What is cystic fibrosis?
Cystic Fibrosis (CF) is an inherited life-threatening condition. It affects many organs in the body, in particular the lungs and digestive tract. Normally cells in the body make secretions and mucus, but in cystic fibrosis these cells do not work properly and the secretions and mucus are much thicker than they should be. This thick, sticky mucus then causes blockages in the lungs and digestive tract making it difficult to breathe and digest food properly.
Just under 300 children are born in the UK with CF every year. It occurs in approximately 1 in 2,500 children. All newborn babies born in the UK are screened for CF shortly after birth as part of a blood test known as the Guthrie test or 'heel prick' test.
The blood is tested for CF along with other inherited conditions such as sickle cell anaemia and phenylketonuria. Pregnant women who are identified as a high risk of having a child with CF can also be tested using the chorionic villus sampling (CVS) technique. This is usually done around the tenth week of pregnancy.
What causes cystic fibrosis?
CF is caused by a faulty gene which controls the movement of salt and water in and out of cells within the body. Approximately 1 in 25 people carry this genetic defect. A baby born to parents who are both carriers of this gene will have a one in four chance of having the condition. If a child inherits just one cystic fibrosis gene, they will also become a carrier but will not develop the condition.
What are the symptoms of cystic fibrosis?
CF is a 'multi system' condition - this means that the excessive build-up of thick mucus affects several organs in the body although primarily the lungs and digestive tract are affected.
Symptoms will usually develop before a baby reaches a year old although some children can develop symptoms later in their childhood.
Lungs
In a healthy person, a continuous flow of mucus helps remove bacteria from the surfaces of the air passages in the lungs. With CF, this mucus is thick and sticky so is unable to remove bacteria effectively - the mucus also provides a perfect environment for the bacteria to live and grow, so a CF sufferer is more at risk of contracting bacterial infections. If these infections are not treated promptly they can be very hard to remedy and this can lead to permanent lung damage.
Digestive system
Cystic fibrosis also affects the digestive system. In a healthy person, the pancreas produces enzymes which help break down fat. In CF the pancreas does not produce these enzymes which means that the fat in foods is not absorbed by the body - this leads to poor weight gain and growth.
Other conditions associated with cystic fibrosis include:
- Liver disease
- Diabetes
- Brittle bones
- Delayed puberty
- Infertility problems.
How is it treated?
There is no cure for CF, but people with the condition are now living into their 30s and beyond. A baby born with CF today is likely to live much longer than this due to advancements in research and treatments.
Children with CF usually need daily sessions of physiotherapy and breathing exercises to clear the lungs and help prevent the build-up of mucus. Parents of young children are usually trained to carry out these vigorous massages, but older children and adults are usually trained to do this for themselves.
With each meal they also need to take enzyme supplements to help them digest their food. They should eat high-energy snacks and take vitamin and mineral supplements for extra nutrients. Children with CF also take daily medication which helps to clear the mucus and protects them against developing infections.
Caring for a CF child in a childcare setting
Parents/carers can spend a lot of time deciding on the right childcare for their child. For those who have a child with cystic fibrosis it is important they feel confident that their childcare provider has a good basic knowledge of the condition.
Staff should work with parents/carers to ensure they know how best to manage the child's condition. They are likely to need a high-calorie diet and possibly help with taking their daily supplements and medication.
- Further information: Contact the Cystic Fibrosis Trust on 0845 859 1000, www.cftrust.org.uk
