Creon necessary?

Question

Hello,

our daughter (18 months) has the F508 mutation (homozygous). I am giving her Creon for children, but am playing it by ear. Usually, the exact Creon dosage is calculated precisely for each fatty meal, but that is not necessary for her since she is getting by with very little Creon. When I realize that her stool is rather hard (small little pellets), I even avoid the Creon altogether for the next meal. My question now is: How can that be? Last year her elastase level was 7. After all, this means that the pancreas is hardly working, doesn’t it? And is it allowable for me to not give her Creon every now and then? She never has diarrhoea. Does the reduced amount of Creon influence growth? She is rather small for her age, 73 cm at 17 months (desired weight 101 percent). I have to add, though, that we as parents are only about 1,70 m tall. However, the CF center will check her growth hormones in November.

Many thanks and kind regards,
A.

Answer

Hello,

you are asking whether your 18-month-old daughter, who has been diagnosed with pancreatic insufficiency, should be given pancreatic enzymes on a regular basis urgently, even though her stools seem normal to you and sometimes the stool even is rather hard. The body weight is at 101 percent of the desired weight, but her growth hormones will be checked because she is very small.

Concerning your problem, let me say that the consistency of the stool in a CF child can vary greatly despite insufficient enzyme dosage. In this situation, there sometimes are typical fat-rich stools, but sometimes also normal-looking ones up to the point of obstipation. Initially, it would be very important to check the excretion of stool fat in your daughter’s 3x24-hour stool, with concurrent logging of the food intake and enzyme dosage, in order to become more certain about the reliability of your judgement of her enzyme need. Based on this result, the doctors at the CF center in cooperation with a dietician will have to give you detailed dosage instructions.

Of course, insufficient enzyme dosage and a resultant increased stool fat excretion will lead to an unnecessary loss of energy with your child. On the other hand, however, increased stool fat excretion will, for example, result in a gradual loss of bile acids in the body, which bears the risk of liver damage. It remains to be seen whether your daughter’s arguably slow growth is caused by insufficient enzyme dosage.

Generally speaking, a CF patient’s enzyme need has to be determined via the stool fat excretion. “Stool cosmetics” via a manipulation of the enzyme dosage are not allowed. In case the patient is prone to obstipation, dietetic therapy measures have to be taken in cooperation with the treating physicians.

We hope you will become more certain about the question of enzyme dosage by the measures suggested here for your daughter’s well-being.

Kind regards
Dr. H.-G. Posselt

13.09.2010