People with cystic fibrosis would be treated with help and advice from a team of healthcare professionals at a cystic fibrosis centre. With regular visits, the patient or parent can learn how to best manage the condition. As each case is different, they can receive tailored care for their or their child’s condition.

There is no cure for cystic fibrosis. The aim of treatment is to ease the symptoms and make the condition easier to live with. It can also prevent or reduce the long-term damage caused by infections and other complications.

The different types of treatment for cystic fibrosis are detailed below.

Medication

Medical treatments for cystic fibrosis can help clear and control infections in the lungs and digestive system. They can also be used to treat some of the other health problems related to cystic fibrosis.

Lungs and airways

1. Bronchodilator drugs are inhaled to help the person breathe more easily. They are used for asthma and relax the muscles that surround the airways in your lungs, helping them to open up.
2. Antibiotics are taken to fight infections in the lungs. They can be taken by mouth as pills or suspensions, be inhaled through a nebuliser (a device that turns drugs into a mist that can be breathed in), or they may be given intravenously (through a tube into a vein) if the infection is more severe. All young children diagnosed with cystic fibrosis will be started on a course of oral antibiotics to protect them from certain bacteria, which will be continued until they are three years of age. For more advice on the use of antibiotics.
3. Steroids reduce the swelling of the airways, which can help with breathing. Steroid nasal drops and sprays can also be used to treat nasal polyps (small growths inside the nostrils).
4. DNase is an enzyme, usually inhaled, which helps to thin and break down the sticky mucus in the lungs so it is easier to cough up.

Digestive system

1. Pancreatic enzymes should be taken before and during every meal and fat-containing snack or drink. They help the digestive system break down food so that it can be digested and absorbed. The number of enzyme capsules taken needs to be adjusted depending on the amount of fat in the meal, snack or drink. The enzymes should be taken with the meal and the timing may vary depending on the age of the person with cystic fibrosis. It is essential that people with cystic fibrosis receive advice about enzymes from a specialist cystic fibrosis dietitian.
2. Fat-soluble vitamin supplements (A, D, E and K) are taken to help replace lost vitamins and to prevent deficiencies. Because people with cystic fibrosis lose fat in their stools, they also lose the fat-soluble vitamins. 
3. Nutritional supplements can help compensate for poor digestion and give additional energy and nutrients.
4. Insulin. People who have diabetes as a result of their cystic fibrosis will need to take insulin and manage their diet to stabilise blood sugar levels. The dietary advice usually given to people with diabetes who do not have cystic fibrosis does not apply to most people with cystic fibrosis-related diabetes.

Other medicines

1. Bisphosphonates can be taken to treat osteoporosis (weak and brittle bones), which can occur as a result of cystic fibrosis. Bisphosphonates help maintain bone density and reduce the risk of fractures.
2. Vaccinations and flu jabs. It is particularly important that people with cystic fibrosis are up to date with all the required vaccinations. People with cystic fibrosis should make sure they have an annual flu jab, as they are more susceptible to complications as a result of infection.

Physiotherapy

The management of cystic fibrosis with physiotherapy differs for each person with the condition, and is specifically tailored to their needs.

Traditionally, physiotherapy for cystic fibrosis focussed mainly on airway clearance (clearing mucus from the lungs). This still makes up a large part of daily treatment, but the role of the physiotherapist in cystic fibrosis has expanded to include daily exercise, inhalation therapy, posture awareness and, for some, the management of incontinence.

There are many airway clearance techniques. A specialist cystic fibrosis physiotherapist will assess a person with cystic fibrosis and recommend the most appropriate technique to use. The technique may change as the person gets older or as their disease changes.

Airway clearance

Some airway clearance techniques are done without any equipment and focus on specific breathing exercises, such as:

• active cycle of breathing techniques (ACBT)
• autogenic drainage

Other techniques use a device to help with the clearance of mucus. The devices use positive pressure to hold open the airways. Some also create vibrations in the airways. Techniques include:

• positive expiratory pressure (PEP)
• oscillating positive expiratory pressure 

Some techniques use large pieces of equipment, which can be very expensive and are usually only available for use in a hospital, such as:

• high frequency chest wall oscillation (HFCWO), where an electric air compressor connects to an inflatable jacket (vest) to vibrate the chest

A physiotherapist will advise on the most appropriate technique to use and also on the length and frequency of treatment sessions. 

Daily physiotherapy is usually required and, if the person with cystic fibrosis has a chest infection, they may need to increase the amount of airway clearance they do.

To encourage independence, older children and adults should use a technique that they can do by themselves and not need help with.

Lung transplants

In severe cases of cystic fibrosis, when the lungs stop working properly and all medical treatments have failed to help, a lung transplant may be recommended.

Both lungs need to be transplanted as they will both be affected by the condition. A lung transplant is a serious operation that carries certain risks, but it can greatly improve the length and quality of life for people with severe cystic fibrosis.

Lung transplantation for cystic fibrosis has a good success rate: 70% of patients survive one or two years after transplantation and the longest surviving patients had their transplant operation over 15 years ago

Related posts:

1. Causes and diagnosing cystic fibrosis
2. Cystic fibrosis
3. Fungal infections: Aspergillosis
4. Bronchiectasis
5. Medicines and other treatments for obstructive pulmonary disease