Sacrosidase is an oral enzyme produced as a by-product of the manufacture of baker's yeast (Saccharomyces cerevisiae). Sacrosidase is FDA-approved for the oral replacement therapy of genetically determined sucrase deficiency, which is part of congenital sucrase-isomaltase deficiency (CSID). CSID is a rare genetic disease in which the body lacks sucrase and isomaltase, enzymes necessary to break down and absorb sucrose and isomaltose, respectively. Symptoms present shortly after sucrose or starches, found in modified milk formulas, fruits, starches, or certain medications, are ingested by an affected infant. Symptoms may include watery diarrhea resulting in dehydration, abdominal distension, and discomfort. Infants may also experience malnutrition and failure to thrive. Symptoms are usually more severe in infants and young children compared to adults. Symptoms often improve as the child grows older, exhibiting only occasional or mild symptoms in adulthood. Treatment involves dietary management through a low-sucrose or sucrose-free diet. A low-starch or starch-free diet may also be recommended, especially in the first few years of life. Sacrosidase is contraindicated in patients hypersensitive to yeast, yeast products, glycerin, or papain. When initiating therapy, administer initial doses near a facility where acute hypersensitivity reactions can be adequately treated. Alternatively, test for hypersensitivity to sacrosidase through skin abrasion testing. If symptoms of hypersensitivity appear, discontinue therapy and initiate symptomatic and supportive therapy.
General Administration Information
For storage information, see the specific product information within the How Supplied section.
Route-Specific Administration
Oral Administration
Oral Liquid Formulations
-One full measuring scoop supplied by the manufacturer equals 1 mL, and 28 drops from the sacrosidase multiple-dose bottle equals 1 mL. The 1 mL dose is available as a multiple-dose bottle. The 2 mL dose is available as a multiple-dose bottle and a single-use container.
-Dilute each 1 mL dose in 60 mL and each 2 mL dose in 120 mL of cold or room temperature water, milk, or infant formula. Do not heat the solution, combine it with hot liquids, or mix or consume with acidic beverages (e.g., fruit juice) because this may reduce the enzyme activity.
-Administer approximately half of the dose before the meal or snack and the remainder during the meal or snack.
-Storage: Discard the multiple-dose bottle 4 weeks after opening, and rinse the measuring scoop with water after each use.
In clinical trials of sacrosidase, adverse reactions were typically mild and often associated with symptoms of the underlying disease.
During clinical trials, severe wheezing was reported 90 minutes after a 2nd dose of sacrosidase was administered to a 4-year old boy and admission into the ICU was required. This patient had a history of asthma which was being treated with steroids. A skin test for sacrosidase confirmed a hypersensitivity reaction.
In sacrosidase clinical trials of up to 54 months duration, gastrointestinal (GI) adverse effects were the most frequently reported. In these trials, which included a total of 52 patients with congenital sucrase-isomaltase deficiency (CSID), abdominal pain (7.7%), nausea (3.8%), vomiting (5.8%), diarrhea (3.8%), and constipation (3.8%) were reported. Because similar GI effects are common symptoms of the underlying disease, a relationship to the treatment is difficult to assess. Specifically, diarrhea and abdominal pain can be a part of the clinical presentation of the genetically determined sucrase deficiency, which is part of CSID.
In sacrosidase clinical trials of up to 54 months duration and including a total of 52 patients, insomnia, headache, and nervousness were reported in one patient each (i.e., a 1.9% frequency).
In sacrosidase clinical trials of up to 54 months duration and including a total of 52 patients, dehydration was reported in one patient (i.e., a 1.92% frequency).
Sacrosidase only provides replacement therapy for sucrase deficiency; it does not provide replacement for isomaltase deficiency. Therefore, dietary starch restriction may still be necessary to minimize the symptoms of congenital sucrase-isomaltase deficiency (CSID). The need for dietary starch restriction should be determined and monitored for each patient.
Safety and efficacy have not been established for the use of sacrosidase in patients with secondary (acquired) disaccharidase deficiencies.
