Retrospective cohort study of hypersomnias of central origin from two Australian tertiary sleep disorders services.

BACKGROUND: Hypersomnias of central origin (HOCO) are diverse in origin and symptomatology and remain poorly described in an Australian population. We hypothesised that the rate of human leukocyte antigen (HLA) DQB1*0602 positivity in the Australian cohort would be comparable to international registries. AIMS: The current study aims to evaluate epidemiological and clinical characteristics of Australian patients with HOCO, including prevalence of HLA DQB1*0602 positivity, the most specific HLA marker associated with narcolepsy. METHODS: This is a retrospective study. Patients ≥ 16 years of age presenting with symptoms of hypersomnolence who attended one of two Australian sleep centres (New South Wales and Queensland) in the preceding 24 months and had undergone both HLA serology and multiple sleep latency tests (MSLTs) were included. Main outcome measures included demographics, HLA DQB1*0602 positivity, MSLT, and clinical parameters (presence of auxiliary narcolepsy symptoms, laboratory tests, relevant prescribed medications). RESULTS: Eighty-eight patients were included. HLA DQB1*0602 positivity was highest in those with type 1 narcolepsy (NT1) (95.7%) and lowest in those without a classifiable disorder (9.1%). Mean sleep latency was lowest and number of sleep-onset rapid eye movement periods (SOREMPs) highest in the NT1 group. Comorbid disorders, particularly depression and overweight/obesity, were prevalent in all cohorts. Across all diagnostic groups, dexamphetamine was the most commonly prescribed agent for excessive daytime sleepiness. CONCLUSIONS: Patients with HOCO assessed in two specialised Australian clinics demonstrate comparable clinical characteristics to other published cohorts internationally; however, available pharmacological agents in Australia do not reflect international standards of care.

Outcomes of non-cystic fibrosis-related bronchiectasis post-lung transplantation.

BACKGROUND: Lung transplantation is a recognised treatment for end-stage lung disease due to bronchiectasis. Non-cystic fibrosis (CF) bronchiectasis and CF are often combined into one cohort; however, outcomes for non-CF bronchiectasis patients vary between centres, and in comparison with those for CF. AIMS: To compare lung transplantation mortality and morbidity of bronchiectasis (non-CF) patients with those with CF and other indications. METHODS: Retrospective analysis of patients undergoing lung transplantation between 1 January 2008 and 31 December 2013. Time to and cause of lung allograft loss was censored on 1 April 2018. A case-note review was conducted on a subgroup of 78 patients, to analyse hospital admissions as a marker of morbidity. RESULTS: A total of 341 patients underwent lung transplantation; 22 (6%) had bronchiectasis compared with 69 (20%) with CF. The 5-year survival for the bronchiectasis group was 32%, compared with CF (69%), obstructive lung disease (OLD) (64%), pulmonary hypertension (62%) and ILD (55%) (P = 0.008). Lung allograft loss due to chronic lung allograft dysfunction with predominant infection was significantly higher in the bronchiectasis group at 2 years. The rate of acute admissions was 2.24 higher in the bronchiectasis group when compared with OLD (P = 0.01). Patients with bronchiectasis spent 45.81 days in hospital per person year after transplantation compared with 18.21 days for CF. CONCLUSIONS: Bronchiectasis patients in the present study had a lower 5-year survival and poorer outcomes in comparison with other indications including CF. Bronchiectasis should be considered a separate entity to CF in survival analysis.

Home-Based Sleep Sensor Measurements in an Older Australian Population: Before and during a Pandemic.

