Cystic Fibrosis database for Great Ormond Street Hospital for Sick Children

Background

Great Ormond Street Hospital (GOSH) is a major centre for treating children with Cystic Fibrosis (CF). CF is the UK's most common life-threatening inherited disease, affecting over 7,500 people. Cystic Fibrosis affects internal organs, particularly the lungs and digestive system. People with CF require daily medication and physiotherapy. The average life expectancy is approximately 31 years, although improvements in treatments mean that a baby born today may be expected to live longer.

The CF Unit at GOSH required a database for both clinical and research purposes; to record, display and analyse information on the patients with CF attending the centre.

Objectives

1. To provide a place to store and retrieve information on CF patients at GOSH, including details of the mode of presentation; clinical assessment, medication; laboratory tests; physiotherapy; exercise and nutrition
2. To store clinical information obtained at annual review and generate a report for the local hospital, community team and GP.
3. To derive values for z scores for growth parameters including height, weight and body mass index.
4. To produce charts which show progress in terms of lung function and growth as well as longitudinal results of various laboratory tests.
5. To generate letters to local consultants, GPs and patients.
6. To provide a system which can be easily queried in order to obtain data for research and audit purposes.

Development and Implementation

Advent IT developed the Microsoft Access CF database which contains a range of menu choices for data-entry and reporting. The data-entry forms are tabbed and sub divided clearly displaying the investigation or data stored. The reporting is complex and allows an Annual Review for each patient to be built and printed containing data tabulations and charts.

Benefits

The benefits of having all GOS CF data available electronically are invaluable. It is used as an administrative resource for the production of letters and reports, saving valuable clinical time. The charts and graphical displays are used for clinical decision making and reporting. The data is used both within the unit and in collaboration with other colleagues in research.

Publications and Research

Modifier effect of the Toll-like receptor 4 D299G polymorphism in children with cystic fibrosis. Arch. Immunol. Ther. Exp. 5 4 2006. Donald S. Urquhart , Jennifer Allen, Mohamed Elrayess, Katy Fidler, Nigel Klein and Adam Jaffé