What is Congenital Sucrase-Isomaltase
Deficiency (CSID)? CSID is mainly a clinical diagnosis.

Congenital Sucrase-Isomaltase Deficiency (CSID) is a rare, inherited condition that has several names. It is sometimes referred to as Genetic Sucrase-Isomaltase Deficiency (GSID), Sucrase-Isomaltase Deficiency, Disaccharide Intolerance, or Congenital Sucrose Intolerance.

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If you have been diagnosed with CSID, ask your GI doctor if Sucraid® is right for you.

If you think you may have CSID, 
you should schedule a visit to discuss
your concerns with a gastroenterologist.

Congenital Sucrase-
Isomaltase Deficiency

CSID, which can occur in males and females of all ages, reduces the ability to digest (break down to an absorbable form) certain foods one eats that contain sugars or starches. For this reason, enzymes like sucrase-isomaltase are called digestive enzymes.

Sucrase-Isomaltase 
digestive enzyme

In people affected by CSID, the enzyme is either not present at all or has very low levels of enzyme activity in the small intestine. The small intestine is the part of the gastrointestinal (GI) tract that is just beyond the stomach and just before the large intestine.

The GI 
symptoms

If you cannot fully digest foods that contain sugar (white table sugar) and/or starch (for example, bread, cookies, crackers, and potatoes)¹, you may develop diarrhea, gas, bloating, and abdominal pain. Undigested foods in your GI tract are eaten by the bacteria that naturally occur in your GI tract.

The science 
behind the enzyme

In the 1960s, Weijers and colleagues discovered a GI condition they named CSID. The condition was originally characterized by GI symptoms such as diarrhea, gas and abdominal pain after eating a meal.²

The condition was also associated with the following changes in enzyme activity:²

  • Sucrase enzyme activity that was undetectable
  • Varying levels of isomaltase enzyme activity
  • Maltase enzyme activity that was reduced to nearly one third the normal level

An enzyme is a naturally-occurring protein that makes the chemical reactions that occur all the time in the body, occur much faster.

We rely on the enzymes our body produces to help the tissues and organs in our body function normally. Sucrase is the enzyme that helps our body digest sucrose, which is a fancy name for white table sugar.

Carbohydrates are one of the three main sources of nutrients in our diets. The other two are proteins and lipids (fats). Carbohydrates are made by plants to store energy. Carbohydrates are complex compounds that must be broken down to a simpler form before they can be absorbed into the bloodstream.

There are three types of carbohydrates. Sugars are simple or complex carbohydrates found in white table sugar, baked goods, fruits, vegetables, and dairy products. Starches are made up of more than one sugar unit bound together and are found in whole grains, potatoes, pasta, vegetables and beans. Dietary fiber is a very complex carbohydrate that your body cannot digest, so it passes through the intestine undigested, becoming a food source for the “good” bacteria that exist naturally in your large intestine. Fiber is present in fruits, vegetables, legumes, nuts, seeds, and whole grains.

How we convert food 
into fuel in our body.

Sucrose (table sugar) is too large to be absorbed from the small intestine. For this reason, sucrose must be digested to its simpler forms, glucose and fructose, before it can be absorbed into the bloodstream and used as an energy source.3 Sucrase is the only naturally-occurring enzyme in humans that can break sucrose down into its simpler forms.

Starches are even larger compounds than sucrose. However, there are several intestinal enzymes that play a role in the digestion of the starches we eat, including: sucrase-isomaltase, maltase-glucoamylase, and α-amylases

So, if you have CSID and the enzyme sucrase-isomaltase is missing from your small intestine or isn’t working or isn’t working well, you may or may not have GI symptoms after eating foods that contain starches. While there is only one naturally-occurring enzyme (sucrase) in our bodies that digests sucrose, there are multiple enzymes in our bodies that act to digest starches. Because only sucrase can digest sucrose, eating foods that contain sucrose usually causes worse GI symptoms in patients with CSID than eating foods that contain starches because some of those starches may be broken down in part by one of the two enzymes other than sucrase-isomaltase.

What are the consequences of not 
absorbing sugars and starches?

Common GI symptoms associated with CSID that follow a meal containing sucrose or starch include watery diarrhea, nausea, bloating and gassiness, abdominal distention (swelling), and pain.4

Failure to break down and absorb foods containing sucrose and starch from the small intestine can:4
  • Slow the normal flow of foods you’ve eaten from the stomach into the small intestine
  • Accelerate the movement of foods from the small intestine into the large intestine
  • Contribute to a reduced absorption of other dietary nutrients, such as other starches and sugars, fats, and proteins
  • Interfere with hormonal regulations of your GI function
  • Lead to GI symptoms
  • Lead to low body weight or malnutrition
For this reason, individuals with CSID are at risk for chronic malnutrition. Indeed, failure to thrive is one of the primary characteristics of the disease in children.5

Specialty
pharmacy

Learn how you can get Sucraid®
from the specialty pharmacy.

IMPORTANT SAFETY INFORMATION:

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.

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