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Sucrose Intolerance What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 1 Sucrose Intolerance Sucrose Intolerance Due to Congenital Sucrase- Isomaltase Deficiency (CSID) Is More Common Than You Think! What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 2 Introduction What is CSID? For many patients with chronic gastrointestinal symptoms, properly CSID, sometimes referred to as Genetic Sucrase- diagnosing Congenital Sucrase-Isomaltase Deficiency (CSID) is a Isomaltase Deficiency (GSID), is a rare disease that difficult journey. With varied health issues and complicated symptoms, it affects a person’s ability to digest sucrose (a type SuCroSe may take months or years to get a correct diagnosis. The time before the of sugar) due to absent or low levels of the diges - In FooD: deficiency is diagnosed can become a very frustrating experience. However, tive enzyme sucrase- isomaltase. Sucrose (sugar) exists in nearly once a patient receives a CSID diagnosis, a positive breakthrough occurs. everything we Finally, proper care and management are within reach. Sucrase-isomaltase is instrumental in the digestion eat. In fact, of sugar and starch. Sucrase-isomaltase is the list of foods may surprise The information in this booklet is designed to be educational. We encourage produced in the small intestine and helps break you. Here are you to share it with your healthcare provider. ONLY a doctor can properly down sucrose into glucose and fructose, which are just a few... diagnose you. used by the body as fuel. It is also one of several Flavored Yogurt enzymes that helps digest starches. Instant oatmeal Salad Dressing Failure to absorb dietary sucrose and starch may energy Drinks Contents impact the absorption of other nutrients and Granola Dried Fruit disrupt the regulation of gastrointestinal function. n What is CSID? Many Frozen Meals Unabsorbed carbohydrates can inhibit gastric n What are the symptoms of CSID? Snack Bars emptying, accelerate small-intestinal transit, and Bottled Sauces n How is CSID diagnosed? contribute to malabsorption of starch, fat, and Coconut Water n Tests that aid in the diagnosis other nutrients.* Cereal n What are treatment options? Some Breads Apples Mangos Fructose oranges Grapefruits Glucose Carrots Sucrose Potatoes Bananas Cantaloupe Pineapples Gluten-Free Bread Brown rice Sucrase Enzyme *Treem WR. Congenital CSID Patients with Congenital Sucrase- sucrase-isomaltase DISEASE deficiency. J Pediatr Isomaltase Deficiency may be at risk Gastroenterol Nutr. INFO.COM for chronic malnutrition. 1995;21(1):1-14. 2 CSIDDiseaseInfo.com 3 What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 4 What are the symptoms of CSID? MoSt CoMMon Symptoms can range from mild to severe chronic, In adults, symptoms persist but may appear to be SYMPtoMS: watery, acidic diarrhea to gas, bloating, less severe than those experienced by children. diarrhea, nausea, and abdominal pain. Infants may not CSID is not a disease that a patient can “outgrow. ” onlIne gas, show symptoms of CSID until they begin to eat In some adults, symptoms may include an reSourCeS: bloating, sucrose- and starch-containing foods such as increase in bowel frequency, nausea, abdominal For the Patient sucroseintolerance.com and juices, solid foods, and medications sweetened with distention, and flatulence, although episodic watery abdominal sucrose. Watery diarrhea, failure to thrive, diaper diarrhea upon ingestion of high levels of sucrose pain sucroseintolerance.com/ rash, irritability, and acidic stools are the most may occur. In some patients, diarrhea may doctor-directory/ common symptoms in infants and toddlers.* Other alternate with constipation, leading to a symptoms include abdominal distention, gassiness, misdiagnosis of irritable bowel syndrome (IBS). For the Doctor colic, excoriated buttocks, indigestion, and vomiting. Call: 1-800-705-1962 to speak with a Normal Intestine CSID Intestine CSID Support Specialist. *Treem WR. Clinical aspects and treatment of A small number of patients may require congenital sucrase- hospitalization for diarrhea and dehydration, isomaltase deficiency. Fructose J Pediatr Gastroenterol malnutrition, muscle wasting, and weakness. Sucrose Sucrose Nutr. 2012;55(suppl 2):S7-13. Glucose Patients with confirmed CSID commonly report †Karakoyun M, Kilicoglu E, being examined for toddler’s diarrhea/irritable SMALL Sahan YO, Baran M, Unal F, INTESTINE Water 9.3% Aydogdu S. Our cases with bowel syndrome (IBS)-D, celiac disease, cystic sucrase isomaltase Sucrase † enzyme deficiency. J Gastrointest fibrosis, and food allergies. Bacteria Irritation, Dig Sys. 2015;5:6. increased motility LARGE Fermentation INTESTINE CSID may be more common Most common Gasses, organic than you think Normal acids, other osmotically Sucrase deficiency may symptoms stool active molecules be more common than CSID sufferers’ most you think. In a recent common symptoms are... retrospective analysis of 27,875 small intestinal As with pediatric patients, the symptoms in adults biopsy samples taken 56% 33% during an endoscopy vary. With the introduction of dietary sucrose, and sent to a specialty some patients may experience several severe effects laboratory for a four- panel disaccharidase from this disease, while others may experience only enzyme activity mild symptoms. assessment , 9.3% of the samples showed a sucrase deficiency. * Diarrhea ‡ Stomach pain ‡ *Nichols BL Jr, Adams B, Roach ‡ Data on file Download the App! Learn more about CSID with CSID CM, Ma CX, Baker SS. Fre - DISEASE our Augmented Reality app; search “ CSID AR .” quency of sucrase INFO.COM deficiency in mucosal biopsies. J Pediatr Gastroenterol Nutr. 2012;55(suppl 2):S28-30. 