What is Cystic Fibrosis?

 Cystic fibrosis is a genetic condition in which the lungs and digestive system become clogged with thick sticky mucus.
Symptoms usually start in early childhood and include:

  • persistent cough
  • recurring chest and lung infections
  • poor weight gain

Symptoms of cystic fibrosis usually become apparent in the first year of life.
An early sign is that an affected child’s sweat is unusually salty, which can be noticeable when you kiss your child.


Treating cystic fibrosis


There is no cure for cystic fibrosis. So the aim of treatment is to ease the symptoms and make the condition easier to live with. Treatment can also prevent or reduce long-term damage caused by infections and other complications. 
Treatment options include:

  • bronchodilators – which are a type of medication that helps expand the airways inside the lungs making it easier to breathe
  • antibiotics – to treat chest and lung infections
  • physiotherapy – there are a range of exercises that can help clear mucus from the lungs

In some cases a lung transplant may be required if the lungs become extensively damaged.


What causes cystic fibrosis?


Cystic fibrosis is caused by a genetic mutation; specifically a mutation in a gene called CFTR. A genetic mutation is when the instructions found in all living cells become scrambled in some way, meaning that one or more of the processes of the body do not work in the way they should.
The CFTR mutation allows too much salt and water into cells. This results in a build-up of thick, sticky mucus in the body's tubes and passageways. These blockages damage the lungs, digestive system and other organs, resulting in the symptoms of cystic fibrosis.

Who is affected


Cystic fibrosis is most common in white people of northern European descent. 
It is estimated that 1 in every 2,500 babies born in the UK will be born with cystic fibrosis and there are over 9,000 people living with the condition in this country.
The condition is much less common in other ethnic groups.


Screening


Babies are screened for cystic fibrosis at birth as part of the NHS newborn screening programme.A small amount of the baby's blood is taken by a heel prick and transferred onto a card. The blood sample on the card is then analysed in the laboratory for cystic fibrosis and other inherited conditions, such as sickle cell anaemia.

Outlook


The outlook has improved considerably in recent years due to advancements in treatment, although most people with cystic fibrosis will have a shorter than average life expectancy. However, this may well improve in the future.  


Websites for advice and support: http://www.cftrust.org.uk/ http://www.patient.co.uk/health/Cystic-Fibrosis.htm http://www.nhs.uk/conditions/Cystic-fibrosis/Pages/Introduction.aspx