The clinical journey of patients with a severe exacerbation of chronic obstructive pulmonary disease (COPD): from the ambulance to the emergency department to the hospital ward.

Background: Exacerbations of chronic obstructive pulmonary disease (COPD) are acute complications that often require emergency management by ambulance, emergency department (ED) and hospital services. Given the high mortality and morbidity of exacerbations, better understanding of the epidemiology of patients with COPD presenting to EDs is needed, as well as identification of predictive factors for adverse outcomes from exacerbations. Methods: This retrospective observational study involved patients who presented to an ED in the state of Queensland and received either an ED or hospital diagnosis of COPD in 2015 and 2016. Administrative data from ambulance, ED, hospital and death registry databases were linked to provide a comprehensive picture of the emergency healthcare pathway for these patients. Results: A total of 16,166 patients (49% female, 51% male) had 29,332 presentations to an ED in Queensland and received either an ED or hospital principal diagnosis of COPD during 2015 and 2016. These patients had a significant comorbidity burden with 54% having two or more comorbidities. Sixty-nine percent of ED presentations involved ambulance transport, and most of these (74%) involved administration of oxygen therapy and/or other medications by paramedics. Prehospital oxygen administration and ≥10 comorbidities were associated with >1 admission [odds ratio (OR) 1.3, 95% confidence interval (CI) 1.1-1.5; OR 4.3, 95% CI: 3.1-5.8, respectively], greater than average lengths of stay (OR 1.5, 95% CI: 1.3-1.6; OR 22.1, 95% CI: 18.1-27.2) and mortality (OR 1.6, 95% CI: 1.5-1.8; OR 5.3, 95% CI: 4.2-6.8). Of the ambulance presentations, 90% were admitted or received ongoing care. Conclusions: COPD places considerable burden on the emergency healthcare pathway including ambulances and EDs in Queensland. Patients with COPD most commonly present to the ED by ambulance and receive extensive pre-hospital management. These patients have significant comorbidity burden and experience high rates of admission and mortality. More research is required to investigate the emergency pathway to further identify reversible factors and enhance healthcare practice and policy for COPD management.

Diagnostic approach to chronic dyspnoea in adults.

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 workup. 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.

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

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 (P<0.001). CONCLUSIONS: In adult patients referred due to chronic dyspnoea to hospital specialist clinics, there is considerable diagnostic uncertainty about its aetiology, for both referring doctors and specialist clinics. These results demonstrate the current difficulty in diagnosing the cause of chronic dyspnoea in adults, and highlight the need for evidence-based diagnostic pathways.

Utility of thrombophilia testing in patients with venous thrombo-embolism.

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. METHODS: A retrospective audit on patients who presented to two Queensland public hospitals from August 2011 to September 2012 with VTE. Data were collected on demographics, yield of the test, and whether the result of the test changed the duration of anticoagulation. Group analysis was performed to identify patients who were more likely to yield positive results. RESULTS: Of the 152 patients, 49% were tested for thrombophilia, of whom 31% returned a positive result. 38% of patients with provoked VTE were tested for thrombophilia, inconsistent with guideline recommendations. In 1.2% of cases there were documented changes to duration of anticoagulation with positive results. The rates of positive results were 45% in unprovoked VTE cases compared with 29% in provoked VTE cases (P=0.054). The rates of positive results were 52% in recurrent VTE cases compared with 27% in those cases with first episode of VTE (P=0.007). CONCLUSIONS: The practice of thrombophilia testing in public hospitals was frequently inconsistent with guidelines, and did not significantly influence clinical decisions. There was higher yield of testing in patients with recurrent episodes of VTE and possibly in patients with unprovoked VTE.

Ivacaftor in severe cystic fibrosis lung disease and a G551D mutation.

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.

Nebulised dornase alpha: adherence in adults with cystic fibrosis.

BACKGROUND: Dornase alpha improves sputum clearance and pulmonary function in patients with moderate bronchiectasis and cystic fibrosis (CF). Several studies have demonstrated variable adherence to treatment regimens in patients with CF. We aimed to compare self-reported adherence with computer based dispensing records of dornase alpha. METHOD: Direct interview of adherence to dornase alpha was compared with pharmacy-dispensing records during a 12-month period. The reasons for dornase alpha usage, and change in lung function in three groups of patients were compared (group 1 (n=42): positive trial, continuing therapy; group 2 (n=13): positive trial, ceased therapy; group 3 (n=22): negative trial). Ninety patients with CF were surveyed. Patients who had ceased dornase alpha provided reasons for drug cessation. Lung function and anthropometric data were collected prior to dornase alpha treatment and at 3 years. Health care professionals rated adherence to therapy. RESULTS: Of the 42 patients on dornase alpha, the average utilisation was 197 days (54%). From dispensing records, 24% of patients were classified as good adherence, 46% as moderate adherence and 30% as poor adherence. Eighty-two percent claimed to use dornase alpha therapy more than 20 days/month, whereas dispensing records showed that only 24% of collected sufficient drug supplies. Health professionals' assessment of adherence of therapy was often incorrect and an overestimation was more common than underestimation. The change in FEV(1) 3 years after trial was not significantly different between groups. There was a negative correlation between initial response to dornase alpha and baseline FEV(1) (r=-0.44, P=0.001), however, initial response was not related to the change in FEV(1) over 3 years (r=0.20, P=0.16). CONCLUSION: There is variability in adherence to dornase alpha in adults with CF and patient self-reporting is likely to over-estimate drug collection from the pharmacy. Further study of the long-term effects of dornase alpha is required. To date, proven interventions to enhance adherence are limited and require investigation in CF.