Cystic Fibrosis is the UK’s most common life-threatening inherited disease with over 1 in 25 people carrying the defective gene. There are currently about 9,000 children and adults living in the UK with the disease. The average life-expectancy is around 41 years and, as yet, there is no cure, although improvements and advances in treatment mean that a baby born with CF today has the chance of living much longer than in the past.
CF mainly affects the lungs and digestive system. Sticky mucus secretions in the lungs cause repeated chest infections, which in time leads to irreparable lung damage. Treatments for the lungs include:-
- Physiotherapy- on the chest to break up some of the mucus. This needs to be performed a number of times daily
- Oral, nebulised or intravenous antibiotics- again to break down mucus and fight infection
- Prophylaxis- preventative therapy
CF causes similar secretions in the digestive tract and pancreas, causing little or no nutrients to be absorbed by the patient. Treatments for this include:-
- Attention to diet- avoiding fat is key here
- Vitamin supplements- to make up for those lost through failing pancreas
- Pancreatic enzyme supplements- to help break down foods
All patients suffer differently with CF, some with far more severe symptoms than others. Sufferers can also develop other problems such as:-
- Insulin dependent diabetes
- Liver disease
- Arthropathy
- Osteoporosis
- Infertility
- Vasculitis
- Cancer of the digestive tract
For more information on CF please visit the Royal Brompton & Harefield NHS Foundation Trust website
