A. Anderson

Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation

Background Extra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study. Methods Data from 12 patients with CF and the G551D mutation prescribed ivacaftor (150 mg bd) was collected at baseline, 6, 26 and 52 weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)). Results Six patients presented EOR at baseline (RSI > 13; median 13; range 2–29) and 5 presented airway reflux (HARQ > 13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P < 0 ∙ 04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire. Conclusion Ivacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration.

Current characteristics, challenges and coping strategies of young people with cystic fibrosis as they transition to adulthood

ABSTRACT This study provides detailed data on the current characteristics, perceptions and outcomes of 45 young people with cystic fibrosis (CF) as they transition into adulthood. Although many had severe disease, they generally coped well, found attendance at a transition clinic helpful and welcomed the increased independence of an adult healthcare environment. Levels of psychological distress were low with only 15.6% having anxiety and 6.7% depression. The main psychological coping strategy used was optimistic acceptance. Overall, most remained stable after transfer but 33% had some decline in lung function and 9% in nutritional status, requiring intensification of treatment. They had high levels of satisfaction with their relationships and life situations and 76% were in employment or education. These results are encouraging and as life expectancy improves, young adults with CF are coping well with transition into adulthood.

A service evaluation of an integrated model of palliative care of cystic fibrosis.

Background: Patients with advanced cystic fibrosis have severe symptoms with a complex trajectory of exacerbations and recovery. They are often awaiting lung transplantation, and many die without receiving specialist palliative care. Aim: We introduced an integrated model whereby palliative specialists joined the cystic fibrosis team to provide palliative care in parallel with standard care. Design: A service evaluation of this model of care was undertaken in a prospective case series documenting symptoms and outcomes, the views of the cystic fibrosis team and the experience of the palliative specialists. Setting/participants: Over 3 years, 28 (10%) of 282 patients attending the cystic fibrosis centre had specialist palliative care. Results: They had advanced lung disease (mean forced expiratory volume in 1 s (FEV1) = 0.86 L (25% predicted)), and 17 died: 6 were on a transplant waiting list at death; 10 were unsuitable and 1 died post transplantation. All who died over these 3 years had specialist palliative care. Four patients had successful transplants. Assessment showed a high prevalence of breathlessness, cough, pain, vomiting and fatigue, with a significant impact on daily life. The cystic fibrosis team rated this model of care highly, felt that palliative care should be members of the team, and thought that patients had found it helpful. The palliative specialists gained knowledge of cystic fibrosis, found it beneficial to meet patients earlier in the disease, and identified unmet needs in managing bereavement and the effects of deaths on other patients with cystic fibrosis. Conclusion: This model has been successful in overcoming the difficulties in access to specialist palliative care for patients with cystic fibrosis.

WS3.1 Gastric aspiration into the CF lung – relationship with reflux symptoms and lung function

WS3.1 Gastric aspiration into the CF lung − relationship with reflux symptoms and lung function G.L. Crossfield1, A. Krishnan2, J. Lordan3, S. Bourke4, A. Anderson4, P.W. Dettmar5, I.A. Brownlee6, C. Ward7, J.P. Pearson1. 1Institute of Cell and Molecular Bioscience, Faculty of Medical Sciences, Newcastle University, Newcastle Upon Tyne, United Kingdom; 2Northern Oesophago-Gastric Unit, Royal Victoria Infirmary, Newcastle Upon Tyne, United Kingdom; 3Institute of Transplantation, The Freeman Hospital, Newcastle Upon Tyne, United Kingdom; 4Royal Victoria Infirmary, Newcastle Upon Tyne, United Kingdom; 5Technostics Ltd, Daisy Building, 2nd Floor, Castle Hill Hospital, Cottingham, East Yorkshire, United Kingdom; 6Food and Human Nutrition Department, Nanyang Polytechnic, Newcastle University, Singapore; 7 Institute of Cellular Medicine, Faculty of Medical Sciences, Newcastle University, Newcastle Upon Tyne, United Kingdom

Travelling abroad with cystic fibrosis: Assessment of risks and healthcare requirements

There is limited information on the risks and healthcare requirements of patients with cystic fibrosis (CF) undertaking travel abroad. Of 100 patients (mean age 24.7 years, mean FEV1 57.3 %predicted) attending a UK adult CF Centre, 96% had travelled abroad but 14% now limited travel on medical advice. They travelled frequently and widely, often undertaking adventurous activities on holidays, but because of the costs involved, 18% travelled without travel insurance and 23% with insurance which did not cover CF. Of those who had ever had an illness abroad 10% had a CF-related illness (7 chest infection, 2 dehydration, 1 pancreatitis) and 12% a non-CF-related illness (4 sunburn, 3 gastroenteritis, 3 ear infection, 1 fall, 1 gastro-oesophageal reflux). There is a wide range of disease severity and assessment of the medical risks and the travel insurance premium to be charged should be based on the individuals health status rather than generically on the basis of a diagnosis of CF.

