Automated Screening to Enhance Proactive Consultation-Liaison Psychiatry Services in Acute Medicine Units: Evaluation of Service Outcomes.

Objective: Proactive consultation-liaison (C-L) psychiatry aims to meet the mental health needs of medical-surgical populations-many of which go unmet by the conventional C-L model-through systematic screening and integrated care. We implemented an automated screening list to enhance case identification of an existing proactive C-L service and evaluated service metrics along with clinician- and patient-reported outcomes.Methods: Service outcomes were evaluated using historical and contemporary comparison data. Adjusted difference-in-difference analyses were used to determine change in consult characteristics, mean length of stay (LOS), and scores on Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS). Practitioners and nurses were surveyed regarding service satisfaction, perceived safety, and burnout.Results: During the intervention, the consult rate was 3-fold higher than at baseline. Change in time to consultation was equivocal. Overall mean LOS was not reduced, but observed LOS was 1.2 days shorter than expected among non-COVID patients receiving psychiatric consultation (P = not significant). Mean patient-rated hospital satisfaction on HCAHPS was 1 point higher on intervention units during the intervention. Surveys revealed broad satisfaction with this model among practitioners and improved perception of safety among nurses.Conclusions: Proactive C-L psychiatry enhanced by automated screening was associated with improved service utilization and evidence suggestive of LOS reduction among those most likely to receive direct benefit from this model of care. Further, both patient and clinician ratings were improved during the intervention. Proactive C-L psychiatry provides benefits to patients, clinicians, and health systems and may be poised to achieve the Triple Aim in health care.Prim Care Companion CNS Disord 2024;26(2):23m03647. Author affiliations are listed at the end of this article.

Real-life impacts of olipudase alfa: The experience of patients and families taking an enzyme replacement therapy for acid sphingomyelinase deficiency.

BACKGROUND: Acid Sphingomyelinase Deficiency (ASMD) is an ultra-rare autosomal recessive lysosomal storage disorder characterized by intracellular lipid accumulation resulting from reduced function of acid sphingomyelinase. Olipudase alfa, an enzyme replacement therapy, was recently approved in several countries for the treatment of the non-neurologic manifestations of ASMD. Studies demonstrate improvement in organomegaly, pulmonary function and lipid profiles with olipudase alfa, yet little is known about its impact on quality of life (QoL) for patients and caregivers. The purpose of this study is to better understand the real-life impact of ASMD on patients and caregivers and assess how olipudase alfa impacts QoL for pediatric patients and their caregivers. METHODS: Caregivers of pediatric patients (≤ 18 years of age) with a confirmed diagnosis of ASMD that received olipudase alfa for at least 12 months were recruited in early 2022 through national patient organizations to participate in a global online questionnaire followed by semi-structured interviews. Ten caregivers of patients with ASMD who utilized olipudase alfa as an experimental therapy for pediatric patients participated in the study. Quantitative analysis of the results was undertaken, and qualitative data was analyzed using an inductive thematic approach. RESULTS: Ten eligible participants completed questionnaires, and 8 of the 10 went on to participate in structured interviews. Symptom burden of ASMD and impact on symptomatology and quality of life after olipudase alfa use are reported here. Five themes emerged from analysis: (1) ASMD is a systemic disease with a wide array of manifestations that significantly impact QoL; (2) Olipudase alfa was associated with improvements in all non-neurologic manifestations of ASMD; (3) Participants perceived the risk associated with olipudase alfa to be low and the benefits to greatly outweigh any risk or burden; (4) Participants reported an unmet need to treat the neurologic manifestations of the disease despite the benefits of olipudase alfa in the management of non-neurological symptoms; (5) Participants felt all patients with ASMD need access to olipudase alfa based on the life-changing experience they perceived. CONCLUSIONS: These findings highlight the sustained positive impact olipudase alfa had in many domains that are deemed important to patients and families living with ASMD and outline the extensive unmet need for patients and families living with ASMD.

Consensus clinical management guidelines for acid sphingomyelinase deficiency (Niemann-Pick disease types A, B and A/B).

BACKGROUND: Acid Sphingomyelinase Deficiency (ASMD) is a rare autosomal recessive disorder caused by mutations in the SMPD1 gene. This rarity contributes to misdiagnosis, delayed diagnosis and barriers to good care. There are no published national or international consensus guidelines for the diagnosis and management of patients with ASMD. For these reasons, we have developed clinical guidelines that defines standard of care for ASMD patients. METHODS: The information contained in these guidelines was obtained through a systematic literature review and the experiences of the authors in their care of patients with ASMD. We adopted the Appraisal of Guidelines for Research and Evaluation (AGREE II) system as method of choice for the guideline development process. RESULTS: The clinical spectrum of ASMD, although a continuum, varies substantially with subtypes ranging from a fatal infantile neurovisceral disorder to an adult-onset chronic visceral disease. We produced 39 conclusive statements and scored them according to level of evidence, strengths of recommendations and expert opinions. In addition, these guidelines have identified knowledge gaps that must be filled by future research. CONCLUSION: These guidelines can inform care providers, care funders, patients and their carers about best clinical practice and leads to a step change in the quality of care for patients with ASMD with or without enzyme replacement therapy (ERT).

