Background: The telehealth service is one of the fastest growing healthcare segments. It is increasingly utilizing computer technology and telecommunication equipment to either provide continuous vital sign monitoring or facilitate patient care at home, rather than relying solely on in-person care. Methods: We conducted a 6-week open study in nineteen patients with cystic fibrosis enrolled from three centers, to investigate patient perception of a telehealth enabled nebulizer system (Prodose Adaptive Aerosol Delivery [AAD] System), which enabled the doorstep delivery of repeat medication. Results: The results showed that patient confidence in the device and perception of ease of use was high with no significant change between the start and end of the trial. Views on the home delivery of medication were split between ‘great’ and ‘inconvenient.’ However, if the delivery system had been more flexible and delivered all the patients' drugs, the majority of patients would have had their medication delivered in this way. Conclusions: The trial showed that it was possible to build telehealth technology into an advanced nebulizer system, and that patient acceptance of the technology was unlikely to be a barrier to the adoption of such a telehealth system.

We undertook assessment of hearing in patients with cystic fibrosis who were taking part in a large randomized controlled trial of once- versus three-times-daily tobramycin for pulmonary exacerbations of cystic fibrosis (the TOPIC study). All patients were eligible to have standard pure tone audiometry performed across the frequency range of 0.25 to 8 kHz. High-frequency pure tone audiometry over 10 to 16 kHz was also performed with a subset of patients. Audiometry was undertaken at the start of tobramycin treatment, at the end of a 14-day course of treatment, and at follow-up 6 to 8 weeks later. We enrolled 244 patients, of whom 219 (125 children and 94 adults) completed treatment. Nineteen patients were excluded from analysis due to abnormal baseline audiometry. Complete pre- and posttreatment standard audiological data were obtained for 168/219 patients. We found no significant differences in hearing thresholds when they were assessed at the baseline, at the end of treatment, and at follow-up 6 to 8 weeks later were compared. In addition, no significant differences in hearing thresholds were detected between treatment regimens. Similar results were obtained for the subset of 63/168 patients who underwent high-frequency audiometry. We conclude that for a single 14-day course of tobramycin treatment in patients with cystic fibrosis with no preexisiting auditory deficit, no measurable effect on hearing was apparent with either once- or three-times-daily treatment. Estimation of the cumulative cochleotoxic risk in cystic fibrosis patients due to repeated aminoglycoside therapy, as evidenced by the patients excluded from this study due to hearing loss, also requires further characterization.

BACKGROUND AND AIM:Life expectancy in patients with cystic fibrosis (CF) has recently improved due to numerous factors, including a multidisciplinary approach to their management. Prolonged survival may have led to an increasing impact of liver disease on the prognosis of CF patients. The aim of this study was to assess the role of liver transplantation in patients with CF.METHODS:The factors influencing outcome in 24 patients (15 adults and nine children) with CF who have received single liver transplantation, triple heart-lung-liver transplantation (tx) or died while being assessed for triple grafting, were analyzed.RESULTS:Median age at tx in single liver recipients (13 years) was lower than in triple graft recipients (21 years) and those who died (23 years). All patients who received single liver tx made an excellent recovery, including significant improvement of their respiratory function (mean forced vital capacity (FVC) increased from 61% before transplantation to 82% of expected, 6-9 months after tx). Four out of five patients who received triple tx died (0-2 months) after operation. On the basis of our retrospective review, we propose modifications to an existing scoring system for liver tx assessment in CF by scoring additional points for elevated white blood count, bilirubin, and impaired pulmonary function. These changes will need to be evaluated prospectively to confirm their predictive value.CONCLUSIONS:Liver transplantation is effective therapy in young patients with cystic fibrosis, portal hypertension and hepatic dysfunction, and is indicated before a critical stage of deteriorating lung function is reached. In patients with both end-stage liver and lung disease, triple tx has a poor prognosis. Pre-emptive liver tx in younger patients with CF not only has a better outcome but improves lung function.

Audit was performed to assess physiotherapy documentation within the adult cystic fibrosis unit at Birmingham Heartlands Hospital, against nationally accepted standards compiled by the Chartered Society of Physiotherapy (1993) and the Association of Chartered Physiotherapists in Respiratory Care (1994). Through their involvement with clinical audit, healthcare professionals derive benefits for themselves, by increasing their knowledge and understanding of effective practice, thus enhancing the effectiveness of the care they deliver. Audit was initially completed between December 1998 and January 1999 using the medical notes of all 43 adult cystic fibrosis patients admitted to the hospital. Physiotherapy documentation was assessed using a proforma designed by the authors. Results of audit concluded that the ‘gold standard’ of 100% completion rate was not met for all criteria of documentation. Recommendations were implemented before re-auditing. These included improving professional standards awareness via a programme of in-service training for all staff, development of local standards, and piloting the development of a new pre-printed assessment sheet. The audit loop was completed by re-auditing six months after initial audit to reassess the effect of recommendations. Documentation was re-audited for all 43 patients admitted between July and August 1999. Results showed that for registration details, 11 out of 12 sections scored 93% or greater completion rates in audit 2 compared with 5 out of 12 in audit 1. There was improvement in completion within 9 out of 12 (75%) of categories (2%-53% range of improvement). All categories of subjective assessment scored 84% or above completion rate in audit 2 compared to 5 out of 7 categories in audit 1. Improvement was noted in 4 out of 7 categories (range of improvement 7%-21%) in audit 2. The objective assessment showed 11 out of 14 sections scored 90% or above in audit 2 compared to 8 out of 14 in audit 1. Improvement in 11 out of 14 categories (78%) demonstrated a range of improvement from 2% to 61% in audit 2. For any test not completed or requested it is recommended that this be documented. It was recommended that all areas of assessment should be completed within 24 hours of referral. All 43 physiotherapy records displayed a treatment plan, and 95% of patients' notes demonstrated ongoing evaluation in audit 2 compared to 93% in audit 1. Of all entries made, 99% in audit 2 were signed compared with 95% in audit 1. 97% were dated in audit 1 and 99% in audit 2. A discharge summary was completed in 77% of all notes in audits 1 and 2. Regular in-service training for all on-call staff will continue to ensure awareness of documentation standards. Re-audit in 12 months will evaluate implementations after audit 2 – the use of a new pre-printed assessment form and development of a CF physiotherapy care pathway. The aim is to achieve the ‘gold standard’ of 100% completion rate for documentation, ensuring ongoing commitment to provision of improved patient care through audit.