Sacrosidase should not be used in patients with a history of yeast hypersensitivity. The enzyme is derived from baker's yeast (Saccharomyces cerevisiae) and may cause sensitivity reactions in patients with hypersensitivity to yeast or yeast products. In addition, patients with papain hypersensitivity should not use this product. Additionally, the commercial formulation contains glycerin (glycerol) and should not be used in patients with a history of glycerin hypersensitivity. Care should be taken to administer initial doses of sacrosidase near a facility where acute hypersensitivity reactions can be adequately treated. Alternatively, the patient may be tested for hypersensitivity to sacrosidase by using skin abrasion testing. One case of a severe hypersensitivity reaction to sacrosidase has been reported in a 4 year old with asthma (see Adverse Reactions); skin testing verified hypersensitivity.
Use sacrosidase with caution in patients with diabetes mellitus. Since sacrosidase hydrolyzes sucrose into glucose and fructose, CSID patients with diabetes mellitus may have higher blood glucose concentrations when using sacrosidase.
The safety and efficacy of sacrosidase have not been established in neonates and infants less than 5 months of age.
Animal reproduction studies have not been conducted with sacrosidase. The enzyme is not expected to cause fetal harm when administered to a pregnant woman or to affect reproductive capacity; however, sacrosidase should be given during pregnancy only if clearly needed.
Clinical trials examining the use of sacrosidase in breast-feeding women have not been conducted; the exact effects on a nursing infant have not been defined. After ingestion, sacrosidase is broken down in the stomach and intestines, and then the remaining amino acids and peptides are absorbed as nutrients. Adverse effects on a nursing infant are not expected. Consider the benefits of breast-feeding, the risk of potential infant drug exposure, and the risk of an untreated or inadequately treated condition. If a breast-feeding infant experiences an adverse effect related to a maternally ingested drug, healthcare providers are encouraged to report the adverse effect to the FDA.
For oral replacement therapy of the genetically determined sucrase deficiency, which is part of congenital sucrase-isomaltase deficiency (CSID):
Oral dosage:
Adults: 2 mL (17,000 international units) or 2 full measuring scoops PO mixed in 120 mL of cool or room temperature water or milk; give with each meal or snack. Administer approximately half of the dosage at the beginning of the meal or snack and the remainder during the meal or snack. Do not mix in or consume with fruit juice.
Infants, Children, and Adolescents 5 months to 17 years weighing more than 15 kg: 2 mL (17,000 international units) or 2 full measuring scoops PO mixed in 120 mL of cool or room temperature water or milk; give with each meal or snack. Administer approximately half of the dosage at the beginning of the meal or snack and the remainder during the meal or snack. Do not mix in or consume with fruit juice.
Infants and Children 5 months and older weighing 15 kg or less: 1 mL (8,500 international units) or 1 full measuring scoop PO mixed in 60 mL of cool or room temperature water, milk, or infant formula with each meal or snack. Administer approximately half of the dosage at the beginning of the meal or snack and the remainder during the meal or snack. Do not mix in or consume with fruit juice.
Maximum Dosage Limits:
-Adults
2 mL PO with each meal or snack.
-Geriatric
2 mL PO with each meal or snack.
-Adolescents
2 mL PO with each meal or snack.
-Children
weighing more than 15 kg: 2 mL PO with each meal or snack.
weighing 15 kg or less: 1 mL PO with each meal or snack.
-Infants
5 months of age and older: 1 mL PO with each meal or snack.
younger than 5 months of age: Safety and efficacy have not been established.
Patients with Hepatic Impairment Dosing
Specific guidelines for dosage adjustments in hepatic impairment are not available; it appears that no dosage adjustments are needed.
Patients with Renal Impairment Dosing
Specific guidelines for dosage adjustments in renal impairment are not available; it appears that no dosage adjustments are needed.
*non-FDA-approved indication
Food: (Moderate) Sacrosidase should not be reconstituted or consumed with acidic food, like fruit juice, since the acidity may reduce the enzyme activity.
Sacrosidase hydrolyzes sucrose (a disaccharide) into its component monosaccharides, glucose and fructose. Sacrosidase replaces the endogenous enzyme sucrase (produced in the small intestine) which is necessary for the digestion of sucrose. Administration of sacrosidase to sucrase deficient patients with congenital sucrase-isomaltase deficiency (CSID) resulted in significantly fewer total stools as well as a reduction in overall symptoms.
Sacrosidase is administered orally. In the stomach and intestines, the enzyme is broken down into amino acids and peptides which are then absorbed as nutrients. No further pharmacokinetic information is available.