Logo Pic

Category: County:

Cystic Fibrosis Symptoms and Suggested Action

  • Cystic Fibrosis (CF) is the UK's most common life-threatening inherited disease.
  • Cystic Fibrosis affects over 7,500 people in the UK.
  • Over two million people in the UK carry the faulty gene that causes Cystic Fibrosis - around 1 in 25 of the population. If you do carry the gene you are unlikely to know.
  • Cystic Fibrosis 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.
  • Each week, five babies are born with Cystic Fibrosis.
  • Each week, three people lose their fight against Cystic Fibrosis.
  • Average life expectancy is around 31 years, although improvements in treatments mean a baby born today could expect to live for longer.

What causes Cystic Fibrosis?
A single faulty gene that controls the movement of salt in the body causes Cystic Fibrosis.

For a baby to be born with Cystic Fibrosis both parents must be carriers of the faulty gene as a person must inherit two copies of this faulty gene - one from each parent - to develop Cystic Fibrosis.

If both parents are carriers, a child has:

  • a one in four chance of being born with CF
  • a two in four chance of being a carrier but not having the disease
  • a one in four chance of being completely free of CF - not having the condition nor being a carrier of the faulty CF gene.

People who are particularly at risk of carrying the faulty gene can be detected by a mouthwash test.

What are the symptoms?
Symptoms usually first develop within the first year of life, but in a minority of cases may not appear until later in childhood. The severity of symptoms can vary and not all children with CF will have all the symptoms.

Lung Symptoms
In those with Cystic Fibrosis, the lungs make thicker sputum (mucus) than normal. This can trap bacteria in the small airways and lead to infection. Symptoms which typically develop include:

  • Persistent cough
  • Wheezing
  • Shortness of breath and breathing difficulties
  • Repeated chest infections

Gut Symptoms
In people with cystic fibrosis, thickened secretions block the normal flow of the digestive juices from the pancreas. This results in food not being digested or absorbed properly, in particular, fatty foods and fat-soluble vitamins (vitamins A, D, E and K). This can cause:

  • Malnutrition leading to poor growth and poor weight gain (even if the child has a good appetite and eats a lot, as the problem is with digesting and absorbing the food)
  • Large, smelly, greasy, fatty faeces (stools or motions) occur in about a third of cases
  • Bloated abdomen and tummy aches
  • Prolonged diarrhoea

Other Symptoms

  • Failure to thrive
  • Repeated sinus infections
  • Polyps forming in the nose
  • Rectal prolapse
  • Constipation
  • The sweat tastes very salty
  • Bowel obstruction in young babies.
  • Adults may develop bone disease
  • Adults may have fertility problems
  • Blockage of small ducts in the liver
  • Diabetes (Rare in children)

How is Cystic Fibrosis diagnosed?

Screening
Antenatal testing can determine early on in pregnancy whether a baby has CF and is usually offered to mothers who are recognised as being high risk of having a child with Cystic Fibrosis.

At present around two thirds of all newborn babies in the UK are screened for Cystic Fibrosis at birth using the heel prick blood test. It is hoped that all new born babies will be tested by mid 2007.

Around 1 in 10 children with cystic fibrosis are diagnosed at or shortly after birth due to a condition called meconium ileus where the gut becomes blocked with meconium - a thick, dark, sticky substance which is made in all babies' intestines before being born. Urgent surgery may be needed to relieve the blockage.

As not all babies are currently screened for Cystic Fibrosis some children will be diagnosed later after they have become unwell and developed symptoms, often involving many dirty nappies each day, chestiness and failure to thrive.

If you think that your child may have CF please go and talk to your GP about the symptoms.

Sweat test
A doctor should arrange a sweat test if he or she suspects cystic fibrosis from the symptoms. This test measures the amount of salt (sodium and chloride) in skin sweat. People with cystic fibrosis have an abnormally high salt level in sweat.

Genetic test
A genetic test can confirm the diagnosis. Some cells are scraped from the inside of the cheek. These can be tested to detect the cystic fibrosis gene.

What is the procedure for treating Cystic Fibrosis?
There is currently no cure for Cystic Fibrosis. In 1964 life expectancy was 5 years, it is now 31.

