Rifat Rashid

Clinical outcomes of pandemic (H1N1) 2009 influenza (swine flu) in adults with cystic fibrosis.

Patients with cystic fibrosis (CF) suffer recurrent bacterial pulmonary infections, but viral infections can also cause acute clinical deterioration.1 Certain patient groups suffer increased morbidity following pandemic (H1N1) 2009 influenza (swine flu),2 but there are few previous reports of outcomes in individuals with CF.3 4 The West Midlands, along with Greater London, has had the highest incidence of H1N1 influenza in the UK.5 We therefore examined the outcomes of patients diagnosed with H1N1 influenza at the West Midlands Adult CF Centre. From June 2009 to April 2010 all adults with CF at our regional centre with potential H1N1 influenza had nasopharyngeal swabs tested …

“Cepacia syndrome” associated with Burkholderia cepacia (Genomovar I) infection in an adolescent with cystic fibrosis

To the Editor: Burkholderia cepacia was first described as an opportunistic organism in patients with cystic fibrosis (CF) in 1972 and was recognized as being pathogenic in CF in the 1980’s. In 1997, Vandamme et al. identified five distinct genomic species of B. cepacia, which collectively they termed the B. cepacia complex (BCC). Subsequent taxonomic studies have revealed that the BCC consists of at least 17 species, with B. cenocepacia (formerly genomovar III) andB. multivorans (formerly genomovar II) most commonly infecting individuals with CF. B. cepacia (formerly genomovar I) is seen in approximately 3% of BCC-infected CF individuals and is generally considered to be less virulent than B. cenocepacia and B. multivorans. Acquisition of BCC is associated with an accelerated decline of lung function, lower body mass index, increased morbidity, more frequent outpatient visits, increased hospitalization and decreased survival, both before and after lung transplantation. Outcomes following acquisition are however very variable, ranging from asymptomatic carriage to ‘‘cepacia syndrome,’’ an almost universally fatal necrotising pneumonia. B. cenocepacia and B. multivorans are the species most commonly implicated in causing cepacia syndrome. We present the first reported case of cepacia syndrome developing in association with B. cepacia infection. A 17-year-old female CF patient was admitted to our regional adult CF centre with worsening respiratory symptoms. She had been diagnosed with CF at 8 months of agewith delta F508 homozygous genotype and positive sweat test. Pseudomonas aeruginosa, Haemophilus influenzae, and Ralstonia pickettii had previously been intermittently cultured from her sputum. B. cepacia was first isolated from her sputumwhen shewas 9 years of age and this organism had been cultured onmultiple occasions over the intervening years. CF-related complications included exocrine pancreatic insufficiency and allergic bronchopulmonary aspergillosis (ABPA) for which she received long-term oral prednisolone. She had also suffered bacterial meningitis at the age of 6 months with consequent significant sensorineural hearing loss. At 16 years of age she transitioned to adult CF care, at which stage she was relatively healthy, with body weight 63 kg (BMI 25.8), FEV1 2.8 L (101% predicted) and FVC 3.35L (105% predicted). She had required intermittent courses of intravenous antibiotics as a child and young adult, most recently a 2-week course of ceftazidime and tobramycin 7 months previously. Due to the patient’s fears of further ototoxicity she was started on nebulised meropenem therapy, rather than nebulised tobramycin, soon after transition. Six months following transition, she was admitted to her local hospital with sore throat, hoarse voice, fevers, streaky haemopytsis and 2 kg weight loss. She was felt to have a viral upper respiratory tract infection and started on oral oseltamivir due to concerns over H1N1 pandemic influenza, although throat swabs tested negative by viral PCR. She received 10 days of intravenous ceftazidime and tobramycin, but since shewas not improving clinically she was admitted to the adult CF centre. She subsequently received intravenous hydrocortisone, ceftazidime, meropenem, and temocillin, as well as chest physiotherapy including use of high-frequency chest wall oscillation (the Vest). In addition she had high random blood glucose measurements suggestive of new onset CF-related diabetes (CFRD), for which she commenced subcutaneous insulin therapy. The chest radiograph showed bilateral multifocal consolidation and high resolution chest CT scan confirmed extensive bilateral consolidation but also demonstrated multiple lung abscesses and mediastinal lymphadenopathy. In view of these radiological findings, flucloxacillin and metronidazolewere added in an attempt to cover potential

Improving the care of patients with cystic fibrosis (CF)

