Associations between resources and practices of ILD centers and outcomes in patients with idiopathic pulmonary fibrosis: data from the IPF-PRO Registry.

BACKGROUND: Performance benchmarks for the management of idiopathic pulmonary fibrosis (IPF) have not been established. We used data from the IPF-PRO Registry, an observational registry of patients with IPF managed at sites across the US, to examine associations between the characteristics of the enrolling sites and patient outcomes. METHODS: An online survey was used to collect information on the resources, operations, and self-assessment practices of IPF-PRO Registry sites that enrolled ≥ 10 patients. Site variability in 1-year event rates of clinically relevant outcomes, including death, death or lung transplant, and hospitalization, was assessed. Models were adjusted for differences in patient case mix by adjusting for known predictors of each outcome. We assessed whether site-level heterogeneity existed for each patient-level outcome, and if so, we investigated potential drivers of the heterogeneity. RESULTS: All 27 sites that enrolled ≥ 10 patients returned the questionnaire. Most sites were actively following > 100 patients with IPF (70.4%), had a lung transplant program (66.7%), and had a dedicated ILD nurse leader (77.8%). Substantial heterogeneity was observed in the event rates of clinically relevant outcomes across the sites. After controlling for patient case mix, there were no outcomes for which the site variance component was significantly different from 0, but the p-value for hospitalization was 0.052. Starting/completing an ILD-related quality improvement project in the previous 2 years was associated with a lower risk of hospitalization (HR 0.60 [95% CI 0.44, 0.82]; p = 0.001). CONCLUSIONS: Analyses of data from patients with IPF managed at sites across the US found no site-specific characteristics or practices that were significantly associated with clinically relevant outcomes after adjusting for patient case mix. Trial registration ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511.

COVID-19 in lung transplant recipients-Risk prediction and outcomes.

Patients after lung transplantation are at risk for life-threatening infections. Recently, several publications on COVID-19 outcomes in this patient population appeared, but knowledge on optimal treatment, mortality, outcomes, and appropriate risk predictors is limited. A retrospective analysis was performed in a German high-volume lung transplant center between 19th March 2020 and 18th May 2021. Impact of COVID-19 on physical and psychological health, clinical outcomes, and mortality were analyzed including follow-up visits up to 12 weeks after infection in survivors. Predictive parameters on survival were assessed using univariate and multivariate proportional hazards regression models. Out of 1,046 patients in follow-up, 31 acquired COVID-19 during the pandemic. 12 of 31 (39%) died and 26 (84%) were hospitalized. In survivors a significant decline in exercise capacity (p = 0.034), TLC (p = 0.02), and DLCO (p = 0.007) was observed at follow-up after 3 months. Anxiety, depression, and self-assessed quality of life remained stable. Charlson comorbidity index predicted mortality (HR 1.5, 1.1-2.2; p = 0.023). In recipients with pre-existing CLAD, mortality and clinical outcomes were inferior. However, pre-existing CLAD did not predict mortality. COVID-19 remains a life-threatening disease for lung transplant recipients, particularly in case comorbidities. Further studies on long term outcomes and impact on pre-existing CLAD are needed.

COVID-19 pandemic restrictions continuously impact on physical activity in adults with cystic fibrosis.

BACKGROUND: We have recently reported reduced physical activity (PA) in people with cystic fibrosis (pwCF) with and without lung transplantation (LTX) during a 6-week stringent lockdown in Switzerland. This follow-up study explores the impact of coronavirus-2019 disease (COVID-19) related pandemic restrictions on individuals' therapy regimens and health-related aspects in pwCF. METHODS: We conducted a cross-sectional web-based national survey in Spring 2021. The survey included questions on daily PA, airway clearance and inhalation therapy, questions on COVID-19-compatible symptoms, diagnostic tests and vaccination status, and enquired health-related aspects covering the pandemic period between March 2020 to April 2021. RESULTS: 193 individuals with CF (53% female; 25% LTX recipients) participated. Among pwCF, 10 reported COVID-19 (n = 2 LTX recipients), two subjects were hospitalized, no invasive ventilation required, no deaths. The clinical course was generally mild. Overall, 46% reported less PA during the pandemic, mostly due to closed fitness facilities (85%), lack of motivation (34%), and changes in daily structures (21%). In contrast, 32/193 (17%) pwCF were able to increase their PA levels: 12 (38%) and 11 (34%) reported undertaking home-based training and outdoor activities more frequently; 6 (19%) reported an increase in routine PA, and another 3 (9%) started new activities. Among pwCF without LTX, 5% and 4% reported to undertake less airway clearance and inhalation therapy, respectively. CONCLUSIONS: Our study reveals unfavorable consequences of COVID-19 pandemic restrictions on PA of pwCF with unknown long-term consequences for their overall physical fitness and lung health. Strategies to overcome this undesirable situation are needed; increased uptake of telehealth PA programs and virtual exercise classes to promote PA participation might be one promising approach along with vaccination of pwCF and their close contacts.

