Reducing the Burden of Care: Multidisciplinary Management of Late-Manifested Crouzon Syndrome—A Case Report

The therapy of patients with Crouzon syndrome involves a multidisciplinary team. In most cases, this therapy is extensive, time-consuming, and exhausting for the patient. This case report illustrates a temporally coordinated therapy plan that succeeds in reducing the burden of care. Showing typical extraoral characteristics of Crouzon syndrome, the patient had a frontal and left-sided crossbite, and impaction of the maxillary canines. Multidisciplinary therapy included the extraction of multiple teeth, midface distraction at Le Fort III level, and alignment of the impacted teeth. Before starting, during, and after completion of the treatment, the patient’s oral health-related quality of life was assessed using COHIP-19. The combination of different treatment steps significantly reduced the duration of therapy. The therapy improved not only the patient’s oro- and craniofacial function, but also the patient’s facial appearance in a short treatment period. The patient’s quality of life improved considerably during this time. In the treatment of severe craniofacial anomalies, the highest priority should be given to keeping the burden of care low. All measures should encourage young patients’ appropriate psychosocial development despite extensive therapies, ensuring at the same time medically satisfactory treatment results.

COVID-19 in German Nursing Homes: A Panel Study on Morbidity, Burden of Care, and Social Isolation

Germany was hit by the second wave of the pandemic much harder than by the first wave. The study aims to describe the extent to which nursing homes (NH) are affected by COVID-19. About 8,000 NHs were invited to participate in two waves of an online survey, with a share of 5-10% participating. The share of all deceased NH-residents with COVID-19 is about 50% (04/2020-02/2021). Factors that increase the risk of an outbreak in NH are the spread of the virus in the population, the size of the institution and staff-resident-ratio. The initial lack of protective equipment has decreased during the second wave, but the facilities have to cope with massive additional care needs with reduced staff. NHs have partly banned contacts between residents and relatives. As a conclusion the support of NH in their attempt to fight the impact of this and further pandemic situations requires highest attention.

The Moderating Effects of Personal Resources on Caregiver Burden in Carers of Alzheimer's Patients

Caring for persons with Alzheimer's disease can be an extremely difficult experience. To date, there has been a lack of research into the role of intermediary variables in the relationship between caregiver personality and psychosocial functioning. The growing numbers of dementia patients worldwide mean that more people are involved in their care, making research into this area a pressing concern. Both a caregiver's personality and personal resources play a key role in their capacity to cope with stressful situations. In order to determine how personal resources moderate the relationship between personality and burden of care, a total of 100 caregivers of Alzheimer's patients (78 women and 22 men) were asked to complete a set of questionnaires to assess personality, personal resources (sense of coherence, generalized self-efficacy, and perceived social support), as well as their levels of stress, depression, and commitment to care. Structural equation modeling and latent growth analysis suggest that personal resources explain the mechanisms underlying burden of care and moderate its relationship with personality. Our findings indicate that personal resources are a critical predictor of burden of care. Therefore, caregivers must be provided with appropriate support, taking into account their resources and personality profiles.

Six-year Burden of Care for Nonsyndromic Unilateral Cleft Lip and Palate Patients: A Comparison Between Cleft Centers and Noncleft Centers

Objective To determine differences in burden of care between nonsyndromic patients with unilateral cleft lip and palate undergoing treatment at American Cleft Palate-Craniofacial Association (ACPA)-accredited centers and nonaccredited centers in New York State. Design A retrospective review of the New York Statewide Planning and Research Cooperative System database from January 2001 to December 2014 was performed using ICD-9 and CPT coding. Patients, participants This study included patients with unilateral cleft lip and palate who underwent both lip and palate repairs during their first 6 years of life. Exclusion criteria included orofacial cleft syndromes, follow-up under 6 years, and one-stage combined cleft lip and palate repairs. Results Eighty-eight patients were treated at cleft centers, and 29 patients at nonaccredited centers ( n = 117). Age at primary palatoplasty (13.0 months vs 18.1 months; p = .019), total number of cleft operations (2.3 vs 2.7; p = .012), and total number of primary cleft-specific procedures (2.2 vs 2.5; p = .0049) were significantly lower for patients treated in cleft centers. Age at primary cheiloplasty (4.8 months vs 4.6 months; p = .865), post-cheiloplasty length of stay (1.2 days vs 1.2 days; p = .673), post-palatoplasty length of stay (1.5 days vs 1.9 days; p = .211), average hospital admissions (2.2 vs 2.3; p = 0.161), and total complication rates (34.1% vs 21.1%; p = 0.517) did not differ significantly between cleft centers and noncenters. Conclusions This data demonstrates some significant differences in overall 6 year burden of care for nonsyndromic patients with unilateral cleft lip and palate treated at ACPA-accredited cleft centers versus nonaccredited centers.

