Cystic Fibrosis – Patient Information

What is Cystic Fibrosis?

Cystic Fibrosis is a hereditary disease resulting from a defective gene which affects the body’s ability to transport salt and water in and out of the cells.

Cystic Fibrosis is a long term life limiting disease which can be managed but not cured. A combination of too much salt and insufficient water leads to a build up of thick, sticky mucus inside the body’s organs and passageways, especially the lungs and digestive system, making it difficult to breathe and digest food.

What are the Symptoms of Cystic Fibrosis?

What are the symptoms of Cystic Fibrosis?

Babies born with Cystic Fibrosis tend to be symptomatic from an early age and are generally diagnosed within a relatively short period of time. Some newborn babies with cystic fibrosis can have dificulties with their bowels which requires an operation to correct the problem.

Long term symptoms of Cystic Fibrosis can include an incessant cough, repeated chest infections, increased phlegm production, prolonged diarrhoea and poor weight gain.  It is common for people with CF to encounter difficulties with their lungs and digestive system. Physiotherapy and medication help to control lung infections and prevent lung damage.

What causes Cystic Fibrosis?

A person carries two copies of the gene associated with Cystic Fibrosis. If one of these genes is defective then this makes the person a carrier. For a child to have the possibility of inheriting the disease, a defective CF gene must be present in both parents. A child born to two carriers of the gene will not automatically inherit Cystic Fibrosis from them but instead, each child has a 1 in 4 chance of suffering from the disease.

Cystic Fibrosis Diagnosis and Treatment

Diagnosis of Cystic Fibrosis

Cystic Fibrosis is a disease which is often diagnosed at birth or shortly afterwards. Babies born with CF suffer with bowel problems and struggle to gain weight.

There is currently no cure for Cystic Fibrosis. The treatment available is designed to alleviate the symptoms and help to manage the condition more effectively to ensure sufferers can lead their lives in as normal a way as possible.

How is Cystic Fibrosis treated?

CF is treated with a combination of medication, physiotherapy and exercise:

Medication – The three main types of drugs used to treat CF in the lungs are Bronchodilator medicines to open up the airways; antibiotics to fight off infection; and steroids to ease inflammation. To aid digestion CF sufferers are also required to take pancreatic enzymes with every meal and snack. Some CF medicines such as Bronchodilators can be administered by inhalation using a nebuliser. A nebuliser is a device which transforms liquid medication into a fine mist for inhalation deep into the lungs using a mask or mouthpiece.

Physiotherapy – Daily physiotherapy is essential for clearing mucus from the airways and minimising infection. Initially physiotherapy is carried out in the home by a parent or carer. Once old enough to manage their own therapy, CF sufferers will carry out their own daily routine.

Exercise – Daily exercise is very important for maintaining good lung health and physical strength and is an integral part of treating and managing Cystic Fibrosis.

Cystic Fibrosis FAQs

  • Who suffers from Cystic Fibrosis?
    CF is a hereditary disease affecting both males and females and is commonly diagnosed within the first year of a baby’s life.
  • How important is diet to a person with CF? 
    Diet is particularly important for Cystic Fibrosis sufferers. Cystic Fibrosis can also affect the pancreas, blocking the transfer of enzymes required for digestion. As food is not digested correctly fat is lost rather than being absorbed by the body and a diet rich in calories and protein is therefore important for maintaining a healthy body weight.
  • I use a nebuliser for taking my medication. Can I use this when travelling abroad?
    It is recommended that you check the voltage requirements for your unit before travelling. Always check with the device manufacturer’s recommendations for travel as you may require a voltage converter or adapter.
  • How do I know if I am a carrier of the gene associated with Cystic Fibrosis?
    Most carriers are unaware that they have the Cystic Fibrosis gene. If there is a history of CF in the family or you are concerned, it is recommended that you seek professional medical advice.
  • What is the advantage of using a nebuliser for my medication?
    Nebulisers transform liquid medication into a mist which can be inhaled through a mask or mouthpiece. This method administers medication deep into lungs where it is most needed.
  • As a sufferer of cystic fibrosis, will I require oxygen?
    In severe cases it may be necessary for a person suffering from CF to receive oxygen to help with their breathing. This is usually due to reduced lung capacity in more advanced or severe cases of the disease. Oxygen can be delivered in the home using an oxygen concentrator. The advantages of oxygen therapy for CF patients include a more restful sleep and improved overall feeling of wellbeing.