Validation of a predictive survival model in Italian patients with cystic fibrosis

Abstract

The Liou model did not adequately predict 5-year survival in our CF population that, compared to the one in which it was originally tested, could benefit from 10 years of improvement in treatments and practice patterns. A new generated model, based on only four variables, was more accurate in predicting 5-year survival in Italian CF patients.

Palliative Care for Patients with Cystic Fibrosis #265

“…1 This improved survival is attributed to advances in the supportive care of patients, including early diagnosis, family support, attention to nutrition, infection control, and the use of nebulised mucolytics and antimicrobials. 2 Chronic progressive pulmonary disease and respiratory failure remain the major cause of morbidity and mortality. End-stage lung disease is characterized by cysts, abscesses, and fibrosis of lungs and airways.…”

“…Factors associated with earlier death in CF include the deltaF508 mutation; pancreatic insufficiency; low body mass index; certain infections (Pseudomonas aeruginosa, Staphylococcus aureus, and Burkholderia cepacia); low socioeconomic status; tobacco smoke exposure; and female sex. 1,2 For select candidates, lung transplantation improves survival and quality of life. The five-year survival posttransplant is about 50%.…”

“…3 Fears of addiction, respiratory depression, and concern that chronic opioid use complicates lung transplant success can create barriers to appropriate opioid administration. 2,4,5 With multiple medications, therapies, and nutritional supplements required each day, routine care can be physically and emotionally exhausting for CF patients and family members. Adolescents with CF report a high quality of life and they abuse substances less frequently than their healthy counterparts.…”

“…These models, however, are designed to stratify lung transplant candidates rather than assist with end-of-life care planning. 2,8 The dynamic, chronic, progressive course of CF creates challenges for timing goals-of-care discussions. Periodic planned meetings amongst patient, family, and care team are advised.…”

Cost of Cystic Fibrosis: Analysis of Treatment Costs in a Specialized Center in Northern Italy

“…In addition, the probability of 5-year survival was calculated for each patient in Group 2, according to the model proposed by Liou et al [24,25]. …”