Assessment and factors affecting quality of life among patients with Wilson's disease.

Wilson's disease is caused by abnormal copper metabolism resulting in deposition in various organs, including the brain, liver, and cornea, thus disrupting organ function. It is characterized by encephalopathy, extrapyramidal symptoms, progressive liver failure, and copper ring deposition in the cornea. Management of this disease should include quality of life maintenance; however, relevant studies on this topic are lacking. This study aimed to assess the factors affecting the quality of life (QoL) of patients with Wilson's disease. A cross-sectional survey using convenience sampling was conducted between July 2020 and March 2021 at the hospital. Data on patient characteristics, 36-item Short-Form General Health Survey, Uniform Wilson Disease Rating Scale, and Hamilton Depression Rating Scale scores were collected. Associations among quality of life depression, anxiety, and Wilson's disease progression were examined using Pearson correlation analysis. Factors affecting the quality of life of patients, including depression, anxiety, liver function, clinical symptoms, diet, liver function, brain magnetic resonance imaging (MRI) findings, disease duration, Barthel Index, and Morse scores were examined using multivariate linear regression analysis. This study included 134 patients with Wilson's disease whose mean age was 29.12 ± 8.59 years. The mean QoL score in the patient group was 71.38 ± 9.55 points and was negatively correlated with anxiety (r = - 0.883, P = 0.000), depression (r = - 0.852 P = 0.000), and clinical symptoms (r = - 0.542, P = 0.000) scores. Anxiety, depression, and clinical symptoms severity are vital factors for the QoL of patients with Wilson's disease. The study provides foundational evidence to design novel interventions, including symptom management, diet, and self-care ability, which can help in improving the quality of life in patients with Wilson's disease and decreasing the burden associated with this disease.

Cost of Total Intravenous Anesthesia Versus Inhalation Anesthesia in Obstructive Sleep Apnea Surgery.

OBJECTIVES: To compare cost and time spent in surgical and postoperative courses in patients with obstructive sleep apnea (OSA) undergoing surgery with either total intravenous anesthesia (TIVA) or inhalational anesthesia. STUDY DESIGN: Retrospective chart review. METHODS: Retrospective review on patients undergoing surgery for OSA under general anesthesia from January 2019 to October 2020. Cost per service was acquired for the day of surgery. RESULTS: A total of 230 patients were included: 95 received TIVA; 135 received inhalation anesthesia. Total cost was significantly higher in the TIVA nasal surgery group by $286 (P = .035). TIVA produced significantly higher pharmacy and operating room costs across all surgeries and OSA severities. These increased costs were offset by significantly lower supply costs in upper airway stimulator (UAS, -$419.50; P = .007) and uvulopalatopharyngoplasty (UPPP, -$115.16; P = .015) patients receiving TIVA. In the TIVA cohort, there was a trend toward lower recovery room costs after UAS (-$111.09; P = .063) and nasal surgery (-$64.45; P = .096) and anesthesia costs after nasal surgery (-$36.67; P = .054). Total recovery time was reduced by 18 minutes (P = .004) for nasal surgery, 25 minutes (P = .043) for UAS, and 27 minutes (P = .147) for UPPP patients receiving TIVA. CONCLUSION: When used in an outpatient setting for patients with OSA, TIVA adds to pharmacy and operating room costs, but this is usually offset by lower supply, anesthesia, and recovery room costs. We found decreased recovery times in the TIVA cohort. TIVA has proven benefits in patient outcomes and can be cost-effective in OSA surgery. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1487-1494, 2022.

A prospective cohort study of condensed low-dose total skin electron beam therapy for mycosis fungoides: Reduction of disease burden and improvement in quality of life.

