18 research outputs found

    Management of cystic fibrosis in adults

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    Person-centered medicine and chronic disease :the role of interdisciplinary education and treatment adherence

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    Rationale, aims and objectives: There is some evidence that a brief training program is effective in developing clinicians’ skills, knowledge and attitudes when dealing with non-adherence with medications. The purpose of this study was to implement and evaluate a one-day training program aimed at improving clinicians’ awareness and responsiveness to clients with poor treatment adherence. Method: Forty two participants completed a one-day training program. Using a workshop format, the program was focused on the use of motivational interviewing as a primary strategy which encouraged participants not only to listen but to understand the client’s perspective concerning treatment adherence. Several measures concerning treatment adherence knowledge, beliefs about medicines, therapeutic tasks, therapeutic optimism and satisfaction with training were used to assess the effectiveness of the program. These measures were applied immediately pre- and post-program and on follow-up at four weeks. Results: There was a significant increase in the adherence knowledge and skills of the participating clinicians’ post-program, which although reduced at one-month follow-up, was still above pre-program knowledge. The results also showed an increase in the therapeutic tasks. Conclusion: Promoting treatment adherence is a pivotal part of clinicians’ work in reducing the burden of chronic disease. These results suggest that a one-day program is effective in improving clinicians’ knowledge, attitude and skills regarding treatment adherence. However, further research is needed to measure sustained improvement of clinicians’ knowledge and the outcomes this may have on clients’ adherence

    Utility of thrombophilia testing in patients with venous thromboembolism

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    Background: Clinical practice of thrombophilia testing those with venous thrombo-embolism (VTE) in public hospitals may not be consistent with the international guidelines. This study aims to assess whether practice of thrombophilia testing in two public hospitals are consistent with international guidelines, and to assess whether certain groups of patients were more likely to benefit from testing

    Ivacaftor in severe cystic fibrosis lung disease and a G551D mutation

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    Ivacaftor is gene-specific oral therapy for patients with cystic fibrosis who have a cystic fibrosis transmembrane conductance regulator mutation, G551D. To date, limited information is available about the benefit in patients with severe CF related lung disease, as such patients were excluded from the phase III trials. We report the early results on clinical outcomes, sweat electrolytes and C-reactive protein in three adults with a G551D mutation and advanced lung disease. A mean increase of 6% in FEV1 was observed at 2 weeks and a mean reduction in sweat chloride of -48.9 mmol/L. While improvements in spirometry, weight gain and reduction in sweat electrolytes are similar with those reported in the phase III trials, a formal comparison was not performed

    Paraquat ingestion in an adult with cystic fibrosis (CF): Diagnostic and management dilemmas

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    Abstract N,N'‐dimethyl‐4,4'bipyridinium dichloride (Paraquat) is a potent herbicide used widely in agriculture. We report the effects of an ingestion of paraquat by a 28 year old male with cystic fibrosis and the diagnostic and management challenges this posed in both the acute and longer term setting. We describe the effects of direct paraquat toxicity on the lung tissue secondary to aspiration and review the long‐term sequelae of paraquat, namely osteonecrosis. Our case is the first to describe osteonecrosis of the knee in the context of paraquat toxicity. Survival following ingestion remains poor with a high associated mortality. However, timely treatment with NAC and immunosuppression may impact on survival. In those patients who do survive the acute phase post ingestion, follow‐up over years may be required to detect the long‐term effects of paraquat on bone health

    Diagnosis of the cause of chronic dyspnoea in primary and tertiary care: characterizing diagnostic confidence