Pseudomonas aeruginosa is a non-capsulate and non-sporing Gram-negative bacillus that most commonly affects the lower respiratory system in humans. Burkholderia (previously Pseudomonas) cepacia has emerged as an important respiratory pathogen in patients with cystic fibrosis (CF). The ability of P. aeruginosa to persist and multiply in moist environments and equipment, such as humidifiers in hospital wards, bathrooms, sinks and kitchens, maybe of importance in cross-infection. P. aeruginosa infections of the lower respiratory tract can range in severity from colonisation (without an immunological response) to a severe necrotising bronchopneumonia. Infection is seen in patients with CF and other chronic lung diseases such as non-CF bronchiectasis. In patients with CF, once P. aeruginosa is established in the airways it is almost impossible to eradicate, but prior to this, aggressive treatment can delay the development of chronic infection. 30 to 40% of the present paediatric population with CF will have chronic pseudomonal infection. B. cepacia has a particular predisposition to infect patients with CF and may be distinguished from P. aeruginosa by accelerated lung disease in about one-third of patients. Overwhelming septicaemia and necrotising pneumonia are well described (cepacia syndrome); events that are rare with P. aeruginosa. With the propensity for social cross-infection, segregation policies have been accepted as means of controlling outbreaks. A number of antipseudomonal agents are available. The most commonly used are the extended-spectrum penicillins, aminoglycosides, cephalosporins, fluoro-quinolones, polymixins and the monobactams. An aminoglycoside with a β-lactam penicillin is usually considered to be the first line treatment. No trial has shown any significant clinical advantage of any particular combination regimen over another. The emergence of resistance continues to be a concern. Pipericillin, piperacillin/tazobactam and meropenem have good but equivalent antibacterial activity against P. aeruginosa. However, B. cepacia is characterised by in vitro resistance to colistin (colomycin), aminoglycosides and ciprofloxacin but better susceptibility to ceftazidime. Nebulised delivery of antipseudomonal antibiotics is thought to prevent recurrent exacerbations, reduce antibiotic usage and maintain lung function, particularly in patients with CF. Colistin, tobramycin and gentamicin are currently the most commonly prescribed nebulised antibiotics. Much effort is directed at treating chronic P. aeruginosa infection but as chronic infection is seldom if ever eradicated when first established, prevention is preferable. Early intensive treatment for P. aeruginosa infection is advocated in order to maintain pulmonary function and postpone the onset of chronic P. aeruginosa infection.

Audit was performed to assess physiotherapy documentation within the adult cystic fibrosis unit at Birmingham Heartlands Hospital, against nationally accepted standards compiled by the Chartered Society of Physiotherapy (1993) and the Association of Chartered Physiotherapists in Respiratory Care (1994). Through their involvement with clinical audit, healthcare professionals derive benefits for themselves, by increasing their knowledge and understanding of effective practice, thus enhancing the effectiveness of the care they deliver.

The lungs of people with cystic fibrosis (CF) are affected by a basic ion transport defect leading to more viscid airway mucus. This is an ideal breeding ground for bacteria with which the lungs can become chronically colonised from an early age, leading to progressive obstructive pulmonary disease. The principles of treatment include airway clearance by physiotherapy and prophylaxis and treatment of infection with antibiotics.

Since March 1980, 309 patients with anaplastic small cell carcinoma of the bronchus (ASCB) have received remission induction therapy prior to randomisation to maintenance (M) or no maintenance (NM) chemotherapy. Induction therapy consisted of six courses of vincristine, doxorubicin and cyclophosphamide (VAC) given IV every 3 weeks. Those with limited disease also received mediastinal irradiation. Consenting patients with no unequivocal residual disease were randomised to have no further treatment until relapse or a further eight courses of VAC, at a lower dosage, every 4 weeks. Patients failing to achieve randomisation status received palliative treatment only. The median survival for all patients with limited disease (LD) is 363 days and that for patients with extensive disease (ED) is 272 days (P<0.00001).