Cystic Fibrosis is a multi-system disease. The Cystic Fibrosis Trust (the national trust for CF) recommends the best care for people with CF is at a specialist CF centre by the multidisciplinary team of doctors, nurses and allied health professionals. This is what CFK supports at the Queen Alexandra Hospital for the children of Portsmouth.
The different ways Cystic Fibrosis is treated can be split in the following sections:
This is a way of clearing the thick, sticky mucus from the lungs. Parents are taught how to do this for their child by the physiotherapist in the CF clinic. Adults with CF can learn how to administer their own physiotherapy.
Physiotherapy should start from the time of diagnosis. Breathing exercises can be introduced in the form of a game from the age of two or three.
From around the age of nine, most children can start doing part of their physiotherapy for themselves. Most teenagers become completely independent and only require help if they have increased secretions.
This can be taken in various ways:
Inhaled into the lungs using nebulisers, taken orally or injected.
These drugs treat the lungs in the following ways:
Bronchodilator drugs open the airways by relaxing the surrounding muscle, they relieve tightness and shortness of breath.
Antibiotics help to treat or control persistent infection and steroids reduce inflammation in the airways.
DNase breaks down mucus making it easier to clear.
Physical activity is an important part of the treatment process. Exercise is particularly important for people with CF as it prevents deterioration of the lungs and improves physical bulk and strength.
Children with CF should be encouraged to take part in as much physical activity as possible - ideally types of exercise that make you out of breath, such as running, swimming, football or tennis.
A suitable diet is very important for people with Cystic Fibrosis. A good body weight can help people to fight chest infections and help the body have reserves should they start losing weight when they are ill.
The right diet for people with Cystic Fibrosis is high in energy (kilocalories), but the exact amount will vary by age and from person to person.
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