Central venous access device insertion and perioperative management of patients with severe haemophilia A: a local experience.

Central venous access device (CVAD) insertion is one of the most common procedures performed on paediatric haemophilia patients. There are no clear guidelines outlining the optimal dosing schedule of factor VIII (FVIII) and duration of treatment required to achieve adequate haemostasis during and after surgery. In this article, we describe the experience at McMaster Children's Hospital using FVIII replacement therapy in 15 children with severe haemophilia A during the course of 7 years. This is a retrospective institutional chart review. Patients between 0 and 18 years of age with severe haemophilia A that underwent CVAD insertion at McMaster Children's Hospital in Hamilton, Ontario, from 2004 to 2010, were identified and charts were reviewed. A total of 15 CVAD insertion surgeries were reviewed. The total average preoperative dose of FVIII was 93.5 IU/kg (range: 53.7-145.4 IU/kg). The total average postoperative dose was 818.7 IU/kg (range: 441-1258 IU/kg). The total perioperative dose was 912.2 IU/kg (range: 495.2-1349 IU/kg). The current study attempts to describe the experience at McMaster Children's Hospital for CVAD insertion surgeries, the average factor dose administered has decreased during the years. These results may be of help in the development of optimal treatment schedules.

Neonatal central venous catheter thrombosis: diagnosis, management and outcome.

Thrombotic occlusion of central venous catheters (CVCs) is a common problem in newborns. There is no guideline that systematically addresses the diagnosis, management, and prevention of this complication. The objective of this review is to establish evidence-based guidance for the management of CVC-related thrombosis. A comprehensive search of the scientific literature was conducted from 1948 to 2012. Twenty-six articles fulfilling four criteria - humans, neonates aged below 28 days, CVC insertion, and English language - were included for analysis. The incidence of thrombosis was 9.2% (308/3332). Singly inserted umbilical venous catheters (UVCs) and peripherally inserted central catheters accounted for over 80% of all CVCs. Frequently reported thrombotic sites were the hepatic vein, right atrium, and inferior vena cava. Symptoms included distal swelling of affected areas and thrombocytopenia. Increased length of catheter stay, infusion of blood products and malpositioned UVCs were identified as risk factors. The commonest diagnostic investigations to confirm thrombosis were echocardiography and ultrasonography. Spontaneous resolution may occur in UVC-related thrombosis, but this warrants close monitoring. Thrombolysis with urokinase alone or combined with low-molecular-weight heparin might be effective and well tolerated as treatment strategies. Prophylactic heparin increases the duration of catheter usability (P < 0.005, 95% confidence interval 0.35-0.81), decreases catheter occlusion, but may not uniformly prevent thrombosis. CVL-related thrombosis is an underreported complication because events in the majority occur silently. Currently, solid evidence-based recommendations for diagnosis and treatment are not possible. Well designed prospective studies are urgently required to establish a concrete investigational approach to CVC-related thrombosis and to institute safe therapeutic modalities.

Spontaneous neonatal arterial thromboembolism: infants at risk, diagnosis, treatment, and outcomes.

Neonatal spontaneous arterial thromboembolism is a rare phenomenon with a high risk of morbidity and mortality. Currently, there is little information regarding common risk factors, diagnostic strategies, therapeutic interventions, and outcomes of this condition. The objective was to nucleate the best evidence regarding the disorder in order to facilitate early detection and treatment recommendations and document adverse outcomes. Web of Science, PubMed, Medline, CINAHL, Cochrane Databases, DARE, and OVID databases were searched using the following keywords: 'arterial' AND 'thrombus' OR 'thrombosis' OR 'thromboembolism' OR 'embolism' AND 'spontaneous' AND 'at birth' OR 'newborn' OR 'neonatal' OR 'fetal' AND 'umbilical cord' OR 'umbilical wall necrosis' AND 'coagulation abnormality' OR 'placenta bits' OR 'ischemic limbs'. The search yielded 172 articles, all of which were case series or single case descriptions. Twenty-seven met inclusion criteria, with a total of 53 newborns and 30 newborn pathology reports. Ultrasound was the preferred method of diagnosis and thromboembolic locations varied with the most common site being umbilical, resulting in embolism and vascular compromise. Treatment interventions and drug dosages were not standardized and ranged from use of anticoagulants to surgery and hyperbaric oxygen. The reported mortality rate was 32.8%. Recurring etiological features facilitated identification of possible sequences of events contributing to the disorder. The literature lacks empirical evidence to affirm causes and predisposing risk factors for timely diagnosis and effective treatment of spontaneous neonatal arterial thromboembolism. Further research is needed to clearly establish the causes and the efficacy of specific treatment options.

