Metabolic Disorders

Imcivree^® (Setmelanotide) Approval Criteria:

• An FDA approved indication of chronic weight management in adult and pediatric members 2 years of age and older with obesity due to 1 of following:
  • Proopiomelanocortin (POMC), proprotein convertase subtilisin/kexin type 1 (PCSK1), or leptin receptor (LEPR) deficiency; or
  • Bardet-Biedl syndrome (BBS); and
• For POMC-, PCSK1-, or LEPR-deficiency, diagnosis must be confirmed by molecular genetic testing to confirm homozygous or compound heterozygous variants in the POMC, PCSK1, or LEPR genes that are interpreted as pathogenic, likely pathogenic, or of uncertain significance (results of genetic testing must be submitted); and
• For BBS, diagnosis must be confirmed by the following:
  • Molecular genetic testing to confirm homozygous or compound heterozygous variants in a BBS gene that are interpreted as pathogenic or likely pathogenic (results of genetic testing must be submitted); and
  • Clinical features of BBS supported by detailed clinical documentation of each feature (medical records/clinical documentation of each feature must be submitted), as follows:
    • Four primary features (i.e., rod-cone dystrophy, polydactyly, obesity, learning disabilities, hypogonadotropic hypogonadism and/or genitourinary anomalies, renal anomalies); or
    • Three of the primary features previously listed in 3.b.i. plus 2 secondary features [i.e., speech disorder/delay, strabismus/cataracts/astigmatism, brachydactyly/syndactyly, developmental delay, poor coordination/imbalance, mild spasticity (especially lower limbs), diabetes mellitus, dental crowding/hypodontia/small roots/high arched palate, left ventricular hypertrophy/congenital heart disease, hepatic fibrosis]; and
• Requests for Imcivree^® for obesity due to suspected POMC-, PCSK1-, or LEPR-deficiency with POMC, PCSK1, or LEPR variants classified as benign or likely benign, or other types of obesity not related to POMC, PCSK1 or LEPR deficiency, or BBS including obesity associated with other genetic syndromes, or general obesity will not be approved; and
• Member is currently on a dietician-guided diet and exercise program and has previously failed a dietician-guided diet and exercise program alone; and
• Member’s baseline weight and body mass index (BMI) must be provided; and
• Baseline BMI must be ≥30kg/m² for adults or ≥95th percentile on BMI-for-age growth chart assessment for children; and
• Member must not be actively suicidal or have uncontrolled depression and prescriber must verify member will be monitored for depression prior to starting Imcivree^® therapy and throughout treatment; and
• Prescriber must verify member has been counseled on potential sexual adverse reactions and when to seek emergency medical care; and
• Prescriber must verify member does not have end stage renal disease [estimated glomerular filtration rate (eGFR) <15mL/min/1.73m²] and must confirm the dose will be adjusted per package labeling for members with severe renal impairment (eGFR 15 to 29mL/min/1.73m²); and
• Prescriber must verify female member is not pregnant or breastfeeding; and
• Prescriber must confirm member or caregiver has been trained on the proper storage and administration of Imcivree^® prior to the first dose; and
• For POMC-, PCSK1-, or LEPR-deficiency, initial approvals will be for the duration of 16 weeks. Reauthorization may be granted if the prescriber documents the member’s current weight or BMI and member has achieved weight loss of ≥5% of baseline body weight or ≥5% of BMI; or
• For BBS, approvals will be for the duration of 1 year. Reauthorization may be granted if the prescriber documents the member’s current weight or BMI and member has achieved weight loss of ≥5% of baseline body weight or ≥5% of BMI; and
• A quantity limit of 9mL per 30 days will apply.

Rezdiffra^® (Resmetirom) Approval Criteria:

