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Sugars and Sugar Substitutes

Sucrose is basic table sugar. All individuals with Congenital Sucrase-Isomaltase Deficiency (CSID) have a significantly decreased ability to digest sucrose, because they lack the enzyme sucrase. Sucrose is known by several different names, such as table sugar, white sugar, cane sugar, beet sugar, evaporated cane juice, raw sugar, brown sugar, turbinado sugar, and others (see table below).

If you or your child have CSID, you should be vigilant about reading food labels to discover hidden sugars. Names of the types of sugar contained in a food are listed in the ingredients section of a food label, while the total sugar content is listed by weight in the nutrition facts section of a food label.

Fructose is also commonly used as a sweetener in many foods. Fructose is usually tolerated fairly well by most individuals with CSID, although it is important to introduce it slowly.

Sugar Substitutes

A sugar substitute is a food additive that mimics the taste and sweetening attributes of sugar. Some sugar substitutes are natural and some are synthetic, known as artificial sweeteners. In the United States, several sugar substitutes are approved for use, including aspartame, sucralose, neotame, acesulfame potassium, and saccharin. Although there is some ongoing controversy about whether artificial sweeteners are safe for consumption, the US Food and Drug Administration (FDA) considers all five of these listed sweeteners to be “generally recognized as safe” (GRAS).

Unfortunately, there are no studies on the effects of these sweeteners or possible long-term consequences of children consuming them. For this reason, artificial sweeteners should be used in moderation. Consult a medical professional when weighing the health risks and benefits in your unique situation.

Sugar Alcohols

Some non-sugar sweeteners are known as sugar alcohols. Sugar alcohols are generally less sweet than sucrose. Sugar alcohols are usually not used when preparing food at home but are widely used in processed foods. Sugar alcohols are considered nutritive sweeteners because they provide calories, but fewer calories than sugar. Sugar alcohols are not completely absorbed by the body, which is why they sweeten with fewer calories than sugar.

A high intake of foods containing some sugar alcohols may lead to gas and diarrhea for all people, not just those with CSID. Contrary to their name, sugar alcohols are neither sugars nor alcohols. Instead, they are carbohydrates with chemical structures that resemble sugar and alcohol. As with artificial sweeteners, the FDA regulates the use of sugar alcohols.

FDA-approved sugar alcohols and novel sweeteners include erythritol, hydrogenated starch hydrolysates, isomalt, lactitol, maltitol, mannitol, sorbitol, xylitol, and stevia preparations that are highly refined (for example, Pure Via®, Truvia®). Currently, the FDA has not approved the use of whole-leaf stevia or crude stevia extracts for use as a sweetener. Approval by the FDA does not imply all these sweeteners are appropriate for you to use. See the table below for acceptable sweeteners and sweeteners to avoid.

