Section 10 - Cystic Fibrosis Clinical Care Pathway

3.2 Pancreatic Insufficiency
Pancreatic insufficiency is present in about 85-90% of the CF population.
Patients who are homozygous for the common ∆F508 mutation have a 99%
chance of being pancreatic insufficient. This can lead to a variety of
manifestations, including steatorrhoea, malnutrition, fat-soluble vitamin
deficiency, growth failure and rectal prolapse. Distal intestinal obstruction
syndrome (DIOS) can also occur.
Pancreatic elastase can be measured on a 1 cubic centimeter stool sample, at
any age. Specimens are sent to virology (for the attention of David Cubbitt)
at GOSH for analysis. Normal levels are in excess of 500µg/g. CF patients
usually have levels below 200µg/g. In pancreatic insufficient CF patients
undetectable levels are frequently reported.
The gold standard for assessing pancreatic insufficiency is by direct sampling
and measurement of the pancreatic juices in the second part of the
duodenum after stimulation. In practice, this is rarely done and is restricted
to specialist paediatric gastroenterology centres.
3.2.1 Pancreatic enzyme supplementation
There is no standard enzyme dose. The correct dose is that which
symptomatically corrects steatorrhoea, abdominal pain and decreases
frequency and mass of stools. More enzymes may be needed with a fatty meal
or if stools are loose, frequent, offensive, pale and oily. Completely normal
stools may never be achieved in some cases.
Infants: Enteric coated mini microsphere enzyme preparations (Creon 10000,
Solvay) are started in young infants. The starting dose is usually a quarter
capsule per feed. The gelatine capsule is opened and the granules mixed with
a little soft food such as fruit puree and given at the start of the feed.
Alternatively, it can be mixed with some breast or formula milk on a spoon
but should not be added to the baby’s bottle.
Children: patients either swallow the capsules whole (over five years) or
empty the granules directly into their mouth. The granules should not be
chewed or crushed as this destroys the enteric coating. High doses of enzyme
have been linked to the occurrence of colonic strictures (fibrosing
colonopathy, see section 3.7). The use of high strength preparations is now
discouraged in children and an upper limit of 10,000 international Lipase
Units/Kg body weight/day has been suggested.
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