Coverage Policies

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Soliris requires pre-authorization.

2)    Soliris is the first medicine approved by the Food and Drug Administration for the treatment of paroxysmal nocturnal Hemoglobinuria (PNH), which is a rare disease characterized by the spontaneous breakdown of red blood cells usually at night.

3)    Soliris is also covered for hemolytic-uremic syndrome, adult patients with generalized myasthenia gravis (gMG) who are anti-acetylcholine receptor (AchR) antibody positive, and adult patients with neuromyelitis optica spectrum disorder who are anti-aquaporin-4 (AQPR) antibody positive.

4)    Soliris is an intravenous drug only available through a specialty pharmacy. It is covered under the medical benefit (see your cost sharing table).

All initial approval durations are for 6 months.

Paroxysmal Nocturnal Hemoglobinuria

Soliris (Eculizumab) is covered only for paroxysmal nocturnal Hemoglobinuria (PMH) when meeting the following criteria:

a)    Flow Cytometric confirmation of at least 10% PNH type III red cells and platelet counts of at least 30,000/microliter and;

b)    Member is either transfusion dependent (i.e., has at least 1 transfusion in the 24 months prior to initiation of Eculizumab due to documented hemoglobin less than 7 g/dl in persons without anemic symptoms or less than 9 g/dl in persons with symptoms from anemia), or member has a documented history of major adverse vascular events from thromboembolism and;

c)    Member has been vaccinated against meningococcal infection (at least 2 weeks prior to Eculizumab treatment, if not previously vaccinated.

d)    Dose does not exceed 600mg per week for the first 4 weeks, followed by 900mg for the fifth dose 1 week later, then 900mg every 2 weeks thereafter

OR

Atypical Hemolytic Uremic Syndrome

a)    A Diagnosis of atypical hemolytic-uremic syndrome (i.e. complement-mediated HUS);

b)    Prescribed by or in consultation with a hemotologist or nephrologist;

c)    Age > 2 months;

d)    Member has signs of thrombotic microangiopathies (TMA) as evidenced by all of the following (a, b, and c):

1.    Platelet count <150 x 109/L;

2.    Hemolysis such as an elevation in serum lactate dehydrogenase (LDH);

3.    Serum creatinine above the upper limits of normal or member requires dialysis;).

e)    Documentation that member does not have either of the following:

1.    A disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency;

2.    STEC-HUS;

f)     Dose does not exceed 900mg per week for the first 4 weeks, followed by 1,200mg for the fifth dose 1 week later, then 1,200mg every 2 weeks thereafter.

OR

Generalized myasthenia gravis (gMG)

b)    Diagnosis of gMG;

c)    Prescribed by or in consultation with a neurologist;

d)    Age > 18 years;

e)    Myasthenia Gravis-Activities of Daily Living (MG-ADL) score > 6 at baseline;

f)     Myasthenia Gravis Foundation of America Clinical Classification (MGFA) Class II to IV;

g)    Member has positive serologic test for ant9-AChR antibodies;

h)    Failure of a corticosteroid unless contraindicated or clinically significant adverse effects are experienced;

i)     Failure of a cholinesterase inhibitor unless contraindicated or clinically significant adverse effects are experienced;

j)      Failure of two (2) immunosuppressive therapies unless contraindicated or clinically significant adverse effects are experienced;

k)     Dose does not exceed 900mg per week for the first 4 weeks, followed by 1,200mg for the fifth dose 1 week later, then 1,200mg every 2 weeks thereafter

Neuromyelitis Optica Spectrum Disorder (NMOSD)

a)    Diagnosis of NMOSD;

b)    Prescribed by or in consultation with a neurologist;

c)    Age > 18 years;

d)    Member has positive serologic test for anti-AQP4 antibodies;

e)    Member has experienced at least one relapse within the previous 12 months;

f)     Member meets one of the following (a or b):

1.    History of at least two relapses during the previous 12 months;

2.    History of three relapses during the previous 24 months;

g)    Baseline expanded disability status score (EDSS) score of <7;

h)    Failure of rituximab at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced.

i)     Dose does not exceed 900mg per week for the first 4 weeks, followed by 1,200mg for the fifth dose 1 week later, then 1,200mg every 2 weeks thereafter.

Continued Therapy

A. Paroxysmal Nocturnal Hemoglobinuria and Atypical Hemolytic Uremic Syndrome (must meet all):

1. Member is responding positively to therapy as evidenced by, including but not limited to, improvement in any of the following parameters (a or b): a. PNH: i. Improved measures of intravascular hemolysis (e.g., normalization of LDH);

2.  Reduced need for red blood cell transfusions;

a)    Increased or stabilization of hemoglobin levels;

b)    Less fatigue;

c)    Improved health-related quality of life;

d)    Fewer thrombotic events;

e)    aHUS: i. Improved measures of intravascular hemolysis (e.g., normalization of LDH);

f)     Increased or stabilized platelet counts;

g)    Improved or stabilized serum creatinine or estimated glomerular filtration rate (eGFR);

h)    Reduced need for dialysis;

3. If request is for a dose increase, new dose does not exceed (a or b):

a. For PNH: 900 mg every 2 weeks;

b. For aHUS: 1,200 mg every 2 weeks.

Approval duration: 6 months

4. Generalized Myasthenia Gravis (must meet all):

a. Member is responding positively to therapy as evidenced by a 2-point reduction in MG-ADL total score;

b. If request is for a dose increase, new dose does not exceed 1,200 mg every 2 weeks.

Approval duration: 6 months

5. Neuromyelitis Optica Spectrum Disorder (must meet all):

a. Member is responding positively to therapy – including but not limited to improvement or stabilization in any of the following parameters:

1. Frequency of relapse;

2. EDSS;

3. Visual acuity;

b. If request is for a dose increase, new dose does not exceed 1,200 mg every 2 weeks.

Approval duration: 6 months

Codes Used In This BI:

J1300   Eculizumab Injection, 10mg

1)    PNH is a rare genetic disease that affects approximately 8,000 to 10,000 people in North America and Europe. The average age of onset is in the early 30’s with an estimated median survival between 10 and 15 years from the time of diagnosis.

2)    Patients with this disorder lack a protein that protects red blood cells from destruction by the body’s immune system. Soliris selectively blocks this protein, known as terminal complement, preventing hemolysis. Symptoms of PNH include severe anemia, disabling fatigue, recurrent pain, intermittent episodes of dark colored urine (Hemoglobinuria), kidney disease and blood clots (thrombosis).

1)    Soliris Prescribing Information. New Haven, CT: Alexion Pharmaceuticals, Inc.; June 2019.

2)    US Food and Drug Administration; FDA Approves First-of-its-Kind Drug to Treat Rare Blood Disorder at; http://www.fda.gov/bbs/topics/NEWS/2007/NEW01589.html

3)    Clinical Pharmacology. Accessed online 07-31-20