BACKGROUND:It is important to monitor health related quality of life in order to determine the efficacy of interventions and physical functioning of patients with cystic fibrosis in their daily activities. There is no a single-item global quality of life scale for routine clinical practice for adult patients with cystic fibrosis. We assessed the reliability and validity of a single-item global quality of life scale and compared with the Cystic Fibrosis Quality of Life Questionnaire (CF-QOL) for adult patients with cystic fibrosis. METHOD:121 (men = 66, women = 55) adult cystic fibrosis patients self-completed the CF-QOL, the Hospital Anxiety Depression Scale, and the single item global quality of life scale at the out patient clinic. 33 (17 women) completed the repeat questionnaires at home within two weeks. Socio-demographic characteristic and lung function data were extracted from the recent medical notes. RESULTS:Mean (SD) age was 29.6 (8.9) years and mean (SD) forced expiratory volume in 1 second was 2.20 (0.94) litres. The test-retest reproducibility using the intra-class correlation coefficient (ICC) for the CF-QOL was 0.83, 95% confidence interval 0.68 to 0.91. The single item global quality of life ICC score was 0.78, 95% confidence interval 0.59 to 0.88. Concurrent validity of the single-item global quality of life was examined in relation to all items of the CF-QOL, frequent episodes of readmission, anxiety and depression (all, p < 0.01) were moderately correlated. CONCLUSION:The study provides preliminary evidence that the single-item quality of life scale is acceptable, valid and repeatable for adult patients with cystic fibrosis. It is a promising tool that can be easily incorporated into a routine clinical practice to assess patients' quality of life.

BACKGROUND:The impact of anxiety and depression on quality of life (QOL) in adult patients with cystic fibrosis (CF) is fully unknown. We investigated the prevalence and factors associated with anxiety and depression, including QOL, in adult CF patients. METHODS:One hundred twenty-one adult CF subjects, age ≥ 18 years were recruited from our out-patient clinic. Participants self-completed the Hospital Anxiety Depression Scale and the Cystic Fibrosis Quality of Life Questionnaire (CF-QOL). Socio-demographic data and values for lung function were extracted from the medical notes. RESULTS:Mean ± SD age was 30 ± 8.8 years, and age ranged 18-70 years. Forty (33%) were identified with anxiety symptoms, 20 (17%) with depressive symptoms. Factors related with depression were impaired QOL and low lung function. Anxiety was associated with difficulty in interpersonal relationships and severity of chest symptoms. The CF-QOL sub-domains (physical functioning, social functioning, treatment issues, chest symptoms, emotional functioning, concerns for the future, interpersonal relationships, body image, future/career concerns, and total CF-QOL) were all significantly correlated with anxiety (P < .001) and with depression (P < .001), respectively. CONCLUSIONS:Anxiety and depressive symptoms are common in adult CF patients. They are associated with poorer QOL, low lung function, reduced physical functioning, and severity of chest symptoms. Therefore, routine screening for symptoms of anxiety and depression is a worthy endeavor, and those identified with elevated clinical symptoms should be referred to receive appropriate treatment.

Background: The telehealth service is one of the fastest growing healthcare segments. It is increasingly utilizing computer technology and telecommunication equipment to either provide continuous vital sign monitoring or facilitate patient care at home, rather than relying solely on in-person care. Methods: We conducted a 6-week open study in nineteen patients with cystic fibrosis enrolled from three centers, to investigate patient perception of a telehealth enabled nebulizer system (Prodose Adaptive Aerosol Delivery [AAD] System), which enabled the doorstep delivery of repeat medication. Results: The results showed that patient confidence in the device and perception of ease of use was high with no significant change between the start and end of the trial. Views on the home delivery of medication were split between ‘great’ and ‘inconvenient.’ However, if the delivery system had been more flexible and delivered all the patients' drugs, the majority of patients would have had their medication delivered in this way. Conclusions: The trial showed that it was possible to build telehealth technology into an advanced nebulizer system, and that patient acceptance of the technology was unlikely to be a barrier to the adoption of such a telehealth system.

Discharge preparation is a multiprofessional activity that is not always conducted efficiently. Perspectives of staff provide an insight into challenges and solutions for this organizational process. The study aimed to examine current discharge practice in one hospital and to compare perceptions of this activity between healthcare workers from different professions. A questionnaire was sent to 1344 staff in an English hospital. It consisted of items related to discharge, which were rated on a Likert scale, and included space for free text. Data were analyzed using descriptive and inferential statistics. The survey method has a number of limitations and conclusions must be considered with caution. A total of 455 usable questionnaires were returned from nurses/midwives, doctors and therapists/allied health professionals. Although respondents showed agreement on a number of areas, differences did arise, which could be linked, in part, to values and roles associated with specific healthcare disciplines. Tensions between professional groups were evident, especially between social workers, nurses and medics. Differences of opinion also appeared between practitioners, patients and carers. Different views among practitioners of discharge preparation could impede efficient joint working. Better understanding of the roles and expertise of team members might improve this process. Factors affecting discharge preparation need to be understood to reduce staff and consumer dissatisfaction and to avoid potential readmissions.

BACKGROUND:Although there is now compelling evidence for cross-infection by strains of Pseudomonas aeruginosa at some specialist (cystic fibrosis [CF]) centers, the clinical impact of infection by transmissible strains is unclear. METHODS:In an 8-year prospective study, we compared the clinical outcome of two groups of patients with CF infected by transmissible (n = 28) and sporadic strains (n = 52) of P aeruginosa. RESULTS:There were no differences between the two groups in survival, annual changes in spirometry, or BMI. There were differences in requirements for IV antibiotic treatment (mean [SD]: 29.3 [21.9] days vs 53.1 [32.5] days) and hospitalization (median [range]: 11.6 [1.1, 49.3] days vs 23.3 [5.5, 103.6] days) between patients infected with sporadic and transmissible strains of P aeruginosa, respectively. CONCLUSIONS:We conclude that infection by transmissible P aeruginosa does not increase mortality but is associated with increased health-care and antibiotic use for patients with CF.

AIMS To understand the perspective of hospital-based health professionals with regard to preparing patients for discharge from an acute hospital in England. BACKGROUND The hospital experience in England over recent years is characterised by increasing admission rates and decreasing length of stay. Legislation and policy initiatives have also focussed upon the need to reduce delayed discharges. Discharge preparation is known to be a complex intervention with multiple obstacles within and outside of the hospital setting. DESIGN Qualitative. METHODS Posters were displayed within a hospital asking health professionals to take part in a focus group. Maximum variation, in terms of job titles, was sought for within the sample. Focus groups were held in December 2006. Six senior members of staff divided into pairs to run them. All groups were taped and transcribed verbatim and analysed using a framework approach. RESULTS Three focus groups were conducted, which involved 11 nurses, 15 allied health professionals, five social workers and one doctor. Analysis identified the following themes and sub themes: 1 Conflicting pressures on staff: Keeping patients in hospital vs. getting them out; Striving for flexibility within a system; A paucity of intermediary provision. 2 Casualties arising from conflicting pressures: Professionals losing their sense of professionalism; Patients being 'systematised'. CONCLUSIONS Pressures described during focus groups stemmed from five main sources: external targets placed upon the system, internal hospital inflexibility and poor communication, the dominance of the medical model of care, a desire to address the complex needs of individuals and a lack of community services. Staff felt themselves to be victims of these competing pressures and that many of the solutions were beyond their influence. Staff described the dehumanising effect of sometimes having to ignore patient concerns, wishes and choices. RELEVANCE TO CLINICAL PRACTICE Understanding of the pressures surrounding discharge could inform relevant service improvements.

Aims. To understand the perspective of hospital-based health professionals with regard to preparing patients

Aims and objectives. To explore patients' responses to developing and managing cystic fibrosis-related diabetes and to contrast their views with those of individuals with type 1 diabetes mellitus.Background. The incidence of diabetes among people with cystic fibrosis has increased with improvement in life expectancy. However, little is known about how patients respond to and manage cystic fibrosis-related diabetes, and how this compares with people living with type 1 diabetes mellitus.Design. Qualitative research was undertaken in order to fully explore meanings and views.Methods. Semi-structured telephone or face-to-face interviews were conducted with patients who had cystic fibrosis-related diabetes or type 1 diabetes mellitus, during which, they discussed diagnosis and management of diabetes. Framework analysis was employed to identify themes and to consider similarities and differences between the two groups.Results. Eleven cystic fibrosis-related diabetes and 12 type 1 diabetes mellitus patients were interviewed in 2006. Patients with cystic fibrosis-related diabetes described their diabetes diagnosis as a progression of their primary illness, management of which was important owing to the benefits it brought to their cystic fibrosis. Those with type 1 diabetes mellitus were more likely to report feeling psychologically low because of diabetes and to list long-term complications as a key factor motivating self-management. Both groups struggled to balance the demands of diabetes with other life and health obligations, and experienced isolation because of diabetes.Conclusions. Variation in perceptions recalled during interviews stemmed from diabetes being part of an existing life-threatening chronic illness in people with cystic fibrosis-related diabetes. Similarities and differences in attitudes and management practices were found, with less urgency regarding glucose monitoring and fewer information resources available for those with cystic fibrosis-related diabetes.Relevance to clinical practice. Both groups need support for optimal diabetes management and access to appropriate resources outside specialist clinics. Web-based technologies could prove useful for those with cystic fibrosis-related diabetes as face-to-face interaction may be prevented owing to the risk of cross-infection.

Objectives: To compare patients with type 1 diabetes mellitus (T1DM) or cystic fibrosis-related diabetes (CFRD) on frequency and severity of symptoms suggestive of hypoglycaemia and diabetes-specific health-related quality of life (HRQoL). Methods: This was a cross-sectional comparison study. Participants with T1DM or CFRD completed a questionnaire that assessed symptoms suggestive of hypoglycaemia and diabetes-specific HRQoL, and recorded episodes of low blood sugars in a prospective diary. Comparisons of responses were conducted, and Pearson product correlations were determined between HRQoL scores and other continuous variables. HRQoL scores were compared between the groups after adjusting for confounding factors. Results: Diabetes in cystic fibrosis (CF) patients had a less negative impact on HRQoL than in T1DM patients. Both groups experienced hypoglycaemia, but loss of consciousness or needing help was more common in T1DM patients. Symptoms suggestive of hypoglycaemia were less of a problem for CFRD patients in terms of severity, with T1DM patients having more neuroglycopenic symptoms. Discussion: Although the same percentage of patients in both groups reported experiencing hypoglycaemia, severity (e.g. symptoms and loss of consciousness) was higher for those with T1DM. Symptoms of hypoglycaemia appear to have a significant impact on HRQoL, and could account for the worse HRQoL in T1DM patients.

BackgroundThis study reviews the impact of changing infection control practices at the Manchester Adult Cystic Fibrosis Centre (MACFC) upon the epidemiology of Burkholderia species infections.MethodsWe reviewed strain and genomovar typing of all available Burkholderia isolates at our centre between 1983–2006.ResultsThe incidence/prevalence of infection with Burkholderia species between 1983–1990 was below 5%/9% each year. There was a rise in incidence/prevalence of Burkholderia species between 1991 and 1994 with a peak of 16.3%/31.2% in 1992. Following complete cohort segregation, the incidence has fallen to below 3% for all but one year and the prevalence has gradually reduced to 9.3% in 2005. Currently, there is an increase in the prevalence to 10.6% for the first time since 1994, predominantly due to patients with unique infections transferring into the unit from referring centres. The presence of unique strains now exceeds transmissible strains for the first time since 1991.ConclusionsInfection control measures including patient segregation have controlled spread of transmissible B. cenocepacia strains, but not the acquisition of unique strains. Unique strains of Burkholderia species now account for the majority of new infections at the Manchester Adult Cystic Fibrosis Centre.

Changes in the provision and availability of oxygen for exercise, travel and in-flight, highlight the need for healthcare professionals to provide comprehensive oxygen assessment and prescription services. For patients, access to new portable equipment, and the ability to travel at home and abroad, may no longer be considered a luxury, and the provision of suitable systems may improve quality of life. This paper aims to provide an overview of the most important practical issues for assessment and provision of ambulatory oxygen and oxygen for travel. This should be incorporated into an evidence-based oxygen assessment service for adults with respiratory conditions, and may result in more efficient use and prescription of oxygen.

"Cystic fibrosis: practical progress and current controversy." Disability and Rehabilitation, 20(6-7), p. 201 Additional informationNotes on contributorsKevin WebbIt is with great sadness that we have to inform the readership of the Journal that shortly after completing the final editorial work on this Special Issue, Professor Kalman Jacob Mann was seriously injured in a car accident and subsequently died.Professor Mann was responsible for establishing the two Hadassah Hospitals and Community Health Centres in Jerusalem and for the past 20 years headed the Presidium of Yad Sarah, Israel's largest community based, volunteer operated organization which provides a spectrum of free or nominal cost home care services nation-wide.We offer our condolences to his family and friends, and trust that this Special Issue stands as a testament to his work in the field.