Evaluation of the use of low-molecular-weight heparin in neonates: a retrospective, single-center study.

Controversies exist over the currently recommended guidelines for the use of low-molecular-weight heparin (LMWH) in neonates. We retrospectively studied 30 neonates treated with LMWH and found a poor therapeutic response to recommended doses as measured by anti-Xa levels. Sixty percent of the study participants required their doses to be increased because of subtherapeutic anti-Xa levels during the initial course of their treatment. The mean starting enoxaparin dose was 1.53 ± 0.38 mg/kg. The mean enoxaparin dose, once therapeutic anti-Xa levels had been achieved, was 1.86 ± 0.50 mg/kg. Preterm and term infants required doses of 2.06 ± 0.61 mg/kg and 1.67 ± 0.26 mg/kg, respectively, to achieve therapeutic anti-Xa levels. In summary, our results suggest that higher initial doses are required to achieve therapeutic anticoagulation in neonates.

Diagnosis and treatment of intracranial hemorrhage in children with hemophilia.

Intracranial hemorrhage (ICH) is a significant complication for children with hemophilia. Identifying risk factors may allow us to establish clinically relevant guidelines for the diagnosis and management of ICH. The purpose of this review is to nucleate evidence from the available literature on the incidence, risk factors, presentation, treatment, and outcomes of ICH that can be utilized to develop a clinically useful framework for the diagnosis and management of hemophiliac patients with the condition. An electronic MEDLINE and EMBASE literature search was undertaken using the key words 'intracranial hemorrhage and hemophilia' and setting limits as: Last 10 years and Review or Randomized Controlled Trial (RCT) or Clinical Trial, or Practice Guidelines. Following review of all articles using predetermined search words and criteria, 31 were retrieved with sufficient data to address our objectives. An algorithm is presented for the management of children (≥3 years-18 years) with hemophilia and suspected ICH. A standardized approach to ICH may reduce unnecessary exposure to radiation via computed tomography scan in a select group of children. Currently there is limited scientific evidence to recommend a diagnostic and therapeutic algorithm for neonates with hemophilia.

Neonatal and infant pulmonary thromboembolism: a literature review.

Pulmonary thromboembolism (PTE) is rare in neonates and infants; however evidence suggests it is underdiagnosed. The primary objective is to conduct a scientific review to determine if the presentation, diagnosis, treatment and outcomes of neonates and infants with PTE are consistent across studies. Secondly, to develop an algorithm to establish the diagnosis and management of the condition based on current information. Two authors searched the literature independently using existing databases and verified that identical articles were assembled. Infants aged less than 1 year with PTE were included and further categorized into neonates 28 days or less and infants 29 days to 1 year or less. Forty-five articles with 157 cases (121 neonates; 36 infants) were identified with PTE. All of the reports were descriptive and neither randomized controlled trials nor prospective or case-control studies were identified. The reports are sub-classified into cases of pulmonary air embolism (PAE) with a higher mortality rate and patients with PTE. Diagnostic and treatment strategies varied widely and were individually case-based, dependent on clinical findings, which influenced patient outcomes. Scientific data to guide an evidence-based, diagnostic and treatment approach to PTE is limited because of the absence of rigorous clinical trials. Large scale, multicenter collaborative studies are required to firmly establish the management of PTE in this population.

Do children with central venous line (CVL) dysfunction have increased risk of symptomatic thromboembolism compared to those without CVL-dysfunction, while on cancer therapy?

