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Background: Cystic fibrosis (CF) is a progressive genetic disease characterized by multisystem symptom burden. Specialist palliative care (PC), as a model of care, has been shown to be effective in improving quality of life and reducing symptom burden in other conditions, but has not been tested in CF. Objectives: To develop and test the feasibility and acceptability of a specialist PC intervention embedded within an outpatient CF clinic. Design: Single-site, equal-allocation randomized pilot study comparing usual care with addition of four protocolized quarterly visits with a PC nurse practitioner. Participants: Adults with CF age ≥18 years with any of the following: FEV 1 % predicted ≤50, ≥2 CF-related hospitalizations in the past 12 months, supplemental oxygen use, or noninvasive mechanical ventilation use, and moderate-or-greater severity of any symptoms on the Edmonton Symptom Assessment Scale. Measurements: Randomization rate, intervention visit completion, data completements, participant ratings of intervention acceptability and benefit, and intervention delivery fidelity. Results: We randomized 50 adults with CF of 65 approached (77% randomization rate) to intervention ( n  = 25) or usual care ( n  = 25), mean age 38, baseline mean FEV 1 % predicted 41.8 (usual care), and 41.2 (intervention). No participants withdrew, five were lost to follow-up, and two died (88% retention). In the intervention group, 23 of 25 completed all study visits; 94% stated the intervention was not burdensome, and 97.6% would recommend the intervention to others with CF. More than 90% of study visits addressed topics prescribed by intervention manual. Conclusions: Adding specialist PC to standard clinic visits for adults with CF is feasible and acceptable.

Context: Patients living with cystic fibrosis (CF) report impaired quality of life. Little is known about unmet supportive care needs among adults living with CF and how they are associated with demographic characteristics., Objectives: The primary objective of this study was to identify associations between demographic variables and unmet supportive care needs regarding anxiety, sadness, pain and uncertainty about the future of living with CF., Methods: We recruited 165 adults with CF from a single academic medical centre to complete a brief demographic survey and the Supportive Care Needs Survey (SCNS-34), a validated self-reported needs assessment that measures the prevalence of and preferences for support for 34 needs that commonly occur in patients with serious illness., Results: Approximately half of the participant sample was male, with a median age of 29 years, varying income levels and a range of lung disease severity. We found statistically significant associations between insufficient income and increased odds of reporting need for support regarding anxiety (OR: 6.48; 95% CI 2.08 to 20.2), sadness (OR: 6.15; 95% CI 2.04 to 18.5), pain (OR: 7.06; 95% CI 2.22 to 22.4) and worries surrounding uncertainty about the future (OR: 3.43; 95% CI 1.18 to 9.99)., Conclusion: Adults with CF report significant unmet needs for support in several physical and emotional domains. Many of these domains were associated with demographic characteristics, most notably, income. Our findings underscore the importance of developing treatment approaches that are sensitive to patient demographics when addressing unmet supportive care needs among adults with CF., Competing Interests: Competing interests: DK receives research support from the NIH/NHLBI (K01HL133466) and the Cystic Fibrosis Foundation (PILEWS14QI0 and KAVAL18QI0). EPD receives research support from the Cystic Fibrosis Foundation (DELLON14QI0 and DELLON16QI0). CHG receives research support from the FDA (R01FD003704), NIH (R01HL113382, R01AI101307, UM1HL119073, P30DK089507 and UL1TR000423) and the Cystic Fibrosis Foundation. JP receives research support from the Cystic Fibrosis Foundation and National Institutes of Health (U01 HL131046, P30 DK072506 and U01 HL128954).National Institutes of Health(U01 HL131046, P30 DK072506 and U01 HL128954)., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

It is increasingly evident that there is little data available to compare the use of various modes of mechanical ventilation or to assess their application. It is difficult to compare the new modes of mechanical ventilation with the conventional modes because of a similar lack of clinical data. The challenge for future research in the area of ventilator technology is to generate controlled clinical studies to support its application. With the increased impact of financial constraints on healthcare, research will also need to examine the economic issues related to the application of newer modes of mechanical ventilation. The critical care nurse will be faced with the continued challenge of being knowledgeable regarding the current trends in ventilatory support and their potential advantages and disadvantages, while keeping in perspective those areas where clinical research is lacking. Possibilities for future nursing research related to mechanical ventilation are endless. The application and refinement of assessment parameters to evaluate the impact of nursing interventions on mechanically ventilated patients should be a key focus. The growing use of SVO2 monitoring in conjunction with other assessment parameters may prove to be useful tools to measure the impact of interventions such as suctioning, positioning, muscle reconditioning, weaning techniques, and comfort measures on mechanically ventilated patients.