This information from Great Ormond Street Hospital is about cystic fibrosis (CF) – an inherited disease primarily affecting the lungs and digestive system. It happens because the gene that is responsible for making mucus is faulty. Normally, the mucus that lines our internal organs is clear, lubricating and protects against infection. In babies with CF, it is thick, congesting and prone to infection.
Clinical outcomes are broadly agreed, measurable changes in health or quality of life that result from our care. Constant review of our clinical outcomes establishes standards against which to continuously improve all aspects of our practice.
Digestive enzymes are made in the pancreas. The fat, protein and carbohydrate in food is broken down by the enzymes to release nutrients. In cystic fibrosis (CF) sticky mucus blocks the passages from the pancreas to the small intestine which stops the enzymes working, so the food cannot be digested or absorbed by the body. This is called malabsorption and causes loose or oily stools, wind, stomach ache and poor weight gain.
Children with cystic fibrosis (CF) may from time to time need an admission to Great Ormond Street Hospital (GOSH) and this may be for a variety of reasons. Usually the admission is discussed with the family and planned ahead of time but occasionally a more urgent admission may be required.
Physiotherapy is an essential part of the treatment for cystic fibrosis (CF) and should start as soon as the diagnosis of CF is confirmed. This page explains the type of physiotherapy treatment used for infants with CF at Great Ormond Street Hospital (GOSH). Further information is available online from the CF Trust website.
Cystic fibrosis related diabetes (CFRD) is different to other types of diabetes. CFRD is caused by the build up of thick secretions in the pancreas. These secretions damage the cells that produce a hormone called insulin.
Children with moderate to severe cystic fibrosis (CF) enrolled on a programme offering physiotherapy, dietary support and personal training sessions at their local gym, were found to spend less time in hospital receiving antibiotics, as well as boosting their exercise capacity.