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Background: Few studies have examined the weight-control practices that promote weight loss and weight-loss maintenance in the same sample.Purpose: To examine whether the weight control practices associated with weight loss differ from those associated with weight-loss maintenance.Methods: Cross-sectional survey of a random sample of 1165 U.S. adults. The adjusted associations of the use of 36 weight-control practices in the past week with success in weight loss (≥10% lost in the past year) and success in weight-loss maintenance (≥10% lost and maintained for ≥1 year) were examined.Results: Of the 36 practices, only 8 (22%) were associated with both weight loss and weight-loss maintenance. Overall, there was poor agreement (kappa=0.22) between the practices associated with weight loss and/or weight-loss maintenance. For example, those who reported more often following a consistent exercise routine or eating plenty of low-fat sources of protein were 1.97 (95% CI=1.33, 2.94) and 1.76 (95% CI=1.25, 2.50) times more likely, respectively, to report weight-loss maintenance but not weight loss. Alternatively, those who reported more often doing different kinds of exercises or planning meals ahead of time were 2.56 (95% CI=1.44, 4.55) and 1.68 (95% CI=1.03, 2.74) times more likely, respectively, to report weight loss but not weight-loss maintenance.Conclusions: Successful weight loss and weight-loss maintenance may require two different sets of practices. Designing interventions with this premise may inform the design of more effective weight-loss maintenance interventions. [ABSTRACT FROM AUTHOR]

Background: Patients with Ehlers-Danlos syndromes (EDS) often experience high rates of joint subluxations and dislocations, and associated pain that may require surgical interventions. Orthopaedic surgical management is challenging in this population, and patients will often undergo multiple unsuccessful surgeries. Outcomes data specific to patients with EDS are sparse in the orthopaedic surgery literature. We conducted a scoping review to evaluate the evidence and outcomes for orthopaedic surgery specifically for the EDS population., Methods: PubMed MEDLINE, Embase, The Cochrane Library, Cochrane Controlled Register of Trials (CENTRAL), CINHL, and Scopus from their inception to February 28, 2024 for all studies that reported outcomes for orthopaedic surgery in patients with EDS. Two reviewers independently determined study eligibility, rated study quality, and extracted data. Methodology followed the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR). The studies in this scoping review include Level III (retrospective cohort and case control) and Level IV (case series) evidence., Results: The literature search yielded a total of 71 citations published between 1990 and 2023. All were primary studies. 38 were single case studies, 14 were case series, and 19 were retrospective cohort studies. No randomized clinical studies or systematic reviews were identified. Overall, the reported findings for the various anatomical sites and procedures indicated that surgery outcomes were inconsistent. Our review highlights the need for future research to determine whether currently established surgical approaches for various orthopaedic conditions offer long-term clinical benefit in patients with EDS. This is clearly a challenging diagnosis, and more rigorous clinical studies are required to identify optimal treatment approaches., Conclusions: Our review found little evidence-based research to guide optimal surgical treatment in EDS. Established surgical techniques that have been shown to be successful in the wider orthopaedic population should be studied to determine their efficacy in the EDS population., (© 2024. The Author(s).)

Introduction: Patients with Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD) have significant health challenges that are well-documented, however their impact in terms of cost is not known. Our research objective was to examine the cost burden of EDS and HSD in the United States. We focused this analysis on those with commercial insurance plans., Methods: We queried the MarketScan® database for year 2021 for claims that contained an ICD-10 diagnosis code for EDS or hypermobility. Excess costs for patients in the EDS and HSD cohorts were determined by matching each patient to one patient in the database that did not have a claim for EDS or HSD and comparing total costs for the calendar year. We determined whether patients had claims for selected comorbid conditions likely to impact costs during the calendar year., Results: Sample sizes were 5,113 for adult (age ≥ 18) patients with EDS, 4,880 for adult patients with HSD, 1,059 for child (age 5-17) patients with EDS, and 2,427 for child patients with HSD. The mean excess costs were $21,100 for adult EDS patients, $11,600 for adult HSD patients, $17,000 for child EDS patients, and $11,000 for child HSD patients. EDS and HSD cohorts, both adults and children, with any of the comorbidities had greater healthcare costs. The largest difference was found in the EDS cohort with gastrointestinal comorbid conditions, with more than double the costs for adults., Discussion: We found that patients in the MarketScan database, adults and children, who had EDS or HSD had substantially higher associated excess healthcare costs than patients without EDS or HSD when considering age, sex, geographic location, and comorbidities. These disproportionate healthcare costs in this population have health policy and economic implications, including the need for rapid diagnosis, access to treatment, and accelerated research to advance treatments., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Schubart, Schaefer, Knight, Mills and Francomano.)

Introduction: Individuals with Ehlers-Danlos syndromes (EDS) often have complex and multi-faceted symptoms across the lifespan. Pain and the related symptoms of fatigue and sleep disorders are common. The objective of this qualitative study was to understand how participants manage their pain and related symptoms., Methods: The design was a qualitative thematic content analysis. Twenty-eight interviews were conducted to collect data from individuals who were participants in a prior quantitative longitudinal study. A semi-structured interview guide was designed to focus on and understand the trajectory of pain, sleep, fatigue, and general function. The interview continued with questions about coping mechanisms and obstacles to maintaining a sense of well-being., Results: Symptoms reported by participants were widespread and often interwoven. Pain was universal and often resulted in fatigue and disordered sleep which impacted physical function. Most participants reported that their symptoms worsened over time. Participants reported a wide range of effective interventions and most reported developing self-care strategies to adapt to their disabilities/limitations. Solutions included complementary interventions discovered when conventional medicine was unsuccessful. Very few relied on a "system" of health care and instead developed their own strategies to adapt to their disabilities/limitations., Discussion: EDS symptoms are often debilitating, and their progression is unknown. For most participants, symptoms worsened over the time. Even though participants in our study, by experience, were self-reliant, the importance of knowledgeable medical providers to help guide self-care should be emphasized., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Author CF declared that she was an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2024 Schubart, Mills, Francomano and Stuckey-Peyrot.)

Craniocervical instability (CCI) is increasingly recognized in hereditary disorders of connective tissue and in some patients following suboccipital decompression for Chiari malformation (CMI) or low-lying cerebellar tonsils (LLCT). CCI is characterized by severe headache and neck pain, cervical medullary syndrome, lower cranial nerve deficits, myelopathy, and radiological metrics, for which occipital cervical fusion (OCF) has been advocated. We conducted a retrospective analysis of patients with CCI and Ehlers-Danlos syndrome (EDS) to determine whether the surgical outcomes supported the criteria by which patients were selected for OCF. Fifty-three consecutive subjects diagnosed with EDS, who presented with severe head and neck pain, lower cranial nerve deficits, cervical medullary syndrome, myelopathy, and radiologic findings of CCI, underwent open reduction, stabilization, and OCF. Thirty-two of these patients underwent suboccipital decompression for obstruction of cerebral spinal fluid flow. Questionnaire data and clinical findings were abstracted by a research nurse. Follow-up questionnaires were administered at 5-28 months (mean 15.1). The study group demonstrated significant improvement in headache and neck pain (p < 0.001), decreased use of pain medication (p < 0.0001), and improved Karnofsky Performance Status score (p < 0.001). Statistically significant improvement was also demonstrated for nausea, syncope (p < 0.001), speech difficulties, concentration, vertigo, dizziness, numbness, arm weakness, and fatigue (p = 0.001). The mental fatigue score and orthostatic grading score were improved (p < 0.01). There was no difference in pain improvement between patients with CMI/LLCT and those without. This outcomes analysis of patients with disabling CCI in the setting of EDS demonstrated significant benefits of OCF. The results support the reasonableness of the selection criteria for OCF. We advocate for a multi-center, prospective clinical trial of OCF in this population., (© 2023. The Author(s).)