BACKGROUND: Thromboembolism (TE) and infection are two common complications of central venous line (CVL). Thrombotic CVL-dysfunction is a common, yet less studied, complication of CVL. Two retrospective studies have reported significant association of CVL-dysfunction and TE. Recent studies indicate association of CVL-related small clot with infection. Infection is the most common cause of non-cancer related mortality in children with cancer. We and others have shown reduced overall survival (OS) in children with cancer and CVL-dysfunction compared to those without CVL-dysfunction. Despite these observations, to date there are no prospective studies to evaluate the clinical significance of CVL-dysfunction and it's impact on the development of TE, infection, or outcome of children with cancer. STUDY DESIGN: This is a prospective, analytical cohort study conducted at five tertiary care pediatric oncology centers in Ontario. Children (≤ 18 years of age) with non-central nervous system cancers and CVL will be eligible for the study. Primary outcome measure is symptomatic TE and secondary outcomes are infection, recurrence of cancer and death due to any cause. Data will be analyzed using regression analyses. DISCUSSION: The overall objective is to delineate the relationship between CVL-dysfunction, infection and TE. The primary aim is to evaluate the role of CVL-dysfunction as a predictor of symptomatic TE in children with cancer. We hypothesize that children with CVL-dysfunction have activation of the coagulation system resulting in an increased risk of symptomatic TE. The secondary aims are to study the impact of CVL-dysfunction on the rate of infection and the survival [OS and event free survival (EFS)] of children with cancer. We postulate that patients with CVL-dysfunction have an occult CVL-related clot which acts as a microbial focus with resultant increased risk of infection. Further, CVL-dysfunction by itself or in combination with associated complications may cause therapy delays resulting in adverse outcome.This study will help to identify children at high risk for TE and infection. Based on the study results, we will design randomized controlled trials of prophylactic anticoagulant therapy to reduce the incidence of TE and infection. This in turn will help to improve the outcome in children with cancer.

Parent health literacy and satisfaction with plain language education materials in a pediatric surgery outpatient clinic: a pilot study.

BACKGROUND: Although significant, the issue of health literacy (HL) among parents attending pediatric surgery outpatient clinics has received little attention. PURPOSE: The objectives of this study are to determine the HL skills of parents attending the pediatric surgery outpatient clinic at McMaster Children's Hospital and to describe parent satisfaction with plain language materials. METHODS: This cross-sectional study was conducted at the pediatric surgery outpatient clinic at McMaster Children's Hospital. Using convenience sampling for 4 months, parents were recruited and interviewed regarding their demographic status. The Newest Vital Sign tool was used to assess HL. Feedback on the plain language education material was received. RESULTS: Seventy-nine individuals were recruited, with a recruitment rate of 62%. Seventy-one percent had adequate HL. English as a first language and Canada as the place of birth were significantly correlated with adequate HL (r = 0.367, P < .001; r = 0.259, P < .05). Parents reported satisfaction with the plain language material, regardless of their HL level. CONCLUSION: Twenty-nine percent of parents showed inadequate HL, likely an underestimate owing to study limitations. Parents expressed satisfaction with the plain language material, emphasizing the need for clear, effective communication with patients and families. Future directions include evaluating staff knowledge of a universal precautions approach to health communication and the accessibility of plain language materials.

Delineating the role of a cohort of clinical research nurses in a pediatric cooperative clinical trials group.

PURPOSE/OBJECTIVES: To describe the roles and responsibilities of the clinical research nurse (CRN). DESIGN: A descriptive design was used to reveal the roles of pediatric oncology CRNs. SETTING: The Children's Oncology Group (COG) password protected Web site. SAMPLE: 85 nurses who performed clinical research associate work within COG. METHODS: The Clinical Trials Nursing Questionnaire was used to investigate the roles and responsibilities of CRNs. MAIN RESEARCH VARIABLES: Protocol assessment, protocol planning, subject recruitment, informed consent process, investigational product, implementation and evaluation, data management, and professional nursing role. FINDINGS: The study found that 55% of respondents (n = 47) were employed in a hospital setting, the majority (81%) had more than five years of oncology experience, and the average age of respondents was 45.56 years (range = 24-65 years). CRNs rated all role components as very important, with the consent process being of greatest importance. Eighty-nine percent reported experiencing autonomy and independence in the role. CONCLUSIONS: Clinical specialization of RNs has increased significantly in the past several decades. Acknowledging that nurses are responsible for performing many different roles that are critical to the successful completion of clinical trials is crucial. IMPLICATIONS FOR NURSING: Evaluation of this dual role is still in its infancy, but articulating the role of CRNs in the conduct and context of clinical research is an important first step.

