Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK.

Background: Nintedanib slows progression of lung function decline in patients with progressive fibrosing (PF) interstitial lung disease (ILD) and was recommended for this indication within the United Kingdom (UK) National Health Service in Scotland in June 2021 and in England, Wales and Northern Ireland in November 2021. To date, there has been no national evaluation of the use of nintedanib for PF-ILD in a real-world setting. Methods: 26 UK centres were invited to take part in a national service evaluation between 17 November 2021 and 30 September 2022. Summary data regarding underlying diagnosis, pulmonary function tests, diagnostic criteria, radiological appearance, concurrent immunosuppressive therapy and drug tolerability were collected via electronic survey. Results: 24 UK prescribing centres responded to the service evaluation invitation. Between 17 November 2021 and 30 September 2022, 1120 patients received a multidisciplinary team recommendation to commence nintedanib for PF-ILD. The most common underlying diagnoses were hypersensitivity pneumonitis (298 out of 1120, 26.6%), connective tissue disease associated ILD (197 out of 1120, 17.6%), rheumatoid arthritis associated ILD (180 out of 1120, 16.0%), idiopathic nonspecific interstitial pneumonia (125 out of 1120, 11.1%) and unclassifiable ILD (100 out of 1120, 8.9%). Of these, 54.4% (609 out of 1120) were receiving concomitant corticosteroids, 355 (31.7%) out of 1120 were receiving concomitant mycophenolate mofetil and 340 (30.3%) out of 1120 were receiving another immunosuppressive/modulatory therapy. Radiological progression of ILD combined with worsening respiratory symptoms was the most common reason for the diagnosis of PF-ILD. Conclusion: We have demonstrated the use of nintedanib for the treatment of PF-ILD across a broad range of underlying conditions. Nintedanib is frequently co-prescribed alongside immunosuppressive and immunomodulatory therapy. The use of nintedanib for the treatment of PF-ILD has demonstrated acceptable tolerability in a real-world setting.

Outcome of Hospitalization for COVID-19 in Patients with Interstitial Lung Disease. An International Multicenter Study.

Rationale: The impact of coronavirus disease (COVID-19) on patients with interstitial lung disease (ILD) has not been established.Objectives: To assess outcomes in patients with ILD hospitalized for COVID-19 versus those without ILD in a contemporaneous age-, sex-, and comorbidity-matched population.Methods: An international multicenter audit of patients with a prior diagnosis of ILD admitted to the hospital with COVID-19 between March 1 and May 1, 2020, was undertaken and compared with patients without ILD, obtained from the ISARIC4C (International Severe Acute Respiratory and Emerging Infection Consortium Coronavirus Clinical Characterisation Consortium) cohort, admitted with COVID-19 over the same period. The primary outcome was survival. Secondary analysis distinguished idiopathic pulmonary fibrosis from non-idiopathic pulmonary fibrosis ILD and used lung function to determine the greatest risks of death.Measurements and Main Results: Data from 349 patients with ILD across Europe were included, of whom 161 were admitted to the hospital with laboratory or clinical evidence of COVID-19 and eligible for propensity score matching. Overall mortality was 49% (79/161) in patients with ILD with COVID-19. After matching, patients with ILD with COVID-19 had significantly poorer survival (hazard ratio [HR], 1.60; confidence interval, 1.17-2.18; P = 0.003) than age-, sex-, and comorbidity-matched controls without ILD. Patients with an FVC of <80% had an increased risk of death versus patients with FVC ≥80% (HR, 1.72; 1.05-2.83). Furthermore, obese patients with ILD had an elevated risk of death (HR, 2.27; 1.39-3.71).Conclusions: Patients with ILD are at increased risk of death from COVID-19, particularly those with poor lung function and obesity. Stringent precautions should be taken to avoid COVID-19 in patients with ILD.

Do temporal scores of motivational constructs correspond with physical activity levels?