Background: Stem cell transplantation is an emerging therapy for severe cardiomyopathy, proffering stem cell recruitment, anti-apoptosis, and proangiogenic capabilities. Angiogenic cell precursors (ACP-01) are autologous, lineage-specific, cells derived from a multipotent progenitor cell population, with strong potential to effectively engraft, form blood vessels, and support tissue survival and regeneration., Methods: This IRB approved outcome analysis reports upon 74 consecutive patients who failed medical management for severe cardiomyopathy, and were selected to undergo transcatheter intramyocardial or intracoronary implantation of ACP-01. Serious adverse events (SAEs) were reported. Cell analysis was conducted for each treatment. The left ventricular ejection fraction (LVEF) was measured by multi-gated acquisition scan (MUGA) or echocardiogram at 4 months ± 1.9 months and 12 months ± 5.5 months. Patients reported quality of life statements at 6 months (± 5.6 months)., Results: Fifty-four of 74 patients met requirements for inclusion (48 males and five females; age 68.1 ± 11.3 years). The mean treatment cell number of 57 × 10 6 ACP-01 included 7.7 × 10 6 CD34 + and 21 × 10 6 CD31 + cells with 97.6% viability. SAEs included one death (previously unrecognized silent MI), ventricular tachycardia (n = 2) requiring cardioversion, and respiratory infection (n = 2). LVEF in the ischemic subgroup (n = 41) improved by 4.7% ± 9.7 from pre-procedure to the first follow-up (4 months ± 1.9 months) (p < 0.004) and by 7.2% ± 10.9 at final follow-up (n = 25) at average 12 months (p < 0.004). The non-ischemic dilated cardiomyopathy subgroup (n = 8) improved by 7.5% ± 6.0 at the first follow-up (p < 0.017) and by 12.2% ± 6.4 at final follow-up (p < 0.003, n = 6). Overall improvement in LVEF from pre-procedure to post-procedure was significant (Fisher's exact test p < 0.004). LVEF improvement was most marked in the patients with the most severe cardiomyopathy (LVEF < 20%) improving from a mean 14.6% ± 3.4% pre-procedurally to 28.4% ± 8% at final follow-up. Quality of life statements reflected improvement in 33/50 (66%), no change in 14/50 (28%), and worse in 3/50 (6%)., Conclusion: Transcatheter implantation of ACP-01 for cardiomyopathy is safe and improves LVEF in the setting of ischemic and non-ischemic cardiomyopathy. The results warrant further investigation in a prospective, blinded, and controlled clinical study., Trial Registration: IRB from Genetic Alliance #APC01-001, approval date July 25, 2022. Cardiomyopathy is common and associated with high mortality. Stem cell transplantation is an emerging therapy. Angiogenic cell precursors (ACP-01) are lineage-specific endothelial progenitors, with strong potential for migration, engraftment, angiogenesis, and support of tissue survival and regeneration. A retrospective outcomes analysis of 53 patients with ischemic and non-ischemic dilated cardiomyopathy undergoing transcatheter implantation of ACP-01 demonstrated improvements in the left ventricular ejection fraction of 7.2% ± 10.9 (p < 0.004) and 12.2% ± 6.4, respectively, at 12 months (± 5) follow-up. Quality of life statements reflected improvement in 33/50 (66%) patients., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Objective: Joint hypermobility in Ehlers-Danlos Syndromes (EDS) predisposes persons with EDS to frequent subluxations and dislocations, chronic arthralgia, and soft-tissue rheumatism. Epidemiologic trends of rheumatologic conditions among persons with EDS are lacking. Prescription claims databases can reflect underlying disease burdens by using medication claims as disease proxies. We examined the prevalence of prescription claims for commonly prescribed immunomodulator and antiinflammatory (IMD) drugs among persons with EDS compared with their matched control person, and hypothesized peripubertal increases among female persons with EDS., Methods: We compared the percentages of IMD drug prescription claims among 3,484 persons with EDS (ages 5-62 years) against their age-, sex-, state of residence-, and earliest claim date-matched control persons using 10 years (2005-2014) of private prescription claims data and a minimum 2-year enrollment inclusion criterion., Results: Our cohort comprised 70% adults and 74% female persons. At least 1 IMD medication was prescribed to 65.4% of persons with EDS compared with 47.4% of control persons. We observed 1.3 to 4.2 times higher odds (P < 0.0001) for 5 out of 6 IMD drug classes among persons with EDS compared with matched control persons, except for biologic agents (conditional odds ratio 1.3, 95% confidence interval 0.8-2.0). Peripubertal increases were observed for nonsteroidal antiinflammatory drugs, oral, and injectable steroids., Conclusions: To our knowledge, our study is the first to examine the full range of IMD drug prescription claim trends among persons with EDS. We believe our research findings can have notable diagnostic and management implications for EDS patients who present with multiple comorbidities and generally require a more granular assessment of their medical conditions., (© 2021 American College of Rheumatology.)

Our study extends a cross-sectional dataset on the Ehlers-Danlos syndromes (EDS) assembled by the National Institute on Aging (NIA), under a protocol entitled Clinical and Molecular Manifestations of Heritable Disorders of Connective Tissue. We were successful in contacting 171 of the original 252 participants with EDS. Our study cohort included 91 participants who completed at least one of the following surveys: Brief Pain Inventory (BPI), Pittsburgh Sleep Quality Index (PSQI), Multidimensional Fatigue Inventory (MFI-20), and Short Form (SF-36) Health Survey, at both baseline and follow-up. Follow-up surveys occurred a median of 11.6 years after the baseline survey. We used mixed effects linear regression models to examine the change in scores for multiple indices reported by participants. There were small mean changes reflected in our estimates for the EDS population as a whole. There was wide heterogeneity between reported individual experiences, with some participants markedly improved and some dramatically worse. Men had a greater increase in mean pain severity over time than women. This is the first study to report a decade of longitudinal data in EDS., (© 2022 Wiley Periodicals LLC.)

We previously reported increased pain and gastrointestinal (GI) medication prescription claims among persons with Ehlers-Danlos syndromes (EDS) and peripubertal increase in opioid and anti-emetic claims among women with EDS. Herein, we hypothesized a higher proportion of respiratory and co-occurring respiratory and GI medication prescription claims among persons with EDS compared to their matched controls with increases among peripubertal women with EDS. We compared the proportions of respiratory and co-occurring respiratory and GI medication prescription claims among persons with EDS (aged 5-62) against their age-, sex-, state of residence-, and earliest claim date-matched controls using 10 years of private prescription claims data. Prescription claims among persons with EDS versus matched controls were increased for eight medication classes (p < .0001): intranasal/inhaled corticosteroids (ICS) (30.8% vs. 19.0%), oral steroids (30.0% vs. 16.5%), H1-antihistamines (26.2% vs. 12.2%), short-acting beta agonists (22.7% vs. 11.6%), decongestants (21.6% vs. 15.9%), leukotriene modifiers (8.9% vs. 3.6%), ICS/long-acting beta agonists (5.7% vs. 2.9%), muscarinic antagonists (2.5% vs. 0.9%), and co-occurring prescriptions (29% vs. 10%). Our results suggest a critical time window for peripubertal intervention and research and a need to focus on the pathogenesis and clinical evaluation of EDS-specific respiratory and aerodigestive disorders., (© 2021 Wiley Periodicals LLC.)

The Ehlers-Danlos Society Extension for Community Health Care Outcomes (EDS ECHO) is a portfolio of teleconferencing programs developed around the principles and practices of Project ECHO®, aimed at increasing awareness of Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD) among clinicians, enhancing their confidence in the assessment and management of these complex conditions, and generating networks of clinicians across specialties. We assessed the outcomes of the first EDS ECHO program, launched in April 2019, with two hub locations: Indiana University Health, Indianapolis, Indiana, USA, and The Royal Society of Medicine, London, UK. Clinicians were surveyed before and 6 months after their participation. We describe the initial outcomes of the first four EDS ECHO programs, each comprising nine sessions. Participants reported increased levels of knowledge and confidence in providing care (93% and 95%, respectively) and an increase in referral network participation (65%). Additionally, 80% reported that their interest in EDS and HSD increased; 57% reporting a great increase. Also, 59% reported a decrease in feeling overwhelmed by EDS and HSD, and nearly half reported a decrease in their level of frustration because of participating in EDS ECHO. Clinicians participating in EDS ECHO programs know more, have more confidence in their ability to provide care, and feel energized by their participation., (© 2021 Wiley Periodicals LLC.)

