Section 10 - Cystic Fibrosis Clinical Care Pathway
Section 10 - Cystic Fibrosis Clinical Care Pathway
Section 10 - Cystic Fibrosis Clinical Care Pathway
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3.2 Pancreatic Insufficiency<br />
Pancreatic insufficiency is present in about 85-90% of the CF population.<br />
Patients who are homozygous for the common ∆F508 mutation have a 99%<br />
chance of being pancreatic insufficient. This can lead to a variety of<br />
manifestations, including steatorrhoea, malnutrition, fat-soluble vitamin<br />
deficiency, growth failure and rectal prolapse. Distal intestinal obstruction<br />
syndrome (DIOS) can also occur.<br />
Pancreatic elastase can be measured on a 1 cubic centimeter stool sample, at<br />
any age. Specimens are sent to virology (for the attention of David Cubbitt)<br />
at GOSH for analysis. Normal levels are in excess of 500µg/g. CF patients<br />
usually have levels below 200µg/g. In pancreatic insufficient CF patients<br />
undetectable levels are frequently reported.<br />
The gold standard for assessing pancreatic insufficiency is by direct sampling<br />
and measurement of the pancreatic juices in the second part of the<br />
duodenum after stimulation. In practice, this is rarely done and is restricted<br />
to specialist paediatric gastroenterology centres.<br />
3.2.1 Pancreatic enzyme supplementation<br />
There is no standard enzyme dose. The correct dose is that which<br />
symptomatically corrects steatorrhoea, abdominal pain and decreases<br />
frequency and mass of stools. More enzymes may be needed with a fatty meal<br />
or if stools are loose, frequent, offensive, pale and oily. Completely normal<br />
stools may never be achieved in some cases.<br />
Infants: Enteric coated mini microsphere enzyme preparations (Creon <strong>10</strong>000,<br />
Solvay) are started in young infants. The starting dose is usually a quarter<br />
capsule per feed. The gelatine capsule is opened and the granules mixed with<br />
a little soft food such as fruit puree and given at the start of the feed.<br />
Alternatively, it can be mixed with some breast or formula milk on a spoon<br />
but should not be added to the baby’s bottle.<br />
Children: patients either swallow the capsules whole (over five years) or<br />
empty the granules directly into their mouth. The granules should not be<br />
chewed or crushed as this destroys the enteric coating. High doses of enzyme<br />
have been linked to the occurrence of colonic strictures (fibrosing<br />
colonopathy, see section 3.7). The use of high strength preparations is now<br />
discouraged in children and an upper limit of <strong>10</strong>,000 international Lipase<br />
Units/Kg body weight/day has been suggested.<br />
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