CYSTIC FIBROSIS

Definition:
An inherited, congenital metabolic disease that is incurable. A faulty gene means that the glands in the body (e.g. in the lung) produce thick mucus, which may already cause major symptoms in childhood. The mucus obstructs the fine branches of the bronchi, the ducts of the pancreas and the bile ducts so that the organs in the body can no longer function properly. The disease may take a very different course from patient to patient: sometimes only individual organs are affected and sometimes quite a number. The extent of organ damage also varies between patients.

Causes / trigger:
Mutated gene on chromosome 7. For the disease to occur, both parents have to pass on the gene mutation.

Symptoms / signs / effects:

  • Sweat containing salt
  • Recurrent obstructive obstructive
    Increased resistance in the airways that reduces the amount of air inhaled in each breath and the oxygen that reaches the pulmonary arteries
    bronchitis bronchitis
    Bronchitis is an inflammation of the bronchi, the larger and medium-sized air passages of the lung.
  • Chronic cough
  • Failure to thrive
  • Diarrhoea with greasy stools
  • Intolerance of greasy foods
  • Inflammation of the pancreas
  • Diseases of the upper respiratory tract, e.g. sinusitis sinusitis
    Sinusitis or rhinosinusitis is an inflammation of the paranasal sinuses.
    , polyps

Diagnosis:

  • Sweat test
  • Lung diagnostics
  • Gastrointestinal diagnostics

Treatment:
Respiratory tract:

  • Administration of mucolytics and antibiotics as well as bronchospasmolytics
  • Drugs used to reduce swelling of the mucous membranes and to control mucus (e.g. inhalation with saline solution or brine)
  • Physiotherapy: Postural drainage and percussion, autogenic drainage, breathing exercises
  • Long-term oxygen therapy
  • With a drastic deterioration in pulmonary function
  • Lung transplant

Gastrointestinal treatment:

  • Pancreatic enzymes
  • Substitution of liposoluble vitamins
  • High-calorie diet