Chronic Iron Overload

POLICY NUMBER

L.5.01.488

Exjade (deferasirox)

Ferriprox (deferiprone)

Jadenu (deferasirox)

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Iron overload occurs when there is an increased absorption of iron from a normal diet or when the subject has received large doses of parenteral iron from multiple red cell transfusions or multiple infusions of intravenous iron, or, rarely, from excessive oral intake of iron. The presence of a serum ferritin >200 to 300 ng/mL in men or >150 to 200 ng/mL in women and/or a transferrin saturation >45 percent obtained on two separate occasions indicates the presence of iron overload. Iron overload should be treated to prevent end-organ damage, and treatment may include chelation therapy to remove excess iron from the body. Deferasirox and deferiprone are orally active iron chelators.

Jadenu (deferasirox) and Exjade (deferasirox) are indicated for treatment of chronic iron overload due to blood transfusions in patients 2 years of age and older and chronic iron overload in patients 10 years of age and older with non-transfusion-dependent thalassemia (NTDT) syndromes and with a liver iron concentration (LIC) of at least 5 mg of iron per gram of dry weight and a serum ferritin greater than 300 mcg/L.

Ferriprox (deferiprone) is indicated for the treatment of transfusional iron overload in adult and pediatric patients with thalassemia syndromes, sickle cell disease, or other anemias. Ferriprox Oral Solution is indicated in patients 3 years of age and older, while Ferriprox Tablets are indicated in patients 8 years of age and older.

Related Medical Policies - 

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements.

Ferriprox (deferiprone) is currently not covered on all BCBSMS formularies.

Initial Criteria

Exjade (deferasirox) or Jadenu (deferasirox) may be considered medically necessary when all of the following criteria are met:

1. ONE of the following:

   1. The individualhas a documented diagnosis of chronic iron overload due to blood transfusions and ALL of the following:

      1. The individual is 2 years of age or older;

      2. Transfusion history of at least 100 mL/kg of packed red blood cells (e.g., at least 20 units of packed red blood cells for a 40 kg person or more in individuals weighing more than 40 kg); AND

      3. Serum ferritin consistently >1000 mcg/L; OR

   2. The individual has a documented diagnosis of chronic iron overload due to a non-transfusion-dependent thalassemia (NTDT) syndrome and ALL of the following:

      1. The individual is 10 years of age or older;

      2. Serum ferritin >300 mcg/L; AND

      3. Liver iron concentration (LIC) of at least 5 milligrams of iron per gram of liver dry weight (mg Fe/g dw);

2. ONE of the following:

   1. The request is for tablets; OR

   2. The request is for sprinkles/oral granules or soluble tablets and ONE of the following:

      1. The individual is <10 years of age; OR

      2. The individual has a documented difficulty swallowing tablets;

3. The individual does not have high-risk myelodysplastic syndromes or advanced malignancies;

4. BOTH of the following:

   1. Duplicate measurements of the individual’s serum creatinine obtained prior to initiation of therapy; AND

   2. The individual’s renal function will be monitored weekly for the first month of therapy and then at least monthly thereafter;

5. BOTH of the following:

   1. The individual’s serum transaminase and bilirubin are measured prior to initiation of therapy; AND

   2. The individual’s serum transaminase and bilirubin will be measured every 2 weeks during the first month of therapy and at least monthly thereafter;

6. The individual does not have any contraindication(s) to therapy with the requested agent;

7. The individual is not currently being treated with other iron chelation therapy; AND

8. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 6 months

Renewal Criteria

Exjade (deferasirox) or Jadenu (deferasirox) may be approved for RENEWAL when ALL of the following criteria are met:

1. The individual has previously been approved for therapy through BCBSMS PA process;

2. The individual has documented clinical improvement (i.e. trend of decreasing ferritin);

3. The individual's blood counts, liver function, renal function and ferritin are checked monthly; AND

4. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of approval: 6 months

POLICY EXCEPTIONS

State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A.  consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B.  appropriate with regard to standards of good medical practice; and

C.  not solely for the convenience of the Member, his or her Provider; and

D.  the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

BCBSMS may request medical records for determination of medical necessity. When medical records are requested, letters of support and/or explanation are often useful, but are not sufficient documentation unless all specific information needed to make a medical necessity determination is included.

POLICY HISTORY

04/05/2016: New policy added. Effective 04/01/2016.

05/31/2016: Policy number L.5.01.488 added.

06/13/2016: Approved by Pharmacy & Therapeutics (P&T) Committee.

05/16/2017: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee.

11/01/2017: Policy section updated to separate and specify coverage criteria for transfusional iron overload and NTDT syndromes for Exjade® and Jadenu®.

03/27/2018: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee.

03/30/2018: Added drug names to the top of the policy. Exjade and Jadenu indication criteria for transfusion timeframe updated to lifetime.

07/30/2020: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding indications for deferasirox. Policy section updated to add that the use of samples by a Member will not be considered current or stable therapy for Medical Policy review. Combined policy statements and revised listed criteria. Added renewal criteria for Exjade and Jadenu. Sources updated.

07/13/2021: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Policy section updated to state that Ferriprox is currently not covered on any BCBSMS formularies. Updated the medical necessity criteria for Jadenu. Policy statement revised to state that Exjade is considered not medically necessary as there are other formulary alternatives covered by the Plan. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity."

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

10/01/2024: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy section updated to change "member" to "individual." Policy statements updated to remove Jadenu. Medically necessary statement updated regarding soluble tablets, measurements of serum creatinine, documentation of monitored renal function, documentation and measurement of serum transaminase and bilirubin, and treatment with other iron chelation therapy. Removed statement that Exjade (deferasirox) is considered not medically necessary as there are other formulary alternatives covered by the Plan. Sources updated. Effective 12/01/2024.

09/09/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding treatment for iron overload and indications for Jadenu (deferasirox), Exjade (deferasirox), and Ferriprox (deferiprone). Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Policy statement regarding coverage of Ferriprox (deferiprone) updated to change "any BCBSMS formularies" to "all BCBSMS formularies." Initial and Renewal policy statements updated to add the brand name drugs. Medically necessary policy statements revised to add age requirement for the diagnosis indicated. Sources updated.

SOURCE(S)

1. Cappellini MD, Cohen A, Eleftheriou A, et al. Guidelines for the Clinical Management of Thalassaemia [Internet]. 2nd Revised edition. Nicosia (CY): Thalassaemia International Federation; 2008. Chapter 3, Iron Overload. Available from: https://www.ncbi.nlm.nih.gov/books/NBK173958/

2. Exjade prescribing information. Novartis Pharmaceuticals Corporation. July 2024. Last accessed June 2025.

3. Ferriprox Oral Solution prescribing information. Chiesi USA, Inc. May 2025. Last accessed June 2025.

4. Ferriprox Tablet prescribing information. Chiesi USA, Inc. March 2025. Last accessed June 2025.

5. Jadenu prescribing information. Novartis Pharmaceuticals Corporation. May 2025. Last accessed June 2025.

CODE REFERENCE

None