The addition of specialist palliative care to standard clinic visits for adults with cystic fibrosis (CF) is feasible, acceptable, and efficacious, according to the results of a pilot, single-site, equal-allocation, randomized controlled study published in the Journal of Palliative Medicine.

The investigators sought to evaluate the feasibility, acceptability, and perceived efficacy of longitudinal specialist palliative care added to routine CF care vs usual CF care alone. As a model of care, specialist palliative care has demonstrated efficacy in improving quality of life (QoL) and burden of symptoms in patients with many conditions, but it has not been tested in those with CF.

Study participants were recruited from the adult CF clinic at the University of Pittsburgh Medical Center between March 2016 and December 2017. All patients were randomized in a 1:1 ratio to the intervention group or the usual-care group.

Study criteria in adults with CF aged 18 or more years included any of the following: (1) forced expiratory volume in 1 second percent predicted (FEV1% predicted) of 50 or less; (2) CF-related hospitalizations in the prior 12 months; (3) supplemental oxygen use or noninvasive mechanical ventilation use: and/or (4) moderate or greater severity of any symptoms on the Edmonton Symptom Assessment Scale.

Learn more about treatments for cystic fibrosis

Fifty of 65 adults were randomized to the study (a 77% randomization rate) to the intervention arm (n=25) or the usual-care arm (n=25). The mean participant age was 38 years. Baseline mean FEV1% predicted was 41.2 in the intervention group and 41.8 in the usual-care group. None of the participants withdrew from the study, although 5 were lost to follow-up and 2 died, which translates to an 88% retention rate.

Overall, 23 of the 25 individuals in the intervention group completed all of their study visits. Among them, 94% of participants indicated that the intervention was not burdensome, and 97.6% stated that they would recommend the intervention to other patients with CF.

In addition, the intervention was perceived to be effective, as measured by patient-reported outcomes in symptom burden and QoL. In fact, 90.9% of surveys from intervention group participants described moderate or greater improvement in physical symptoms, QoL, and mood. Further, 72% of participants’ surveys reported that the intervention improved their understanding of CF or what the future holds regarding the chronic disease process and their ability ot cope.

The authors concluded that because the study was limited to adults aged 18 years and older, “As CF is often diagnosed early in childhood, future studies should examine the effect of introducing [palliative care] concepts much earlier in the disease course.”


Kavalieratos D, Lowers J, Moreines LT, et al. Embedded specialist palliative care in cystic fibrosis: results of a randomized feasibility clinical trial. J Palliat Med. Published online November 9, 2022. doi:10.1089/jpm.2022.0349