Cystic Fibrosis Learning Network Learning Structure for Multicenter Spread of Co-Production and Timely Patient Registry Data Entry Practices

Background: The Cystic Fibrosis Learning Network (CFLN) is a group of 34 programs that work and learn together with shared measures and processes to improve patient outcomes. Interventions are organized into change packages (collected, actionable concepts to share tested, refined ideas across multiple care centers). Since 2016, these change packages have helped advance team level co-production and improve timely data entry (TDE) and quality and use of Cystic Fibrosis Foundation Patient Registry (CFFPR) data. In the context of the COVID-19 pandemic, in-person meetings were curtailed, and team membership changed often. New learning structures to promote peer-to-peer learning were needed to spread and sustain these interventions. The objective was to describe the shared multicenter learning method used to spread practices in two series: co-production (recruitment and onboarding of patient and family partners (PFPs)) and TDE entry into the CFFPR. Method(s): In the design phase of the learning structure, we developed objectives specific to each series. Community content experts refined the curriculum from the established change package concepts. Teams were recruited through an open invitation to all CFLN sites. and met virtually biweekly for 30-minute sessions for 10 to 12 weeks. CFLN content experts used the change packages to coach teams and share their experiences during learning structure huddles. These sessionswere followed by 2-week action periods to review and test change package ideas. Teams shared progress at each meeting in round-robin format. Progress toward smart aims, team experience, and participation were assessed using descriptive surveys before, during, at the end of the series, and 6 months after it closed. Result(s): In initial surveys, teams self-reported awide range of experiences with co-production and TDE into the CFFPR. Participating teamswere from pediatric and adult programs that varied in number of patients and geographic location. Four teams participated in the co-production series to recruit and onboard PFPs within 6 months of completion. In the 6-month follow-up survey, two of the four teams met their goal of recruiting and onboarding a new PFP. The remaining teams reported barriers related to institutional policies that limited training for volunteers. In the TDE series, five teams joined and aimed to improve TDE into the CFFPR within 8 months. All five teams are on target to meet this goal. For both series, action-period surveys revealed completion of tasks assigned (e.g., reviewof change package concepts, testing tools, process maps, barriers, facilitators). Feedback surveys collected during the final sessions of each series indicated that the learning structure helped teams meet expectations, learn something new, and increase confidence in the interventions. Conclusion(s): This learning structure for spreading standard interventions helped teams meet series' aims. The small-group structure allowed teams to learn and adapt coproduction and timely data change package ideas and sustain practices for at least 6 months. In future iterations, this learning structure could be used as a model to spread standard interventions to other programs in the CFLN and the larger care center network.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved

Decreased Utilization of Low-Value Health Care Services during the Covid-19 Pandemic

INTRODUCTION AND OBJECTIVE: Low value health care is defined as care in which the potential to cause harm is greater than benefit. We hypothesize that rationing of health care services during the pandemic decreased the delivery of low value services. METHOD(S): Data was retrieved from the Mass General Brigham Research Patient Data Registry. High value care services were defined by U.S. Preventive Services Task Force guidelines, while low value care services were adapted for claims as described in the literature. Twenty-one services (4 high value and 17 low value) had adequate volume for analysis. Three month periods were considered, consisting of the pandemic period (Q4: 3/2/20 to 6/1/20) and control periods preceding the pandemic (Q1: 12/1/18 to 3/1/19;Q2: 3/2/19 to 6/1/19;and Q3: 12/1/19 to 3/1/20). Ratio measures of services per period were used to account for seasonality and differences in frequency.The 2019 high value (H) care ratio (Y0H = NHQ2/NHQ1) illustrates relative service counts during a typical year and the 2020 ratio (Y1H = NHQ4/NHQ3) represents the change due to the pandemic. Difference in ratios YH=Y1H-Y0H less than zero reflects a reduction in high value services during the pandemic. The same calculation was made for low value (L) procedures;YL=Y1LY0L. The difference between YL and YH is the difference in differences (DID) estimator and illustrates the differential decline in services. YH- YL greater than zero suggests that low value care declined to a greater degree than high value care. Subdivision DID in ratio analyses were performed for cancer and non-cancer care. RESULT(S): Included in this analysis were 3,271,957 patients. Mean age was 51.4 years, 59.1% of patients were female, and 71.7% were non-Hispanic. Of 21 identified services, 18 had a reduction in volume during the pandemic. The YL for PSA testing in men older than 75 was -0.81. The DID in ratios of all care was 0.08 (p<0.01), suggesting a modest decline in low-value care (Figure 1). The reduction was more pronounced for cancer care with a DID in ratios of 3.39 (p<0.01). CONCLUSION(S): We observed a reduction in both low and high value care with a greater reduction in low value services, especially for cancer care. Limitations include use of data from a single health system, limited number of services, and short time periods given the rapid onset of the pandemic.

