Congenital Sucrase-Isomaltase
Deficiency (CSID)
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Induction Diet
This induction diet should not be used without consulting your physician first and utilization should be monitored by a licensed dietician. Calculations for adequate carbohydrate consumption, based on age and body weight, utilizing fructose or glucose or enzyme supplementation must be completed by a licensed physician or dietician to safely utilize this diet. A copy of this induction diet, food composition tables, enzyme replacements and information on sweeteners should be given to your physician and dietician for review before beginning this diet. For the physician and dietician: The induction diet controls each week for the amount of sucrose, lactose, starch and maltose per 100 g of a specific food being added to the diet. The induction diet begins by slowly adding in foods containing sucrose increasing the load each week, adding in a sucrose enzyme supplement, then foods containing lactose, (lactose enzyme supplement if necessary) followed by foods containing starch. Finally foods which contain maltose are added last to the diet. Children and adults report varying levels of success utilizing sucrose, lactose, starch and maltose enzyme supplements. To date, more than 30 mutations that cause CSID have been identified, thus children and adults present with a wide variety of food tolerances with and without enzyme supplementation for sucrose, lactose, starch and maltose. The phenotypes A-F are a starting point based on small bowel biopsy's of over 9,000 children and adults, but there has been a wide variation of tolerated foods reported. Regarding lactose: If the small biopsy indicates a normal level for lactase, and there is no known allergies to dairy or dairy proteins, you the physician or dietician, may wish to consider adding the dairy items listed in weeks eight through ten to the foods in week one or two. If the levels are below normal or zero you may wish to consider the use of alternate dairy products such as Lactaid milk and cheese products, or lactose enzyme replacement when age appropriate. When introducing juices, you may want to suggest to the client beginning with 1/4 strength mixed with water, soda water, diet ginger ale, or diet tonic water, then proceeding to 1/2 strength, 3/4 strength and finally to full strength due to the natural laxative effect of some fruits or that they should only be used with meals. More than 80% of our families do not use any enzyme replacement for sucrose, and limit their food intake to weeks one and two and dairy products found in weeks eight through ten if lactose levels are normal in their small bowel biopsy. If they have half to normal levels of starch enzymes in their biopsy they will also add those foods that contain less than 2 g of sucrose to their diet without an enzyme replacement. Children and adults in Phenotype D with a genetic history from New Zealand, and Australia have a reported severe reactions to foods marked with a numbers (#) sign. Upon ingestion of these foods parents report children breaking out in hives, rashes, welts, and blisters and other severe reactions. Consider avoidance of these foods for Phenotype D clients. Week One (Phenotypes A,B,C,D,E,F, without enzyme replacement for sucrose) The following foods contain 1 gram or less of sucrose and no lactose, or maltose and <1 gram of starch. Normally all children and adults with CSID can tolerate these foods without the use of an enzyme replacement. If additional information regarding a specific food please refer to the food composition tables. Children and adults may have have allergies to a specific food that is unrelated to CSID. Foods designated for each week do not need to be measured or weighed. Physicians and dieticians should be aware a child may become symptomatic with or without an enzyme replacement due to a carbohydrate load in a meal and across the day. The dietician may wish to instruct the parent to introduce one serving the first day, two the next, and three the following to determine tolerated loads per meal and in the same day. The dietician may wish families to utilize a food journal when introducing new foods. Foods in weeks one and two (except for phenotype F requiring 1 gram or less) are normally safe foods for all children and adults who have CSID without an enzyme replacement. This allows children to learn what foods are safe to eat when they do not have their medications/enzyme replacements with them at a friend's house, restaurants, with grandparents or other relatives, at school, or in the care of a babysitter.
Week Two (Phenotypes A,B,C,D,E, without an enzyme replacement for sucrose), (Phenotype F, will require an enzyme replacement for sucrose contained in these foods) These additional foods are added to the diet during this week. The following foods contain 2 grams or less of sucrose and no lactose, starch or maltose. Normally all children and adults with CSID except phenotype F (<1% of CSID population based on unique mutation) can tolerate these foods without the use of an enzyme replacement for sucrose. If additional information regarding a specific food please refer to the food composition tables.
