Cystic Fibrosis - information from a Pediatric Nurse

What is CF?

Cystic fibrosis (CF) is a life long inherited illness. CF means that glands in the body that produce mucus become faulty and produce very thick sticky mucus. The organs mainly affected by this abnormal mucus production are the lungs and the pancreas in the digestive system.

The Lungs

Normally the mucus produced by glands in our lungs is responsible for clearing harmful bacteria out that we have breathed in. This is achieved via small oscillating hairs (cilia) that line the inside of our lungs pushing the mucus up where we eventually swallow it. In Cystic Fibrosis this mechanism for clearing mucus and bacteria out of the lungs is impaired. The cilia malfunction due to the presence of the thick sticky mucus.

In CF the presence of this mucus in the lungs is cause for concern as many bacterial organisms thrive causing repeated and often continuous infection in the airways. Every individual with Cystic Fibrosis is at risk from chest infections often just after they have contracted a 'cold'. The frequency of these infections is numerous, but unpredictable - however more likely in the cooler months of the year. In CF the main treatments to protect the lungs from the affects of infection are physiotherapy and antibiotics.

Physiotherapy

The aim of physiotherapy is to loosen the mucus in the lungs so that it can more readily move out of the lungs to be swallowed or spat out. Physiotherapy is tailored to the individual child and family and needs to be performed everyday even if they are well. When unwell with a chest infection physiotherapy will often be increased, individual sessions may be lengthened or the sessions may become more frequent. During chest infections extra physiotherapy may be required at night to ease coughing and other symptoms.

Antibiotics

Antibiotics are given to a child with Cystic Fibrosis at higher strengths and for a longer duration (normally 2 weeks). Many children require continuous antibiotics for ongoing infections (also known as colonised) or to protect them from chest infections. Most antibiotics are given orally, but occasionally intravenous and/or nebulised antibiotics are necessary to treat bacterial infections.

The Pancreas

Part of our digestive tract is the pancreas; it normally produces digestive enzymes and delivers them via small tubes (ducts) into our gut (small intestine). These enzymes are required to break down the food that we eat so that we can absorb the resulting nutrients into our bloodstream. Once in the bloodstream these nutrients are distributed around the body and are generally used for energy, the body's growth (particularly in children) and repair. In most people with CF the ducts leading from the pancreas into the small intestine are permanently blocked as a result of damage by thick sticky mucus. For these individuals to remain adequately nourished and in order that their growth and development is optimal they must take the following oral treatments:

Information written by Jason Witts, a pediatric nurse who works at Queen Alexandra Hospital in Portsmouth