Children with Cystic Fibrosis: Causes, Symptoms, Remedies, Treatment

Posted on October 27, 2009 by TDR in Childrens Health

What is Cystic Fibrosis?

Cystic fibrosis (CF) which is also known as mucoviscoidosis, is an autosomal recessive disorder and is major cause of pancreatic insufficiency and respiratory failure in childhood. Cystic fibrosis is characterized by generalized dysfunction of all exocrine glands, including those which secrete mucus.
Blockage by viscid secretion causes cystic changes in the pancreas and also bronchiectasis.

This is an inherited disease (that has been passed on from parents to young ones). Children with cystic fibrosis can be easily affected with infections. In such cases a number of tissues in the body produce mucus that is abnormally thick.

For example:

  • In normal cases the lining of the air passages in the lungs produce thin mucus that keeps the lining moist. But in cystic fibrosis the mucus is thickened and blocks the air passages and makes it susceptible to infection.
  • The intestine of the fetus which is filled with meconium also contains mucus. So a body with cystic fibrosis may develop a blocked intestine during the initial days of life.
  • Pancreas can also be affected by cystic fibrosis. Pancreas produces digestive juices. Food will not be digested properly if it is affected by cystic fibrosis.
    Because of this the child’s bowels are loose, fatty and foul smelling.

What You Can Do

  • There is no cure for Cystic Fibrosis.
  • To diagnose cystic fibrosis the amount of salt that is present in the sweat is tested.
  • Early diagnosis helps prevent damage to the lungs.
  • Children with Cystic Fibrosis may suffer from chest infections. Antibiotics are usually prescribed because they are likely to be bacterial.
  • Chest physiotherapy is quite beneficial for Cystic Fibrosis. The child should be made to lie with head downwards. Then pat on the chest with cupped hands to dislodge (remove) thick phlegm. Doing this daily has shown wonderful results.
  • Affected children will need special enzyme supplements and also vitamin supplements because of poor digestion and absorption.
  • Affected children should eat a low fat diet.

Causes of Cystic Fibrosis

  • Cystic fibrosis or also known as mucoviscoidosis in the new born and may be associated with intestinal obstruction due to inspissated meconeum the so called meconeum ileus, sometimes with non-canalization of the part near about the region of ceacum.
  • In surviving infants, it may be followed with respiratory disease with pneumonia and bronchio-pneumonia.
  • In older children, however, respiratory symptoms are less prominent; where as, complication like those of celiac disease are prominent. There is excessive secretion of all the glands of the body in this condition.

Symptoms of Cystic Fibrosis

  • In infancy cystic fibrosis may be present frequently with repeated attacks of respiratory infection.
  • Defective pancreatic enzymes give rise to impaired digestion and mal-absorption of the fats.
  • Stool are bulky, foamy and foul smelling.
  • The child is often poorly nourished. Complications include intussusceptions and obstruction due to faecal masses.
  • The respiratory problems become progressively more important in the adolescent and adults and determine the fate of the individual by contrast mal-absorption is less troublesome.
  • There may be bronchiectasis, recurrent haemoptysis, pulmonary fibrosis and right ventricular failure.
  • Some times there is cirrhosis leading to portal hypertension.
  • Females with cystic fibrosis may become pregnant but males are always infertile.

Cystic Fibrosis Diagnosis

Investigations
In the child, the diagnosis is established by the finding of an increase in the concentration of sodium in the sweat.

  1. Sodium (pilocarpin iontophoresis).
  2. Sodium < 85mmol/l
  3. Potassium < 25mmol/l

Minimum sample of 50ml.
These are the normal levels.

The test is difficult to interpret after adolescence, the diagnosis is then made in the presence chronic pulmonary disease, pancreatic insufficiency and a family history of cystic fibrosis.

Leave a Reply

Your email address will not be published.