Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis.

The improved survival in people with cystic fibrosis has led to an increasing number of patients reaching adulthood. This trend is likely to be maintained over the next decades, suggesting a need to increase the number of centres with expertise in the management of adult patients with cystic fibrosis. These centres should be capable of delivering multidisciplinary care addressing the complexity of the disease, in addition to addressing the psychological burden on patients and their families. Further issues that require attention are organ transplantation and end of life management.Lung disease in adults with cystic fibrosis drives most of the clinical care requirements, and major life-threatening complications, such as respiratory infection, respiratory failure, pneumothorax and haemoptysis, and the management of lung transplantation require expertise from trained respiratory physicians. The taskforce therefore strongly reccommends that medical leadership in multidisciplinary adult teams should be attributed to a respiratory physician adequately trained in cystic fibrosis management.The task force suggests the implementation of a core curriculum for trainees in adult respiratory medicine and the selection and accreditation of training centres that deliver postgraduate training to the standards of the HERMES programme.

Longitudinal association between lung function and health-related quality of life in cystic fibrosis.

BACKGROUND: Lung function is an important indicator of cystic fibrosis disease status and those with better forced expiratory volume in 1 s (FEV(1))% predicted have tended to report a better health-related quality of life (HRQoL) in cross-sectional studies. The relationship between lung function and HRQoL over time is unknown. This work assesses the natural progression of HRQoL reporting over many years and compares assessments across a whole decade and evaluates the relationship between lung function and HRQoL longitudinally. METHODS: Demographic (age, gender), clinical (FEV(1)% predicted, body mass index, diabetes, Burkholderia cepacia complex, intravenous access device and nutritional status) and HRQoL (Cystic Fibrosis Quality of Life Questionnaire) variables were obtained every 2 years over a 12-year period (seven time points from 1998 to 2010). RESULTS: HRQoL and lung function declined slowly over time and significant decade changes were observed for FEV(1)% predicted and the nine domains of the Cystic Fibrosis Quality of Life Questionnaire. The results of random coefficient modelling indicated that, at the population level, decreasing FEV(1)% predicted was associated with decreasing HRQoL after adjusting for confounding variables. However, the percentage of patients for whom a decrease in lung function was associated with a decrease in HRQoL differed according to the quality of life domain. CONCLUSIONS: HRQoL and FEV(1)% predicted decline slowly; nevertheless, a decrease in lung function predicted a decrease in HRQoL over time.

PmrB mutations promote polymyxin resistance of Pseudomonas aeruginosa isolated from colistin-treated cystic fibrosis patients.

Pseudomonas aeruginosa can develop resistance to polymyxin and other cationic antimicrobial peptides. Previous work has shown that mutations in the PmrAB and PhoPQ regulatory systems can confer low to moderate levels of colistin (polymyxin E) resistance in laboratory strains and clinical isolates of this organism (MICs of 8 to 64 mg/liter). To explore the role of PmrAB in high-level clinical polymyxin resistance, P. aeruginosa isolates from chronically colistin-treated cystic fibrosis patients, most with colistin MICs of >512 mg/liter, were analyzed. These cystic fibrosis isolates contained probable gain-of-function pmrB alleles that conferred polymyxin resistance to strains with a wild-type or pmrAB deletion background. Double mutant pmrB alleles that contained mutations in both the periplasmic and dimerization-phosphotransferase domains markedly augmented polymyxin resistance. Expression of mutant pmrB alleles induced transcription from the promoter of the arnB operon and stimulated addition of 4-amino-l-arabinose to lipid A, consistent with the known role of this lipid A modification in polymyxin resistance. For some highly polymyxin-resistant clinical isolates, repeated passage without antibiotic selection pressure resulted in loss of resistance, suggesting that secondary suppressors occur at a relatively high frequency and account for the instability of this phenotype. These results indicate that pmrB gain-of-function mutations can contribute to high-level polymyxin resistance in clinical strains of P. aeruginosa.

Enteral tube feeding for cystic fibrosis.

BACKGROUND: Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. OBJECTIVES: To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 03 September 2012.Date of the most recent hand search of PubMed and conference abstract books: 15 June 2012. SELECTION CRITERIA: All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. DATA COLLECTION AND ANALYSIS: Thirty-one trials were identified by the searches; however, none were eligible for inclusion in this review. MAIN RESULTS: There are no trials included in this review. AUTHORS' CONCLUSIONS: Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status necessitates it.

