The journey for adolescents and young adults with chronic conditions transitioning to adult care with successful warfarin management.

UNLABELLED: Health transition of youth from a child-centered care model to the adult model has been recognized to be of critical importance due to the increasing numbers of children now surviving chronic conditions. A formalized transition process is required adequately assess the AYA's readiness for transition and to move towards adult care. Indefinite warfarin therapy poses challenges as warfarin is a narrow therapeutic index drug that requires frequent monitoring and attentiveness to warfarin interactions and affects. OBJECTIVE: The objective of this study was to evaluate transition to adult care for AYAs requiring indefinite warfarin therapy within a structured self-management program. OUTCOME MEASURES: Results were compared between Phase 1 (enrollment to patient self-management) and Phase 2 (independent warfarin management) 6months following confirmation of transition to adult care. There was no statistical difference between outcome measures except INR testing frequency, and no adverse events. CONCLUSIONS: This transition process resulted in successful transition as measured by TTR and other clinical end-points from pediatric to adult care. Implementing a formal transition process for young adults with chronic health conditions that considers patient preferences motivates and empowers them over time to develop autonomy with warfarin self-management, results in successful transition and warfarin management.

Four-factor prothrombin complex concentrates in paediatric patients - a retrospective case series.

BACKGROUND: Four-factor prothrombin complex concentrates (PCCs) are human plasma-derived products containing coagulation factors II, VII, IX and X as well as proteins C and S. They are licensed in many countries for treatment of bleeding or urgent periprocedural prophylaxis in patients with acquired deficiency of prothrombin complex coagulation factors, typically in the setting of vitamin K antagonist (VKA) usage. Efficacy and safety have been established in the adult population, but there is little information in the literature regarding their use for these indications in the paediatric population. We report on our institution's experience with these products in paediatric patients over a five-year period. METHOD: A retrospective case series study was performed, whereby any patient aged 0-16 years who had received PCCs between 2009 and 2013 was identified. Details regarding patient demographics, indications, dose, relevant bloodwork, additional blood products used, adverse effects and general outcomes were obtained through chart and blood bank reviews. RESULTS: A total of sixteen patients were included in our study, fourteen of whom received PCCs in a perioperative setting. Eleven received product for VKA reversal. Significant improvements in PT INRs were observed in those patients who had timely bloodwork drawn. Five others received product for reasons not related to VKA use. One thrombotic event was detected a day following PCC infusion, but causation is uncertain. CONCLUSION: Within study limitations, when used for rapid reversal of VKAs, efficacy and safety of these products in a paediatric population appear to be similar to those reported in adults.

EMPoWARed: Edmonton pediatric warfarin self-management study.

BACKGROUND: Patient self-management (PSM) in adults is safer and more cost effective than conventional management. Warfarin is a narrow therapeutic index drug with individual patient response to changes and frequently a long-term therapy. Children and their families are proposed to be able to effectively manage their child's warfarin therapy. Increased health related quality of life is highly associated with effective therapy in patients with chronic conditions. OBJECTIVES: The aim of this study is to evaluate the safety and efficacy of PSM over time including HRQOL and variables that may influence PFU success at PSM. PATIENTS/METHODS: Children and their family units (PFUs) current performing patient self-testing/monitoring for ≥ 3 months were enrolled in this cohort study. PFUs participated in comprehensive education on warfarin testing and management followed by an apprenticeship. Socio-demographic, clinical, and laboratory data were collected to evaluate safety and efficacy and health related quality of life. Outcomes were compared between the first 6 months on PSM (phase 1) and the last 6 months data collected on PSM (phase 2). RESULTS: Forty-two patients performed PSM for a median of 2.7 years (range: 1.1-6.2 years). Time in therapeutic range was 90% and 92.9% (p=0.30) in phases 1 and 2 respectively. All measures were strongly associated with improved heath related quality of life. PFUs socio-demographic status did not influence success at PSM. All PFUs maintained warfarin knowledge and INR testing competency. Warfarin dosing decision errors median 0 (range: 0-5, p=0.73) and a median 0 (range 0-4, p=0.55) per patient in phases 1 and 2 respectively. There were no adverse hemorrhagic or thrombotic events. CONCLUSIONS: Empowering PFUs to self-manage warfarin results in increased knowledge and understanding of their health condition, improved commitment to their health care and adherence to medication regimens and is demonstrated to be sustainable over time.

EMPoWarMENT: Edmonton pediatric warfarin self-management pilot study in children with primarily cardiac disease.

UNLABELLED: Increasing numbers of children require warfarin thromboprophylaxis. Home INR testing by the patient (PST) has revolutionized warfarin management. However, the family/patient must contact the health team for guidance for warfarin dosing. Patient self management(PSM) prepares a patient performing PST to take an active role in warfarin dosing. Adult studies demonstrate that PSM is safe and effective with improved adherence and treatment satisfaction quality of life (QOL). OBJECTIVE: To estimate the safety and efficacy in children performing PSM or PST, to evaluate warfarin dose decision making in PSM, and warfarin related QOL. METHODS: Warfarinized children performing PST for >3m were randomized to PST or PSM. The PSM group underwent warfarin management education and assumed independent warfarin management. INRs were collected for a year prior to and for 1 year of study to determine TTR and warfarin decision making. QOL was assessed through inventory completion and interviews. RESULTS: 28 children were randomized and followed for 12 months. TTR was (83.9% pre/ post), and 77.7% pre to 83.0% post for PST and PSM (p=0.312). Appropriate warfarin decision making was 90% with no major bleeding episodes and no thromboembolic events. PSM was preferred by families. CONCLUSIONS: PSM for children may be a safe and effective management strategy for warfarinized children. Clinical studies with larger sample size are required.

Development and preliminary evaluation of the KIDCLOT PAC QL: a new health-related quality of life measure for pediatric long-term anticoagulation therapy.

UNLABELLED: Long term anticoagulation (LTA) is hypothesized to induce treatment dissatisfaction influence quality of life (QOL). QOL is measured by a tool developed specific to the patient condition. Pediatric QOL inventory for children on LTA should assess constructs salient for this population. Identification and evaluation of QOL constructs, critical to improve care, and is accepted as the "gold-standard" measurement for patient-centered outcomes in clinical research. OBJECTIVES: To develop and preliminarily validate a pediatric QOL inventory for children/families receiving LTA. Secondary objective was to determine how anticoagulation disrupts children's life. METHODS: Stage 1: Item/theme generation through focus groups and existing inventories, Stage 2: Item reduction, inventory generation and content validity. Stage 3: Inventory refinement, implementation and reliability testing. Responses were evaluated for variability, internal consistency, and scale structure. Item reduction was based on response rate, item variability, and clinical utility. RESULTS: Two inventories, KIDCLOT-PAC-Child -Tween QL and KIDCLOT-PAC Parent-proxy-QL were developed. Content and face validity was assessed by experts, parents, and patients. Internal consistency determined by Cronbach's alpha was good for parent-proxy(0.82) and child(0.89). Pearson correlation was acceptable with >0.5 for test-retest reliability (parent inventory). CONCLUSIONS: KIDCLOT-PAC-QL is the first preliminarily validated inventory to assess QOL in anticoagulated children. The inventory identifies barriers in care and areas for improvement in order to modify care to provide the "best" management (improved QOL associated with safety and efficacy) for children requiring LTA.