CurrentCignaPolicy IP0447

Enzyme Replacement Therapy - Sucraid

This Cigna coverage policy governs prior authorization and medical necessity criteria for Sucraid (sacrosidase oral solution) for treatment of congenital sucrase-isomaltase deficiency and applies to health benefit plans administered by Cigna Companies.

Policy Summary

PayerCigna

PolicyEnzyme Replacement Therapy - Sucraid

Policy CodePolicy IP0447

Change TypeMaterial revision — approval duration and criteria wording updated

Effective DateJul 15, 2025

Next Review Date

Key ActionObtain prior authorization; approvals are provided for 1 year when criteria are met.

SourceLink

POLICY UPDATE CHANGES

Policy name updated from 'Sacrosidase' to 'Enzyme Replacement Therapy - Sucraid' and approval duration updated from 6 months to 12 months.

Criteria wording for isomaltase and lactase levels changed from 'decreased to normal' to 'decreased or normal'.

1 yearapproval duration for Sucraid when criteria met

IP0447coverage policy number

CSIDFDA-approved indication

Prior Authauthorization requirement

Coverage Criteria for Sucraid (sacrosidase)

Policy Summary

PayerCigna

PolicyEnzyme Replacement Therapy - Sucraid

Policy CodePolicy IP0447

Change TypeMaterial revision — approval duration and criteria wording updated

Effective DateJul 15, 2025

Next Review Date

Key ActionObtain prior authorization; approvals are provided for 1 year when criteria are met.

SourceLink

On This Page

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FDA-Approved Indication — Congenital Sucrase-Isomaltase Deficiency

Approve for 1 year if the patient meets ALL of the following (A, B, and C):

Overall requirement: All A, B, and C must be met

A) Diagnostic confirmation (one of i or ii): Diagnosis established by either (i) endoscopic small bowel biopsy with disaccharidase levels consistent with CSID OR (ii) molecular genetic testing demonstrating homozygous or compound heterozygous pathogenic or likely pathogenic sucrase-isomaltase variants.

A.i) Biopsy disaccharidase criteria: Endoscopic biopsy shows ALL of: (a) decreased (usually absent) sucrase level (normal reference: >25 U/g protein); AND (b) decreased or normal isomaltase (palatinase) level (normal reference: >5 U/g protein); AND (c) decreased maltase level (normal reference: >100 U/g protein); AND (d) decreased or normal lactase level (normal reference: >15 U/g protein).

Isomaltase and lactase may be decreased or normal per policy wording.

A.ii) Genetic testing: Molecular genetic test demonstrating homozygous or compound heterozygous pathogenic or likely pathogenic sucrase-isomaltase gene variant.

B) Symptomatic disease: Prior to starting therapy, patient had symptomatic congenital sucrase-isomaltase deficiency (e.g., diarrhea, bloating, abdominal cramping).

C) Prescriber specialty: Medication prescribed by or in consultation with a geneticist, gastroenterologist, metabolic disorder sub-specialist, or physician who specializes in treatment of congenital diarrheal disorders.

Prior authorization required; approvals provided for 1 year when criteria are met.

Sucraid for any other use is considered not medically necessary.

Use of Sucraid for indications other than congenital sucrase-isomaltase deficiency is considered not medically necessary and will not be covered.

Provider Actions, Authorization, and Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Sucraid. Approvals are provided for a duration of 1 year when criteria are met. Because specialized skills are required for evaluation and diagnosis and for monitoring, Sucraid must be prescribed by or in consultation with a physician who specializes in the condition being treated (geneticist, gastroenterologist, metabolic disorder sub-specialist, or physician experienced in congenital diarrheal disorders).

Approval duration: 1 year
Prescriber requirement: geneticist, gastroenterologist, metabolic disorder sub-specialist, or physician who specializes in congenital diarrheal disorders

Step Therapy

No step therapy requirements are specified for Sucraid in this policy.

