An overview of the fibrocystic disease of the pancrease and mucoviscoidosis, also known as Cystic Fibrosis

Cystic Fibrosis


Cystic Fibrosis (CF), also known as fibrocystic disease of the pancreas and mucoviscoidosis, is an hereditary disease that can be life threatening. CF affects 1 in every 1500 of the population with at least one CF affected child born every day in the UK. It is therefore a major cause of infant mortality.

In CF, there is an increase of activity in the glands that produce mucus, sweat and digestive juices. The results of this are seen in the lungs, the sweat glands and in the pancreas.

In the lungs the mucus glands produce a thick sticky sputum which blocks the air passages causing a high suscepibilty to recurring lung infections and severe breathing difficulties.

In the sweat glands, the disease produces an unusually salty sweat. This is a means to diagnosing the condition using the `Sweat Test', where levels of sodium chloride are actually measured in the child's sweat.

In the pancreas, which produces juices needed for the digestion and absorption of fats, thickened mucus blocks the gland causing degeneration of the pancreas. The effect of this process is to interfere with the absorption of foods causing the child to develop growth problems.

CF attacks at any time between infancy and adolescence. It may show itself, in an infant often with a voracious appetite as `failing to thrive', e.g. not gaining weight. Because the child cannot digest fats, they are passed out the body causing loose, greasy, smelly, bulky stools. An affected child may also suffer recurring chest infections leading to lung distruction and increasing shortness of breath.

As CF patients can't produce pancreatic juices, they must be given an equivalent in the form of a medication which will have to be taken with every single meal for the rest of their life! They must also take a high protein diet and a good supply of vitamins. Attention must be paid to the prevention and rapid treatment of lung infections. Special physiotherapy techniques are taught to parents to help drain the child's lungs of thickened mucus.

If CF is not diagnosed and treated early, it causes irreversible damage to the lungs, which may prove fatal. However, with more advanced methods of diagnosis and treatment, over two thirds of CF sufferers now survive into adulthood.

IMPORTANT NOTICE : This content is from the Dr Chris Steele personal archive and is provided for convenience only. Information contained here may no longer agree with the most up to date medical advice. Please check with a medical professional before taking any action.

More like this
Babies and Children
Illnesses
Treatments
Use this page



Privacy Terms | Advertisers | Legal Disclaimer | Contact
© 2007 TheFamilyGP.com