The mucus is thicker than normal because CF affects cells in the epithelium (pronounced: eh-puh-THEE-lee-um), the layer of cells that lines the passages in the body's organs.
In a person who does not have CF, the epithelial cells produce a thin, watery mucus that acts like a lubricant and helps protect the body's tissues.
CF is a serious but variable disease which requires regular and continuous treatment.
The severity of the lung disease is the key to the quality and the length of life.
In the gut, the thick mucus prevents normal digestive enzymes to flow into the bowel.
This causes incomplete digestion and malabsorption which can affect the growth of the baby and child.
The patients were over the age of 12 and had a lung function between 50 per cent and 90 per cent of healthy individuals.
Because the mucus can block the path between the pancreas and the intestines, people with CF have trouble digesting food and getting the vitamins and nutrients they need from it.However, the members of the UK Cystic Fibrosis Gene Therapy Consortium believe they have now made the key initial breakthrough that allows them to proceed with further clinical trials involving more active forms of the gene-therapy treatment.“We are looking to undertake follow-up studies assessing higher, more frequent doses as well as combinations with other treatments,” Professor Alton said.The latest trial involved 40-minute inhalations once a month for year with a nebulising spray containing fatty droplets or “liposomes” wrapped around a healthy, synthetic copy of the cystic fibrosis gene.This can happen if the parents are "carriers" of the faulty gene.This means they don't have cystic fibrosis themselves, but they can have a child with the condition if their partner also carries the faulty gene.