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Summary: Sickle cell disease (SCD) arises from beta‐globin gene mutations, with global estimates indicating around 500 000 affected neonates in 2021. In the United States, it is considered rare, impacting fewer than 200 000 individuals. The key pathogenic flaw lies in mutant haemoglobin S, prone to polymerization under low oxygen conditions, causing erythrocytes to adopt a sickled shape. This leads to complications like vascular occlusion, haemolytic anaemia, inflammation and organ damage. Beyond erythrocyte abnormalities however, there is a body of literature highlighting the hypercoagulable state that is likely a contributor to many of the complications we see in SCD. The persistent activation of the coagulation cascade results in thromboembolic events, notably venous thromboembolism (VTE) which is independently associated with increased mortality in both adults and children with SCD. While the increased risk of VTE in the SCD population seems well established, there is a lack of guidelines for thromboprophylaxis in this population. This Wider Perspective will describe the hypercoagulable state and increased thrombosis risk in the SCD population, as well as advocate for the development of evidence‐based guidelines to aid in the prevention of VTE in SCD. [ABSTRACT FROM AUTHOR]

OBJECTIVES: In 2009, a large multicenter study demonstrated that the rate of pediatric venous thromboembolism (VTE) across US children's hospitals had significantly increased from 2001 to 2007. The objective of this study was to evaluate the rate of pediatric VTE from 2008 to 2019 using similar methodology. METHODS: A retrospective cohort study using the Pediatric Health Information System (PHIS) database. Subjects from birth to <18 years admitted from 2008 through 2019 who had an ICD-9-CM or ICD-10-CM code for VTE were included. Demographics, underling medical comorbidities and mortality were collected. VTE location and anticoagulation data during admission were extracted. RESULTS: During the 12-year study period, there were 52 401 hospital admissions among 39 713 pediatric patients with a diagnosis of VTE. The VTE admission rate increased from 46 VTE cases per 10 000 admissions in 2008 to 106 VTE cases per 10 000 admissions in 2019, a 130% increase (P < .0001) in VTE events. The median age at admission was 6.1 years, and almost one-third (31.3%) of patients with VTE were in the adolescent age group (13-17 years). Most patients (78%) had an underlying chronic medical condition. CONCLUSIONS: The rate of VTE in hospitalized pediatric patients continues to increase from a 70% increase reported from 2001 to 2007 to the 130% increase from 2008 to 2019. These findings support the need for more effective VTE prevention strategies. Clinical trials focused on risk stratification and VTE prevention are needed. [ABSTRACT FROM AUTHOR]

Heavy menstrual bleeding (HMB) occurs in up to 40% of adolescent girls, significantly affecting their daily activities. Identifying alternative treatment strategies for HMB is particularly important for adolescents who prefer not to take hormonal contraception. Our objective was to determine whether use of tranexamic acid (TA) would increase health-related quality of life and decrease menstrual blood loss (MBL) in adolescents with HMB. In an open-label, multi-institutional, single-arm, efficacy study, patients 18 years of age or younger with HMB were treated with oral TA 1300 mg 3 times daily during the first 5 days of menses and monitored over the course of 4 menstrual cycles (1 baseline; 3 treatment cycles). Assessment of MBL was performed using the Menorrhagia Impact Questionnaire (MIQ) and the Pictorial Blood Assessment Chart. The MIQ includes Likert scale items, validated to assess the influence of HMB on quality of life. In previous studies, a 1-point decrease or more in score correlated with clinically significant improvement. Thirty-two patients enrolled in the study, and 25 had sufficient follow-up data to be deemed evaluable. The mean age of the participants was 14.7 years (range, 11-18 years). There was an overall improvement in all items of the MIQ, with a greater than 1-point improvement in the MIQ perceived blood loss scale. When using TA, mean Pictorial Blood Assessment Chart score improved by 100 points. There were no medication-related serious adverse events. Use of TA in female adolescents with HMB is well tolerated and leads to clinically meaningful reduction in MBL. [ABSTRACT FROM AUTHOR]

Heavy menstrual bleeding (HMB) is frequently reported by adolescents. The role of the hematologist is threefold in evaluating such patients: (1) perform a clinical and laboratory evaluation for an underlying bleeding disorder on the basis of the degree of clinical suspicion, (2) identify and manage any concomitant iron deficiency, and (3) provide input to the referring provider regarding the management of HMB, particularly for patients with identified hemostatic defects. Several clues in the menstrual history should raise suspicion for an underlying bleeding disorder, such as menses lasting >7 days, menstrual flow which soaks >5 sanitary products per day or requires product change during the night, passage of large blood clots, or failure to respond to conventional therapies. A detailed personal and family history of other bleeding symptoms should also be obtained. Iron deficiency with and without anemia is commonly found in young women with HMB. Therefore, it is important to obtain measures of hemoglobin and ferritin levels when evaluating these patients. Iron supplementation is often a key component of management in the adolescent with heavy menses and is still needed in those who have received packed red cell transfusions as a result of severe anemia. Strategies for decreasing menstrual blood flow are similar for adults and adolescents with heavy menses, with combined hormonal contraceptives recommended as first-line therapy. However, there are adolescentspecific considerations for many of these agents, and they must be incorporated into shared decision-making when selecting the most appropriate treatment. [ABSTRACT FROM AUTHOR]

Anticoagulant-associated heavy menstrual bleeding (HMB) is an underrecognized but not uncommon problem in clinical practice. Premenopausal women should be advised of the potential effect of anticoagulant therapy on menstrual bleeding at the time of treatment initiation. Consequences of HMB should be assessed and treated on an ongoing basis. In the acute setting, the decision to withhold anticoagulants is based on an individual patient's risk of thrombosis and the severity of the bleeding. For women who require long-term anticoagulation, a levonorgestrel intrauterine system, tranexamic acid (during menstrual flow), high-dose progestin-only therapy, or combined hormonal contraceptives are effective for controlling HMB. The risk of thrombosis during anticoagulant therapy with these treatments is not well studied but is likely to be low. Selection of type of hormonal therapy is based on patient preference, other indications for and contraindications to therapy, adverse effect profile, and ongoing thrombotic risk factors. Women who do not respond to medical treatment or who do not wish to retain their fertility should be considered for surgical management. [ABSTRACT FROM AUTHOR]

Objective: To investigate safety of hormonal contraception with regard to thromboembolic events in women with type 1 or 2 diabetes.Research Design and Methods: We used data from 2002-2011 in Clinformatics Data Mart to identify women in the U.S., 14-44 years of age, with an ICD-9-CM code for diabetes and a prescription for a diabetic medication or device. We examined contraceptive claims and compared time to thromboembolism (venous thrombosis, stroke, or myocardial infarction) among women with diabetes dispensed hormonal contraception using a modification of Cox regression to control for age, smoking, obesity, hypertension, hyperlipidemia, diabetic complications, and history of cancer; we excluded data for 3 months after women gave birth.Results: We identified 146,080 women with diabetes who experienced 3,012 thromboembolic events. Only 28% of reproductive-aged women with diabetes had any claims for hormonal contraception, with the majority receiving estrogen-containing oral contraceptives. Rates of thromboembolism were highest among women who used the contraceptive patch (16 per 1,000 woman-years) and lowest among women who used intrauterine (3.4 per 1,000 woman-years) and subdermal (0 per 163 woman-years) contraceptives. Compared with use of intrauterine contraception, progestin-only injectable contraception was associated with increased risk of thromboembolism (12.5 per 1,000 woman-years; adjusted hazard ratio 4.69 [95% CI 2.51-8.77]).Conclusions: The absolute risk of thromboembolism among women with type 1 or 2 diabetes using hormonal contraception is low. Highly effective, intrauterine and subdermal contraceptives are excellent options for women with diabetes who hope to avoid the teratogenic effects of hyperglycemia by carefully planning their pregnancies. [ABSTRACT FROM AUTHOR]