• An FDA approved indication of noncirrhotic nonalcoholic steatohepatitis (NASH); and
• Member must be 18 years of age or older; and
• Member must have moderate-to-advanced liver fibrosis (e.g., stage F2 or F3) confirmed by at least 1 of the following (results of the selected test must be submitted with the request):
  • FibroScan with vibration controlled transient elastography (VCTE) ≥8.5kPa and controlled attenuation parameter (CAP) ≥280dB/min; or
  • Enhanced Liver Fibrosis (ELF) biochemical test score ≥9; or
  • Liver biopsy showing stage F2 or F3 fibrosis with NASH; and
• Member must not have known liver cirrhosis (e.g., stage F4); and
• Must be used in conjunction with diet and exercise [clinical documentation (e.g., office notes) of member’s diet and exercise program must be included with the request]; and
• Prescriber must attest that metabolic comorbidities are being appropriately managed, including treatment for all of the following, if applicable:
  • Type 2 diabetes; and
  • Dyslipidemia; and
  • Hypertension; and
• Member must not be taking strong CYP2C8 inhibitors (e.g., gemfibrozil) or OATP1B1/OATP1B3 inhibitors (e.g., cyclosporine) concurrently with Rezdiffra; and
• If member is taking a moderate CYP2C8 inhibitor (e.g., clopidogrel) concurrently with Rezdiffra^®, prescriber must agree to reduce the dose as required in the package labeling; and
• If the member is taking a statin, prescriber must agree to adjust the statin dosage (when necessary) and monitor for statin-related adverse reactions; and
• A trial of Wegovy^® (semaglutide injection) at maintenance dosing for at least 3 months (unless contraindicated) that did not provide an adequate response; and
  • If combination therapy of Rezdiffra^® with Wegovy^® is being requested, a patient-specific, clinically significant reason why the member requires combination therapy must be provided; and
• Must be prescribed by, or in consultation with, a gastroenterologist or hepatologist (or an advanced care practitioner with a supervising physician who is a gastroenterologist or hepatologist); and
• Initial approvals will be for the duration of 6 months. Subsequent approvals (for the duration of 1 year) will be approved if the prescriber documents the member is tolerating and responding well to the medication; and
• A quantity limit of 30 tablets per 30 days will apply.

Wegovy^® (Semaglutide Injection and Tablets) Approval Criteria [Cardiovascular (CV) Risk Reduction Indication Only]:

• An FDA approved indication to reduce the risk of major adverse cardiovascular (CV) events in members with established CV disease (CVD) and either obesity or overweight; and
  • Wegovy^® will not be approved for obese or overweight members in the absence of established CVD; and
• Member must be 45 years of age or older; and
• Member must have established CVD with a history of 1 of the following (documentation must be submitted with the request):
  • Previous myocardial infarction; or
  • Previous stroke; or
  • Symptomatic peripheral arterial disease confirmed by 1 of the following:
    • Intermittent claudication with ankle-brachial index <0.85 at rest; or
    • Peripheral arterial revascularization procedure; or
    • Amputation due to atherosclerotic disease; and
• Member has a body mass index (BMI) ≥27kg/m²; and
• Member does not have type 1 diabetes mellitus (T1DM) or type 2 diabetes mellitus (T2DM); and
• Member has a hemoglobin A1C (HbA1c) <6.5%; and
• Member will not be using Wegovy^® in combination with other semaglutide-containing products or any other glucagon-like peptide-1 (GLP-1) receptor agonist; and
• Member is currently receiving guideline-directed management and therapy (GDMT) for CVD (e.g., antihypertensives, lipid-lowering agents, antiplatelets), as documented in the member’s pharmacy claims history, unless contraindicated; and
• Wegovy^® must be used in conjunction with diet and exercise [clinical documentation (e.g., office notes) of this discussion with the member must be included with the request]; and
• Initial approvals will be for the titration period to allow initial and escalation dosing. A separate prior authorization request must be submitted for each dose; and
  • Approvals will be for 8 weeks at a time to allow for proper dose escalation; and
  • An additional 8 weeks for each dose may be approved for those who experience intolerable adverse effects during dose escalation with proper documentation; and
  • Members who cannot tolerate dose escalation after an additional 8 week approval will not be approved for continuation; and
• Subsequent approvals for the maintenance dose (1.7mg or 2.4mg for the injection and 25mg for the tablets) will be approved for 1 year if the prescriber documents the following:
  • Member is tolerating maintenance dosing; and
  • Member has not developed T1DM or T2DM; and
  • Member is continuing all of the following in conjunction with Wegovy^®:
  • Reduced calorie diet; and
  • Increased physical activity; and
  • GDMT for CVD where applicable; and
• A quantity limit of 4 pens per 28 days or 30 tablets per 30 days will apply; and
• Wegovy^® should be discontinued in members who cannot tolerate at least the 1.7mg once weekly maintenance dosing.

