Cystic Fibrosis

What causes Cystic Fibrosis?


CF is a genetically inherited condition caused by a faulty gene passed from parent to child.


1 in 25 people within the general population carry the faulty CF gene. A carrier of the gene does not have any symptoms and remains free of CF related symptoms throughout life. Both parents have to be carriers of the faulty gene for a child to be born with Cystic Fibrosis.


If both parents have the gene, there is a 25% chance the child will have CF. If both parents carry the gene there is also a 50% chance of the child being a gene carrier and a 25% chance they will not have the faulty CF gene.


Cystic Fibrosis


Cystic fibrosis is a complex condition that affects many different organs and causes most problems in the lungs and the digestive system in the early years.  Thick mucus in the lungs and in the digestive system causes repeated chest infections and difficulty in gaining weight.  Other symptoms can include a troublesome and tiring cough.


Over time the infections leave tissue damage in the lungs that can affect lung function. Physiotherapy and medication can help to control the level of mucus in the lungs and minimise the number of infections.


The thick mucus prevents enzymes from reaching the intestines to aid digestion.


This can result in frequent, unpredictable bowel function, which can restrict normal growth and physical strength.  Digestive enzymes in capsule form are taken with food to help digest food.


Some older people with CF develop CF related diabetes and they need additional treatment to manage this.


Other problems experienced, mostly by adults, include sinusitis, nasal polyps, osteoporosis, arthritis and liver disease.

Medical Treatment and Care for Cystic Fibrosis


People with Cystic Fibrosis receive their health care from a multidisciplinary team of specialist doctors, nurses, physiotherapists, dieticians and other health professionals. These professionals work together to provide nationally recognised NHS standards of care.


They will attend regular hospital appointments to have their health monitored and the CF nurse will provide outreach support to the family who provide the daily care at home.


Their transition from paediatric care, where they will have formed attachments with the medical professionals who care for them, to adult care where the personnel and the procedures are different, can be very challenging for these young people. They are supported through this transition process by both teams working together for a period of time as the young person settles into the new environment and becomes familiar with the team.


Everyone with Cystic Fibrosis will also have a daily treatment regime that is carried out at home


Treatment includes:


Physiotherapy to help clear thick mucus from the lungs and help reduce chest infections and prevent lung damage.


Parents are taught how to do physiotherapy with their child by the physiotherapist in the CF clinic. Adults with CF can learn to carry out their own physiotherapy.


There are lots of different techniques to help clear the mucus from the lungs and the CF physiotherapist will advise and train the parents and later the young person carry out effective physiotherapy for each individual.


The physiotherapist will also advise the parent on the frequency and duration of physiotherapy required and this may change as the person grows or when they have a bout of infection.


Different techniques may be introduced as the person gets older.


Antibiotics and other medication to treat repeated chest infections and breathlessness; special high fat and protein diet with vitamin supplements and pancreatic enzymes to aid digestion and prevent weight loss.