Can health-related quality of life predict survival in adults with cystic fibrosis?

RATIONALE: Advances in the management of cystic fibrosis have led to a significant improvement in survival, although marked differences between individuals are still observed. The value of patient-reported health-related quality of life scores in predicting survival in adults with cystic fibrosis is unknown. OBJECTIVES: To evaluate whether patient-reported health-related quality of life could predict survival in cystic fibrosis. METHODS: From 1996 to 1997 a consecutive series of 223 patients were recruited to evaluate the Cystic Fibrosis Quality of Life Questionnaire. Demographic (age, sex), clinical (FEV(1)% predicted, body mass index, diabetes, B. Cepacia complex, intravenous access device, nutritional and lung transplant status) and health-related quality of life variables were recorded (Cystic Fibrosis Quality of Life Questionnaire and the SF-36). These data were used as baseline measures to explore the prognostic association of health-related quality of life and subsequent survival. MEASUREMENTS AND MAIN RESULTS: At the census date (December 31, 2006) 154 (69.1%) adults were alive, 66 (29.6%) had died, and three (1.3%) were lost to follow-up. Cox proportional hazards models and bootstrapping procedures examined if health-related quality of life domains predicted survival after adjusting for the demographic and clinical factors. The physical functioning domain of the Cystic Fibrosis Quality of Life Questionnaire and the pain domain of the Short Form-36 had the strongest statistical associations with survival. CONCLUSIONS: Aspects of patient-reported quality of life serve as prognostic measures of survival beyond a number of previously known factors in cystic fibrosis. This needs to be investigated further in a larger longitudinal study.

Regimens for eradicating early Pseudomonas aeruginosa infection in children do not promote antibiotic resistance in this organism.

BACKGROUND: This study was designed to address the concern that intensive regimens to eradicate early Pseudomonas aeruginosa infection in patients with cystic fibrosis may lead to the emergence of antibiotic-resistant isolates. METHODS: Data was analysed retrospectively over a 10 year period at the Leeds Regional Paediatric Cystic Fibrosis Centre. All patients with first-ever isolation of P. aeruginosa who successfully completed an eradication regimen were included. Antibiotic sensitivities of P. aeruginosa were compared between initial and subsequent re-isolations in patients where eradication was successful and in those where treatment had failed. RESULTS: Forty one patients with first acquisitions for P. aeruginosa and who completed eradication treatment were identified. Eradication treatments consisted of oral, intravenous, nebulised antibiotics or a combination of these. The antibiotic sensitivity of P. aeruginosa in first growths was high and remained so on subsequent re-isolations. A repeated measures logistic model found no significant difference with time of isolation. There was no statistically significant difference in antibiotic sensitivity between P. aeruginosa isolated after successful and failed eradication. CONCLUSIONS: Repeated intensive regimens for P. aeruginosa eradication did not result in a significant increase in overall antibiotic resistance between initial and subsequent growths of this organism over the period of this study.

Decision-making about pregnancy for women with cystic fibrosis.

OBJECTIVES: This study aims to generate experiential data about the decision-making process encountered by 12 women with cystic fibrosis (CF) considering pregnancy. DESIGN: This study uses a qualitative grounded theory methodology to analyse the data and develop a conceptual hierarchical model of salient issues. METHODS: Twelve women completed a semi-structured interview accompanied by quantitative self-report measures of quality of life and psychological well-being, and indicators of health and treatment status were used. RESULTS: Descriptive data demonstrated that the sample did not differ significantly from other published samples. Four core categories emerged: (1) impact of the decision; (2) preparation for making and living with the decision; (3) owning the decision; and (4) personal dilemmas. Each comprised a number of overlapping conceptual and subcategories. Influencing factors included the impact of pregnancy on participants' health, and upon their ability to care for the child, the pregnancy experiences of other women with CF, the moral implications of having a child and the support and beliefs of family members. DISCUSSION: The findings extend the current knowledge in this area. We recommend development of guidelines for supporting and advising chronically ill young women about the psychosocial and emotional issues.

Clinical impact of reducing routine susceptibility testing in chronic Pseudomonas aeruginosa infections in cystic fibrosis.

