Medical management of cystic fibrosis.

The manifestations of cystic fibrosis make it a complex disease, in the 
management of which the respiratory physician, the microbiologist, the gastroenterologist and 
last but not least, the psychologist, all are or should be 
involved. About 1 in 10 babies with cystic fibrosis are born 
with acute intestinal obstruction due to plugging of the small intestine 
with very sticky meconium. Diagnosis is made by the test for 
excess salt in the sweat, and although possible very soon after 
birth, it is easier to carry out at about three weeks 
of age when the baby sweats more freely. The great majority 
of children, if not treated, tend to suffer progressively more frequent 
chest infections or chronic chest infections. The reason for the tendency 
of the lungs of children with cystic fibrosis to become colonised 
by harmful bacteria is not known. Most cystic fibrosis children absorb 
carbohydrate normally, but only about 50% (range 45% to 80%) of 
the protein and fats they eat. The only other major problem 
for which any active measures may be required is the occurrence 
of fibrosis of the liver in about 5% of these children. 
Children with cystic fibrosis should be brought up from the beginning 
to be like other children. 