# data[].attributes.attachment_caption
We are still reintroducing foods from the CSID food list.  In addition to sucrose and starch, fructose also seems to create a problem.  

# data[].attributes.body
Here are items we have successfully been using to treat low blood sugar that do not create gastrointestinal discomfort.

Smarties candy

Bottlecaps candy

Lego block candy made with Dextrose

Glass of Milk with a scoop of powdered Dextrose

Glass of homemade Lemonade made with powdered Dextrose

Glass of homemade Decaffeinated Iced Tea made with powdered Dextrose

# data[].attributes.attachment_caption
Here is an example of a starter CSID Elimination Diet.  A registered dietician can guide you how to take enzymes and reintroduce foods.

# data[].attributes.attachment_caption
Right now, these can only be ordered from Germany.  Intoleran will be opening a US Storefront and selling on Amazon soon.

# data[].attributes.attachment_link.description
Starchway contains the enzymes Invertase and Gluco-Amylase. It contains 7500 units of Invertase and 2500 units of Gluco-Amylase per capsule.

# data[].attributes.attachment_caption
Sucraid can only be ordered from one specialty pharmacy in the United States.  It has to be shipped overnight in a refrigerated cooler box.  

# data[].attributes.attachment_link.readable_text
If you're suffering from Sucrose Intolerance due to Congenital Sucrase-Isomaltase Deficiency (CSID), ask your doctor if Sucraid ® (sacrosidase) Oral Solution is right for you.

If you're suffering from Sucrose Intolerance due to Congenital Sucrase-Isomaltase Deficiency (CSID), ask your doctor if Sucraid ® (sacrosidase) Oral Solution is right for you.

Sucraid ® is the only FDA-approved enzyme replacement therapy for the treatment of CSID.

Congenital Sucrase-Isomaltase Deficiency (CSID), also known as Genetic Sucrase-Isomaltase Deficiency (GSID).

Sucraid ASSIST ™ is a comprehensive support program that provides information and support.

CSID is a disorder that reduces the effectiveness of enzymes that digest carbohydrates (sugar and starch). Congenital Sucrase-Isomaltase Deficiency, also known as Genetic Sucrase-Isomaltase Deficiency (GSID), is an inherited disorder that causes these digestive enzymes either to be missing or to not work as well.

About CSID

Download a CSID Doctor Discussion Guide

Talking to your doctor about your CSID symptoms - especially when they involve bloating, gassiness, and diarrhea - might seem embarrassing. But sharing these details and your medical history helps your doctor make a diagnosis.

Just as your doctor has questions for you, it's natural for you to have questions for your doctor. Using a tool like the CSID Doctor Discussion Guide helps you organize your personal information and any questions you may have so that you are prepared for your appointment.

Just click on the bar below and download the discussion guide, fill it out, and share the information with your doctor during your next visit.

Enter your email so that we may keep you up to date with the latest information about disease and drug therapy.

Download a CSID Doctor Discussion Guide

Talking to your doctor about your CSID symptoms - especially when they involve bloating, gassiness, and diarrhea - might seem embarrassing. But sharing these details and your medical history helps your doctor make a diagnosis.

Just as your doctor has questions for you, it's natural for you to have questions for your doctor. Using a tool like the CSID Doctor Discussion Guide helps you organize your personal information and any questions you may have so that you are prepared for your appointment.

Just click on the bar below and download the discussion guide, fill it out, and share the information with your doctor during your next visit.

Enter your email so that we may keep you up to date with the latest information about disease and drug therapy.

What is Sucraid®?

Sucraid ® is an FDA-approved drug for the treatment of genetically determined sucrase deficiency, which is part of Congenital Sucrase-Isomaltase Deficiency (CSID). Sucraid ® is an enzyme replacement therapy, providing a substitute for the sucrase enzyme in the small intestine that is missing or not working in people who have CSID. CSID may also be called Sucrose Intolerance, carbohydrate maldigestion or intolerance or Genetic Sucrase-Isomaltase Deficiency (GSID).