Week Three (All Phenotypes A,B,C,D,E,F, will require an enzyme replacement for sucrose contained in these foods) These additional foods are added to the diet during this week. The following foods contain 2.1-5 grams or less of sucrose and no lactose, no starch, or maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Four (Phenotypes A,B,C,D,E,F, will require an enzyme replacement for sucrose contained in these foods) These additional foods are added to the diet. The following foods contain 5.1-7 grams or less of sucrose and no lactose, starch or maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Five (Phenotypes A,B,C,D,E,F, will require an enzyme replacement for sucrose contained in these foods) These additional foods are added to the diet. The following foods contain 7.1-10 grams or less of sucrose and no lactose, starch, or maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Six (Phenotypes A,B,C,D,E,F, will require an enzyme replacement for sucrose contained in these foods) These additional foods are added to the diet. The following foods contain 10.1-13 grams or less of sucrose and no lactose, starch or maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Seven (Phenotypes A,B,C,D,E,F, will require an enzyme replacement for sucrose contained in these foods) These additional foods are added to the diet. The following foods contain 13.1-40 grams or less of sucrose and no lactose, starch or maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements. To physicians and dieticians note: Some families report that at this point the enzyme replacement Sucraid at the dose of 1 ml for infants and 2ml for children and adults is ineffective for the following foods that contain sucrose or added sucrose in baked goods. It is likely based on a specific mutations. Most of the additional reporting families continue to progress to 20, 25, and even higher grams of sucrose per 100 ml of food with the same 1-2ml. dose of Sucraid respectively. If the child or adult falls into this category consider skipping the foods in week seven and baked goods that have more than 13 grams of sucrose added and proceed to week eight to the introduction of Dairy products. If you are skipping all dairy products proceed to week eleven.
Week Eight (Phenotypes A,B,C,D,E,F, introduction of Dairy products) These additional foods are added to the diet. The following foods contain 1 gram or less of sucrose and 2g or less lactose, no starch or maltose. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements. Children and adults with no/low small bowel biopsy results for lactose may require lactase enzyme replacement to utilize these foods.
Week Nine (Phenotypes A,B,C,D,E,F, introduction of Dairy Products continued) These additional foods are added to the diet. The following foods contain 10 grams or less of sucrose and 6g or less lactose, no starch or maltose. An enzyme replacement for sucrose will be required by all phenotypes marked with an asterisk (*). If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements. Children and adults with no/low small bowel biopsy results for lactose likely will require lactase enzyme replacement to utilize these foods.
Week Ten (Phenotypes A, B, C, D, E, F, introduction of Dairy Products continued) These additional foods are added to the diet. The following foods contain 15 grams or less of sucrose and 15g or less of lactose, no starch or maltose. An enzyme replacement for sucrose will be required by all phenotypes with an asterisk (*). If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements. Children and adults with no/low small bowel biopsy results for lactose will require lactase enzyme replacement to utilize these foods. The following foods have 30-80 grams of lactose per 100 grams and should only be considered for individuals who had normal biopsys for lactase enzynes. They include: Milk, powdered, skim or whole; Yogurt, cow, powder; and Whey, dried.
Week Eleven (Phenotypes B,C,E, introduction of starch) These additional foods are added to the diet. The following foods contain 2-5 g or less of sucrose and no lactose, 2 grams or less starch, no maltose. For the physician and dietician: All foods with greater than 2 grams of sucrose require a sucrase enzyme replacement. Some phenotype A, D, and F children and adults may be able to use the foods being added this week in very small amounts, some may require a starch enzyme replacement to do so. Most Phenotype B children and adults will tolerate between 3 and 11 grams of starch. Phenotype C children and adults can tolerate between 0 and 50 grams with 20, 25, 32 and 50g variations in tolerance. Each child is different. Some respond well to starch enzyme replacements and some do not. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Twelve (Phenotypes B,C,E, introduction of Starch continued) These additional foods are added to the diet. The following foods contain 12 grams or less of sucrose and no lactose, 2.1-5g or less of starch, no maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in foods marked with an asterisk (*). If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Thirteen (Phenotypes B,C,E, introduction of Starch continued) These additional foods are added to the diet. The following foods contain 15 g or less of sucrose and no lactose, 5.1-10 g or less of starch, no maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Fourteen (Phenotypes C,E, introduction of Starch continued) These additional foods are added to the diet. The following foods contain 2-20 g or less of sucrose and no lactose, 10.1-20 g or less of starch, no maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in foods marked with an asterisk (*). If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Fifteen (Phenotypes C,E, introduction of Starch continued) These additional foods are added to the diet. The following foods contain 2-5 g or less of sucrose, 2 g or less of lactose, 20.1-30 g or less of starch, no maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Sixteen (Phenotypes C,E, introduction of Starch continued) These additional foods are added to the diet. The following foods contain 2-5 grams or less of sucrose and 3g or less of lactose, 30.1 -50 g or less of starch, no maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in foods marked with an asterisk (*). If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Seventeen (Phenotypes C,E, introduction of Starch continued) These additional foods are added to the diet. The following foods contain 2-10 g or less of sucrose and no lactose, 50.1-100 g of starch or maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Eighteen (Phenotype C introduction of Maltose) These additional foods are added to the diet. The following foods contain 2-50 grams or less of sucrose and no lactose, 10-100 g of starch or 5 grams or less of maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
Week Nineteen (Phenotype C, introduction of Maltose continued) These additional foods are added to the diet. The following foods contain 2-50 grams or less of sucrose and 0-5 g of lactose, 10-100 g of starch and greater than 5 g maltose. All children and adults with CSID will require the use of an enzyme replacement for sucrose contained in these foods. If additional information regarding a specific food please refer to the food composition tables. For information on enzyme supplementation please refer to enzyme replacements.
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