Recurrent exacerbations affect FEV1 decline in adult patients with cystic fibrosis

Abstract

Obstructive lung disease is the major cause of morbidity and mortality in cystic fibrosis (CF). To identify risk factors contributing to FEV(1) decline in CF patients, we carried out a retrospective analysis of clinical and pulmonary function data in a population of CF patients followed up for 5 years and studied the correlation between clinical data and FEV(1) decline. Fifty-one adult CF patients were studied. The FEV(1) decline was related to the following clinical characteristics: CFTR genotype, age, gender, weight, height, age at diagnosis, baseline FEV(1), pancreatic function, presence of airway infection, pancreatic insufficiency and diabetes, number of exacerbations/year and intravenous (i.v.) antibiotic courses/year. Both the number of exacerbations/year and the number of i.v. antibiotic courses/year were strongly related to the FEV(1) decline. Patients with airway infection or with diabetes had significantly lower FEV(1) values during the study as compared with non-infected patients or patients without diabetes; however, both the presence of airway infection or diabetes did not affect the FEV(1) decline. These results suggest that the aggressive treatment of disease exacerbations is crucial for delaying lung function decline in CF.

Cystic Fibrosis Chronic Rhinosinusitis: A Comprehensive Review

“…46 The general lack of chronic ear disease in CF patients and normal temporal bone development argues against this hypothesis. 47 However, genotype does appear to influence paranasal sinus development as individuals homozygous for the F508del mutation have shown a significantly increased frequency of underdeveloped frontal (98%), maxillary (70%), and sphenoid (100%) sinuses when compared with other genetic mutations (69,8, and 50%, respectively), suggesting CFTR may be a primary contributor to sinus development. 45 Studies from the recently developed CF pig model support the premise that CFTR dysfunction as opposed to chronic infection is responsible for decreased sinus pneumatization because pigs lacking intact CFTR have sinus underdevelopment before the development of infection.…”

“…Several studies showing severe CF sinusitis as a risk factor for forced expiratory volume in 1 second (FEV 1 ) decline and thus worsened prognosis in children suggest aggressive treatment may have a role in select patients. [69][70][71] …”

Exposure Memory and Lung Regeneration

“…Respiratory distress syndromes can occur acutely, and prior exposures may determine which repair and regenerative methods are needed to prevent permanent loss of function (12). Similarly, in cystic fibrosis, recurring exacerbations result in long-term lung failure (13), and the lifelong history of exposures may define the need for different conditioning regimens to support regeneration. Improved lung regeneration in this diverse spectrum, as well as in chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, sarcoidosis, bronchiolitis obliterans, and other chronic lung diseases, will have considerable impact in pulmonary medicine.…”

Analysis of Changes in Diversity and Abundance of the Microbial Community in a Cystic Fibrosis Patient over a Multiyear Period

“…Pulmonary exacerbations (PEs), which may develop multiple times per year in CF patients, are often caused by a disturbance to a stable chronic bacterial infection (5,6). The exact cause of a PE, often identified by an increase in pulmonary disease symptoms, remains uncertain but is commonly attributed to factors associated with established bacteria, and possibly to viruses or newly acquired bacterial strains (7,8).…”

“…The frequency of PEs has been connected to increased mortality and results in a permanent impairment in lung function (6). Early treatment intervention might reduce the length and severity of a PE; however, attempts at developing tools to predict a PE have had limited success (10,11).…”