& Starch in Foods
Sucrose Free Drugs
Sucraid (sacrosidase) oral solution is an enzyme replacement
therapy for use in the treatment of Congenital Sucrase-Isomaltase Deficiency (CSID)
developed initially by Orphan Medical, Inc. CSID is a condition where your body lacks
the enzymes needed to properly break down and absorb sucrose (table sugar) and isomaltase
(a type of starch) from the intestines.
Sucraid can help improve the breakdown and absorption of sucrose (table sugar)
from the intestine and can help relieve the symptoms of CSID. Sucraid may also
possibly improve growth in young children and make it easier to sleep by relieving
Sucraid does not contain the enzyme needed to break down and absorb isomaltase (
a type of starch) from the intestine. Your doctor should be consulted to determine
if starch intake needs to be restricted.
For more information on Sucraid you can contact
www.ivpcare.com or call 800-424-9002.
In May of
2003, Orphan Medical sold Sucraid
to QOL Medical and enclosed a letter from QOL Medical that highlighted the
Frequently Asked Questions about Sucraid
provided by QOL Medical
SucraidŽ Description: Chemical Name: B,D fructofuranoside
SucraidŽ(sacrosidase) oral solution is an enzyme replacement therapy for
the treatment of the genetically determined sucrase deficiency, which is
part of congenital sucrase-isomaltase deficiency (CSID). SucraidŽ is a
pale yellow, clear solution with a pleasant, sweet taste.
The active ingredient is sacrosidase [enzyme derived from bakers yeast (saccharomyces
cerevisiae)]. SucraidŽ also contains 50% glycerol in an aqueous
solution, with no expected toxicity at recommended SucraidŽ dose levels.
The solution is fully soluble with water, milk and infant formula. DO
NOT HEAT SOLUTIONS CONTAINING SucraidŽ. Do not put SucraidŽ in warm or
hot liquids. DO NOT MIX WITH SUGARY DRINKS SUCH AS FRUIT JUICES.
Does SucraidŽ contain isomaltase?
SucraidŽ does not contain isomaltase.
Does SucraidŽ contain gluten?
SucraidŽ does not contain gluten.
Is a support group available?
Yes. A parent support group is available. Visit www.csidinfo.com
Mechanism of Action
SucraidŽ facilitates breakdown and absorption of sucrose (table sugar)
from the intestine and can help relieve the gastrointestinal symptoms of
SucraidŽ does not break down starch. Therefore, you may need to restrict
the amount of starch in your diet. Your doctor will tell you if you
should restrict the amount of starch in your diet.
What causes CSID?
It is a genetic, inheritable disease the consequences of which interfere
with the enzyme transport mechanism. Sucrase is a naturally-occurring
enzyme that is produced in the brush-border of the small intestine,
primarily in the distal duodenum and jejunum. Sucrase hydrolyzes
sucrose, a disaccharide, into its component monosaccharides, glucose and
fructose. Congenital sucrase-isomaltase deficiency (CSID) is a rare,
chronic, autosomal recessive, phenotypically heterogenous disease with
very variable enzyme activity. CSID is usually characterized by a
complete or almost complete lack of endogenous sucrase activity, a very
marked reduction in isomaltase activity, a moderate decrease in maltase
activity and normal lactase levels.
What are the symptoms of CSID?
Symptoms may include some or all of the following: excessive gas,
bloating, abdominal cramps, watery diarrhea, nausea, vomiting, and
failure to thrive.
Sucrase is naturally produced in the brush border of the small
intestine. Sucrase hydrolyzes the disaccharide sucrose into its
component monosaccharides, glucose and fructose. In the absence of
endogenous human sucrase, sucrose is not metabolized. This may lead to
an osmotic retention of water, which causes loose stools. Unabsorbed
sucrose in the colon is fermented by bacterial flora to produce
increased amounts of hydrogen, methane and water. As a consequence,
excessive gas, bloating, abdominal cramps, nausea and vomiting may
Chronic malabsorption of disaccharides may result in malnutrition.
Undiagnosed/untreated CSID patients often fail to thrive and fall behind
in their expected growth and development.
How do you diagnose CSID?
CSID is difficult to diagnose. The definitive test for diagnosis of CSID
is measurement of intestinal disaccharidases following small bowel
Less invasive diagnoses are: A positive breath hydrogen test following
an oral challenge with sucrose and a negative breath hydrogen test
following oral challenge with lactose along with a stool pH of less than
6 provides an acceptable diagnosis of CSID.
Alternatively, because of the difficulty in diagnosing CSID a physician
may prescribe a short therapeutic trial in patients suspected of having
sucrase deficiency. Therapeutic trial supplies may be ordered by
physicians by calling 1-800-424-9002
Are there any other treatments available for CSID?
There are no other FDA approved treatments for CSID. Historically the
only way to manage CSID was to eat a restricted diet containing very
little sucrose. In clinical trials, patients still suffered symptoms of
watery diarrhea, abdominal pain and cramps even when on a fully
A two phase (dose-response preceded by a breath hydrogen phase), double
blind, multi-site, crossover trial was conducted in 28 patients (aged 4
months to 11.5 years) with confirmed CSID, as published in, Treem WR, Mc
Adams L, Stanford L, Kastoff G, Justinich C, Hyams J. Sacrosidase
Therapy for Congenital Sucrase-Isomaltase Deficiency. Journal of
Pediatric Gastroenterology and Nutrition 1999; 28(2):137-142.