Background: The incidence of thyroid cancer in women is increasing at an alarming rate, with greatest risk in the reproductive years. Establishing relationships of hormonally related reproductive factors with thyroid cancer has been difficult. We aimed to elucidate potential risk factors for thyroid cancer in a large cohort of women., Methods: Among 116,228 women in the Nurses' Health Study II followed from 1989 to 2013, 620 cases of thyroid cancer were identified. We examined reproductive and hormone-related factors, including age at menarche, age at menopause, parity, oral contraceptive use, and postmenopausal hormone therapy use. Pregnancy, reproductive years, and months of breastfeeding were used as surrogate markers for exposure to endogenous reproductive hormones. We used multivariable Cox models to calculate relative risks and 95% confidence intervals for the associations between these factors and risk of thyroid cancer., Results: Number of reproductive years of 41 years or more was associated with more than double the risk of thyroid cancer compared with 30 years or fewer (relative risk, 2.20; 95% confidence interval, 1.19-4.06). The other variables analyzed (parity number, months of breastfeeding, age at menarche, menopausal status, and postmenopausal hormone therapy) were not associated with the risk of thyroid cancer. Women who entered menopause at age 45 years or older had a higher risk of thyroid cancer compared with women who entered menopause at a younger age. This result did not reach statistical significance; however, there was a linear trend between later age at menopause and increased risk of thyroid cancer (p trend  = .009)., Conclusions: This study used a unique large, longitudinal dataset to assess thyroid cancer risk factors and potential confounders over an extended time frame. Our key finding suggests increased risk of thyroid cancer may be associated with a variety of indicators of longer reproductive years. The Nurses' Health Study II has provided new insights into the hormonal risks associated with thyroid cancer., (Copyright © 2021 Jacobs Institute of Women's Health. Published by Elsevier Inc. All rights reserved.)

Background: The Ehlers-Danlos syndromes (EDSs) are a group of heritable disorders of connective tissue associated with an increased prevalence of both structural and functional GI conditions., Methods: We used 10 years (2005-2014) of administrative claims data comprised of 4294 people with clinician-diagnosed EDS, aged 5-62 years, and compared their frequency of GI drug prescription claims to their age-, sex-, state of residence-, and earliest claim date-matched controls. We categorized the GI medications into the following groups: acid suppressants, anti-emetics, irritable bowel syndrome drugs, and visceral hypersensitivity (VHS) medications., Key Results: Compared to controls, a significantly higher proportion of persons with EDS had prescription claims for at least one GI drug group, as well as for drugs in each of the four GI drug groups included in our study. By age-group, 25.7% children and 45.1% adults with EDS had prescription claims for at least one GI drug group compared with only 7.4% and 21.0% of controls, respectively (p < 0.0001). By gender, 44.0% of women and 25.3% of men with EDS had prescription claims for at least one class of GI drugs compared with 19.2% and 9.6% of controls, respectively (p < 0.0001)., Conclusions and Key Inferences: Predominant medication burden occurs among women with EDS, beginning peri-pubertally for anti-emetics and VHS drugs. High GI medication burden underscores previous evidence that GI dysmotility is common among persons with EDS., (© 2021 John Wiley & Sons Ltd.)

Objective: To examine differences in outcomes after tonsillectomy in children who received outpatient narcotics prescriptions compared to those who did not., Methods: The MarketScan database was analyzed for claims made for 14 days following tonsillectomy/adenotonsillectomy between 2008 and 2012 for privately insured children 1 to 17 years. Post-op bleeding, dehydration, emergency department (ED) visits, readmissions, and mean total costs for the 14 days after tonsillectomy were compared., Results: Of the 294 795 patients included, 60.9% received a narcotic prescription. Acetaminophen/hydrocodone bitartrate was received by 53.2% of the group receiving narcotic drugs, 42.5% received acetaminophen/codeine phosphate, 3.0% received acetaminophen/oxycodone hydrochloride, and 0.5% received oxycodone hydrochloride alone. Children who had been prescribed narcotics had significantly higher percentages of bleeding complications (2.7% vs 2.5%, P < .001), and ED visits (6.8% vs 6.6%, P < .001) within 14 days, but a lower percentage of readmissions (1.0% vs 1.5%, P < .001). No significant difference was observed between groups for dehydration. There were some age-related differences. The mean total health-care costs for 14 days post-op were the same in each group, except for the 4- to 6-year-olds, where the narcotic group had higher costs (US $7060 vs US $5840, P = .006)., Conclusion: In this large-scale study, we found small but statistically significant differences in outcomes related to use of narcotics. The only outcome that benefitted the narcotics group was a lower readmission rate.

Ehlers-Danlos Syndromes (EDS) are a group of heritable disorders of connective tissue (HDCT) characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Orthostatic intolerance (OI) is highly prevalent in EDS however mechanisms linking OI to EDS remain poorly understood. We hypothesize that impaired blood pressure (BP) and heart rate control is associated with lower arterial stiffness in people with EDS. Orthostatic vital signs and arterial stiffness were assessed in a cohort of 60 people with EDS (49 female, 36 ± 16 years). Arterial elasticity was assessed by central and peripheral pulse wave velocity (PWV). Central PWV was lower in people with EDS compared to reference values in healthy subjects. In participants with EDS, central PWV was correlated to supine systolic BP (r = 0.387, p = 0.002), supine diastolic BP (r = 0.400, p = 0.002), and seated systolic BP (r = 0.399, p = 0.002). There were no significant correlations between PWV and changes in BP or heart rate with standing ( p > 0.05). Between EDS types, there were no differences in supine hemodynamics or PWV measures ( p > 0.05). These data demonstrate that increased arterial elasticity is associated with lower BP in people with EDS which may contribute to orthostatic symptoms and potentially provides a quantitative clinical measure for future genotype-phenotype investigations.

Background: People with the Ehlers-Danlos Syndromes (EDS), a group of heritable disorders of connective tissue, often report experiencing dental procedure pain despite local anesthetic (LA) use. Clinicians have been uncertain how to interpret this apparent LA resistance, as comparison of EDS and non-EDS patient experience is limited to anecdotal evidence and small case series. The primary goal of this hypothesis-generating study was to investigate the recalled adequacy of pain prevention with LA administered during dental procedures in a large cohort of people with and without EDS. A secondary exploratory aim asked people with EDS to recall comparative LA experiences., Methods: We administered an online survey through various social media platforms to people with EDS and their friends without EDS, asking about past dental procedures, LA exposures, and the adequacy of procedure pain prevention. Among EDS respondents who both received LA and recalled the specific LA used, we compared agent-specific pain prevention for lidocaine, procaine, bupivacaine, mepivacaine, and articaine., Results: Among the 980 EDS respondents who had undergone a dental procedure LA, 88% (n = 860) recalled inadequate pain prevention. Among 249 non EDS respondents only 33% (n = 83) recalled inadequate pain prevention (P < 0.001 compared to EDS respondents). The agent with the highest EDS-respondent reported success rate was articaine (30%), followed by bupivacaine (25%), and mepivacaine (22%)., Conclusions: EDS survey respondents reported nearly three times the rate of LA non-response compared to non-EDS respondents, suggesting that LAs were less effective in preventing their pain associated with routine office dental procedures., Competing Interests: DECLARATION OF INTEREST: The authors had no conflicts of interest to declare., (Copyright © 2019 Journal of Dental Anesthesia and Pain Medicine.)

