When people think about CF, they typically think of the respiratory side of the disease, but that’s only part of the condition. The digestive system is also heavily involved, and the vast majority of patients are pancreatic insufficient, meaning their pancreas doesn’t function properly because of the thick, sticky CF mucus clogging it up. It is believed that over 90% of CF patients are pancreatic insufficient and need enzymes to properly digest and absorb nutrients from food. So far, Judith is one of the very lucky minority that is pancreatic sufficient and does not need to take enzymes, although we did give them briefly early on while we were trying to figure out some GI issues.
As mentioned earlier, pancreatic enzymes help CF patients absorb nutrients, to digest carbs, proteins, and fats, and to help them gain weight. Without enzymes, patients who are pancreatic insufficient will suffer from malabsorption and will not be able to gain weight (as a side note, some patients who are pancreatic sufficient, like Judith, may still have problems with weight gain because of the disease, so weight gain issues aren’t necessarily isolated to those who are pancreatic insufficient).
Enzymes are taken with meals, and while there are some exceptions of foods that do not require enzymes, most do require them. Dosing depends on the patient, and the CF team will help determine the proper amount, but a lot of parents and patients also get really good at adjusting the dosing depending on what was consumed.
For more information on pancreatic enzymes, you can read the informational pamphlet from the Cystic Fibrosis Foundation: http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Diet/EnzymeReplacement/Nutrition-Pancreatic-Enzyme-Replacement.pdf