Risk factors associated with meeting the standard of care and use of telehealth in 2020 among persons with cystic fibrosis in the United States

Background: The COVID-19 pandemic led to a dramatic decrease in clinic visits for essential health care needs for individuals with cystic fibrosis (CF), but the decline in in-person visits was accompanied by a rapid pivot by many CF care centers to provide telehealth services, similar to the U.S. health care system as a whole. In the absence of in-person visits, we hypothesized that individuals with CF who used telehealth services would be more likely to meet standard of care as defined by Cystic Fibrosis Foundation (CFF) guidelines than individuals who did not use or did not have access to telehealth. We also hypothesized that telehealth use or access would be lower in particular demographic groups based on disparities seen in non-CF telehealth studies. Method(s): We used 2019 and 2020 data from the U.S. CFF Patient Registry (CFFPR) to describe patterns of telehealth use and evaluate associations between patient-level characteristics and use of telehealth in 2020 to achieve standard of care. We quantified the extent to which persons with CF received the recommended components of the care model, comparing 2019 and 2020. A risk factor analysis was implemented to identify patient characteristics associated with attaining standard of care and use of any telehealth in 2020 using multivariable logistic regression. Result(s): A total of 28,132 CFFPR participants were included in the study. The proportion of individuals meeting the individual standards of CF care was lower in 2020 than 2019 for every indicator and lower in adults than in children. In 2020, telehealth use was high among CFFPR participants, with 71% of children and 73% of adults reporting one or more telehealth encounter. In adults, demographic, socioeconomic and CF-related disease covariateswere significantly associated with achieving the overall standard of care and use of telehealth. In the pediatric population, Black race, Hispanic ethnicity, and markers of lower socioeconomic status were associated with lower odds of telehealth use. In all analyses, having received the standard of care in 2019 was associated with higher odds of reported telehealth use (odds ratio (OR) 1.28, 95% CI, 1.16-1.41 in children) and achieving the recommended elements of the CF care model in 2020 (OR 2.36, 95% CI, 2.11-2.64 in children;OR 2.61, 95% CI, 2.19-3.12 in adults). Conclusion(s): Fewer individuals with CF met standards of care in the United States in 2020 than in 2019, probably because of pandemic-related effects, although use of telehealth services increased adherence to some standards of care, such as four or more encounters of any type, mental health screening, and annual ancillary consultations, but not four pulmonary function tests or bacterial cultures annually. The analysis suggests that there are disparities in access to telehealth services. Adults or children who were Black or Hispanic or whose self or parents had lower levels of education had lower odds of telehealth use. Overall, CF care centers in the United States have proven remarkably adept in pivoting care models during the pandemic, and some aspects of these models are important to be retained in a post-pandemic era.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved

Characteristics Of Heart Failure Clinical Trials Registered In Clinicaltrials.gov- A Cross Sectional Analysis

Purpose: To perform an analysis of trends and characteristics of heart failure (HF) trials registered in ClinicalTrials.gov (CTG). Design(s): Cross-sectional study Methods: All heart failure clinical trials registered on CTG from September 27, 2007 to March 31, 2022 were identified using Advanced Search feature in ClinicalTrials.gov. Trial characteristics were assessed through relative frequency calculations. Hazard ratio and 95% confidence intervals were determined for characteristics associated with early discontinuation. Result(s): A total of 4272 clinical trials were registered in CTG during this period. There were 68.8% interventional studies and 31.2% were observational studies. Patient registry studies constituted 15.8% of observational studies. Interestingly, registry studies in HF were more frequent compared overall clinical studies (4.8% vs 2.5%). In HF, 32.4% trials were funded by industry, 6.6% trials were by funded the National Institute of Health (NIH) and other U.S Federal agencies (FA) and 61.8% trials by all others which includes individuals, universities, research organizations etc. Observational studies constituted 28% of the industry sponsored trials in HF versus 15% of the total registered clinical trials. Completion rate were 58.9% for NIH and FA funded trials as compared to 50% for industry sponsored and 38.9% for other sponsored. Discontinuation rates were 9.6% for NIH and FA funded trials as compared to 16.8% for industry sponsored. On the other hand unknown status of the trial was higher for other sponsors (19.8%) as compared to NIH and FA (3.2%) and industry sponsors (9.3%). Trend analysis for last 15 years showed an increasing number of clinical trials in HF each year. During the two years of COVID-19 pandemic (April 2020 to March 2022) 15.5% total number of trials registered were in HF even though the number of NIH and FA funded trials decreased by 28.9%. This was much higher than the previous two years (April 2018 to March 2020). Total of 492 trials (11.52%) were withdrawn, suspended or discontinued early. Trials were less likely to be discontinued if funded by sources other than industry and NIH considered together (OR 1.93;X2= 47.50;p<0.01). A detailed analysis of various phases of trials and trends of discontinued trials will also be presented. Conclusion(s): Number of clinical trials in HF are increasing. Registry trials and observational studies are more common in HF. Number of HF trials increased during COVID-19 pandemic. Trials funded by NIH and FA have better completion rate, whereas discontinuation rates were much higher for industry sponsored trials.Copyright © 2022