Context: The Ehlers-Danlos Syndromes (EDSs) are a set of rare heritable disorders of connective tissue, characterized by defects in the structure and synthesis of extracellular matrix elements that lead to a myriad of problems including joint hypermobility and skin abnormalities. Because EDS affects multiple organ systems, defining clear boundaries and recognizing overlapping clinical features shared by disease phenotypes is challenging., Objectives: The objective of this study was to seek evidence of phenotypic subgroups of patients with distinctive symptom profiles and describe these resulting subgroups., Methods: Data were extracted from a repository assembled 2001-2013 by the National Institute on Aging Intramural Research Program. Agglomerative hierarchical clustering was used to form distinct subgroups of patients with respect to the domains of pain, physical and mental fatigue, daytime sleepiness, and nighttime sleep. Domains were selected based on literature review, clinician expertise, and guidance from patient advisors., Results: One hundred seventy-five patients met all inclusion criteria. Three subgroups were identified. The Pain Dominant subgroup (39 patients) had the highest mean pain values, but lowest mean values of other symptoms. The High Symptom Burden subgroup (71 patients) had high mean values in all domains. The Mental Fatigue subgroup (65 patients) had a high mean value for mental fatigue and daytime sleepiness, but a lower mean value for pain., Conclusion: The subgroups aligned with clinical observation of the heterogeneous nature of EDS, with overlapping symptoms between subtypes and a wide divergence in degree of symptoms within subtypes. This exploratory study helps characterize the various phenotypes and comorbidities of patients with EDS., (Copyright © 2019 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.)

We describe a data repository on heritable disorders of connective tissue (HDCT) assembled by the National Institutes of Health's National Institute on Aging (NIA) Intramural Research Program between 2001 and 2013. Participants included affected persons with a wide range of heritable connective tissue phenotypes, and unaffected family members. Elements include comprehensive history and physical examination, standardized laboratory data, physiologic measures and imaging, standardized patient-reported outcome measures, and an extensive linked biorepository. The NIA made a commitment to make the repository available to extramural investigators and deposited samples at Coriell Tissue Repository (N = 126) and GenTAC registry (N = 132). The clinical dataset was transferred to Penn State University College of Medicine Clinical and Translational Science Institute in 2016, and data elements inventoried. The consented cohort of 1,009 participants averaged 39 ± 18 years (mean ± SD, range 2-95) at consent; gender distribution is 71% F and 83% self-report Caucasian ethnicity. Diagnostic categories include Ehlers-Danlos syndrome (classical N = 50, hypermobile N = 99, vascular N = 101, rare types and unclassified N = 178), Marfan syndrome (N = 33), Stickler syndrome (N = 60), fibromuscular dysplasia (N = 135), Other HDCT (N = 72). Unaffected family members (N = 218) contributed DNA for the molecular archive only. We aim to develop further discrete data from unstructured elements, analyze multisymptom HDCT manifestations, encourage data use by other researchers and thereby better understand the complexity of these high-morbidity conditions and their multifaceted effects on affected persons., (© 2019 Wiley Periodicals, Inc.)

Against the backdrop of increased opioid prescribing in the United States and the associated high rate of side effects, dependence, and addiction, our study examined how opioids and other medications are being used among persons with Ehlers-Danlos syndrome (EDS). EDS is a set of heritable connective tissue disorders with high symptom burden, including chronic pain. Prescription medication use among persons with EDS was compared to a cohort of matched controls using 10 years of administrative claims data from a large database of privately insured patients (2005-2014). Our dataset included 4,294 persons with EDS, ages 5-62 years old. In both adults and children, we found that the percentage of persons with a prescription drug claim was higher in the EDS cohort for all prescription drug classes examined. Among children, opioid use was double in the EDS cohort compared to the control group (27.5% vs. 13.5%); in adults, it was nearly double in EDS patients (62% vs. 34.1%). Among persons who were prescribed opioids, those with EDS had higher cumulative dosages over a 2-year time period versus controls. Our study aids in understanding opioid and prescription drug use patterns in a vulnerable population with high symptom burden and chronic pain that is often severe., (© 2019 Wiley Periodicals, Inc.)

Purpose: Advance directives (AD) have been heralded as vehicles to promote patient autonomy and have been decried as ineffective. Efforts to improve advance care planning (ACP) and AD documents are wide ranging but have not been prospectively studied., Materials and Methods: In an institutional review board-approved, single-blind, randomized, controlled trial, we compared an interactive, educational ACP decision aid to standard ACP among patients with advanced cancer. We hypothesized that use of the decision aid would increase physician awareness of patients' health care wishes and increase physician adherence to patients' end-of-life wishes compared with standard ACP., Results: A total of 200 patients were randomly assigned to two study arms. We analyzed data from medical records and interviews with physicians and family members for 121 patients who died by August 2016. No differences in physician awareness or adherence were found between the ACP decision aid and standard ACP groups. End-of-life treatment wishes and discussion of wishes were documented for 70% and 64% of the patients, respectively, but only 35% had an actual AD in the medical record. According to family members, end-of-life care was consistent with the patients' stated wishes 94% of the time. Similarly, according to physicians, it was consistent for 98%. However, according to AD documents, delivered care was consistent with desired care in only 65%. Considerably fewer patients than predicted died, and data from physicians were difficult to obtain., Conclusion: ACP type did not influence documentation of patient wishes or end-of-life care received. Future prospective studies must account for challenges in prognostication and point-of-care data collection at the end of life.

Background:: Optimizing recruitment efficiency is an important strategy to address the resource limitations that typically constrain clinical research. Surprisingly, little empiric data exist to guide research teams attempting to recruit a difficult population into similar studies. Our objective was to investigate factors associated with enrollment into an advance care planning interventional trial., Methods:: This study used secondary data of patients with advanced cancer receiving treatment at an academic medical center in central Pennsylvania who were referred to a randomized controlled trial of an advance care planning intervention. Enrolled participants were compared to nonparticipants with regard to age, gender, race, season of recruitment, elapsed time between recruitment stage, distance to study site, and number of recruitment calls., Results:: Of the 1988 patients referred, 200 participants were enrolled yielding a recruitment efficiency of 10%. Two-thirds of all enrolled participants were recruited with 1 or less phone calls, whereas only 5% were enrolled after 3 calls. There were no statistically significant differences in enrollment based on gender ( P = .88) or elapsed time between recruitment contacts ( P = .22). However, nonparticipants were slightly older ( P = .02)., Conclusions:: Our finding that individuals were more likely to enroll within the first 3 phone calls suggests that recruitment efforts should be focused on making initial contacts with potential participants, rather than continuing attempts to those who are unable to be contacted easily. Researchers could optimize their recruitment strategy by periodically performing similar analyses, comparing differences between participants and nonparticipants.

Objective: Evaluate post-tonsillectomy outcomes in children discharged with ibuprofen versus those without., Methods: This was a retrospective review of children who underwent tonsillectomy ± adenoidectomy from 2012 to 2016 at a tertiary care children's hospital. Main outcome measures included bleed rates, ER visits, and nurse phone calls., Results: Seven hundred and seventy-three patients were included; 504 had ibuprofen at discharge (ID) and 269 did not (NID). There were significant differences in mean age, 6.7 years in the ID group years versus 8.6 for the NID group (P < 0.001). Indication for surgery was sleep apnea in 70.5% of ID patients and 44.0% of NID patients (P < 0.001). Post-tonsillectomy bleeds occurred in 8.7% in the ID group and 5.9% of the NID group (P = 0.168). Other outcome measures revealed no significant differences between the two groups. There was no significant difference in the outcome measures between patients with sleep apnea or recurrent tonsillitis. Age was important; 12.1% of children 9-18 years versus 4.8% in children 3.1-6 years (P = 0.006) had post-tonsillectomy bleeding. For children 9-18 years old, 16.7% in the ID group bled versus 7.5% in the NID group (P = 0.039). Logistical regression revealed that age contributed to post-op bleeding, and ibuprofen contributed to number of ER visits., Conclusion: Ibuprofen is associated with significantly elevated post-tonsillectomy bleeding in older children, further research is needed and other analgesics should be considered., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Advance care planning (ACP) has been shown to benefit patients and families, yet little is known about how an ACP event impacts communication and conversation about end-of-life treatment wishes and the content of such conversations between patients and family caregivers., Objective: To characterize post-ACP conversations regarding medical wishes between seriously ill patients and their family caregivers., Participants: Patients with advanced illness and family caregivers., Outcome Measured: Post-ACP conversations., Design: As part of a larger randomized controlled trial, dyads consisting of seriously ill patients and their identified family caregiver engaged in ACP and created an advance directive for the patient. Approximately 4 to 6 weeks later, semistructured interviews were conducted with the family caregivers to elucidate the subsequent communications regarding medical wishes. If the dyad did not have any conversations post-ACP, reasons and barriers were explored., Results: The majority of dyads (131/188, 69.7%) had 2 to 3 conversations lasting 3 to 5 minutes each in the weeks immediately following ACP. These conversations most commonly addressed general patient wishes about quality of life and specific medical treatments. The most common reasons for not having conversations were a general discomfort with the topic (13/57, 22.8%) and previously having discussed medical wishes (16/57, 28.1%)., Conclusion: The ACP events promote conversation regarding quality of life, general wishes at the end of life, and specific medical wishes. Barriers to conversation following ACP were similar to barriers to ACP in general, suggesting that a more intentional focus on addressing these barriers pre- and post-ACP may be necessary to improve communication.

