MUCOVISCIDOSIS/CYSTIC FIBROSIS

Definition of Mucoviscidosis/Cystic fibrosis:
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
of Mucoviscidosis/Cystic fibrosis:
Faulty gene on chromosome 7. For the disease to occur, both parents have to pass on the gene mutation.

Symptoms/signs/effects of Mucoviscidosis/Cystic fibrosis

  • Sweat containing salt
  • Recurrent obstructive bronchitis
  • 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, polyps

Diagnosis of Mucoviscidosis/Cystic fibrosis:

  • Sweat test
  • Lung diagnostics
  • Gastrointestinal diagnostics

Treatment of Mucoviscidosis/Cystic fibrosis:

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 of Mucoviscidosis/Cystic fibrosis:

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