Kreon and pancreatic sufficiency

Question

Our child is a heterozygot for deltaF508 and R1070W, with minimum clinical manifestation so far (pancreatic sufficiency, no pulmonary complications, just episodes of severe dehydration). At 3 years of age he weighs 15 kg and is 102 cm tall, he has solid, normal stools. He never recieved Kreon or other pancreatic enzymes. In this case, of pancreatic sufficiency established by pancreatic elastase, should we administer Kreon or other pancreatic enzymes? What about vitamins A, D, E, K supplement? Thank you very much for your answers.

Answer

Hello,
Concerning the first point of your question:
in CF it is important to moitor the intestinal absorption, in order to find out which patients require an enzyme treatment. Clinical signs of malabsorption are poor weight gain and growth, abdominal distension, discomfort and loose oily pale stools. A dietary assessment, the measurment of the fat excretion in the stool over 3 days and/or the measurement of the faecal elastase can objectify the degree of malabsorption. If a child has no clinical signs and those test were unsuspicious (like seemingly in your case), the child does not suffer from pancreatic insufficiency and does not require the intake of Kreon. Kreon is a drug containig pancreatic enzymes which can cause side-effects and should be titrated according to the degree of malabsorption and not be given if it is not needed.
Concerning the second point of your question, liposoluble vitamin supplements are recomended only for the patients with pancreatic insufficiency, which is not your boy’s situation, who is in a very good shape. Also, the recommendation for liposoluble vitamins administration is made only if there are clinical signs present and if the vitamin deficit is demonstrated.
The question you asked already recieved an answer according to the guides, provided by Dee Shimmin.
"Thank you for your question regarding vitamin supplementation with ADEK for your 3 year old baby. From the information you have provided it seems like your baby is healthy, is growing well and is pancreatic sufficient.

Malabsorption of fat soluble vitamins (A, D E and K) is likely in most patients with CF particularly those who are pancreatic insufficient. Current recommendations favour routine supplementation of vitamin A, D and E for those individuals who have low blood levels or clinical evidence of deficiency.
ADEK fat soluble vitamin preparation is no longer available and has been replaced by AQUADEKS (www.aquadeks.eu). This is a complete nutritional supplement specifically designed to meet the needs of those who have difficulty in absorbing fat-soluble vitamins and nutrients (mainly CF patients who have pancreatic insufficiency).

It is recommended that your baby has his/her fat soluble blood vitamin levels checked (A D E). Based on the results your CF team can recommend a dose of fat soluble vitamins to normalise blood levels without causing hypervitaminosis. Your physician may recommend Aquadek if it is felt that your child requires supplementation with vitamins A D and E. However, complete supplementation of all fat soluble vitamins may not be necessary. In this case separate vitamin preparations may be recommended. The blood levels should be checked again in a few months and the dose of vitamin supplements adjusted accordingly. Thereafter your child should have their blood fat soluble vitamin levels measured annually.
Kind regards
Dee Shimmin
Cystic Fibrosis Trust Nutrition Working Group. Nutritional Management of Cystic Fibrosis. London. Cystic Fibrosis Trust, April 2002
Sinaasappel M, Stern M, Littlewood J, et al. Nutrition in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2002; 2: 51-75."

Best regards,
the Romanian ECORN-CF team and Dr. Daniela d'Alquen

22.03.2012