleviate nephrotoxicity.

Retinoblastoma: one world, one vision.

Retinoblastoma is curable when diagnosed early and treated appropriately; however, the prognosis is dismal when the basic elements of diagnosis and treatment are lacking. In developing countries, poor education, lower socioeconomic conditions, and inefficient health care systems result in delayed diagnosis and suboptimal care. Furthermore, the complexity of multidisciplinary care required is seldom possible. Whereas ocular salvage is a priority in the Western world, death from retinoblastoma is still a major problem in developing countries. To bring the 2 ends of this spectrum together and provide a forum for discussion, the "One World, One Vision" symposium was organized, at which clinicians and researchers from various cultural, geographic, and socioeconomic backgrounds converged to discuss their experiences. Strategies for early diagnosis in developing countries were discussed. Elements of the development of retinoblastoma centers in developing countries were discussed, and examples of successful programs were highlighted. An important component in this process is twinning between centers in developing countries and mentor institutions in high-income countries. Global initiatives by nongovernmental organizations such as the International Network for Cancer Treatment and Research, Orbis International, and the International Agency for Prevention of Blindness were presented. Treatment of retinoblastoma in developing countries remains a challenge; however, it is possible to coordinate efforts at multiple levels, including public administrations and nonprofit organizations, to improve the diagnosis and treatment of retinoblastoma and to improve the outcome for these children.

Childhood cancer epidemiology in low-income countries.

Global studies of childhood cancer provide clues to cancer etiology, facilitate prevention and early diagnosis, identify biologic differences, improve survival rates in low-income countries (LIC) by facilitating quality improvement initiatives, and improve outcomes in high-income countries (HIC) through studies of tumor biology and collaborative clinical trials. Incidence rates of cancer differ between various ethnic groups within a single country and between various countries with similar ethnic compositions. Such differences may be the result of genetic predisposition, early or delayed exposure to infectious diseases, and other environmental factors. The reported incidence of childhood leukemia is lower in LIC than in more prosperous countries. Registration of childhood leukemia requires recognition of symptoms, rapid access to primary and tertiary medical care (a pediatric cancer unit), a correct diagnosis, and a data management infrastructure. In LIC, where these services are lacking, some children with leukemia may die before diagnosis and registration. In this environment, epidemiologic studies would seem to be an unaffordable luxury, but in reality represent a key element for progress. Hospital-based registries are both feasible and essential in LIC, and can be developed using available training programs for data managers and the free online Pediatric Oncology Networked Data Base (www.POND4kids.org), which allows collection, analysis, and sharing of data.

Impact of an education program on late diagnosis of retinoblastoma in Honduras.

BACKGROUND: In developed countries, more than 90% of children with retinoblastoma present with limited-stage disease and are cured; however, in countries with limited resources, like Honduras, most patients present with advanced disease and cure rates are less than 50%. Early diagnosis is necessary to improve the survival of children with retinoblastoma in these countries. PROCEDURE: We describe the preliminary results of a retinoblastoma education program linked to a national vaccination campaign in Honduras. Posters and flyers were designed to be accessible to poorly educated readers, to convey the severity of retinoblastoma, and to provide contact information. Charts and an electronic database were reviewed to determine age at diagnosis, presenting signs and symptoms, date of diagnosis, and outcome. RESULTS: During the eight previous years (July 1995-June 2003), 73% of the 59 diagnosed cases of retinoblastoma were extraocular; in contrast, during the post-campaign period (June 2003-January 2005), only 35% of the 23 diagnosed cases showed extraocular spread (P = 0.002). More than one-third of patients in both time periods either refused therapy or abandoned treatment. CONCLUSION: This inexpensive approach is an effective first step toward improving survival of childhood retinoblastoma. Abandonment and refusal of therapy are continuing obstacles.

Managing children's cancer pain in Morocco.

PURPOSE: To identify issues in managing pain of children with cancer in the two pediatric oncology centers in Morocco. METHODS: Focus groups were conducted with pediatric oncology nurses and physicians. FINDINGS: Four themes were identified: (a) children's cancer pain is an overwhelming concern to the Moroccan nurses and physicians who participated in this study; (b) training and resources for children's cancer pain management are lacking in Morocco; (c) some impediments to pain relief were verbalized, such as a stoic approach to suffering and limited use of some drugs; and (d) a critical need exists for a comprehensive pain management approach for children with cancer in Morocco. CONCLUSIONS: This study elucidated issues in managing children's cancer pain in Morocco and increased knowledge of current practice issues. A program of policy research has been initiated with the aim of establishing guidelines for practice policies for managing children's cancer pain in Morocco.

Significance of pleural effusion at diagnosis of Wilms tumor.

BACKGROUND: Pleural effusion is uncommon at diagnosis of Wilms tumor. Because the clinical significance of this finding has not been reported, a retrospective review of this entity was conducted. PROCEDURE: The radiology reports and medical records of 233 patients with Wilms tumor who were treated at St. Jude Children's Research Hospital between 1985 and 2001 were reviewed. RESULTS: Ten of the 233 patients (4.3%) had pleural effusions detected on pre-nephrectomy imaging studies. Computed tomography (CT) scans were more sensitive than chest X-rays in identifying pleural effusions. All of the patients with pleural effusions had tumors of favorable histology. Two patients had stage II disease, five had stage III disease, two had stage IV disease, and one had stage V (bilateral) disease. All of the effusions occurred on the same side as the primary kidney tumors and six effusions were associated with ascites. The two effusions that were tapped were negative for tumor cells. All 10 patients in our series were alive without recurrence with a median follow-up duration of 68 months, yet only two patients were treated with lung irradiation. CONCLUSION: Pleural effusion at presentation of Wilms tumor is uncommon and not associated with adverse prognosis. We recommend that pleural effusions be tapped whenever feasible if the results will change therapy. Patients with pleural effusions that are small and difficult to tap may be treated according to their local stage without pulmonary irradiation. Further analysis of pleural effusions in a larger group of patients is necessary to confirm our preliminary observations.