BACKGROUND: Low-dose total skin electron beam therapy (TSEBT) for mycosis fungoides is popular because of reduced toxicity with effective palliation. We condensed TSEBT, reducing visits by half and overall treatment length by one third. OBJECTIVE: To determine the efficacy and safety of a novel condensed low-dose TSEBT for mycosis fungoides. METHODS: We conducted a cohort study (2014-2018) with a median follow-up of 22.8 months. We delivered 12 Gy per 6 fractions with the modified Stanford technique, 3 fractions per week, with boosts to shadowed sites at risk between treatments, completing in 2 weeks. Primary outcomes included clinical response, duration of and time to response, and toxicity. Secondary outcomes included patient-reported quality of life (pain, pruritus, and Dermatology Life Quality Index) and physician-scored disease burden (body surface area involvement and Modified Skin Weighted Assessment Tool). RESULTS: Of 25 patients, stage IB was most common at the time of TSEBT (36%). The overall response rate was 88%. Most common was a near complete response (36%), and complete response was achieved in 6 (24%) patients. The median duration of response was 17.5 months (3.5-44.2), and the median time to response was 2 months (range, 0.9-4.1). No patients had toxicity of grade 3 or greater. QOL and disease burden showed significant benefit after TSEBT (P < .001). LIMITATIONS: Cohort study with limited sample size. CONCLUSIONS: Condensed, low-dose TSEBT has favorable outcomes and toxicity with logistical convenience.

Awareness of ocular diagnosis, transportation means, and barriers to ophthalmology follow-up in the Philadelphia Telemedicine Glaucoma Detection and Follow-up Study.

Purpose: The purpose of this study was to assess factors affecting follow-up eye care in participants enrolled in the Philadelphia Telemedicine Glaucoma Detection and Follow-up Study, such as awareness of ocular diagnosis, availability of transportation methods, and reasons for missing eye care appointments. Methods: The sample included 172 participants who were randomized to the intervention group and contacted by the social worker. Results: A total of 155 participants completed the assessment form, which was used as an instrument to assess factors affecting adherence to follow-up eye care. The main reasons for missing eye exam appointments were feeling ill (38.1%, n = 59) and forgetting the appointment (34.2%, n = 53). In addition, 45 (29.2%) participants were unaware of or did not comprehend the severity of their ocular diagnosis. Common methods of transportation included public transportation (31.6%, n = 49), driving (29.7%, n = 46), and being driven (27.7%, n = 43) to their appointment. Conclusion: These results suggest that individuals in need of eye care may benefit from additional assistance of a social worker regarding ongoing eye exam appointment reminders and in-depth explanation of their ocular diagnosis.

Impact of a Social Worker in a Glaucoma Eye Care Service: A Prospective Study.

The purpose of this study was to evaluate the effects of a six-month social worker intervention for participants with a glaucoma-related diagnosis in an urban ophthalmic setting (N = 40). The social worker assessed common barriers to eye care, helping participants with transportation, access to low-vision resources, and supportive counseling. The Distress Thermometer, Patient Health Questionnaire-9, and a satisfaction survey were administered to determine the effect of the social worker intervention and participants' overall impressions of the social worker. The most common barrier to eye care was emotional distress (77.5 percent). There was a significant decrease in the number of participants with symptoms of major and moderate depression. Fourteen participants experienced a clinically relevant decrease in depression, 37 participants felt that the social worker's support addressed their issues, and 29 participants thought it helped them in managing their glaucoma. This study provides evidence that a social work intervention may improve care coordination and management of individuals afflicted with vision impairment due to glaucoma and other ocular diseases.

Philadelphia Telemedicine Glaucoma Detection and Follow-up Study: Ocular Findings at Two Health Centers.

Blindness from glaucoma can be prevented by early detection and treatment. Telemedicine improves access to treatment in high-risk populations that face barriers to receiving adequate ophthalmic care. We used a practice-based telemedicine screening model at two health centers. Telemedicine imaging of the fundus and optic nerve, followed by a complete eye exam at the same location for participants with abnormal findings, unreadable images, or ocular hypertension (OHTN), was performed. A total of 182 participants were screened, of whom 108 qualified for a complete eye exam. Of these, 62 (34.1%) had abnormal images, 12 (6.6%) had OHTN, and 34 (18.7%) had unreadable images. Eighty-nine of the 108 (82%) returned for the complete eye exam. Predominantly, participants were African American with glaucoma suspect and cataract. Screening location was an important factor for follow-up. Assessment of the fundus and optic disc using telemedicine resulted in early detection of glaucoma and other ocular pathology.

