Management of CF Lung Disease

Prognosis

Improved Prognosis, But No Cure

Although disease management and prognosis for patients with CF has improved over the past 50 years, it still:

Is a progressive disease
Has no cure and results in early death
Often affects those in the prime of life

One of the biggest challenges is to understand why patients with the same disease have so many variations, which makes it difficult to establish a prognosis for patients. There have been many inconclusive studies conducted attempting to find prognostic indicators in growth patterns, in environmental factors, or in the number or pattern of gene mutations.

Only one thing is certain – the severity of CF is largely determined by how much the lungs are involved.

Prognostic Factors

Data shows that 80% to 95% of patients with CF succumb to respiratory failure that is the result of bacterial infections and inflammation.⁴⁰ Furthermore, chronic infection with Pseudomonas aeruginosa is the leading cause of lung function decline.⁴⁰ Bacterial infections appear to pass through a series of stages from the nonmucoid strains of Pseudomonas aeruginosa to the mucoid strains that correlate strongly with poor prognosis.

There is an accompanying inflammatory response that becomes self-sustaining as infection is established. Not only is this damaging to lung tissue, it also diminishes the immune response and further compromises the patient.⁴⁰

Assessment of lung function is the most reliable determinant of the progression of CF. Of all current measures of long function, none is more effective or more frequently utilized than spirometry for the assessment of FEV₁. (forced expiratory volume in 1 second).^3,41

References:
3. Welsh M. Cystic Fibrosis. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 22nd ed. Philadelphia: WB Saunders; 2004. p. 1-22.
40. Lyczak JB, Cannon CL, Pier GB. Lung infections associated with cystic fibrosis. Clin Microb Reviews 2002;15(2):194-222.
41. Moorcroft AJ, Dodd ME, WebbAK. Exercise testing and prognosis in adult cystic fibrosis. Thorax 1997;52:291-3.