Liver and lung transplantation in cystic fibrosis: an adult cystic fibrosis centre's experience.

Liver disease develops in one-third of patients with cystic fibrosis (CF). It is rare for liver disease to have its onset after 20 years of age. Lung disease, however, is usually more severe in adulthood. A retrospective analysis was performed on nine patients. Three patients required lung transplantation approximately a decade after liver transplant, and another underwent combined liver and lung transplants. Four additional patients with liver transplants are awaiting assessment for lung transplants. One patient is awaiting combined liver and lung transplants. With increased survival in CF, several patients may require more than single organ transplantation.

Osmotic stimuli increase clearance of mucus in patients with mucociliary dysfunction.

Mucociliary dysfunction results in mucus accumulation, airway obstruction, bacterial colonization, recurrent infective exacerbations, and an increase in morbidity and mortality. Studies in patients with cystic fibrosis, established that inhalation of hypertonic saline (HS) increases clearance of mucus acutely in a dose-dependent manner. Clearance over 90 min was 23.8 +/- 4.0% and 26.0 +/- 3.1% in response to 7% and 12% saline, which was significantly enhanced compared to 12.7 +/- 1.4% and 19.7 +/- 3.1% in response to 0.9% and 3% saline. Mannitol (approximately 300 mg) inhaled as a dry powder had a marked acute effect in patients with bronchiectasis. Clearance over 75 min was 34.0 +/- 5.0% with mannitol, 17.4 +/- 3.8% with control, and 11.7 +/- 4.4% at baseline. Further studies in patients with bronchiectasis showed that mannitol reduces the 24-h retention of radiolabeled mucus, suggesting that the effect of mannitol extends beyond the acute phase. Mannitol helped patients to clear mucus within 2 h that would have taken 24 h to clear without mannitol. A further study in CF patients showed that mannitol was equally effective as 6% HS at improving ciliary and cough clearance. The total clearance over 120 min with mannitol (27.6 +/- 3.7%) and with HS (31.0 +/- 5.5%) was significantly increased compared to their respective controls (18.6 +/- 3.8% and 20.9 +/- 3.6%). These preliminary results suggest that long-term treatment with HS or mannitol may benefit patients with mucociliary dysfunction.

Supported and unsupported arm exercise capacity following lung volume reduction surgery: a pilot study.

STUDY OBJECTIVES: Lung volume reduction surgery (LVRS) has been shown to improve lung function, leg exercise capacity and quality of life in subjects with severe COPD. This is the first study to examine the effect of LVRS on supported and unsupported arm exercise capacity. DESIGN: Eight subjects with COPD (% pred FEV1 +/- SD = 31.1 +/- 9.8%) completed testing. At baseline (T1), after eight weeks pulmonary rehabilitation (T2) and four months after LVRS (T3), each subject had tests of lung function, and performed three symptom-limited exercise tests to peak work capacity: supported arm exercise (SAE), unsupported arm exercise (UAE) and leg exercise (LE). MEASUREMENTS: The FEV1 (% pred) increased from 27.8 +/- 7.4 (mean +/- SD) at T2 to 36.3 +/- 7.1 at T3 (P < 0.05). Peak oxygen consumption (VO2) remained similar from T1 to T2 for SAE, UAE and LE (all P = 1.0) but increased from T2 to T3 (P < 0.05) (SAE: T2 = 0.59 +/- 0.2 L/min, T3 = 0.72 +/- 0.1 L/min; UAE: T2 = 0.45 +/- 0.1 L/min, T3 = 0.54 +/- 0.1 L/min; LE: T2 = 0.68 +/- 0.2 L/min, T3 = 0.81 +/- 0.2 L/min). The ratio of end-expiratory lung volume to total lung capacity was reduced at peak SAE and LE from T2 to T3 (P < 0.01) (SAE: T2 = 81 +/- 4.0%, T3 = 76 +/- 2.7%; LE: T2 = 81 +/- 5.1%, T3 = 75 +/- 3.6%). CONCLUSION: There was a significant increase in SAE and UAE capacity following LVRS. Dynamic hyperinflation was reduced during SAE following LVRS.

Effect of airway clearance techniques on the efficacy of the sputum induction procedure.

