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About CSID

What Are the Symptoms of CSID?

What Are the Symptoms of CSID?

In general terms, when individuals with Congenital Sucrase-Isomaltase Deficiency (CSID) eat food containing sucrose, they are unable to digest the sucrose and they develop gastrointestinal symptoms. Most of those with CSID have very little or absent sucrase activity, which is the enzyme needed for sucrose digestion. They also have varying levels of activity of the enzymes required for starch digestion (for example, isomaltase [palatinase] and maltase), so they may also experience gastrointestinal symptoms from consuming foods containing starch.1

Gastrointestinal symptoms for those with CSID can vary from mild to severe. These symptoms occur because dietary sucrose moves through the small intestine undigested. The undigested sugar enters the colon, which causes diarrhea; and the normal bacteria present in the intestine digest the sugar by fermentation, which results in gas production. Diarrhea occurs when substances are not absorbed from the small intestine, causing water to be retained in the colon. This is referred to as osmotic diarrhea. Presenting symptoms may differ among infants, children, and adults.2

Infants and Children​

Infants do not manifest symptoms of CSID until they begin to ingest sucrose- and starch-containing foods (for example, fruit juices, solid foods, formula, and medications sweetened with sucrose). Infants who are exclusively breastfed may not show symptoms until the introduction of solid foods or until weaning. Common symptoms are chronic, watery diarrhea with or without failure to thrive (poor physical growth) and abdominal pain.1

Infants may also present with abdominal swelling (distension), gassiness, colic, irritability, excoriated (abrasions and irritations) buttocks, diaper rash, and vomiting. A minority of infants may require hospitalization due to dehydration, malnutrition, muscle wasting, and weakness. Because children have shorter gastrointestinal tracts, their symptoms may be more severe than adults.1

Some children with CSID are not diagnosed as infants and are presumed to have1 chronic, nonspecific diarrhea (toddler’s diarrhea). In older children, symptoms include intermittent diarrhea and abdominal pain or discomfort from excessive gas, bloating, or cramps. They may be incorrectly diagnosed with irritable bowel syndrome (IBS). In these children, a prolonged period of misdiagnoses may occur as individuals learn to live with their symptoms. The potential reasons for a delay in the diagnosis of CSID could be mistaken diagnoses of milk protein intolerance, food allergies, and chronic, nonspecific diarrhea. CSID is a genetic disease that individuals will not outgrow.1

Adults

Adult symptoms are similar to those of infants and children but may be less severe due to the adult’s longer gastrointestinal tract. In adults, a prolonged period of misdiagnoses may occur as the adult learns to live with their uncomfortable gastrointestinal symptoms, or assumes their ongoing gastrointestinal problems to be normal. Recurrent symptoms similar to those caused by CSID, such as frequent bowel movements, chronic diarrhea, unexplained abdominal cramps, gassiness, bloating, weight loss, and vomiting should not be ignored.1

In some adults, symptoms may be limited to an increase in bowel movement frequency, abdominal distention, and flatulence, with the occurrence of episodic watery diarrhea when eating foods containing a high concentration of sucrose. In some adults, diarrhea may alternate with constipation, leading to a misdiagnosis of irritable bowel syndrome (IBS). Consultation with a gastroenterologist is recommended to discuss any recurring, persistent gastrointestinal symptoms.1

Failure to absorb dietary sucrose and starch may impact the absorption of other nutrients as well as the hormonal regulation of gastrointestinal function. Carbohydrates that have been consumed but not digested or absorbed accelerate the rate food moves from the small intestine to the large intestine (called transit time), a factor that contributes further to the malabsorption of dietary starch, fat, and monosaccharides. The severity of gastrointestinal symptoms is affected by several factors, including the quantity of sucrose or starch consumed, the level of bacterial activity present in the intestine, the absorptive capacity of the colon, the rate of gastric emptying, and small bowel transit time.1

References
  1. Treem WR. Congenital sucrase-isomaltase deficiency. J Pediatr Gastroenterol Nutr. 1995;21(1):1-14. doi:10.1097/00005176-199507000-00001
  2. Gericke B, Amir M, Naim HY. The multiple roles of sucrase-isomaltase in the intestinal physiology. Mol Cell Pediatr. 2016;3(1):2-6. doi:10.1186/s40348-016-0033-y

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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.