Managing CSID in Adult Patients

Monitoring adult patients with Congenital Sucrase-Isomaltase Deficiency (CSID) requires a periodic review of their responses to therapeutic and dietary interventions and the impact of CSID on the patients’ overall dietary intake. Particular attention must be paid to dietary carbohydrates and the relationship between dietary sucrose and starch consumption and gastrointestinal symptoms while taking enzyme replacement therapy.

Consider the typical case of a patient, a woman in her late thirties. Clinically underweight, she says she has had “stomach problems” since childhood, including chronic intermittent diarrhea, abdominal pain, and bloating. This typical patient has seen a variety of specialists over the years who had diagnosed her with various conditions, including gastroesophageal reflux disease, irritable bowel syndrome, and lactose intolerance. No therapy prescribed for any of these conditions has provided any notable clinical benefit. Finally diagnosed with CSID at the age of 36 years, she has been following a low-sucrose and modified-starch diet and using sacrosidase enzyme replacement therapy to help minimize her symptoms.

Tools for Counseling Adult Patients with CSID

Getting patients to communicate their symptoms effectively and accurately can be challenging, but it’s imperative for achieving the correct diagnosis and for monitoring responses to both dietary intervention and sacrosidase enzyme replacement therapy. Below are a few tools to facilitate effective communication and tracking of patient symptoms.

Bristol Stool Chart

Many patients do not understand what comprises diarrhea or constipation. Discussing the normal frequency of bowel movements for adults, which ranges from three bowel movements per day to three per week, does not include the stool form, consistency, urgency or comfort factors. The Bristol stool chart is a schematic that helps patients recognize and accurately describe whether they are experiencing diarrhea, constipation, or a mixture of the two.

Diet Management

Typically, patients starting enzyme replacement therapy are instructed to take Sucraid® (sacrosidase) Oral Solution with every meal or snack before making substantive dietary modifications. Improvement in gastrointestinal symptoms is generally noted within the first two weeks on therapy. Patients on therapy typically tolerate a normal amount of sucrose in the diet, and dietary sucrose restriction may not be necessary.1 If symptom relief is not adequate with therapy alone, dietary restriction of sucrose and starch may need to be considered. Although sacrosidase does not aid in starch digestion, the types and amounts of starch tolerated varies for each patient with CSID. Patients who are symptomatic while on therapy are encouraged to work with a registered dietitian who has experience with CSID.

 

References

  1. Treem WR, McAdams L, Stanford L, Kastoff G, Justinich C, Hyams J. Sacrosidase therapy for Congenital Sucrase-Isomaltase Deficiency. J Pediatr Gastroenterol Nutr. 1999;28(2):137-142.
  2. Almario CV, Chey W, Kaung A, et al. Computer-generated vs. physician-documented history of present illness (HPI): results of a blinded comparison. Am J Gastroenterol. 2015;110(1):170-179.