Efficacy and outcomes of a summer-based pediatric vision screening program.

PURPOSE: To investigate the prevalence of decreased visual acuity and uncorrected refractive error in school-aged children participating in summer programs. METHODS: During the summers of 2014-2016, Wills Eye Hospital collaborated with summer programs in Philadelphia to provide vision screenings for underserved children. Parental consent was obtained prior to vision screening. Fail criteria included children in grades K-1 (ages 5-6) with visual acuity worse than 20/40 in either eye, children in grades 2-6 (ages 7-13) with visual acuity worse than 20/30 in either eye, or children with ≥2 lines of interocular difference. If decreased visual acuity was correctable to ≥20/30 by the onsite optometrist, two pairs of free eyeglasses were provided. Children with other ocular abnormalities were referred to pediatric ophthalmology. RESULTS: Of 1,627 children screened, 360 children (22.1%) did not pass vision screening, and 64 (3.9%) were referred. The prevalence of decreased distance visual was 34.1%. Younger children were more likely to have worse visual acuity than older children (OR = 0.943; P = 0.023; 95% CI, 0.896-0.992). Myopia (73%), astigmatism (56.8%), hyperopia (15.5%), spherical anisometropia (12.5%), and cylindrical anisometropia (11.9%) presented in the 303 children who underwent a manifest refraction. Myopia increased with age (OR = 0.818; P = 0.001; 95% CI, 0.724-0.922), whereas astigmatism decreased (OR = 0.817; P < 0.001; 95% CI, 0.728-0.913) with age. Two pairs of glasses were provided to 301 children. CONCLUSIONS: Partnership with summer programs and other community initiatives to provide vision screenings facilitates access to eye care ultimately aimed at improving social functioning and academic performance.

The Influence of Health Insurance Policy on Radiation Oncology Physician SBRT/SABR Use Practices: A North American Survey.

PURPOSE: European data suggest that 8-fraction stereotactic body radiation therapy (SBRT) regimens may be similar in efficacy with less toxicity than ≤5-fraction SBRT for central lung lesions. However, under current Centers for Medicare and Medicaid Services guidelines, SBRT in the United States (US) is reimbursed for only ≤5 fractions, whereas there are no such restrictions for reimbursement in Canada. We hypothesize that US-specific SBRT reimbursement policies influence the use of ≥5-fraction SBRT in US academic centers in comparison with comparable Canadian centers. METHODS AND MATERIALS: A 15-question electronic survey was distributed to radiation oncologists at National Cancer Institute-designated cancer centers in the US and the 10 highest research-funded cancer centers in Canada. Fisher exact test or exact logistic regression if applicable was used, where P<.05 was considered statistically different from neutral. RESULTS: Of the 143 radiation oncologists from 60 US cancer centers and 6 Canadian cancer centers who completed the survey (17.6% response rate), 125 routinely prescribe SBRT. Fifty percent of US physicians versus 0% of Canadian physicians indicated that there are instances when they would like to prescribe >5-fraction SBRT but prescribe ≤5 fractions because of insurance reimbursement (P=.076 and P=.001, respectively). Seventy percent (P=.006) of US radiation oncologists versus 0% (P=.001) of Canadian radiation oncologists report that SBRT clinical investigation is constrained by the insurance reimbursement. The most common reported deterrent to prescribing >5-fraction SBRT in the US was insurance reimbursement (49.5%). CONCLUSIONS: US radiation oncologists are more likely than those in Canada to report that SBRT clinical investigation and >5-fraction SBRT use may be negatively influenced by health insurance reimbursement; this perception was not held by physicians in Canada. Health care environment may significantly affect radiation therapy decision making and practice patterns.