IMPORTANT REMINDER
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan or government program (e.g., TennCare), the express terms of the health plan or government program will govern.
POLICY
I. INDICATIONS
The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.
FDA-Approved Indications
Crysvita is indicated for the treatment of:
1. X-linked hypophosphatemia (XLH) in adult and pediatric patients 6 months of age and older.
2. FGF23-related hypophosphatemia in tumor-induced osteomalacia (TIO) associated with phosphaturic mesenchymal tumors that cannot be curatively resected or localized in adult and pediatric patients 2 years of age and older.
All other indications are considered experimental/investigational and not medically necessary.
II. DOCUMENTATION
A. X-linked hypophosphatemia
Submission of the following information is necessary to initiate the prior authorization review for X-linked hypophosphatemia (XLH):
1. Initial requests:
a. Radiographic evidence of rickets or other bone disease attributed to XLH
b. At least one of the following:
i. Genetic testing results confirming the member has a PHEX (phosphate regulating gene with homology to endopeptidases located on the X chromosome) mutation
ii. Genetic testing results confirming a PHEX mutation in a directly related family member with appropriate X-linked inheritance
iii. Lab test results confirming the member’s serum fibroblast growth factor 23 (FGF23) level is above the upper limit of normal or abnormal for the assay.
2. Continuation of therapy requests: documentation (e.g., chart notes, lab test results) of a response to therapy (e.g., increase or normalization in serum phosphate, improvement in bone and joint pain, reduction in fractures, improvement in skeletal deformities).
B. Tumor induced osteomalacia
Submission of the following information is necessary to initiate the prior authorization review for tumor induced osteomalacia (TIO):
1. Initial requests:
a. Lab test results confirming the member’s serum fibroblast growth factor 23 (FGF23) level is above the upper limit of normal or abnormal for the assay.
b. Fasting serum phosphorus levels less than 2.5 mg/dL
c. Ratio of renal tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) less than 2.5 mg/dL
2. Continuation of therapy requests: documentation (e.g., chart notes, lab test results) of a response to therapy (e.g., increase or normalization in serum phosphate, improvement in bone and joint pain, reduction in fractures, improvement in skeletal deformities).
III. CRITERIA FOR INITIAL APPROVAL
A. X-linked hypophosphatemia (XLH)
Authorization of 12 months may be granted for treatment of X-linked hypophosphatemia (XLH) when both of the following criteria is met:
1. The member meets one of the following:
a. Genetic testing was conducted to confirm a PHEX mutation in the member and genetic testing results were submitted confirming diagnosis.
b. Genetic testing was conducted to confirm a PHEX mutation in a directly related family member with appropriate X-linked inheritance and genetic testing results were submitted confirming diagnosis.
c. Member’s FGF23 level is above the upper limit of normal or abnormal for the assay and lab test results were submitted confirming diagnosis.
2. Member has radiographic evidence of rickets or other bone disease attributed to XLH.
B. Tumor-induced osteomalacia (TIO)
Authorization of 12 months may be granted for treatment of tumor-induced osteomalacia (TIO) when the following criteria is met:
1. Member’s diagnosis is confirmed by ALL of the following and lab test results were submitted confirming diagnosis:
a. FGF23 level is above the upper limit of normal or abnormal for the assay.
b. Fasting serum phosphorus levels are less than 2.5 mg/dL
c. Ratio of renal tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) is less than 2.5 mg/dL
2. Member’s disease is associated with phosphaturic mesenchymal tumors that cannot be curatively resected or localized.
IV. CONTINUATION OF THERAPY
Authorization of 12 months may be granted for continued treatment in members requesting reauthorization for an indication listed in Section III who are currently receiving the requested medication through a paid pharmacy or medical benefit and who are experiencing benefit from therapy as evidenced by disease improvement or disease stability (e.g., increase or normalization in serum phosphate, improvement in bone and joint pain, reduction in fractures, improvement in skeletal deformities).
APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS
BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.
ADDITIONAL INFORMATION
For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).
REFERENCES
1. Crysvita [package insert]. Bedminster, NJ: Kyowa Kirin, Inc.; March 2023
2. NIH. U.S. National Library of Medicine. ClinicalTrials.gov website. http://clinicaltrials.gov/ct2/show/NCT02163577. Accessed October 24, 2018.
3. NIH. U.S. National Library of Medicine. ClinicalTrials.gov website. http://clinicaltrials.gov/ct2/show/NCT02526160. Accessed October 24, 2018.
4. Dieter, H., Emma, F., Eastwood, D.M., et.al. Clinical Practice Recommendations for the Diagnosis and Management of X-linked Hypophosphataemia. Nature Reviews Nephrology 15, 435-455 (2019).
5. NIH. U.S. National Library of Medicine. ClinicalTrials.gov website. http://clinicaltrials.gov/ct2/show/NCT02304367. Accessed June 30, 2020.
6. Chong WH, Molinolo AA, Chen CC, et.al Tumor-induced Osteomalacia. Endocrine Related Cancer 18:R53-R77 (2011).
7. Fauconnier C, Roy T, Gillerot G, et al. FGF23: Clinical usefulness and analytical evolution. Clin Biochem. 2019
ORIGINAL EFFECTIVE DATE: 5/25/2018
MOST RECENT REVIEW DATE: 4/9/2024
ID_CHS
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
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