Heavy menstrual bleeding (HMB) is a common symptom in patients who present to the obstetrician-gynecologist or adolescent medicine specialist and might result from an underlying inherited bleeding disorder. Whereas relatively common bleeding disorders such as von Willebrand disease are often included in standard laboratory assessments, rarer platelet function disorders can be challenging to diagnose. Additionally, HMB can be a particularly difficult symptom to manage in adolescents with platelet function disorders, and it is associated with decreased quality of life. We review the diagnostic and management issues of patients with platelet function disorders through the presentation of 2 patient case reports, with a focus on a diagnosis of Glanzmann thrombasthenia, an inherited qualitative disorder that affects platelet function. Whereas the first patient presented to the emergency department before the diagnosis of a bleeding disorder and required a hematologic referral and extensive laboratory assessments, the second patient had been diagnosed with Glanzmann thrombasthenia as a child but experienced severe management challenges at the onset of menarche. In both patients, collaboration between the obstetrician-gynecologist or adolescent medicine specialist and the hematologist was critical for achieving acute management of the bleeding symptoms and for ensuring optimal long-term disease management. Together, these cases highlight the importance of properly identifying females with HMB who might have an undiagnosed bleeding disorder and of consulting with a hematologist to determine an appropriate management plan throughout all life stages. [ABSTRACT FROM AUTHOR]

Venous thromboembolism (VTE) is a rare but serious complication of combined hormonal contraception. While the absolute risk of VTE is low in adolescents, thrombotic events in contraception users younger than the age of 20 years account for 5 to 10% of total contraception-related VTE events in population studies, because of the high frequency of contraception use in adolescents. An increased risk of VTE exists not only with oral contraceptives, but also the contraceptive patch and vaginal ring. Most adolescents who experience contraception-related VTE have additional transient or inherited thrombotic risk factors at the time of VTE. Although the presence of inherited thrombophilia impacts the risk of contraception-related VTE, thrombophilia screening before contraception prescribing should be targeted only to high-risk populations. Pediatric institutions, caregivers, and young women need to be aware of the risk of VTE with estrogen-containing contraception, and maintain a high index of suspicion for this complication in women using these agents. [ABSTRACT FROM AUTHOR]

Given the rising incidence of thrombotic complications in paediatric patients, understanding of the pharmacologic behaviour of anticoagulant drugs in children has gained importance. Significant developmental differences between children and adults in the haemostatic system and pharmacologic parameters for individual drugs highlight potentially unique aspects of anticoagulant pharmacology in this special and vulnerable population. This review focuses on pharmacologic information relevant to the dosing of unfractionated heparin, low molecular weight heparin, warfarin, bivalirudin, argatroban and fondaparinux in paediatric patients. The bulk of clinical experience with paediatric anticoagulation rests with the first three of these agents, each of which requires higher bodyweight-based dosing for the youngest patients, compared with adults, in order to achieve comparable pharmacodynamic effects, likely related to an inverse correlation between age and bodyweight-normalized clearance of these drugs. Whether extrapolation of therapeutic ranges targeted for adult patients prescribed these agents is valid for children, however, is unknown and a high priority for future research. Novel oral anticoagulants, such as dabigatran, rivaroxaban and apixaban, hold promise for future use in paediatrics but require further pharmacologic study in infants, children and adolescents. [ABSTRACT FROM AUTHOR]

Abstract: Background: Access to effective family planning is of great importance for women with sickle cell disease (SCD) due to the increased frequency of pregnancy complications. However, little is known regarding use of hormonal contraception, complications of contraception, and pregnancy in young women with SCD. Study Design: We abstracted diagnostic codes, procedure codes and pharmacy claims from women with SCD, aged 13–21 years, enrolled in Michigan Medicaid between 1/1/2000 and 12/31/2003 with ≥9 months of continuous eligibility. Results: We identified 250 women with SCD with an average period of continuous Medicaid enrollment of 20 months. Claims for hormonal contraception could be identified in only 20 patients (8%, 95% CI 5–12%). The most commonly prescribed method (n=12) was depot medroxyprogesterone (DMPA) acetate. Venous thromboembolism and osteopenia were rarely identified (n≤10) and occurred only in women with no recorded use of hormonal contraception. In the total population, 64 patients had one pregnancy and 16 had ≥2 pregnancies during the 4-year study period. Among adolescents (13–18 years), 49 (30%) of 195 patients (95% CI 24–37%) had 59 pregnancies. By 12 weeks postpartum, few patients (n≤10) with a record of delivery had hormonal contraception claims. Conclusions: In a publicly insured population of young women with SCD, pregnancy was more commonly identified than hormonal contraception use. Our work suggests that significant gaps may exist in family planning care for young women with SCD. [ABSTRACT FROM AUTHOR]

There are few studies in the pediatric population regarding the use of magnetic resonance venography to rule out cerebral venous sinus thrombosis in patients presenting with signs and symptoms of idiopathic intracranial hypertension. The purpose of this study was to compare the clinical characteristics of children with presumed idiopathic intracranial hypertension who did and did not undergo venography during their evaluation at a single children's hospital. The authors found that 45 of 68 (66%) patients underwent magnetic resonance venography. Five of 45 (11%) venography studies revealed a thrombosis. There were no significant clinical differences within patients based on the completion of venography or the presence of thrombosis. Larger studies are needed to identify risk factors for thrombosis in children with presumed idiopathic intracranial hypertension, as well as factors influencing physician decision making in the use of magnetic resonance venography in this evaluation. [ABSTRACT FROM AUTHOR]

Abstract: Background: Little is known regarding how prescribing patterns of hormonal contraceptives to adolescents in the United States have changed over time as new formulations become available and knowledge of thrombosis risk increases. Desogestrel and high-dose estrogen-containing formulations have been associated with a higher risk of thrombosis than other methods. Study design: We used the National Ambulatory Medical Care/National Hospital Ambulatory Medical Care surveys to describe trends in contraception prescriptions for 11- to 21-year-old females between 1993 and 2004. Results: The majority of prescriptions arose from obstetrics/gynecology clinics. The proportion of desogestrel-containing prescriptions was ∼10% throughout the study period. Ortho Evra (transdermal norelgestromin, ethinyl estradiol) and Yasmin (oral drospirenone, ethinyl estradiol) accounted for a steadily increasing proportion of prescriptions. Conclusion: With regard to contraception and thrombosis risk, a significant minority of adolescents are prescribed desogestrel-containing contraceptives, and a greater number are exposed to higher levels of estrogen from transdermal contraception. Future studies are needed to evaluate the absolute risk of thrombosis in adolescents using these formulations so clinicians have accurate safety information when prescribing to this unique population. [Copyright &y& Elsevier]

The authors discuss the activated partial thromboplastin time (aPTT) in children who receive unfractionated heparin (UFH), which one of the significant therapeutic modality for anticoagulation in children. They explore a study by Hanslik and colleagues within the issue of "Blood" journal, which compares two dose protocols for UFH in children with cardiac catheterization and shows poor agreement between anti-Xa, aPTT, and activated clotting times (ACTs).

