Medical_Policy

Highmark Commercial Medical Policy - Pennsylvania

Medical Policy:	L-188-004
Topic:	Huntington Disease Testing
Section:	Laboratory
Effective Date:	November 13, 2017
Issue Date:	November 13, 2017
Last Reviewed:	June 2017

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder causing progressive cognitive, motor, and psychiatric disturbances. Testing for Huntington disease is performed by determining the number of CAG repeats in the HTT gene.

This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.

Policy Position Coverage is subject to the specific terms of the member’s benefit plan.

Testing for HD may be considered medically necessary when the following criteria are met:

Clinical Consultation:
- Pre and post-test genetic counseling by an appropriate; and
- Examination by a geneticist or physician familiar with genetic movement disorders; and
Previous Genetic Testing:
- No previous genetic testing of HTT; or
- Diagnostic Testing for Symptomatic Individuals:
- For individuals greater than or equal to 18 years:(at least 2 of the following)
  - Progressive motor disability featuring involuntary movements (chorea) and gait disturbance; and/or
  - Behavioral disturbances including:
    - Personality change
    - Depression
    - Cognitive decline; and/or
  - Family History of Huntington’s Disease
- For individuals less than or equal to 17 years: (at least 2 of the following)
  - Progressive motor disability featuring involuntary movements (chorea) and gait disturbance; and/or
  - Cognitive decline; and/or
  - Stiffness or rigidity; and/or
  - Epilepsy/myoclonus and tremor; and/or
  - Family History of Huntington’s disease; or
Predictive Testing for Presymptomatic/Asymptomatic At-Risk Individuals*:
- Known CAG trinucleotide repeat expansion in HTT in 1st, 2nd, or 3rd degree biologic relative; or
- One or more 1st degree biologic relative(s) with clinical diagnosis of HD and mutation unknown/not yet tested; and
- 18 or older.

*Includes prenatal testing for at-risk pregnancies.

Procedure Codes

81401

Professional Statements and Societal Positions

The United States Huntington's Disease Genetic Testing Group (2003) has guidelines regarding genetic testing for Huntington disease.

Symptomatic testing: "Confirmatory testing by analysis of the HD gene may be offered at or after the time of the clinical diagnosis of HD. The presence of a CAG repeat expansion in a person with HD symptoms confirms the clinical impression and supports a diagnosis of HD."

Asymptomatic (predictive) testing is supported in the context of a predictive testing protocol that includes optional neurological exam, psychological exam, social support, pre- and post-test counseling regarding implications of positive and negative test results, and documented informed consent.

The predictive testing protocol is also supported by guidelines from the International Huntington Association and the World Federation of Neurology Research Group on Huntington's Chorea (1994), the American College of Medical Genetics, and the National Society of Genetic Counselors, as well as recent literature.

Place of Service: Outpatient

HD testing is typically an outpatient procedure which is only eligible for coverage as an inpatient procedure in special circumstances, including, but not limited to, the presence of a co-morbid condition that would require monitoring in a more controlled environment such as the inpatient setting.

The policy position applies to all commercial lines of business

Denial Statements

Services that do not meet the criteria of this policy will not be considered medically necessary. A network provider cannot bill the member for the denied service unless: (a) the provider has given advance written notice, informing the member that the service may be deemed not medically necessary; (b) the member is provided with an estimate of the cost; and (c) the member agrees in writing to assume financial responsibility in advance of receiving the service. The signed agreement must be maintained in the provider’s records.

Links

Link to References

Medical policies do not constitute medical advice, nor are they intended to govern the practice of medicine. They are intended to reflect Highmark's reimbursement and coverage guidelines. Coverage for services may vary for individual members, based on the terms of the benefit contract.

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Provides free aids and services to people with disabilities to communicate effectively with us, such as:

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If you need these services, contact the Civil Rights Coordinator.

If you believe that the Claims Administrator/Insurer has failed to provide these services or discriminated in another way on the basis of race, color, national origin, age, disability, or sex, you can file a grievance with: Civil Rights Coordinator, P.O. Box 22492, Pittsburgh, PA 15222, Phone: 1-866-286-8295, TTY: 711, Fax: 412-544-2475, email: CivilRightsCoordinator@highmarkhealth.org. You can file a grievance in person or by mail, fax, or email. If you need help filing a grievance, the Civil Rights Coordinator is available to help you.

You can also file a civil rights complaint with the U.S. Department of Health and Human Services, Office for Civil Rights electronically through the Office for Civil Rights Complaint Portal, available at https://ocrportal.hhs.gov/ocr/portal/lobby.jsf, or by mail or phone at:

U.S. Department of Health and Human Services
200 Independence Avenue, SW
Room 509F, HHH Building
Washington, D.C. 20201
1-800-368-1019, 800-537-7697 (TDD)

Complaint forms are available at http://www.hhs.gov/ocr/office/file/index.html.

Insurance or benefit/claims administration may be provided by Highmark, Highmark Choice Company, Highmark Coverage Advantage, Highmark Health Insurance Company, First Priority Life Insurance Company, First Priority Health, Highmark Benefits Group, Highmark Select Resources, Highmark Senior Solutions Company or Highmark Senior Health Company, all of which are independent licensees of the Blue Cross and Blue Shield Association, an association of independent Blue Cross and Blue Shield plans.

Highmark retains the right to review and update its medical policy guidelines at its sole discretion. These guidelines are the proprietary information of Highmark. Any sale, copying or dissemination of the medical policies is prohibited; however, limited copying of medical policies is permitted for individual use.