Ototoxicity is a recognised dose-related complication of aminoglycoside antibiotics. Predisposition to these ototoxic effects, even when levels are within the therapeutic range, is associated with an inherited mitochondrial DNA mutation known as m.1555A>G.1 2 We report a case of a 22-year-old woman with cystic fibrosis (CF), genotype delta F508 homozygous, with bilateral high frequency hearing loss. This was discovered when an audiogram (not previously performed) was requested for routine screening by our centre before commencement of nebulised TOBI (preservative-free pH-adjusted preparation of tobramycin for inhalation). She had a history of diabetes mellitus, exocrine pancreatic insufficiency and developed chronic Pseudomonas aeruginosa infection when aged 5 …

Data collection: A screening tool was developed by practitioners working at the Manchester Adult Cystic Fibrosis Centre. The tool was based on a review of the literature and clinical experience. It contained items thought to be associated with development of CFRD. A retrospective review of medical notes was conducted. Patients with an abnormal oral glucose tolerance test (OGTT) in 2005 or 2006 were compared with patients who had normal OGTT results in the same years using information from annual reviews to complete the screening tool.

Clinical practice guidelines recommend annual screening for neuropathy for all people with diabetes.Rapid and reliable sensory tests appropriate for the diagnosis of neuropathy in Type 1 (T1) and Type 2 diabetes -which have been shown to be predictors of foot complications -are established in routine diabetes practice.As yet the reliability of these tests in Cystic Fibrosis related diabetes (CFRD) is unknown.These sensory tests are not specific for diabetes neuropathy and may be affected by factors relevant to CF such as age, height and exposure to neurotoxic agents.The object of this study was to determine and explore any differences in the results of three simple sensory tests to establish validity when screening for neuropathy in patients with CFRD.Research design and Methods: Three simple sensory tests were performed: the 10-g Semmes-Weisnstein monofilament examination (SMWE), superficial pain sensation (SP) and vibration perception threshold (VPT) in 4 groups each of 30 subjects: CF patients, CFRD patients, T1 patients, and normal controls.Results: The groups were sex and age matched (p = 0.26) with matching of the diabetic groups for disease duration (T1 11.3 years, CFRD 10.1years).There was no significant difference between the groups for SMWE or VP.The control groups showed a tendency to higher values for SP when compared to CFRD patients (p = 0.066), however this was the least reproducible test.There were no differences between CF patients and controls for any of the tests Conclusion: The lack of a significant difference between the CF group and controls in all three tests suggests that abnormalities detected on the tests would be likely due to specific pathological processes rather than an inherent feature of CF.

Objective: As patients with cystic fibrosis (CF) are living longer, transfer from a pediatric to an adult clinic has become a more challenging issue.Despite increased availability of adult providers, approximately 25% of adults with CF receive care from a pediatrician (Anderson, Flume, Hardy, & Gray, 2002).This study assesses relationships between readiness to transfer and CF-related knowledge, parental distress, and executive and family functioning.Methods: Participants were patients (n = 50) from three pediatric CF centers in the United States (M age = 15.93 years).Patients completed measures of executive function and CF knowledge.Parents completed measures of demographics, family functioning, and parental distress.Patients, parents, and CF team members rated patients' readiness to transfer to adult clinic.Results: In a linear regression model, older age (b = 0.80, p < 0.001) and higher FEV 1 (b = 0.37, p < 0.001) were associated with physicians' ratings of greater readiness, accounting for 60% of the variance (F(3,34) = 16.48,p < 0.001).In separate analyses, worse family functioning (F(3,32) = 7.38, p < 0.001) and greater knowledge (F(3,38) = 10.94,p < 0.001) accounted for significant proportions in nurses' ratings, after controlling for age and health status.Conclusions: Older and healthier adolescents were rated as more ready to attend adult clinic.Surprisingly, worse family functioning in relation to transfer may have reflected increased adolescent independence and rebellion.Limitations included small sample size and cross-sectional design.Future studies should investigate longitudinal predictors of successful transition to adult care.

BackgroundA cost-effectiveness evaluation comparing home-based and hospital-based treatment with intravenous antibiotics for respiratory exacerbations in adults with cystic fibrosis (CF) has not been previously undertaken.MethodsThe study was conducted in a UK adult CF centre from a health service perspective. Clinical outcome and resource use data were obtained from a retrospective one-year study and combined with unit cost data in an incremental economic analysis. The primary outcome measure was percentage change in FEV1; “effectiveness” was defined as maintenance of baseline average FEV1 over the one-year study period.Results116 patients received 454 courses of intravenous antibiotics. At the end of 1 year, there had been a mean percentage decline in FEV1 compared with baseline average for home-treated patients but an improvement for hospital-treated patients (Tukey's HSD mean difference 10.1%, 95% CI 2.9 to 17.2, p=0.003). Treatment was deemed “effective” in more hospital (58.8%) than home (42.6%) patients. The cost of hospital treatment was higher than home treatment (mean difference £9005, 95% CI 3507 to 14,700, p<0.001). The mean ICER was £46,098 (2.5th and 97.5th percentiles −374,044 and 362,472).ConclusionsHospital treatment was more effective but more expensive than home treatment. Potential methods to improve outcome at home should be considered but these may have resource implications.

Objectives: This study aimed to produce valid patient-based UK National Health Service (NHS) costs for adults with cystic fibrosis to identify differences between hospital- and home-based treatments for infections.Methods: A costing study was carried out in adults with cystic fibrosis (CF) in the United Kingdom, who required intravenous antibiotic treatments for respiratory infections, administered either at home or in the hospital. The perspective was that of the NHS hospital trust. Data were collected retrospectively for each patient for 1 year using clinical records. Data were collected for 116 adults with CF between 2000 and 2001, when 42,382 treatment days (454 courses) of intravenous antibiotics were administered; 213 courses with intention-to-treat at home and 241 courses with intention-to-treat in the hospital. The mean length of a course was 15.3 days.Results: Patients who had >60 percent of courses at home over 1 year had a mean cost of £13,528, compared with £22,609 for patients who had >60 percent of courses in the hospital, and a mean cost of £19,927 for patients who had an equal mix of home and hospital care (p = .0001).Conclusions: The key cost-generating events in CF respiratory infections are hospital admissions. Future studies assessing costs should concentrate on factors affecting admissions, length of stay, staff input, and alternative methods of home-care provision, rather than marginal effects, such as using different antibiotics.

Background: Several studies have suggested that clinical outcomes in adults with cystic fibrosis (CF) are equivalent after home and hospital treatment with intravenous antibiotics, but these studies were small and selective and only considered one course of treatment. Methods: A retrospective longitudinal study was performed to compare the clinical outcome over a period of 1 year of all patients attending the Manchester Adult CF Unit who received intravenous antibiotics at home or in hospital. The primary outcome measure was percentage change in forced expiratory volume in 1 second (FEV1) at the end of the 1 year period. Baseline ‘‘best’’ and ‘‘average’’ FEV1 values were established for each patient for the year before the study. The secondary outcome measures were percentage changes in forced vital capacity (FVC) and body weight. Results: A total of 116 patients received 454 courses of intravenous antibiotics. At the end of 1 year there had been a mean percentage decline in FEV1 compared with the baseline ‘‘average’’ for patients treated mostly at home but an improvement in patients treated mostly in hospital (Tukey’s HSD mean difference 10.1%, 95% CI 2.9 to 17.2, p = 0.003). For all patients there was a mean percentage decline in FEV1 from the baseline ‘‘best’’ value. For each course of treatment the mean percentage improvements in FEV1 at the end of the course from the start of the course were significantly higher for patients treated in hospital than for those treated at home. Conclusions: Clinical outcome, as defined by spirometric parameters and body weight, was better after a course of treatment in hospital than after home treatment, and this benefit was maintained over 1 year of treatment. The results suggest that patients treated at home need closer supervision.

The harmful effects of smoking are now well documented. The UK figures are disturbing: although the prevalence of smoking for adults is falling, in children it has been steadily rising since 1992. Passive smoking has been implicated in lung cancer, heart disease and respiratory diseases. Children are most vulnerable to passive smoking, especially if they already have preexisting lung disease. Cigarette advertising has been implicated in the increasing incidence and prevalence of smoking, especially in children. The majority (96%) of 11?15- year-olds can recall cigarette advertising, and 56% can name one sport linked with cigarette advertising. There have been several papers looking at the incidence, prevalence and effects of active and passive smoking in the cystic fibrosis (CF) population. This paper reviews the current literature on active and passive smoking in cystic fibrosis and reports the findings of a recent questionnaire to adult CF patients attending the Manchester Unit. The survey details the levels of active and passive smoking in CF patients and their parents, the perceptions of how smoking affects their health, and provides recommendations for care and cessation.

AIMSTo evaluate the sensitivity and specificity of two selective media for the isolation of Burkholderia cepacia from sputum specimens in patients with cystic fibrosis (CF).METHODSIn total, 149 expectorated sputum specimens from 113 patients with CF (32 cepacia colonised patients and 81 non-cepacia colonised patients) attending three CF centres were examined for the presence of B cepacia on two selective media: (1) MAST selective agar, a commercially available selective medium widely used in the UK and (2) BCSA (B cepacia selective agar), a new medium recently described, which is used predominantly in North America.RESULTSBurkholderia cepacia was isolated from 53 of 149 (35.6%) specimens examined, representing 32 of 113 (28.3%) patients, using both the MAST and BCSA media. Growth was most rapid on BCSA with all (53 of 53) isolates detectable after 48 hours, compared with 50 of the 53 isolates on MAST agar, with the remaining three isolates detectable at five days. Twenty eight contaminants were identified on MAST agar and 13 on BCSA agar; mainly Alcaligenes xylosoxidans and yeast on MAST agar and Flavobacterium indologenes on BCSA medium. BCSA was equivalent to MAST agar in its ability to isolate B cepacia from patients with CF with a history of B cepacia infection.CONCLUSIONSThe increased selectivity and reduced time to detection of BCSA makes it an attractive alternative to MAST. However, its present limited commercial availability in the UK may delay its use in routine diagnostic laboratories because of complications with media preparation and quality control.

Eight patients with cystic fibrosis [CF] colonized with Pseudomonas aeruginosa (P. aeruginosa) had serial lung function, peripheral blood inflammatory markers, and serum IgG antibodies to Burkholderia cepacia (B. cepacia) lipopolysaccharide measured in the months preceding and following colonisation with B. cepacia.