Real-time tracking of menstrual bleeding is a barrier to research due to limitations with traditional data collection tools. This prospective cohort study utilized a mobile application (TDot app) in young adolescents aged 10-14 years to assess the relationship between heavy menstrual bleeding (HMB), dysmenorrhea, and activity limitation. Menstrual cycles were captured over six months in real-time using the Pictorial Blood loss Assessment Chart (PBAC). A median PBAC score of >100 was used to identify participants with HMB. Participants also completed a modified WaLIDD (Working ability, Location, Intensity, Days of pain, Dysmenorrhea) scale. Impact of menses on daily activities was collected for each cycle. A total of 160 participants enrolled and 100 (63%) participants with ≥3 cycles recorded in the mobile app were analyzed. HMB was noted in 41% of participants. Median modified WaLIDD score was significantly higher in participants with HMB than those without HMB (p=0.01). No significant differences were found in activity limitations between participants with and without HMB (p=0.34). Median modified WaLIDD score for participants with activity limitation was significantly higher than those without activity limitation (p=0.01). Utilizing mobile app technology, we were able to gather real-time menstrual outcome data from young adolescents on heaviness of flow, dysmenorrhea and activity limitations. While we did not find that patients with HMB were more likely to have activity limitations, we did find that those with limitations had modestly higher dysmenorrhea scores. Future studies should focus on identifying additional variables that impact activity limitation during menstruation. [ABSTRACT FROM AUTHOR]

The article focuses on the difference in symptoms for von Willebrand disease (VWD) between men and women. It discusses the heavy menstrual bleeding (HMB) in women as a common symptom, which mostly goes underreported, how procedure codes were revealed for the identification of women who underwent hysterectomy.

Purpose: To examine processes, barriers, and facilitators to sperm banking counseling and decision-making for adolescent males newly diagnosed with cancer from the perspective of clinicians who completed Oncofertility communication training. We also identify opportunities for improvement to inform future interventions and implementation. Methods: A survey (N=104) and subsequent focus groups (N=15) were conducted with non-physician clinicians practicing in pediatric oncology who completed Oncofertility communication training. Results: Most survey participants were confident in communicating about the impact of cancer on fertility (n=87, 83.7%) and fertility preservation options (n=80, 76.9%). Most participants reported never/rarely using a sperm banking decision tool (n=70, 67.3%), although 98.1% (n=102) said a decision tool with a family-centered approach would be beneficial. Primary themes in the subsequent focus groups included variable processes/workflows (inconsistent approaches to consult initiation; involvement of adolescents, caregivers, and various clinician types; assessment of puberty/sexual experience), structural and psychosocial barriers (cost and logistics, developmental, cultural, clinical acuity/prognosis), and facilitators (educational materials, alternative options for banking). Opportunities and strategies for improvement (including fertility preservation in existing research protocols; additional staffing/resources; oncologist education and buy-in; and development of decision tools) were informed by challenges identified in the other themes. Conclusion: Barriers to adolescent sperm banking remain, even among clinicians who have completed Oncofertility training. Although training is one factor necessary to facilitate banking, structural and psychosocial barriers persist. Given the complexities of offering sperm banking to pediatric populations, continued efforts are needed to mitigate structural barriers and develop strategies to facilitate decision-making before childhood cancer treatment. [ABSTRACT FROM AUTHOR]

Abstract Thrombotic events in children differ from those in adults in epidemiology, pathophysiology, and anatomical location. However, anticoagulation in children is mostly based on evidence from adults while scarce evidence exists from children. The classical anticoagulants currently used in children have several limitations, resulting in the need for regular monitoring. Several direct oral anticoagulants (DOACs) are now authorized for adults in whom they have established efficacy and safety without the need for monitoring. Given their pharmacological properties and the special characteristics of children requiring anticoagulation, the DOACs have the potential to be particularly suitable for children. All currently approved DOACs have paediatric development plans, targeting various indications for prevention and treatment of thrombosis. Paediatric formulations are being developed and systematic age-specific dosing information will be generated. Whether therapeutic drug monitoring will be necessary in certain situations in children remains to be elucidated. The results of ongoing clinical studies still need to demonstrate whether there is a positive benefit-risk balance in all targeted paediatric indications and age-groups, particularly in indications that have not been explored in adults, such as catheter-related thrombosis or congenital heart disease. If the advantages of DOACs bear out in the results of the current paediatric studies, they will likely be used widely in children. As of now, the DOACs should not be used routinely in children as there is still insufficient information on appropriate dosing, safety and efficacy. The paediatric community is encouraged to promote participation of children and adolescents into the multiple ongoing studies of DOACs. Highlights • The classical anticoagulants have several limitations for use in children. • The direct oral anticoagulants (DOACs) may be particularly suitable for children. • All DOACs have developmental plans ongoing in children for various indications. • Paediatric formulations, dosing, efficacy and safety are being assessed in children. • Participation of children in studies of DOACs should be encouraged. [ABSTRACT FROM AUTHOR]

Background The number of adolescents who are prescribed hormonal contraception (HC) for contraceptive and noncontraceptive indications is increasing. Approximately 1 of 4 female adolescents will use some form of HC by age 19 years. Venous thromboembolism (VTE) is a rare, but life-threatening complication associated with HC use. Although adolescents aged 15 to 19 years have the lowest absolute HC-associated VTE risk, they still account for 5.9% of HC-associated VTE. The absolute HC-associated VTE risk for those younger than 15 years of age is not well described. Objective The objectives of this report are to describe the current literature regarding HC-associated VTE in adolescents, to review the coagulation cascade and in vivo coagulation, to highlight differences between the adolescent and adult coagulation profiles, to discuss coagulation profile changes related to HC use, and to identify knowledge deficits for future study. Conclusion The mechanisms and confounding variables that lead to HC-associated VTE in all adolescents are not well understood because many large HC studies exclude female adolescents younger than 18 years of age. Because of the paucity of data in this age group, observational studies and randomized controlled trials of VTE risk in adolescents on varying forms of HC are needed. Studies should include female adolescents across the entire adolescent age span (12-21 years) to better understand HC-associated VTE risk in this population. [ABSTRACT FROM AUTHOR]

To evaluate the use of intrauterine devices (IUDs) in two young women's hematology clinics and compare adverse events in adolescents with and without inherited bleeding disorders (BDs) Retrospective multicenter cohort study from February 2014 through February 2020 Young women's hematology clinics at Nationwide Children's Hospital in Columbus, Ohio, and Children's Medical Center in Dallas, Texas Female patients evaluated for heavy menstrual bleeding (HMB) who underwent IUD placement Rates of IUD expulsion, malposition, and ongoing HMB requiring additional medical treatment We identified 43 patients with BDs and 35 patients without BDs who underwent placement of an IUD for HMB. The mean age was 14.9 years (range 11.0-21.4 years) at the time of presentation and 15.8 years (range 11.0-21.4 years) at IUD placement. Those with BDs were younger at the time of IUD insertion. Most patients (90%) had previously failed other methods to control HMB. The annual rate of IUD adverse events was 0.25 per year of use, and all adverse events occurred in the first 20 months after placement. There were no significant differences in adverse IUD events in patients with and without BDs, although those without BDs requested IUD removal more frequently. In this cohort of adolescent females, the presence of a BD was not associated with a higher IUD expulsion rate. IUD placement should be considered a first-line option for adolescents with BDs who experience HMB. [ABSTRACT FROM AUTHOR]