Dose did not vary with age or sucrose intake;
Dose-response relationship was shown between the two higher and the two
The two higher doses of sacrosidase were associated with significantly
fewer total stools and higher proportions of patients having lower total
symptom scores, the primary efficacy endpoints;
Higher doses of sacrosidase were associated with a significantly greater
number of hard and formed stools as well as with fewer watery and soft
stools, the secondary efficacy endpoints; and
Analysis of the overall symptomatic response as a function of age
indicated that in CSID patients up to 3 years of age, 86% became
asymptomatic. In patients over 3 years of age, 77% became asymptomatic.
Thus, the therapeutic response did not differ significantly according to
The effects of SucraidŽ have not been evaluated in patients with
secondary (acquired) disaccharidase deficiencies.
Do I need to restrict my diet and watch my carbohydrate intake?
Patients in the clinical trial consumed a normal diet consisting of
2g/kg sucrose (table sugar) and 5 g/kg carbohydrates. It is generally
recommended to avoid adding starch immediately back into the diet and to
gradually introduce sucrose. Once a patient is well maintained on their
normal sucrose diet (over approximately 2 weeks), starhc van be added
back into the diet and symptoms monitored. Because CSID has many
phenotypes, a small number of patients migh need to remain starch-free.
What precautions should be discussed with my physician before starting
Tell your doctor if you are allergic to, have ever had a reaction to, or
have ever had difficulty taking yeast, yeast products, or glyercin
Tell your doctor if you have diabetes. With SucraidŽ, sucrose (table
sugar) can be absorbed from your diet and your blood glucose levels may
change. Your doctor will tell you if your diet or diabetes medicines
need to be changed.
Neither drug-drug nor drug-food interactions are expected or have been
reported with the use of SucraidŽ. However, SucraidŽ should not be
reconstituted or consumed with fruit juice, since it's acidity may
reduce the enzyme activity.
Are there any tests available to determine if a patient is allergic to
The patient may be tested for hypersensitivity to SucraidŽ through skin
abrasion testing. The patient should discuss this with the primary care
physician or allergist.
How do you treat an overdose?
No incidents of overdose have been reported. Glycerin, a component of
SucraidŽ, is an osmotic laxative; so dehydration may occur if too much
SucraidŽ is ingested. If an overdose should occur, adequate hydration
should be maintained.
1 mL (one full measuring scoop or 22 drops) per meal or snack for
patients up to 15 kg in body weight. 2 mL (two full measuring scoops or
44 drops) per meal or snack for patient over 15 kg in body weight. It is
suggested that half of the dose be taken before the meal and the other
half be taken half way through the meal.
Can I put SucraidŽ in other drinks such as water or milk?
SucraidŽ is completely soluble in water, milk, and infant formula.
Beverages containing SucraidŽ should be consumed cold or at room
temperature. Heating beverages containing SucraidŽ, or adding SucraidŽ
to hot beverages, will likely decrease the potency. SucraidŽ should not
be reconstituted or consumed with fruit juice, since its acidity may
reduce the enzyme activity. KEEP SucraidŽ REFRIGERATED
How long will SucraidŽ be stable once it is put into another liquid such
as water or baby formula?
We recommend preparation immediately prior to the meal or snack, with
half of the dosage taken before the meal or snack and the remainder of
the dosage half way through.
We just received a new shipment of SucraidŽ and it was not refrigerated
right away. Is it still good?
SucraidŽ is an enzyme product and needs to be maintained at refrigerated
conditions. However, brief exposure to ambient room temperature should
not affect the potency. If you have concerns, please call 1-800-424-9002
Food and Drug Sucraid
| | |
SUCRAID (Brand Name Drug)
FDA Application No.
Original Approval or Tentative Approval Date
April 9, 1998
1 New molecular entity (NME)
P Priority review drug
O Orphan drug
Products on Application (NDA) #020772
Click on a
column header to re-sort the table:
||Orphan Medical Inc.
|*Date Approved by
||April 9, 1998
|*Approval by FDA does
not mean that the drug is available for consumers at this
|What is Sucraid used for? Sucraid oral
solution is used as a replacement for patients who do not
have the enzymes needed to properly break down and absorb
sucrose (table sugar) and isomaltose (a type of starch) in
Who should not take Sucraid?
You should not take Sucraid if you are allergic to:
- Yeast products
- Glycerin (glycerol)
Special Warning(s) for Sucraid:
Sucraid may cause an allergic reaction in some
people. Your doctor may have you take your first and second
doses while in the office to observe how your body reacts,
or you may be tested in advance to check your sensitivity to
the drug. At any time, if you notice any swelling of the
face, wheezing or difficulty breathing, stop taking Sucraid
and seek emergency medical help right away.
General Precautions with Sucraid:
Tell your health care provider if you are allergic
to or have ever had a reaction to yeast, yeast products, or
glycerin (glycerol). If you have diabetes, your health care
provider will want to discuss adjustments to your diet plan
since using Sucraid can cause sucrose (table sugar) to be
absorbed by the body and cause changes in blood sugar
Your health care provider may want you to limit
starch in your diet to help reduce symptoms even more.
What are some possible side effects of Sucraid?
(This is NOT a complete list of side
effects reported with Sucraid. Your health care provider can
discuss with you a more complete list of side effects.)
- Stomach ache
- Difficulty sleeping
For more detailed information about Sucraid, ask your
health care provider.
updated 12/16/2004 ghc