BACKGROUND Susceptibility testing results are not predictive of clinical response to antibiotic therapy in chronic Pseudomonas aeruginosa infections in cystic fibrosis (CF). We assessed the impact of reducing the number of routine susceptibility tests performed on clinical outcome in these cases. METHODS In June 2006, we introduced a protocol whereby susceptibility tests of P. aeruginosa isolates obtained from respiratory samples of people with CF were limited to those taken at the commencement of antibiotic therapy, when there was evidence of clinical failure or routinely if not tested in the previous 3 months. At all other times, isolates were identified and reported as normal but P. aeruginosa isolates were not subjected to susceptibility testing. RESULTS Over a 6 month period, P. aeruginosa was isolated on at least one occasion from 193 patients attending the Adult Cystic Fibrosis Unit. In this period, we reduced the number of routine susceptibility tests by 56% (from a projected 2231 tests on 872 samples to an actual 972 tests on 427 samples). We assessed the response to courses of intravenous antibiotic treatment administered during the 6 month study period in 2006 and for courses administered in the same patients during the same calendar months in 2005. No significant differences in median change of FEV1, FVC, C-reactive protein (CRP), white cell count, weight or duration of intravenous antibiotics were observed. The projected savings of this intervention were 3500 euros in consumables and 170 h (costed at 6500 euros) of laboratory staff time per annum, a total annual saving of 10,000 euros (6500 pounds sterling). CONCLUSIONS For CF units sending regular, routine sputum samples, a reduction in the number of susceptibility tests performed in cases of chronic P. aeruginosa infection can be carried out without impacting on short-term clinical outcomes.

Serologic diagnosis of allergic bronchopulmonary aspergillosis in patients with cystic fibrosis through the detection of immunoglobulin G to Aspergillus fumigatus.

Allergic bronchopulmonary aspergillosis (ABPA) is seen in approximately 10% of patients with cystic fibrosis (CF) and can be difficult to diagnose. Consensus criteria require the presence of multiple elevated immunologic markers such as total immunoglobulin E (IgE), Aspergillus IgE and Aspergillus IgG, or precipitins for a robust diagnosis. There is some degree of standardization of total IgE and Aspergillus IgE levels, but there is no standardization in the measurement of IgG antibodies or precipitins to Aspergillus. The interpretation of results may, therefore, be confusing. Eighty-seven patients with CF were categorized as having ABPA or as controls, using the consensus criteria and an in-house enzyme immunoassay to measure IgG levels to Aspergillus. All sera from patients were then analyzed by commercial fluorescent immunoassay (FEIA) for the quantitative detection of anti-Aspergillus IgG. FEIA results were analyzed against the consensus conference minimum diagnostic criteria to ascertain a cutoff point, which could predict a diagnosis of ABPA in CF. Eighty patients with CF and with no or incomplete evidence of ABPA had a mean FEIA score of 51.1 mg/L, whereas 7 CF patients with ABPA had a mean FEIA score of 132.5 mg/L. Using receiver operator characteristic curve analysis of the ImmunoCAP (Phadia) IgG score on ABPA versus all other patients gave an area under the curve of 0.933 (estimated SE, 0.027). This analysis provisionally suggested that a score of 90 mg/L may be used as a cutoff point, which would give a sensitivity of 91% and specificity of 88.0% for the diagnosis of ABPA, though this requires further validation. This quantitative approach to Aspergillus IgG measurement in patients with CF along with the results of other tests will hopefully provide a more accurate approach to the diagnosis of ABPA.

A cross-sectional study of bone mineral density in children and adolescents attending a Cystic Fibrosis Centre.

BACKGROUND: Low bone mineral density is common in adults with cystic fibrosis. Children with good lung function compared to controls matched for body size have normal bone mineralisation. There are few data in large unselected populations of children. METHODS: All children between five and 16 years were invited to take part. Disease severity was assessed. Bone mineral measurements using a GE-Lunar Prodigy densitometer were expressed as age and gender matched Z-scores. Bone mineral apparent density for L2-L4 was estimated and data from UK Caucasian children used to create age and gender specific reference ranges for predicted values. Z-scores were calculated. Total body analysis utilised the Molgaard method. Blood was sampled for measurement of 25-hydroxyvitamin D, and parathyroid hormone levels. RESULTS: 107 children entered the study. 18 and 10 children had low areal and apparent bone mineral density respectively. Short, narrow bones were common. Fifteen children reported 22 fractures, 20 with associated trauma. The best predictors of bone status were ZBMI and percent predicted FEV(1). CONCLUSIONS: Bone mineral density corrected for body size was normal in over 90% of children. These results are similar to previously reported results in small studies of children with well preserved respiratory function.