Approximately 8% of African Americans born annually have sickle cell trait (SCT), a public health concern that may contribute to health disparities if individuals with SCT do not know it and lack access to understandable information about reproductive implications. Pre-pandemic, Ohio offered in-person SCT education for parents of SCT-affected children but many did not attend. Those with limited health literacy (HL) were less likely to achieve high knowledge. We used a HL-focused evaluation of this education to develop a virtual program (SCTaware) to communicate clear, actionable information and promote knowledge retention. Seven English-speaking parents, three with limited HL, were recruited in 2019 for in-person session videotaping and SCT knowledge assessments. Clinicians, HL experts, educators, genetic counselors, and parent stakeholders reviewed sessions, assessments, and accompanying visuals. Evaluators: observed parents asked few questions; noted undefined technical terms, closed questions, key concept omission, and limited explanation of visuals scoring low for understandability, actionability, and clarity; and developed SCTaware for individual videoconference delivery (knowledge objectives; plain language guide; HL-informed communication strategies; new visuals scoring highly for understandability, actionability, and clarity; narrated post-education version; standardized educator training). Using a HL-focused evaluation, our diverse team created a promising virtual SCT education program addressing a common issue affecting populations at risk for disparities. Given virtual education will likely continue post-pandemic and limited HL is common, this approach may replicable for other public health education programs, especially those transitioning to virtual formats, to convey clear, actionable information and promote health equity. [ABSTRACT FROM AUTHOR]

To assess initial evaluation patterns of patients presenting to the Emergency Department (ED) with abnormal uterine bleeding (AUB) including differences by race Retrospective multicenter cohort study from October 2015 through September 2020 Forty-seven children's hospitals submitting data to the Pediatric Health Information System Female patients aged 8-21 with an ED encounter with AUB as the primary diagnosis code Proportion of visits with at least 1 laboratory assessment for the evaluation of anemia, iron deficiency, and/or hemostatic disorders We identified 17,759 unique patients with AUB seen in the ED who met inclusion criteria. Median age was 16.3 years (IQR, 14.1-17.8 years). Most encounters (n = 11,576, 65.2%) included evaluation for anemia, but only 6.8% (n = 1,215) included assessment for iron deficiency and 26.2% (n = 4,654) for hemostatic disorders. Black patients accounted for 34.7% (n = 6,155) of AUB encounters yet constituted only 25% of all ED encounters (n = 198,192). Black patients with AUB were less likely to undergo bleeding disorder evaluation (OR = 0.76; 95% CI, 0.69-0.83) but more likely to receive evaluation for sexually transmitted infections (OR = 1.63; 95% CI, 1.48-1.80) compared with White patients, despite controlling for age and concomitant pain. In a national cohort of adolescents presenting to the ED with AUB, evaluations for anemia and hemostatic disorders were infrequently performed, and racial differences existed regarding initial assessment. Further studies are needed to understand the factors underlying racial differences in hematologic testing and the impact of this disparity on health outcomes for females with AUB. [ABSTRACT FROM AUTHOR]

Many childhood cancer survivors desire biological children but are at risk for infertility after treatment. One option for mitigating risk is the use of fertility preservation prior to gonadotoxic therapy. Adolescents and emerging adults may rely on their parents to help them decide whether to use fertility preservation. While this is often a collaborative process, it is currently unknown how parents can optimally support adolescents and emerging adults through this decision. To address this gap, we developed a family-centered, psychoeducational intervention to prompt adolescents and emerging adults to reflect on their future parenthood goals and attitudes towards fertility preservation, as well as to prompt their parents (or other caregivers) to reflect on their own and their child's perspectives on the topic. In this randomized controlled trial, families will be randomized to either the standard of care control group (fertility consult) or the intervention group. After their fertility consult, adolescents and emerging adults and parents in the intervention group will complete a fertility preservation values clarification tool and then participate in a guided conversation about their responses and the fertility preservation decision. The primary expected outcome of this study is that participation in the intervention will increase the use of fertility preservation. The secondary expected outcome is an improvement in decision quality. Chi-square analyses and t-tests will evaluate primary and secondary outcomes. The goal of this intervention is to optimize family-centered fertility preservation decision-making in the context of a new cancer diagnosis to help male adolescents and emerging adults achieve their future parenthood goals. [ABSTRACT FROM AUTHOR]

Introduction: In the era of electronic medical records, pen‐and‐paper‐based physician‐administered bleeding assessment tools (BAT) remain under‐utilized in the clinical setting, as they are noted to be time‐consuming. Aim: The current study reviews the use of an electronic self‐administered bleeding assessment tool (eBAT) prospectively in a paediatric haematology clinic and in comparison with a physician administered BAT (pBAT). Materials and Methods: This was reviewed and approved in the current form because the aims statement includes the method regarding comparison of 2 groups. So no additional section required. Results: A total of 94 BAT response pairs were available for analysis. The median time required for patients or parents to complete the eBAT was 8 min, with less than a third of the patients requiring over 10 min. The median bleeding scores noted in this study were 4 for both the BATs, with strong positive correlation between the eBAT and the physician administered bleeding questionnaire. The eBAT had a sensitivity of 93.8% (95% CI 82.8%–98.7%), a specificity of 34.8% (95% CI 21.4%–50.3%), a positive predictive value (PV) of 60.0% (95% CI 54.5%–65.2%) and a negative PV of 84.2% (95% CI 62.5%–94.5%) for identifying a bleeding disorder. Conclusions: Findings indicate that eBAT is a valid and time‐efficient screening tool for evaluating patients' bleeding symptoms, which can improve clinical applicability of BATs by reducing time for bleeding history review. [ABSTRACT FROM AUTHOR]

Reproductive tract bleeding (RTB) is an important outcome in menstruating females on anticoagulant therapy (AC). The diagnosis and management of AC-RTB in adolescent and young adult (AYA) females is unknown. The aim of this study was to survey the contemporary patterns of diagnosis and management of AC-RTB in AYA females. SurveyMonkey® questions were sent to members of 1) Pediatric and Neonatal Thrombosis Hemostasis Subcommittee and Women's Health Subcommittee of the International Society on Thrombosis and Haemostasis and 2) Hemostasis and Thrombosis Research Society. Results are reported using descriptive statistics. Response rate was 33% (251 out of 753). AC-RTB was infrequently reported. Menstrual history was not routinely reviewed prior to initiation of AC. Respondents indicated a differential risk of AC-RTB, most frequently with Rivaroxaban. Respondents continued hormonal therapy (HT) if an AYA female was on it at the start of AC. When AC-RTB occurred, management strategies were variable with initiation of HT or antifibrinolytic therapy being the most frequent. The timing of AC-RTB after the thrombotic event influenced the respondents' choice of therapy. Differences were seen in the management strategies between US and non-US participants, with more US respondents initiating HT while more non-US respondents modifying the AC regimen. Respondents uniformly reported complications with AC-RTB and with its treatment. This survey highlights the need to review menstrual history at the start of and during AC and for future research into choosing the optimal AC in AYA females. The results can inform the design of future studies. • Menstrual history was not routinely reviewed prior to initiation of anticoagulation • Respondents identified a differential risk of reproductive tract bleeding with anticoagulants, mostly with Rivaroxaban • Wide variability was seen in the management of anticoagulation associated reproductive tract bleeding • The timing of bleeding after the thrombotic event influenced the respondents' choice of therapy • Differences were seen in the management strategies between US and non-US participants [ABSTRACT FROM AUTHOR]

Purpose: Over half of males experience fertility impairment after childhood cancer therapy, which often causes psychosocial distress. Yet, fertility preservation (FP) remains underutilized. The goals of this study were to determine the feasibility and impact of implementing a family-centered FP values clarification tool on sperm banking attempts among adolescent males newly diagnosed with cancer, and identify key determinants of banking attempts. Methods: A prospective pilot study was conducted among families of males (12–25 years old), prior to cancer therapy. Thirty-nine of 41 families agreed to participate (95%); 98 participants (32 adolescents, 37 mothers, 29 fathers) completed the Family-centered Adolescent Sperm banking values clarification Tool (FAST). Analyses assessed the impact of the FAST on banking attempts and examined associations between demographic/medical characteristics, FAST subscales (perceived threat, benefits, barriers), and banking attempts. Results: Twenty-three (59%) adolescents attempted to bank, compared to 8 adolescents (33%) during baseline assessment (p=.04). Significant associations were identified between banking attempts and adolescents' report of perceived threat (rpb=.45, p=.01) and benefits (rpb=.57, p=.01). Only mothers' proxy reports of adolescent perceived threat (rpb=.42, p=.01) and benefits (rpb=.47, p=.003) were associated with banking attempts, while fathers' self-reported perceived benefits (rpb=.43, p=.03), self-reported barriers (rpb=.49, p=.01), and proxy reports of adolescent perceived threat (rpb=.38, p=.04) and benefits (rpb=.59, p=.02) were associated with banking attempts. Conclusion: Adolescent sperm banking attempt rates significantly increased after implementation of a family-centered FP values clarification tool prior to cancer treatment. Findings underscore the importance of targeting both adolescents and their parents, particularly fathers, in FP efforts. [ABSTRACT FROM AUTHOR]

