Adult Dosing
Sucrase deficiency (congenital sucrase-isomaltase deficiency)
>15 kg body wt
- 2 mL (17,000 IU)/meal or snack diluted with 2-4 ounces of water or milk
Notes:- Half of the dosage should be taken at the beginning of the meal or snack and the remaining to be taken during the meal or snack
- Serve the beverage at cold or room temperature and do not heat before or after addition of sacrosidase
- Do not reconstitute or consume sucrosidase with fruit juice
Pediatric Dosing
Sucrase deficiency (congenital sucrase-isomaltase deficiency)
15 kg body wt
- 1 mL (8,500 IU)/meal or snack diluted with 2-4 ounces of water, milk or infant formula
>15 kg body wt
- 2 mL (17,000 IU)/meal or snack diluted with 2-4 ounces of water, milk or infant formula
Notes:- Half of the dosage should be taken at the beginning of the meal or snack and the remaining to be taken during the meal or snack
- Serve the beverage or infant formula at cold or room temperature and do not heat before or after addition of sacrosidase
- Do not reconstitute or consume sucrosidase with fruit juice
[Outline]
See Supplemental Patient Information
- Severe wheezing 90 minutes after a second dose of sacrosidase was reported in a patients having asthma, caution advised
- There is a risk of acute hypersensitivity reactions while administering initial doses of sacrosidase hence administer initial doses near a facility where such reactions can be treated adequately. Alternately test patient for hypersensitivity to sacrosidase through skin abrasion testing. Discontinue treatment on occurence of symptoms related to such reactions and institute symptomatic and supportive therapy
- Sacrosidase does not provide specific replacement therapy for the deficient isomaltase hence consider restricting starch in diet to reduce symptoms and evaluates its need in each patients
- It is warranted to conduct short therapeutic trial (e.g. 1 wk) of sacrosidase if the diagnosis of CSID is in doubt to assess response in patient suspected of sucrase deficiency
- Sacrosidase efficiency has not been evaluated in patients with secondary (acquired) disaccharidase deficiencies
Cautions: Use cautiously in
- Hypersensitivity reaction
- Asthma
- Secondary (acquired) disaccharidase deficiencies
Supplemental Patient Information
- Advise patient not to warm or heat the beverage or infant formula before or after the addition of sacrosidase because heating is likely to decrease its potency
- Advise patient not to reconstitute or consume sacrosidase with fruit juice since its acidity may reduce the enzyme activity
Pregnancy Category:C
Breastfeeding: As per manufacturer information sacrosidase is broken down in the stomach and intestines and the component amino acids and peptides are then absorbed as nutrients.