Hematologic findings in children with rotavirus-positive and -negative diarrhea.

The authors studied neutropenia in 101 children hospitalized for gastroenteritis between 1 December 2000 and 30 June 2001 and identified children tested for rotavirus by reviewing their laboratory records. Rotavirus-positive and rotavirus-negative subjects did not differ significantly in their white blood cell counts, absolute neutrophil counts, or frequency of neutropenia (defined as an absolute neutrophil count < 1.0 x 10(9)/L), which accompanied 8.6% of rotavirus-positive cases and 9.3% of rotavirus-negative cases of gastroenteritis. The authors conclude that mild neutropenia accompanying diarrhea does not require further evaluation unless it persists or is associated with other factors such as sepsis.

Training subspecialty nurses in developing countries: methods, outcome, and cost.

BACKGROUND: As infections are controlled in developing countries, other pediatric subspeciality programs such as oncology become increasingly important. A major impediment to the development of such programs is a lack of trained nurses. Therefore, education of pediatric subspecialty nurses becomes a priority. PROCEDURE: We describe three models we have used for education of pediatric oncology nurses: a short series of classes or lectures with additional training of key nurses, an expanded 12 week series of classes at centers combining didactic and clinical instruction and a regional residential school offering regular 12 week courses in theory and clinical practice. RESULTS: Cost analysis showed that the cost per nurse trained was, respectively, 3,700; 4,350; and 5,500 US dollars. Early effectiveness indicators show that retention rates are high, home institutions are satisfied, and nurses trained shared their knowledge with other nurses and improved nursing practices. CONCLUSIONS: Programs to teach subspecialty nursing in developing countries are effective and can improve medical care. Such programs should be based on past experience and evaluated as to cost and effectiveness.

Improved survival for patients with recurrent Wilms tumor: the experience at St. Jude Children's Research Hospital.

BACKGROUND: Reported estimates of survival for patients with recurrent Wilms tumor are 24% to 43%. Because published survival data are more than a decade old and do not reflect advances in therapy, the authors reviewed their experience in treating recurrent Wilms tumor to determine whether the probability of survival has increased. PATIENTS AND METHODS: The authors reviewed the cases of 54 patients with recurrent Wilms tumor who were treated on one of six consecutive clinical trials at St. Jude Children's Research Hospital between 1969 and 2000. RESULTS: Five-year overall survival estimates after relapse were 63.6 +/- 15.7% for patients treated during or after 1984 (n = 20) and 20.6 +/- 6.5% for patients treated before 1984 (n = 34) (P = 0.002). When the analysis was restricted to patients with high-risk clinical features, 5-year overall survival estimates were 47.6 +/- 15.7% for those treated in the modern era (n = 16) and 11.1 +/- 5.2% for those treated in the earlier era (n = 25) (P = 0.005). Only three patients received high-dose chemotherapy with autologous stem cell rescue; one survived. No patients with recurrent anaplastic histology disease survived. CONCLUSIONS: Significant progress has been achieved in the treatment of recurrent favorable-histology Wilms tumor using multimodality salvage regimens with conventional doses of chemotherapy. Novel therapeutic strategies will be necessary to cure patients with recurrent anaplastic Wilms tumor.

Comparing the results of coagulation tests on blood drawn by venipuncture and through heparinized tunneled venous access devices in pediatric patients with cancer.

PURPOSE/OBJECTIVES: To compare the accuracy of three coagulation tests (prothrombin time [PT], activated partial thromboplastin time [aPTT], and fibrinogen [FBG]) performed on blood samples collected through heparinized tunneled venous access devices (TVADs) with those from venipuncture. DESIGN: Descriptive comparative with patients serving as their own controls. SETTING: Pediatric comprehensive care setting for children and adolescents experiencing catastrophic diseases. SAMPLE: 53 patients who had TVADs, had not received asparaginase during the previous 14 days, and had coagulation studies ordered. Patients ranged in age from 2-20 years (mean = 9.2 years, SD = 5). The most common diagnoses were neuroblastoma and acute myelocytic leukemia. METHODS: Blood was collected through TVADs within seconds of collection of the venipuncture sample. The first 3 ml of blood from a TVAD was discarded; the research nurse then drew three sequential samples of 3 ml each. Laboratory personnel were blinded to the source of all four samples until all analyses had been completed. MAIN RESEARCH VARIABLES: PT, aPTT, and FBG. FINDINGS: For all patients, results of PT, aPTT, and FBG tests on each of the three blood samples obtained through the TVAD differed significantly from results of the same tests on blood obtained by venipuncture. CONCLUSIONS: These findings indicate that neither a 6 ml, 9 ml, nor 12 ml discard from a heparinized TVAD is sufficient to yield clinically trustworthy PT, aPTT, or FBG values. IMPLICATIONS FOR NURSING: Nurses who have been persuaded by patients or parents to withdraw blood samples for coagulation indicators from a TVAD rather than from a venipuncture should have access to this research-based information that the three indicators, particularly aPTT, differ significantly from each other as to make it unreliable and potentially unsafe to sample blood from a TVAD to assess coagulation.