Wegovy^® (Semaglutide Injection) Approval Criteria [Metabolic Dysfunction-Associated Steatohepatitis (MASH) Diagnosis Only]:

• An FDA approved indication of noncirrhotic MASH; and
  • Wegovy^® will not be approved for obese members in the absence of MASH; and
• Member must be 18 years of age or older; and
• Member must have moderate-to-advanced liver fibrosis (e.g., stage F2 or F3) confirmed by at least 1 of the following (results of the selected test must be submitted with the request):
  • FibroScan with vibration controlled transient elastography (VCTE) ≥8kPa and controlled attenuation parameter (CAP) ≥280dB/min; or
  • Enhanced Liver Fibrosis (ELF) biochemical test score ≥9; or
  • Liver biopsy showing stage F2 or F3 fibrosis with MASH; and
• Member must not have chronic liver disease other than metabolic dysfunction-associated steatotic liver disease (MASLD); and
• Member does not have type 1 diabetes mellitus (T1DM) or type 2 diabetes mellitus (T2DM); and
• Wegovy^® must be used in conjunction with diet and exercise [clinical documentation (e.g., office notes) of this discussion with the member must be included with the request]; and
• Prescriber must attest that metabolic comorbidities are being appropriately managed, including treatment for all of the following, if applicable:
  • T2DM; and
  • Dyslipidemia; and
  • Hypertension; and
• Member will not be using Wegovy^® in combination with other semaglutide-containing products or any other glucagon-like peptide-1 (GLP-1) receptor agonist; and
• Must be prescribed by, or in consultation with, a gastroenterologist or hepatologist (or an advanced care practitioner with a supervising physician who is a gastroenterologist or hepatologist); and
• Initial approvals will be for the titration period to allow initial and escalation dosing. A separate prior authorization request must be submitted for each dose; and
  • Approvals will be for 8 weeks at a time to allow for proper dose escalation; and
  • An additional 8 weeks for each dose may be approved for those who experience intolerable adverse effects during dose escalation with proper documentation; and
  • Members who cannot tolerate dose escalation after an additional 8 week approval will not be approved for continuation; and
• Subsequent approvals for the maintenance dose (1.7mg or 2.4mg) will be approved for 1 year if the prescriber documents the following:
  • Member is tolerating maintenance dosing; and
  • Member has not developed T1DM or T2DM; and
  • Member is continuing a reduced calorie diet and increased physical activity in conjunction with Wegovy^®; and
• A quantity limit of 4 pens per 28 days will apply; and
• Wegovy^® should be discontinued in members who cannot tolerate at least the 1.7mg once weekly maintenance dosing.

Wegovy® PA Form

PA Approval Criteria:

• An FDA approved diagnosis of acute hepatic porphyria (AHP) confirmed by:
  
  • Genetic testing; OR
  • Elevated urinary porphobilinogen (PBG) and signs/symptoms of AHP; AND
   
• Member must be 18 years of age or older; AND  
• Givlaari™ must be administered in a health care setting by a health care professional prepared to manage anaphylaxis; AND
  
  • Givlaari™ must be shipped to the health care setting where the member is scheduled to receive treatment; AND
   
• The prescriber must agree to monitor liver function tests prior to initiating treatment with Givlaari™, every month during the first 6 months of treatment, and as clinically indicated thereafter; AND  
• The prescriber must agree to monitor renal function during treatment with Givlaari™ as clinically indicated; AND
• Member must not be taking sensitive CYP1A2 or CYP2D6 substrates (e.g., caffeine, dextromethorphan, duloxetine, amitriptyline, olanzapine, fluoxetine, paroxetine, hydrocodone, tramadol) concomitantly with Givlaari™; AND
• The member’s recent weight must be provided on the prior authorization request in order to authorize the appropriate amount of drug required according to package labeling; AND
• Initial approvals will be for the duration of 6 months. Further approval may be granted if the prescriber documents that the member is responding well to treatment as indicated by less porphyria attacks and that the member does not have elevated transaminase levels.