Older adults are susceptible to poor night-time sleep, characterized by short sleep duration and high sleep disruptions (i.e., more frequent and longer awakenings). This study aimed to longitudinally and objectively assess the changes in sleep patterns of older Australians during the 2020 pandemic lockdown. A non-invasive mattress-based device, known as the EMFIT QS, was used to continuously monitor sleep in 31 older adults with an average age of 84 years old before (November 2019-February 2020) and during (March-May 2020) the COVID-19, a disease caused by a form of coronavirus, lockdown. Total sleep time, sleep onset latency, wake after sleep onset, sleep efficiency, time to bed, and time out of bed were measured across these two periods. Overall, there was no significant change in total sleep time; however, women had a significant increase in total sleep time (36 min), with a more than 30-min earlier bedtime. There was also no increase in wake after sleep onset and sleep onset latency. Sleep efficiency remained stable across the pandemic time course between 84-85%. While this sample size is small, these data provide reassurance that objective sleep measurement did not deteriorate through the pandemic in older community-dwelling Australians.

Safety of IV amikacin in the treatment of pulmonary non-tuberculous mycobacterial disease.

BACKGROUND AND OBJECTIVE: Pulmonary non-tuberculous mycobacterial (NTM) disease has a high mortality rate and often requires treatment with intravenous amikacin. We report on safety data in patients treated with intravenous amikacin for pulmonary. METHODS: A retrospective observational study (2002-2012) was performed including 45 patients that met American Thoracic Society criteria for pulmonary NTM disease and were treated with intravenous amikacin at three hospitals in Brisbane, Australia. The aim was to define the rates of common adverse effects, the patient and regimen factors associated with these adverse effects and describe the rates of treatment success and associated factors. RESULTS: Forty-five patients (34 women; median age 63 years) were treated for Mycobacterium intracellulare (25), Mycobacterium abscessus (13), Mycobacterium avium (6) and Mycobacterium fortuitum (1) using multi-drug therapy that included IV amikacin. Transient ototoxicity was seen in eight (18%) but long-term ototoxicity was seen in only three (7%). There were no cases of nephrotoxicity and no long-term vestibulotoxicity. Sustained culture conversion at 6 months was only found in 17 (38%), however, the majority (34 patients, 76%) had a clinical response to treatment determined by an improvement in symptoms. CONCLUSION: Carefully selected and closely monitored patients with pulmonary NTM can be treated using IV amikacin safely with low rates of toxicity. No pretreatment patient or regimen factors were predictive of toxicity or treatment success in this small cohort. Lower treatment success rates were found than previous trials suggest there is a difficult balance in this patient group between treatment success and toxicities.

Management of obstructive sleep apnoea in primary care.

BACKGROUND: Obstructive sleep apnoea (OSA) is common in the community and is increasing in prevalence. Primary care plays a pivotal role in the diagnosis and management of OSA. OBJECTIVE: This article focuses on the management options for a patient with an established diagnosis of OSA and provides a guide for driving licensing requirements. Indications for continuous positive airway pressure (CPAP) are discussed and tips provided to consider when conducting a review appointment, including trouble shooting. DISCUSSION: There are several treatment options available for patients with an established diagnosis of OSA. Selecting the optimal therapy involves aligning the symptoms and severity of OSA with the presence of comorbidities. CPAP is a highly effective therapy for symptomatic adults with moderate-to-severe OSA and for some symptomatic patients with mild OSA. Early trouble shooting of side effects and using supportive interventions increases the probability of long-term adherence, which is key to symptomatic improvement.

Prospective crossover trial of positional and CPAP therapy for the treatment of mild-to-moderate positional obstructive sleep apnea.

