Drug Therapy Guidelines

Transcription

1 Drug Therapy Guidelines Applicable* Hereditary Angiedema (HAE) Agents: Berinert (C1 esterase inhibitr [human]), Cinryze (C1 esterase inhibitr [human]), Haegarda (C1 esterase inhibitr [human]) Kalbitr (ecallantide), Firazyr (icatibant), Rucnest (C1 esterase inhibitr [recmbinant]), Takhzyr TM (lanadelumab-fly) Medical Benefit x Effective: 1/3/19 Pharmacy- Frmulary 1 x Next Review: 6/19 Pharmacy- Frmulary 2 x Date f Origin: 3/09 Pharmacy- Frmulary 3/Exclusive x Review Dates: 10/15/06, 11/5/07, 12/15/08,12/09, 12/10, 12/11, Pharmacy- Frmulary 4/AON x 12/12, 12/13, 12/14, 6/15, 6/16, 9/17, 6/18, 9/18 I. Medicatin Descriptin Hereditary Angiedema (HAE) is an autsmal dminant disease caused by mutatin f the C1 inhibitr gene (SERPING1), resulting in lw levels f C1 inhibitr prtein, r lss f functinality f this prtein. Althugh multiple mutatins have been identified, the exact cause f HAE is still unclear. C1 inhibitr prtein plays a rle in fur enzymatic cascades in the bdy, all f which are interrelated t eventually cause the prductin f the peptide bradykinin. Deficiency in C1 inhibitr prtein results in lack f inhibitin f these cascades and increased levels f bradykinin. This uninhibited prductin f bradykinin causes angiedema in patients with HAE. Labratry measurement f C1-INH, C1-INH activity and serum C4 levels are perfrmed fr diagnsis. II. Psitin Statement Cverage is prvided thrugh a prir authrizatin prcess with supprting clinical dcumentatin fr every request. Medical benefit drugs: Cinryze (when administered by a healthcare prfessinal) Haegarda (when administered by a healthcare prfessinal) Berinert (when administered by a healthcare prfessinal) Rucnest (when administered by a healthcare prfessinal) Kalbitr Takhzyr (when administered by a healthcare prfessinal) Pharmacy benefit drugs: Berinert (when self-administered) Cinryze (when self-administered) Firazyr Haegarda (when self-administered) Rucnest (when self-administered) Takhzyr (when self-administered) Page 1 f 6

2 III. Plicy In all cases, the diagnsis f Hereditary Angiedema (HAE) must have (at sme time) been cnfirmed by an allergist, immunlgist, r hematlgist. Cverage f the prphylactic use f Cinryze, Haegarda, r Takhzyr is prvided fr the fllwing: Fr lng-term prphylaxis against angiedema attacks when: The disease is severely symptmatic AND The member is at least: 6 years f age fr Cinryze 10 years f age fr Haegarda 12 years f age fr Takhzyr AND When requesting cverage f a brand medicatin fr which an A/B rated generic is available, cverage will be prvided when there is sufficient evidence that the use f the A/B rated generic equivalent has resulted in inadequate results AND Cverage will be prvided when the member has experienced intlerance r therapeutic failure with ONE plan-preferred medicatin such as attenuated andrgen (i.e. Danazl) r antifibrinlytic (i.e. tranexamic acid) first OR when at least ONE f the fllwing criteria have been met: The plan-preferred medicatins are cntraindicated r will likely cause an adverse reactin by r physical r mental harm t the member. The plan-preferred medicatins are expected t be ineffective based n the knwn clinical histry and cnditins f the member and the member s prescriptin drug regimen. The member has tried the plan-preferred medicatins r anther prescriptin drug in the same pharmaclgic class r with the same mechanism f actin and such prescriptin drug was discntinued due t lack f efficacy r effectiveness, diminished effect, r an adverse event. The member is stable n the medicatin selected by their healthcare prfessinal fr the medical cnditin under cnsideratin (where stable is defined as receiving the medicatin fr an adequate perid f time, have achieved ptimal respnse, and cntinued favrable utcmes are expected UNLESS the medicatin was initially selected slely due t the availability f a drug sample r a cupn card and the member des nt therwise meet the definitin f stable ). The plan-preferred medicatin is nt in the best interest f the member because it will likely cause a significant barrier t the member s adherence r t cmpliance with the member s plan f care, will likely wrsen a cmrbid cnditin f the member, r will likely decrease the member s ability t achieve r maintain reasnable functinal ability in perfrming daily activities. Fr shrt-term prphylaxis against angiedema attacks: Befre surgeries, where endtracheal intubatin is required, where upper airway r pharynx is manipulated, r befre brnchscpy r endscpy AND When the member is at least 6 years f age Page 2 f 6