 Prior Authorization form

Kebilidi™ (Eladocagene Exuparvovec-tneq) Approval Criteria:

• An FDA approved diagnosis of aromatic L-amino acid decarboxylase (AADC) deficiency; and
• Diagnosis must be confirmed by
  • Genetic testing confirming biallelic pathogenic or likely pathogenic mutations in the DDC gene (results of genetic testing must be submitted); and
  • Functional confirmation with measured diagnostic variations in AADC enzyme activity in plasma and/or levels of neurotransmitter metabolites in cerebrospinal fluid (CSF) (results of testing must be submitted); and
• Member must be 16 months of age or older; and
• Female members of reproductive potential must not be pregnant and must have a negative pregnancy test prior to Kebilidi™ administration; and
• Must be prescribed by a neurologist, neurosurgeon, or a specialist with expertise in the treatment of AADC deficiency; and
• Prescriber must verify the member has confirmed skull maturity as assessed by neuroimaging; and
• Must be administered by intraputaminal infusion in a medical center that specializes in stereotactic neurosurgery in addition to the preparation and infusion of Kebilidi™; and
• Must be shipped via cold chain supply to the facility where the member is scheduled to receive treatment, and the facility must be capable of adhering to the storage, handling, and preparation requirements as described in the package labeling; and
• Must only be administered using an FDA-authorized cannula for intraparenchymal infusion (e.g., ClearPoint^® SmartFlow^® Neuro Cannula); and
• Approvals will be for 1 treatment per member per lifetime.

• An FDA approved indication for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, or related variants; AND 
• Prescriber documentation that all non-pharmacological treatments failed including the following:
  • Hyperkalemic periodic paralysis:
    • Acute attacks can be aborted with sugar or mild exercise 
    • Avoiding foods rich in potassium 
    • Avoiding fasting 
    • High-carbohydrate diet 
    • Avoiding strenuous activity 
    • Avoiding prolonged cold exposure 
  • Hypokalemic periodic paralysis:
    • Low-carbohydrate diet (avoiding carbohydrate loading) 
    • Avoiding vigorous exercise (some mild attacks can be aborted by low level exercise) 
• Prescriber documentation of frequent and severe attacks requiring pharmacological treatment (at least one attack per week but no more than three attacks per day); AND 
• A four-week trial within the last 90 days of acetazolamide in combination with
  • Spironolactone or triamterene in hypokalemic periodic paralysis; OR 
  • Hydrochlorothiazide in hyperkalemic periodic paralysis 
• A quantity limit of four tablets per day will apply. 
• Initial approvals will be for the duration of three months after which time compliance will be required for continued approval. Additionally, for continuation the prescriber must include information regarding reduced frequency or severity of attacks.

• An FDA approved diagnosis of leptin deficiency in patients with congenital or acquired generalized lipodystrophy; and
• Approvals will not be granted for the following diagnoses:
  • Metabolic disease without current evidence of generalized lipodystrophy
  • HIV-related lipodystrophy
  • General obesity not associated with congenital leptin deficiency
• Myalept™ must be prescribed by an endocrinologist; and
• Prescriber must agree to test for neutralizing antibodies in patients who experience severe infections or if they suspect Myalept™ is no longer effective.
  • Baseline HbA1c, fasting glucose, and fasting triglycerides must be stated on prior authorization request
  • Re-approvals will require recent lab values (HbA1c, fasting glucose, and fasting triglycerides) to ensure neutralizing antibodies have not developed; and
• Prescriber and pharmacy must be enrolled in the Myalept™ REMS program; and
• Approvals will be for the duration of three months to evaluate compliance and ensure the prescriber is assessing continued efficacy; and
• A quantity limit of one vial per day will apply.  
  

Prior Authorization form

Harliku™ (Nitisinone), Nityr^® (Nitisinone), and Orfadin^® (Nitisinone) Approval Criteria [Alkaptonuria (AKU) Diagnosis]:

• An indication to reduce urine homogentisic acid (HGA) in patients with alkaptonuria (AKU); and
  • The diagnosis of AKU must be confirmed by 1 of the following (results of the selected test must be submitted with the request):
    • Genetic testing identifying biallelic pathogenic or likely pathogenic variants in the homogentisate 1,2-dioxygenase (HGD) gene; or 
    • Urine test for HGA showing >0.4 grams of HGA excreted in 24 hours; and
• Nitisinone must be prescribed by, or in consultation with, a geneticist, rheumatologist, or specialist with expertise in the treatment of AKU; and
• The prescriber must confirm the member will receive a baseline ophthalmologic examination prior to initiating nitisinone treatment; and
• The prescriber must confirm the member has been counseled to report any unexplained ocular, neurologic, or other symptoms to their health care provider; and
• Use of Harliku™ will require a documented failed trial of both generic nitisinone 2mg capsules and Nityr^® (nitisinone) 2mg tablets and clinical justification as to why Harliku™ would be expected to confer a different response since it contains the same active ingredient (nitisinone); and
• A quantity limit of 30 tablets for 30 days will apply; and
• Initial approvals will be for the duration of 6 months; and
• Subsequent approvals will be for the duration of 1 year; and
• Reauthorization requires the following:
  • Verification from the prescriber of continued response to therapy (i.e., decrease in urine HGA levels, improvement in joint pain, decrease in visible ochronosis).