Time Spent at Home Among Older Adults with AML Treated with Hypomethylating Agents and Venetoclax

Introduction The prognosis for older adults with acute myeloid leukemia (AML) is poor. Of approximately 12,000 adults age ≥ 60 diagnosed with AML in the U.S. annually, less than 40% survive 1 year from diagnosis. Prior research has shown that adults with AML feel time at home is a critical consideration in treatment selection. However, to date, no study has adequately described the amount of time older adults can expect to spend at home following initiation of AML therapy. In this study, we aimed to (1) quantify this time and (2) assess the impact of venetoclax (VEN) combination therapy on time at home for older adults with AML. Methods We queried records from University of North Carolina Health to identify individuals age ≥ 60 newly diagnosed with AML from 2015 to 2020. First-line AML therapy was identified, and those receiving azacitidine (AZA) and/or VEN were included. Dates of diagnosis, first remission, death, last follow up, and all oncology clinic / emergency department (ED) / inpatient encounters were captured. Patients with incomplete records were excluded. The primary outcome was proportion of days at home (PDH). PDH was calculated for each patient by subtracting the number of care days (days hospitalized or seen in an ED / oncology clinic / infusion center) from the total days of follow up, divided by total days of follow up. Overall survival (OS) was calculated via the Kaplan-Meier method. Covariates including demographics and disease risk (per European Leukemia Net 2017) were captured. PDH was evaluated via summary statistics for the full cohort and stratified by each categorical variable, both for the full follow up period and on a month-by-month basis among survivors. Associations between PDH and covariates were further assessed via linear regression with adjustment for length of follow up, with significance assessed via Wald Chi-square test. Results From 2015-2020, 137 older adults receiving first-line AZA and/or VEN were identified. 24 were excluded due to incomplete records. Among the remaining 113, mean age was 76 (range 60-99), 80.4% were white, and 43.4% were female. Most received AZA+VEN (51.3%) followed by AZA monotherapy (39.0%) and other VEN-containing combinations (9.7%). The majority had adverse-risk AML (61.3%). Baseline covariates including age and ELN risk were similar across therapy groups. Over the full follow up period, mean PDH was 0.58 (95% confidence interval 0.54-0.63) with a median of 0.63. PDH was similar among those with adverse-risk (mean 0.55, CI 0.49-0.61) and intermediate-risk AML (0.64, CI 0.56-0.72). PDH did not differ among therapy groups: AZA+VEN (0.60, CI 0.54-0.66), AZA alone (0.57, CI 0.49-0.66), and other VEN-containing regimens (0.54, CI 0.40-0.69). When adjusted for length of follow up, no covariate had a significant association with PDH (all p > 0.05). When evaluated month-by-month, the proportion of days at home rose over time among survivors. For example, of patients who survived the full month, mean PDH was 0.51 (CI 0.47-54, n = 105) in month 1 following diagnosis and 0.77 (CI 0.72-0.82, n = 57) in month 6. Figure 1 summarizes person-days at home or engaged in care for 12 months following diagnosis (including contributions from those who did not survive the full month). 34.5% of patients achieved composite complete remission, with higher rates among those receiving AZA+VEN (51.7%) than AZA alone (13.6%) (OR 6.8, p = 0.0002). Median OS was 0.64 years (CI 0.32 - 0.91) and was similar across therapy groups. Conclusion Burden of care for older adults with AML treated with first-line AZA or VEN is high. These individuals spend nearly half (cohort mean 42%) of days following diagnosis engaged in oncology care. In randomized studies, the addition of VEN to AZA improves OS and increases remission rates though results in higher rates of neutropenic fever. Although this study may be underpowered to detect small differences, the superior remission rates with AZA+VEN did not translate to more days at home, perhaps due to increased admissions for neutropenic fever and office/infusion visits. Given the importance of time at home to older adults with AML identified in prior research, these data provide new information to support shared decision making regarding treatment options. Future prospective trials should evaluate burden of care, including time at home, as an endpoint. While awaiting these data, larger analyses of the impact of treatment regimen on burden of care would be useful. Figure 1 Figure 1. Disclosures Bryant: Carevive: Consultancy, Research Funding; Jazz Pharmaceuticals: Consultancy, Research Funding; Servier Pharmaceuticals: Honoraria, Speakers Bureau. Coombs: Alexion Pharmaceuticals: Research Funding; Machaon Diagnostics: Research Funding. Foster: Macrogenics: Research Funding; Rafael Pharmaceuticals: Research Funding; Macrogenics: Consultancy; Daiichi Sankyo: Consultancy; Agios: Consultancy; Bellicum Pharmaceuticals: Research Funding.

Immunoglobulin replacement for primary immunodeficiency: Indications for initiating and continuing treatment

Background: Immunoglobulin replacement therapy (IGRT) is the foundation of treatment for the majority of patients with primary immunodeficiency. Clinical history and laboratory evaluation define the patients for whom IGRT is necessary and appropriate. During the 70 years since the first patient was treated, new products have led to the development of several modes of administration that facilitate the individualization of treatment that enables the optimization of care. Objective: The objective was to explain the assessment of candidates for IGRT and approaches to reevaluating recipients of IGRT to decide on the need to continue treatment and to review the approaches to optimize IGRT. Methods: The relevant literature was reviewed in the context of the author's experience supervising > 20,000 IGRT treatments over a 40-year period. Results: Providing the most appropriate form of IGRT for individual patients ameliorates disease and lessens the burden of care for patients with primary immunodeficiency. Conclusion: IGRT is safe and effective when used to treat patients with primary immunodeficiency who meet established and appropriate clinical and laboratory criteria.

Sleep-Related Problems and Associations with Occupational Factors among Home Care Personnel

Recent demographic developments in Europe have increased the demand for home care. Working in other people’s home environment is challenging. Home care personnel’s musculoskeletal disorders are common, and care personnel overall often have sleep disturbances. In this study, associations between occupational physical and psychosocial factors and possible sleep-related problems among home care personnel were explored using a questionnaire. The questionnaire was distributed to 19 workplaces in Stockholm County in 2017–2019, and 665 home care personnel answered. Several factors, including job contentment, physical burden of care, client-related burnout, quantitative demands, and pain, were significantly associated with sleep-related problems. The results highlight the need for implementing measures to improve psychosocial and organizational working conditions in home care service.