What is cystic fibrosis and what are the symptoms?
INCURABLE illness cystic fibrosis impacts thousands of lives in the UK, making it hard for sufferers to breathe or go about their daily lives.
Every one in every 2,500 babies are diagnosed with the genetic mutation and sadly there is no cure for cystic fibrosis.
What is cystic fibrosis?
Cystic fibrosis is a condition that affects the lungs and digestive system.
The lungs particularly become clogged with thick, sticky mucus.
It can lead to breathing problems and trouble digesting food.
In most cases, the condition is diagnosed in childhood.
Over the years, a person’s lungs are damaged to the extent that they can stop working properly.
In 2016 cystic fibrosis claimed the life of campaigner Kirstie Tancock, who inspired many to become organ donors.
More recently in 2022, ex-CBBC star Chelsie Whibley lost her life to cystic fibrosis.
What causes cystic fibrosis?
It is estimated that around one in every 2,500 babies are born in the UK every year with the condition, which is also known as CF, mucovoidosis, or mucoviscidosis.
One person in 25 carries the faulty gene, often without knowing.
The faulty gene means some cells in the body cannot move salt and water across the cell wall.
- recurring chest infections
- difficulty putting on weight
- frequent, wet-sounding coughs
- diarrhoea
- occasional wheezing and shortness of breath
Those diagnosed with the disease are also at risk of other conditions, including diabetes, osteoporosis and liver problems.
What is the life expectancy of someone with cystic fibrosis?
The average life expectancy for someone with cystic fibrosis can vary, with the current predicted age just 48 years.
CF patients aged 12 years and older who either have two copies of the F508del mutation or one copy of the F508del mutation and a copy of one of the other 14 licensed mutations could be prescribed Symkevi (tezacaftor/ivacaftor) in combination with ivacaftor.
People with the condition also use inhaled and injected drugs to treat their symptoms and often take enzyme pills.
Lung transplants are also common for people with cystic fibrosis but other organs affected also include the kidneys or pancreas.
Many people with the illness end up taking a mixture of treatments to manage and hopefully improve their condition.