Pathway for prescribing PCSK9 Inhibitors; Alirocumab (Praluent, Sanofi) ▼and Evolocumab (Repatha, Amgen) ▼

PRESCRIBING CRITERIA PRIMARY PREVENTION: If LDL cholesterol persistently > 5 mmol/L in Heterozygous Familial Hypercholestereolaemia SECONDARY PREVENTION:  If LDL cholesterol persistently > 4 mmol/L in Non-familial hypercholesterolaemia or mixed dyslipidaemia, with high risk of cardiovascular disease (acute coronary syndrome e.g. myocardial infarction or unstable angina requiring hospitalisation, coronary or other arterial revascularisation procedures, coronary heart disease, ischaemic stroke, peripheral arterial disease)  If LDL cholesterol persistently > 3.5 mmol/L in;  Heterozygous Familial Hypercholestereolaemia with CVD  Non-familial hypercholesterolaemia or mixed dyslipidaemia with very high risk of cardiovascular disease (recurrent cardiovascular events or polyvascular disease)

Patient newly referred to Secondary Care or Team or already on active Caseload Patient meets Alirocumab / Evolocumab prescribing criteria

Prescribing initiated and retained within Patient to phone the relevant Consultant if side effects Discuss treatment options Secondary Care by Consultant Chemical develop. with patient, including Pathologist (Lipid Clinic) continuation and stopping /Cardiologist/Endocrinologist Follow up in Outpatients at 3 months and six months after initiating treatment. Annually thereafter. Patient to have criteria. Ensure that the Either Alirocumbab 75 mg subcutaneous fasting blood tests one week prior to their appointments. patient understands the every 2 weeks or Evolocumab 140 mg Patients to be monitored for efficacy, adherence to treatment, importance of adherence subcutaneous every 2 weeks. and adverse effects and that treatment will be Respective Patient Support Programme stopped if the required LDL- Enrolment form signed by the Consultant If Alirocumab 75 mg was started and LDL cholesterol is > 2 C concentration is not and patient encouraged to engage to aid mmol/L, dose to be increased to 150 mg every 2 weeks. achieved adherence If treatment with one of the PCSK9 needs to be stopped, the Patient supplied prescriptions via other PCSK9 can be prescribed. Baseline renal and liver Homecare Company function tests The Company Nurse will communicate to the relevant Patient is trained to self-administer by Consultant any concern regarding the patient including side Company nursing support who visits effects and non-adherence to treatment. patient at home

CONTRAINDICATIONS STOPPING CRITERIA Pregnancy, breastfeeding, <18 years old. -If planning pregnancy Hypersensitivity to the active substance or to any of the excipients as stated on corresponding - If side effects develop e.g. local injection site SPCs reactions, allergy, upper respiratory tract symptoms, nausea, arthralgia, back pain, etc. Should be used with caution in patients with severe hepatic impairment (Child-Pugh C) and severe -If < 30% reduction in pre-treatment LDL- renal impairment (eGFR < 30 mL/min) cholesterol is not achieved at 3 months Alirocumab and Evolocumab have been licensed and approved by NICE (TA 393 and 394) to treat patients with primary hypercholesterolaemia or mixed dyslipidaemia whose Low-density lipoprotein (LDL) cholesterol concentrations are persistently above the thresholds specified under “Prescribing criteria “, despite maximal tolerated lipid-lowering therapy ie maximum doses have Alirocumab and Evolocumab are black triangle been reached including the use of combination therapy in line with the following NICE publications, ▼medicines . All side effects must be reported as appropriate for the patient’s diagnosis or further titration or initiation is limited by intolerance as via the yellow card system (submitted to MHRA, defined by NICE CG71 and the relevant SPC. record in patient’s notes, complete Datix)

 Familial hypercholesterolaemia: identification and management (CG71)  Ezetimibe for treating primary heterozygous-familial and non-familial hypercholesterolaemia (TA385) The use of Evolocumab for the treatment of  Cardiovascular disease: risk assessment and reduction, including lipid modification (CG181) homozygous familial hypercholesterolaemia is an  Cardiovascular risk assessment and lipid modification (QS100) NHSE commissioning responsibility.  Familial hypercholesterolaemia (QS41)

Produced by West Essex CCG Medicines Optimisation Team; Approved September 2016;Formatting updated July 2019; Review Date July 2021