Exploring Associations Between Self-Compassion, Self-Criticism, Mental Health, and Quality of Life in Adults with Cystic Fibrosis: Informing Future Interventions.

Self-compassion is increasingly recognised as an important and beneficial factor in quality of life and mental health-related research, but research within the adult cystic fibrosis (CF) population is scarce. In a cross-sectional study, 114 (56 female, 58 male) adults with CF completed and returned a series of validated questionnaires that assessed CF-related quality of life, negative emotional states (depression, anxiety and stress), self-compassion, and self-criticism. Quality of life and self-compassion were positively correlated, and each in turn were inversely correlated with negative emotional states and self-criticism. Negative emotional states correlated positively to self-criticism. Self-compassion and/or self-criticism moderated ten relationships between various sub-domains of quality of life and negative emotions. Psychological interventions that increase self-compassion may be beneficial for enhancing mental health and quality of life for adults with CF.

Mindfulness moderates the relationship between emotional eating and body mass index in a sample of people with cystic fibrosis.

PURPOSE: Self-regulation in eating is significant for enhancing life expectancy of people with cystic fibrosis (CF), but research with this population is scarce. METHODS: In a cross-sectional study, adults with CF completed a number of psychometric scales exploring typical eating behaviours that may increase calorific intake including motivations to eat palatable foods and scales that may be associated with decreased calorific intake: mindfulness, mindful eating and self-compassion. RESULTS: Findings suggested that motivations to eat palatable foods and eating behaviours correlate with higher BMI, while mindfulness, mindful eating and self-compassion did not reach significance. Mindfulness and mindful eating moderated the relationship between emotional eating and BMI, while self-compassion did not moderate this relationship. CONCLUSIONS: There is a need to develop healthy and effective means of enhancing calorific intake, where this is indicated, adapting mindful eating principles to focus on increasing both self-regulation and pleasure in eating while reducing emotional eating may be one means of doing this. LEVEL OF EVIDENCE: Level V, cross-sectional descriptive study.

Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis.

Early liver transplant (LT) has been advocated for patients with cystic fibrosis liver disease (CFLD) and evidence of deterioration in nutritional state and respiratory function to prevent further decline. However, the impact of single LT on long-term respiratory function and nutritional status has not been adequately addressed. We performed a retrospective analysis of the outcomes of 40 (21 adult/19 pediatric) patients with CFLD transplanted between 1987 and 2009 with median follow-up of 47.8 months (range 4-180). One and five-year actuarial survival rates were 85%/64% for adult and 90%/85% for pediatric LT cohorts, respectively. Lung function remained stable until 4 years (FEV(1) % predicted; pretransplant 48.4% vs. 45.9%, 4 years posttransplant) but declined by 5 years (42.4%). Up to 4 years posttransplant mean annual decline in FEV(1) % was lower (0.74%; p = 0.04) compared with the predicted 3% annual decline in CF patients with comorbidity including diabetes. Number of courses of intravenous antibiotics was reduced following LT, from 3.9/year pretransplant to 1.1/year, 5 years posttransplant. Body mass index was preserved posttransplant; 18.0 kg/m(2) (range 15-24.3) pretransplant versus 19.6 kg/m(2) (range 16.4-22.7) 5 years posttransplant. In conclusion, LT is an effective treatment for selected patients with cirrhosis due to CFLD, stabilizing aspects of long-term lung function and preserving nutritional status.

Outcomes of patients with cystic fibrosis undergoing lung transplantation with and without cystic fibrosis-associated liver cirrhosis.

People with severe cystic fibrosis (CF) lung disease with co-existent CF-associated liver disease (CFLD) are often excluded from consideration of sole lung transplantation, largely because of the concerns that they will subsequently develop hepatic decompensation. This retrospective cohort study aimed at determining whether patients with severe cirrhosis caused by CFLD have any differences in perioperative and relevant post-transplant outcomes compared to CF patients without CFLD when undergoing sole lung transplantation. Six patients with CFLD were matched with 18 CF patients without CFLD undergoing sole lung transplant at the same institution. There were no differences in total operative time or intra-operative requirements for cardiopulmonary bypass or blood products. Over a period of five yr post-transplant, no differences were observed between the two groups in body mass index, six-min walk, lung function, and survival. None of the CFLD subjects developed variceal bleeding; however, one developed hepatocellular and renal failure at four yr post-transplant and is being assessed for liver-kidney transplant. One additional patient with CFLD required repeat lung transplantation for bronchiolitis obliterans syndrome. This study provides evidence that CF patients with liver cirrhosis caused by CFLD can safely be considered for sole lung transplantation provided there is no evidence of significant hepatocellular dysfunction with decompensated cirrhosis or hepatic synthetic failure.

Survival of Burkholderia cepacia sepsis following lung transplantation in recipients with cystic fibrosis.

Cystic fibrosis (CF) lung transplant recipients infected with Burkholderia cenocepacia have a worse survival rate after lung transplantation than those who are not infected with this organism. The decreased survival is predominantly due to recurrent B. cenocepacia infection, with the majority of affected recipients succumbing within 3 months after transplant. B. cepacia complex (BCC) sepsis is one of the defining criteria for cepacia syndrome, an almost universally fatal necrotizing pneumonic illness. We report 2 CF patients who were long-term survivors of B. cenocepacia sepsis after lung transplantation. The aim of this report is to demonstrate that, although survival of B. cenocepacia sepsis after lung transplantation is extremely uncommon, with aggressive multidisciplinary management, long-term survival remains a realistic objective.

Microbial contamination of non-invasive ventilation devices used by adults with cystic fibrosis.

BACKGROUND: There is currently little evidence regarding potential risks of bacterial contamination of non-invasive ventilation (NIV) devices used by cystic fibrosis (CF) patients. AIM: The aim of this study was to determine the extent of bacterial contamination of NIV devices in our regional adult CF centre. METHODS: Seven NIV devices recently used by CF patients chronically infected with Pseudomonas aeruginosa or Burkholderia cepacia complex (BCC) were swabbed in seven areas, both external and internal. Two devices had undergone ethylene oxide (EtO) sterilization between patient use and swabbing, and five devices had not undergone EtO sterilization. FINDINGS: Swabs from five devices had insignificant growth of environmental organisms and two devices had significant growth of environmental organisms. No CF pathogens were isolated from any machine. CONCLUSIONS: No evidence was found of pathogenic microbial contamination of NIV devices used by CF patients in this small study. We suggest that further studies examine for evidence of bacterial contamination of NIV devices and that this issue should be included in future CF infection control guidelines.