PULMOZYME, Dornase Alfa Form

Dornase alfa (Pulmozyme®) is a recombinant DNase enzyme. FDA Approved Indication(s) Pulmozyme is indicated in conjunction with standard therapies for the management of cystic fibrosis (CF) patients to improve pulmonary function. In CF patients with a forced vital capacity ≥ 40% of predicted, daily administration of Pulmozyme has also been shown to reduce the risk of respiratory tract infections requiring parenteral antibiotics. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that Pulmozyme is medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Cystic Fibrosis (must meet all):

1. Diagnosis of CF;
   2. Prescribed by or in consultation with a pulmonologist or an expert in treatment of cystic fibrosis;
2. Therapeutic plan includes concomitant use of standard CF therapies (e.g., antimicrobials, bronchodilators, mucolytics, chest physiotherapy);
3. Dose does not exceed both of the following (a and b): a. 5 mg per day; b. 2 ampules per day. Approval duration: 6 months B. Other diagnoses/indications (must meet 1 or 2):

4. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: Page 1 of 6

   CLINICAL POLICY Dornase Alfa CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

5. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
   II. Continued Therapy A. Cystic Fibrosis (must meet all):
6. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B);
   2. Member is responding positively to therapy;
   3. If request is for a dose increase, new dose does not exceed both of the following (a and b): a. 5 mg per day; b. 2 ampules per day. Approval duration: 12 months B. Other diagnoses/indications (must meet 1 or 2):
7. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

8. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. Page 2 of 6

   CLINICAL POLICY Dornase Alfa III. Diagnoses/Indications for which coverage is NOT authorized:
   A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policy – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid, or evidence of coverage documents.
   IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key CF: cystic fibrosis FDA: Food and Drug Administration Appendix B: Therapeutic Alternatives Not applicable Appendix C: Contraindications/Boxed Warnings • Contraindication(s): known hypersensitivity to dornase alfa, Chinese Hamster Ovary cell products, or any component of the product • Boxed warning(s): none reported Appendix D: General Information • Dornase alfa is recommended for chronic use in both mild and moderate-to-severe disease per the American Thoracic Society 2013 CF guidelines.
   • Severity of lung disease is defined by FEV1 predicted as follows: normal, > 90% predicted; mildly impaired, 70-89% predicted; moderately impaired, 40-69% predicted; and severely impaired, < 40% predicted. V. Dosage and Administration
   Indication Dosing Regimen CF One 2.5 mg ampule inhaled QD; some patients may benefit from BID administration Maximum Dose 5 mg/day VI. Product Availability
   Inhalation solution in single-use ampules: 2.5 mg/2.5 mL VII. References

   1. Pulmozyme Prescribing Information. South San Francisco, CA: Genentech, Inc.; July 2021. Available at: https://www.gene.com/download/pdf/pulmozyme_prescribing.pdf. Accessed October 7, 2022.
9. Mogayzel PJ, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. April 1, 2013; 187(7): 680-689.

10. Kapnadak SG, Dimango E, Hadjiliadis D, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros 2020 May;19(3):344-354. Doi: 10.1016/j.jcf.2020.02.015. Page 3 of 6

    CLINICAL POLICY Dornase Alfa

11. Cystic Fibrosis Foundation: Clinical Care Guidelines. Available at: https://www.cff.org/medical-professionals/clinical-care-guidelines. Accessed October 7,

    2022. Coding Implications Codes referenced in this clinical policy are for informational purposes only. Inclusion or exclusion of any codes does not guarantee coverage. Providers should reference the most up-to- date sources of professional coding guidance prior to the submission of claims for reimbursement of covered services. HCPCS Codes
          J7639

    Dornase alfa, inhalation solution, FDA-approved final product, noncompounded, administered through DME, unit dose form, per mg Reviews, Revisions, and Approvals 1Q18 annual review:

    • Medicaid: Removed initial requirement that therapeutic plan includes concomitant use of standard CF therapies as this is non- specific.
    • HIM: policy revised to apply
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