Cystic Fibrosis (CF) is a genetic condition that causes damage to the lungs, digestive system and other organs of the body. It primarily affects cells that produce mucus, sweat and digestive juices. These secretions are generally thin and protect the body’s tubes and ducts to promote smooth pathways. In people with CF, these secretions become thick and sticky clogging up these pathways particularly the lung and pancreas.
CF is caused by a mutation in the CFTR gene. This gene assists in controlling salt and water in the body’s cells affecting the production of mucus, sweat and digestive juices.
In Australia, approximately 1 in 25 people carry this gene, most are not even aware they carry it. When carrying the gene, people do not have symptoms. Both parents must carry the gene for it to be passed to their child.
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In Australia, 1 in 2,500 babies are born with CF.
Signs and symptoms can reveal in childhood, however some people may not start experiencing symptoms until teenage years or adulthood. People who are undiagnosed until adulthood often have milder and ore atypical symptoms of the condition. Signs and symptoms can vary depending on which organs are affected and the severity of the condition. Symptoms can vary within the individual as well from 1 time to the next.
Symptoms of CS are related to the organs that the condition affects.
The lungs are the most common organ affected by CF. Thick, sticky mucus clogs up the respiratory tubes such as bronchi, bronchiole and the small sacks (alveoli) that are essential for O2 and CO2 to be exchanged.
Symptoms can include:
This thick mucus can block the tubes that transport digestive enzymes from the pancreas to the small intestine. This inhibits the body’s ability to absorb nutrients in food.
Symptoms can include:
Comprehensive input and treatment is required in the management of CF, especially during periods of inflammation. The team will usually include:
Physiotherapy is an essential part of treatment. Due to chronic mucus build up, physiotherapy helps to mobilise mucus, improve breathing, reduce risk of infection, improve lung function, increase exercise tolerance and build strength. Techniques are often taught to family members so that daily intervention can be performed between physio sessions. This is especially important at times with the person has an infection and may be required multiple times a day.
People with CF may require a lung, liver or pancreas transplant to prolong their lives.
Historically, treatment has been focused on controlling symptoms and preventing complications, however with newer types of medications ‘modulator therapies’ that target the cause of cystic fibrosis treatment is improving significantly.
My Lotus Care supports individuals and their families who have a variety of conditions. We build a multidisciplinary team involving both formal and informal supports and a variety of therapists to manage symptoms and improve independence and quality of life.
02 9889 5171
www.cysticfibrosis.org.au/
(07) 3359 8000
admin@cfqld.org.au
My Lotus Care Group supports a number of families living with ASD We provide support and education for families including:
To find out more please contact info@mylotus.com.au.
Lotus Disability Care is able to assist their participants to connect with a variety of allied Health services.