Health insurance literacy and health services access barriers in Niemann-Pick disease: the patient and caregiver voice.

BACKGROUND: Major challenges to health care access include low health insurance literacy, prohibitive costs, and insurance barriers. Niemann-Pick disease (NPD), comprising acid sphingomyelinase deficiency (ASMD) and Niemann-Pick type C (NPC), is a group of rare, autosomal recessive, highly heterogeneous, neurovisceral, life-threatening, relentlessly progressive lysosomal disorders. Patients experience debilitating systemic and neurological symptoms and substantial emotional and financial stress. Currently, these multifaceted disorders are managed symptomatically as there are no approved therapies. Given the considerable disease burden of NPD, timely access to quality health care is paramount for improving outcomes in these life-threatening disorders. Understanding health insurance literacy and access challenges among patients with NPD and their caregivers is a first step to overcoming treatment barriers. RESULTS: Patients from the Niemann-Pick community participated in a health insurance literacy survey and follow-up telephone interviews on perceived access challenges. Of the 79 respondents who completed the survey, 67 participated in interviews. All respondents had stable health insurance coverage. However, 61% of respondents were unaware of Medicaid waivers and did not avail of them. Overall, 50% of respondents with childhood onset NPC selected Medicaid/Medicare and private insurance; 35% utilized Medicaid waivers. Most respondents with ASMD had private insurance only. Although the Niemann-Pick community demonstrated greater health insurance literacy than the general population, knowledge gaps exist in calculating insurance coverage, out-of-pocket maximums, and defining a formulary. The most frequently cited access burden was the process of obtaining medical care and services. Among respondents with ASMD, the greatest access burden was fear of unavailability of or access to medications and treatment. Access challenges adversely impacted patients' mental health and exacerbated physical symptoms. Delays and denials in obtaining essential medication, equipment, and services contributed to disease progression. Caregivers faced burnout and often questioned the utility of their advocacy. CONCLUSIONS: This study identified knowledge gaps in health insurance literacy and challenges to access medication and health care services among individuals impacted by NPD. Patients and caregivers need the knowledge and skills to navigate a complicated health care system, understand their rights to medication and services and, ultimately, benefit from improved outcomes, especially in a post-drug approval era.

Impact and burden of acid sphingomyelinase deficiency from a patient and caregiver perspective.

Acid sphingomyelinase deficiency (ASMD), historically known as Niemann-Pick disease (NPD) types A, A/B, and B, is a rare, progressive, potentially fatal lysosomal storage disease with a spectrum of phenotypes. Little is known about how ASMD symptoms affect the lives of patients and their caregivers. In a cross-sectional qualitative study conducted in the US and UK, and in collaboration with the National Niemann-Pick Disease Foundation (US) and Niemann-Pick UK, we investigated the symptom experience of patients with ASMD types B and A/B and explored how the disease impacts their and their caregivers' lives. The study included 17 adult patients (mean age 38.7 years, 12 female), three caregivers of adults with ASMD, 12 pediatric/adolescent patients with ASMD (mean age 10.5 years, six female), and 12 caregivers of pediatric/adolescent patients with ASMD. The most commonly reported disease manifestations were respiratory (n = 26, 89.7%), abdominal (n = 25, 86.2%), and musculoskeletal symptoms (n = 23, 79.3%); excessive bleeding or bruising (n = 20, 69%); fatigue (n = 20, 69%); gastrointestinal symptoms (n = 18, 62.1%); and headache (n = 15, 51.7%). ASMD was reported to negatively impact patients' physical function (n = 23, 79.3%), self-esteem (n = 18, 62.1%), emotions (n = 16, 55.2%), social function and relationships (n = 16, 55.2%), and personal care (n = 9, 31%). Providing care for individuals with ASMD negatively affected caregivers' emotional well-being (n = 12, 80%), social function (n = 4, 26.7%), relationships (n = 6, 40%), and financial security (n = 7, 46.7%). The physical toll of providing care, the need for lifestyle changes, and the responsibility for making medical decisions added to the burden for caregivers. Alternatively, some caregivers noted that caring for a loved one enhanced their spirituality, providing them with a different outlook on life and a deeper personal resolve. This study showed that ASMD is a substantial burden for patients and caregivers, with long-term physical, emotional, social, and financial impacts. The study confirmed commonly known manifestations of ASMD, especially respiratory problems, but also identified less recognized ones, such as dermatological complications. The data collected and insight gained from this study should enhance clinical care, help evaluate new treatments, and inform health care decision making for patients with ASMD.

