About the authors
Ms Sharon Hunt
Dr Peter Cooper
Download full article (.pdf 110kB)
Cystic fibrosis clinics are located at the following hospitals:
Cystic fibrosis is the most common genetic disease in Caucasian people. It affects approximately one in every 2500 children born in Australia.
Cystic fibrosis was first recognised as a condition in the 1930s. At that time affected children rarely lived to their teenage years. However, with greatly improved treatments and understanding of the condition, average life expectancy is now well into the third decade, with many affected people living longer than this.
Cystic fibrosis is a disease caused by a change (mutation) in a person’s genetic makeup. This mutation brings about an abnormality in the way salt and water move in and out of the cells of the body, causing the body to secrete thick, sticky mucus. This leads to difficulties in the lungs, pancreas, digestive system and reproductive organs. It is usually diagnosed at birth through the newborn screening program.
There is no cure for cystic fibrosis, so treatment is life long and is based on the prevention of illness and the management of symptoms. It is in no way contagious – people must be born with this condition to have it.
A person with cystic fibrosis has inherited the disease by either having:
Cystic fibrosis does not affect the student’s intellectual abilities and in general children with cystic fibrosis are keen to learn and do well at school.
Children with cystic fibrosis may be smaller in stature and thinner than their peers. Most live with a level of lung disease, which is usually mild in children, as well as gastrointestinal difficulties.
Another feature of cystic fibrosis is shortness of breath. It is reasonable to allow children at school to sit and catch their breath if they need to, especially when doing vigorous sports.
People with cystic fibrosis will cough frequently due to increased amounts of sputum in the lungs. The severity and frequency of coughing episodes varies between individuals.
Three important points:
There are several parts of the gastrointestinal system that may be affected by cystic fibrosis. The pancreas and the intestines are the most significant. The majority of people with cystic fibrosis (85%) are pancreatic insufficient. This means the pancreas is unable to produce the enzymes required to digest food properly.
Most people with cystic fibrosis still experience a degree of malabsorption which potentially causes:
These students may require extra toilet privileges and extended time in the bathroom.
It is not advised to make the student wait until the next break if he or she needs the toilet.
Children with cystic fibrosis lose significantly more salt in their sweat, which increases the chance of becoming dehydrated. This is especially problematic in summer or during periods of increased exercise. Salt supplements and fluid replacement are used to minimise this.
During periods of prolonged high temperatures students with cystic fibrosis:
Other complications of cystic fibrosis can include:
Every person is affected differently and may be affected by some, none, or all of these potential complications. More information on individual students and their management can always be sought from parents or staff at the hospital the child attends, in conjunction with the parent or carer.
Many factors contribute to fatigue in children with cystic fibrosis – for example, waking early to undergo treatments, chronic
infection, dehydration and decreased nutrition. It is helpful to bear this in mind when setting homework tasks and with requests
Management of cystic fibrosis involves medication, enzyme supplements, physiotherapy, a highly calorific diet and may require various treatment aids. Hospitalisation for intravenous antibiotics or routine monitoring of the condition will mean absence from school at various times.
Due to the effects of poor absorption and the body’s use of energy to deal with infection, children with cystic fibrosis require substantially more calories to grow than do children without cystic fibrosis.
In order to achieve this, children with cystic fibrosis have a diet that is high in fats, proteins and carbohydrates. In addition, a high salt diet is necessary due to salt loss.
It is important not to criticise the high calorie, high fat, high salt diet required for these children. When looking at healthy diets in class, it would be helpful to note that whilst there is a standard diet considered healthy for the general community, there are some people who have different needs.
Some children with cystic fibrosis have implanted devices to aid their management, including:
If a student has a port or a gastrostomy button, the parent/carer or treating physician will inform the school of this.
Whilst cystic fibrosis does not affect the brain in any way, it must be remembered that these students have a chronic illness. Aspects of the disease can cause lethargy, dehydration, or anxiety, which may all affect school performance from time to time. Understanding is required around need for medication, treatment, school absence and possible work volume allowances.