Cystic Fibrosis

The Cystic Fibrosis (CF) Unit at Great Ormond Street Hospital (GOSH) is a designated specialist centre with around 180 children aged 0-16 years under its care. The unit has a full specialist multidisciplinary team of doctors, nurses, physiotherapists, dietitians, pharmacist, psychologists, social workers and administrative staff. The department is actively involved in research to benefit children and families with cystic fibrosis. 

The CF Unit works closely with a number of other departments within GOSH, in particular Paediatric Radiology, Endocrinology and Gastroenterology. 

Outcomes

One of the key measures undertaken to monitor CF lung function is the Forced Expiratory Volume in 1 second (FEV1 ). This measures the proportion of expired air blown out in the first second during a hard and fast breath out.

• In 2016 the national average FEV1 per cent predicted for all those with CF aged 6 and over was 91 per cent. The result for our clinic at GOSH in 2016 was 91 per cent, the same as the national average value and within the control limits. We continue, with our quality improvement programme and other projects (such as the INSPIRE study) striving to increase this value further. It is our goal to transfer our young people to adult services with normal lung function. 

• In 2016, the national average BMI percentile for all those with CF aged 2 to 15 was 53. The BMI percentile for our clinic at GOSH in 2016 was 45.1. Although national guidelines state that the majority of children with CF should have a BMI percentile of 50 or greater, a high BMI does not mean a healthy nutritional state. 

• In 2016 the national average percentage of patients with chronic Pseudomonas aeruginosa infection in paediatric centres in the UK was 7.5 per cent. The proportion of GOSH patients with chronic Pseudomonas aeruginosa was 5 per cent, below the national average and within the control limits.