3 Cverage f the fllwing is prvided fr acute treatment f HAE attacks: Cinryze when the member is at least 6 years f age Haegarda when the member is at least 10 years f age Kalbitr when the member is at least 12 years f age Rucnest when the member is at least 13 years f age Firazyr when the member is at least 18 years f age Berinert when the member is at least 6 years f age IV. Quantity Limitatins Haegarda Medical benefit: sufficient quantity t allw a dse f 60 internatinal units (6 billable units)/kg bdy weight twice weekly Pharmacy benefit: sufficient quantity t allw a dse f 60 internatinal units/kg bdy weight twice weekly Cinryze Medical benefit: 1000 billable units/30 days Pharmacy benefit: 20 vials/30 days Berinert Medical benefit: 800 billable units/30 days Pharmacy benefit: 16 vials/30 days Kalbitr Medical benefit: 240 billable units/30 days Firazyr Pharmacy benefit: 4 syringes/30 days Rucnest Medical benefit: 1680 billable units/30 days Pharmacy benefit: 8 vials/30 days Takhzyr Medical benefit: up t 2 vials/28 days Pharmacy benefit: up t 2 vials/28 days Additinal quantities f each medicatin may be available thrugh cverage review f supprting dcumentatin (i.e., bdy weight, frequency f attacks, etc.). V. Cverage Duratin Cverage is prvided fr 6 mnths and may be renewed. Cverage f shrt-term prphylaxis is limited t 1 mnth. VI. Cverage Renewal Criteria Cverage can be renewed based upn the fllwing criteria: Prphylaxis f hereditary angiedema attacks: Page 3 f 6

4 Dcumentatin must be prvided f a decrease in frequency f HAE attacks versus baseline and/r significant imprvement in the severity r duratin f attacks AND Absence f significant adverse reactin r txicity t medicatin is shwn Acute treatment f hereditary angiedema attacks: Subsequent requests will be authrized based n dcumentatin that member has respnded t prir treatments AND Absence f significant adverse reactin r txicity t medicatin is shwn VII. Billing/Cding Infrmatin Haegarda Haegarda is available as single-use vials cntaining 2000 r 3000 IU f C1-INH Medical when administered by a healthcare prfessinal Pharmacy when self-administered C9015: 1 billable unit = 10 units f drug Cinryze: Medical when administered by a healthcare prfessinal Pharmacy when self-administered Medical J0598 (1 billable unit = 10 units f drug) Pharmacy- 500 units f drug per each single-use vial Berinert: Medical when administered by a healthcare prfessinal Pharmacy when self-administered Medical- J0597 (1 billable unit = 10 units f drug) Pharmacy- 500 units f drug per each single-use vial Kalbitr: Medical benefit nly J1290 (1 billable unit = 1mg f drug; 10mg/ml single use vials cntaining 10mg each, 3 vials per cartn) Firazyr: Pharmacy benefit nly 3ml prefilled syringe (10mg/ml) Rucnest: Medical when administered by a healthcare prfessinal Pharmacy when self-administered Medical J0596 (1 billable unit = 10 units f drug) Pharmacy 2100 IU f drug per each single use vial Takhzyr: Medical when administered by a healthcare prfessinal Pharmacy benefit when self-administered Medical J3590 (1 billable unit = 1 vial) C9399 (1 billable unit = 1 vial) Page 4 f 6

