Enzymes

Question

Dear ladies and gentlemen,
my daughter has Cystic Fibrosis. She has DF508 and G542X. She is 3.5 years old. We live in Santigo, Chile. She was born with meconium ileus and has been operated within 24 hours after birth. She got an ileostomy. in October, 2011, her bowel has been reanastomozed successfully. At the moment she is doing quite well, even if her cultures of the lung show from time to time Stapyhlococcus aureus. My daughter got in her first year of life intravenous treatments, combined with oral antibiotics. But instead of getting rid of Staphyloccus, the therapy in a public hospital had as a consequence, that she got additionally E. coli. She has no symptoms at the moment, no cough, twice daily physiotherapy.
My questions is about the enzymes. She is taking Panzytrat® 10.000 at the moment, 7 capsules per meal, with 4 meals per day. She weighs 14kg. She has bowel movements at least once daily, sometimes 2-3 times. The consistency seems to be normal, and is not fatty. To adress the dosage: she is taking 70.000 IU lipase per meal, what is in my opinion much for her weihgt. The leaflet of Panzytrat® advises a maximum dosage of 10.000 IU lipase per kilogram body weight, whereas my daughter takes 20.000 IU. On the other hand it is difficult for her in spite of the high dosage, to gain weight. In Chile, only a single physician recommended me to keep this high dosage and even to increase it, in case her weight should again decrease. All other physicians are of the opinion, that the dosage is simply too high and I have to reduce it. I have even tried this, with the result, that my daughter stopped gaining weight.
What is your opinion on the dosage of the enzymes? She is investigated two times a year with ultrasound and none of the results showed blockage of the bowel or the like. Her pancreatic insufficiency is absolute. Furthermore, it is not clear to me, how long the enzymes can have an effect after intake. A meal for her lasts about 45 minutes. What do you think could be a better alternative? To give all enzymes at the beginning of the meal or half of them at the beginning of the meal and the other half in the middle of the meal? How long are the enzymes effective after intake? Is yoghurt suitable for the enzyme intake? I do this varaint for breakfast and in the afternoon, as my daughter does not like milk.
I would be very glad about some information.
Yours sincerely,

Answer

Hello,
you report about your 3.5 year-old girl, who has been born with meconium ileus and who got an ileostoma within the first 22 hours of life, that could be replaced in the course of time. You report, that your daughter takes 7 capsules of Panzytrat® à 10.000 IU of lipase for each meal. With 4 meals a day, she has a daily dosage of 280.000 IU lipase with a body weight of 14 kg. You worry about this high dosage, as in the leaflet a maximum dosage of 10.000 IU per kg body weight and day is recommended and because nearly all CF physicians in Chile judge the given dosage to be too high.
Generally it can be said about the therapy with pancreatic enzymes in case of CF, that the individual need is very different. In here it is not infrequently like this, that children, who had a meconium ileus, have a higher need for enzymes in the following time. It is right, that one should only in rare cases exceed the dosage of 10.000 IU lipase and that in those cases, close controls are necessary, in order to depict possible side effects early. Justifiably, you point at the individual intake of the enzymes. As your daughter needs quite a lot of time for a meal, it would be good, if you give the enzymes in three portions. 3 capsules at the beginning, 2 capsules in the middle and 2 capsules at the end. Also you should not give always 7 caspules per meal, however you should orientate yourself to the amount of fat in the meal. Here the standard value of about 2.000 IU lipase per gram fat pertains. A dietary specialist could help you with the calculation of the fat. Probably you come on this way to an a bit reduced amount of enzymes, without that the weight gain stagnates.
In some patients, also the intake of so-called proton pump inhibitors (omeprazole) is necessary, in order to enhance efficacy of enzymes and therefore come to a reduced dosage of enzymes. Sometimes it also helps, to change the product, that means to change from Panzytrat® to Kreon®.
Furthermore you should discuss with your CF physician in charge, if a preparation of Rizopuslipase (Nortase®) can be ordered via an international pharmacy. You could replace then about a third of the product Panzytrat® with the Nortase®, without that this lipase has to be counted on the maximum dosage.
I hope to have helped you with my advice and wish you and your daughter all the best.
Dr. H.-G. Posselt

17.12.2014