Objectives: Assuming a causal relationship between depression and the progression of CF, the aim of our study was to show that elevated symptoms of depression could be a predictor of worse lung function.Methods: 22 CF patients were recruited form our adult CF centre (13 women, 9 men, mean age 24, 18-33 years).The patients completed Patient Health Questionnaire-9 (PHQ 9) and the Generalized Anxiety Disorder 7item scale (GAD 7).Body mass indeX (BMI) and pulmonary function testing were measured.Results: Level of anxiety and depression showed no significant association with decreased lung function.This can be explained by the fact that none of the subjects had serious anxiety and/or depression and prospective study should bed one. Conclusion:The interaction between depression and disease progression could be linked to the lack of hope, optimism and perceived self-efficacy in depressed patients, consequently inhibiting their fighting spirit against the disease and necessary adherence to treatment.Thus, depressed patients may fail to respond to standard medical treatment due to the nature of their mental health problems, and may experience a greater loss of lung function than necessary.This effect may then reinforce the negative thoughts related to their own prognosis, triggering a vicious psychosomatic cycle.In contrast, patients who are not depressed and already have severely restricted lung function might be more able to make use of resources for their daily treatment routines.This, in turn, might result in stable or even slightly improved outcomes.

The onset of lung disease in cystic fibrosis (CF) begins early in life with respiratory infection, inflammation, and structural lung damage all reported in infants with CF in the first months of life. As new treatments become available, it is essential that we have outcome measures that can be used to track disease progression and treatment efficacy. In this review, we have examined the role of lung function testing in infants and preschool children with CF. In particular, we have focused on the ability of the various lung function tests to detect the presence of respiratory pathogens and structural lung disease, increased inflammation, and the onset of acute exacerbations.

Background: Fathers are a neglected group in parenting research, yet they have significant influence on family systems. This paper reports on the experiences of a group of fathers as they manage the impact on the family of the chronic medical challenge of Cystic Fibrosis (CF) in their child.Method: Fifteen fathers of children diagnosed with CF under the newborn screening (NBS) procedures in Western Australia (WA) participated in semi-structured interviews. The transcribed interviews were analysed for common themes using qualitative content analysis, following established procedures for process and rigour. Results: Receipt of a diagnosis of CF for a child altered relationships within the family system. Fathers in this study mostly used internalised and pragmatic coping strategies to assist them to manage anxiety and to come to terms with their child’s diagnosis. These strategies allowed these fathers to shift their focus from the crisis of diagnosis to the functioning of their family. Conclusions: Fathers play an important protective role in families, often so well that their own needs for support are overlooked. Clinical implications are discussed.

BACKGROUND:Lung disease in cystic fibrosis (CF) is established in early childhood with recurrent bacterial infections and inflammation. Using spirometry, the effect of this early lung damage cannot be measured until a child is 6 years of age when some irreversible lung damage may already have occurred. Techniques for measurement of lung function in infants and young children include raised volume rapid thoracic compression (RVRTC) and low frequency forced oscillation (LFFOT). The aim of this study was to investigate the role of inflammation and infection on a population of infants and young children with CF and to determine whether lung function in this population (measured by LFFOT) is affected by early lung disease.METHODS:Lung function was measured by LFFOT in 24 children undergoing bronchoalveolar lavage (BAL) on 27 occasions as part of an annual programme while still under general anaesthesia. Following lung function testing, three aliquots of saline were instilled into the right middle or lower lobe. The first aliquot retrieved was processed for the detection of microbes, and the remaining aliquots were pooled to assess inflammatory markers (cytology, IL-8, NE, LTB(4)).RESULTS:Inflammation (percentage and number of neutrophils) was significantly higher in children with infections (p<0.001, p = 0.04, respectively), but not in those with symptoms. Several markers of inflammation significantly correlated with LFFOT parameters (R, G, and eta).CONCLUSION:Infections and inflammation are established before symptoms are apparent. Inflammation is correlated with measures of parenchymal changes in lung function measured by LFFOT.