Sputum induction is used in the early identification of tuberculosis (TB) and pneumocystis infections of the lung. Although manual physiotherapy techniques to clear the airways are often incorporated in the sputum induction procedure, their efficacy in this setting is unknown. This randomised, crossover trial enrolled adults referred for sputum induction for suspected TB and pneumocystis infections of the lung. All participants underwent two sputum induction procedures, inhaling 3% saline via ultrasonic nebuliser. During one randomly allocated procedure, airway clearance techniques (chest wall percussion, vibration, huffing) were incorporated. In total, 59 participants completed the trial. The airway clearance techniques had no significant effect on how the test was tolerated, the volume expectorated or the quality of the sample obtained (assessed by the presence of alveolar macrophages). The techniques did not significantly affect how often the test identified a suspected organism, nor the sensitivity or specificity of sputum induction. In conclusion, the study was unable to demonstrate any effect of airway clearance techniques on the sputum induction procedure. The results provide some justification for not including airway clearance techniques as part of the sputum induction procedure.

Method for detection of respiratory viruses in the sputa of patients with cystic fibrosis.

Since the role of respiratory viruses in lung exacerbations of patients with cystic fibrosis has been hampered by the difficulty of detecting viruses in viscous sputum specimens, a multiplex reverse transcriptase PCR (RT-PCR) assay combined with colorimetric amplicon detection was tested for the identification of seven common respiratory viruses in the sputa of cystic fibrosis patients. Of 52 sputa from 38 patients, 12 (23%) samples from 12 patients were positive for a respiratory virus (4 for influenza B, 3 for parainfluenza 1, 3 for influenza A and 2 for respiratory syncytial virus). These results suggest that the RT-PCR method carried out on sputum may provide a convenient means of investigating the role of virus infection in lung exacerbations of cystic fibrosis patients.

Adults with cystic fibrosis: meeting the challenge!

The number of adults with cystic fibrosis (CF) is increasing. They are striving for independence and a fulfilling life with focus on career, relationships, education and finances at a time when lung function is likely to be declining and complications of this multi-system disease are increasing. Maintaining the quality and improving the duration of life are continuing challenges for the -clinician and the patient. Increased hope and greater expectations have been provided by a number of recent clinical advances and active research into novel treatments, including gene therapy. There has been increased recognition of the necessity for early diagnosis, adequate monitoring and effective intervention for complications such as diabetes and osteoporosis. Research into multi-resistant bacteria and clonal strains of Pseudomonas aeruginosa is ongoing and attention has focused on infection control policies. Although more high-level evidence is required on many issues confronting people with CF, a considerable effort has been made over the last decade to provide a more evidence-based approach to therapy with a number of large controlled clinical trials. For the adult with CF, there are also more decisions to be made. There is focus on reproductive health, with most couples enjoying the real possibility of having children. For those with advanced disease, the option for lung transplantation is well established. Maintenance of quality care will require adequate planning, effective transition programmes from paediatric to adult care, specialized training for doctors, nurses and allied health professionals and the allocation of sufficient resources to accommodate the inevitable increase in patient numbers.

Reduction in resting energy expenditure following lung volume reduction surgery in subjects with chronic obstructive pulmonary disease.

STUDY OBJECTIVES: Some subjects with COPD have an elevated resting energy expenditure (REE) which may be related to an increased work of breathing at rest. The purpose of this study was to examine the effect of lung volume reduction surgery (LVRS) on REE and body weight. DESIGN: Ten subjects with COPD were recruited (mean age +/- SD = 61.4 +/- 6.1 years). At baseline (which was following preoperative pulmonary rehabilitation) and four months following LVRS (combined with postoperative pulmonary rehabilitation), each subject had tests of lung function, REE via indirect calorimetry using a canopy system, six minute walk distance (6MWD) and quality of life (QoL) using the St George's Hospital Respiratory Questionnaire (SGRQ). MEASUREMENTS: The FEV1 (% predicted) increased from 27.7 +/- 5.8% (mean +/- SD) at baseline to 33.9 +/- 7.8% following LVRS (P < 0.05). REE (% predicted) was 110 +/- 9.8% at baseline and decreased to 106 +/- 6.7% following LVRS (P = 0.04). Body mass index (BMI) following LVRS was unchanged (P = 0.67). No correlation between the change in BMI and change in REE was shown (r2 = 0.3, P = 0.1). There was a significant improvement in QoL following LVRS (P < 0.001). 6MWD also significantly increased from 354 +/- 83 m to 412 +/- 82 m following LVRS (P = 0.001). CONCLUSION: Whilst there was an increase in lung function and a reduction in REE following LVRS, there was no corresponding change to body weight. The improvement in REE following LVRS may be related to an improvement in work of breathing.