Acceptable Sweeteners and Sweeteners to Avoid

Sweeteners Tolerated by Most Individuals with CSID
Aspartame
(NutraSweet®)
  • 200x sweeter than sucrose
  • has no calories
Dextrose
(another name for glucose)
  • a monosaccharide
  • 75% as sweet as sucrose
Fructose
(crystalline fructose or crystalline fructose syrup)
  • a monosaccharide
  • 110% as sweet as sucrose
Glucose
  • a monosaccharide
  • 75% as sweet as sucrose
Lactose
(milk sugar)
  • a disaccharide that breaks down into glucose and galactose
Sweeteners Tolerated by Some Individuals with CSID
Acesulfame-K
(acesulfame potassium, Ace-K, Sunett®, Sweet One®)
  • 200x sweeter than sucrose
  • often mixed with other sweeteners
  • has no calories
Agave nectar
  • consists primarily of glucose and fructose
  • 1.5x sweeter than sucrose
Corn syrup
  • primarily glucose syrup
Equal® Original
in blue packaging
  • contains dextrose, maltodextrin, aspartame, and acesulfame K
High fructose corn syrup
(HFCS)
  • corn syrup with some of the glucose replaced with fructose
  • sweetens similar to sucrose
  • HFCS comes in 2 main types:
    1. HFCS 55 (55% fructose and 45% glucose); commonly used in soft drinks, very similar in sweetness to table sugar
    2. HFCS 42 (42% fructose and 58% glucose); commonly used in ice cream, canned fruits, ice cream, and other sweetened processed foods
    Honey
    • consists primarily of glucose (≈38-39%), fructose (≈30-31%), and sucrose (≈1-2%)
    • 1-1.5x sweeter than sucrose
    Hydrogenated starch hydrosylates
    (HSH)
    • a mixture of several sugar alcohols
    • 40-90% as sweet as sucrose
    Inverted sugar syrup
    (invert sugar)
    • a mixture of glucose and fructose obtained by heating sucrose to produce these two components
    Maltose
    (malt sugar)
    • a disaccharide formed from two units of glucose
    Saccharin
    (Sweet’N Low®)
    • 200-700x sweeter than sucrose
    • has no calories
    Stevia
    (Truvia®, Pure Via®)
    • South American herb used as a sweetener for centuries
    • 200-400x sweeter than sucrose
    • has no calories
    Sucralose
    (Splenda®)
    • 600x sweeter than sucrose
    • sometimes maltodextrin (a starch) is used as an additive in sucralose
    • has no calories
    Sugar alcohols
    (sorbitol, mannitol, maltitol, xylitol, erythritol, lactitol)
    • neither a sugar nor an alcohol
    • excessive consumption can cause abdominal discomfort and/or a laxative effect for everyone
    • has fewer calories than sucrose
    Sweeteners to AVOID by All Individuals with CSID
    Beet sugar
    Brown sugar
    • a combination of sucrose & molasses
    Cane juice
    Cane sugar
    Caramel, caramel-based sauces
    Coconut sugar
    Confectioner’s sugar
    Date sugar
    Maple syrup
    Molasses
    Raw sugar
    Sucanat™
    (natural cane sugar)
    Sucrose
    Sugar
    Turbinado sugar

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    IMPORTANT SAFETY INFORMATION:

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    Important Safety Information for Sucraid® (sacrosidase) Oral Solution

    • Tell your doctor if you are allergic to, have ever had a reaction to, or have ever had difficulty taking yeast, yeast products, papain, or glycerin (glycerol).
    • Sucraid® may cause a serious allergic reaction. If you notice any swelling or have difficulty breathing, get emergency help right away.
    • Sucraid® does not break down some sugars that come from the digestion of starch. You may need to restrict the amount of starch in your diet. Your doctor will tell you if you should restrict starch in your diet.
    • Tell your doctor if you have diabetes, as your blood glucose levels may change if you begin taking Sucraid®. Your doctor will tell you if your diet or diabetes medicines need to be changed.
    • Some patients treated with Sucraid® may have worse abdominal pain, vomiting, nausea, or diarrhea. Constipation, difficulty sleeping, headache, nervousness, and dehydration have also occurred in patients treated with Sucraid®. Check with your doctor if you notice these or other side effects.
    • Sucraid® has not been tested to see if it works in patients with secondary (acquired) sucrase deficiency.
    • NEVER HEAT SUCRAID® OR PUT IT IN WARM OR HOT BEVERAGES OR INFANT FORMULA. Do not mix Sucraid® with fruit juice or take it with fruit juice. Take Sucraid® as prescribed by your doctor. Normally, half of the dose of Sucraid® is taken just before a meal or snack and the other half is taken during the meal or snack.
    • Sucraid® should be refrigerated at 36°F-46°F (2°C-8°C) and should be protected from heat and light; single-use containers can be removed from refrigeration and stored at 59°F-77°F (15°C-25°C) for up to 3 days (72 hours). Refer to Instructions for Use for full information on how to take Sucraid®.

    You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.

    Indication

    Sucraid® (sacrosidase) Oral Solution is indicated for the treatment of sucrase deficiency, which is part of congenital sucrase-isomaltase deficiency (CSID), in adult and pediatric patients 5 months of age and older.