CurrentCignaPolicy IP0447

Enzyme Replacement Therapy - Sucraid

Defines Cigna's coverage and prior authorization requirements for Sucraid (sacrosidase oral solution) to treat congenital sucrase-isomaltase deficiency (CSID) for covered members under Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyEnzyme Replacement Therapy - Sucraid

Policy CodePolicy IP0447

Change TypeRevised criteria and extended approval duration

Effective Date

Next Review Date

Key ActionObtain prior authorization and document diagnostic confirmation (biopsy or genetic test), symptoms, and appropriate prescriber specialty to support approval for up to 1 year.

SourceLink

POLICY UPDATE CHANGES

Policy name updated from 'Sacrosidase' to 'Enzyme Replacement Therapy - Sucraid.'

Isomaltase and lactase acceptance criteria changed from 'decreased to normal' to 'decreased or normal' for biopsy disaccharidase levels.

Duration of approval updated from 6 months to 12 months.

1 yearstandard approval duration for Sucraid when criteria met

IP0447coverage policy identifier

FDA-approvedindication basis cited (CSID)

Coverage Criteria for Sucraid (sacrosidase)

FDA-Approved Indication - Congenital Sucrase-Isomaltase Deficiency

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

Overall approval

A: Diagnostic confirmation
- i. Biopsy disaccharidase criteria
  - a: Decreased (usually absent) sucrase level (normal reference: > 25 U/g protein)
  - b: Decreased or normal isomaltase (palatinase) level (normal reference: > 5 U/g protein)
  - c:

Policy Summary

PayerCigna

PolicyEnzyme Replacement Therapy - Sucraid

Policy CodePolicy IP0447

Change TypeRevised criteria and extended approval duration

Effective Date

Next Review Date

Key ActionObtain prior authorization and document diagnostic confirmation (biopsy or genetic test), symptoms, and appropriate prescriber specialty to support approval for up to 1 year.

SourceLink

On This Page

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Provider Actions, Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for benefit coverage of Sucraid. Approvals are provided for 1 year when specified diagnostic, symptomatic, and prescriber specialty criteria are met. Because evaluation and monitoring require specialized skills, Sucraid must be prescribed by or in consultation with a physician who specializes in the condition being treated.

Prior authorization required for Sucraid
Approvals granted for 1 year when criteria met
Prescriber specialty requirement: geneticist, gastroenterologist, metabolic disorder sub-specialist, or physician specializing in congenital diarrheal disorders

Step Therapy

No step therapy requirements specified. Providers do not need to complete step therapy prior to requesting prior authorization for Sucraid; submit prior authorization per requirements below.

Documentation Required

Required Documentation

Documentation must demonstrate diagnosis and clinical necessity. Provide one of the diagnostic evidences listed and documentation of symptoms prior to starting therapy, plus prescriber specialty or consultation note.

Evidence of diagnosis: either small bowel endoscopic biopsy with disaccharidase levels consistent with congenital sucrase-isomaltase deficiency OR molecular genetic testing showing homozygous or compound heterozygous pathogenic/likely pathogenic sucrase-isomaltase gene variants
If biopsy used, include lab results showing: decreased (usually absent) sucrase level (normal reference: >25 U/g protein); decreased or normal isomaltase (palatinase) (normal reference: >5 U/g protein); decreased maltase (normal reference: >100 U/g protein); decreased or normal lactase (normal reference: >15 U/g protein)
Documentation of symptomatic congenital sucrase-isomaltase deficiency prior to starting Sucraid (e.g., diarrhea, bloating, abdominal cramping)
Prescription or consultation note from an appropriate specialist (geneticist, gastroenterologist, metabolic disorder sub-specialist, or physician specializing in congenital diarrheal disorders)
Receipt of sample product does not satisfy documentation requirements

Denial Risk

Denial Triggers / Documentation Risks

Claims submitted without a covered diagnosis/procedure code or not accompanied by the required documentation will be denied. Failure to obtain prior authorization when required may result in denial of coverage.

Denial triggers: missing covered diagnosis/procedure codes
Denial triggers: absence of required diagnostic documentation or specialist prescription/consultation
Denial triggers: no prior authorization when required

Background

Congenital sucrase‑isomaltase deficiency (CSID) is an autosomal recessive intestinal disorder caused by reduced or absent activity of the sucrase‑isomaltase enzyme complex, leading to malabsorption of sucrose and some starches. Patients typically present with osmotic diarrhea, abdominal bloating, cramping, vomiting, and in some cases failure to thrive or dehydration. Diagnosis is established by either endoscopic small intestine biopsy demonstrating disaccharidase levels consistent with CSID or by molecular genetic testing identifying pathogenic sucrase‑isomaltase variants. Enzyme replacement with Sucraid is indicated only for confirmed CSID.

Definitions

CSID (Congenital sucrase-isomaltase deficiency)

DefinitionCongenital sucrase-isomaltase deficiency (CSID) is an autosomal recessive intestinal disorder with reduced or absent activity of the sucrase-isomaltase complex causing carbohydrate malabsorption and gastrointestinal symptoms.

Gold-standard diagnostic testEndoscopic small intestine biopsy with disaccharidase assay demonstrating decreased (usually absent) sucrase and concordant disaccharidase pattern.

Genetic confirmationMolecular genetic testing demonstrating homozygous or compound heterozygous pathogenic or likely pathogenic sucrase-isomaltase gene variants can establish the diagnosis.

Common clinical manifestationsOsmotic diarrhea, vomiting, bloating, abdominal pain; may include failure to thrive, dehydration, and other complications.

OpenPayer

Enzyme Replacement Therapy - Sucraid

Coverage Criteria for Sucraid (sacrosidase)

Coding and Laboratory Reference Values

Provider Actions, Authorization, and Documentation

Background

Definitions