Nityr^® (Nitisinone) and Orfadin^® (Nitisinone) Approval Criteria [Hereditary Tyrosinemia (HT-1) Diagnosis]:

• An FDA approved diagnosis of HT-1; and
  • The diagnosis of HT-1 must be confirmed by 1 of the following (results of the selected test must be submitted with the request):
    • Genetic testing identifying biallelic pathogenic or likely pathogenic variants in the fumarylacetoacetase hydrolase (FAH) gene; or
    • Elevated succinylacetone concentrations in the blood or urine; and
• Documentation of active management with a tyrosine and phenylalanine restricted diet; and
• Nitisinone must be prescribed by, or in consultation with, a geneticist or specialist with expertise in the treatment of HT-1; and
• The prescriber must verify the member will receive appropriate ophthalmologic examinations; and
• The prescriber must confirm the member has been counseled to report any unexplained ocular, neurologic, or other symptoms to their health care provider; and
• The member’s recent weight must be provided on the prior authorization request in order to authorize the appropriate amount of drug required according to the package labeling; and
• Initial approvals will be for the duration of 6 months; and
• Subsequent approvals will be for the duration of 1 year; and
• Reauthorization requires the following:
  • Documentation of active management with a tyrosine and phenylalanine restricted diet; and
  • Verification from the prescriber of continued response to therapy (i.e., decrease in plasma and/or urine succinylacetone concentration).

Olpruva™ (Sodium Phenylbutyrate Pellets for Oral Suspension) Approval Criteria:

• An FDA approved diagnosis of urea cycle disorder (UCD); AND
• Member must be actively managing UCD with a protein restricted diet; AND
• A patient-specific, clinically significant reason why the member cannot use sodium phenylbutyrate powder and tablets (generic Buphenyl^®), which are available without a prior authorization, must be provided; AND
• A patient-specific, clinically significant reason why the member cannot use Pheburane^® (sodium phenylbutyrate oral pellets) must be provided; AND
• A maximum daily dose of 20g of sodium phenylbutyrate will apply.

Palynziq^® (Pegvaliase-pqpz) Approval Criteria:

• An FDA approved diagnosis to reduce blood phenylalanine concentrations in patients with phenylketonuria who have uncontrolled blood phenylalanine concentrations >600µmol/L on existing management; AND
• Documentation of active management with a phenylalanine restricted diet; AND
• Baseline phenylalanine concentration must be documented on the prior authorization request and must be drawn within the last 30 days; AND
• Palynziq^® must be prescribed by, or in consultation with, a geneticist, neurologist, or specialist with expertise in the treatment of PKU; and 
• Concomitant use with Kuvan® (sapropterin) or Sephience™ (sepiapterin) will not be approved except to allow for temporary coverage during the titration of Palynziq^®; AND
• Prescriber, pharmacy, and member must be enrolled in the Palynziq™ Risk Evaluation and Mitigation Strategy (REMS) program and maintain enrollment throughout therapy; AND  
• Initial dose must be administered under the supervision of a health care provider equipped to manage anaphylaxis and observe the member for at least 60 minutes following injection; AND
• Member must be prescribed auto-injectable epinephrine and be counseled on its appropriate use; AND
• Initial approvals will be for 1 year to allow for initial titration and maintenance treatment. Reauthorization may be granted if the following information is provided (documentation must be submitted):
  • Member has achieved a 20% reduction in blood phenylalanine concentration from pre-treatment baseline; or
  • Member has achieved a blood phenylalanine concentration ≤600micromol/L; or
  • Member is currently in the titration/maintenance phase of treatment, and the dose is being titrated up to the maximum daily dose of 60mg once daily. Slower dose titrations may be approved based on member’s response and tolerability; and 
• Members who do not achieve at least a 20% reduction in blood phenylalanine concentration from pre-treatment baseline or a blood phenylalanine concentration ≤600µmol/L after at least 16 weeks of continuous treatment with the maximum dosage of 60mg once daily will not be approved for subsequent approvals; AND
• Dose titrations up to the maximum daily dose of 60mg once daily will be permitted to allow members to achieve a blood phenylalanine level ≤360micromol/L based on the current treatment guideline goal for blood phenylalanine level; and 
• Subsequent approvals will be for the duration of one year.
• Reauthorization will require the following:
  • Documentation of active management with a phenylalanine restricted diet; AND
  • Verification from the prescriber of continued response to therapy (i.e., blood phenylalanine level, increase in dietary phenylalanine tolerance, improvement in clinical symptoms). 