Collaborative multidisciplinary team approach to fertility issues among adolescent and young adult cancer patients.

Cancer treatment and the field of reproductive technology have each made impressive advancements in the last decade. Improved cancer treatment and survival rates have increased the number of cancer survivors, who might benefit from an array of fertility preservation strategies provided by emerging and advanced assisted conception technology. The challenge becomes bridging the gap between these two separate disciplines to ultimately improve the quality of life for cancer survivors. This paper discusses the issues and process involved with bringing these two teams of health-care professionals together. This model provides a framework for coordinating efforts in providing fertility preservation options to patients undergoing treatment for cancer. Effective multidisciplinary teams that include: oncologists, nurses in the specialties of oncology and infertility, social workers, reproductive endocrinology and infertility specialists, andrologists, and embryologists are required to work together in order to achieve success. The result of this unique team approach is not only a cancer survivor, but one whose quality of life might be enhanced by being able to have a child of his or her own in the future.

Availability and use of palliative care and end-of-life services for pediatric oncology patients.

PURPOSE: Palliative care prevents or relieves the symptoms caused by life-threatening medical conditions. Previous surveys have shown both underuse and lack of availability of these services for children with cancer throughout North America. We sought to investigate the current practices and resources surrounding palliative and end-of-life care among participating institutions of the Children's Oncology Group (COG). METHODS: A survey regarding practices and resources was developed by the COG palliative care subcommittee and was sent to all 232 institutions to complete for the calendar year 2005. RESULTS: The survey was completed by 81% of the institutions. Per institution, there were a mean of 64.6 newly diagnosed patients and 17.7 patients experiencing relapse. A palliative care team was available in 58% of institutions, a pain service in 90%, a hospice in 60%, a psychosocial support team in 80%, and a bereavement program in 59%. Complementary and alternative medicine was available in 39% of institutions and in 95% of the COG institution's community. Most services, even when available, were not well used by patients. CONCLUSION: Despite the well-established benefit of pediatric palliative care, it is only offered in 58% of COG institutions caring for children with cancer. In an era where the benefit of palliative care has been clearly established, this number should approach 100%. Efforts should be directed toward understanding barriers to provision of such services, so that they are available and well used at all childhood cancer centers.

Pediatric oncology sedation trial (POST): A double-blind randomized study.

BACKGROUND: There is limited evidence to support the use of an anti-emetic with the administration of intra-thecal chemotherapy. Nor is there adequate clarity on analgesic strategies for children with cancer undergoing painful procedures. PROCEDURES: A double-blind, randomized, placebo-controlled, factorial trial was performed in children with acute lymphoblastic leukemia undergoing combined bone marrow aspirations and lumbar punctures during maintenance therapy. The study was designed to measure the effect of adding ondansetron and fentanyl to a standard combination of midazolam and propofol. RESULTS: During the first 12 hr following the procedures, patients experienced significantly less vomiting/retching and less disruption of activity while receiving ondansetron, and recorded significantly lower pain scores while receiving fentanyl. CONCLUSIONS: This study provides evidence that the addition of an analgesic (fentanyl) and an anti-emetic (ondansetron) to the combination of a sedative (midazolam) and an anesthetic (propofol) is of measurable benefit in children who undergo procedures that are painful and risk the consequence of nausea and vomiting.

Talking with others who "just know": perceptions of adolescents with cancer who participate in a teen group.

