What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 1 Intolerance Sucrose Intolerance Due to Congenital - 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 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 . Sucrase-isomaltase is the list of foods may surprise The information in this booklet is designed to be educational. We encourage produced in the and helps break you. Here are you to share it with your healthcare provider. ONLY a doctor can properly down sucrose into and , which are just a few... diagnose you. used by the body as fuel. It is also one of several Flavored Yogurt that helps digest . 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 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 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.

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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 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 , leading to a symptoms include abdominal distention, gassiness, misdiagnosis of (IBS). For the Doctor colic, excoriated buttocks, , 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 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.

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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. , 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.

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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. from Congenital Sucrase-Isomaltase Deficiency

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A Relief of P 81% Symptoms Patients who became asymptomatic with Sucraid ® in a clinical trial.* † *Patients who took Sucraid ® with each meal were considered asymptomatic if they reported no GI Indication symptoms for at least 7 of the ® 10 study days. Sucraid (sacrosidase) Oral Solution is an enzyme replacement therapy for the treatment of genetically CSID †Treem WR, McAdams L, Stand - ford L, Kastoff G, Justinich C, determined sucrase deficiency, which is part of congenital Hyams J. Sacrosidase sucrase-isomaltase deficiency (CSID). therapy for congenital More Common Than sucrase-isomaltase deficiency. You Think! J Pediatr Gastroenterol Nutr. Important Safety Information for 1999;28:137-142. Sucraid ® (sacrosidase) Oral Solution n Sucraid ® may cause a serious allergic reaction. If you Please see additional notice any swelling or have difficulty breathing, get Important Safety emergency help right away. Information on page S2 and enclosed n Tell your doctor if you are allergic to, have ever had a Find out more. Call: 1-800-705-1962 Email: [email protected] Full Prescribing reaction to, or have ever had difficulty taking yeast, yeast Visit: CSIDDiseaseInfo.com or Sucraid.com Information. products, papain, or glycerin (glycerol). © 2019 QOL Medical, LLC. All Rights Reserved. 3405 Ocean Drive, Vero Beach, FL 32963 S1 CSIDDiseaseInfo.com

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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 QOL Sucraid Oral Solution Rev. 11/14 Size: 10.5 x 6.5 Type: Insert: 5 pt Pat Instr. 10 pt. Side A 12/3/14 JB

Sucraid® (sacrosidase) Oral Solution: a sucrose challenge (a measurement of excess hydro- gen excreted in exhalation) in CSID patients has shown PATIENT PACKAGE INSERT Sucraid can help improve the breakdown and absorption of DESCRIPTION levels as great as 6 times that in normal subjects. Sucraid® (sacrosidase) Oral Solution is an enzyme sucrose (table sugar) from the intestine and can help relieve replacement therapy for the treatment of genetically A generally accepted clinical definition of CSID is a con- determined sucrase deficiency, which is part of con- dition characterized by the following: stool pH < 6, an INFORMATION FOR PATIENTS the gastrointestinal symptoms of CSID. genital sucrase-isomaltase deficiency (CSID). increase in breath hydrogen of > 10 ppm when chal- lenged with sucrose after fasting and a negative lactose CHEMISTRY breath test. However, because of the difficulties in diag- Sucraid® (sacrosidase) Oral Solution Sucraid is a pale yellow to colorless, clear solution with nosing CSID, it may be warranted to conduct a short Sucraid does not break down some sugars resulting from a pleasant sweet taste. Each milliliter (mL) of