Lung Transplantation for Severe Post-coronavirus Disease 2019 Respiratory Failure.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has resulted in >72 million cases and 1.6 million deaths. End-stage lung disease from COVID-19 is a new and growing entity that may benefit from lung transplant; however, there are limited data on the patient selection, perioperative management, and expected outcomes of transplantation for this indication. METHODS: A systematic review of the literature was performed with searches of MEDLINE and Web of Science databases as well as the gray literature. All manuscripts, editorials, commentaries, and gray literature reports of lung transplantation for COVID-related respiratory failure were included. A case from the University of Virginia is described and included in the review. RESULTS: A total of 27 studies were included: 11 manuscripts, 5 commentaries, and 11 gray literature reports. The total number of transplantations for COVID-related lung disease was 21. The mean age was 55±12 years, 16 (76%) were male individuals, and the acuity was high, with 85% on extracorporeal membrane oxygenation preoperatively. There was a 95% early survival rate, with 1 additional late death. There is growing histopathologic evidence for permanent structural damage with no replicating virus at the time of transplantation. CONCLUSIONS: Bilateral lung transplantation is an effective treatment option with reasonable short-term outcomes for patients with end-stage lung failure secondary to COVID-19. However, specific considerations in this new population require a multidisciplinary approach. As we move into the second wave of the COVID-19 global pandemic, lung transplantation will likely have a growing role in management of these complex patients.

Geographic Differences in Lung Transplant Volume and Donor Availability During the COVID-19 Pandemic.

BACKGROUND: Regional variation in lung transplantation practices due to local coronavirus disease 2019 (COVID-19) prevalence may cause geographic disparities in access to lung transplantation. METHODS: Using the United Network for Organ Sharing registry, we conducted a descriptive analysis of lung transplant volume, donor lung volume, new waitlist activations, and waiting list deaths at high-volume lung transplant centers during the first 3 months of the pandemic (March 1. 2020, to May 30, 2020) and we compared it to the same period in the preceding 5 years. RESULTS: Lung transplant volume decreased by 10% nationally and by a median of 50% in high COVID-19 prevalence centers (range -87% to 80%) compared with a median increase of 10% (range -87% to 80%) in low prevalence centers (P-for-trend 0.006). Donation services areas with high COVID-19 prevalence experienced a greater decrease in organ availability (-28% range, -72% to -11%) compared with low prevalence areas (+7%, range -20% to + 55%, P-for-trend 0.001). Waiting list activations decreased at 18 of 22 centers. Waiting list deaths were similar to the preceding 5 years and independent of local COVID-19 prevalence (P-for-trend 0.36). CONCLUSIONS: Regional variation in transplantation and donor availability in the early months of the pandemic varied by local COVID-19 activity.

Lung transplantation during the outbreak of Coronavirus Disease 2019 in China.

OBJECTIVES: The study objectives were to illustrate our workflow for lung donation and transplantation during the Coronavirus Disease 2019 crisis and to report our preliminary experience with perioperative care. METHODS: We retrospectively analyzed data in the China Lung Transplantation Registration from January 23, 2020, to March 23, 2020 (2020 cohort), compared with the same period in 2019 (2019 cohort). Pre- and post-lung transplantation management strategies, including measures aiming to prevent severe acute respiratory syndrome coronavirus 2 infection, were applied to all recipients, including 5 post-Coronavirus Disease 2019 transplants during the Coronavirus Disease 2019 pandemic period in China. RESULTS: Twenty-eight lung transplant procedures were performed, including lung transplant for 5 patients with acute respiratory distress syndrome due to Coronavirus Disease 2019-related pulmonary fibrosis. Compared with the 2019 cohort, more patients with urgent conditions received transplantation in 2020, with a shorter pre-lung transplant admission time and early mobilization post-lung transplant. A large proportion (60%) of lung donations were transported on high-speed trains and commercial flights or highways and commercial flights. Grafts in the preservation containers were handed over to the receiving staff at the airport for 40% (10/25) of donations, which reduced the unnecessary quarantine of transporting staff entering the city. Listed candidates were urgently transferred to other qualified centers in 17.9% of cases (5/28), which reduced the risk of severe acute respiratory syndrome coronavirus 2 exposure in Coronavirus Disease 2019-designated hospitals. The 90-day survival of the transplant recipients in 2020 was 85.7%, including 3 of 5 recipients (60%) who had critically severe Coronavirus Disease 2019. CONCLUSIONS: Lung transplant and donation amid Coronavirus Disease 2019 can be performed safely with coordinated efforts on medical resource sharing and medical staff protection based on stratification of the infection risk. Outcomes were not compromised during the Coronavirus Disease 2019 outbreak. Lung transplantion can be regarded as salvage therapy for critical patients with Coronavirus Disease 2019 with a confirmed positive turned negative virology status.

Trends in Lung Transplantation Practices Across the United States During the COVID-19 Pandemic.