Support for patients with MBC

Over the last decade we have witnessed rapid advances in the treatment of patients with metastatic breast cancer (MBC) with seminal discoveries in cancer biology, correlative biomarkers and clinical trials leading to multiple new drug approvals. While these milestones have improved survival, the science of survivorship in this population is just beginning. The diagnosis of MBC is life-changing and requires individualized and multidisciplinary support. The NCI defined the areas of epidemiology and surveillance, symptom management, psychosocial research, health-care delivery, and health behaviors as necessary fields to advance the state of the science in advanced cancer survivors. A multifaceted program addressing these domains is needed to assess MBC patients and their unique and ever-changing needs. With input from patients and providers, program components should include: therapeutic clinical trials, multidisciplinary specialty care, individualized patient navigation, peer support, continuing education, and patient reported outcome (PRO) collection to support patients living with MBC. Input for a program for MBC patients can be guided by a multidisciplinary steering committee in which patient advocates are a major voice. Patients can provide insight into what works for them, and what they are facing may be very different from the experience of an early-stage breast cancer patient. Clinical trials designed to advance the current scientific knowledge of breast cancer treatment are essential to patients living longer, more fulfilled lives with MBC. Clinical trials may include systemic therapy, local therapies such as surgery and radiation for MBC patients, side-effect management and quality of life (may put elsewhere). A comprehensive systemic therapy portfolio should include all biological subtypes as well as recommended treatment options (hormonal therapy, targeted therapy, chemotherapy, and immunotherapy). Multidisciplinary care is necessary to diagnose and treat any condition the MBC patient may encounter and is essential in providing quality care. Comorbidities and debilitating side effects arising from cancer treatment are known to be associated with inferior outcomes. MBC patients may experience lack of familiarity of some providers with novel MBC cancer treatment, side effects, and interactions of their cancer treatment with non-cancer conditions and treatment. With the increasing life expectancy of MBC patients, it is important to manage the medical comorbidities in coordination with the MBC patient's cancer treatment. Integrative Medicine helps support the quality of life of patients through providing clinical modalities such as stress management, yoga, meditation, acupuncture, massage and lifestyle counseling. Supportive care helps support cancer related fatigue and sleep challanges, geriatrics and hospice and palliative care for advanced cancer patients. The role of navigation for MBC patients is unique and should be designed to support the patient's many individual needs. Navigation requires assessment of individual knowledge deficit, coordination of care challenges, internal resource utilization, cultural requests, and emotional health. Navigation should also address the patient's financial and disability questions, medication assistance, symptom management, advanced care planning and goals of care discussions. Additional items to be discussed during navigation visits include primary care provider utilization, COVID-19 vaccination, illness and medication questions, and other patient questions as they arise. A comprehensive registry of MBC patient's medical records and histories will assist researchers in designing future therapeutic and quality of life clinical trials. The categories of patient demographics, clinical variables, pathological variables, treatment variables, outcomes of MBC, and PROs will create a robust registry. A comprehensive patient registry can create a rich database which can guide and inspire future innovative research. Peer support through support groups and peer-to-peer matching s pivotal to MBC patients finding and utilizing their patient voice, emotionally supporting each other and learning from other's similar experiences. Connection between patients and the creation of a community of survivors can empower patients to positively impact their care through self-advocacy and self-efficacy. Continuing patient education is also essential to providing quality cancer care. The format of a weekly virtual education webinars are helpful in creating an engaged patient community and a platform to disseminate educational resources in a reoccurring digestible format. Frequent educational webinars covering a wide variety of topics can positively influence patient interactions with their healthcare providers and influence how patients living with MBC view their own cancer experience. Educational webinars provide opportunities for patients to connect with subject matter experts, other patients like themselves, and share information with their family and friends. Informed patients can discuss and ask questions more confidently with their health care providers about information and services presented during the educational webinars. The symptom profile of patients living with MBC are impacted by numerous variables such as disease burden, treatment plan, comorbidities, supportive regimen etc. The collection of PROs has been shown to improve patient satisfaction with his/her care, improve quality of life, decrease emergency room visits and hospitalizations, and increased overall survival. The routine measurement and management of MBC patients' symptoms has been found to be integral in providing comprehensive cancer treatment. The collection of PROs improves patient and provider communication and elicits the outcome to symptoms that matter most to each patient. Patients diagnosed with MBC are living longer because of the recent advancements in therapeutic treatments. A multifaceted and comprehensive program consisting of therapeutic clinical trials, multidisciplinary specialty care, individualized patient navigation, peer support, continuing education, and PROs collection is integral to fully support patients living with MBC.

COVID-19 in rheumatoid arthritis patients receiving baricitinib: Observations from patient registry

Background/Purpose: Rheumatoid arthritis (RA) patients have higher COVID-19 risks [1,2]. Data suggest that some RA biologics, including baricitinib, may be beneficial for COVID-19 outcomes [3,4]. We used data from RA registry to evaluate impact of COVID-19 on RA activity in patients receiving baricitinib. Method(s): Current study is a single center registry of RA patients receiving baricitinib as a part of routine treatment. Study center accumulates most of RA patients who started baricitinib in Moscow (Russia) from July 2020 to data cutoff (January 2022). We analyzed medical records data for demographics, disease history, and change of disease activity indexes. Medical record data were allocated to visit 1 (baseline), closest to 4 and 8 months after baricitinib initiation (visits 2 and 3). Patients, who had no baricitinib interruptions, were divided in strata according to COVID status between visits 1 and 2. Result(s): At the time of data cutoff registry included data from 142 RA patients receiving baricitinib. Median duration of treatment was 14.5 (interquartile range [IQR] 10-29) weeks. Clinical RA indexes measures are compiled in Table 1. Of 142 patients, 52 had COVID-19 between visits 1 and 2 without baricitinib interruption. Swollen joint counts (SJCs) and tender joint counts (TJCs) were comparable across 3 visits except TJC at visit 3 (P < 0.05). Disease Activity Score-28 for Rheumatoid Arthritis with C-Reactive Protein (DAS28-CRP), Disease Activity Score-28 for Rheumatoid Arthritis with Erythrocyte Sedimentation Rate (DAS28-ESR) had comparable change regardless of COVID-19 status (P > 0.05). Simplified Disease Activity Index for Rheumatoid Arthritis (SDAI) and Clinical Disease Activity Index (CDAI) were higher in COVID-19 survivors at visit 3 (P < 0.05). (Table Presented) Conclusion(s): We conclude that, overall, COVID-19 had no significant impact on RA activity during baricitinib treatment. Further follow-up needed to find out reasons for TJC/SDAI/CDAI increase in COVID-19 survivors >=4 months after infection.