BACKGROUND: Adults with physical disabilities typically engage in low levels of physical activity (PA), thus a better understanding of how motivational factors and behavior vary over time and in relation to disability type and severity is needed. OBJECTIVE/HYPOTHESIS: To examine temporal changes in theory of planned behavior (TPB) constructs and PA by disability type (multiple sclerosis vs. spinal cord injury) and severity (mild, moderate, and severe). METHODS: Participants were 77 adults with physical disabilities who completed a web-based survey that assessed the TPB constructs and PA participation. The survey was completed once every four months for a total of three time points. Two-way repeated measures ANOVAs were used to examine if the TPB constructs and PA changed over time and in relation to disability type and severity. RESULTS: There was only one significant between-subjects effect for PA by disability severity, F (2, 68) = 5.94, p = .004, η2 = 15%. People with mild disabilities exhibited higher mean PA participation over the three time periods (M = 21.06 ±â€¯11.49 MET-hrs/day) than those with moderate disabilities (M = 11.27 ±â€¯9.3 MET-hrs/day) and severe disabilities (M = 13.43 ±â€¯14.74 MET-hrs/day). CONCLUSIONS: Although participants possessed positive PA motivational factors that remained stable over time, their PA participation was low and differed as a function of disability severity. Therefore, PA interventions should be tailored such that individuals with severe disabilities receive the greatest amount of exercise prescription consultations and supervision compared to those with more moderate and mild disabilities.

Contemporary challenges for specialist nursing in interstitial lung disease.

The role of clinical nurse specialists (CNSs) in interstitial lung disease (ILD) is evolving in response to clinical guidelines and the growth of clinical research. The role is well established in the UK, although more ILD posts are needed to ensure supply meets clinical demand. This phenomenon is also happening across Europe. An appreciation of the similarities and differences between CNS and advanced nurse practitioners is important given the challenges in defining, developing and supporting this nursing specialisation. Globally, different models exist. In some countries charitable organisations take a leading role in supporting patients. Many European centres look to the National Institute for Health and Care Excellence guidelines and quality standards as a template to develop and evaluate the role of the ILD CNS. We present a UK perspective in the context of a government subsidised healthcare system to promote professional discussion and debate regarding the future of nursing practice in the ILD specialty. KEY POINTS: ILDs are often complex and associated with significant mortality, morbidity and co-morbid conditions that require a technical healthcare skill setThere is worldwide shortage of nurses, low retention rates and retirement of many skilled nursesCollaboration across the ILD interdisciplinary community is needed to safeguard the future of our professions and high-quality patient careThe ILD interdisciplinary and nurse network has identified key priorities to help secure the future of the ILD clinical and academic nurse specialism. EDUCATIONAL AIMS: To explain the similarities and differences between clinical nurse specialists (CNSs) and advanced nurse practitioners (ANPs) in the context of ILD specialismTo review contemporary nursing specialism in the UK's government subsidised healthcare systemTo stimulate discussion and debate across the European/international respiratory community regarding the clinical and academic development of the ILD CNSTo identify key priorities that will support collaboration across the ILD interdisciplinary workforce in clinical practice and research.

The effect of intellectual disability on children's recall of an event across different question types.

This research examined the performance of 80 children aged 9-12 years with either a mild and moderate intellectual disability when recalling an innocuous event that was staged in their school. The children actively participated in a 30-min magic show, which included 21 specific target items. The first interview (held 3 days after the magic show) provided false and true biasing information about these 21 items. The second interview (held the following day) was designed to elicit the children's recall of the target details using the least number of specific prompts possible. The children's performance was compared with that of 2 control groups; a group of mainstream children matched for mental age and a group of mainstream children matched for chronological age. Overall, this study showed that children with either a mild or moderate intellectual disability can provide accurate and highly specific event-related information. However, their recall is less complete and less clear in response to free-narrative prompts and less accurate in response to specific questions when compared to both the mainstream age-matched groups. The implications of the findings for legal professionals and researchers are discussed.