Document ID: chunk:federal_register_of_legislation:F2025C00124:clause:3_1:p344
Version: federal_register_of_legislation:F2025C00124
Segment Type: clause
Provision Reference: sch 3 cl 1 (pt 344/476)
Character Range: 3060568–3070597

regression in ability to perform age‑appropriate motor milestones; or
                                                                                                                                            iii) Proximal weakness; or
                                                                                                                                            iv) Hypotonia; or
                                                                                                                                            v) Absence of deep tendon reflexes; or
                                                                                                                                            vi) Failure to gain weight appropriate for age; or
                                                                                                                                            vii) Any active chronic neurogenic changes; or
                                                                                                                                            viii) A compound muscle action potential below normative values for an age‑matched child.
                                                                                                                                            Defined signs and symptoms of type II SMA are:
                                                                                                                                            i) Onset between 6 and 18 months; and
                                                                                                                                            ii) Failure to meet or regression in ability to perform age‑appropriate motor milestones; or
                                                                                                                                            iii) Proximal weakness; or
                                                                                                                                            iv) Weakness in trunk righting/derotation; or
                                                                                                                                            v) Hypotonia; or
                                                                                                                                            vi) Absence of deep tendon reflexes; or
                                                                                                                                            vii) Failure to gain weight appropriate for age; or
                                                                                                                                            viii) Any active chronic neurogenic changes; or
                                                                                                                                            ix) A compound muscle action potential below normative values for an age‑matched child.
                                                                                                                                            Defined signs and symptoms of type IIIa SMA are:
                                                                                                                                            i) Onset between 18 months and 3 years of age; and
                                                                                                                                            ii) Failure to meet or regression in ability to perform age‑appropriate motor milestones; or
                                                                                                                                            iii) Proximal weakness; or
                                                                                                                                            iv) Hypotonia; or
                                                                                                                                            v) Absence of deep tendon reflexes; or
                                                                                                                                            vi) Failure to gain weight appropriate for age; or
                                                                                                                                            vii) Any active chronic neurogenic changes; or
                                                                                                                                            viii) A compound muscle action potential below normative values for an age‑matched child.
                                                                                                                                            Invasive permanent assisted ventilation means ventilation via tracheostomy tube for greater than or equal to 16 hours per day.
                                                                                                                                            Application for authorisation of initial treatment must be in writing and must include:
                                                                                                                                            (a) a completed authority prescription form; and
                                                                                                                                            (b) a completed Spinal muscular atrophy PBS Authority Application Form which includes the following:
                                                                                                                                            i) specification of SMA type (I, II or IIIa); and
                                                                                                                                            (ii) sign(s) and symptom(s) that the patient has experienced; and
                                                                                                                                            (iii) patient's age at the onset of sign(s) and symptom(s).
                                                                                                                                            The approved Product Information recommended dosing is as follows:
                                                                                                                                            (i) 16 days to less than 2 months of age: 0.15 mg/kg
                                                                                                                                            (ii) 2 months to less than 2 years of age: 0.20 mg/kg
                                                                                                                                            (iii) 2 years of age and older weighing less than 20 kg: 0.25 mg/kg
                                                                                                                                            (iv) 2 years of age and older weighing 20 kg or more: 5 mg
                                                                                                                                            In this authority application, state which of (i) to (iv) above applies to the patient. Based on (i) to (iv), prescribe up to:
                                                                                                                                            1 unit where (i) applies;
                                                                                                                                            2 units where (ii) applies;
                                                                                                                                            3 units where (iii) applies;
                                                                                                                                            3 units where (iv) applies.
                                                                       C14392                                                               Symptomatic type IIIB/IIIC spinal muscular atrophy (SMA)                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                              Compliance with Authority Required procedures
                                                                                                                                            Continuing/maintenance treatment in a child or adult, but where treatment was initiated during childhood
                                                                                                                                            Patient must be undergoing continuation of existing PBS‑subsidised treatment with this drug; OR
                                                                                                                                            Patient must be undergoing a change in prescribed SMA drug to this drug ‑