STUDY OBJECTIVES: To evaluate efficacy of vibrotactile positional therapy (PT) compared to standard CPAP therapy in mild-to-moderate positional obstructive sleep apnea (pOSA). METHODS: Prospective crossover randomized controlled trial of adult patients with treatment-naïve, symptomatic, mild-to-moderate pOSA - defined as ≥5 total apnea-hypopnea index (AHI) <30 with supine-to-non-supine (s:ns)AHI ratio ≥2. Participants were randomized to in-laboratory treatment initiation polysomnography with either PT or CPAP on sequential nights before an eight-week trial of each therapy. The primary endpoint was symptomatic improvement (Epworth Sleepiness Scale; ΔESS). Secondary endpoints included patient preference, usage, sleep architecture and quality of life (QoL) measures. RESULTS: 52 participants were enrolled and completed both arms of the study. Participants were symptomatic with median ESS 12 (IQR 10-14). Treatment resulted in a significant (p<0.001) symptomatic improvement with both PT and CPAP (ΔESS 4; IQR 6-11) without a significant difference between treatment arms (p=0.782). PT was effective at restricting supine sleep and demonstrated improved sleep efficiency compared with CPAP, although no better than baseline. Both therapies were effective at reducing AHI, although CPAP demonstrated superior AHI reduction. There were otherwise no clinically significant differences in sleep architecture, usage, or secondary outcomes including overall patient preference. CONCLUSIONS: In this cohort, treatment with PT or CPAP resulted in clinically significant symptomatic improvement (ΔESS) that was not significantly different between treatment arms. No real difference was seen in other secondary outcome measures. This study provides further evidence to support the use of PT as an alternative first-line therapy with CPAP in appropriately selected patients with pOSA. CLINICAL TRIAL REGISTRATION: Registry: Australian New Zealand Clinic Trials Registry; Name: Prospective crossover trial of Positional and Continuous positive airway pressure Therapy for the treatment of mild-to-moderate positional obstructive sleep apnoea; Identifier: ACTRN12619000475145; URL: https://anzctr.org.au/Trial/Registration/TrialReview.aspx?id=377221&isReview=true.

Australasian Sleep Association 2024 guidelines for sleep studies in adults.

Executive summary: This document is a consensus statement of a subcommittee of experienced sleep physicians and scientists, tasked to review the literature and formulate recommendations on the indications, performance, and reporting of sleep studies, to update clinical practice from the 2017 Australasian Sleep Association (ASA) guidelines for sleep studies in adults (Douglas JA, Chai-Coetzer CL, McEvoy D, et al. Guidelines for sleep studies in adults - a position statement of the Australasian Sleep Association. Sleep Med. 2017;36(Suppl 1):S2-S22. doi:10.1016/j.sleep.2017.03.019). This document moves the focus beyond important discussions outlined in the 2017 guidelines, particularly surrounding the sensitivity and specificity of validated questionnaires and home sleep studies. The 2024 guide outlines the performance of the broad range of sleep testing available for the investigations of sleep disorders in adults including indications, strengths, limitations, and reporting standards.

Health literacy assessment in the clinic: benefits, pitfalls and practicalities.

Approximately 60% of Australians have low or marginal health literacy, which is associated with poorer outcomes in patients with chronic disease. Patient-centred strategies (such as reduced medical jargon, use of pictograms, multimedia narratives) are effective in improving outcomes for many chronic diseases, with the impact being greatest in individuals with low health literacy. However, clinicians need a reliable and practical tool for assessing health literacy, the results of which help inform the choice of communication techniques best tailored to deliver information to patients. This article reviews the evidence of health literacy as an independent predictor of poor disease outcomes, describes feasible methods for assessing health literacy and presents communication strategies aimed at facilitating shared decision-making among those with low health literacy.

Randomized controlled trial on the efficacy of audio-visual health educational materials on CPAP adherence: the AHEAD trial.

