Ruth Dentice

An investigation into the quality of life of individuals after laryngectomy.

Little longitudinal data are available on the general physical, psychological, and social problems experienced as a result of laryngectomy or on the preoperative status of patients in regard to these aspects. In particular, prospective longitudinal data gathered from the same group of subjects over time and examining a variety of such outcomes is rare. This descriptive study addresses these issues.

A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis

Background The mucoactive effects of hypertonic saline should promote exacerbation resolution in people with cystic fibrosis (CF). Objectives To determine the effects of hypertonic saline inhalation during hospitalisation for exacerbation of CF on length of stay, lung function, symptoms, oxygenation, exercise tolerance, quality of life, bacterial load and time to next hospitalisation. Methods 132 adults with an exacerbation of CF were randomised to inhale three nebulised doses a day of either 4 mL 7% saline or a taste-masked control of 0.12% saline, throughout the hospital admission. The primary outcome measure was length of hospital stay. Results All participants tolerated their allocated saline solution. There was no significant difference in length of stay, which was 12 days in the hypertonic saline group and 13 days in controls, with a mean between-group difference (MD) of 1 day (95% CI 0 to 2). The likelihood of regaining pre-exacerbation FEV1 by discharge was significantly higher in the hypertonic saline group (75% vs 57%), and the number needed to treat was 6 (95% CI 3 to 65). On a 0–100 scale, the hypertonic saline group had significantly greater reduction in symptom severity than the control group at discharge in sleep (MD=13, 95% CI 4 to 23), congestion (MD=10, 95% CI 3 to 18) and dyspnoea (MD=8, 95% CI 1 to 16). No significant difference in time to next hospitalisation for a pulmonary exacerbation was detected between groups (HR=0.86 (CI 0.57 to 1.30), p=0.13). Other outcomes did not significantly differ. Conclusions Addition of hypertonic saline to the management of a CF exacerbation did not reduce the length of hospital stay. Hypertonic saline speeds the resolution of exacerbation symptoms and allows patients to leave hospital with greater symptom resolution. Trial registration number ACTRN12605000780651.

Pregnancy outcomes in the current era of cystic fibrosis care: a 15-year experience.

Background:  With improvement in clinical care and longer survival of patients with cystic fibrosis (CF), pregnancy has become commonplace. However, the impact of pregnancy on maternal health and fetal outcomes requires ongoing review.

Recombinant Activated Factor VII for Massive Hemoptysis in Patients With Cystic Fibrosis

Massive hemoptysis is a common complication in patients with cystic fibrosis (CF) and is associated with significant morbidity and mortality. Conventional treatment with antibiotic therapy and early bronchial artery embolization (BAE) is usually successful in achieving hemostasis in the majority of patients. Recombinant activated factor VII (rFVIIa), originally developed for use in patients with hemophilia, has emerged as a general hemostatic agent that is potentially useful in the management of many life-threatening bleeding conditions. In this article, we present four patients with CF lung disease and massive hemoptysis who were treated successfully with rFVIIa. We suggest that in patients with CF who present with massive hemoptysis, the use of rFVIIa can be considered in patients with refractory hemoptysis despite conventional therapy or as a temporizing therapy when BAE is not immediately available.

Adults with cystic fibrosis prefer hypertonic saline before or during airway clearance techniques: a randomised crossover trial

QUESTION Among adults with cystic fibrosis, does the timing of hypertonic saline relative to airway clearance techniques affect lung function, perceived efficacy, tolerability, or satisfaction with the entire airway clearance regimen, and is the preferred timing regimen stable over time? DESIGN A randomised crossover trial with concealed allocation, intention-to-treat analysis, and blinded assessors. PARTICIPANTS 50 adults with cystic fibrosis and stable lung function at the end of a hospital admission. INTERVENTION Participants performed 3 sessions of airway clearance techniques per day for 3 days. On each day, participants were randomised to inhale hypertonic saline either before, during, or after the airway clearance techniques. Participants readmitted within one year repeated the 3-day study. OUTCOME MEASURES The primary outcome was the change in forced expiratory volume in one second (FEV(1)) from before to 2 hours after an entire airway clearance session. Secondary outcomes were change in forced vital capacity, perceived efficacy, tolerability, satisfaction, adverse events, and adherence. RESULTS All 50 participants completed the study. The effects on lung function were non-significant or were of borderline statistical significance favouring inhalation of hypertonic saline before airway clearance techniques. Satisfaction was rated significantly worse on a 100mm scale when hypertonic saline was inhaled after the airway clearance techniques: mean differences 20mm (95% CI 12 to 29) compared to before the airway clearance techniques and 15 mm (95% CI 6 to 24) compared to during the techniques. Perceived effectiveness showed similar effects but other outcomes were unaffected. All 14 participants who were readmitted repeated the study and most preferred the same timing regimen. Conclusion People with cystic fibrosis could be encouraged to time hypertonic saline before or during airway clearance techniques to maximise perceived efficacy and satisfaction, even though lung function may not be better with these timing regimens. TRIAL REGISTRATION ACTRN12611000673943.

Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline

Physiotherapy management is a key element of care for people with cystic fibrosis (CF) throughout the lifespan. Although considerable evidence exists to support physiotherapy management of CF, there is documented variation in practice. The aim of this guideline is to optimize the physiotherapy management of people with CF in Australia and New Zealand. A systematic review of the literature in key areas of physiotherapy practice for CF was undertaken. Recommendations were formulated based on National Health and Medical Research Council (Australia) guidelines and considered the quality, quantity and level of the evidence; the consistency of the body of evidence; the likely clinical impact; and applicability to physiotherapy practice in Australia and New Zealand. A total of 30 recommendations were made for airway clearance therapy, inhalation therapy, exercise assessment and training, musculoskeletal management, management of urinary incontinence, managing the newly diagnosed patient with CF, delivery of non‐invasive ventilation, and physiotherapy management before and after lung transplantation. These recommendations can be used to underpin the provision of evidence‐based physiotherapy care to people with CF in Australia and New Zealand.

Spontaneous coronary artery dissection associated with coughing

a Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia b Department of Respiratory Medicine, University of Sydney, NSW 2006, Australia c Department of Respiratory and Sleep Medicine, St. George Hospital, 30 Gray Street, Kogarah, NSW 2217, Australia d Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia e Department of Cardiology, St. George Hospital, 30 Gray Street, Kogarah, NSW 2217, Australia

A randomised, cross-over trial of upright sitting versus alternate side lying during nebulised delivery of medication in cystic fibrosis

Introduction: Studies show Non-Invasive Ventilation (NIV) in Cystic Fibrosis (CF) has reduced respiratory failure and aided secretion clearance. Our aim was to examine the use of NIV in CF patients over 12 months (Jan–Dec 2008). Method: A retrospective observational study of adult CF inpatients treated with NIV was performed. Indication for NIV treatment, tolerance, complications and perceived benefit were recorded. Results: 20 patients were treated with NIV over 35 episodes, 9 male with a mean age of 31 years (SD 9). Median predicted lung function on admission was 23% (10−53%). 13 patients had 1 NIV episode; 7 patients had 2−6 episodes. NIV was used to aid sputum clearance in 5 patients (12 episodes) (34%), control hypercapnic symptoms in compensated type II respiratory failure in 10 patients (17 episodes) (49%) and to reverse acidosis in acute type II respiratory failure in 5 patients (6 episodes) (17%). NIV was tolerated well in 18 patients (33 episodes) (94%), with subjective benefit in all episodes. In patients with raised PaCO2, reduction of headache was reported in 9 patients (17 episodes) (74%), and decreased work of breathing in 3 patients (3 episodes) (13%). All 5 patients (12 episodes) started on NIV to aid sputum clearance and 1 patient with acute respiratory failure reported increased sputum clearance with NIV. 2 patients were unable to tolerate the mask and felt no subjective benefit. The most common complications reported by those patients who tolerated NIV were; aerophagia 4 (22%), pressure sores 3 (17%), difficulty sleeping 2 (11%) and dehydration 2 (11%). Conclusion: NIV was used successfully to control symptoms of hypercapnic respiratory failure and sputum retention and was generally well tolerated.

Use of extracorporeal membrane oxygenation in cystic fibrosis in an Australian cystic fibrosis centre

Extracorporeal membrane oxygenation (ECMO) support is used in selected patients with cystic fibrosis (CF) as a bridge to transplantation. Our aim was to describe briefly treatment and outcomes of six CF patients who received ECMO. One patient received a lung transplant and another recovered from acute respiratory failure. Four died despite ECMO support. Lack of timely availability of suitable donor lungs and patient selection are contributing factors.

Drug Therapies that Augment Airway Surface Liquid

This chapter briefly describes the airway surface liquid (ASL) and the critical role of its hydration status and reviews the pathophysiology initiated by insufficient hydration of ASL. It then presents detailed evidence about medications intended to restore ASL and their effectiveness in people with CF. Inhaled osmotic agents are one class of ASL-augmenting medications. Nebulized hypertonic saline significantly improves lung function, reduces exacerbations, and improves the quality of life of people with CF. Dry powder mannitol also significantly improves lung function and reduces the incidence of exacerbations in this population, but it does not significantly improve quality of life. Ion transport modifiers are another class of inhaled medications that augment the ASL. These medications address the underlying ion transport defects by either inhibiting sodium absorption (e.g., amiloride) or by stimulating chloride secretion (e.g., denufosol). Despite promising preliminary research, ion transport modifiers have not demonstrated clinical benefits to date. In summary, osmotic agents, such as hypertonic saline and dry powder mannitol, have clinical worthwhile benefits in people with CF and are currently used in clinical practice.