Cystic fibrosis Cystic fibrosis

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Ms Sharon Hunt
Cystic Fibrosis Clinical Nurse Consultant
Cystic Fibrosis Clinic
The Children’s Hospital at Westmead

Dr Peter Cooper
Consultant Paediatric Respiratory Physician
Director, Cystic Fibrosis Clinic
The Children’s Hospital at Westmead
NSW Australia

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Cystic fibrosis clinics are located at the following hospitals:

  • The Children’s Hospital at Westmead
    Ph: +61 2 9845 0719

  • Sydney Children’s Hospital
    Ph: +61 2 9382 1111

  • John Hunter Children’s Hospital
    Ph: +61 2 4985 5407

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:

  • a parent who is heterozygous for the disease, being a carrier without symptoms (heterozygote), or

  • parents who have cystic fibrosis themselves, or

  • received a mutated gene for cystic fibrosis from each parent (homozygote).

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.

  • The mucus which normally lines and cleans the airways is thicker and stickier than that secreted by people who do not have cystic fibrosis.

  • This thick mucus traps infection and blocks the small airways in the lungs.

  • The lungs are unable to clear this mucus in the normal fashion, so people with cystic fibrosis may experience severe and frequent chest infections. These infections are not contagious.

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:

  • This cough is not contagious.

  • It is important that coughing is not discouraged. Students with cystic fibrosis must cough to clear their lungs. They should not be made to feel ashamed of their coughing or have unnecessary attention drawn to it, for example by asking them to leave the classroom.

  • Although coughing bouts may sometimes be severe and sound alarming, people with cystic fibrosis will not choke from coughing.

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:

  • abdominal pain and cramping

  • bloating, bowel obstruction and urgency when needing to use the toilet.

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:

  • may be more tired and have difficulty concentrating

  • must have access to fluids and be able to drink when they want to

  • may need to be excused from physically strenuous exercise in hot weather.

Other complications of cystic fibrosis can include:

  • asthma [Link to Asthma article

  • diabetes [Link to Diabetes article]

  • liver disease

  • sinus disease

  • delayed puberty and reproductive issues (in males with cystic fibrosis the vas deferens is usually obstructed, resulting in sterility).

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 for extensions.

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.

  • Medications used in the management of cystic fibrosis are numerous and their administration is time-consuming. They are usually given at home, orally and via nebuliser. Many patients will also require hospitalisation from time to time for intravenous medication.

  • The use of medication at school is consciously kept to a minimum. It may be necessary, however, for the student with cystic fibrosis to take medication during school time.

  • Around 85% of children with cystic fibrosis will require enzyme supplements to help them digest their food.

  • They must be taken at the same time as eating most foods, except fruits, vegetables and sugar lollies. Students will usually carry the enzyme capsules with them at school in their lunch box or school bag.

  • Enzyme supplements are available without a prescription. However, they are prescribed in order to obtain the Pharmaceutical Benefits Scheme (PBS) benefit. They are not harmful to others if accidentally consumed.

  • All patients with cystic fibrosis should have chest physiotherapy once or twice per day to help clear their lungs of the thick sputum that is produced.

  • The formal physiotherapy is conducted outside of school. However, informal forms of physiotherapy can be carried out at school in the form of physical exercise.

  • Hospitalisation for intravenous antibiotics is a part of treatment for many patients with cystic fibrosis, although the frequency is different for each individual.

  • Most large paediatric hospitals have a hospital school that will arrange to continue the work the student is doing at the school he or she usually attends.

  • Students will need to have absent days to attend routine clinics to monitor progress, and there may need to be a number of sick days at home.

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:

  • ports

  • gastrostomy buttons

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.