STUDY OBJECTIVES: Educational interventions have been proposed to improve continuous positive airway pressure (CPAP) adherence; however, studies to date have not demonstrated robust effectiveness, due to methodological issues. Furthermore, these educational interventions have not specifically targeted low health literacy communication techniques, which have been demonstrated to improve outcomes in numerous other chronic diseases. We hypothesized that the addition of low-cost audio-visual educational videos (EVs) to usual standard-of-care (SC) education would improve CPAP adherence in adults with obstructive sleep apnea (OSA) syndrome. METHODS: At CPAP initiation, treatment-naïve adults with OSA syndrome were randomized to (1) usual SC or (2) SC + 5 EVs showing a patient's journey, designed with low health literacy communication techniques to teach patients about OSA and CPAP therapy. The primary endpoint was CPAP usage at 2 months (hours/night) and secondary endpoints were CPAP usage at 12 months and percentage of patients with ≥ 4 hours/night use at 2 months and 12 months. RESULTS: One hundred and ninety-five patients were randomized (SC 99, EV 96), with a mean age of 57 years (interquartile range [IQR] 44.1-64.8 years). There were no statistically significant differences in patient characteristics at baseline between the SC vs EV groups, with the diagnostic apnea-hypopnea index of 34 events/h (IQR 21-59 events/h) vs 30 events/h (IQR 20-50 events/h) and Epworth Sleepiness Scale score of 12.8 ± 6 vs 11.7 ± 5. At 2 months, there was no significant difference in hours of CPAP usage (SC: 3.45 hours/night [95% confidence interval (CI): 2.76 to 4.13] vs EV: 3.75 hours/night [95% CI: 3.14 to 4.37]) nor in proportion with adequate usage or overall commencement rate. However, at 12 months, there was a significant difference in hours of CPAP usage (SC: 2.50 hours/night [95% CI: 1.94 to 3.06] vs EV: 3.66 hours/night [95% CI: 2.92 to 4.40]). The probability of adequate CPAP usage at 12 months was higher in the intervention arm (odds ratio: 1.33; 95% CI: 1.04 to 1.7; P = .013). Patients with low education backgrounds benefitted substantially from the EV intervention compared with SC (mean difference at 12 months = 2.47 hours/night usage; 95% CI: 1.01 to 2.93; P < .01). CONCLUSIONS: Low health literacy-designed EVs improve CPAP adherence at 12 months compared with SC, with the greatest impact in patients with a low educational background. CLINICAL TRIAL REGISTRATION: Registry: Australian New Zealand Clinical Trials Registry; Name: Randomised controlled trial on the efficacy of audio-visual health educational materials on sleep health literacy and continuous positive airway pressure (CPAP) adherence in Sleep Clinic patients; URL: https://www.australianclinicaltrials.gov.au/anzctr/trial/ACTRN12619000523101; Identifier: ACTRN12619000523101. CITATION: Ellender CM, Samaranayake C, Reid N, et al. Randomized controlled trial on the efficacy of audio-visual health educational materials on CPAP adherence: the AHEAD trial. J Clin Sleep Med. 2022;18(11):2617-2625.

Adequate health literacy is associated with adherence to continuous positive airway pressure in adults with obstructive sleep apnea.

Study Objectives: Obstructive sleep apnea (OSA) is a chronic disease with significant health implications and adequate adherence to continuous positive airway pressure (CPAP) is essential for effective treatment. In many chronic diseases, health literacy has been found to predict treatment adherence and outcomes. In this study, the aim was to determine the health literacy of a sleep clinic population and evaluate the association between health literacy and CPAP adherence. Methods: A prospective cohort study was undertaken, recruiting 104 consecutive patients with a variety of sleep diagnoses. The Short Form Rapid Estimate of Adult Literacy in Medicine (REALM-SF), a validated questionnaire, was administered to measure health literacy. In a sub-group of 91 patients prescribed CPAP for OSA, CPAP usage was measured, with adequate usage defined as greater than 4 h/night CPAP therapy. Results: Seventy-one percent of the sleep clinic cohort was found to have adequate health literacy, as measured by the REALM-SF. In those prescribed CPAP for OSA, inadequate health literacy was associated with a twofold increased risk for inadequate CPAP usage (adjusted odds ratio [OR] 2.9, 95% CI: 1.1 to 8.22, p = 0.045). There was a 1.7 h/night difference in median CPAP usage comparing those with adequate to inadequate health literacy (4.6 h vs. 6.3 h/night). Conclusions: The majority of this sleep disorders cohort had adequate health literacy as measured by the REALM-SF questionnaire. However, inadequate health literacy appears to be an independent predictor of treatment adherence and may represent a modifiable risk factor of poor treatment outcomes in OSA.

Prospective cohort study to evaluate the accuracy of sleep measurement by consumer-grade smart devices compared with polysomnography in a sleep disorders population.

OBJECTIVES: Consumer-grade smart devices are now commonly used by the public to measure waking activity and sleep. However, the ability of these devices to accurately measure sleep in clinical populations warrants more examination. The aim of the present study was to assess the accuracy of three consumer-grade sleep monitors compared with gold standard polysomnography (PSG). DESIGN: A prospective cohort study was performed. SETTING: Adults undergoing PSG for investigation of a suspected sleep disorder. PARTICIPANTS: 54 sleep-clinic patients were assessed using three consumer-grade sleep monitors (Jawbone UP3, ResMed S+ and Beddit) in addition to PSG. OUTCOMES: Jawbone UP3, ResMed S+ and Beddit were compared with gold standard in-laboratory PSG on four major sleep parameters-total sleep time (TST), sleep onset latency (SOL), wake after sleep onset (WASO) and sleep efficiency (SE). RESULTS: The accelerometer Jawbone UP3 was found to overestimate TST by 28 min (limits of agreement, LOA=-100.23 to 157.37), with reasonable agreement compared with gold standard for TST, WASO and SE. The doppler radar ResMed S+ device underestimated TST by 34 min (LOA=-257.06 to 188.34) and had poor absolute agreement compared with PSG for TST, SOL and SE. The mattress device, Beddit underestimated TST by 53 min (LOA=-238.79 to 132) on average and poor reliability compared with PSG for all measures except TST. High device synchronisation failure occurred, with 20% of recordings incomplete due to Bluetooth drop out and recording loss. CONCLUSION: Poor to moderate agreement was found between PSG and each of the tested devices, however, Jawbone UP3 had relatively better absolute agreement than other devices in sleep measurements compared with PSG. Consumer grade devices assessed do not have strong enough agreement with gold standard measurement to replace clinical evaluation and PSG sleep testing. The models tested here have been superseded and newer models may have increase accuracy and thus potentially powerful patient engagement tools for long-term sleep measurement.

Cholecystectomy under thoracic epidural anaesthesia for patient with severe intra-pulmonary shunt.

Pulmonary arteriovenous malformations are common in patients with hereditary haemorrhagic telangiectasia and can be associated with significant hypoxia and intra-pulmonary shunt. We present a case of a young man with a known 57% calculated shunt requiring abdominal surgery and the multidisciplinary decisions required in the preoperative period to minimize post-operative complications.

Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis.

This case reports the successful management of chylothorax in a non-transplanted patient with pulmonary lymphangioleiomyomatosis (pLAM). Prolonged initial therapy failed, including total parenteral nutrition, pleural drainage, surgical pleurodesis, and pleurectomy. Commencement of sirolimus 2 mg daily (2 mg alternating days had failed) led to resolution of chylothorax after 20 days. Discontinuation of sirolimus for abdominal surgery led to recurrence of the chylothorax. Reinstitution of sirolimus led to rapid resolution of the effusion, stabilization of lung function, and there has been no recurrence in the ensuing 4 years. We conclude that sirolimus should be considered in the management of pLAM-related chylothorax, perhaps before surgical intervention.

Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension.

A 33-year-old woman with past history of Sjögren's syndrome and systemic lupus erythematosus presented with dyspnea and syncope secondary to pulmonary hypertension. After progressive symptoms over 4 years, she received bilateral lung transplantation. Histopathology of the explanted lungs showed isolated pulmonary amyloid light-chain amyloidosis and pulmonary cysts. No evidence of systemic amyloidosis was found at the time of transplantation. Seven years post lung transplantation, she remains well with no evidence of systemic amyloidosis recurrence.