P231 A prospective cohort study of integrated palliative care of cystic fibrosis (CF)

There are 140 deaths in the UK each year from CF, often on a transplant waiting list and often without specialist palliative care. A palliative physician and nurse joined our team in 2011, providing palliative care in parallel with standard CF care. We undertook a prospective study documenting symptoms and outcomes, the views of the CF team and the experience of the palliative specialists. Over 3 years, 28 (10%) of 282 patients at our Centre had palliative input; their mean age was 31 (range 18–47) years and mean FEV1 was 0.86 L (24%); 17 (61%) died - 6 were on a transplant waiting list, 10 were unsuitable, and one died post transplantation; 4 have had transplantation and no longer need palliative input, 7 are in on-going care; 15 (88%) of deaths were on the CF ward and 2 at home. All patients who died had had palliative care. The main symptoms were breathlessness, cough, pain, vomiting, fatigue and low mood. The mean palliative assessment score was high at 2.9, indicating that life was dominated by symptoms. Palliative interventions included opioid, benzodiazepine, anti-emetic and anti-depressant medications and non-pharmacological interventions included relaxation techniques, massage, acupuncture and cognitive therapy. A survey was completed by 16 members of the CF team: all felt that palliative specialists should be part of the team and rated the model of care highly with a mean score of 4.1 (scale 1–5); 11 thought that patients had found input very helpful and 5 helpful; one patient declined a palliative consultation. The palliative specialists had increased their knowledge of CF, found it useful to meet patients earlier and had no difficulty in providing palliation in parallel with standard CF care. Their workload was high and they identified additional needs of bereavement counselling and managing the effects of deaths on other CF patients. This integrated model was successful in overcoming barriers to specialist palliative care. Palliative specialists have improved their knowledge of CF and the CF team have learnt palliative skills.

Overweight and obesity challenges in the Cystic Fibrosis population – a need for revision in nutritional advice?

G. L. Crossfield, A. Krishnan, S. Bourke, A. Anderson, A. Gurney, P.W. Dettmar, I. Brownlee, C. Ward and J. P. Pearson Institute of Cell and Molecular Bioscience, Faculty of Medical Sciences, Newcastle University, Newcastle Upon Tyne, NE2 4HH, Northern Oesophago-Gastric Unit, Royal Victoria Infirmary, Newcastle Upon Tyne, NE1 4LP, Royal Victoria Infirmary, Newcastle Upon Tyne, NE1 4LP, Technostics Ltd, Daisy Building, 2nd Floor, Castle Hill Hospital, Cottingham, East Yorkshire, HU16 5JQ, Food and Human Nutrition Department, Nanyang Polytechnic, Newcastle University, Singapore and Institute of Cellular Medicine, Faculty of Medical Sciences, Newcastle University, Newcastle Upon Tyne, NE2 4HH

WS5.2 Travelling abroad with cystic fibrosis (CF): current practice and problems

Background: The CF Foundation encourages CF centres to undertake local projects with the aim of improving CF Care. In 2012 a retrospective audit of the CF Annual Review (AR) process at Mater Children’s Hospital (MCH) was conducted. Whilst the percentage of ARs completed was found to be high, it was recognised that the vast amount of patient information being obtained was being underutilised. Aims: a. To increase the AR completion rate. b. To ensure that 100% of ARs have finalised documentation. c. To ensure all AR are discussed by the multi-disciplinary team (MDT). Methods: A new system was developed. A colour-coded spread sheet was used allowing easy identification of the point at which the patient had reached in the AR process; hence termed ‘the rainbow project’. Collected information was presented weekly at the CF MDT meeting. A proposed plan for each patient was discussed and specific goals for the following year agreed by the MDT. AR letters were finalised following discussion and reviewed with families when next seen. Results: A review was undertaken 6-months after commencement and demonstrated successful introduction of the process. 100% of patients undergoing an AR were discussed by the MDT with the identification of specific goals for the following year. Additionally, in all cases an AR letter was sent to both the GP and family. Conclusion: Improved adherence was seen with the new system. We speculate that refinement of the AR process has led to improvements in CF care delivered at our centre. A further audit is currently underway to assess this.