Impact of the COVID-19 pandemic on patients with myasthenia gravis: A survey of the Myasthenia Gravis Foundation of America MG patient registry.

INTRODUCTION/AIMS: Factors associated with coronavirus disease 2019 (COVID-19) infection among the myasthenia gravis (MG) population are incompletely understood. This study aimed to characterize the behavior of MG patients during the pandemic and to examine risk factors associated with COVID-19 infection. METHODS: A "COVID-19 Survey" was sent to MG Patient Registry participants in the summer of 2020 (CSS20) and winter of 2021 (CWS21). Survey results were summarized descriptively. Demographics, disease characteristics, medication use, and survey results were compared between those reporting COVID-19 diagnosis (COVID), COVID-19 like symptoms without diagnosis (COVID-Like), and asymptomatic participants. RESULTS: A total of 454 and 665 participants completed the CSS20 and CWS21 surveys respectively; 326 participants completed both. Most continued follow-up visits and MG treatments. The frequency of COVID-like symptoms was similar between CSS20 and CWS21, while COVID-19 exposure (6% vs. 27%), COVID-19 testing among symptomatic individuals (35% vs. 78%), and COVID-19 diagnosis (0.2% vs. 6%) were higher in the CWS21. Cough, fever, fatigue, myalgia, anosmia/ageusia, and hospital and intensive care unit (ICU) admissions were more frequent in the COVID compared to the COVID-Like group. COVID-19 exposure (odds ratio [OR] 7.88), number of people in the household (OR 1.31), and report of MG exacerbation before the pandemic (OR 2.6) were independently associated with COVID-19 infection. DISCUSSION: COVID-19 affected MG patients increasingly through the early pandemic. While face-to-face contact with a COVID-19 infected individual was an obvious risk factor, MG patients who had more people in the household and unstable disease were at elevated risk for COVID-19 infection.

Clinical Features of the UK Myotonic Dystrophy Patient Registry

The UK Myotonic Dystrophy Patient Registry is a patient self-enrolling online database collecting clinical and genetic information about myotonic dystrophy type 1 (DM1) and type 2 (DM2). The registry was established in May 2012 with support from Muscular Dystrophy UK and the Myotonic Dystrophy Support Group and is coordinated Newcastle University. The registry aims to facilitate academic and clinical research, better characterise and understand DM, and disseminate information relating to upcoming studies and research advancements. The registry is used to capture longitudinal, selfreported data through an online portal available to patients and clinicians. Where specialised clinical or genetic information is required, the neuromuscular specialist involved in the patient's care can be invited to provide some additional information and the patient can select them from a pre-populated list at the registration stage. The registry is a Core Member of the TREAT-NMD Global Registries Network for DM1. Between May 2012 and January 2022, there were 834 patient registrations. On average there are 5 new registrations per month. For those reporting a clinical diagnosis, 96% have DM1 (of which 14% have a diagnosis of congenital DM) and 4% have DM2. Overall, 40% of patients have had genetic confirmation of their condition provided. The registry has previously supported almost 30 research enquiries to date. Since 2020, the registry has facilitated 11 enquiries including an industry enquiry, three COVID-19 surveys, and various surveys capturing information on dysphagia, pregnancy, patient preferences for future treatments and the patient/ caregiver experience. The registry continues to be a versatile, cost-effective research tool, helping facilitate and advance a range of DM research. Additional work continues to be done to improve reporting of genetic information on the registry and there are future data linkage plans between the registry and the Newcastle Research Biobank for Rare and Neuromuscular Diseases.

Clinical Features of the UK FSHD Patient Registry

The UK Facioscapulohumeral Muscular Dystrophy (FSHD) Patient Registry is a patient self-enrolling online database collecting clinical and genetic information about FSHD type 1 (FSHD1) and type 2 (FSHD2). The registry was established in May 2013 with support from Muscular Dystrophy UK and is coordinated by Newcastle University. The registry aims to facilitate academic and clinical research, better characterise and understand FSHD, and disseminate information relating to upcoming studies and research advancements. The registry is used to capture longitudinal, selfreported data through an online portal available to patients and clinicians. Where specialised clinical or genetic information is required, the neuromuscular specialist involved in the patient's care can be invited to provide some additional information and the patient can select them from a pre-populated list at the registration stage. The registry is a Core Member of the TREAT-NMD Global Registries Network for FSHD. Between May 2013 and January 2022, there were 1,074 patient registrations, with 84% based in the UK. On average, there are 9 new registrations per month. For those reporting a clinical diagnosis, 97% have FSHD or FSHD1, and 3% have FSHD2. Overall, 46% of patients have had genetic confirmation of FSHD1 provided. The registry has previously supported almost 30 registry enquiries to date. Since 2020, the registry has facilitated 12 enquiries including, three COVID-19 surveys, and various surveys capturing information on dysphagia, pregnancy, sleep and the patient/caregiver experience. The registry is currently one of the largest national FSHD patient registries and is an example of a versatile, cost-effective research tool, helping facilitate and advance a wide range of FSHD research. Additional work continues to be done to improve reporting of genetic information on the registry and there are future data linkage plans between the registry and the Newcastle Research Biobank for Rare and Neuromuscular Diseases.

