**Author details**

**Name of Score Year**

**CF-ABLE Score**

**22. Conclusion**

**[93]**

**Liou Predictive 5 year survivorship model of Cystic Fibrosis [145]**

**Published**

24 Cystic Fibrosis in the Light of New Research

**Number of patients in cohort**

2001 Retrospective data collected 5820 patients in US registry between 1986 and 1993

2013 49 CF patients

**Table 2.** Summary of Clinical Prediction Tools in Cystic Fibrosis.

followed over a 7 year period. Validated in 370 patients collected from national registry over 5 years

**Parameters Used Strengths Limitations**






Composite score of the presence or value of following parameters to predict 5-year survival:



7-point scale based on evaluation of: - Age <24 = 1 point - BMI <20.1 kg/m2


Score of >5 indicates 26% chance of poor outcome in

the next 4 years

Over the last two decades, prognosis has improved significantly in CF and this has led to the need to better predict outcomes. Subsequently, many studies have looked at all aspects of the disease and identified modifiable risks, as well as identified biomarkers to follow disease progression. This increased knowledge of the condition has allowed improved treatment and more phenotypically specific therapy to be developed. Many different clinical features and markers predict outcome and this is a complex area, and a multifaceted approach to risk stratifying patients is needed. However, what must be not overlooked as the survival increases in CF are the extrapulmonary manifestations and the significant psychosocial issues associated with chronic illness. It is important to note that up to 22% of CF patients demonstrate symptoms of depression, 10% report anxiety symptoms, and up to 5% report suicidal thoughts [146]. The

= 1


(0-5)

points

Cormac McCarthy\* , Orla O'Carroll, Alessandro N. Franciosi and Noel G. McElvaney

\*Address all correspondence to: cmccarthy@rcsi.ie

Respiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland