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    Background: Chronic dyspnoea (breathlessness) in adults is a common symptom, the exact cause of which may be difficult to diagnose on initial presentation. We characterised the diagnostic complexity of chronic dyspnoea in primary care and tertiary care.Methods: This retrospective observational study screened consecutive referral letters of all adult patients referred to cardiology or respiratory clinics at a tertiary referral hospital, during May to June 2015. For patients referred due to chronic dyspnoea, data were analysed from the initial referral letter and subsequent specialist clinic letters for the 6-month time period after referral.Results: Of 1,370 patient referrals, 122 patients (mean age 63 y, 55% female) were referred due to chronic dyspnoea. One hundred and five patients (86%) were referred from primary care and 17 (14%) from other hospital clinics. Sixty-one percent were referred with an evident diagnosis (referrer was confident of the initial diagnosis), whereas 39% had non-evident or unclear diagnoses. By 6 months after referral, a definitive final diagnosis had been determined by the specialist clinics in 62% of patients. The majority of patients had been referred appropriately to a cardiology or respiratory clinic, based on the final diagnosis. However, only 26% of diagnoses were fully concordant between the initial referrer diagnosis and the final specialist clinic diagnosis, with 26% being partially concordant and 48% non-concordant. Diagnostic certainty of the referrer was associated with concordance of diagnosis between the referrer and specialist clinic (

    Diagnostic approach to chronic dyspnoea in adults

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    Chronic dyspnoea, or breathlessness for more than four weeks duration, is a common symptom in adults presenting to primary and tertiary care. It often presents a diagnostic challenge due to the wide spectrum of underlying disease, which is multifactorial in approximately one third of cases. Challenges in diagnosis include an often non-diagnostic clinical assessment, difficulty in selecting the most appropriate investigations and correct speciality referral for further diagnostic assessment. In patients presenting with chronic dyspnoea, history and physical examination are often non-specific with key findings more useful as negative predictive factors. There is a broad range of simple to specialised investigations that may be utilised in the diagnostic work-up. Several diagnostic algorithms incorporating different tiers of investigations have been tested in studies of chronic dyspnoea patients but there is currently very limited data that test a diagnostic algorithm against standard clinical care. In this review we propose a diagnostic pathway with primary, secondary and tertiary level investigations for patients with chronic dyspnoea. This pathway is based on the combination of previously tested diagnostic algorithms in the literature, to assist clinicians in their diagnostic workup of chronic dyspnoea patients. Further research is needed to further evaluate diagnostic algorithms in this setting and to test this diagnostic pathway in clinical practice

    Evaluation of an innovative mobile health programme for the self-management of chronic obstructive pulmonary disease (MH-COPD): Protocol of a randomised controlled trial

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    Introduction Chronic obstructive pulmonary disease (COPD) is the fourth leading cause of death globally. In outpatient care, the self-management of COPD is essential, but patient adherence to this remains suboptimal. The objective of this study is to examine whether an innovative mobile health (mHealth)-enabled care programme (MH-COPD) will improve the patient self-management and relevant health outcomes. Methods and analysis A prospective open randomised controlled trial has been designed. In the trial, patients with COPD will be recruited from The Prince Charles Hospital, Brisbane, Australia. They will then be randomised to participate in either the MH-COPD intervention group (n=50 patients), or usual care control group (UC-COPD) (n=50 patients) for 6 months. The MH-COPD programme has been designed to integrate an mHealth system within a clinical COPD care service. In the programme, participants will use a mHealth application at home to review educational videos, monitor COPD symptoms, use an electronic action plan, modify the risk factors of cigarette smoking and regular physical activity, and learn to use inhalers optimally. All participants will be assessed at baseline, 3 months and 6 months. The primary outcomes will be COPD symptoms and quality of life. The secondary outcomes will be patient adherence, physical activity, smoking cessation, use of COPD medicines, frequency of COPD exacerbations and hospital readmissions, and user experience of the mobile app. Ethics and dissemination The clinical trial has been approved by The Prince Charles Hospital Human Research Ethics Committee (HREC/16/QPCH/252). The recruitment and follow-up of the trial will be from January 2019 to December 2020. The study outcomes will be disseminated according to the Consolidated Standards of Reporting Trials statement through a journal publication, approximately 6 months after finishing data collection

    Superior vena cava obstruction due to total implantable venous access devices in cystic fibrosis: Case series and review

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    As the life expectancy of patients with cystic fibrosis (CF) improves, treatment related morbidity is increasingly recognised. Totally implantable venous access devices (TIVADs) offer reliable long term central venous access but are associated with recognised complications including venous thrombosis. Superior vena cava obstruction (SVCO) however has been rarely reported in this setting. We report a single CF centre's experience of SVCO associated with TIVADs in patients with CF, followed by a review of the published literature on risk factors, preventative strategies and treatment approaches
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