Congenital Sucrase-Isomaltase Deficiency (CSID) Society

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Frequently Asked Questions

How is this disorder diagnosed? 

CSID is usually diagnosed with a small bowel biopsy although genetic testing is now available for some mutations.  The Hydrogen Breath test is not considered a reliable way to diagnose CSID.  Enzyme levels are also used to identify mutations for CSID patients.  This is important as some mutations have a more severely restricted diets than others and can greatly contribute to the child's quality of life, and long term health.

Is this disorder fatal?

Of the children we currently track, all lead normal lives once they have been diagnosed and restrict their diet to foods that do not cause symptoms.  The diet is very important to good health and we strongly recommend that you consult a dietician or a Pediatric Gastrointeroligist to monitor your child's diet for the first five years after being diagnosed. Children who are fed or "sneak" foods not advisable for this disorder do tend to continue to be unhealthy and some have developed other intestinal disorders.  

Should my child wear a medical alert bracelet?

Yes! The bracelet helps caretakers, nurses and emergency room personal avoid an accidental ingestion of sucrose.  The earlier your child begins to wear a medical alert bracelet the less resistant they will be to wear one when older.  

Will my child ever outgrow this illness?

No. This is a genetic disorder not an illness like the flu.  To date there is no cure for this genetic disorder.  Some children experience improvement in starch digestion between the ages of one and three and a half years old.  After that the society has not been able to document any improvement in starch digestion.  We also have no data to support any child improving sucrose digestion regardless of age for all of the children we track.

How do I know which mutation my child has?

The only way to determine which mutation your child has is with a small bowel biopsy or genetic testing. Phenotypes and diet plans can be determined using the same information.

Are there any side effects of Sucraid?

The FDA provides an information sheet regarding Sucraid that may be helpful.

Sucraid does not seem to be working, am I doing something wrong?

The three main reasons we can document for families reporting that Sucraid seems ineffective are:

They did not realize that Sucraid only aids in sucrose digestion not starch digestion.

The bottle of Sucraid was allowed to come to room temperature, causing it to become ineffective.  Sucraid must be refrigerated. 

Finally, for each child there seems to be an upward limit of sucrose a given dose of Sucraid (2 ml) can aide in digestion.  This varies widely.  Children have been documented as tolerating as little as 12 grams per 100 grams of food in one serving to 28 grams per 100 grams in one serving. With 36 identified mutations there is a great deal of variance between tolerance levels.

What have other parents done when their child is underweight because of the vomiting and loose stools?

A physician or dietician should be consulted if your child is failing to thrive or is underweight. Some of our parents have reported success in achieving weight gain by adding the following to their children's diets: 

  • 1 teaspoon of olive oil to each bottle of formula
  • Adding two bottles of lemonade made with lemon juice and crystalline fructose two times a day
  • Adding olives, cheese cubes, chicken chunks, avacado to toddler's diets
  • Using whole milk instead of 2%, 1% or nonfat milk to a toddler's diet.

My child has trouble getting to sleep, are there other children with CSID who do the same thing?

Yes! Small children especially cannot tell you they have gas pains, abdominal cramping or a stomach ache.  As the child becomes accustomed to the new diet and symptoms are eliminated they normally return to a normal sleep schedule. Many of our parents continued to put their children to bed at their normal time each night even if they stayed awake for several hours at first. Within three to six months almost all of our parents have reported their children returning to a normal sleeping schedule.

My child gets a lot of upper respiratory infections, are there other children with CSID who get this frequently?

Yes. Many of our families complain that their children with CSID seem to get sicker faster then siblings and have a greater number of upper respiratory infections and viral pneumonia. Some physicians have elected to vaccinate children each year for influenza, and give the chicken pox and pneumonia vaccination when age appropriate. Because many pharmacies do not keep a ready supply of cold medications that are sucrose free, lactose or starch free you may want to prepare before your child gets ill.  Please refer to our list of Sucrose-free drugs.  Many parents obtain a prescription for Sucrose-free Robitusson and store it in their refrigerator. Another drug they have reported keeping on hand is Feverall (small children need a prescription) a suppository that will reduce a fever in a child who is vomiting.

How do I cope with holidays like Easter, Christmas and Halloween?

Each year moms share sources for sucrose free candy and other treats.  We also list a number of sources that you can mail order from.  You can also find links to recipes that many of our parents use to make their own sucrose-free treats.

My child's doctor and small bowel biopsy indicate they have CSID but my child does not get a lot of vomiting and loose stools discussed in the literature for this disorder. Instead he suffers from gas, bloating, skin rashes-sometimes all over the body, and abdominal cramping or constapation. Does he  still have CSID?

We have a group of children from New Zealand and Australia that are emerging who have CSID but rarely get vomiting or loose stools.  Most of these children also are lactose intolerant. Instead they suffer from gas, bloating, skin rashes-sometimes all over the body, and severe abdominal cramping within hours of ingesting sucrose or starch.

Some children suffer from constapation due to a lack of fiber and chronic dehydration.

What can I feed my child who has just been diagnosed with CSID?

We have collected as much data as possible from other parents who have children with CSID.  With this information, a dietician at the University of Washington was consulted, and subsequently we have developed a diet that seems to work for Groups A, B and C that eliminates the child's symptoms.  Please refer to the Induction Diet. Several mothers in the New Zealand/Australia areas with children in Phenotype D painstakingly kept records of her children and supplied us with her diet that we have successfully used with other children from the same phenotype.

The literature suggests copper malabsorption may be an issue for children with CSID, what is copper malabsorption?

"Various conditions associated with copper deficiency are now recognized:  anemia, neutropenia, lukopenia, and skeletal demineralization, all of which are reversable by copper administration.  The normal concentration of serum copper ranges from 75 to 150 ug/dL and is slightly higher in females than in males. ... Serum copper is generally measured by atomic absorption spectrophotometry (AAS)." Advanced Nutrition and Human Metabolism by Sara M. Hunt and James L. Groff , West Publishing Company: St. Paul; 1990 pg 310.

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