About Sucraid®

Complete Resolution

Patients who became asymptomatic* with Sucraid® in clinical trials. ¹

*Patients who took Sucraid® with each meal were considered asymptomatic if they reported no GI symptoms for at least 7 of the 10 study days. 1 Treem WR, McAdams L, Standford L, Kastoff G, Justinich C, Hyams J. Sacrosidase Therapy for Congenital Sucrase-Isomaltase Deficiency. J Pediatr Gastroenterol Nutr. 1999;28(2):137-42.

Complete Resolution

Patients who became asymptomatic* with Sucraid® in clinical trials. ¹

*Patients who took Sucraid® with each meal were considered asymptomatic if they reported no GI symptoms for at least 7 of the 10 study days. 1 Treem WR, McAdams L, Standford L, Kastoff G, Justinich C, Hyams J. Sacrosidase Therapy for Congenital Sucrase-Isomaltase Deficiency. J Pediatr Gastroenterol Nutr. 1999;28(2):137-42.

Sucraid ® is a drug that is only available by prescription, but a prescription for Sucraid ® cannot be filled by your corner drugstore. The only way to get a Sucraid ® prescription filled is through the specialty pharmacy. Call the specialty pharmacy at 1-833-800-0122.

If your doctor has prescribed Sucraid ® for you, it is either because you have been diagnosed with a sucrase-isomaltase enzyme deficiency or because your doctor suspects the reason why you have frequent GI symptoms is because the sucrase-isomaltase enzymes in your small intestine are not working. If that is the case, your doctor wishes to determine if a trial of Sucraid ® therapy would help relieve some of your GI symptoms.

Sucraid® comes in a translucent plastic bottle containing approximately 118 mL of concentrated solution, packaged with two bottles per box

Each mL of solution contains 8,500 international units (IU) of sacrosidase

A 1-mL measuring scoop is provided with each box

Watch Now

Behind the Mystery: Rare & Genetic Diseases is a special video on the balancing act of treating individuals who have CSID. This is an interview with Dr. William Treem, a pediatric gastroenterologist who has studied and treated patients with CSID for years.

QOL Medical contributed financially to the production and air time of this video, which appeared on the Lifetime Television Network. Dr. William Treem, who appears in this video, was a paid consultant of QOL Medical and was compensated for his time participating in this video. Please see the indication and important safety information for Sucraid ® below.

# data[].attributes.attachment_link.title
Sucraid® (sacrosidase) Oral Solution - Patient Information

# data[].attributes.attachment_link.description
If you're suffering from Sucrose Intolerance due to Congenital Sucrase-Isomaltase Deficiency (CSID), ask your doctor if Sucraid ® (sacrosidase) Oral Solution is right for you. If you're suffering from Sucrose Intolerance due to Congenital Sucrase-Isomaltase Deficiency (CSID), ask your doctor if Sucraid ® (sacrosidase) Oral Solution is right for you.

# data[].attributes.attachment_link.provider_name
CSID Disease Information | Congenital Sucrase-Isomaltase Deficiency

# data[].attributes.attachment_link.readable_text
In patients with Congenital Sucrase-Isomaltase Deficiency (CSID) and gastrointestinal symptoms that warrant treatment, three major treatment options exist:

Before Sucraid was available, severe restriction of dietary carbohydrate intake was the only treatment option for patients with CSID.

Given the sucrose content of Western diets and the difficulties of dietary adherence generally, patient compliance with a sucrose-free diet is challenging and often accompanied by chronic gastrointestinal symptoms and decreased body weight for a given height and age.¹⁻³ In a recent observational study, only 50% of children with CSID complied with dietary reductions of sucrose and starch, and 60 to 75% still experienced frequent diarrhea and abdominal pain, presumably due to a lack of adherence to the sucrose elimination diet.⁴

Many Foods Contain Sucrose

The average American adult consumes about 130 pounds of sugar per year, about 70 pounds of which is sucrose.⁵ Sucrose can be found in a surprising number of foods. Sucrose is the naturally-occurring sugar in many fruits, juices, and some vegetables, and is the sugar often added in baked goods and processed foods. Based on a consumption of 2,000 calories per day, sucrose makes up 10% of the total American diet.⁶

Figure 1: Adapted from Treem 2012: J Pediatr Gastroenterol Nutr.⁴

Given the amount of sucrose Americans eat every year, it is no surprise that maintaining a low sucrose diet can be challenging. Patients with CSID must avoid eating a surprising number of foods, all of which are high in starches and/or sucrose.