Abstract: Study Objective: To define the thrombotic risk factors of young women presenting to a children''s hospital with hormonal contraception-related venous thromboembolism (VTE). We hypothesized that the majority of patients would have additional risk factors for VTE. Design: Clinical and laboratory data obtained retrospectively from electronic medical records concerning history of presentation, body mass index (BMI), medical and family history, medication profile, and relevant laboratory studies. Setting: Nationwide Children''s Hospital in Columbus, Ohio, November 2008-May 2012. Participants: Twenty-six female patients, age 12-21 years, admitted for hormonal contraception-related VTE. Results: Fifty-seven VTE cases were reviewed, and 26 were identified as contraception-related VTE. 96% of patients had at least 1 additional risk factor for VTE, and 42% of patients had 2 or more additional risk factors. 50% patients had a BMI ≥25 kg/m2. 35% of patients had a positive family history of VTE in a first or second-degree relative. 27% of patients were subsequently diagnosed with an inherited thrombophilia, 5 of whom had a positive family history. Conclusion: In a small population of adolescents presenting to a children''s hospital with contraception-related VTE, the majority of patients had multiple risk factors for VTE. Obesity was the most common additional risk factor (50%) identified in our study population. More research is needed regarding the impact of obesity on contraception-related VTE in young women, and whether the presence of obesity should influence thrombophilia screening practices prior to prescribing contraception. [Copyright &y& Elsevier]

Purpose: Research among adults shows benefits and low perceived burden of engaging in behavioral research. However, questions remain regarding the ethics of conducting behavioral research in pediatric populations during sensitive situations, including during a new life-threatening diagnosis or at end-of-life. We examined reactions to participating in a behavioral reproductive research study among male adolescents newly diagnosed with cancer and their parents, as a step towards optimizing fertility preservation utilization in a population where future infertility is common. Methods: Pediatric literature regarding the ethics of behavioral research was reviewed. In our pilot, forty-four participants (19 mothers, 11 fathers, 14 male adolescents newly diagnosed with cancer) from 20 families completed demographic questionnaires and a fertility preservation decision tool developed by the study team. Qualitative interviews exploring the impact of study participation were subsequently conducted. Verbatim transcripts were coded for thematic content using the constant comparison method. Results: Literature review showed positive reactions to research participation among youth/caregivers. In our pilot study, 89% (n = 17) of mothers, 64% (n = 7) of fathers, and 71% (n = 10) of adolescents reported at least one benefit of participating. Eleven percent (n = 2) of mothers, 36% (n = 4) of fathers, and 29% (n = 4) of adolescents said they were not affected; none of the participants reported a negative effect. Conclusion: Consistent with prior literature, our study suggests behavioral reproductive research prior to cancer treatment can offer direct benefits to participants and society, without increasing burden. These findings will inform future interventions to improve long-term psychosocial and reproductive outcomes for youth with cancer. [ABSTRACT FROM AUTHOR]

Purpose: Approximately half of all males experience fertility impairment after cancer treatment, which can diminish quality of life. Parents are often responsible for sharing health-related information, and parental recommendation strongly impacts fertility-related decisions; yet it remains unclear whether adolescents and young adults' (AYAs) and their parents' fertility-related goals/attitudes align. This study examined parent–AYAs congruence on fertility-related attitudes and (grand) parenthood goals during survivorship, and if parents were aware of their sons' parenthood goals and reproductive concerns. Methods: Males (15–25 years) and their parents were recruited within 1–8 years of completing cancer treatment. Based on the Health Belief Model, AYAs (N = 38), mothers (N = 33), and fathers (N = 24) reported on parenthood goals, perceived benefits/barriers of fertility testing/preservation, perceived susceptibility/severity of infertility, and fertility knowledge. Analyses included Pearson's correlations and paired-sample t-tests. Results: More than 80% of mothers, fathers, and AYAs desired future (grand) children. Mother–son dyads had differences in fertility knowledge (p = 0.037), and father–son dyads differed in parenthood goals (p = 0.024). AYAs perceived more fertility-related barriers than their mothers (p = 0.014) and fathers (p = 0.006). AYA survivors were less likely to report they could accept a life without a biological child compared with their mothers (p = 0.009) and fathers (p = 0.024). Conclusions: These findings suggest some similarities, yet important differences between male AYA survivors' and their parents' attitudes toward fertility and parenthood. As infertility is common in this population, and is associated with uncertainty and distress, these findings underscore the need for family-centered fertility-related interventions at the time of cancer diagnosis and throughout survivorship. [ABSTRACT FROM AUTHOR]

• Compared with children (age <15 years), adolescents and young adults (AYA; age 15 to 39 years) with acute leukemias and myelodysplastic syndromes have greater hematopoietic cell transplantation (HCT)-related costs and rates of health care utilization. • Although the median hospital length of stay was similar in children and AYA HCT recipients, the median cost per hospital day was significantly higher in AYA. • Factors driving increased costs included older age, use of cord blood, unrelated donors, occurrence of graft-versus-host disease, any infection, use of dialysis, and mechanical ventilation. Allogeneic hematopoietic cell transplantation (HCT) is a curative option for patients with acute leukemia and myelodysplastic syndromes (MDS) but is associated with significant cost. Compared with children (age <15 years), adolescents and young adults (AYA; age 15 to 39 years) undergoing HCT have an increased risk for transplantation-related complications. However, whether such complications translate into increased resource utilization and costs during HCT remains unknown. Therefore, we conducted a multicenter database study using the Pediatric Health Information System database, an administrative database containing resource utilization data from 49 US tertiary children's hospitals to compare inpatient costs and resource utilization in children and AYA undergoing HCT for acute leukemia and MDS. The International Classification of Diseases, Ninth Revision, Clinical Modification codes were used to identify HCT recipients and transplantation-related complications occurring up to 1 year post-HCT. We identified 1693 HCT recipients at pediatric centers between January 2010 and September 2014. Eighty percent of the total costs (from admission for HCT up to 1 year post-HCT) occurred during the initial transplantation admission. During initial admission, although AYA and children had a similar median length of stay (LOS) of 43 days, AYA incurred significantly greater adjusted costs ($338,458 versus $275,723; P <.001) and costs per hospital day ($7122 versus $5838; P <.001). Median total costs and costs per day during subsequent time periods post-HCT were also significantly greater in the AYA group. In multivariable analysis, increasing age at HCT, LOS, use of cord blood or an unrelated donor, occurrence of any graft-versus-host disease, infection, and use of dialysis or mechanical ventilation were significant drivers of increased cost at initial admission. In conclusion, allogeneic HCT for acute leukemia and MDS is associated with higher costs in AYA recipients than in children. Therefore, directing efforts and resources aimed at reducing HCT-related costs may be advantageous in this high-risk group. [ABSTRACT FROM AUTHOR]