5 300 mg/2 ml (150 mg/ml) slutin in single-dse glass vial Related diagnsis: Other deficiencies f circulating enzymes: D84.1 VIII. Summary f Plicy Changes 9/1/11: Remved requirement f trial f C-1 esterase inhibitr prir t Kalbitr Renewal criteria defined 6/15/12: Firazyr added t plicy 6/2012: Cinryze and Berinert made available under the pharmacy benefit 3/15/13: n changes 3/15/14: Allwed Berinert fr prphylaxis if requested Simplified acute attack cverage criteria Expanded shrt-term prphylaxis cverage situatins 10/22/14: Rucnest added t plicy 3/15/15: change in age requirement fr cverage f Kalbitr 7/1/15: frmulary distinctins made 9/15/15: change in age requirement fr Cinryze and Berinert (frm 13 years t 12 years) 1/1/16: drug cde updated fr Rucnest 7/19/16: n plicy changes 5/1/17: step therapy criteria added 6/21/17: updated age limitatins and Berinert use 1/1/18: Haegarda added; updated age requirements 6/15/18: n plicy changes 11/1/18: Takhzyr added; updated age requirements 1/3/19: updated billing/cding infrmatin IX. References 1. Berinert [C-1 esterase inhibitr (human)] prescribing infrmatin, CSL Behring LLC, Kankakee IL 60901, Revised 9/ Cinryze [C-1 esterase inhibitr (human)] prescribing infrmatin, VirPharma Incrprated, Extn PA 19341, Revised 12/ Kalbitr (ecallantide), prescribing infrmatin, Dyax Crp., Cambridge MA 02139, Revised 3/ Firazyr (icatibant), prescribing infrmatin, Shire Orphan Therapies Inc., Lexingtn MA 02421, Revised 12/ Rucnest [C1 esterase inhibitr [recmbinant]), prescribing infrmatin, Santarus Inc. Raleigh, NC Revised 12/ UpTDate, retrieved May 2015 Page 5 f 6

6 7. Clinical Pharmaclgy, Accessed 5/ Facts and Cmparisns On-line, retrieved May Zuraw B, Lumry W, Hurewitz D, et al. Results f pen-label administratin f nanfiltered C1-inhibitr fr the treatment f acute HAE attacks. Abstract presented at the American Cllege f Allergy, Asthma, and Immunlgy Annual Scientific Meeting. Seattle, WA; 2008 Nv Abstract Zuraw B, Schaefer O, Grant JA, et al. Results f a randmized duble-blind placeb cntrlled study f nanfiltered C1-inhibitr fr the treatment f HAE attacks. Abstract presented at the American Cllege f Allergy, Asthma, and Immunlgy Annual Scientific Meeting. Dallas, TX; 2007 Nv Abstract Bwen T, Cicardi M, Farkas H, et al Internatinal cnsensus algrithm fr the diagnsis, therapy and management f hereditary angiedema. Allergy Asthma Clin Immunl. 2010;6(1): Farkas H, Gyeney L, Gidófalvy E, Füst G, Varga L. The efficacy f shrt-term danazl prphylaxis in hereditary angiedema patients underging maxillfacial and dental prcedures. J Oral Maxillfac Surg. 1999;57(4): Craig T, Pursun EA, Brk K, et. al. WAO Guideline fr the Management f Hereditary Angiedema. WAO Jurnal 2012; 5: Dunkle, M. (2011). Natinal Organizatin fr Rare Disrders (NORD). Encyclpedia f Clinical Neurpsychlgy, Haegarda (C-1 esterase inhibitr[human]) prescribing infrmatin. CSL Behring LLC, Kankakee IL. Revised 07/ Adlescent Health. Wrld Health Organizatin. WHO Web. Accessed 12/21/ Centers fr Medicare and Medicaid Services. Medicare Benefit Plicy Manual: Chapter 15. (CMS Publicatin N ). Retrieved frm Takhzyr TM (lanadelumab-fly) prescribing infrmatin. Dyax Crp, Lexingtn, MA. Revised *These guidelines are nt applicable t benefits cvered under Medicare Advantage. Medicare Advantage benefit cverage requests are reviewed in accrdance with the guidance set frth in Chapter 15 Sectin 50 f the Centers fr Medicare & Medicaid Services Medicare Benefit Plicy Manual. The Plan fully expects that nly apprpriate and medically necessary services will be rendered. The Plan reserves the right t cnduct pre-payment and pst-payment reviews t assess the medical apprpriateness f the abve-referenced therapies. The preceding plicy applies nly t members fr whm the abve named pharmacy benefit medicatins are included n their cvered frmulary. Members with clsed frmulary benefits are subject t trying all apprpriate frmulary alternatives befre a cverage exceptin fr a nn-frmulary medicatin will be cnsidered. The preceding plicy is a guideline t allw fr cverage f the pertinent medicatin/prduct, and is nt meant t serve as a clinical practice guideline. Page 6 f 6