The following is a list of foods with high levels of starch (denoted with *) and/or sugar (more than 1 gram of sucrose per 100 grams of food):

Fruit: apples, apricots, bananas*, cantaloupe, dates, grapefruit, guava, honeydew melon, mango, nectarine, oranges, passion fruit, peaches, persimmon, pineapple, plums, tangelos, tangerines, watermelon

Vegetables: beets, carrots, cassava (yucca)*, chickpeas*, coleslaw, corn, edamame*, green peas*, jicama*, kidney beans*, lima beans*, okra, onion, parsnips*, pumpkin, snow peas, split peas*, sweet pickles, sweet potatoes*, yams*

Dairy: chocolate and flavored milks, milk shakes, sweetened condensed milk, malted milk, yogurt sweetened with sucrose or with fruits containing sucrose, processed cheese or cheese products with additives, fillers or preservatives that may contain sucrose or starch

Protein: prepared, processed, breaded, or cured meats; for example, bacon, sausage, luncheon meat, pate, liverwurst, tofu

Nuts and seeds: almonds, cashews*, chestnuts*, filberts/hazelnuts, macadamia nuts, peanuts*, pecans, pine nuts, pistachios, nut butters with added sucrose, sesame seeds

Other starches: baked goods sweetened with sucrose; for example, breads, cereals, snacks, muffins, pancakes, pastries, waffles

Fats: some mayonnaises and salad dressings

Sweeteners and ingredients: table, brown, granulated, powdered or raw sugar, beet sugar, cane sugar/syrup/juice, coconut sugar, date sugar, maple syrup/sugar, molasses, syrup, jelly, jam, brown rice syrup, caramel, corn syrup/syrup solids, dextrins, glucose polymers, maltodextrin, modified tapioca starch

Desserts: ice cream, popsicles, pudding, sherbet, sorbet, brownies, cakes, cookies, muffins, pastries, pies, whipped toppings, carob, caramel, chocolate, chocolate sauce/syrup

Beverages: all sucrose-sweetened beverages, juices made from fruits containing sucrose (see above), energy drinks, sports drinks, fruit-flavored drinks, sucrose-sweetened soda or drink mixes, specialty/flavored coffees containing sucrose

Condiments: BBQ sauce, ketchup, chutney, seasonings or sauces containing sucrose, salad dressings, sweet pickles

Enzyme Replacement Therapy

Enzyme replacement therapy with Sucraid (sacrosidase) Oral Solution offers a pharmacologic alternative to sucrose-free restricted diets. Sucraid is the only FDA-approved therapy for the treatment of genetically determined sucrase deficiency, which is part of CSID. The basis for regulatory approval of Sucraid was two clinical trials that demonstrated the ability of sacrosidase to significantly reduce breath hydrogen, which is a measure of GI symptoms in CSID patients when challenged with a normal sucrose-containing diet.6,7 In the clinical trial that quantified treatment responses, 81% of 28 children with CSID aged 5 months to 11 years became asymptomatic with sacrosidase replacement of sucrase.⁷ In both studies, study participants who received higher doses of sacrosidase had fewer stools, a greater number of formed or hard stools, as well as fewer symptoms of gas, abdominal cramping, and bloating compared to those who received lower doses of sacrosidase.6,7 In a third clinical trial, conducted over a period of several years in 32 participants diagnosed with CSID, Sucraid again demonstrated it is an effective option for the management of sucrase deficiency, reducing or eliminating the need for restrictions of dietary sucrose, as well as reducing or eliminating the symptoms of CSID in the majority of patients.8

It is important to note that CSID patients generally have a reduced capacity for the digestion of sugars from dietary starches due to reduced isomaltase and maltase enzyme activities. Sucraid does not provide specific replacement therapy for deficient starch digestion.⁹ Therefore, restricting starch in the diet is usually necessary in patients with CSID. The need for dietary starch restriction in patients using Sucraid should be evaluated on a patient-by-patient basis. Consultation with a registered dietitian experienced with the dietary management of starch for CSID patients taking Sucraid is available at www.sucraidassist.com.