Sucraid therapeutic trial (e.g., one week) to assess response in contains 8,500 International Units (I.U.) of the enzyme patients suspected of having CSID. the digestion of starch. Therefore, you may need to restrict sacrosidase, the active ingredient. The chemical name Please read this leaflet carefully before you take Sucraid® of this enzyme is ß,D-fructofuranoside fructohydrolase. CLINICAL STUDIES the amount of starch in your diet. Your doctor will tell you The enzyme is derived from baker’s yeast A two-phase (dose response preceded by a breath (sacrosidase) Oral Solution or give Sucraid to a child. (Saccharomyces cerevisiae). hydrogen phase) double-blind, multi-site, crossover trial was conducted in 28 patients (aged 4 months to Please do not throw away this leaflet. You may need to read if you should restrict the amount of starch in your diet. It has been reported that the primary amino acid struc- 11.5 years) with confirmed CSID. During the dose ture of this protein consists of 513 amino acids with an response phase, the patients were challenged with an it again at a later date. This leaflet does not contain all the apparent molecular weight of 100,000 g/mole for the ordinary sucrose-containing diet while receiving each Discuss the following important information with your glycosylated monomer (range 66,000-116,000 g/mole). of four doses of sacrosidase: full strength (9000 Reports also suggest that the protein exists in solution I.U./mL) and three dilutions (1:10 [900 I.U./mL], 1:100 information on Sucraid. For further information or advice, doctor before you begin to take Sucraid: as a monomer, dimer, tetramer, and octomer ranging [90 I.U./mL], and 1:1000 [9 I.U./mL]) in random order from 100,000 g/mole to 800,000 g/mole. It has an iso- for a period of 10 days. Patients who weighed no more ask your doctor or pharmacist. electric point (pI) of 4.5. than 15 kg received 1 mL per meal; those weighing Tell your doctor if you are allergic to, have ever had a Sucraid may contain small amounts of papain. Papain more than 15 kg received 2 mL per meal. The dose did is known to cause allergic reactions in some people. not vary with age or sucrose intake. A dose-response BEFORE TAKING SUCRAID Papain is a protein-cleaving enzyme that is introduced relationship was shown between the two higher and the reaction to, or have ever had difficulty taking yeast, yeast in the manufacturing process to digest the cell wall of two lower doses. The two higher doses of sacrosidase WARNING: Sucraid may cause a serious allergic reaction. the yeast and may not be completely removed during were associated with significantly fewer total stools products, papain, or glycerin (glycerol). subsequent process steps. and higher proportions of patients having lower total symptom scores, the primary efficacy end-points. In If you notice any swelling or have difficulty breathing, get Sucraid contains sacrosidase in a vehicle comprised of addition, higher doses of sacrosidase were associated Tell your doctor if you have diabetes. With Sucraid, sucrose glycerol (50% wt/wt), water, and citric acid to maintain with a significantly greater number of hard and formed emergency help right away. Before taking your first and the pH at 4.0 to 4.7. Glycerol (glycerin) in the amount stools as well as with fewer watery and soft stools, the (table sugar) can be absorbed from your diet and your consumed in the recommended doses of Sucraid has secondary efficacy end-points. second doses, be sure that there are health professionals no expected toxicity. Analysis of the overall symptomatic response as a blood glucose levels may change. Your doctor will tell you This enzyme preparation is fully soluble with water, function of age indicated that in CSID patients up to 3 nearby (within a few minutes of travel) just in case there is milk, and infant formula. DO NOT HEAT SOLUTIONS years of age, 86% became asymptomatic. In patients if your diet or diabetes medicines need to be changed. CONTAINING SUCRAID. Do not put Sucraid in warm or over 3 years of age, 77% became asymptomatic. Thus, an allergic reaction. hot liquids. the therapeutic response did not differ significantly CLINICAL PHARMACOLOGY according to age. Side effects to watch for: Congenital sucrase-isomaltase deficiency (CSID) is a A second study of similar design and execution as the INFORMATION ABOUT YOUR MEDICINE chronic, autosomal recessive, inherited, phenotypically first used 4 different dilutions of sacrosidase: 1:100 (90 heterogeneous disease with very variable enzyme Some patients may have worse abdominal pain, vomiting, I.U./mL), 1:1000 (9 I.U./mL), 1:10,000 (0.9 I.U./mL), ® activity. CSID is usually characterized by a complete or and 1:100,000 (0.09 I.U./mL). There were inconsistent The name of your medicine is Sucraid (sacrosidase) Oral nausea, or diarrhea. Constipation, difficulty sleeping, almost complete lack of endogenous sucrase activity, a results with regards to the primary efficacy parameters. very marked reduction in isomaltase activity, a moderate Solution. It can be obtained only with a prescription from decrease in activity, and normal lactase levels. In both trials, however, patients showed a marked headache, nervousness, and dehydration have also decrease in breath hydrogen output when they received Sucrase is naturally produced in the brush border of sacrosidase in comparison to placebo. your doctor. the small intestine, primarily the distal and occurred. Other side effects may also occur. If you notice . Sucrase hydrolyzes the sucrose INDICATIONS AND USAGE into its component , glucose and Sucraid® (sacrosidase) Oral Solution is indicated as The purpose of your medicine: these or any other side effects during treatment with fructose. Isomaltase breaks down from oral replacement therapy of the genetically determined starch into simple sugars. Sucraid does not contain sucrase deficiency, which is part of congenital sucrase- Sucraid is an enzyme replacement therapy for the treatment Sucraid, check with your doctor. isomaltase. isomaltase deficiency (CSID). In the absence of endogenous human sucrase, as in CONTRAINDICATIONS of the genetically determined sucrase deficiency, which is CSID, sucrose is not metabolized. Unhydrolyzed Patients known to be hypersensitive to yeast, yeast Stop taking Sucraid and get emergency help immediately if sucrose and starch are not absorbed from the intestine products, glycerin (glycerol), or papain. part of congenital sucrase-isomaltase deficiency (CSID). and their presence in the intestinal lumen may lead to WARNINGS any of the following side effects occur: difficulty breathing, osmotic retention of water. This may result in loose Severe wheezing, 90 minutes after a second dose of CSID is a condition where your body lacks the enzymes stools. sacrosidase, necessitated admission into the ICU for a wheezing, or swelling of the face. Unabsorbed sucrose in the colon is fermented by bac- 4-year-old boy. The wheezing was probably caused by needed to break down and absorb sucrose (table sugar) terial flora to produce increased amounts of hydrogen, sacrosidase. He had asthma and was being treated with methane, and water. As a consequence, excessive gas, steroids. A skin test for sacrosidase was positive. and other sugars from starch. How to take your medicine: bloating, abdominal cramps, nausea, and vomiting may Other serious events have not been linked to Sucraid. occur. Each bottle of Sucraid is supplied with a plastic screw cap PRECAUTIONS Chronic malabsorption of disaccharides may result in Care should be taken to administer initial doses of The symptoms of CSID often include frequent watery diar- which covers a dropper dispensing tip. Remove the outer malnutrition. Undiagnosed/untreated CSID patients Sucraid near (within a few minutes of travel) a facility often fail to thrive and fall behind in their expected where acute hypersensitivity reactions can be ade- rhea, abdominal pain, bloating, and gas. In many cases, the growth and development curves. Previously, the treat- quately treated. Alternatively, the patient may be tested cap and measure out the required dose. Reseal the bottle ment of CSID has required the continual use of a strict for hypersensitivity to Sucraid through skin abrasion symptoms of CSID are similar to other medical problems. sucrose-free diet. testing. Should symptoms of hypersensitivity appear, after each use by replacing and twisting the cap until tight. CSID is often difficult to diagnose. Approximately 4% discontinue medication and initiate symptomatic and Only your doctor can make a definite diagnosis of CSID. to 10% of pediatric patients with chronic diarrhea of supportive therapy. unknown origin have CSID. Measurement of expired Skin testing as a rechallenge has been used to verify breath hydrogen under controlled conditions following hypersensitivity in one asthmatic child who displayed QOL Sucraid Oral Solution Rev. 11/14 Size: 10.5 x 6.5 Type: Insert: 5 pt Pat Instr. 10 pt. Side B 12/3/14 JB