Microvascular complications in patients with cystic fibrosis-related diabetes (CFRD).

BACKGROUND: This study aims to quantitatively and qualitatively assess microvascular complications and their risk factors in patients with cystic fibrosis-related diabetes (CFRD) compared to those with type 1 diabetes mellitus (DM1). METHODS: 79 patients with CFRD were matched with 79 patients with DM1 according to sex, age and duration of insulin therapy. Retinopathy, peripheral neuropathy, nephropathy and microalbuminuria were the microvascular complications assessed. Risk factors studied were: smoking, BMI, HbA1c, cholesterol, cholesterol/HDL ratio, diastolic and systolic blood pressure. RESULTS: Both groups had the same number of microvascular complications (29%). CFRD patients showed more microalbuminuria (21% versus 4.1%; p=0.003), while retinopathy was more common in patients with DM1 (24% versus 10%; p=0.044). The prevalences of peripheral neuropathy and nephropathy were similar. Patients with CFRD had lower BMI (p<0.0001), total cholesterol (p<0.0001) and HbA1c (p=0.056) levels, and a lower prevalence of smokers (p<0.0001). Cholesterol/HDL ratio and diastolic and systolic blood pressure were similar in both groups. CONCLUSIONS: The microvascular complications shown by patients with CFRD are similar to those seen in patients with DM1 but with a lower prevalence of retinopathy and a higher prevalence of microalbuminuria. The latter may reflect the influence of other cystic fibrosis-related factors on renal function.

Health-related quality of life in adults with cystic fibrosis: the role of coping.

OBJECTIVE: This study examined the role of coping in explaining health-related quality of life (HRQoL) in cystic fibrosis (CF). METHODS: Coping, quality of life, and clinical variables were assessed. The CF Coping Scale measures four ways of coping: optimistic acceptance, hopefulness, distraction, and avoidance. The Cystic Fibrosis Quality of Life Questionnaire (CFQoL) comprises 9 domains: physical, social, treatment, chest symptoms, emotional, body image, relationships, career, and future. Step-wise multiple regression and path analysis were used to model the associations between coping and CFQoL domains, taking into account important demographic and clinical factors. RESULTS: One hundred and sixteen people with CF were recruited to the study. Their mean FEV(1)% predicted and BMI were 59.3% and 21.2 kg/m(2), respectively. Mean scores were highest for optimism and lowest for distraction coping. Coping had an inequitable influence across the CFQoL domains: it had negligible influence on domains that incorporate symptoms and aspects of physical functioning but considerable influence on psychosocial domains. Optimism and distraction were strongly associated with emotional responses, social functioning, and interpersonal relationships. A high level of optimism was associated with a better HRQoL and high levels of distraction with a poorer HRQoL. For some domains, optimism and distraction had a counterbalancing effect so the difference between them could be an important predictor of HRQoL. CONCLUSION: Coping has emerged as an important factor in explaining some quality-of-life domains but not others. This has important implications especially when employing HRQoL as an outcome measure in clinical trials.

Therapeutic drug monitoring of once daily tobramycin in cystic fibrosis--caution with trough concentrations.

BACKGROUND: : Once daily intravenous aminoglycoside dosing (ODD) is widely used to treat acute Pseudomonas aeruginosa exacerbations in patients with cystic fibrosis. Controversy exists as to what is the most appropriate method of therapeutic drug monitoring (TDM) of such therapy with recommendations including trough plasma concentrations of <1 mg/L or <2 mg/L, area under curve (AUC) and various nomograms. This study aimed to compare the exposures to ODD of tobramycin in adults and children with cystic fibrosis using the AUC and trough TDM approaches. METHODS: : Using a mono-exponential software program to calculate AUC from 2 plasma concentrations, AUCs were determined in 22 adults with pre-dose tobramycin concentrations <1 mg/L. The exposure of 5 children with reduced tobramycin clearances was simulated at the usual recommended dose of 10 mg/kg/daily but retaining a trough <1 mg/L. RESULTS: : A tobramycin dose of 10 mg/kg of tobramycin in these patients with normal serum creatinine and a trough concentration <1 mg/L resulted in exposures in excess of those associated with conventional 8-hourly dosing. CONCLUSIONS: : The TDM approach of a trough <1 mg/L, as used with conventional 8-hourly tobramycin dosing, is not relevant to ODD.

Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis.

BACKGROUND & AIMS: Achieving and maintaining an ideal nutritional status is the primary aim of the nutritional management of cystic fibrosis (CF). It is unclear how nutritional interventions impact on patients' perceptions and behaviours concerning body image and eating. This work aimed to provide a psychosocial profile and compare CF patients receiving (a) enteral tube feeding, (b) nutritional supplements, (c) no nutritional interventions, and (d) healthy controls. METHODS: A cross-sectional questionnaire design was employed. Age, gender, lung function, and body mass index were recorded. Subjects completed measures of eating attitudes, perceived and desired body shape, body image, self-esteem and quality of life (QoL). RESULTS: A minority of CF patients reported disordered eating. Those receiving nutritional interventions engaged in less dieting behaviour. All CF groups, especially intervention groups, received more pressure from others to eat. For females, control groups desired to be slimmer whereas intervention groups desired to be heavier. Healthy males were content with their body whereas CF males wished to be heavier. Patients receiving enteral tube feeding were less satisfied with their body image, reported lower self-esteem and poorer QoL. CONCLUSION: Body image and eating behaviours are important considerations of nutritional interventions for maintaining QoL.

Nutritional intake and status in children with cystic fibrosis: does age matter?

OBJECTIVES: To determine whether nutritional intake and status vary with age in children with cystic fibrosis (CF). METHODS: Case-control study examining differences in nutritional parameters and intakes in 58 children with CF recruited from a regional centre (2000-2001) and 45 controls. Participants were divided into age groups of 5-8 years, 9-12 years and 13-16 years. Weight, height, body mass index and standard deviation scores were recorded. A 4-day food diary (51 CF, 31 controls) was calculated for macronutrients and micronutrients. RESULTS: Energy intakes (%EAR) increased with age (112%, 115% and 116%, respectively) and were significantly higher in children with CF than controls. Lower weight and growth trends were observed in children ages 5 to 8 years (NS). Weight gain and growth was normal in children with CF ages 9 to 12 years but declined at 13 to 16 years (weight z score -0.85 vs 0.68 P = 0.003, height z score -0.54 vs 0.53 P = 0.002, body mass index z score -0.72 vs 0.41 P = 0.03). Lung function was the most significant predictor of nutritional status at 9 to 12 years (r2 = 0.37, P = 0.006) and 13 to 16 years (r2 = 0.31, P = 0.01), but was not significant in children ages 5 to 8 years. CONCLUSION: Energy intakes increased with age in children with CF and exceeded that of healthy peers in all age groups. Weight gain and growth equaled that of healthy peers at 9 to 12 years but was suboptimal at 5 to 8 years and dramatically declines at 13 to 16 years. Energy intakes were unable to meet the clinical demands of children in these age groups. Both remain vulnerable and require greater nutritional targeting.

Aspergillus bronchitis in cystic fibrosis.

Aspergillus fumigatus, a widely distributed spore-bearing fungus, is commonly grown in sputum cultures of patients with cystic fibrosis (CF). A fumigatus may cause allergic bronchopulmonary aspergillosis (ABPA), a complex condition that leads to worsening of airway inflammation and progressive damage and is diagnosed by specific criteria. In this report, we present six CF patients with respiratory deterioration that did not respond to appropriate antibiotic treatment. All had had A fumigatus in sputum cultures but did not fulfill the criteria of ABPA. Treatment with antifungal agents was followed by improvement in clinical condition. We suggest that in patients with CF, A fumigatus should be considered as a pathogen that may directly cause respiratory exacerbations. Antifungal therapy should be considered when deteriorating respiratory function is not responding to antibacterial therapy and A fumigatus is growing in sputum cultures.

Diagnosis and treatment of intestinal malabsorption in cystic fibrosis.

Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes. Bicarbonate deficiency, abnormal bile salts, mucosal transport problems, motility differences, and anatomical structural changes are other contributory factors. Effective treatment should allow a normal to high-fat diet to be taken, control symptoms, correct malabsorption, and achieve a normal nutritional state and growth. Appropriate pancreatic enzyme replacement therapy will achieve normal or near-normal absorption in most people with cystic fibrosis. Early identification and treatment of intestinal malabsorption is critical to achieving optimal nutritional status. The occurrence of fibrosing colonopathy in a few patients on very high doses of those enzymes which have the copolymer Eudragit L30 D55 in their covering resulted in guidelines in the UK to avoid doses equivalent to more than 10,000 IU lipase per kg per day, and also to avoid preparations containing this copolymer in children and adolescents. For patients not responding to 10,000 IU lipase per kg per day review of adherence to treatment, change of enzyme preparation, variation in time of administration, and reduction in gastric acid may improve absorption. The importance of early investigation to exclude other gastrointestinal disorders as a cause of the patient's symptoms, rather than merely increasing the dose of enzymes, is stressed. With modern pancreatic enzymes in doses up to or only slightly in excess of 10,000 IU lipase per kg per day, adequate control of gastrointestinal symptoms and absorption can be achieved, and a normal nutritional state and growth rate maintained in most people with cystic fibrosis.

Risk factors for acquisition of methicillin-resistant Staphylococcus aureus (MRSA) by patients with cystic fibrosis.

Methicillin-resistant Staphylococcus aureus (MRSA) is an increasing problem for patients with cystic fibrosis (CF). It has been associated with clinical deterioration in some patients with CF, creates additional infection control problems, and may affect acceptance onto transplant waiting lists. Recent attempts to eradicate the organism have met with only moderate success. An understanding of those factors which increase the risk of acquisition of MRSA by CF patients will aid the development of effective preventative strategies. We conducted a retrospective case-control study comparing a variety of risk factors for 15 MRSA-positive patients and 30 age-sex-matched MRSA-negative controls who attended the Regional Paediatric or Regional Adult Cystic Fibrosis Units in Leeds. During the year prior to initial isolation, MRSA-positive CF patients spent more days in hospital (mean 19.8 days versus 5.5 days, p=0.0003), received more treatment days of oral ciprofloxacin (43.5 days versus 13.9 days, p=0.03) more treatment days of oral/intravenous cephalosporins (42.7 days versus 15.4 days, p=0.04) and were more likely to be chronically infected with Aspergillus fumigatus (40% versus 10%, p=0.04) than the age-sex-matched MRSA-negative controls. There were no significant differences in observed clinical parameters (clinical and X-ray scores) with between the two groups. Minimising the number and length of hospital admissions and judicious use of antibiotics, particularly ciprofloxacin, should be the key components of any strategies designed to reduce the risk of MRSA acquisition by patients with CF.

Associations between clinical variables and quality of life in adults with cystic fibrosis.

BACKGROUND: The disease progression of cystic fibrosis (CF) is marked by an increase in clinical conditions and therapeutic interventions, which have the potential to affect health-related quality of life (HRQoL). This cross-sectional study explored associations between clinical variables and HRQoL. METHODS: HRQoL was measured using the Cystic Fibrosis Quality of Life (CFQoL) questionnaire, which consists of nine domains: physical, social, treatment, chest symptoms, emotional functioning, concerns for the future, relationships, body image, and career concerns. The CFQoL was completed by 223 adults with CF. Clinical and demographic data collected were: age, gender, FEV1% predicted, BMI, Burkholderia cepacia status, lung transplant status, diabetic status, level of nutritional intervention, and presence of an intravenous access device. Multiple regression using forward selection was used to construct models relating these variables to each HRQoL domain. RESULTS: Despite many of the variables being inter-related, some variables were associated with CFQoL domains even in the presence of other important clinical factors. FEV1% predicted was weakly positively associated with all nine domains. Strong evidence emerged that patients who had received a lung transplant reported a higher HRQoL in physical and social functioning, chest symptoms, and treatment issues. Females tended to report a lower quality of life for chest symptoms and career issues, but higher values for body image. Patients with an access device expressed more career concerns. There was no evidence of an association between B. cepacia and any of the nine CFQoL domains. The model for the body image domain explained a high percentage of the variance (R2=30%): negative body image was associated with lower BMI, having an access device, diabetes, and enteral feeding. CONCLUSIONS: While important associations were identified, much of the variance in HRQoL remains unexplained. Other clinical and psychosocial variables merit investigation. A longitudinal study is required to investigate how the disease trajectory and associated treatments affect an individual's quality of life.