Introduction: Heavy menstrual bleeding (HMB) may be expected for many adolescents after menarche. Accurate assessment of HMB, a key component in the diagnosis of a haemostatic defect (HD), is a well‐recognized challenge. Aim: Our objective was to determine the diagnostic accuracy of an HMB‐specific screening tool for HDs in adolescents with HMB, presenting to a secondary care setting. Methods: Adolescents with HMB were evaluated for a HD at 4 US centres. A screening tool, the Philipp Tool, developed and validated in adult women with HMB, was administered. We modified the tool by assigning a score based on the number of affirmative responses. Sensitivity, specificity and likelihood ratios (LRs) of a positive tool, modified tool, with a pictorial blood assessment chart (PBAC) score >185, and with serum ferritin ≤20 ng/mL were calculated for HDs. Results: Among 248 adolescents with HMB, 29% were diagnosed with HDs. Sensitivity, specificity and LR of a positive screening tool for HDs were 95% (range 88‐99), 14% (9‐21) and 1.1 (1‐1.2), respectively. A score of ≥2, addition of a PBAC score >185 and ferritin ≤20 ng/mL changed the sensitivity, specificity and LR of the tool to 72% (61‐81), 94% (83‐99), 76% (65‐85); 60% (53‐68), 24% (16‐34) and 39% (31‐47) and 1.8 (1.4‐2.2), 1.2 (1.1‐1.4) and 1.2 (1‐1.4), respectively. Conclusion: Although sensitive, the discriminative ability of the tool to identify adolescents with HDs from those without, who presented with HMB, was low. Further research is needed to optimize or develop an adolescent‐specific HMB tool for secondary care settings. [ABSTRACT FROM AUTHOR]

Study Objective To assess the frequency, severity, and inpatient management of girls admitted with heavy menstrual bleeding and iron deficiency anemia at US children's hospitals, with a focus on hematologic considerations. Design Retrospective multicenter cohort study from October 2012 through September 2015. Setting Children's hospitals submitting data to the Pediatric Health Information System. Participants Female patients, age 8-18 years, admitted with heavy menstrual bleeding and anemia as either a primary or secondary diagnosis. Patients with cancer, immune thrombocytopenic purpura, aplastic anemia, and pregnancy were excluded. Interventions and Main Outcome Measures Hemostatic evaluation; provision of iron therapy. Results We identified 1183 admissions (1134 unique patients). Patients’ median (interquartile range) age was 14 (11-17) years. Forty-one percent were Caucasian (n = 480), 31% African American (n = 371), and 26% Hispanic ethnicity (n = 310). Intensive care use occurred in 5% of admissions (n = 56). Hemostatic assessment was inconsistent; 15% (n = 182) had no such evaluation. Two-thirds (n = 797; 67%) involved transfusions, 37% (n = 433) received no inpatient iron therapy, and 17% (n = 197) received no hormonal or antifibrinolytic therapy. Hemostatic evaluation was associated with intensive care use: odds ratio (OR), 4.80 (95% confidence interval [CI], 1.16-19.86; P = .03); emergency department visit: OR, 2.60 (95% CI, 1.86-3.65; P < .01); private insurance: OR, 1.62 (95% CI, 1.12-2.35; P = .01); and younger age: OR, 0.84 (95% CI, 0.77-0.92; P < .01). Conclusion Hundreds of girls with heavy menstrual bleeding and anemia are hospitalized at US children's hospitals each year with variable inpatient hematologic evaluation and management. Future guidelines should emphasize early identification of at-risk patients and promote effective implementation strategies to reduce the burden of this preventable complication. [ABSTRACT FROM AUTHOR]

Objective: To estimate the frequency of von Willebrand disease screening and factors that affect screening frequency in a national sample of girls and adolescents with heavy menstrual bleeding.Methods: In this retrospective cohort study, we used a national claims database for privately and publicly insured patients between 2011 and 2013 for girls aged 10-17 years. Diagnostic criteria of heavy menstrual bleeding were the presence of one inpatient or two outpatient International Classification of Diseases, 9th Revision codes for heavy menstrual bleeding. We defined severe heavy menstrual bleeding as heavy menstrual bleeding plus an inpatient stay for menstrual bleeding, iron deficiency anemia, or blood transfusion. To assess whether patient- or facility-level characteristics affected screening, we performed logistic regression analysis including patient age, health care provider type seen at first visit for menorrhagia, patient residence in a metropolitan statistical area (proxy for urban vs rural inhabitance), and approximate travel time to the nearest hemophilia treatment center.Results: We identified 23,888 postpubertal girls and adolescents with heavy menstrual bleeding (986 with severe heavy menstrual bleeding). Von Willebrand disease screening was performed in 8% of females with heavy menstrual bleeding and 16% with severe heavy menstrual bleeding. Younger age at diagnosis, commercial insurance, and living within a metropolitan statistical area were associated with higher screening rates. Patients who underwent testing for iron deficiency anemia had the highest likelihood of undergoing screening (odds ratio 7.08, 95% CI 6.32-7.93). Among patients living in a metropolitan statistical area, those 60 minutes or more from a hemophilia treatment center were less likely to undergo screening.Conclusion: Despite recommendations by the American College of Obstetricians and Gynecologists for more than 15 years, fewer than 20% of postpubertal girls and adolescents with heavy menstrual bleeding underwent screening for von Willebrand disease in this cohort. Increased clinician awareness and adherence to recommended screening recommendations may increase diagnosis of von Willebrand disease. [ABSTRACT FROM AUTHOR]

Study Objective Heavy menstrual bleeding is a common symptom reported by approximately 30% of women. The Pictorial Blood Assessment Chart (PBAC) score is often used to quantify severity of menstrual bleeding. However, the traditional PBAC paper diary might be subject to recall bias and compliance issues, especially in adolescents. We developed a mobile application (app) version of the PBAC score and evaluated patient satisfaction and compliance with app reporting vs paper reporting. Design, Setting, Participants, Interventions, and Main Outcome Measures This study was a randomized cross-over study of 25 postmenarchal female adolescents and young women ages 13-21 years. Participants agreed to track bleeding in 2 consecutive menstrual cycles and were randomized to use the PBAC paper diary or mobile app format first. At the end of each cycle, a satisfaction survey and system usability scale (app only) was used to assess the acceptability of the format used. Results Twenty-five participants had a median age of 15 years. Cross-over analysis showed that satisfaction level was significantly higher for the app ( P < .001). Twenty of 25 (80%) participants preferred the app over the paper diary. For the app, 20 of 25 participants (80%) had high compliance for reporting bleeding, with a mean of 2 app entries per day. Participants' PBAC scores did not vary significantly between the paper diary (median, 95) and mobile app (median, 114). All paper diaries met definition for high compliance. There was no significant period or carryover effect. Conclusion This study showed that a PBAC app compared with the paper diary was the preferred method of recording menstrual bleeding in adolescents and showed feasibility as a research data collection tool. [ABSTRACT FROM AUTHOR]

