What is Cystic Fibrosis? Everything you need to know about the genetic condition

In the wake of the current EastEnders storyline, we take a look at the facts surrounding CF

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New character Jade is living with cystic fibrosis – but what exactly is it?

An EastEnders storyline has recently been praised by a leading charity for raising awareness of a life-shortening condition.

Bosses at Cystic Fibrosis Trust hope that ‘feisty, young character’ Jade – the daughter of Dean and Shabnam – will help to accurately portray what it’s like to live with cystic fibrosis.

But how much do we all know about the condition? Here are the facts according tothe NHS and Cystic Fibrosis Trust:

What is cystic fibrosis?

“Cystic fibrosis is a genetic condition in which the lungs and digestive system become clogged with thick, sticky mucus.

“It is caused by a faulty gene (known as the CFTR gene) which normally creates a protein that moves salt and water out of a cell.

“If this gene is defective, it results in the build up of thick, sticky mucus in the body’s tubes and passageways which damage the lungs, digestive system and other organs.

“Symptoms can vary in severity from person to person. They usually start in early childhood and include a persistent cough, recurring chest and lung infections and poor weight gain.”

Lungs

“It is common for people with cystic fibrosis to experience persistent coughing as the body tries to remove the thick mucus from the lungs. Sudden coughing fits can occur, which sometimes lead to vomiting.

“Inflammation in the lungs may cause wheezing, shortness of breath and breathing difficulties, particularly after exercise.”

Digestive system

CF can cause mucus to block the ducts in the pancreas, which produces essential food-digesting enzymes.

When it is blocked, not enough of the enzymes reach the intestines to help break down food, which can cause malnutrition and problems with passing stools.

In school-age children and older people with cystic fibrosis, the pancreas can become more damaged and diabetes can develop.

Other symptoms include sinusitis, nasal polyps, arthritis, infertility and liver damage.

How does cystic fibrosis affect daily life?

The Cystic Fibrosis Trust says that it “affects everyone differently, but for many it involves a rigorous daily treatment regime including physiotherapy, oral, nebulised and occasionally intravenous antibiotics, and taking enzyme tablets with food. Some people with cystic fibrosis will have a feeding tube overnight.”

Diagnosis

Most cases of cystic fibrosis in the UK are now identified through screening carried out early in life, before symptoms appear.

There are three main ways of diagnosing cystic fibrosis:

Newborn testing

Babies are offered screening for CF at birth as part of the NHS newborn blood spot screening programme.

Antenatal testing

A test can be done on a pregnant woman to see if her unborn baby has CF, but is usually only offered to mothers who are thought to be at high risk of having a child with the disease.

This can be carried out from 10 weeks of pregnancy. The test uses chorionic villus sampling, where a fine needle is passed through the abdomen into the womb.

Sweat testing

If someone has cystic fibrosis, their sweat will have higher levels of salt than normal. A parent may first notice their child’s symptoms when they kiss them, as their skin can taste salty.

What is the life expectancy of someone with cystic fibrosis?

According to the Cystic Fibrosis Trust, “it is misleading to suggest that there is an ‘average life expectancy’ that can be applied to everyone with cystic fibrosis.

“Life expectancy is likely to be different for people of different ages; and this is before taking into account the fact that cf can also affect people differently.

“The median predicted survival for someone with cystic fibrosis currently stands at 41 years old. This means that according to the statistics on our clinical CF Registry, currently half of those with cystic fibrosis will live to over 41 years old, although a baby born today can be expected to live longer.”

Oli Lewington, Engagement Director at the trust, said:

“We’re proud and excited to have worked with EastEnders on the introduction of Jade to the soap and we hope that this feisty, young character with cystic fibrosis will help raise awareness of this hidden, genetic condition which affects thousands of people in the UK.

To read more about the condition, visit the NHS website. You can also find advice, support and information on the Cystic Fibrosis Trust website.

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