CHARACTERISTICS ASSOCIATED with POOR COVID-19 OUTCOMES in PEOPLE with PSORIASIS and SPONDYLOARTHRITIS: DATA from the COVID-19 PSOPROTECT and GLOBAL RHEUMATOLOGY ALLIANCE PHYSICIAN-REPORTED REGISTRIES

Background: Some factors associated with severe COVID-19 outcomes have been identifed in patients with psoriasis (PsO) and infammatory/autoimmune rheumatic diseases, namely older age, male sex, comorbidity burden, higher disease activity, and certain medications such as rituximab. However, information about specifcities of patients with PsO, psoriatic arthritis (PsA) and axial spondyloarthritis (axSpA), including disease modifying anti-rheumatic drugs (DMARDs) specifcally licensed for these conditions, such as IL-17 inhibitors (IL-17i), IL-23/IL-12 + 23 inhibitors (IL-23/IL-12 + 23i), and apremilast, is lacking. Objectives: To determine characteristics associated with severe COVID-19 outcomes in people with PsO, PsA and axSpA. Methods: This study was a pooled analysis of data from two physician-reported registries: the Psoriasis Patient Registry for Outcomes, Therapy and Epidemiology of COVID-19 Infection (PsoProtect), comprising patients with PsO/PsA, and the COVID-19 Global Rheumatology Alliance (GRA) registry, comprising patients with PsA/axSpA. Data from the beginning of the pandemic up to 25 October, 2021 were included. An ordinal severity outcome was defned as: 1) not hospitalised, 2) hospitalised without death, and 3) death. A multivariable ordinal logistic regression model was constructed to assess the relationship between COVID-19 severity and demographic characteristics (age, sex, time period of infection), comorbidities (hypertension, other cardiovascular disease [CVD], chronic obstructive lung disease [COPD], asthma, other chronic lung disease, chronic kidney disease, cancer, smoking, obesity, diabetes mellitus [DM]), rheumatic/skin disease (PsO, PsA, axSpA), physician-reported disease activity, and medication exposure (methotrexate, lefunomide, sulfasalazine, TNFi, IL17i, IL-23/IL-12 + 23i, Janus kinase inhibitors (JAKi), apremilast, glucocorticoids [GC] and NSAIDs). Age-adjustment was performed employing four-knot restricted cubic splines. Country-adjustment was performed using random effects. Results: A total of 5008 individuals with PsO (n=921), PsA (n=2263) and axSpA (n=1824) were included. Mean age was 50 years (SD 13.5) and 51.8% were male. Hospitalisation (without death) was observed in 14.6% of cases and 1.8% died. In the multivariable model, the following variables were associated with severe COVID-19 outcomes: older age (Figure 1), male sex (OR 1.53, 95%CI 1.29-1.82), CVD (hypertension alone: 1.26, 1.02-1.56;other CVD alone: 1.89, 1.22-2.94;vs no hypertension and no other CVD), COPD or asthma (1.75, 1.32-2.32), other lung disease (2.56, 1.66-3.97), chronic kidney disease (2.32, 1.50-3.59), obesity and DM (obesity alone: 1.36, 1.07-1.71;DM alone: 1.85, 1.39-2.47;obesity and DM: 1.89, 1.34-2.67;vs no obesity and no DM), higher disease activity and GC intake (remission/low disease activity and GC intake: 1.96, 1.36-2.82;moderate/severe disease activity and no GC intake: 1.35, 1.05-1.72;moderate/severe disease activity and GC intake 2.30, 1.41-3.74;vs remission/low disease activity and no GC intake). Conversely, the following variables were associated with less severe COVID-19 outcomes: time period after 15 June 2020 (16 June 2020-31 December 2020: 0.42, 0.34-0.51;1 January 2021 onwards: 0.52, 0.41-0.67;vs time period until 15 June 2020), a diagnosis of PsO (without arthritis) (0.49, 0.37-0.65;vs PsA), and exposure to TNFi (0.58, 0.45-0.75;vs no DMARDs), IL17i (0.63, 0.45-0.88;vs no DMARDs), IL-23/IL-12 + 23i (0.68, 0.46-0.997;vs no DMARDs) and NSAIDs (0.77, 0.60-0.98;vs no NSAIDs). Conclusion: More severe COVID-19 outcomes in PsO, PsA and axSpA are largely driven by demographic factors (age, sex), comorbidities, and active disease. None of the DMARDs typically used in PsO, PsA and axSpA, were associated with severe COVID-19 outcomes, including IL-17i, IL-23/IL-12 + 23i, JAKi and apremilast.