Proper dosing is essential for Sucraid to be effective. The proper dosage is 1 mL or 2 mL (one or two full measuring scoops) with each meal or snack. Each full measuring scoop equals 1 mL. The proper dose of Sucraid by body weight is:

For patients weighing up to 33 lb (15 kg): 1 mL (one full measuring scoop)

For patients weighing more than 33 lb (15 kg): 2 mL (two full measuring scoops).

The appropriate dose of Sucraid is to be taken orally with each meal or snack, diluted with 2 to 4 ounces (60 to 120 mL) of water, milk, or infant formula. Because heat and acidity can decrease the potency of Sucraid, the diluting liquid should not be a warmed or acidic liquid such as fruit juice. It is recommended that approximately half of the dose be taken at the beginning of a meal or snack and the remainder taken during a meal or snack.
Practice Tip
The low-FODMAP diet is not recommended for patients with CSID. Although it is a standard diet for patients with irritable bowel syndrome (IBS), it will not address symptoms in patients with CSID since it contains many foods that are high in sucrose, including: banana, cantaloupe, grapefruit, honeydew melon, mandarin orange, orange, passion fruit, pineapple, tangelo, carrot, parsnip, potato, silver beet, sweet potato, taro, yam, gluten-free breads and cereals, 100% spelt bread, rice, oats, polenta, arrowroot, millet, psyllium, quinoa, sorghum, tapioca, lactose-free yogurt, gelato, sorbet, table sugar, golden syrup, maple syrup, and molasses.
References

1. Treem WR. Congenital Sucrase-Isomaltase Deficiency. J Pediatr Gastroenterol Nutr. 1995;21(1):1-14.

2. Gudmand-Høyer E. Sucrose malabsorption in children: a report of thirty-one Greenlanders. J Pediatr Gastroenterol Nutr. 1985;4(6):873-877.

3. Gudmand-Høyer E, Krasilnikoff PA, Skovbjerg H. Sucrose-isomaltose malabsorption. In: Draper H, ed. Adv Nutr Res. 1984;5:233-269.

4. Puntis JWL, Zamvar V. Congenital Sucrase-Isomaltase Deficiency: diagnostic challenges and response to enzyme replacement therapy. Arch Dis Child. 2015;100(9):869-871.

5. US Department of Agriculture. Table 50. U.S. per capita caloric sweeteners estimated deliveries for domestic food and beverage use, by calendar year. https://www.ers.usda.gov/data-products/sugar-and-sweeteners-yearbook-tables.aspx. Last updated September 6, 2018.

6. US Department of Agriculture. Table 51. Refined cane and beet sugar: estimated number of per capita calories consumed daily, by calendar year. https://www.ers.usda.gov/data-products/sugar-and-sweeteners-yearbook-tables.aspx. Last updated September 6, 2018.

7. Treem WR, Ahsan N, Sullivan B, et al. Evaluation of liquid yeast-derived sucrase enzyme replacement in patients with sucrase-isomaltase deficiency. Gastroenterology. 1993;105(4):1061-1068.

8. Treem WR, McAdams L, Kastoff G, Justinich C, Hyams J. Sacrosidase therapy for Congenital Sucrase-Isomaltase Deficiency. J Pediatr Gastroenterol Nutr. 1999;28(2):137-142.

9. QOL Medical, LLC. Open-label, long-term study of sucrase enzyme therapy for Congenital Sucrase-Isomaltase Deficiency. Data on file, OMC-SUC-3. 1997.

10. Sucraid [package insert]. Vero Beach, FL: QOL Medical, LLC; 2018.

Related Posts

# data[].attributes.attachment_link.title
Treatment Options for Managing CSID - CSID For Healthcare Providers

# data[].attributes.attachment_link.description
In patients with Congenital Sucrase-Isomaltase Deficiency (CSID) and gastrointestinal symptoms that warrant treatment, three major treatment options exist: Before Sucraid was available, severe restriction of dietary carbohydrate intake was the only treatment option for patients with CSID.