Background The West Midlands Adult Cystic Fibrosis (CF) Centre based at Birmingham Heartlands Hospital provides care for adults with CF in the West Midlands. People with CF are prone to pulmonary exacerbations, which often require inpatient admission for intravenous antibiotics. We observed that the admission process was efficient during working hours (9:00–17:00, Monday–Friday) when the CF team are routinely available, but out-of-working hours, there were delays in these patients being clerked and receiving their first antibiotic dose. We were concerned that this was resulting in quality and potential safety issues by causing delays in starting treatment and prolonging hospital inpatient stays. We therefore undertook a quality improvement project (QIP) aimed at addressing these issues. An initial survey showed median time to clerk of 5 hours, with 60% of patients missing their first dose of antibiotics and mean length of stay of 16 days. Methods We applied the Plan-Do-Study-Act (PDSA) cycle approach, with the first PDSA cycle involving raising awareness of the issue through education to doctors, nurses and patients. Results This led to a reduction of median time to clerk from 5 to 2 hours with 23% of patients missing their first antibiotic dose and mean length of stay reducing to 14 days. The second cycle involved introducing an admissions checklist and displaying education posters around the hospital, resulting in median time to clerk remaining at 2 hours but only 20% of patients missing their first antibiotic dose and the mean length of stay remaining at 14 days. Conclusion This QIP has improved the out-of-hours admissions process for adults with CF in our centre. We plan to review the longer term effects of the project including sustainability, effects on clinical outcomes and patient satisfaction.

Physical activity in adults with cystic fibrosis receiving intravenous antibiotics in hospital and in the community.

Introduction : Intravenous antibiotic therapy (IVAT) for CF acute pulmonary exacerbations (APE) can be delivered in hospital or in the community. This study aimed to compare physical activity in CF patients receiving hospital and community-delivered IVAT, as well as other health outcomes. Materials and Methods : This was a non-randomised parallel group prospective observational study. Hospitalised and community-treated CF adults receiving IVAT for APE were asked to wear ActiGraph® activity monitors, complete the habitual activity estimation scale (HAES), food diary, modified shuttle test (MST) and CFQ-R at the start and end of therapy. Nutritional and clinical outcomes were also compared between the cohorts. The primary outcomes was physical activity measured by the ActiGraph® activity monitors at the beginning and end of treatment in both cohorts. Results : Physical activity (measured and self-reported) was no different between the cohorts, with both hospitalised and community-treated subjects being generally sedentary. Body weight increased significantly in the hospitalised cohort, whereas no difference was seen in the community-treated cohort. FEV1 % predicted and FVC % predicted increased in community-treated subjects, whereas only FVC % predicted increased in hospitalised subjects. CFQ-R respiratory domain increased to a greater extent in community-treated subjects. Conclusion : CF adults receiving IVAT for APE, both in hospital and in the community, are generally sedentary and we found no difference in physical activity between the two groups. These findings suggests the need to further promote physical activity in suitable patients during APE where considered appropriate.

P281 The effectiveness of acupuncture in managing symptoms in CF adults

Introduction and objectives People with cystic fibrosis (CF) experience a wide spectrum of symptoms, both physical and psychological, which are often troublesome and can result in significantly impaired quality of life. Acupuncture has a strong evidence base in the treatment of a wide variety of symptoms, however there is currently very limited evidence for its role in the management of symptoms in people with CF. The objective of this pilot study was to provide preliminary evidence of the effectiveness and patient acceptance of this treatment in our large regional adult CF centre. Methods In this observational prospective study, we offered acupuncture to CF inpatients attending our large regional adult CF centre if they were suffering from range of symptoms. Patients were asked to specify the symptoms being treated and its severity before and after treatment, as well as whether treatment achieved the desired outcome, whether they suffered any ill effects, whether it is a valuable service and whether they feel we should continue to offer acupuncture to patients. They were also offered the option of making free-text comments on their experience of the service. Results 106 patients were included over a 12-month period. 50 patients were treated for pain (back/neck/shoulder pain, n = 28; unspecified location, n = 12; headache, n = 5, chest pain, n = 4, toe pain, n = 1) with pain significantly reducing after treatment (median severity 7 (IQR 6–8) vs. 4 (IQR 3–5), p < 0.001). 25 patients were treated for stress/anxiety with symptoms significantly reducing after treatment (median severity 8 (IQR 7–9) vs. 5 (IQR 3.6–6), p < 0.001). 10 patients were treated for breathlessness/tight chest with symptoms significantly reducing after treatment (median severity 7 (IQR 6–8) vs. 5 (IQR 4–6), p = 0.001). 21 patients were treated for a range of other symptoms including low energy levels, reduced appetite and constipation. 95 patients reported that treatment had achieved the desired result and 10 patients reported that it was too early to tell. 1 patient felt ‘a bit sick’ after treatment, but no other adverse effects were reported. All patients felt that acupuncture was a valuable service and should continue to be offered. 30 patients commented that the service should be available more frequently. Conclusions Acupuncture was greatly appreciated by CF adults, with significant improvements in a wide variety of symptoms. A randomised controlled study is required to confirm these benefits.