Adolescents with cancer have social support needs that are challenging to meet. A qualitative descriptive study was done using in-person interviews with 11 participants between the ages of 14 and 20. The purpose of the study was to explore and describe adolescent's perceptions of a teen support group. Two main themes emerged: Satisfying Elements and Challenges in the teen group. The subthemes under Satisfying Elements include participants being able to talk to others who "just know," sharing experiences while having fun as a normal teenager, and giving or receiving inspiration. Subthemes under Challenges as identified by participants include ensuring that activities during teen events meet the needs of all participants and dealing with the death of a group member. Study findings suggest that adolescent's feel supported when they are among others in similar circumstances while engaging in normal activity. Offering this type of group for adolescents with cancer provides them with the ongoing opportunities for connection with their peers that is often needed throughout the treatment journey.

Using plain language skills to create an educational brochure about sperm banking for adolescent and young adult males with cancer.

Writing in plain language makes it easier for patients to read, understand, and make informed decisions about sperm banking. Greater attention to the issue and properly designed educational brochures for use by nurses in oncology and reproductive health is of evident importance but of unknown impact. A multidisciplinary clinical team followed an evidence-based, patient-centered approach to develop "plain language" patient education materials about sperm banking for adolescent and young adult (AYA) males with cancer. A patient education booklet was produced and implemented as part of the planned patient education for AYA male oncology patients at McMaster Children's Hospital, Hamilton Health Sciences, in Hamilton, Ontario, Canada. The patient education booklet for use by health professionals as a teaching tool to facilitate discussion with AYA males has been produced with the hope that it will contribute to better informed decision making regarding sperm banking and increased use of this technology for fertility preservation.

Creatine monohydrate attenuates body fat accumulation in children with acute lymphoblastic leukemia during maintenance chemotherapy.

BACKGROUND: Corticosteroids are an important component of the treatment of acute lymphoblastic leukemia (ALL), with known significantly negative effects on bone and muscle. Creatine monohydrate (CrM) supplementation may be an adjunctive therapeutic strategy to attenuate some of these adverse effects. PROCEDURE: Nine children with ALL in the maintenance phase of treatment on the Dana-Farber Cancer Institute (DFCI) protocol 2000-2001 were treated with CrM (0.1 g/kg/day) for two sequential periods of 16 weeks (16 weeks treat > 6 weeks wash-out > 16 weeks treat). A cohort of children (N = 50) who were receiving the same chemotherapy at the same time served as natural history controls. Measurements included height, weight, body mass index (BMI), and lumbar spine bone mineral density (LS-BMD), whole body bone mineral content (WB-BMC), fat-free mass (FFM), and percent body fat (%BF) using dual-energy X-ray absorptiometry. RESULTS: Despite the long course of corticosteroid treatment for ALL, children showed significant increases in height, LS-BMD, WB-BMC and FFM over approximately 38 weeks (P < 0.05) during the study. There was an increase in BMI over time, but children taking CrM had a reduction, while the natural history group showed an increase in % BF (P < 0.05 for interaction). CONCLUSIONS: Children with ALL treated with corticosteroids as part of a maintenance protocol of chemotherapy showed an increase in % BF that was attenuated by CrM supplementation.

Pediatric healthcare quality: A novel approach to lifelong child health research.

Medical science historically focuses on biomedical research about causes and treatment of disease. McMaster Child Health Research Institute (MCHRI), in Hamilton, Ontario, Canada, takes a different approach to research about lifelong health in children with varied chronic conditions. Staff at MCHRI propose to develop research programs to help "children with complicated lives and their families" meet the challenges of growing up in the face of threats to their health and development in order to help both children and families reach their full potential. This "noncategorical approach" to health services research is timely for addressing chronic childhood illnesses and disabilities across the life span.

Discussions regarding sperm banking with adolescent and young adult males who have cancer.