To investigate the impact of medications on outcomes after tonsillectomy, a retrospective review using the MarketScan database was performed. A total of 306,536 privately insured children and adolescents (1 to 17 years old) who underwent tonsillectomy/adenoidectomy were identified from 2008 to 2012. Pharmaceutical claims identified patients who received outpatient prescriptions for ibuprofen, steroids, or topical anesthetics until discharge and for medications for the treatment of attention deficit hyperactivity disorder (ADHD) or montelukast up to 14 days postoperatively. Logistic regression compared prescription claims to outcomes, including postoperative bleeding, dehydration, emergency department visits, and readmissions. Ibuprofen was the only medication associated with increased odds of postoperative bleeding (odds ratio [OR]: 1.45, 95% confidence interval [CI]: 1.07 to 1.95). Patients receiving steroids had lower odds of dehydration (OR: 0.74, 95% CI: 0.65 to 0.84) and emergency department visits (OR: 0.82, 95% CI: 0.76 to 0.88). Odds of dehydration were highest in patients taking ADHD medications (OR: 1.38, 95% CI: 1.15 to 1.66) and topical anesthetics (OR: 1.32, 95% CI: 1.10 to 1.59). Although causality cannot be assumed in observational studies, steroids and ibuprofen should be used judiciously.

Children with autism spectrum disorders (ASDs) face unique challenges when preparing for and undergoing surgery in the perioperative setting. Our goal was to describe this experience via the qualitative evaluation of interviews with parents whose children with ASD had recently undergone surgery in a tertiary medical center. Twelve parents or guardians participated in these interviews. Two independent researchers recorded interviews and analyzed transcripts. The researchers analyzed the interview transcripts using qualitative software to determine the categories of frequent answers to interview questions. Important categories that emerged included behavioral triggers (ie, response to sounds, expression of anxiety and pain), objects used for comfort, communication issues, important people, and advice. We found that children with ASD have specific and unique needs for reassurance and comfort during a perioperative visit. We created a tool, included in this article, to provide a patient-centered framework for interactions with children with ASD in the perioperative environment., (© AORN, Inc, 2018.)

Objective: To evaluate 2 strategies for preparing family members for surrogate decision-making., Design: A 2 × 2 factorial, randomized controlled trial testing whether: (1) comprehensive online advance care planning (ACP) is superior to basic ACP, and (2) having patients engage in ACP together with family members is superior to ACP done by patients alone., Setting: Tertiary care centers in Hershey, Pennsylvania, and Boston, Massachusetts., Participants: Dyads of patients with advanced, severe illness (mean age 64; 46% female; 72% white) and family members who would be their surrogate decision-makers (mean age 56; 75% female; 75% white)., Interventions: Basic ACP: state-approved online advance directive plus brochure. Making Your Wishes Known (MYWK): Comprehensive ACP decision aid including education and values clarification., Measurements: Pre-post changes in family member self-efficacy (100-point scale) and postintervention concordance between patients and family members using clinical vignettes., Results: A total 285 dyads enrolled; 267 patients and 267 family members completed measures. Baseline self-efficacy in both MYWK and basic ACP groups was high (90.2 and 90.1, respectively), and increased postintervention to 92.1 for MYWK ( P = .13) and 93.3 for basic ACP ( P = .004), with no between-group difference. Baseline self-efficacy in alone and together groups was also high (90.2 and 90.1, respectively), and increased to 92.6 for alone ( P = .03) and 92.8 for together ( P = .03), with no between-group difference. Overall adjusted concordance was higher in MYWK compared to basic ACP (85.2% vs 79.7%; P = .032), with no between-group difference., Conclusion: The disconnect between confidence and performance raises questions about how to prepare family members to be surrogate decision-makers.

Objective: To define current practices in management of secondary post-tonsillectomy hemorrhage (PTH) in children by pediatric and general otolaryngologists., Introduction: Bleeding after tonsillectomy is common. Our goal was to describe management methods across the U.S., Methods: Questions regarding perioperative management and treatment in response to three hypothetical cases featuring secondary post-tonsillectomy bleeding were posed via REDCap survey. Comparisons were made for pediatric otolaryngology fellowship training, regions of residency training and current practice, practice type, and number of years in practice., Results: A total of 400 surveys were distributed with 104 responses. Fellowship-trained respondents were more likely to have been in practice for less than ten years (41.5% versus 17.8%) and to practice in an academic setting (67.3% versus 13.6%). They were less likely to prescribe antibiotics after tonsillectomy and more likely to prescribe acetaminophen (98.3% versus 80.4%), ibuprofen (79.3% versus 56.5%), and narcotics (74.1% versus 50.0%) compared to general otolaryngologists. When faced with a post-tonsillectomy patient with visible clot but no active bleeding, pediatric otolaryngologists were less likely to remove the clot (31.6% versus 54.3%) and more likely to proceed to the OR (75.9% versus 56.5%) and admit the patient (87.9% versus 68.9%). Few regional differences were encountered; however, factors influencing the decision to pursue operative intervention varied by region., Conclusion: Pediatric otolaryngologists are more likely to follow American Academy of Otolaryngology guidelines for tonsillectomy perioperative management. They also manage patients with secondary PTH differently than general otolaryngologists. Management trends are similar by region but salient factors considered in the decision-making process vary., (Copyright © 2018. Published by Elsevier B.V.)

