title: Advance care planning in adolescents with cystic fibrosis: A quality improvement project.
abstract: Advance care planning (ACP), though recommended, has not been studied in adolescents with cystic fibrosis (CF). This quality improvement project engaged adolescents with advanced CF disease in ACP and assessed patient and CF provider attitudes and preferences regarding ACP discussions and tools.
  
  
  
  Patients ≤22 years with advanced CF (FEV1≤40% predicted, >2 pulmonary exacerbations requiring IV antibiotics in 1 year, and/or use of home oxygen or non-invasive ventilation) were referred to the pediatric palliative care team (PC). After establishing rapport, ACP was discussed using Voicing My CHOiCES™: An Advanced Care Planning Guide (VMC). Patients completed a survey assessing attitudes and preferences around ACP. PC also led a training session for CF providers around ACP and VMC and provider attitudes were assessed via a pre- and post-training survey.
  
  
  
  Twelve patients (mean age 17.9 ± 2.2 years) reviewed VMC and completed the ACP survey. The majority (83%) found ACP helpful. None felt it was harmful. All found VMC easy to understand and 90% felt it was appropriate for patients with CF. Of participating CF providers (pre-training, n = 6; post-training, n = 7), 83% found ACP worthwhile, but desired more training in this area. All found the training session useful and felt VMC was appropriate for patients with CF.
  
  
  
  Adolescents with advanced CF disease felt ACP was a positive experience and not harmful. CF providers valued ACP, but desired more training. Both patients and providers felt that VMC was a useful, disease-appropriate tool. Pediatr Pulmonol. 2016;51:1304-1310. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Division of Pulmonary Medicine, Allergy, and Immunology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Traci M
authors.initials: TM
authors.lastname: Kazmerski
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Division of Pulmonary Medicine, Allergy, and Immunology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Weiner
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Division of Pulmonary Medicine, Allergy, and Immunology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Janice
authors.initials: J
authors.lastname: Matisko
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Division of Pulmonary Medicine, Allergy, and Immunology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Diane
authors.initials: D
authors.lastname: Schachner
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Supportive Care Program, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Whitney
authors.initials: W
authors.lastname: Lerch
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Supportive Care Program, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Carol
authors.initials: C
authors.lastname: May
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Supportive Care Program, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Scott H
authors.initials: SH
authors.lastname: Maurer
mesh_terms: D000293:Adolescent; D032722:Advance Care Planning; D016223:Advance Directives; D001294:Attitude to Health; D003550:Cystic Fibrosis / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D063087:Noninvasive Ventilation; D010102:Oxygen Inhalation Therapy; D010347:Patient Care Planning; D019727:Proxy; D058996:Quality Improvement; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D013727:Terminal Care; D055815:Young Adult
keywords: advance care planning; cystic fibrosis; end-of-life care