There are a number of ways to treat Cystic Fibrosis -

Physiotherapy
This is a way of clearing the thick, sticky mucus from the lungs. Cystic Fibrosis clinics will train parents to do this for their children. Physiotherapy should start from the time of diagnosis. Initially parents should do the physiotherapy, relatives or friends should also become involved to help with the routine. Breathing exercises can be introduced from the age of two or three and from the age of nine most children can start doing the physiotherapy themselves.

Exercise
Physical activity is an important part of the treatment process for people with cystic fibrosis. Exercise is particularly important for people with CF as it acts as a form of physiotherapy, helping to keep the lungs clear and also helps maintain physical fitness. People with CF should be encouraged to take exercise and participate in sport.

Medication
The lungs are treated with oral, nebulised and intravenous drugs.

Bronchodilator drugs open the airways by relaxing the surrounding muscles; they relieve tightness and shortness of breath.

Antibiotics help to treat or control persistent infection.

Steroids reduce inflammation in the airways.

DNase breaks down Mucus making it easier to clear.

In most people with CF the small channels that carry the digestive juices from the pancreas to the intestine become clogged. The enzymes then build up in the the pancreas, which becomes inflamed. Enzyme pills should be taken with meals and snacks to replace pancreatic enzymes to enable people with Cystic Fibrosis absorb and digest their food properly. Nutritional supplements may also help to compensate for ineffective digestion.

Bisphosphonates can be used to treat weak/brittle bones caused by Cystic Fibrosis.

Nutrition
As well as taking enzyme pills to help digest food most people with CF require 20-50% more calories each day than people without CF, however some may need considerably more than this. It is important for those with CF to maintain a healthy weight as it helps the body deal more effectively with chest infections or weight loss caused by illness.

Is Cystic Fibrosis Contagious?
No - it is a genetic illness. A baby is born with Cystic Fibrosis and will have the condition for life.

What care is available for Cystic Fibrosis sufferers and their families?
If a child is diagnosed with Cystic Fibrosis a multidisciplinary team treats them and the family is put in contact with the Cystic Fibrosis Trust.

This article was written using information provided by the Cystic Fibrosis Trust.

The Cystic Fibrosis Trust was founded in 1964, since then the Trust has been working to improve the lives of people with Cystic Fibrosis, raise the profile of Cystic Fibrosis and to fund research into a cure.

The main objectives of the Trust are -

To fund medical and scientific research to develop a cure and provide effective treatments for Cystic Fibrosis.

Ensure appropriate clinical care for Cystic Fibrosis sufferers

Provide information, support, and where appropriate, financial assistance to anyone affected by Cystic Fibrosis.

To find out more about Cystic Fibrosis and the Cystic Fibrosis Trust visit www.cftrust.co.uk

NOTE:
The health section of Parenting.co.uk is not to be used as a substitute for your GP; if your child is ill then seek the advice of a qualified doctor or other health professional without delay.

 

What do you think?
Login or Register to add a comment or ask a question.

parenting divider

FURTHER PARENTING HELP

Parenting.co.uk offers a range of options tailored to your individual parenting needs. Help options include both telephone and email based support. Please note there are charges for these services. If you would like to find out more about how we can help you lead a more fulfilling life as a parent or overcome specific problems you are having email andy@parenting.co.uk with details of the areas you would like help, support and guidance.

LOOKING FOR SOMETHING? TRY THE PARENTING.CO.UK DIRECTORY

The Parenting.co.uk Directory covers the entire range of parenting subjects from health to activities, shopping to education and sports to days out. You can also search your local area for everything from drama lessons to babysitters and sports clubs to baby photographers.

Check out the directory here: Parenting Directory

ADVERTISE YOUR BUSINESS IN THE PARENTING.CO.UK DIRECTORY

If you are looking to reach thousands of parents and families to boost your business then advertising in our directory is a simple way for you to do it.

Join our directory here: Join the Directory

Other Sections: Local Stuff : Days Out : Health : Education : General Help : Competitions : Directory : Forum : Holidays : Shopping : Blog