Objective: Evaluate the internal consistency and temporal stability of advance directives (ADs) generated by an interactive, online computer program., Methods: 33 participants completed the program at three visits, 2 weeks apart. Agreement rates were calculated for the General Wishes component of the AD. The test-retest method was used to examine the temporal stability of the Specific Wish for Treatment component which contains five clinical scenarios., Results: General Wishes remained stable with 94% selecting the identical response at each visit. For the Specific Wish for Treatment scale , significant variations in test-retest correlations existed (ie, ρ=0.32-0.78, between time points 1 and 2); however within scenario, correlations did not significantly vary between time points. Temporal stability was lower in the Specific Wish for Treatment scale compared with General Wishe s (average ρ=0.59, between time points 1 and 2; and ρ=0.75, between time points 2 and 3)., Conclusions: ADs generated by an online decision aid demonstrate good temporal stability, with highest stability for General Wishes and moderate stability for Specific Wish for Treatment regarding medical treatments in specific clinical scenarios. Internal consistency for wish for treatment across all time points and scenarios was high (Cronbach α>0.90)., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Background: Learner mistreatment has been a long-standing example of unprofessional behavior in medical training. Alignment of perceptions of professional behavior is a critical component of developing a defined organizational culture. Clinical vignettes addressing learner mistreatment can help to achieve this goal. Our aim was to determine whether using clinical vignettes to address learner mistreatment during onboarding can reduce variability in the perceptions of mistreatment., Materials and Methods: External experts in the field of labor and employment relations embedded in the clinical learning environment identified six thematic areas of potential mistreatment. Corresponding clinical case vignettes were developed and presented to incoming trainees during the onboarding process, followed by facilitated discussion. Perceptions of mistreatment before and after discussion were assessed on a Likert scale, with results compared using F-test and t-test., Results: There were 145 participants. Most participants reported previously witnessing or experiencing episodes of mistreatment before matriculation (84%), with the majority reporting multiple events. The most common offenders were faculty (57%), residents/fellows (49%), and nurses (33%). Only 10% of incoming trainees reported a previous incident of mistreatment. Postintervention scores demonstrated decreased variability (P < 0.05) in perceptions of mistreatment in all but one vignette (withholding learning opportunities). Two vignettes demonstrated higher perception of mistreatment after intervention (noneducational tasks and gender or racial discrimination, P < 0.05)., Conclusions: Mistreatment remains a prevalent phenomenon in medical training involving a wide cross-section of healthcare providers. Trainees arrive with discordant definitions of mistreatment. Alignment of individuals' definitions can be achieved through the use of carefully crafted clinical vignettes and facilitated discussion., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Mistreatment of trainees remains a frequently reported phenomenon in medical education. One barrier to creating an educational culture of respect and professionalism may be a lack of alignment in the perceptions of mistreatment among different learners. Through the use of clinical vignettes, our aim was to assess the perceptions of trainees toward themes of potential mistreatment at different stages of training., Methods: Based on observations from external experts embedded in the clinical learning environment, six thematic areas of potential mistreatment were identified: verbal abuse, specialty-choice discrimination, non-educational tasks, withholding/denying learning opportunities, neglect and gender/racial insensitivity. Corresponding clinical vignettes were created and distributed to 1) medical students, 2) incoming interns, 3) residents/fellows. Perceptions of the appropriateness of the interactions depicted in the vignettes were measured on a 5-point Likert scale. Scores were categorized into neutral or appropriate (≤3) or inappropriate (i.e. mistreatment) (>3) and compared using chi-squared tests., Results: Four hundred twenty seven trainees participated (182 students, 120 interns, 125 residents/fellows). Proportions of students perceiving mistreatment differed significantly from those of interns and residents/fellows in domains of verbal abuse, specialty discrimination and gender/racial insensitivity (p < 0.05). In scenarios comparing interns to residents/fellows, no significant differences were noted in perceptions of mistreatment in the domains of non-educational tasks, withholding learning and neglect., Conclusions: Perceptions of mistreatment differ at different developmental stages of medical training. After exposure to the clinical learning environment, perceptions of incoming interns did not differ from those of residents/fellows, implicating clinical rotations as a key period in indoctrinating students into the prevailing culture. More longitudinal studies are needed to confirm or better examine this phenomenon.

Objective: Many patients with chronic illnesses report a desire for increased involvement in medical decision-making. This pilot study aimed to explore how patients with exacerbation-prone disease trajectories such as advanced heart failure or chronic obstructive pulmonary disease experience advance care planning using an online decision aid and to compare whether patients with different types of exacerbation-prone illnesses had varied experiences using the tool., Methods: Pre-intervention questionnaires measured advance care planning knowledge. Post-intervention questionnaires measured: (1) advance care planning knowledge; (2) satisfaction with tool; (3) decisional conflict; and (4) accuracy of the resultant advance directive. Comparisons were made between patients with heart failure and chronic obstructive pulmonary disease., Results: Over 90% of the patients with heart failure (n = 24) or chronic obstructive pulmonary disease (n = 25) reported being "satisfied" or "highly satisfied" with the tool across all satisfaction domains; over 90% of participants rated the resultant advance directive as "very accurate." Participants reported low decisional conflict. Advance care planning knowledge scores rose by 18% (p < 0.001) post-intervention. There were no significant differences between participants with heart failure and chronic obstructive pulmonary disease., Discussion: Patients with advanced heart failure and chronic obstructive pulmonary disease were highly satisfied after using an online advance care planning decision aid and had increased knowledge of advance care planning. This tool can be a useful resource for time-constrained clinicians whose patients wish to engage in advance care planning., (© The Author(s) 2016.)

Introduction: Advanced endoscopic techniques provide novel therapies for complications historically treated with surgical interventions. Over-the-scope clips (OTSCs) have recently been shown to be effective at endoscopic closure of gastrointestinal (GI) defects. We hypothesize that by following classic surgical principles of fistula management, a high rate of long-term success can be achieved with endoscopic closure of non-acute GI tract defects., Methods: A retrospective review of a single-institution prospectively maintained database (2012-2015) of all patients referred for the management of GI leaks or fistulae who underwent attempted closure with the OTSC system (Ovesco, Germany) was performed. Acute perforations were excluded. The primary endpoint was long-term success defined by the absence of radiographic or clinical evidence of leak or fistula during follow-up. Patients were stratified by success or failure of OTSC closure and compared with Fisher's exact and Mann-Whitney U tests., Results: We identified 22 patients with 28 defects (22 fistulae and 6 leaks). Most patients were female (59 %) with a mean age of 54 years (±14), median BMI of 29, and prior bariatric procedure (55 %). Comorbidities included smoking history (68 %) and diabetes (23 %). The majority of defects were solitary (64 %), involved the upper GI tract (82 %), and had been present for >30 days (50 %). Multiple therapeutic interventions were necessary in 46 % of defects. There were no adverse outcomes related to OTSC placement or misfiring. Endoscopic adjuncts were used in 61 % of cases. Overall success rate was 82 % (100 % for leaks and 76 % for fistulae) at a median follow-up of 4.7 months (IQR 2.1-8.4 months). Predictors of success and failure could not be distinguished due to limited sample size., Conclusions: Over-the-scope clips can be safely and effectively used in patients presenting with GI leaks and fistulae. Further research is required to characterize the determinants of long-term success and risk factors for failure.

Background: Surgical costs are influenced by perioperative care, readmissions, and further therapies. We aimed to characterize costs in hepato-pancreato-biliary surgery in the United States., Methods: The MarketScan database (2008-2010) was used to identify privately insured patients undergoing pancreatectomy (n = 2254) or hepatectomy (n = 1702). Costs associated with the index surgery, readmissions, and total short-term costs were assessed from a third party payer perspective using generalized linear regression models., Results: Mean total costs of pancreatectomy and hepatectomy were $107,600 (95% confidence interval [CI], 101,200-114,000) and $81,300 (95% CI, 77,600-85,000), respectively, with corresponding surgical costs of 69.2% and 60.9%. Ninety-day readmission costs were $36,200 (95% CI, 32,000-40,400) and $34,100 (95% CI, 28,100-40,100), respectively. In multivariate analysis, readmissions were associated with an almost two-fold increase in total costs in both pancreatectomy (cost ratio = 1.98; P < 0.001) and hepatectomy (cost ratio = 1.92; P < 0.001)., Conclusions: Hepato-pancreato-biliary surgery is associated with significant economic burden in the privately insured population. Substantial costs are incurred beyond the index surgical admission, with readmissions representing a major source of potentially preventable health care spending. Sustained efforts in defining high-risk populations and decreasing the burden of postoperative complications through a combination of prevention and improved outpatient management offer promising strategies to reduce readmissions and control costs., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: People with cancer face complex medical decisions, including whether to receive life-sustaining treatments at the end of life. It is not unusual for clinicians to make assumptions about patients' wishes based on whether they had previously chosen to pursue curative treatment., Objective: We hypothesized that cancer patients who initially underwent curative intent surgery (CIS) would prefer more aggressive end-of-life treatments compared to patients whose treatment was noncurative intent (non-CIT)., Methods: This study was a retrospective review of data from a large, randomized controlled trial examining the use of an online decision aid for advance care planning, "Making Your Wishes Known" (MYWK), with patients who had advanced cancer. We reviewed patients' medical records to determine which patients underwent CIS versus non-CIT. In the parent trial, conducted at an academic medical center (2007-2012), 200 patients were enrolled with stage IV malignancy or other poor prognosis cancer. Patients' preferences for aggressive treatment were measured in two ways: using patient-selected General Wishes statements generated by the decision aid and patient-selected wishes for specific treatments under various hypothetical clinical scenarios (Specific Wishes)., Results: We evaluated 79 patients. Of these, 48 had undergone initial CIS and 31 had non-CIT. Cancer patients who initially underwent CIS did not prefer more aggressive end-of-life treatments compared to patients whose treatment was non-CIT., Conclusions: Clinicians should avoid assumptions about patients' preferences for life-sustaining treatment based on their prior choices for aggressive treatment.

