Research
Audit and review
Our team undertake regular reviews to analyse the benefits of the treatments that we offer (how we are doing), and new information which may benefit the cystic fibrosis community is presented at national and international respiratory and cystic fibrosis conferences. The anonymised data on the port cystic fibrosis database will be used to compare our service to others in the UK and as a tool for quality control.
Presentations
Presentations that our team has conducted include:
- The benefit of home respiratory physiotherapy and its acceptance by cystic fibrosis patients.
- The role of continuous subcutaneous glucose monitoring system in cystic fibrosis-related diabetes management.
- The relationship between glucose control and lung function and weight in cystic fibrosis-related diabetes.
- Lung function decline in cystic fibrosis patients who subsequently develop cystic fibrosis-related diabetes.
- The effect of chronic azithromycin use on lung function in adults with cystic fibrosis
In the last three years, members of our cystic fibrosis team have presented 7 posters of our research at the European Cystic Fibrosis Society Annual Conference, the American Thoracic Society Annual Conference, and the British Thoracic Society annual winter meeting.
Developing new treatments
Our team has developed a modified method of delivering laxative therapy for DIOS (distal intestinal obstruction syndrome) and severe constipation.
Previously cystic fibrosis adults with DIOS that did not respond to oral laxative therapy needed to have a colonoscopy to insert large volumes of gastrograffin into the beginning of the large bowel or distal end of the small bowel. We tried using a nasojejunal feeding tube (similar to a standard nasogastric tube but ending further down in the small bowel) to deliver the gastrograffin. This was successful but some patients found it uncomfortable and coughed the tube out.
We then changed to using a special gastrostomy button with a second tube ending in the mid small bowel which could be inserted under x-ray guidance. This has worked successfully in a number of patients, avoiding the need for a colonoscopy, and has allowed them to manage their constipation successfully at home. A paper is being prepared for publication.
Research
Pharmaceutical Research
We are recruiting patients to two clinical trials run by pharmaceutical companies to investigate new drug treatments in cystic fibrosis. Dr Kuitert is chief investigator for the UK for one trial (Novartis, formally Chiron). Our service has achieved the highest recruitment rates for both trials. These trials are co-ordinated through the Lung Research Centre, based at the London Chest Hospital.
Local Research
Vitamin K and bone density
Our specialist cystic fibrosis dietitian has an interest in bone disease in cystic fibrosis and has a research grant to study the effect of vitamin K supplementation on bone density.
Lung inflammation during pulmonary exacerbations and the impact of cystic fibrosis-related diabetes.
Dr Kuitert, Director of the Cystic Fibrosis Centre for adults is studying the changes in sputum mediators (markers of inflammation in the lung that are part of the lung infection process) when a pulmonary exacerbation (chest infection) is treated and comparing the changes in cystic fibrosis patients with and without cystic fibrosis-related diabetes.
She is also developing studies looking at more intensive treatment of diabetes and its effect on acute exacerbations in cystic fibrosis.
The preliminary research evaluating the rate of decline of lung function in cystic fibrosis patients who then developed diabetes found that an accelerated decline in FEV1 began 6 years before the diagnosis of diabetes and was much worse in females than males. Contrary to one previous study, we did not find a reduction in body mass index (BMI). This research was accepted as an oral presentation at the European Cystic Fibrosis Society meeting in Copenhagen in 2006.
Interaction of cystic fibrosis-related diabetes and cystic fibrosis liver disease.
A pilot study looking at parameters of liver disease and markers of diabetes control in cystic fibrosis is being undertaken by Dr Kuitert with our diabetes specialist (Professor Graham Hitman), and hepatologist (Professor Graham Foster) through the Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry.
Publications
(1). Kuitert LME, Driver R, Butler M, Hitman GAH. Lack of correlation between lung function, body mass index (BMI), and hyperglycemia in cystic fibrosis-related diabetes (cystic fibrosis RD). Am J Respir Crit Care Med 2006;173:A370
(2). Brown J, Elston C, Driver R, Hitman GA, Kuitert LM. Decline in lung function and BMI prior to the diagnosis of CYSTIC FIBROSISRD and the impact of gender. J Cystic fibrosis 2006;5(suppl.1):S63
(3). Butler M, Driver R, Elston C, Hitman GA, Kuitert LM. Use of Continuous Subcutaneous Glucose Monitoring System (CSGMS) in the Management of cystic fibrosis RD: the cystic fibrosis RD team and patient perspective. J Cystic fibrosis 2006;5 (suppl 1):S63
(4).Barton A, Choudhury A, Shanmugasundaran P, McAvinia P, Coyle F, Foster GR, Kuitert L. Association between abnormal hepatic enzymes, fatty change in the liver, and acute pulmonary infection in Cystic fibrosis. Thorax 2006;61(suppl. 1): ii121
(5). Keane J, Townsend J. Should community physiotherapy be a standard care package with all home intravenous courses? J Cystic fibrosis 2006;5 (suppl. 1):S77
(6). Butler M, Kuitert L. The impact of pulmonary gender, pulmonary disease severity, and presence of diabetes on azithromycin benefit. J Cystic fibrosis 2007;6(suppl 1);S40
(7). Turner S, Kuitert L. The effect of maximal exercise on FEV1 in adult cystic fibrosis (cystic fibrosis). J Cystic fibrosis 2007;6(suppl 1):S64
