Treatment

What are the Options for Treating CSID?

For patients with Congenital Sucrase-Isomaltase Deficiency (CSID) who present with gastrointestinal symptoms that warrant intervention, three major treatment options exist:

  • Severe diet restriction

  • Sucraid

  • Sucraid with moderate diet restriction

Before Sucraid was available, treatment of CSID consisted of lifelong adherence to a strict sucrose-free diet. However, given the high sucrose content of Western diets, patient compliance with a sucrose-free diet is challenging. Patient noncompliance is often accompanied by chronic gastrointestinal (GI) symptoms and a low body mass index (BMI) that falls below an age-adjusted growth curve.1-3 In a clinical trial observing the response to a restricted diet, up to 75% of patients remained symptomatic, mostly due to noncompliance with the diet.4

Enzyme replacement therapy with Sucraid offers a pharmacologic alternative to sucrose-free diets. Sucraid is an effective option for the management of sucrase deficiency and reduces or eliminates the need for dietary restrictions of sucrose and the symptoms associated with CSID in the majority of patients. In a long-term clinical trial, 81% of patients with CSID managed by Sucraid became asymptomatic.4 Note that Sucraid does not provide specific replacement therapy for the deficient isomaltase.5 Therefore, restricting starch in the diet may still be necessary in order to minimize symptoms. The need for dietary starch restriction in patients using Sucraid should be evaluated on a patient-by-patient basis.

Treatment Percent of CSID Patients Treated With Sucraid Became Asymptomatic

Figure 1. In a long-term clinical trial, 81% of study subjects with CSID who were treated with Sucraid became asymptomatic.

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Important Safety Information for Sucraid® (sacrosidase) Oral Solution

  • Sucraid may cause a serious allergic reaction. Patients should stop taking Sucraid and get emergency help immediately if any of the following side effects occur: difficulty breathing, wheezing, or swelling of the face. Care should be taken when administering initial doses of Sucraid to observe any signs of acute hypersensitivity reaction.
  • Do not use Sucraid (sacrosidase) Oral Solution with patients known to be hypersensitive to yeast, yeast products, papain, or glycerin (glycerol).
  • Although Sucraid provides replacement therapy for the deficient sucrase, it does not provide specific replacement therapy for the deficient isomaltase.
  • Adverse reactions as a result of taking Sucraid may include worse abdominal pain, vomiting, nausea, diarrhea, constipation, difficulty sleeping, headache, nervousness, and dehydration.
  • Before prescribing Sucraid to diabetic patients, the physician should consider that Sucraid will enable sucrose hydrolysis and the absorption of those hydrolysis products, glucose and fructose.
  • The effects of Sucraid have not been evaluated in patients with secondary (acquired) disaccharidase deficiency.
  • DO NOT HEAT SOLUTIONS CONTAINING SUCRAID. Do not put Sucraid in warm or hot fluids. Do not reconstitute or consume Sucraid with fruit juice since the acidity of the juice may reduce the enzyme activity of Sucraid. Half of the reconstituted Sucraid should be taken at the beginning of the meal or snack and the other half during the meal or snack.
  • Sucraid should be refrigerated at 36°F-46°F (2°C-8°C) and should be protected from heat and light.

Indication

Sucraid®(sacrosidase) Oral Solution is an enzyme replacement therapy for the treatment of genetically determined sucrase deficiency, which is part of Congenital Sucrase-Isomaltase Deficiency (CSID).