Objectives: Although ineffective communication is known to influence patient and family satisfaction with care in intensive care unit [ICU] settings, there has been little systematic analysis of the features of the perceived problem from a communication theory perspective. This study was undertaken to understand perceptions of miscommunication and the circumstances in which they present., Research Methodology and Design: Semi-structured interviews were conducted with 22 health care professionals [HCPs] in five adult ICUs at an academic medical centre in the United States., Findings: From qualitative analysis of the transcribed interviews, four themes emerged, each containing multiple subthemes. Person factors are problems that originate within individuals, related to education, cultural background and emotion. Structural factors are associated with boundaries and coordination of institutional roles. Information management problems result from social and psychological processes by which HCPs and family members seek, distribute and understand information. Relationship management problems arise from difficulties in interpersonal interactions., Conclusions: Ineffective communication is not a single problem, but rather several distinct problems that exist at different levels of abstraction and vary in over-time stability. These findings provide a framework for designing interventions to improve the well-being of patients and family members., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Prior research has used focus group methodology to investigate cultural factors impacting the breast cancer experience of women of various ethnicities including African-Americans; however, this work has not specifically addressed treatment decision-making. This study identifies key issues faced by African-American women diagnosed with breast cancer regarding treatment decisions. We used an interpretive-descriptive study design based on qualitative data from three focus groups (n = 14) representing a population of African-American women in central Pennsylvania. Participants were asked to think back to when they were diagnosed with breast cancer and their visit with the breast surgeon. Questions were asked about the actual visit, treatment choices offered, sources of information, and whether the women felt prepared for the surgery and subsequent treatments. The prompts triggered memories and encouraged open discussion. The most important themes identified were fear across the breast cancer disease trajectory, a preference for visual information for understanding the diagnosis and surgical treatment, and support systems relying on family and friends, rather than the formal health-care system. Our results have implications for practice strategies and development of educational interventions that will help breast cancer patients better understand their diagnosis and treatment options, encourage their participation in treatment decision-making, and provide psychosocial support for those at high risk for emotional distress.

Context: Many physicians avoid advance care planning (ACP) discussions because they worry such conversations will lead to psychological distress., Objectives: To investigate whether engaging in ACP using online planning tools adversely affects hope, hopelessness, or anxiety among patients with advanced cancer., Methods: Patients with advanced cancer and an estimated survival of two years or less (Intervention group) and a Control group were recruited at a tertiary care academic medical center (2007-2012) to engage in ACP using an online decision aid ("Making Your Wishes Known"). Pre/post and between-group comparisons were made, including hope (Herth Hope Index), hopelessness (Beck Hopelessness Scale), and anxiety (State Trait Anxiety Inventory). Secondary outcomes included ACP knowledge, self-determination, and satisfaction., Results: A total of 200 individuals completed the study. After engaging in ACP, there was no decline in hope or increase in hopelessness in either the Control or Intervention group. Anxiety was likewise unchanged in the Control group but decreased slightly in the Intervention group. Knowledge of ACP (% correct answers) increased in both the groups, but more so in the Intervention group (13% increase vs. 4%; P<0.01). Self-determination increased slightly in both groups, and satisfaction with the ACP process was greater (P<0.01) in the Intervention than Control group., Conclusion: Engaging in ACP with online planning tools increases knowledge without diminishing hope, increasing hopelessness, or inducing anxiety in patients with advanced cancer. Physicians need not avoid ACP out of concern for adversely affecting patients' psychological well-being., (Copyright © 2015 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.)

Importance: The incidence of thyroid cancer has increased over the past 30 years. Thyroid cancer is less common in blacks than in persons of white descent, and it has been most common in Asians/Pacific Islanders until recently., Objective: To determine whether the incidence of thyroid cancer is increasing at disproportionate rates for different races and ethnicities., Design, Setting, and Participants: Retrospective review. Study participants were individuals with thyroid cancer in the US National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) 13 database from 1992 through 2010. The SEER 13 registry consists of records from Atlanta (Georgia), Connecticut, Detroit (Michigan), Hawaii, Iowa, New Mexico, San Francisco-Oakland (California), Seattle-Puget Sound (Washington), Utah, Los Angeles (California), San Jose-Monterey (California), rural Georgia, and the Alaska Native Tumor Registry., Main Outcomes and Measures: The SEER*Stat Joinpoint Regression Program was used to determine the average annual percentage change in thyroid cancer incidence for different races and ethnicities from 1992 through 2010. Trends in thyroid cancer incidence were compared between groups using comparability testing., Results: During the study period, the average annual percentage change for thyroid cancer was 5.3% (95% CI, 4.8%-5.7%) per year. Stratification of the study population by race revealed that whites experienced the largest increase in age-adjusted thyroid cancer incidence (5.6% per year), followed by blacks (4.8% per year), American Indian/Alaskan natives (3.2% per year), and Asians/Pacific Islanders (2.3% per year). Joinpoint regression comparability testing showed that the increase in disease incidence was not significantly different between whites and blacks (P = .25). However, the increase in incidence for Asians/Pacific Islanders was significantly lower than that for whites and blacks (P < .05). Stratification of the study population by ethnicity revealed that non-Hispanics experienced a larger increase in incidence (5.5% per year) than Hispanics (3.3% per year)., Conclusions and Relevance: The incidence of thyroid cancer continues to increase in all races and ethnicities. No significant difference was observed between the increase in incidence for whites and blacks. However, the increase in incidence for non-Hispanics was significantly larger than that for Hispanics. The increase in incidence of thyroid cancer was greater in whites than in Asians/Pacific Islanders, so whites now have a higher incidence of thyroid cancer than persons of Asian/Pacific Islander descent.

Aim: Compared with standard laparoscopic (SDL) approaches, less is known about the incidence of hernias after single-site laparoscopic (SSL) colorectal surgery. This study hypothesized that SSL colorectal surgery was associated with an increased risk of hernia development., Method: Institutional retrospective chart review (September 2008-June 2013) identified 276 evaluable patients who underwent laparoscopic colorectal procedures. The following data were collected: demographic data, risk factors for the development of a hernia, operative details and postoperative course including the development of a hernia. Patients were stratified by laparoscopic technique to compare the characteristics of those undergoing SDL and SSL. Patients were subsequently stratified by the presence or absence of a hernia to identify associated factors., Results: One hundred and nineteen patients (43.1%) underwent SDL and 157 patients (56.9%) underwent SSL surgery. The development of an incisional hernia was observed in 7.6% (9/119) of SDL patients compared with 17.0% (18/106) of SSL patients (P = 0.03) over a median 18-month follow-up. Similar proportions of patients developed parastomal hernias in both groups [SDL 16.7% (10/60) vs SSL 15.9% (13/80)]. Hernias were diagnosed at a median of 8.1 (SDL) and 6.5 (SSL) months following the index operation and were less likely to be incarcerated in the SSL group [SDL 38.9% (7/18) vs SSL 6.5% (2/31), P = 0.01]., Conclusion: SSL colorectal surgery is associated with an increase in the incidence of incisional hernias but not parastomal hernias. Site of specimen extraction in SSL may contribute to the development of an incisional hernia., (Colorectal Disease © 2014 The Association of Coloproctology of Great Britain and Ireland.)

