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Cystic Fibrosis – Condition and Treatment

Cystic Fibrosis (SIS-tik fi-BRO-sis), or CF, also known as mucoviscidosis, is an inherited disease of the secretory glands. Secretory glands include glands that make mucus and sweat. Although it mainly affects mostly the lungs, it also the pancreas, liver, kidneys, intestines, sinuses, and sex. Long-term issues include difficulty breathing and coughing up sputum as a result of frequent lung infections.

Other symptoms include sinus infections, poor growth, fatty stool, clubbing of the finger and toes, and infertility in males, among others.

Different people may have different degrees of symptoms.

These secreted fluids are normally thin and slippery. But in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.

Although Cystic fibrosis is a life-threatening disorder that causes severe damage to the lungs and digestive system, recent improvements in screening and treatments mean most people with cystic fibrosis now live into their 20s and 30s, and some are living into their 40s and 50s.

Lack of Cures for Cystic Fibrosis

There is no cure for cystic fibrosis. Lung infections are treated with antibiotics which may be given intravenously, inhaled, or by mouth. Sometimes the antibiotic azithromycin is used long term. Inhaled hypertonic saline and salbutamol may also be useful.

Lung transplantation may be an option if lung function continues to worsen. Pancreatic enzyme replacement and fat-soluble vitamin supplementation are important, especially in the young. While not well supported by evidence, many people use airway clearance techniques such as chest physiotherapy.

The average life expectancy is between 37 and 50 years in the developed world. Lung problems are responsible for death in 80% of people.

Treatment for Cystic Fibrosis

Treatment for CF can be intensive and time-consuming. At present, there is no cure for CF. Treatment aims to slow the progression of the condition and includes:

  • Chest physiotherapy
  • Antibiotics
  • Inhalations via a compressed air pump and nebuliser
  • Enzyme replacement capsules with meals and snacks
  • Well balanced diet high in protein, fat and kilojoules
  • Supplementary vitamins
  • Salt supplements
  • Regular exercise

Regular attendance at a major CF clinic is beneficial and recommended.

Some Lung Conditions Attributed to CF 

How Does AirPhysio Help Cystic Fibrosis?

By using the AirPhysio device to clean the airway and condition the lungs, this will assist in the clearance of excess mucus and fluid and assists in the maintenance of Cystic Fibrosis.

AirPhysio assists in 2 distinct ways:

1. Firstly the clearance process of AirPhysio assists in clearing any blockage or build-up of excess mucus through the 2 stage mobilisation and elimination method.

2. Secondly, the vibration or flutter effect of the AirPhysio, including the deep breathing method assist in conditioning the airway and assists to improve lung capacity through a physiotherapy method of exercising the lungs, similar to exercise.

For more information about Bronchitis, please refer to the following web pages and articles:
Cystic fibrosis (CF)
Mayo Clinic – Cystic fibrosis
National Heart, Lung, and Blood Institute – Cystic Fibrosis

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