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Browsing by Author "Haley, Kristina M."
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Item Laboratory misdiagnosis of von Willebrand disease in post-menarchal females: A multi-center study(Wiley, 2020-09) Jaffray, Julie; Staber, Janice M.; Malvar, Jemily; Sidonio, Robert; Haley, Kristina M.; Stillings, Amy; Weyand, Angela; Hege, Kerry; Jain, Shilpa; Gupta, Sweta; Agnew, Caroline; Wheeler, Allison; Pawar, Anjali; Sharma, Mukta; Chitlur, Meera; O'Brien, Sarah H.; Kouides, Peter; Pediatrics, School of MedicineIncreased awareness of von Willebrand Disease (VWD) has led to more frequent diagnostic laboratory testing, which insurers often dictate be performed at a facility with off-site laboratory processing, instead of a coagulation facility with onsite processing. Off-site processing is more prone to preanalytical variables causing falsely low levels of von Willebrand Factor (VWF) due to the additional transport required. Our aim was to determine the percentage of discordance between off-site and onsite specimen processing for VWD in this multicenter, retrospective study. We enrolled females aged 12 to 50 years who had off-site specimen processing for VWF assays, and repeat testing performed at a consulting institution with onsite coagulation phlebotomy and processing. A total of 263 females from 17 institutions were included in the analysis. There were 251 subjects with both off-site and onsite VWF antigen (VWF:Ag) processing with 96 (38%) being low off-site and 56 (22%) low onsite; 223 subjects had VWF ristocetin co-factor (VWF:RCo), 122 (55%) were low off-site and 71 (32%) were low onsite. Similarly, 229 subjects had a Factor VIII (FVIII) assay, and 67 (29%) were low off-site with less than half, 29 (13%) confirmed low with onsite processing. Higher proportions of patients demonstrated low VWF:Ag, VWF:RCo, and/or FVIII with off-site processing compared to onsite (McNemarʼs test P-value <.0005, for all assays). These results emphasize the need to decrease delays from sample procurement to processing for VWF assays. The VWF assays should ideally be collected and processed at the same site under the guidance of a hematologist.Item Physician decision making in selection of second-line treatments in immune thrombocytopenia in children.(Wiley, 2018-07) Grace, Rachael F.; Despotovic, Jenny M.; Bennett, Carolyn M.; Bussel, James B.; Neier, Michelle; Neunert, Cindy; Crary, Shelley E.; Pastore, Yves D.; Klaassen, Robert J.; Rothman, Jennifer A.; Hege, Kerry; Breakey, Vicky R.; Rose, Melissa J.; Shimano, Kristin A.; Buchanan, George R.; Geddis, Amy; Haley, Kristina M.; Lorenzana, Adonis; Thompson, Alexis; Jeng, Michael; Neufeld, Ellis J.; Brown, Travis; Forbes, Peter W.; Lambert, Michele P.; Pediatrics, School of MedicineImmune thrombocytopenia (ITP) is an acquired autoimmune bleeding disorder which presents with isolated thrombocytopenia and risk of hemorrhage. While most children with ITP promptly recover with or without drug therapy, ITP is persistent or chronic in others. When needed, how to select second-line therapies is not clear. ICON1, conducted within the Pediatric ITP Consortium of North America (ICON), is a prospective, observational, longitudinal cohort study of 120 children from 21 centers starting second-line treatments for ITP which examined treatment decisions. Treating physicians reported reasons for selecting therapies, ranking the top three. In a propensity weighted model, the most important factors were patient/parental preference (53%) and treatment-related factors: side effect profile (58%), long-term toxicity (54%), ease of administration (46%), possibility of remission (45%), and perceived efficacy (30%). Physician, health system, and clinical factors rarely influenced decision-making. Patient/parent preferences were selected as reasons more often in chronic ITP (85.7%) than in newly diagnosed (0%) or persistent ITP (14.3%, P = .003). Splenectomy and rituximab were chosen for the possibility of inducing long-term remission (P < .001). Oral agents, such as eltrombopag and immunosuppressants, were chosen for ease of administration and expected adherence (P < .001). Physicians chose rituximab in patients with lower expected adherence (P = .017). Treatment choice showed some physician and treatment center bias. This study illustrates the complexity and many factors involved in decision-making in selecting second-line ITP treatments, given the absence of comparative trials. It highlights shared decision-making and the need for well-conducted, comparative effectiveness studies to allow for informed discussion between patients and clinicians.Item Second-line treatments in children with immune thrombocytopenia: Effect on platelet count and patient-centered outcomes(Wiley, 2019-04-03) Grace, Rachael F.; Shimano, Kristin A.; Bhat, Rukhmi; Neunert, Cindy; Bussel, James B.; Klaassen, Robert J.; Lambert, Michele P.; Rothman, Jennifer A.; Breakey, Vicky R.; Hege, Kerry; Bennett, Carolyn M.; Rose, Melissa J.; Haley, Kristina M.; Buchanan, George R.; Geddis, Amy; Lorenzana, Adonis; Jeng, Michael; Pastore, Yves D.; Crary, Shelley E.; Neier, Michelle; Neufeld, Ellis J.; Neu, Nolan; Forbes, Peter W.; Despotovic, Jenny M.; Pediatrics, School of MedicineImmune thrombocytopenia (ITP) is an autoimmune bleeding disorder with isolated thrombocytopenia and hemorrhagic risk. While many children with ITP can be safely observed, treatments are often needed for various reasons, including to decrease bleeding or improve health related quality of life (HRQoL). There are a number of available second-line treatments, including rituximab, thrombopoietin-receptor agonists, oral immunosuppressive agents, and splenectomy, but data comparing treatment outcomes are lacking. ICON1 is a prospective, multi-center, observational study of 120 children starting second-line treatments for ITP designed to compare treatment outcomes including platelet count, bleeding, and HRQoL utilizing the Kids ITP Tool (KIT). While all treatments resulted in increased platelet counts, romiplostim had the most pronounced effect at 6 months (p=0.04). Only patients on romiplostim and rituximab had a significant reduction in both skin-related (84% to 48%, p=0.01 and 81% to 43%, p=0.004) and non-skin-related bleeding symptoms (58% to 14%, p=0.0001 and 54% to 17%, p=0.0006) after 1 month of treatment. HRQoL significantly improved on all treatments. However, only patients treated with eltrombopag had a median improvement in KIT scores at 1 month that met the minimal important difference (MID). Bleeding, platelet count, and HRQoL improved in each treatment group, but the extent and timing of the effect varied among treatments. These results are hypothesis generating and help to improve our understanding of the effect of each treatment on specific patient outcomes. Combined with future randomized trials, these findings will help clinicians select the optimal second-line treatment for an individual child with ITP.