Objective

Recurrence risk of congenital heart disease (CHD) in families with an affected first-degree relative is increased as compared with the general population. Advances in genetic testing and evidence that preconceptual folic acid supplementation may decrease risk of CHD warrant preventative counseling for at-risk families. Our goal was to document patterns of preconceptual folic acid supplementation and recurrence risk counseling in at-risk families in order to identify opportunities for improved preventative care.

Design

Mothers referred for a fetal echocardiogram were prospectively enrolled. Cases were defined as mothers deemed to be at higher risk of having an affected fetus with CHD given an affected parent or affected previous pregnancy with CHD. Controls were defined as mothers with no prenatal risk factors. Mothers completed a validated questionnaire assessing use of folic acid supplementation and receipt of recurrence risk counseling. Chi-square analyses were performed to analyze questionnaire responses and demographic data.

Results

A total of 314 subjects participated (controls = 216, cases = 98). Cases took preconceptual folic acid supplementation more often than controls (P < .001), but only 55% started preconceptually. Maternal advanced education and counseling (P < .001) were associated with preconceptual supplementation, whereas complexity of CHD in the relative was not. While 70% of cases received some recurrence risk counseling, those with advanced education and complex CHD in the affected relative were more likely to receive counseling. Few at-risk cases interacted with genetic services (19%).

Conclusions

At-risk mothers with lower education are less likely to take preconceptual folic acid supplementation or receive recurrence risk counseling. Health care providers should proactively provide this information to all at-risk patients and develop collaborations with genetic services.

Despite the likely benefits of palliative care (PC) for patients with cirrhosis, physician experiences and perspectives about best practices are variable. We aimed to assess PC experience and gaps in training among transplant hepatology fellows. We conducted a national survey of all transplant hepatology fellows enrolled in accredited fellowship programs during the 2020-2021 academic year. We assessed the frequency of PC provision and comfort with physical and psychological symptom management, psychosocial care, communication skills, advance care planning, and end-of-life care. A total of 45 of 56 (79%) of transplant hepatology fellows responded to the survey; 50% (n = 22) were female. Most trained at centers performing over 100 transplants per year (67%, n = 29) distributed evenly across geographic regions. Most fellows (69%, n = 31) had a PC or hospice care rotation during residency, and 42% (n = 19) of fellows received education in PC during transplant hepatology fellowship. Fellows reported feeling moderately to very comfortable with communication skills such as breaking bad news (93%, n = 41) and leading family meetings (75%, n = 33), but nearly one-third (30%, n = 13) reported feeling not very or not at all comfortable assessing and managing anxiety and depression (30%, n = 13) and spiritual distress (34%, n = 15). Nearly one-quarter (22%, n = 10) had never discussed or documented advance care plans during fellowship. Fellows wished to receive future instruction on the assessment and management of physical symptoms (68%, n = 30) and anxiety and depression (64%, n = 28). Conclusion: Our survey highlights gaps in PC experience and education during transplant hepatology fellowship, lack of comfort in managing psychological distress and advance care planning, and desire to improve skills, particularly in symptom management. Future studies should investigate how to enhance transplant hepatology competencies in these PC domains and whether this impacts clinical care, advance care planning, or patient experience.

Background

Why physicians use surveillance imaging for asymptomatic cancer survivors despite recommendations against this is not known.

Methods

Physicians surveilling head and neck cancer survivors were surveyed to determine relationships among attitudes, beliefs, guideline familiarity, and self-reported surveillance positron-emission-tomography/computed-tomography use.

Results

Among 459 responses, 79% reported using PET/CT on some asymptomatic patients; 39% reported using PET/CT on more than half of patients. Among attitudes/beliefs, perceived value of surveillance imaging (O.R. 3.57, C.I. 2.42-5.27, P = <.0001) was the strongest predictor of high imaging, including beliefs about outcome (improved survival) and psychological benefits (reassurance, better communication). Twenty-four percent of physicians were unfamiliar with guideline recommendations against routine surveillance imaging. Among physicians with high perceived-value scores, those less familiar with guidelines imaged more (O.R. 3.55, C.I. 1.08-11.67, P = .037).

Conclusions

Interventions to decrease routine surveillance PET/CT use for asymptomatic patients must overcome physicians' misperceptions of its value. Education about guidelines may modify the effect of perceived value.

OBJECTIVE: The antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are multiorgan diseases. Patients with AAV report impairment in their health-related quality of life (HRQOL) and have different priorities regarding disease assessment compared with physicians. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group previously received endorsement for a core set of domains in AAV. Two approaches to measure patient-reported outcomes (PRO) were presented at OMERACT 2016. METHODS: A novel 5-step tool was used to facilitate assessment of the instruments by delegates: the OMERACT Filter 2.0 Instrument Selection Algorithm, with a red-amber-green checklist of questions, including (1) good match with domain (face and content validity), (2) feasibility, (3) do numeric scores make sense (construct validity)?, (4) overall ratings of discrimination, and (5) can individual thresholds of meaning be defined? Delegates gave an overall endorsement. Three generic Patient-Reported Outcomes Measurement Information System (PROMIS) instruments (fatigue, physical functioning, and pain interference) and a disease-specific PRO, the AAV-PRO (6 domains related to symptoms and HRQOL), were presented. RESULTS: OMERACT delegates endorsed the use of the PROMIS instruments for fatigue, physical functioning, and pain interference (87.6% overall endorsement) and the disease-specific AAV-PRO instrument (89.4% overall endorsement). CONCLUSION: The OMERACT Vasculitis Working Group gained endorsement by OMERACT for use of the PROMIS and the AAV-PRO in clinical trials of vasculitis. These instruments are complementary to each other. The PROMIS and the AAV-PRO need further work to assess their utility in longitudinal settings, including their ability to discriminate between treatments of varying efficacy in the setting of a randomized controlled trial.