Establishment of Hong Kong Neuromuscular Disorder Patient Registry

Introduction: Neuromuscular disorders (NMDs) are a group of diseases affecting the peripheral nervous system (1). Many NMDs cause disability or even premature death (2). We aim to design and establish a robust NMD patient registry in Hong Kong. Methods: By modelling international NMD patient registries, we designed patient-professional reported questionnaires to collect the demographic, clinical c haracteristics, genetic details, family history, investigation findings and specific treatment of NMD patients. Patients were recruited through Hong Kong West Cluster (DKCH, QMH) and Kowloon Central Cluster (HKCH). We also developed self-registration online platform. p<0.05 was considered statistically significant. Findings: Since June 2019, 125 NMD patients have been enrolled in the registry with 12 participants registered online. The registry recruited 13 types of NMDs, including spinal muscular atrophy (SMA) (n=31), Duchenne muscular dystrophy (DMD) (n=19) and congenital myopathy (n=18). The age range was 7 months to 63 years old. 65.6% of those enrolled were children (<18 years old). 63.2% were male. 64.8% of the patients had genetic diagnosis. The registry has contributed to two studies. The first one is a prospective study of clinical efficiency of Nusinersen in SMA patients (n=22). 14/16 SMA patients showed improvement in at least one of motor performance (CHOP intend/RULM/HINE/HFMSE) and health-related quality of life after 1st year of treatment. The second study is the reactogenicity and immunogenicity study of the COVID-19 vaccine in DMD patients (n=4). Data will be available in October. Conclusion: Hong Kong Patient registry has contributed to ongoing and new research study to optimise medical care.

The characterisation of a patient cohort that had home spirometry devices and opted into sharing their data with the US Cystic Fibrosis Foundation

Objectives: To analyse characteristics of people with cystic fibrosis (PwCF) who were using home spirometry devices (HS) during 2020–2021 Methods: During the COVID-19 pandemic, the CF Foundation (CFF) partnered with a technology vendor, ZephyRx, to distribute MIR HS devices to eligible PwCF. During 04/2020–12/2021, 20,157 spirometers were shipped to PwCF. PwCF enrolled in the CFF patient Registry (CFFPR) provided an additional consent to have their HS values linked to their CFFPR data. An application programming interface (API) was built to allow transfers of HS data (FEV1, FVC, FEF25–75, sex, date of birth, height) from each device. Each record contained a CFFPR ID to enable its linkage to the CFFPR. This analysis uses CFFPR data to describe the HS cohort and the data obtained through API to characterise HS utilisation trends. Demographic and clinical characteristics between the HS cohort and the 2019–2020 CFFPR population ages 7 and older were also compared. Results: 272 (94.4%) CF programs participated in the HS program. Records of 1,537 patients, who had activated their device by January 10, 2021, or earlier were linked to CFFPR. The cohort was 69.8% adult, 89.5% Caucasian, 57.8% female, and had a mean age of 27.8, and mean FEV1 of 79.9% predicted. When compared to the CFFPR population, the HS cohort was older, contained more Caucasians and females, and had lower lung function. The median number of acceptable FEV1 measurements supplied per PwCF was 4 (IQR 2–8). 1065 (69%) PwCF in the HS cohort continued to use their device 6 months from activation. Conclusions: HS data has the potential to augment care and research databases like the CFFPR. Little is known about PwCF’s long-term usage of HS devices in a real-world setting. While the HS cohort is small and may be biased compared to the CFFPR population, we have established a reliable channel for collecting HS data and that PwCF’s usage patterns suggests that most are using the devices on a regular basis.

Living conditions of people with diabetes: A scoping review on the impact of the COVID-19 pandemic in Germany

The present study gives an overview on the effects caused by the ongoing COVID-19 pandemic on the living and care situation of people with diabetes in Germany. For this purpose, a systematic search was conducted using the scoping review methodology. On the one hand, a systematic literature search was accomplished in scientific databases for empirical studies and in other search areas for other non-empirical publications. On the other hand, routinely collected electronic health data (routine data;e. g., health insurers administrative data, data from patient registers, medical billing, and drug care data from contractual physicians) were requested from health insurance companies, patient registries or other institutions to gain insight into the care situation of people with diabetes. The literature search identified a total of 53 publications (12 empirical studies and 41 other publications) which were included in the data extraction. Additionally, the methodological quality of the empirical studies was assessed. Due to the small number of empirical studies and their low methodological quality, the evidence gaps regarding the impact of the COVID-19 pandemic on care of people with diabetes are large. However, the empirical studies provide little evidence that the pandemic had a negative impact on the use of diabetes-specific services. The studies show fewer new and reenrolments in disease management programs for diabetes;fewer changes in prescriptions of blood glucose-lowering drugs;fewer diabetes diagnoses and a higher rate of diabetic ketoacidosis in children and adolescents. Additionally, the COVID-19 pandemic has encouraged the use of digital tools for the care of people with diabetes. The search for routine data remained without results. In summary, very limited reliable data on the effects of the COVID-19 pandemic on the care of people with diabetes in Germany was available.