wheezing after oral sacrosidase. effective for the treatment of the genetically acquired Write down the date the sealed bottle is first opened in the Figure 2. Mix dose in beverage or infant formula. sucrase deficiency, which is part of CSID. GENERAL Although Sucraid provides replacement therapy for the ADVERSE REACTIONS space provided on the bottle label. Always throw away the deficient sucrase, it does not provide specificreplace- Adverse experiences with Sucraid in clinical trials were bottle four weeks after first opening it because Sucraid con- ment therapy for thedeficient isomaltase. Therefore, generally minor and were frequently associated with restricting starch in the diet may still benecessary to the underlying disease. tains no preservatives. For the same reason, you should reduce symptoms as much as possible. The needfor In clinical studies of up to 54 months duration, physi- dietary starch restriction for patients using Sucraid cians treated a total of 52 patients with Sucraid. The should be evaluated in each patient. adverse experiences and respective number of patients rinse the measuring scoop with water after each time you reporting each event (in parenthesis) were as follows: It may sometimes be clinically inappropriate, difficult, abdominal pain (4), vomiting (3), nausea (2), diarrhea or inconvenient to perform a small bowel biopsy or finish using it. breath hydrogen test to make a definitive diagnosis of (2), constipation (2), insomnia (1), headache (1), nerv- ousness (1), and dehydration (1). CSID. If the diagnosis is in doubt, it may be warranted to conduct a short therapeutic trial (e.g., one week) Note: diarrhea and abdominal pain can be a part of the To get the full benefits of this medicine, it is very important with Sucraid to assess response in a patient suspected clinical presentation of the genetically determined of sucrase deficiency. sucrase deficiency, which is part of congenital to take Sucraid as your doctor has prescribed. The usual sucrase-isomaltase deficiency (CSID). The effects of Sucraid have not been evaluated in patients with secondary (acquired) disaccharidase defi- One asthmatic child experienced a serious hypersensi- dosage is 1 to 2 milliliters (mL) with each meal or snack: ciencies. tivity reaction (wheezing) probably related to sacrosi- 1mL = 1 full measuring scoop (28 drops from the bottle tip) It is recommended that approximately half of your dosage INFORMATION FOR PATIENTS dase (see Warnings). The event resulted in withdrawal See Patient Package Insert. Patients should be instruct- of the patient from the trial but resolved with no sequelae. and 2 mL = 2 full measuring scoops (56 drops from the bot- be taken at the beginning of each meal or snack and the ed to discard bottles of Sucraid 4 weeks after opening OVERDOSAGE due to the potential for bacterial growth. For the same Overdosage with Sucraid has not been reported. reason, patients should be advised to rinse themeas- tle tip). remainder of your dosage be taken during the meal or uring scoop with water after each use. DOSAGE AND ADMINISTRATION The recommended dosage is 1 or 2 mL (8,500 to snack. Sucraid is fully soluble with water, milk, and infant for- 17,000 I.U.) or 1 or 2 full measuring scoops (each full mula, but it is important to note that this product is measuring scoop equals 1 mL; 28 drops from the Measure your dose with the measuring scoop provided (see sensitive to heat. Sucraid should not be reconstituted Sucraid container tip equals 1 mL) taken orally with Storing your medicine: or consumed with fruit juice, since its acidity may each meal or snack diluted with 2 to 4 ounces (60 to Figure 1). Do not use a kitchen teaspoon or other measur- reduce the enzyme activity. 