Many lung transplant candidates and recipients are older and frailer compared to previous eras. Older patients are at increased risk for pre- and posttransplant mortality, but this risk is not explained by numerical age alone. This manuscript represents the product of the American Society of Transplantation (AST) conference on frailty. Experts in the field reviewed the latest published research on assessment of elderly and frail lung transplant candidates. Physical frailty, often defined as slowness, weakness, low physical activity, shrinking, and exhaustion, and frailty evaluation is an important tool for evaluation of age-associated dysfunction. Another approach is assessment by cumulative deficits, and both types of frailty are common in lung transplant candidates. Frailty is associated with death or delisting before transplant, and may be associated with posttransplant mortality. Sarcopenia, cognitive dysfunction, depression, and nutrition are other important components for patient evaluation. Aging-associated inflammation, telomere dysfunction, and adaptive immune system senescence may also contribute to frailty. Developing tools for frailty assessment and interventions holds promise for improving patient outcomes before and after lung transplantation.

Background: The COVID-19 pandemic resulted in a rapid shift from center-based rehabilitation to telerehabilitation for chronic respiratory disease and lung transplantation due to infection control precautions. Clinical experience with this delivery model on a large scale has not been described. Objective: The aim of this study is to describe usage and satisfaction of providers and lung transplant (LTx) candidates and recipients and functional outcomes following the broad implementation of telerehabilitation with remote patient monitoring during the first wave of the COVID-19 pandemic. Methods: This study was a program evaluation of providers, LTx candidates, and early LTx recipients who used a web-based, remote monitoring app for at least four weeks between March 16 and September 1, 2020, to participate in telerehabilitation. Within-subjects analysis was performed for physical activity, Self-efficacy For Exercise (SEE) scale score, aerobic and resistance exercise volumes, 6-minute walk test results, and Short Physical Performance Battery (SPPB) results. Results: In total, 78 LTx candidates and 33 recipients were included (57 [51%] males, mean age 58 [SD 12] years, 58 [52%] with interstitial lung disease, 34 [31%] with chronic obstructive pulmonary disease). A total of 50 (64%) LTx candidates and 17 (51%) LTx recipients entered ≥10 prescribed exercise sessions into the app during the study time frame. In addition, 35/42 (83%) candidates agreed the app helped prepare them for surgery and 18/21 (85%) recipients found the app helpful in their self-recovery. The strongest barrier perceived by physiotherapists delivering the telerehabilitation was patient access to home exercise and monitoring equipment. Between the time of app registration and ≥4 weeks on the waiting list, 26 LTx candidates used a treadmill, with sessions increasing in mean duration (from 16 to 22 minutes, P=.002) but not speed (from 1.7 to 1.75 mph, P=.31). Quadriceps weight (pounds) for leg extension did not change (median 3.5, IQR 2.4-5 versus median 4.3, IQR 3-5; P=.08; n=37). On the Rapid Assessment of Physical Activity questionnaire (RAPA), 57% of LTx candidates scored as active, which improved to 87% (P=.02; n=23). There was a decrease in pretransplant 6-minute walk distance (6MWD) from 346 (SD 84) meters to 307 (SD 85) meters (P=.002; n=45) and no change in the SPPB result (12 [IQR 9.5-12] versus 12 [IQR 10-12]; P=.90; n=42). A total of 9 LTx recipients used a treadmill that increased in speed (from 1.9 to 2.7 mph; P=.003) between hospital discharge and three months posttransplant. Quadriceps weight increased (3 [IQR 0-3] pounds versus 5 [IQR 3.8-6.5] pounds; P<.001; n=15). At three months posttransplant, 76% of LTx recipients scored as active (n=17), with a high total SEE score of 74 (SD 11; n=12). In addition, three months posttransplant, 6MWD was 62% (SD 18%) predicted (n=8). Conclusions: We were able to provide telerehabilitation despite challenges around exercise equipment. This early experience will inform the development of a robust and equitable telerehabilitation model beyond the COVID-19 pandemic.

Low muscle mass is common in lung transplant (LTx) candidates; however, the clinical implications have not been well described. The study aims were to compare skeletal muscle mass in LTx candidates with controls using thoracic muscle cross-sectional area (CSA) from computed tomography and assess the association with pre- and post-transplant clinical outcomes. This was a retrospective, single-center cohort study of 527 LTx candidates [median age: 55 IQR (42–62) years; 54% male]. Thoracic muscle CSA was compared to an age- and sex-matched control group. Associations between muscle CSA and pre-transplant six-minute walk distance (6MWD), health-related quality of life (HRQL), delisting/mortality, and post-transplant hospital outcomes and one-year mortality were evaluated using multivariable regression analysis. Muscle CSA for LTx candidates was about 10% lower than controls (n = 38). Muscle CSA was associated with pre-transplant 6MWD, but not HRQL, delisting or pre- or post-transplant mortality. Muscle CSA (per 10 cm² difference) was associated with shorter hospital stay [0.7 median days 95% CI (0.2–1.3)], independent of 6MWD. In conclusion, thoracic muscle CSA is a simple, readily available estimate of skeletal muscle mass predictive of hospital length of stay, but further study is needed to evaluate the relative contribution of muscle mass versus functional deficits in LTx candidates.

Background: Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD) are a heterogeneous group of heritable genetic connective tissue disorders with multiple characteristics including joint hypermobility, tissue fragility, and multiple organ dysfunction. Respiratory manifestations have been described in EDS patients, but have not been systematically characterized. A narrative review was undertaken to describe the respiratory presentations and management strategies of individuals with EDS and HSD. Methods: A broad literature search of Medline, Embase, Cochrane Database of Systematic Reviews, and Cochrane CENTRAL was undertaken from inception to November 2020 of all study types, evaluating EDS/ HSD and pulmonary conditions. This narrative review was limited to adult patients and publications in English. Results: Respiratory manifestations have generally been described in hypermobile EDS (hEDS), classical and vascular EDS subtypes. Depending on EDS subtype, they may include but are not limited to dyspnea, dysphonia, asthma, sleep apnea, and reduced respiratory muscle function, with hemothorax and pneumothorax often observed with vascular EDS. Respiratory manifestations in HSD have been less frequently characterized in the literature, but exertional dyspnea is the more common symptom described. Respiratory symptoms in EDS can have an adverse impact on quality of life. The respiratory management of EDS patients has followed standard approaches with thoracotomy tubes and pleurodesis for pleural manifestations, vocal cord strengthening exercises, continuous positive pressure support for sleep apnea, and exercise training. Reduced respiratory muscle function in hEDS patients responds to inspiratory muscle training. Conclusion: Respiratory symptoms and manifestations are described in EDS and HSD, and have generally been managed using conservative non-surgical strategies. Research into the prevalence, incidence and specific respiratory management strategies in EDS and HSD is needed to mitigate some of the associated morbidity.