Proactive Integration of Mental Health Care in Hospital Medicine: PRIME Medicine.

BACKGROUND: Proactive consultation-liaison (C-L) psychiatry has been shown to reduce hospital length of stay (LOS), increase psychiatric C-L consult rate, and improve hospital staff satisfaction. Nursing attrition has not been studied in relation to proactive C-L. OBJECTIVE: Our primary aim in evaluating the proactive C-L service called Proactive Integration of Mental Health Care in Medicine (PRIME Medicine) is to analyze change in LOS over 10 months using historical and contemporary comparison cohorts. As secondary aims, we assess change in psychiatric consultation rate, time to consultation, and change in nurse attrition. METHODS: PRIME Medicine was implemented on 3 hospital medicine units as a quality-improvement project. Team members systematically screened patients arriving to assigned units for psychiatric comorbidity. Identified patients were reviewed with hospitalist teams and nurses with the goal of early intervention. RESULTS: Including historical and contemporary comparison cohorts, the mean sample age was 62.4 years (n = 8884). Absolute LOS was unchanged, but difference-in-difference analysis trended toward reduced LOS by 0.16 day (P = 0.08). Consultation rate increased from 1.6% (40 consults) to 7.4% (176 consults). Time to consultation was unchanged (4.0-3.8 d). Annual per-unit nursing turnover increased from 4.7 to 5.7 on PRIME units but from 8.5 to 12.0 on comparison units. Nurses citing "population" as the reason for leaving decreased from 2.7 to 1.7 on PRIME units but increased from 1.5 to 4.5 on comparison units. PRIME Medicine led to increased consultation rate, and our unit-wide outcomes provide a conservative estimate of effect. Factors that may have influenced effect size include our cohort's advanced age, considerable emergency department boarding times, increasing proportion of patients discharged to skilled nursing facilities, and concurrent LOS-reduction initiatives on all units. The favorable trends in nursing attrition on PRIME units may be explained in part by our prior finding that PRIME Medicine was associated with enhanced nursing satisfaction. CONCLUSIONS: While PRIME Medicine had no more than a modest effect on LOS, it was associated with a markedly increased psychiatric consult rate and favorable trends in nursing retention. This analysis highlights important factors that should be considered when implementing and determining value metrics for a proactive C-L service.

Human Rabies - Wyoming and Utah, 2015.

In September 2015, a Wyoming woman was admitted to a local hospital with a 5-day history of progressive weakness, ataxia, dysarthria, and dysphagia. Because of respiratory failure, she was transferred to a referral hospital in Utah, where she developed progressive encephalitis. On day 8 of hospitalization, the patient's family told clinicians they recalled that, 1 month before admission, the woman had found a bat on her neck upon waking, but had not sought medical care. The patient's husband subsequently had contacted county invasive species authorities about the incident, but he was not advised to seek health care for evaluation of his wife's risk for rabies. On October 2, CDC confirmed the patient was infected with a rabies virus variant that was enzootic to the silver-haired bat (Lasionycteris noctivagans). The patient died on October 3. Public understanding of rabies risk from bat contact needs to be improved; cooperation among public health and other agencies can aid in referring persons with possible bat exposure for assessment of rabies risk.

Prolonged Exposure Dermatosis: Reporting High Incidence of an Undiagnosed Facial Dermatosis on a Winter Wilderness Expedition.

OBJECTIVE: Previously unclassified inflammatory skin lesions referred to as sun bumps have been observed throughout the year on participants of wilderness trips; however, the underlying cause and diagnosis remain unclear. The purpose of this prospective observational study was to document the incidence, characteristics, and risk factors associated with these skin lesions as they occurred on a winter wilderness expedition. METHODS: For this study, the lesions were defined as pruritic or erythematous skin lesions occurring while in the wilderness. Seventy-four participants in a wilderness ski touring course in Wyoming fully completed a 44-question written survey concerning occurrence and risk factors for these lesions. Weather information and photographs were collected. RESULTS: Twenty-six percent of participants had similar lesions. The lesions were described as edematous pale papules and plaques with erosions and crusts on an erythematous background. The face was involved in 90% of affected persons. Lesions occurred after an average of 8.7 days in the wilderness and resolved 10.6 days later. Skin that was less prone to sunburn was associated with a decreased incidence (odds ratio 0.44). No association could be found between lesion incidence and history of polymorphous light eruption, sun exposure, ambient temperature, affected contacts, sex, or body mass index. CONCLUSIONS: Overall, the lesions were common among study participants but occurred only after prolonged exposure to wilderness conditions. It was not possible to classify the skin condition as an example of any known diagnosis. We propose the name "prolonged exposure dermatosis" for this condition until further studies better define its etiology, prevention, and treatment.