Effects of a primary palliative care intervention on quality of life and mental health in cystic fibrosis

Abstract

Background: Despite the significant impact of chronic symptoms on quality of life with cystic fibrosis (CF), the role of palliative care in management of this disease is not well defined. The coping, goal assessment, and relief from evolving CF symptoms (CF-CARES) model is a primary palliative care intervention designed to provide chronic symptom management at all stages of the disease. The goal of this pilot study was to estimate the effectiveness of the CF-CARES intervention on improving chronic symptoms and quality of life for people living with CF. Methods: A structured assessment was used to guide referral to supportive services intended to address burdensome symptoms. Follow-up assessments were performed approximately 3 and 6 months later. Longitudinal regression analyses of changes in symptoms and quality of life were performed for all participants regardless of utilization of supportive services. Subgroup analyses were performed for subjects participating in mental health and alternative health services. Results: Forty-one subjects completed assessment and referral processes. The mean number of CF-associated symptoms decreased over time, as did respiratory symptom-related distress and depressive symptoms. Subjects utilizing alternative health services reported less psychological distress at follow-up. Among subjects with severe disease, mental health, and quality of life improved, especially for those using mental health services. Conclusions: The CF-CARES model resulted in significant mental health and quality-oflife benefits, suggesting the value of integrating symptom management interventions Pediatric Pulmonology. 2019;54:984-992. wileyonlinelibrary.com/journal/ppul 984 | Questionnaire 9-item scale; ppFEV 1 , percent predicted forced expiratory volume in 1 second; QoL, quality of life. Moskowitz and Yonker have contributed equally to this work.

Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease

“…Some unique barriers to ACP exist in CF and especially ACFLD including variable disease progression among those with advanced disease, the potential for rapid deterioration in some individuals, and the perceived conflict between advanced planning and pursuit of lung transplantation. Early introduction of palliative care in pulmonary disease is associated with more consistent ACP conversations, reduced healthcare utilization, and improved symptoms without reducing survival [14][15][16] . Literature supports early ACP for individuals with CF even before or while pursuing transplant [16][17][18][19] , emphasizing that ACP conversations and aggressive treatments should not be mutually exclusive ( Fig.…”

“…15 The CF Foundation found insufficient evidence to make a recommendation regarding routine screening for gastroesophageal reflux in individuals with advanced CF lung disease. 16 The CF Foundation recommends the use of enteral tube feeds for individuals with advanced CF lung disease and malnutrition after consideration of procedural risks versus benefits. 17 For individuals with advanced CF lung disease with frequent prior and continuing exposure to nephrotoxic and ototoxic agents, the CF Foundation recommends increased monitoring for accumulating toxicity.…”

Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines

“…This recommendation reinforces the importance of PPC as a part of usual CF care and recognizes the capacity of CF care teams to deliver these elements throughout the entire disease trajectory. [19][20][21] Ideally, the provision of palliative care, like standard CF care, is a coordinated, patient-driven partnership by an interdisciplinary team of professionals.…”

Adding Life to Their Years: The Current State of Pediatric Palliative Care in CKD

“…74 Promisingly, a pilot study of a primary PPC intervention for CF patients demonstrated improvement in CF-associated symptoms, decreased depressive symptoms, and diminished psychological suffering. 75 Based on these findings, there has been a call for routine integration of PPC as standard of care for children with CF. 76 Finally, a similar shift to integrating PPC has taken place in the care of children with hypoplastic left heart syndrome.…”