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Cystic Fibrosis

What is it? What are the symptoms? What are the treatments?

What is Cystic Fibrosis?

Cystic Fibrosis (CF) is a life-threatening inherited disease that affects the internal organs, especially the lungs and digestive system, by clogging them with thick sticky mucus. This makes it hard to breathe and digest food. There are around 9,000 people living with CF in the UK today.

Around one person in 25 in the UK carries the faulty gene for CF, and if a couple both carry the gene, their babies have a one in four chance of having the disease.

You can find out if you are a carrier of the faulty gene by using a simple mouthwash. It's really important to find out, especially if your partner is a known carrier. There's also a test you can have in pregnancy if you're considered at high risk of having a baby with CF.

Following a successful campaign by the Cystic Fibrosis Trust, all newborns are now screened for CF via blood taken during the heel-prick Guthrie test.

Infertility can be a problem for people with CF. Most male patients have blocked sperm ducts and, although females do produce fertile eggs, their cycles can be sporadic because of underweight caused by nutritional problems. Problems with nutrition occur because the pancreas is also affected by CF. Longer term, some patients develop type 2 diabetes.

Other problems associated with CF include brittle bone disease.

Research is going on all the time to improve the lifespan of people living with CF.


What are the symptoms of Cystic Fibrosis?

Early symptoms of CF include a persistent cough; repeated chest infections, ongoing diarrhoea and poor weight gain, although these symptoms are not exclusive to CF.

Because diagnosis is most often made through screening tests, parents usually know if their baby has CF very early on.

People with CF are more prone to hayfever and sinusitis.


What are the treatments and remedies of Cystic Fibrosis?

Babies can be weaned in exactly the same way as healthy babies at around 6 months, but they'll be prescribed extra vitamin supplements, too.

Physiotherapy plays a large part in the treatment of CF, and you'll be taught how to do this for your child. Sometimes the hospital may need to use special devices to clear mucus from the airways. Breathing exercises are also important, and your child can be taught these from a young age.

Your child may have to take enzyme pills to help her digest food. This is because the small vessels that carry digestive juices can also become clogged up with sticky mucus. A high-calorie diet will also be prescribed.

Exercise is very important to help keep the lungs healthy, so you should encourage your child to take up sport, dance, swimming or another activity she enjoys.

Medication to treat the lungs can be given either intravenously (into a vein) or through a nebuliser (inhaled medication). Your child may need frequent courses of antibiotics to control or treat persistent infections, and sometimes steroid medication is also prescribed to reduce the inflammation of the airways. Mucus-reducing medicines can also be given.

If diabetes is diagnosed, your child may need to take tablets or have insulin injections.

This guide 

The information in this Bounty A-Z of Family Health is not a substitute for an examination, diagnosis or treatment by a doctor, midwife, health visitor or any other qualified health professional. If in doubt, always speak to a doctor.

Bounty will not be held responsible or liable for any injury, loss, damage, or illness, however this occurs or appears, after using the information given on this website and in particular the A-Z of Family Health.

Further help

For health advice and information 24 hours a day, 365 days a year, the NHS offers call and web services. You can also visit NHS websites for services, health information and health news at nhs.uk 

  • England – call 111 from any landline or mobile phone free of charge, or visit nhs.uk 
  • Scotland – call 111 from any landline or mobile phone free of charge, or visit nhs24.com 
  • Wales – call 0845 4647 , or visit nhsdirect.wales.nhs.uk 
  • Northern Ireland – visit hscni.net

 

Cystic Fibrosis