Hematopoietic cell transplant (HCT) is associated with a proinflammatory, procoagulant environment that places recipients at increased risk of venous thromboembolism (VTE). Although the incidence of VTE in adult HCT recipients has been extensively studied, similar data for children are lacking. We conducted a multicenter retrospective study to analyze the prevalence of VTE and associated risk factors in a large cohort of patients who underwent HCT at tertiary care US children's hospitals. The Pediatric Health Information System database, a large administrative database that contains clinical and resource utilization data from 49 freestanding children's hospitals in the United States, was used to extract data. International Classification of Diseases, Ninth Revision, Clinical Modification codes were used to identify HCT recipients, VTE events, post-HCT complications, and associated risk factors up to 1 year post-transplant. Data on patients who received HCT from January 2010 through September 2014 were collected. A total of 4158 unique patients mean ± standard deviation age at transplant admit, 8.8 ± 6.5 years; range, birth to 33.4 years) were identified. After HCT 290 subjects (6.9%) developed VTE. VTE prevalence was greater in patients aged ≥ 13 versus <13 years (8.54% versus 6.33%; P = .01) and in recipients of allogeneic versus autologous grafts (7.7% versus 5%; P ≤ .01). VTE was associated with prolonged median duration of hospitalization (81 versus 54 days; P ≤.01) and increased 1-year mortality (13.9% versus 5.9%; P ≤ .01). Infections and presence of any graft-versus-host disease (GVHD) were significantly associated with VTE occurrence in recipients of allogenic grafts. Prevalence of VTE in patients who underwent HCT at pediatric tertiary care hospitals is about 7%. Age ≥ 13 years and allogeneic grafts were significant pre-HCT VTE risk factors, with GVHD and infections seen more frequently in patients with VTE. [ABSTRACT FROM AUTHOR]

Background Despite universal screening that detects sickle cell trait ( SCT) in infancy, only 16% of Americans with SCT know their status. To increase SCT status awareness, effective education for patients and their families is needed. The objective of this study was to assess caregivers' SCT knowledge before and after an in-person SCT education session. Methods A trained educator provides in-person SCT education to caregivers of referred infants with SCT at Nationwide Children's Hospital. From August 2015 to July 2016, primarily English-speaking caregivers of infants with hemoglobin S-trait were recruited and completed a health literacy assessment and a SCT knowledge assessment ( SCTKA) before and after receiving education. Caregivers repeated the SCTKA again after ≥6 months, if they could be contacted. Results Thirty-eight (38.1%) percent of 113 caregivers had high SCTKA scores (≥75%) before education but 90.3% achieved high scores after education. Caregivers with low SCTKA scores after education had significantly lower health literacy ( P = 0.029) and baseline SCTKA scores ( P = 0.003) compared to those with higher scores after education. At ≥6 months, caregivers' scores were significantly higher ( P = 0.014) than baseline, but only 73.3% scored ≥75%. Conclusion Our results suggest that caregivers' baseline SCT knowledge is low, improves with in-person education but may decline with time. Caregivers who do not achieve high SCT knowledge after education had lower health literacy and baseline knowledge. Future studies should determine if adapting in-person education to caregivers' health literacy and knowledge levels results in high and sustained SCT knowledge among all caregivers and more individuals who know their SCT status. [ABSTRACT FROM AUTHOR]

The article explores the challenges and strategies in recruiting children and families with cancer for behavioral research. Topics mentioned include the treatment-related decisions and emotional adjustment that such patients faced, the barriers that limit access to the patient and family, and the importance for research team to partner with medical team for the design of the study, identification of participants, and in recruitment visits plan.

Study Objective To assess the proportion of iron deficiency that is not detected with a screening hemoglobin or complete blood count (CBC) alone in young women with heavy menstrual bleeding. Design Retrospective review of electronic medical records. Setting Nationwide Children's Hospital in Columbus, Ohio. Participants, Interventions, and Main Outcome Measures One hundred fourteen young women aged 9-19 years consecutively referred to a young women's hematology clinic with a complaint of heavy menstrual bleeding. Results Fifty-eight (50.9%) of all patients had ferritin <20 ng/mL indicating iron deficiency. Of the 58 patients with iron deficiency, only 24 (41.4%) were anemic and 25 (46.3%) were microcytic. The sensitivity of hemoglobin alone and CBC alone for identifying women with ferritin <20 ng/mL was 41.4% (95% confidence interval [CI], 28.7-54.1) and 46.3% (95% CI, 33.0-59.6), respectively. Both tests had reasonable specificity at 91.1% (95% CI, 83.6-98.5) for hemoglobin and 83.9% for CBC (95% CI, 74.3-93.6). Patients had significantly higher odds of having iron deficiency if they were overweight or obese (odds ratio, 2.81; 95% CI, 1.25-6.29) compared with patients with normal body mass index. Age at presentation for heavy menstrual bleeding, presence of an underlying bleeding disorder, and median household income were not significantly associated with iron deficiency. Conclusion In adolescents with heavy menstrual bleeding, fewer than half of iron deficiency cases are detected when screening is performed with hemoglobin or blood count alone. Measuring ferritin levels in at-risk patients might allow for earlier implementation of iron therapy and improvement in symptoms. [ABSTRACT FROM AUTHOR]

Choosing Wisely® is a medical stewardship initiative led by the American Board of Internal Medicine Foundation in collaboration with professional medical societies in the United States. The American Society of Hematology (ASH) released its first Choosing Wisely® list in 2013. Using the same evidence-based methodology as in 2013, ASH has identified 5 additional tests and treatments that should be questioned by clinicians and patients under specific, indicated circumstances. The ASH 2014 Choosing Wisely® recommendations include: (1) do not anticoagulate for more than 3 months in patients experiencing a first venous thromboembolic event in the setting of major, transient risk factors for venous thromboembolism; (2) do not routinely transfuse for chronic anemia or uncomplicated pain crises in patients with sickle cell disease; (3) do not perform baseline or surveillance computed tomography scans in patients with asymptomatic, early-stage chronic lymphocytic leukemia; (4) do not test or treat for heparin-induced thrombocytopenia if the clinical pretest probability of heparin-induced thrombocytopenia is low; and (5) do not treat patients with immune thrombocytopenia unless they are bleeding or have very low platelet counts. [ABSTRACT FROM AUTHOR]

Objectives: Although critically ill children are at increased risk for developing deep venous thrombosis, there are few pediatric studies establishing the prevalence of thrombosis or the efficacy of thromboprophylaxis. We tested the hypothesis that thromboprophylaxis is infrequently used in critically ill children even for those in whom it is indicated.Design: Prospective multinational cross-sectional study over four study dates in 2012.Setting: Fifty-nine PICUs in Australia, Canada, New Zealand, Portugal, Singapore, Spain, and the United States.Patients: All patients less than 18 years old in the PICU during the study dates and times were included in the study, unless the patients were 1) boarding in the unit waiting for a bed outside the PICU or 2) receiving therapeutic anticoagulation.Interventions: None.Measurements and Main Results: Of 2,484 children in the study, 2,159 (86.9%) had greater than or equal to 1 risk factor for thrombosis. Only 308 children (12.4%) were receiving pharmacologic thromboprophylaxis (e.g., aspirin, low-molecular-weight heparin, or unfractionated heparin). Of 430 children indicated to receive pharmacologic thromboprophylaxis based on consensus recommendations, only 149 (34.7%) were receiving it. Mechanical thromboprophylaxis was used in 156 of 655 children (23.8%) 8 years old or older, the youngest age for that device. Using nonlinear mixed effects model, presence of cyanotic congenital heart disease (odds ratio, 7.35; p < 0.001) and spinal cord injury (odds ratio, 8.85; p = 0.008) strongly predicted the use of pharmacologic and mechanical thromboprophylaxis, respectively.Conclusions: Thromboprophylaxis is infrequently used in critically ill children. This is true even for children at high risk of thrombosis where consensus guidelines recommend pharmacologic thromboprophylaxis. [ABSTRACT FROM AUTHOR]