120 mL) of water, milk, or infant formula. The beverage Sucraid is available in 4 fluid ounce (118 mL) see-through USE IN DIABETICS or infant formula should be served cold or at room ing device since it will not measure an accurate dose. temperature. The beverage or infant formula should The use of Sucraid will enable the products of sucrose , glucose and fructose, to be absorbed. This not be warmed or heated before or after addition of plastic bottles, packaged two bottles per box. A 1 mL meas- Sucraid because heating is likely to decrease potency. fact must be carefully considered in planning the diet of Figure 1. Measure dose with measuring scoop. diabetic CSID patients using Sucraid. Sucraid should not be reconstituted or consumed with uring scoop is provided with each bottle. Always store fruit juice since its acidity may reduce the enzyme activ- LABORATORY TESTS ity. Sucraid in a refrigerator at 36°F - 46°F (2°C - 8°C). Protect The definitive test for diagnosis of CSID is the meas- It is recommended that approximately half of the urement of intestinal followingsmall bowel biopsy. dosage be taken at the beginning of the meal or snack Sucraid from heat and light. and the remainder be taken during the meal or snack. Other tests used alone may be inaccurate: for example, the breath hydrogen test (high incidence of false nega- The recommended dosage is as follows: If your bottle of Sucraid has expired (the expiration date is tives) or oral sucrose tolerance test (high incidence of 1 mL (8,500 I.U.) (one full measuring scoop or 28 false positives). Differential urinary disaccharide test- drops) per meal or snack for patients up to 15 kg in printed on the bottle label), throw it away. ing has been reported to show good agreement with body weight. small intestinal biopsy for diagnosis of CSID. 2 mL (17,000 I.U.) (two full measuring scoops or 56 DRUG INTERACTIONS drops) per meal or snack for patients over 15 kg in Keep this medicine in a safe place in your refrigerator where Neither drug-drug nor drug-food interactions are body weight.

expected or have been reported with the use of Sucraid. children cannot reach it. However, Sucraid should not be reconstituted or con- Dosage may be measured with the 1 mL measuring scoop (provided) or by drop count method (1 mL sumed with fruit juice, since its acidity may reduce the enzyme activity. equals 28 drops from the Sucraid container tip). QOL Medical, LLC CARCINOGENESIS, MUTAGENESIS, IMPAIRMENT OF HOW SUPPLIED ® FERTILITY Sucraid (sacrosidase) Oral Solution is available in Mix your dose in 2 to 4 ounces of water, milk, or infant for- 118 mL (4 fluid ounces) translucent plastic bottles, Vero Beach, FL 32963 Long-term studies in animals with Sucraid have not been performed to evaluate the carcinogenic potential. packaged two bottles per box. Each mL of solution con- mula (see Figure 2). Sucraid should not be dissolved in or tains 8,500 International Units (I.U.) of sacrosidase. A Studies to evaluate the effect of Sucraid on fertility or its mutagenic potential have not been performed. 1 mL measuring scoop is provided with each bottle. A taken with fruit juice. www.sucraid.net full measuring scoop is 1 mL. PREGNANCY Store in a refrigerator at 2°- 8° C (36°- 46°F). Discard For questions call 1-866-469-3773 Teratogenic effects. Pregnancy Category C. Animal reproduction studies have not been conductedwith four weeks after first opening due to the potential NEVER HEAT SUCRAID OR PUT IT IN WARM OR HOT BEV- for bacterial growth. Protect from heat and light. Sucraid. Sucraid is not expected to cause fetalharm Rx only. Rev / when administered to a pregnant woman or to affect ERAGES OR INFANT FORMULA. Heating Sucraid causes it reproductive capacity. Sucraid should be given to a 09 18 Distributed by: pregnant woman only if clearly needed. to lose its effectiveness. The beverage or infant formula Part No. 0110 QOL Medical, LLC NURSING MOTHERS Vero Beach, FL 32963 should be taken cold or at room temperature. The Sucraid enzyme is broken down in thestomach To order, or for any questions, call 1-866-469-3773 and intestines, and the component amino acidsand www.sucraid.net peptides are then absorbed as nutrients. NDC# 67871-111-04 PEDIATRIC USE Sucraid has been used in patients as youngas 5 months of age. Evidence in one controlled trial in pri- marily pediatric patients shows that Sucraid is safe and