With improved survival rates among cancer patients, attention to fertility preservation is of paramount importance. An open-ended survey was used to question oncology and reproductive health nurses regarding their interactions with male adolescents and young adults with cancer about sperm banking. Four main areas of interest were identified: (a) key staff need to be identified to initiate discussions about sperm banking, (b) most staff felt unprepared for these discussions, (c) staff education was needed, and (d) provision of educational materials would benefit both staff and patients. The use of sperm banking, as part of the treatment protocol for adolescent and young adult males with cancer, requires the expertise and cooperation of a multidisciplinary team of experts from both oncology and fertility. Because nurses are the primary contacts with patients, nurses' role in promoting the discussion around sperm banking is crucial.

Thromboembolism in children with lymphoma.

PURPOSE: Cancer is a major underlying disease in children with thromboembolism. However, the epidemiology of thrombosis in children with cancer is largely unknown. We undertook the following study to define the epidemiology and to identify the risk factors predisposing children with lymphoma to thrombosis. PATIENTS AND METHODS: We reviewed the medical records of 75 children (<18 years of age) diagnosed with lymphoma from January 1990-December 2004. RESULTS: Nine of 75 patients (12%, 95% CI; 5.6-21.6%) were diagnosed with a total of 16 thrombotic events. Twelve of 16 (75%) events were venous thrombosis; 11 were related to the central venous line. The prevalence of pulmonary embolism was 2.6%. Nine of 51 (17.6%) patients with mediastinal lymphadenopathy developed thrombosis compared to none of 21 patients without mediastinal lymphadenopathy (Z=3.31, p=0.001, 95%CI; 7.2-28.1%). Older children and children with advanced stage disease were at higher, but statistically non-significant risk of thrombosis. There was no effect of gender, lymphoma type, or B-symptoms on the occurrence of thrombosis. Four of 9 patients (44%) experienced recurrent clots and 3 (33.3%) patients had post-thrombotic syndrome. Children with thrombosis had a higher rate of adverse events compared to those without thrombosis (41% vs. 21.2%, p=0.126). CONCLUSION: Thrombosis is a frequent complication in children with lymphoma with over a 40% recurrence rate and significant morbidity. Children with a mediastinal mass were at significantly increased risk of thrombosis. Larger prospective studies are required to confirm these findings and to identify children at an increased risk for the development of thrombosis.

Effectiveness of sperm banking in adolescents and young adults with cancer: a regional experience.

BACKGROUND: Improving success in the treatment of cancer has resulted in an increasing number of survivors. An important quality of life issue among younger survivors is the ability to have a family. Current gonadotoxic treatments for cancer pose a challenge to future fertility. Preservation of fertility after gonadotoxic therapy is an important consideration for these patients. In a regional center, the authors evaluated efficacy and utilization of sperm banking for preservation of male fertility in adolescents and young adults (AYA) with cancer. METHODS: A retrospective chart review was conducted to obtain data on clinical features, andrology, and fertility from patients (ages < 30 years) who cryopreserved samples of semen from 1995-2005. RESULTS: Of 821 newly diagnosed male AYA cancer patients, aged 14-30 years, only 146 (17.8%) used sperm cryopreservation technology. Patients who used their cryopreserved semen for attempted conception had a 36.4% success rate with intrauterine insemination (IUI) and a 50.0% clinical pregnancy rate with in vitro fertilization (IVF) and intracytoplasmic sperm injection (ICSI). CONCLUSIONS: Sperm cryopreservation by AYA males with cancer is an efficacious method for preserving future fertility. Awareness and employment of assisted reproductive technologies needs to be implemented by an interdisciplinary team of experts caring for these patients and can result in successful paternity in males after treatment for cancer.

Complementary and alternative medicine in pediatric oncology: availability and institutional policies in Canada--a report from the Children's Oncology Group.

Complementary and alternative medicine (CAM) is used frequently by pediatric oncology patients. A survey exploring the institutional practices and policies surrounding CAM use in pediatric oncology patients was completed by 17 pediatric hematology/oncology centers in Canada. We found that CAM was offered in only 18% of the institutions, but 94% of the communities. Only 6% of oncology divisions made direct referrals to community CAM providers, and only 20% of the centers had policies regarding use of CAM therapies for their patients. Despite published widespread use of CAM therapies, our study demonstrates that institutional CAM resources and policies on CAM are present in much lower proportions.