STROKE CARE QUALITY BETWEEN 2017- 2020: OBSERVATIONAL DATA FROM THE AUSTRALIAN STROKE CLINICAL REGISTRY

Background and aims: National clinical quality registries facilitate reliable monitoring of stroke care by providing local hospital teams with data on their performance compared to national benchmarks. We aimed to assess changes in stroke care over time from public hospitals participating in the Australian Stroke Clinical Registry (AuSCR). Methods: AuSCR stroke quality care indicators were compared between 2017 and 2020, using a matched-hospital design. Analyses were limited to adults with stroke or transient ischaemic attack admitted to hospitals contributing ≥30 episodes each year during the study period. Descriptive statistics and linear tests for trend were used to assess changes in quality indicators across years. Results: Among 47 eligible hospitals, admissions increased from 13,508 (2017) to 18,139 (2020). Overall, half were aged ≥75 years, 45% were female, and 59% had a severe stroke (no differences by year). Between 2017 and 2020, improvements were observed for: endovascular retrieval (+8%;P<0.001), hyperacute antithrombotics (+6%;P<0.001), mobilisation during admission (+3%;P<0.001), swallow screen/assessment within 4 hours (+12%;P<0.001), discharge care planning (+11%;P<0.001), and discharge secondary prevention medications (+10%;P<0.001). However, delivery of thrombolysis remained unchanged (-1%;P=0.07), door-toneedle within 60 minutes decreased (-6%;P=0.008), and access to stroke unit care declined in 2020 (76% 2019 vs 72% 2020;P<0.001). Conclusion: Improvements in many indicators of quality stroke care have been observed within Australian hospitals participating in a national registry. Declines in timeliness to thrombolysis and access to stroke units in 2020 represent a likely consequence of the COVID-19 pandemic that requires national action.

Understanding the Resiliency of the Muscular Dystrophy Community One-Year into the COVID-19 Pandemic

Objective: To assess the long-term social and health impacts of the COVID-19 pandemic on people with muscular dystrophy (MD). Background: As the COVID-19 pandemic has continued, it has produced lasting impacts on daily life worldwide. People with muscular dystrophy are potentially at a higher risk for complications when infected with COVID-19, but little is known about the continued impact of COVID-19 on the muscular dystrophy population. Design/Methods: We modified our prior COVID-19 Impact Survey (K. Eichinger, et al) to assess impacts from the continuing pandemic using feedback from muscular dystrophy experts, patients, and advocacy group/registry representatives. The survey assessed COVID-19 medical history, and the effects of the pandemic on social aspects, muscle disease, and medical care. We also used the Perceived Stress Scale, a validated 10-item scale. The de-identified, electronic survey was distributed to adults with muscular dystrophy via international patient registries or advocacy group websites from February 8, 2021 to March 22, 2021. Results: Respondents (n=1243: 49% FSHD;43% DM, and 8% LGMD) were slightly more women and middle-aged (range 18-90). COVID-19 infection rates were 8%. Reported recovery times were typically less than 2 weeks with only 9% reporting recovery greater than 8 weeks, and 7% requiring hospitalization. Major challenges reported during the pandemic included stress management (27%) and wearing a mask (24%). The majority reported a slight worsening of their disease. Respondents reported moderate stress levels (average= 15.8;range= 0-39), with higher stress levels reported by women and those under age 30 years. Of the participants who had telemedicine visits, 70% reported satisfaction;however, most preferred in-person visits. Conclusions: People with muscular dystrophy reported moderate stress and challenges during the COVID-19 pandemic. COVID-19 infection rates and medical complications were similar to a general population. Telemedicine visits may have a more permanent role in care, though inperson visits are still preferred.

Post-acute neurological sequalae of SARS-COV-2 infection

Objective: To investigate associations of COVID-19 illness severity in individuals who have developed objective or subjective neurologic findings after infection. Background: Following recovery from acute COVID-19 illness many patients report onset of new cognitive and neurological symptoms which can be disabling. Design/Methods: Early in the pandemic, in response to clinical experience and emerging research on post-acute neurological sequelae (PANS) of COVID-19, we created an IRB-approved patient registry in the Department of Neurology. Participants included are both retrospectively identified patients located through a search of all existing patients from Neurology outpatient practices at Columbia University Irving Medical Center with any COVID-19 related diagnosis, plus newly referred patients with PANS. Those included met CDC criteria of either suspected, probable, or confirmed COVID-19 (N=121). Information was obtained retrospectively through chart review and prospectively through symptom questionnaire and mini-MoCA. Analysis was performed with Chi-squared test and Pearson's correlation. Results: Our cohort was 72.7% women, mean age 47.9, 54.2% white, 16.7% Hispanic/Latino, 6.7% Black/African American, and 5% Asian. 55.45% had a prior neurological diagnosis, most commonly headache (23.1%). 68.8% had both clinical and lab definite COVID-19 infection, 23.1% required hospitalization, and 9.1% ICU care. 72.2% reported no worsening of prior neurological symptoms but 81.8% developed new neurological symptoms including general cognitive complaints (47.9%), attention difficulty (42.1%), word finding difficulty (36.4%), vestibular complaints (23.1%), and fatigue (19.8%). Mini-MoCAs were administered to 37 subjects (median score 12/15). Hospitalization for COVID-19 correlated with subjective “brain fog” (p= .009) and attention difficulty (p= .011). ICU requirement correlated with subjective word finding difficulty (p= .049), “brain fog” (p= .034), and attention difficulty (p= .020). There was a relationship between length of hospitalization and mini MoCA score (p= .006). Conclusions: In this patient sample, severity of infection assessed through surrogate measures of hospitalization and ICU requirement are associated with subjective and objective post COVID19 neurological dysfunction.