Documentation Required

Documentation Requirements

Documentation submitted with authorization requests must include definitive evidence establishing the diagnosis of congenital sucrase‑isomaltase deficiency by ONE of the following: (1) small bowel endoscopic biopsy with disaccharidase levels demonstrating decreased (usually absent) sucrase and the pattern of disaccharidase results described in the policy; OR (2) molecular genetic testing showing homozygous or compound heterozygous pathogenic or likely pathogenic sucrase‑isomaltase gene variants. Also include documentation of symptomatic disease prior to therapy (e.g., diarrhea, bloating, abdominal cramping) and that the medication is prescribed by or in consultation with an appropriate specialist.

Acceptable diagnostic evidence: endoscopic small bowel biopsy with disaccharidase levels OR molecular genetic testing confirming homozygous or compound heterozygous pathogenic/likely pathogenic SI gene variants
Clinical documentation: symptoms prior to therapy (e.g., diarrhea, bloating, abdominal cramping)
Prescriber documentation: specialty or consultation note from geneticist, gastroenterologist, metabolic disorder sub‑specialist, or physician experienced in congenital diarrheal disorders

Denial Risk

Claims Without Covered Codes Will Be Denied

Claims submitted without covered diagnosis or procedure codes specified in the policy (i.e., without the covered diagnosis/procedure codes required by this Coverage Policy) will be denied as not covered.

Denial Risk

Use Limited to FDA‑Approved Indication

Requests for Sucraid for any use other than congenital sucrase‑isomaltase deficiency (the FDA‑approved indication) will be considered not medically necessary and denied.

Definitions

CSID (Congenital sucrase-isomaltase deficiency)

DefinitionCongenital sucrase-isomaltase deficiency (CSID) is an autosomal recessive intestinal disorder characterized by reduced or absent activity of the sucrase-isomaltase complex leading to malabsorption and gastrointestinal symptoms (e.g., osmotic diarrhea, vomiting, bloating, abdominal pain, steatorrhea).

PathophysiologyReduced or absent sucrase-isomaltase activity impairs hydrolysis of complex sugars and starches into absorbable simple sugars, causing accumulation in the small intestine and clinical manifestations.

Diagnosis (gold standard)Small intestine biopsy with disaccharidase assay demonstrating reduced or absent enzyme activity.

Diagnosis (genetic)Molecular genetic testing identifying pathogenic sucrase-isomaltase gene variants can confirm the diagnosis.

Sucraid (sacrosidase)

Generic nameSacrosidase (oral solution)

Brand nameSucraid

IndicationEnzyme replacement therapy indicated for treatment of sucrase deficiency as part of congenital sucrase-isomaltase deficiency (CSID).

Approval contextPrior authorization required; when criteria met, approvals provided for 1 year.

Background

Congenital sucrase-isomaltase deficiency (CSID) is an autosomal recessive disorder characterized by reduced or absent activity of the sucrase-isomaltase complex, leading to malabsorption of sucrose and some starches and resulting in symptoms such as osmotic diarrhea, vomiting, bloating, abdominal pain, and steatorrhea.

Diagnosis is established by either endoscopic small intestine biopsy with disaccharidase assay (the gold standard) showing reduced sucrase activity and characteristic disaccharidase patterns or by molecular genetic testing identifying homozygous or compound heterozygous pathogenic or likely pathogenic variants in the sucrase-isomaltase gene.

Sucraid (sacrosidase oral solution) is an enzyme replacement therapy indicated for treatment of sucrase deficiency as part of CSID. Prior authorization is required for coverage, and when criteria are met approvals are provided for a duration of 1 year.

Reference disaccharidase levels used in diagnostic interpretation include: sucrase >25 U/g protein (normal), isomaltase (palatinase) >5 U/g protein (normal), maltase >100 U/g protein (normal), and lactase >15 U/g protein (normal).

OpenPayer

Enzyme Replacement Therapy - Sucraid

Coverage Criteria for Sucraid (sacrosidase)

Coding and Reference Values

Provider Actions, Authorization, and Documentation Requirements

Definitions

Background