Prior Authorization form

Pheburane^® (Sodium Phenylbutyrate Oral Pellets) Approval Criteria:

• An FDA approved diagnosis of urea cycle disorder (UCD); AND
• Member must be actively managing UCD with a protein restricted diet; AND
• A patient-specific, clinically significant reason why the member cannot use sodium phenylbutyrate powder and tablets (generic Buphenyl^®), which are available without a prior authorization, must be provided; AND
• A maximum daily dose of 20g of sodium phenylbutyrate will apply; AND
• A quantity limit of 1,218g of pellets (equivalent to 588g of sodium phenylbutyrate) per 29 days will apply. 

• An FDA approved diagnosis of urea cycle disorder (UCD); AND
• Active management with protein restricted diet; AND
• A patient specific, clinically significant reason why member cannot use Buphenyl® (sodium phenylbutyrate); AND
• A patient-specific, clinically significant reason why the member cannot use Pheburane^® (sodium phenylbutyrate oral pellets) must be provided; AND
• A maximum daily dose of 17.5mL (19g) of glycerol phenylbutyrate will apply; AND
• A quantity limit of 525mL per 30 days will apply. 

Javygtor™ (Sapropterin), Kuvan^® (Sapropterin), and Zelvysia™ (Sapropterin) Approval Criteria:

• An FDA approved diagnosis of phenylketonuria; and
• Documentation of active management with a phenylalanine restricted diet; and
• Member must not have two null mutations in trans; and
• Baseline phenylalanine concentration must be documented on the prior authorization request and must be drawn within the last 30 days; and
• Sapropterin must be prescribed by, or in consultation with, a geneticist, neurologist, or specialist with expertise in the treatment of PKU; and 
• Concomitant use with Palynziq™ (pegvaliase-pqpz) will not be approved except to allow for temporary coverage during the titration of Palynziq^®; and
• Use of Javygtor™ (sapropterin) or Zelvysia™ (sapropterin) will require a patient specific, clinically significant reason why other generic formulations of sapropterin cannot be used; and 
• Initial approvals will be for the duration of 30 days.  After which time, the prescriber must verify that the member responded to treatment as defined by laboratory documentation of greater than or equal to a 30% decrease in blood phenylalanine levels from baseline.
  • If the member was initiated at 10mg/kg/day dose, then a subsequent trial of 20mg/kg/day for a duration of 30 days can be approved.  After which time, the prescriber must verify that the member responded to treatment as defined by laboratory documentation of greater than or equal to a 30% decrease in blood phenylalanine levels from baseline.
  • If the member was initiated at 20mg/kg/day dose, then no additional approvals will be granted after a trial period of 30 days if the member did not respond to treatment as defined by laboratory documentation of greater than or equal to a 30% decrease in blood phenylalanine levels from baseline. 
• Subsequent approvals will be for the duration of one year. 
• Reauthorization will require the following:
  
  • Documentation of active management with a phenylalanine restricted diet; and
  • Verification from the prescriber of continued response to therapy (i.e., blood phenylalanine level, increase in dietary phenylalanine tolerance, improvement in clinical symptoms).