BACKGROUND: The purpose of this study is to examine the effects of the coronavirus disease 2019 (COVID-19) pandemic on adult lung transplants and report practice changes in the United States. METHODS: A retrospective analysis of a public dataset from the United Network for Organ Sharing was performed regarding adult lung transplantation (January 19, 2020-June 30, 2020). Data were stratified into 3 periods: pre-COVID-19 (January 19, 2020-March 14, 2020), first COVID-19 era (March 15, 2020-May 8, 2020), and second COVID-19 era (May 9, 2020-June 30, 2020). Weekly changes in waitlist inactivations (COVID-19 precautions or not), waitlist additions, transplant volume, and donor recovery were examined across eras and changes across era were correlated. RESULTS: During the first COVID-19 era, 301 patients were added to the waitlist, representing a 40% decrease when compared to the prior 8-week period. This was followed by a significant increase in listing during the second COVID-19 era (t = 2.16, P = 0.032). Waitlist inactivations decreased in the second COVID-19 era from the first COVID-19 era (t = 3.60, P < 0.001). There was no difference in waitlist inactivations between the pre-COVID era and the second COVID-19 era (P = 0.10). Weekly volume was not associated with trends in COVID-19 cases across any era, but was negatively associated with waitlist inactivations due to COVID-19 precautions entering the first COVID-19 era (r = -0.73, P = 0.04) and second COVID-19 era (r = -0.89, P = 0.003). CONCLUSIONS: Due to the COVID-19 pandemic, the United States experienced a decrease in lung transplant volume. While overall volume has returned to normal, additional studies are needed to identify areas of improvement to better prepare for future pandemics.

The global impact of SARS-CoV-2 in 181 people with cystic fibrosis.

With the growing SARS-CoV-2 pandemic, we need to better understand its impact in specific patient groups like those with Cystic Fibrosis (CF). We report on 181 people with CF (32 post-transplant) from 19 countries diagnosed with SARS-CoV-2 prior to 13 June 2020. Infection with SARS-CoV-2 appears to exhibit a similar spectrum of outcomes to that seen in the general population, with 11 people admitted to intensive care (7 post-transplant), and 7 deaths (3 post-transplant). A more severe clinical course may be associated with older age, CF-related diabetes, lower lung function in the year prior to infection, and having received an organ transplant. Whilst outcomes in this large cohort are better than initially feared overall, possibly due to a protective effect of the relatively younger age of the CF population compared to other chronic conditions, SARS-CoV-2 is not a benign disease for all people in this patient group.

Recommended shielding against COVID-19 impacts physical activity levels in adults with cystic fibrosis.

BACKGROUND: Severe acute respiratory syndrome - coronavirus-2 (SARS-CoV-2) has caused a pandemic threatening the life of people with chronic respiratory diseases including cystic fibrosis (CF). This study was designed to investigate health-related aspects of individuals with CF, with and without lung transplantation (LTX), their communication with their specialist healthcare providers during the pandemic, potential changes in peoples' individual therapy regimes and daily physical activity levels. METHODS: A web-based survey was conducted among Swiss adults with CF with and without LTX, study period from March 16th, 2020 - the day the "extraordinary situation" was officially declared in Switzerland introducing stringent measures protecting the public - until May 16th, 2020. RESULTS: 327 individuals (25% LTX recipients) were included, 45 individuals reported coronavirus-2019 disease (COVID-19) like symptoms. Of 28 subjects tested, only three subjects were tested positive, all with mild symptoms, no hospitalization required. Almost half of the survey respondents (45%) reported undertaking less physical activity during the lockdown, while 79% and 91% of participants reported no change in traditional airway clearance and inhalation therapies, respectively. Distress regarding a potential SARS-CoV-2 infection or worsening of lung disease were no major concerns for subjects. CONCLUSIONS: Our study reveals that the direct impact of SARS-CoV-2 on clinical outcomes of individuals with CF was mild although people with chronic lung diseases like CF are considered a high-risk population; overall, this is reassuring. However, strict lockdown measures substantially affected peoples' physical activity levels, a vital cornerstone of CF therapy; and this is worrisome.

The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and paediatric patients.

BACKGROUND: Little is known about the impact of COVID-19 on patients with cystic fibrosis (CF), despite being considered a high-risk group. This study explored the early impact of COVID-19 on the emotional well-being of patients and self-reported changes in their home therapy since the start of the pandemic. METHODS: Adult patients with CF, lung-transplanted (LTX) CF patients and parents of children with CF completed an online questionnaire, securely linked to their medical files. The questionnaire covered the emotional impact of the pandemic, changes in CF and LTX treatment, changes in health-protecting behaviours and CF-related concerns, and their perception of their COVID-19 status. RESULTS: The response rate was 63% (80 CF, 66 LTX and 73 parents). A wide range of illness severity was included. None of the respondents had contracted COVID-19 and all strictly followed the social distancing rules. There was evident psychological impact, with many reporting increased stress, fear and worry about CF and the future. Changes in treatment were positive, including more physiotherapy for adults and better-quality nebulizing. Changes in routine were reported, such as different treatment timing. Adult patients and parents had cancelled their CF appointments more often since the start of the pandemic. CONCLUSIONS: The initial psychological impact of COVID-19 was evident. The impact on home treatment was reassuringly small. Psychological care is needed for patients suffering prolonged psychological impact, and CF teams need to contextualize the information that patients and parents receive from the media and support them to balance the perceived risk with true risk.