What is Cystic Fibrosis - page

What is Cystic Fibrosis?

Symptoms - Therapy
Molecular Biology of C.-F

 

Cystic Fibrosis

Cystic Fibrosis (Mucoviscidosis) or simply CF, is one of the most frequent hereditary illnesses effecting the lungs, and to a lesser degree, the pancreas.

Viscous mucus / sputum lines the bronchi, offering the perfect basis/milieu for bacteria and inflammation.
The ducts of the pancreas suffer similarly, viscous mucus blocks them, prohibiting the entry of sufficient digestive enzymes into the small intestine making a life-long substitution of pancreatic enzymes necessary.
An insulin - dependent DM can also develop in CF.


The following symptoms can be an indication of CF:

  • Salty-tasting skin due to the high salt content of sweat (already) seen in babyhood
  • Chronic bronchitis
  • Drum....
  • Insufficient weight gain
  • Food-stuff intolerance, especially fatty foods
  • Severe stomach/abdominal pains accompanied by flatulence
  • Chronic diarrhoea
  • Nasal polyps
  • Sinusitis.

Only consistent therapy can hold off the illness or delay it's progression:

  • Regular inhalations
  • Continuous medication
  • Autogenous drainage / postular drainage
  • Physiotherapy
  • High caloric intake
  • Regular sport
  • Timely intervention of IV Antibiotics
  • To be consistent in the use of natural healing
  • Education about all accounts of illness, therapy, etc.
  • Psychological support through the illness-progress.
Cystic Fibrosis natural healing
Top

 

Molecular biology of CF

Cystic Fibrosis (Mucoviscidosis): : illness caused by thick viscous mucous in secretory organs, most frequent autosomal- recessive hereditary decease in the white population. Characteristics from which it got its name: Viscous mucous being secreted in the bronchial/ respiratory track and digestive organs, causing progressive damage. This leads to tissue becoming fibrous with cavities of fluid (cysts) in there organs.


Organs concerned:

  • Lungs: displacement of the bronchi and bronchioles by viscous mucous. This leads to breathing being impeded, oxygen deficiency and to chronic infections due to permanent static sites of bacteria.

  • Liver / gall bladder: Blockage of the fine gall bladder 'corridors' impedes the availability of gall-fluid, which is necessary for digestion. This can lead to liver cirrhosis, although relatively seldom seen in CF.

  • Pancreas: Displacement of the canals, demise of the secretion-forming cells: scarring of the organ. Digestive-disturbance as a result of insufficient digestive fluids can cause under- nourishment and growth impairment. Fibrous (fibrotic), scarred tissue can inhibit the production of insulin, leading to diabetes.

  • Small intestine: blockage can be caused by meconium ( the first bowel content of the new-born) often necessitating surgical intervention.

  • Nose & throat glands: due to the secretory anomaly, this is often the site for descending bronchial bacterial infections - ENT complications.

  • Skin: as a consequence of the impaired sweat glands, sweat contains an unusually high amount of sodium chloride. Measuring this content is part of the diagnostic process in CF.

  • Cell-defects: impaired chloride transport; the bronchial epithelial layer ( this is the inner and outer lining, often secretory, of the organs) controls the metabolism between inner and outer layer - it can give up none, or too little chloride into the respiratory tract; the disturbed chloride transport causes the cells to take up even more sodium. The disturbance of the ion-milieu leads to the mucous film becoming firm, extending over the respiratory tract. Conversely with sweat: the epithelial cells of the exit canals / channels of the sweat glands should reabsorb from the chloride, here, although in the other direction, the chloride transport is disturbed.

  • Causes of the impaired transport: the CL (chloride?) is channelled in and out through a canal. This canal is represented by the CFTR-protein.

  • The genetic fault: in CF, the CFTR-gene sitting on chromosome 7 is defective, causing the formation of a 'false' CFTR protein. More than 1000 mutations of the CFTR gene have been discovered. In the most frequent mutation - Delta F 508 - 3 nucleotides are missing from one place. This means that on the position 508 of the protein, the AS Phenylalanin is not built in.
 Research results of the last years have led to new therapy forms being developed.
Copyright © by Jason Kuhlmann 1998. All rights reserved.


Top      Home - to the main page