PANDEMIC POPULATION MENTAL HEALTH: PAST, PRESENT AND FUTURE

Background: The challenges of the COVID-19 pandemic have dominated Australian healthcare. Counter-intuitively, despite baseline workforce, infrastructure and access problems, population mental health and mental health services have weathered the first two years of the pandemic. However, there remain especially vulnerable sub-populations. Objectives: To provide a clinical update for psychiatrists and trainees on specific aspects of population mental health and mental health service performance during the COVID-19 pandemic. Methods: We review the relevant research and policy data. Conclusions: COVID-19 pandemic population mental health policy research and analysis is essential to improve clinical care and will be needed for future pandemics. The establishment of clinical registries for population mental health data can help guide future pandemic mental health policy, planning and intervention.

Follow-up of hospitalized patients diagnosed with COVID-19 after recovery from the acute episode: COVID-19 registry (NCT04347278)

Objective: To assess the quality of life perceived by patients after recovery from COVID-19 in a period of time between 6 and 12 months after hospital discharge. Material and/or methods: Prospective observational study, IDI-REM-2020 code and EUPAS34551 registration, non-interventional, telephone survey from 6 months after discharge date. Health questionnaire consisted of 13 questions covering 8 dimensions (physical functioning, bodily pain, general health, vitality, social functioning and emotional role). Results: Out of 250 patients included and hospitalized in the first wave of the pandemic, from March to April 2020, 161 patients were surveyed. As for the data recorded: at the beginning of the questionnaire general health was fair or poor in 39.8% of respondents and in the rest there was no impact. Social activities had no impact in 39% of the respondents, and only 26.3% had appreciable impact. As for bodily pain, although it did not hinder the usual work in 83.6% of the respondents, in the rest it was a major limitation. Emotional problems affected daily activity in 29.4% of cases. After suffering COVID infection, 50.3% of the participants indicated that their health worsened while in 45.9% it remained the same or even improved in 3.1% of the participants. Conclusions: The quality of life questionnaire is essential to complete a clinical data registry and to understand whether COVID-19 infection is affecting health in the months following infection. In this cohort of patients, a negative impact on health was seen in half of the patients followed.

An illicit drug early warning system utilising comprehensive toxicological analysis of emergency department presentations in Victoria, Australia

Objective: In response to the evolving threat of illicit drug use, combined with anticipated SARS-CoV-2 (COVID-19) pandemicrelated market volatility, we created a multi-institution network supplying high-quality data on illicit drug presentations to Victorian emergency departments (EDs). Primary objective: timely data provision to a state Early Warning System (EWS) utilising multiple intelligence sources (including syringe residue and wastewater analysis) to inform public health interventions. Methods: The Emerging Drugs Network of Australia VIC (EDNAV) project is a multi-site prospective observational study collating de-identified clinical and analytical information within an electronic clinical registry (Research Electronic Data Capture secure web-based software platform). Case inclusion criteria: individuals ≥16 years of age presenting with suspected illicit drug toxicity requiring venepuncture as part of standard care. Hospital ethics committee approved waiver of patient consent for inclusion of deidentified data. Nine metropolitan and one regional ED contributed blood samples for weekly toxicological analysis at the Victorian Institute of Forensic Medicine. Liquid chromatographytandem mass spectrometry (LC-MS/MS) screened for 327 pharmaceuticals and illicit substances, as well as 268 novel psychoactive substances. EDNAV data was reviewed weekly as a component of the state EWS. High-risk signals were disseminated to government and external stakeholders. Results: During September 2020 - March 2021, 320 cases were analysed (70% male, mean age 30 years, 72% ambulance arrival). Sedation (Glasgow Coma Score (GCS)<9, 35%) and agitation (33%) were the commonest reasons for presentation;33% of patients required parenteral sedation, and 18% were administered naloxone. In addition, 8% were intubated and 11% required critical care admission;85% had a Poisoning Severity Score of ≥2. There were two deaths. There were 815 separate detections (345 illicit substances, 470 pharmaceuticals). At least one illicit drug was detected in 87% of cases (> 1 illicit drug in 43%). Common illicit drugs included methylamphetamine (52% of cases), gamma-hydroxybutyrate (GHB), 3,4-methylenedioxymethamphetamine (MDMA), cocaine and opioids. Eight novel benzodiazepines, 7 cathinones, 5 hallucinogens, 3 synthetic cannabinoid receptor agonists (SCRAs) and one novel opioid (Beta-U10) were detected. In 90% of cases, reported exposure differed from analytical findings. During COVID-19 related lockdowns, there was evidence of substance substitution including benzodiazepines in products sold as heroin. Three public health warnings were released in association with EDNAV findings (Nethylpentylone in cocaine, 25B-NBOH sold as lysergic acid diethylamide (LSD), paramethoxymethamphetamine (PMMA) sold as MDMA). Conclusion: For the first time in Victoria, a network of healthcare institutions working together enabled timely detection of illicit drug related harm, facilitating early public health warnings and notification of peer-based harm reduction services.