Craniocervical instability in patients with Ehlers-Danlos syndromes: outcomes analysis following occipito-cervical fusion.

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.

Longitudinal analysis of symptoms in the Ehlers-Danlos syndromes.

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.

The power of patient-led global collaboration.

In late 2017, the global nonprofit, The Ehlers-Danlos Society (TEDS), was awarded a "Pipeline to Proposal" Tier A award from the Patient-Centered Outcomes Research Institute (PCORI). The goal of this application was to form a team of patients, researchers, other community support groups and stakeholders who are focused on establishing the Ehlers-Danlos Comorbidity Coalition to address the common health issues associated with this group of high morbidity, high disease burden conditions. The ongoing Coalition that is now funded by individual donors and expanding in its mission and members, is an example of successful collaboration spanning over borders and priorities.

A case-control study of respiratory medication and co-occurring gastrointestinal prescription burden among persons with Ehlers-Danlos syndromes.

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.

Initial description and evaluation of EDS ECHO: An international effort to improve care for people with the Ehlers-Danlos syndromes and hypermobility spectrum disorders.

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.

Use of prescription opioid and other drugs among a cohort of persons with Ehlers-Danlos syndrome: A retrospective study.

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.

Heritable disorders of connective tissue: Description of a data repository and initial cohort characterization.

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.

Association of ibuprofen use with post-tonsillectomy bleeding in older children.

OBJECTIVE: Evaluate post-tonsillectomy outcomes in children discharged with ibuprofen versus those without. METHODS: This was a retrospective review of children who underwent tonsillectomy ±â€¯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.

Sleep study indices and early post-tonsillectomy outcomes.

OBJECTIVES: To investigate the relationships between preoperative sleep study findings of children undergoing adenotonsillectomy anesthesia emergence time, recovery room time, and length of stay. STUDY DESIGN: Retrospective case series with chart review. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: All children aged 1-17 years who had undergone adenotonsillectomy between 2013 and 2016 were included. Apnea-hypopnea index (AHI), central apnea index (CAI), oxygen saturation nadir, and end-tidal carbon dioxide were compared with the in-operating room times, recovery room time, and length of stay. RESULTS: Three hundred and fourteen patients with a mean age of 6.67 (95% CI 6.25-7.09) years were included. Mean AHI was 9.14 (95% CI 7.33-10.95), mean CI was 0.88 (95% CI 0.50-1.26), mean oxygen saturation nadir was 82.9% (95% CI 81.41-84.32), mean end-tidal carbon dioxide was 50.3 (95% CI 49.39-51.15). Mean emergence time was 16 min (95% CI 15:11-17:13 min), recovery room time was 66 min (95% CI 1:00-1:11 h), and length of stay was 25.7 h (95% CI 21:43-30:00 h). When controlled for age, gender and BMI, linear regression showed that children with a higher AHI had a significantly longer operating room and operative times (p < 0.001), emergence time (p < 0.001) and length of stay (p = 0.01). CAI was related to shorter total operating room times (p = 0.03). AHI, oxygen saturation nadir, CAI and end-tidal carbon dioxide were not associated with recovery room time. CONCLUSION: Preoperative sleep study indices are associated with longer in-operating room times and length of stay, and can be useful in planning operating room and hospital flow.

Aligning perceptions of mistreatment among incoming medical trainees.

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.

Perceptions of mistreatment among trainees vary at different stages of clinical training.

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.

Management of non-acute gastrointestinal defects using the over-the-scope clips (OTSCs): a retrospective single-institution experience.

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.

Costs of hepato-pancreato-biliary surgery and readmissions in privately insured US patients.

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.

Use of a Computer Program for Advance Care Planning with African American Participants.

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.

Accuracy of the Emotion Thermometers (ET) screening tool in patients undergoing surgery for upper gastrointestinal malignancies.

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.

Breast Cancer Surgery Decision-Making and African-American Women.

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.

Screening for psychological distress in surgical breast cancer patients.

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.

Overall survival by pattern of recurrence following curative intent surgery for colorectal liver metastasis.

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.

Factors that affect decisions to receive (or not receive) life-sustaining treatment in advance care planning.

This study identifies factors that affect decisions people make regarding whether they want to receive life-sustaining treatment. It is an interpretive-descriptive study based on qualitative data from three focus groups (N = 23), representing a diverse population in central Pennsylvania. Study sites included a suburban senior center serving a primarily White, middle-class population; an urban senior center serving a frail, underserved, African American population; and a breast cancer support group. The most important factors affecting whether participants wished to receive life-sustaining medical treatment were prognosis, expected quality of life, burden to others, burden to oneself in terms of the medical condition and treatment, and effect on mental functioning and independence. Our findings contribute to the knowledge of the complex factors that influence how people make decisions about advance care planning and life-sustaining treatments. This understanding is critical if nurses are to translate the patient's goals, values, and preferences into an actionable medical plan.

Estimates of the excess cost burden of Ehlers-Danlos syndromes: a United States MarketScan® claims database analysis.

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.