Cystic Fibrosis Treatment

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What is the treatment of CF?

1.     Physiotherapy

cystic fibrosis

cystic fibrosis

Physiotherapy is a way of clearing the thick, sticky mucus from the lungs. Parents are taught how to do this for their child by the physiotherapist in the CF clinic. Adults with cystic fibrosis can learn how to manage their own physiotherapy.

Why is chest physiotherapy important?

Chest physiotherapy is important because helps to prevent the thick, sticky lung secretions from blocking the air tubes. This helps to reduce infection and prevent lung damage.

What different physiotherapy techniques are there?

There are a wide variety of techniques of clearing airways  and a Specialist Cystic Fibrosis Physiotherapist will assess a person with CF and advise on the most appropriate technique to use, and also on the length and frequency of treatment sessions. The technique used may change wth the passage of time. The amount of mucus you clear will also vary as your disease progresses.

Some techniques are done without any equipment and focus on specific breathing exercises:

  • Active Cycle of breathing Techniques (ACBT)
  • Autogenic Drainage

Other techniques use a device to help clearance of mucus, by using positive pressure to hold open the airways, and some also create vibrations within the airways:

  • Positive Expiratory Pressure (PEP)
  • Oscillating Positive Expiratory Pressure – e.g Flutter, Acapella

Some equipment for physiotherapy can be very expensive and are only available for use in hospital:

  • High frequency chest wall oscillation (HFCWO) – an electric air compressor connects to an inflatable jacket (vest) to vibrate your chest.

    chest physiotherapy in cystic fibrosis in infants

How much physiotherapy is needed?

The length of treatment sessions varies according to need. Daily physiotherapy is usually required and if you have a chest infection you may need to increase the  time of physiotherapy. If there are few or no secretions, treatment sessions may only need to last 10-15 minutes. If there are many secretions, it could take as long as 45-60 minutes.

The number of treatment sessions vary from person to person. Most people require two a day when all is well, increasing to four a day when necessary. If no secretions are present, some people with CF only need physiotherapy once a day.

Your physiotherapist can advise you on how much physiotherapy is appropriate.

When should physiotherapy start?

Physiotherapy should be started at the time of diagnosis.

Who should do physiotherapy?

At first, the adults that care for the child should do it. In time, relatives or friends should learn so that no one person becomes obligatory.

Breathing exercises can be introduced in the form of a game from the age of two or three. From around the age of nine, most children can start doing part of their physiotherapy for themselves.

Most teenagers become completely independent and only require help if they have increased secretions.

2.     Nutrition:

In many people with Cystic Fibrosis, the small channels that carry the digestive juices become clogged with sticky mucus. The enzymes then build up in the pancreas, which becomes inflamed and damaged over time.

The effect of Cystic Fibrosis on the pancreas varies from person to person and around 5-10% of people with CF retain some useful function. Most people need  enzyme pills to help digest food.

A suitable diet is also very important for people with Cystic Fibrosis. There should be proper diet plan for cystic fibrosis. The right diet for people with Cystic Fibrosis is high in energy (kilocalories), but the exact amount will vary by age and from person to person.

3.     Exercise:

Physical activity is an important part of the treatment process as it prevents deterioration of the lungs and improves physical strength. Children with CF should be encouraged to take part in as much physical activity as possible. Ideally types of exercise that make you out of breath such as running, swimming, football or tennis.

It is important to inform teachers at school that exercise should be encouraged, as they may be unsure whether exercise is good for people with Cystic Fibrosis.

4.     Posture and chest mobility:

The spine, ribcage and shoulders should remain fully flexible as far as possible and good posture should be maintained.

Older children or adults may need to do stretching exercises to maintain full movement of the joints and muscles around the shoulders and chest.

Your hospital physiotherapist can advise you on the right exercises and activities.

5.     Medication for Cystic Fibrosis

Cystic Fibrosis affects the lungs and the digestive tract – these areas are likely to require medication. Infections can be cleared or controlled by a variety of drugs. Here is a guide to the most commonly used medication for people with Cystic Fibrosis.

Lungs

Medication can be administered in various ways: inhaled into the lungs using nebulisers, taken orally or taken intravenously (injected). These drugs treat the lungs in the following ways:

  • Bronchodilator drugs open the airways by relaxing the surrounding muscle. They relieve tightness and shortness of breath.
  • Antibiotics help to treat or control persistent infection.
  • Steroids reduce inflammation in the airways.
  • DNase breaks down mucus making it easier to clear.

    nebulizer

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Digestive system

Cystic Fibrosis affects the pancreas, so enzyme pills should be taken with meals and snacks to replace pancreatic enzymes and enable people with CF to gain more energy from the food they eat. Your dietitian or doctor can advise you on the appropriate type/dosage of enzyme supplement.

Nutritional supplements such as high-energy drinks can also help to compensate for ineffective digestion.

Anyone suffering from CF related diabetes will need to balance food intake with appropriate diabetic treatment such as tablets or insulin.

Other affected areas

Bisphosphonates have been shown to be beneficial for the treatment of osteoporosis in Cystic Fibrosis.

Research is being carried out to investigate the benefits of high doses of vitamin D and calcium.

If someone with CF is having liver problems, they will be treated in the same way as other people with this condition. Promising results have been reported following early treatment with ursodeoxycholic acid.

Travel and Cystic Fibrosis

As medications and healthcare for those with Cystic Fibrosis have improved, it is now common for people with Cystic Fibrosis and their families to travel abroad. However there are some issues to consider when travelling if you or a family member has Cystic Fibrosis.

  • Travel insurance
  • Medication – including taking sufficient, sterilising tablets, salt tablets, antibiotics etc
  • Air travel
  • Oxygen
  • Nebulisers and voltage
  • Clothing
  • Visits to theme parks
  • Driving abroad
  • Financial assistance
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