4 CSIDDiseaseInfo.com 5 What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 6 FULL PRESCRIBING INFORMATION How is CSID diagnosed? Tests that aid in the diagnosis Additional Important Safety Who MIGht Diagnosing CSID can be difficult ® hAve CSID? Information for Sucraid (sacrosidase) because the symptoms are similar to Oral Solution Infants, endoscopy DID You KnoW? Sucraid ® other gastrointestinal disorders. n Sucraid ® does not break down some sugars that come children, n The disaccharidase assay directly measures In some is ONLY from the digestion of starch. You may need to restrict the and Your healthcare provider can choose enzyme activity levels in biopsy samples societies, like Greenland amount of starch in your diet. Your doctor will tell you if you available at adults from several testing methods to obtained from the small intestine during an upper should restrict starch in your diet. can suffer eskimos and US Bioservices from CSID. help diagnose you. Here is a list of GI endoscopy . some Alaskan n Tell your doctor if you have diabetes, as your blood Specialty natives, glucose levels may change if you begin taking Sucraid ®. Pharmacy. tests and resources for your doctor n For more information about disaccharidase a low- Your doctor will tell you if your diet or diabetes medicines assay testing, call 1-800-705-1962. carbohydrate, need to be changed. For the Doctor to consider. high-protein, high-fat n Some patients treated with Sucraid ® may have worse The physician must diet may mask abdominal pain, vomiting, nausea, or diarrhea. Constipation, complete the Sucraid ® Sucrose Breath test CSID difficulty sleeping, headache, nervousness, and dehydration Prescription Form symptoms. have also occurred in patients treated with Sucraid ®. Check and fax it to n The test is noninvasive, short in duration, and with your doctor if you notice these or other side effects. 1-866-850-9155. can be administered by patients at home. n Sucraid ® has not been tested to see if it works in patients rm with secondary (acquired) sucrase deficiency. est Fo n Patients with CSID may experience symptoms ONLY T due to consumption of table sugar during the test. n NEVER HEAT SUCRAID ® OR PUT IT IN WARM OR HOT YOUR BEVERAGES OR INFANT FORMULA. Do not mix Sucraid ® with n For more information, call 1-800-705-1962. fruit juice or take it with fruit juice. Take Sucraid ® as prescribed *DOCTOR by your doctor. Normally, half of the dose of Sucraid ® is taken OR CSID DISEASE before a meal or snack and the other half is taken during the HEALTHCARE 4-4-4 At-home Food INFO.COM meal or snack. Sucraid ® should be refrigerated at 36°F-46°F (2°C-8°C) and PROVIDER Because CSID n should be protected from heat and light. CAN Challenge symptoms are Step 1: Stir 4 tablespoons of ordinary table similar to other ORDER n gastrointestinal You are encouraged to report negative side effects sugar into a 4-ounce glass of water. Mix until disorders, of prescription drugs to the FDA. Visit A TEST www.FDA.gov/medwatch or call 1-800-FDA-1088. sugar is completely dissolved. patients are often n Step 2: Drink it on an empty stomach. misdiagnosed or delayed in ® n Step 3: See if symptoms such as bloating, gas, reaching mith and diarrhea occur during the next 4-8 hours; a diagnosis. Dr. S this suggests Sucrose Intolerance is possible. (sacrosidase) Oral Solution n NOTE: This test is not appropriate for infants, young children, geriatric patients, and those with Please see additional co-morbid conditions like diabetes. Patients may Important Safety Call: 1-800-705-1962 Information on page have severe symptoms if they are very sensitive S1 and enclosed to sugar. A physician should be consulted before Email: [email protected] Full Prescribing this test is taken. Information. 6 CSIDDiseaseInfo.com 7 Sucraid.com S2 What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 9 What are treatment options? Sucraid ® (sacrosidase) Oral Solution is the only FDA-approved enzyme replacement therapy indicated for the treatment of genetically determined sucrase deficiency. Ask your doctor if Sucraid ® is right for you. Clinical Trials Confirm Efficacy for Use of Sucraid ® in Both Children SuCrAID ® and Adults the goal with Sucraid ® therapy is to eat as normal and O as healthy TM P a diet as E Sucrose Intolerance N possible.
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