P233 Cough swabs should not be used to exclude non-tuberculous mycobacterial (NTM) infection in adults with cystic fibrosis

Introduction and objectives People with cystic fibrosis (CF) are prone to airway infection with non-tuberculous mycobacteria (NTM) including M. abscessus, M. avium and M. intracellulare. Routine NTM screening is recommended for all patients at least once a year.1 In patients not able to produce sputum, cough swabs are often sent for NTM analysis. Anecdotally, we observed that several patients cared for in our centre had cultured NTM in sputum having previously been culture-negative from cough swabs. The objective of this observational study was to examine the diagnostic yield of cough swabs and sputum samples from CF adults with known NTM infection. Methods We identified all CF patients being cared for in our large regional adult CF centre that had cultured any NTM species from cough swabs or sputum samples and are currently attending our NTM clinic. Demographics, clinical parameters and microbiology results were recorded and analysed. Results 26 patients (19 male) were included: median age 24 years, 92% chronically infected with P. aeruginosa, 100% pancreatic insufficient, 62% CF-related diabetes, 27% ABPA. 381 sputum samples and 55 cough swabs were analysed. 251 (66%) sputum samples and 4 (7%) cough swabs cultured NTM (see Table 1). In the 4 cough swabs that cultured NTM, sputum samples also cultured the same species. Conclusions Cough swabs have a very low diagnostic yield and their use did not contribute to identification of NTM infection in our adult CF patient population. We have therefore stopped sending cough swabs for NTM culture in our centre and our data suggests that cough swabs should not be used to screen for these organisms. Reference 1 Mycobacterium abscessus Suggestions for infection prevention and control. CF Trust, 2013

P92 A Retrospective Study to Evaluate the Use of Nebulised Meropenem at a Large UK Adult CF Centre

Background Meropenem is commonly used intravenously in CF patients infected with Pseudomonas aeruginosa, but there is limited evidence of its tolerability and effectiveness when used as a nebulised treatment. Aim To evaluate the use of nebulised meropenem in our large UK adult CF centre. Methods Medical records of patients who had trialled nebulised meropenem between 2008–2012 were reviewed for: demographics, lung function, BMI, sputum microbiology, indications and tolerability and number of exacerbations requiring IV antibiotics. Results 37 patients (18 male) trialled nebulised meropenem, with median age (IQR) 27 (20–37) years and FEV1% predicted 48% (35–64%). Patients received a 250 mg dose reconstituted with water for injection preceded by a bronchodilator. All patients were chronically infected with P. aeruginosa. The most common treatment indications were deteriorating condition or intolerance to other antibiotic nebulisers (21/37), with additional indications including treatment of Burkholderia cepacia complex (8/37) and non-tuberculous mycobacteria (8/37). 22/37 continue to tolerate the drug well. 10 of these 22 had previously been intolerant to Tobramycin Inhalation Solution (TIS) and 1 intolerant to nebulised amikacin. 4/37 discontinued due to no benefit or poor concordance. 11/37 did not tolerate nebulised meropenem, reporting adverse effects including chest tightness, increased cough, chest discomfort, nausea and lethargy. 6 of these 11 had previously been intolerant to TIS and 2 were intolerant to amikacin. There were no significant changes in lung function, BMI or requirement for IV antibiotics comparing the 12-month periods before and after commencing nebulised meropenem. Conclusion Nebulised meropenem is generally well tolerated in CF adults and offers an alternative antibiotic choice for people with CF unable to tolerate other treatments. Abstract P92 Table 1 No. courses of IVs 12/12 pre mero No. courses of IVs 12/12 post mero Days in hosp 12/12 pre mero Days in hosp 12/12 post mero M. abscessus 7 10 43 44 B. cepacia complex 8 5 43 11 Other 23 25 211 293