# data[].attributes.attachment_link.readable_text
CSID is also known as genetic sucrase deficiency and congenital sucrose intolerance. This disorder occurs with equal frequency in males and females.

Sugar

Although simple sugars are the main carbohydrate absorbed in the digestive tract, monosaccharides ( glucose, fructose, and galactose) are rarely found in human diets. Instead, disaccharides (for example, sucrose, lactose, and maltose) are more common in the human diet and must be broken down into monosaccharides by small intestinal enzymes before they can be absorbed into the bloodstream for use by the body. Without this breakdown, none of these disaccharides can be used for energy because they are too big to be absorbed by the intestine.

The small intestine is lined with finger-like projections called microvilli. The microvilli are known collectively as the brush border. The microvilli absorb nutrients from food as it passes through the small intestine.

Enzymes are proteins that catalyze (speed up) chemical reactions in the body.

The molecules that are affected by enzymes are called substrates.

Substrate molecules temporarily bind to the active site of an enzyme to form an enzyme-substrate complex.

The enzyme bends the substrate in a way that breaks the molecule apart.

The result of an enzymatic reaction is called a product.

Individuals with CSID have varying amounts of the enzymes required for starch digestion (for example, isomaltase [ palatinase] and maltase), so they may also experience gastrointestinal symptoms after eating foods containing starch.

Carbohydrates are classified as either simple or complex. Classification depends on the chemical structure of the food, and how quickly the sugar is digested and absorbed. Simple carbohydrates have one sugar ( monosaccharides) or two sugars ( disaccharides). Complex carbohydrates have three or more sugars linked together ( polysaccharides).

Many complex carbohydrates are starchy carbohydrates, which require ample time and adequate enzymatic activity in the digestive tract to be digested properly. Starch digestion depends on the combination of enzyme activities that occur at several sites in the gastrointestinal tract, from the salivary gland in the mouth, to the pancreas, to the brush border in the small intestine.

The digestion of starches begins in the mouth with an enzyme in saliva called salivary amylase, sometimes called ptyalin. Chewing grinds the food and breaks it apart, allowing salivary amylase to work more effectively. After swallowing, the starchy carbohydrates reach the stomach where gastric enzymes begin to break down food.

The stomach plays a major role in digestion by mechanically mixing and crushing the food and enzymatically by further breaking down food. The stomach also acts as a reservoir to facilitate the movement of smaller amounts of food into the small intestine at any given time. The stomach secretes a form of amylase called gastric amylase that further digests starches.

Many individuals with CSID have some of the necessary components needed for starch digestion, which includes functional salivary amylase and pancreatic amylase. Problems digesting starch arise with the decreased activity of sucrase-isomaltase and maltase-glucoamylase (brush border enzymes) in the final digestive stages. Approximately 60% to 80% of starch digestion in the small intestine is accounted for by sucrase-isomaltase and 20% to 40% is accounted for by maltase-glucoamylase.

Due to the presence of maltase-glucoamylase activity, which adds to the digestion of starches, combined with the fact that as one grows, the size of the area that produces digestive enzymes in the small intestine also grows, those with CSID may be able to gradually better tolerate starches as they get older. They should consult their healthcare provider to help determine how much dietary starch they can tolerate.
References

1. Robayo-Torres CC, Opekun AR, Quezada-Calvillo R, et al. ¹³C-breath tests for sucrose digestion in congenital sucrase isomaltase-deficient and sacrosidase-supplemented patients. J Pediatr Gastroenterol Nutr. 2009;48(4):412-8. www.ncbi.nlm.nih.gov/pmc/articles/PMC3955999/

2. Semenza G, Auricchio S.. In: Scriver CR ed. The Metabolic Basis of Inherited Disease, Volume 2. 6th ed. New York, NY: McGraw-Hill Inc.: 1989:2975-97.

# data[].attributes.attachment_link.title
About Congenital Sucrase-Isomaltase Deficiency | CSID Cares

# data[].attributes.attachment_link.description
CSID is also known as genetic sucrase deficiency and congenital sucrose intolerance. This disorder occurs with equal frequency in males and females. Although simple sugars are the main carbohydrate absorbed in the digestive tract, monosaccharides ( glucose, fructose, and galactose) are rarely found in human diets.