Objectives: Although critically ill children are at increased risk for developing deep venous thrombosis, there are few pediatric studies establishing the prevalence of thrombosis or the efficacy of thromboprophylaxis. We tested the hypothesis that thrombopro-phylaxis is infrequently used in critically ill children even for those in whom it is indicated. Design: Prospective multinational cross-sectional study over four study dates in 2012. Setting: Fifty-nine PICUs in Australia, Canada, New Zealand, Portugal, Singapore, Spain, and the United States. Patients: All patients less than 18 years old in the PICU during the study dates and times were included in the study, unless the patients were 1) boarding in the unit waiting for a bed outside the PICU or 2) receiving therapeutic anticoagulation. Interventions: None. Measurements and Main Results: Of 2,484 children in the study, 2,159 (86.9%) had greater than or equal to 1 risk factor for thrombosis. Only 308 children (12.4%) were receiving pharmacologic thromboprophylaxis (e.g., aspirin, low-molecular-weight heparin, or unfractionated heparin). Of 430 children indicated to receive pharmacologic thromboprophylaxis based on consensus recommendations, only 149 (34.7%) were receiving it. Mechanical thromboprophylaxis was used in 156 of 655 children (23.8%) 8 years old or older, the youngest age for that device. Using nonlinear mixed effects model, presence of cyanotic congenital heart disease (odds ratio, 7.35; p < 0.001) and spinal cord injury (odds ratio, 8.85; p = 0.008) strongly predicted the use of pharmacologic and mechanical thromboprophylaxis, respectively. Conclusions: Thromboprophylaxis is infrequently used in critically ill children. This is true even for children at high risk of thrombosis where consensus guidelines recommend pharmacologic thromboprophylaxis. [ABSTRACT FROM AUTHOR]

Choosing Wisely® is a medical stewardship and quality improvement initiative led by the American Board of Internal Medicine Foundation in collaboration with leading medical societies in the United States. The ASH is an active participant in the Choosing Wisely® project. Using an iterative process and an evidence-based method, ASH has identified 5 tests and treatments that in some circumstances are not well supported by evidence and which in certain cases involve a risk of adverse events and financial costs with low likelihood of benefit. The ASH Choosing Wisely® recommendations focus on avoiding liberal RBC transfusion, avoiding thrombophilia testing in adults in the setting of transient major thrombosis risk factors, avoiding inferior vena cava filter usage except in specified circumstances, avoiding the use of plasma or prothrombin complex concentrate in the nonemergent reversal of vitamin K antagonists, and limiting routine computed tomography surveillance after curativeintent treatment of non-Hodgkin lymphoma. We recommend that clinicians carefully consider anticipated benefits of the identified tests and treatments before performing them. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Although recent evidence-based guidelines for the management of immune thrombocytopenia (ITP) recommend a conservative, observation-based approach for the majority of patients with newly diagnosed pediatric ITP, current practice patterns are unknown. This study used the Pediatric Health Information System database to examine patterns of inpatient care in newly diagnosed ITP in freestanding US children's hospitals and to examine geographic differences in care. METHODS: Data were extracted from Pediatric Health Information System for all newly diagnosed ITP admissions aged 1 to 18 years discharged between January 2008 and December 2010. Clinical data obtained included age, gender, length of stay, diagnoses, medications, and discharge status. RESULTS: We identified 2314 unique patients meeting the study diagnosis of newly diagnosed ITP. Noncutaneous bleeding occurred in 12% of patients (intracranial hemorrhage 0.6%), with epistaxis the most commonly reported symptom. Ninety percent of hospitalized patients received ITP-directed therapy, with intravenous immunoglobulin G the most commonly used therapy (78% of patients). We identified significant variation by geographic region in treatment strategies, length of stay, hospital charges, and likelihood of readmission. CONCLUSIONS: A substantial number of children with newly diagnosed ITP continue to be hospitalized and receive intravenous medications, although the majority of these patients do not have clinical bleeding events during the admission. By using these results as a backdrop, future studies will be able to identify if the number of ITP admissions, costs of care, and geographic variability in care decrease with the dissemination and implementation of recently published guidelines. Pediatrics 2013;131:880-885 [ABSTRACT FROM AUTHOR]

Introduction It is unknown if improvements in supportive care have altered the risk of calcineurin inhibitor (CNI) mediated posterior reversible encephalopathy syndrome (PRES) in patients with hemoglobinopathies such as sickle cell disease (SCD) and β-thalassemia major. We conducted a multicenter retrospective study to determine prevalence of PRES and associated risk factors in a contemporary cohort of patients with hemoglobinopathies transplanted at US children's hospitals. Methods The Pediatric Health Information Systems database, an administrative database containing information from 49 pediatric hospitals, was used to extract data on eligible patients who underwent allogeneic hematopoietic cell transplant (HCT) from 2003 to 2017. Outcomes were compared between SCD and β-thalassemia cohorts and analyzed using non-parametric methods. Results 788 patients (586 SCD, 202 β-thalassemia) were included (Table 1). PRES was seen in 8.1% (n=64) of the cohort with greater prevalence in SCD than β-thalassemia patients (9.7% vs 3.4%; p=0.005). All patients received CNI therapy post-HCT. Hypertension (HTN) pre-HCT, iron overload, use of unrelated donors, fludarabine-based regimen and corticosteroid therapy post-HCT were significant risk factors in SCD cohort only. In both cohorts, increasing age and graft versus host disease (GvHD) were significant risk factors but not neurological deficits pre-HCT, sex, and graft source. One-year-mortality was increased in PRES affected SCD vs unaffected patients (19% vs 5% p=0.0002) but not in β-thalassemia patients with PRES vs without (29% vs 9% p=0.133) Conclusion In our study, prevalence of PRES was higher in SCD than β-thalassemia patients. Studies evaluating CNI- free regimens are needed to abrogate the risk of PRES in this high-risk population. [ABSTRACT FROM AUTHOR]

Simple Summary: Fertility impairment is common among male childhood cancer survivors and negatively impacts quality of life. Sperm banking, before starting cancer treatment, is an established fertility preservation option, yet it remains underutilized at many pediatric centers. Although survivors often report regret about missed banking opportunities, little is known about short-term decisional satisfaction. The aim of this mixed methods study was to examine decisional satisfaction one month after diagnosis by comparing satisfaction among families of adolescents who did or did not attempt to bank. Quantitatively, families reported satisfaction regardless of the banking decision, while qualitatively, families of adolescents who did not attempt to bank reported potential for future regret. Thus, decisional dissatisfaction may not present after one month but could emerge in the future. The findings underscore the importance of longitudinal research to examine satisfaction over time, and why quantitative and qualitative discrepancies exist, as well as psychosocial support across the care continuum as survivors approach their reproductive years. Half of male childhood cancer survivors experience treatment-related fertility impairment, which can lead to distress. Survivors often regret forgoing fertility preservation (FP), and decisional dissatisfaction is associated with a lower quality of life. This mixed methods study examined short-term FP decisional satisfaction among families of male adolescents newly diagnosed with cancer who received an initial fertility consult and completed an FP values clarification tool. One-two months after the FP decision, thirty-nine families completed the Brief Subjective Decision Quality measure. Decisional satisfaction was compared for participants (mothers, fathers, adolescents) who did and did not attempt to bank. Semi-structured interviews included the following question: How do you/your family feel about the banking decision now/in the future? Decisional quality scores were moderate-high (M = 5.74–6.33 out of 7), with no significant differences between non-attempter (n = 15) and attempter (n = 24) families (adolescents: p = 0.83, d = 0.08; mothers: p = 0.18, d = 0.45; fathers: p = 0.32, d = 0.44). Three qualitative themes emerged among non-attempter families: (1) satisfaction with decision (50% of participants), (2) acceptance of decision (60%), and (3) potential for future regret (40%). Satisfaction with decision was the only theme identified in attempter families (93%). Quantitively, short-term decisional satisfaction was high regardless of the banking attempt. However, the qualitative findings suggest that the experiences of families who did not bank may be more nuanced, as several participants discussed a potential for future regret, highlighting the importance of ongoing support. [ABSTRACT FROM AUTHOR]