Prior Authorization form

PA Approval Criteria:

• An FDA approved indication to increase pain-free light exposure in adult patients with a history of phototoxic reactions from erythropoietic protoporphyria (EPP); AND  
  • The diagnosis of EPP must be confirmed by genetic testing; AND  
• Member must be 18 years of age or older; AND  
• Scenesse® must be administered by a health care professional who is proficient in the subcutaneous (subQ) implantation procedure and has completed the training program provided by the manufacturer prior to administration of the Scenesse® implant; AND
  • Scenesse® must be shipped via cold chain supply shipping and delivery to the health care setting where the member is scheduled to receive the implant administration; AND
  • Scenesse® must be stored under refrigeration (36 to 46°F) and protected from light prior to implantation; AND  
• The Scenesse® implant should be inserted using an SFM Implantation Cannula or other implantation device that has been determined by the manufacturer to be suitable for implantation of Scenesse®; AND
• The prescriber must agree that the member will be monitored by a health care provider for at least 30 minutes after the implant administration; AND  
• The prescriber must agree that the member will have a full body skin examination performed at least twice yearly while the member is being treated with Scenesse® to monitor pre-existing and new skin pigmentary lesions; AND
• Documentation that member will maintain sun and light protection measures during treatment with Scenesse® to prevent phototoxic reactions related to EPP; AND
• A quantity limit of 1 implant per 60 days will apply. Initial approvals will be for 2 implants for the duration of 4 months. Further approval may be granted if the prescriber documents that the member is responding well to treatment as indicated by increased tolerance of sunlight (i.e., less phototoxic reactions).   

Prior Authorization form

Sephience™ (Sepiapterin) Approval Criteria:

• An FDA approved diagnosis of phenylketonuria (PKU); and
• Documentation of active management with a phenylalanine restricted diet; and
• Baseline phenylalanine concentration must be documented on the prior authorization request and must be drawn within the last 30 days; and
• Sephience must be prescribed by, or in consultation with, a geneticist, neurologist, or specialist with expertise in the treatment of PKU; and
• Concomitant use with Palynziq^® (pegvaliase-pqpz) will not be approved except to allow for temporary coverage during the titration of Palynziq^®; and
• Member must meet 1 of the following (documentation must be provided): 
  • A 3-month trial with sapropterin with inadequate response, defined as blood phenylalanine ≥360micromol/L, despite consistent use in combination with dietary phenylalanine restriction; or
  • Member is a non-responder to sapropterin defined as ≤30% decrease in phenylalanine after 30 days of sapropterin therapy in combination with dietary phenylalanine restriction; or
  • A diagnosis of classic PKU (blood phenylalanine ≥1,200micromol/L at diagnosis or 2 null mutations in trans); or
  • A patient specific, clinically significant reason why the member cannot use generic Kuvan^® (sapropterin) must be provided; and
• Initial approvals will be for 2 weeks. After which time, the prescriber must verify that the member responded to treatment as defined by laboratory documentation of ≥30% reduction in blood phenylalanine levels from baseline; and
  • Members younger than 2 years of age will be approved for a longer dosage titration per the package labeling up to the maximum daily dosage of 60mg/kg/day. After which time, the prescriber must verify that the member responded to treatment as defined by laboratory documentation of ≥30% reduction in blood phenylalanine levels from baseline; or
  • If the member was initiated at 60mg/kg/day, then no additional approvals will be granted after a trial period of 2 weeks if the member did not respond to treatment as defined by laboratory documentation of ≥30% reduction in blood phenylalanine levels from baseline; and
• Subsequent approvals will be for the duration of 1 year; and
• Reauthorization requires the following:
  • Documentation of active management with a phenylalanine restricted diet; and
  • Verification from the prescriber of continued response to therapy (i.e., blood phenylalanine level, increase in dietary phenylalanine tolerance, improvement in clinical symptoms).

Strensiq^® (Asfotase Alfa) Approval Criteria:

• An FDA approved indication for the treatment of members with perinatal/infantile-onset and juvenile-onset hypophosphatasia (HPP); and
• Confirmed diagnosis by the following (results of selected tests must be submitted with the request):
  • Low age- and sex-adjusted alkaline phosphatase (ALP) activity; and
  • At least 1 of the following:
    • Elevated level of a tissue non-specific alkaline phosphatase (TNSALP) substrate [e.g., inorganic pyrophosphate (PPi), phosphoethanolamine (PEA), pyridoxal 5’-phosphate (PLP)]; or
    • Molecular genetic testing documenting pathogenic or likely pathogenic variants in the ALPL gene; and
• Member’s weight (kg) must be provided and must have been taken within the last 4 weeks to ensure accurate weight-based dosing per package labeling; and
• The 80mg/0.8mL vial should not be used in pediatric members weighing <40kg.