Acknowledgments: The authors wish to acknowledge the support and assistance of Dr. William Lawrence for his contribution to the M.A.UT model used in the decision aid, Making Your Wishes Known: Planning Your Medical Future (MYWK), Dr. Cheryl Dellasega for her leadership in focus group activities, Charles Sabatino for his review of legal aspects of MYWK, Dr. Robert Pearlman and his collaborative team for use of the advance care planning booklet "Your Life, Your Choices," Megan Whitehead for assistance in grant preparation and project organization, and the Instructional Media Development Center at the University of Wisconsin as well as JPL Integrated Communications for production and programming of MYWK., Background: For various cultural and historical reasons, African Americans are less likely than Caucasians to engage in advance care planning (ACP) for healthcare decisions., Purpose: This pilot study tested whether an interactive computer program could help overcome barriers to effective ACP among African Americans., Methods: African American adults were recruited from traditionally Black churches to complete an interactive computer program on ACP, pre-/post-questionnaires, and a follow-up phone interview., Results: Eighteen adults (mean age =53.2 years, 83% female) completed the program without any problems. Knowledge about ACP significantly increased following the computer intervention (44.9% → 61.3%, p=0.0004), as did individuals' sense of self-determination. Participants were highly satisfied with the ACP process (9.4; 1 = not at all satisfied, 10 = extremely satisfied), and reported that the computer-generated advance directive accurately reflected their wishes (6.4; 1 = not at all accurate, 7 = extremely accurate). Follow-up phone interviews found that >80% of participants reported having shared their advance directives with family members and spokespeople., Conclusion: Preliminary evidence suggests that an interactive computer program can help African Americans engage in effective advance care planning, including creating an accurate advance directive document that will be shared with loved ones., (© 2015 National Medical Association. Published by Elsevier Inc. All rights reserved.)

Distress is common in patients with gastrointestinal cancers. Most conventional scales are too long for routine clinic use. We tested the Emotion Thermometers (ET) tool, a brief visual-analogue scale. There are four emotional upset thermometers: distress, anxiety, depression, and anger. Sixty-nine surgical patients were recruited from an academic hospital clinic in 2012; 64 had complete data for Beck depression inventory and ET. The sample size was modest due to the specialist nature of the sample. We examined sensitivity, specificity, and area under the receiver-operator-curve. A dimensional multi-domain approach to screening for emotional disorders is preferable to using the distress thermometer alone and can be achieved with little extra time burden to clinicians. The ET is a diagnostic tool that is primarily designed for screening to identify cancer patients who would benefit by enhanced psychosocial care.

Background: Following curative intent surgery (CIS) for colorectal liver metastasis (CRLM), patterns of recurrence and subsequent survival outcomes are not widely reported., Methods: An institutional database (January 2002-December 2012) was reviewed to evaluate patterns of recurrence following CIS for CRLM., Results: 163 patients with CRLM underwent successful CIS. Median follow-up and disease-free interval were 33 and 16 months, respectively. 5-year overall survival (OS) was 55%. After initial CIS, 102 (63%) patients recurred: liver-44% (5-year OS 55%), lung-15% (5-year OS 45%), and other/multifocal-41% (5-year OS 24%). OS for isolated liver and lung recurrences were not significantly different. Liver only recurrence was associated with 1-5 mm liver resection margins (P = 0.048). Lung only recurrence was associated with extrahepatic metastasis (at the time of initial CRLM) (P = 0.025). Other/multifocal recurrence was associated with bilobar CRLM (P = 0.026), and extrahepatic metastasis (P = 0.028)., Conclusions: Patterns of recurrence following CIS for CRLM have important implications for OS. 5-year OS was similar between isolated lung and liver recurrences. During CIS, decreased liver resection margin may be associated with increased risk of liver only recurrence. Patients with aggressive primary or metastatic liver disease are at higher risk for pulmonary or other/multifocal recurrence., (© 2014 Wiley Periodicals, Inc.)

Introduction: Following curative intent surgery (CIS) for colorectal liver metastasis (CRLM), repeat CIS for recurrence improves survival. The factors associated with repeat CIS are not widely reported., Methods: An institutional database (January 2002-December 2012) was reviewed to evaluate factors influencing repeat CIS., Results: One hundred sixty-three patients with colorectal liver metastasis (CRLM) underwent successful CIS. Median follow-up and disease-free interval (DFI) was 33 and 16 months, respectively. After initial CIS, 102 patients (63%) recurred. Fifty-three patients (52%) underwent a repeat CIS. After repeat CIS, 33 patients (62%) developed a second recurrence, and in 13 patients (39%), a third CIS was possible. DFI decreased following initial CIS (first CIS vs. second CIS vs. third CIS [20 vs. 15 vs. 8.5 months], p < 0.001). Overall 5-year survival in all patients was 55%; patients who recurred had a 5-year survival of 67% if they underwent repeat CIS vs. 7.8% if they were managed palliatively. Second CIS was less likely with a postoperative complication, other/multifocal recurrence, or DFI <12 months., Conclusion: Despite high recurrence and decreasing DFI, repeat CIS provides a survival benefit. Postoperative complications, DFI, number, and pattern of recurrence influence the decision to pursue repeat CIS.

Background: The American Association of Endocrine Surgeons initiated a fellowship match in 2007. The profile of applicants who successfully match into an endocrine surgery (ES) fellowship has not previously been characterized., Methods: An institutional review board-approved, web-based survey was distributed to recent and current ES fellows., Results: The survey response rate was 62% (56/90). The overall mean age was 33 years (standard deviation ±3), 54% were female, and 37% self-identified as non-white. Only 5% entered their surgical training with the aim of specializing in ES. During residency, respondents were exposed to high volumes of index ES cases. Sixty-two percent had dedicated research time. At the time of fellowship application, the median number of publications was 5 (range, 0 to 25), and 30% of respondents had additional advanced degrees., Conclusion: Entering ES fellows has diverse backgrounds, with strong academic credentials. These data help inform the career mentoring of aspiring ES applicants., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: This pilot study assessed the levels of patient emotional distress and impact on clinic throughput time., Methods: From April through August 2012, 149 breast cancer patients at the Penn State Hershey Breast Center were screened with the emotions thermometer (ET), a patient-rated visual 0-10 scale that measures distress, anxiety, depression, anger, burden, and need for help. Also, patients indicated their most pressing cancer-related concerns. Clinic visit time was computed and compared with a control group., Results: Using a previously validated cut point ≥4 for any thermometer, we found emotional difficulty in the following proportions: distress 22 %, anxiety 28 %, depression 18 %, anger 14 %, burden 16 %, and need for help 10 %; 35 % scored above the cut point on at least 1 thermometer. We found higher levels of distress in all domains associated with younger age at diagnosis. More extensive surgery (bilateral mastectomy vs unilateral mastectomy vs. lumpectomy) was correlated with higher levels of psychosocial distress. Most often cited concerns, experienced by >20 %, included eating/weight, worry about cancer, sleep problems, fatigue, anxiety, and pain. Mean clinic visit time for evaluable patients screened using the ET (n = 109) was 43.9 min (SD 18.6), compared with 42.6 min (SD 16.2) for the control group (n = 50)., Conclusions: Utilizing the ET, more than one-third of women screened met criteria for psychological distress. Younger age at diagnosis and more extensive surgery were risk factors. The ET is a simple validated screening tool that identifies patients in need of further psychological evaluation without impacting clinic throughput time.

Background: Pleural effusion is a potential complication following blunt splenic injury. The incidence, risk factors, and clinical management are not well described in children., Methods: Ten-year retrospective review (January 2000-December 2010) of an institutional pediatric trauma registry identified 318 children with blunt splenic injury., Results: Of 274 evaluable nonoperatively managed pediatric blunt splenic injures, 12 patients (4.4%) developed left-sided pleural effusions. Seven (58%) of 12 patients required left-sided tube thoracostomy for worsening pleural effusion and respiratory insufficiency. Median time from injury to diagnosis of pleural effusion was 1.5days. Median time from diagnosis to tube thoracostomy was 2days. Median length of stay was 4days for those without and 7.5days for those with pleural effusions (p<0.001) and 6 and 8days for those pleural effusions managed medically or with tube thoracostomy (p=0.006), respectively. In multivariate analysis, high-grade splenic injury (IV-V) (OR 16.5, p=0.001) was associated with higher odds of developing a pleural effusion compared to low-grade splenic injury (I-III)., Conclusions: Pleural effusion following pediatric blunt splenic injury has an incidence of 4.4% and is associated with high-grade splenic injuries and longer lengths of stay. While some symptomatic patients may be successfully managed medically, many require tube thoracostomy for progressive respiratory symptoms., (Copyright © 2014 Elsevier Inc. All rights reserved.)