(242) Palliative Care Needs in Cystic Fibrosis (CF): Baseline Data from the Improving Life with CF Multi-site Implementation Trial for Primary Palliative Care Intervention

Background: To evaluate the nature and severity of illness burden among people with CF, we evaluated baseline data collected during a pragmatic implementation trial of a new model of primary palliative care now underway at 5 US CF centers.

Methods: Before launching the intervention, people with CF aged ≥12 years at the participating sites were invited to complete measures including the CFF-recommended [1], 10-item Integrated Palliative Care Outcome Scale (IPOS) [2]. Data were collected from June 2021-February 2022.

Results: The study sample (N=317) included 273 adults (mean age=38.7 years, range 18-79; 59.6% female, 91.2% White, 8.2% Hispanic) and 44 adolescents (mean age=14.1 years, range 12-17; 45.5% female, 84.1% White, 7.0% Hispanic); 30.6% had CF-related diabetes, 7.3% had FEV₁% predicted <40 (median=82) and 83.3% took CFTR modulators. Of the sample, 308 (97%) completed the IPOS, representing 62% of adults and 31% of adolescents with CF treated at the study sites. The mean IPOS total score was 11.4/68 (SD=8.6, range 0-43) for adults and 5.7 (SD=5.9, range 0-22) for adolescents. The most common physical symptoms in the past week described by adults as “moderate”, “severe”, or ‘‘overwhelming” were fatigue (30.8%), pain (17.7%), and drowsiness (16.2%); 24.4% reported “feeling anxious or worried” about their illness or treatment and 18.1% experienced “feeling depressed” either “sometimes”, “most of the time” or “always” (Table 1). In contrast, fewer than 10% of adolescents rated any physical or psychological symptom as moderate-severe. Feeling “at peace” only “sometimes”, “occasionally”, or “not at all” was experienced by 38.7% of adults and 19.1% of adolescents. Perceiving that feelings could be shared with family or friends only “sometimes”, “occasionally”, or “not at all” was reported by 36.5% of adults and 14.3% of adolescents. About 1 in 5 adults and adolescents reported that family or friends were “anxious or worried” about them.

Conclusions: This study used a multidimensional palliative care measure to assess illness burden in a diverse sample of people with CF, providing the first data of this type since the advent of highly effective modulator therapy (HEMT). Adults endorsed elevated responses to all IPOS items at higher rates than adolescents. Not feeling at peace and communication difficulties with loved ones were more frequent sources of illness burden than symptom distress. These data illuminate the nature of illness burden in the era of HEMT and underscore the need for a model of palliative care that, in addition to managing physical symptoms, can address the psychosocial and spiritual needs of people with CF throughout the lifespan. Our multicomponent primary palliative care model, which includes screening-and-triage workflows, patient and provider education, and best practice treatment guidelines for high frequency problems, is implementing such an approach.

Acknowledgements: This work was supported by the Cystic Fibrosis Foundation [grant number CFFGEORGI20QI0].

References: [1] Kavalieratos D, Georgiopoulos AM, Dhingra L, Basile MJ, Rabinowitz E, Hempstead SE, Faro A, Dellon EP. Models of palliative care delivery for individuals with CF: Cystic Fibrosis Foundation evidence-informed consensus guidelines. J Palliat Med. 2021 Jan;24(1):18-30.

[2] Sandham MH, Medvedev ON, Hedgecock E, Higginson IJ, Siegert RJ. A Rasch analysis of the Integrated Palliative Care Outcome Scale. J Pain Symptom Manage. 2019 Feb;57(2):290-296.