Document ID: chunk:federal_register_of_legislation:F2025C00124:clause:3_1:p331
Version: federal_register_of_legislation:F2025C00124
Segment Type: clause
Provision Reference: sch 3 cl 1 (pt 331/476)
Character Range: 2969577–2977188

response assessment is not undertaken and submitted within these timeframes, the patient will be deemed to have failed to respond to treatment with ravulizumab.
                                                                       C14780                                                               Atypical haemolytic uraemic syndrome (aHUS)                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                           Compliance with Written Authority Required procedures
                                                                                                                                            Initial treatment - Initial (new patient) loading dose
                                                                                                                                            Patient must have active and progressing thrombotic microangiopathy (TMA) caused by aHUS; AND
                                                                                                                                            Patient must have ADAMTS-13 activity of greater than or equal to 10% on a blood sample taken prior to plasma exchange or infusion; or, if ADAMTS-13 activity was not collected prior to plasma exchange or infusion, patient must have platelet counts of greater than 30x10^9/L and a serum creatinine of greater than 150 mol/L; AND
                                                                                                                                            Patient must have a confirmed negative STEC (Shiga toxin-producing E.Coli) result if the patient has had diarrhoea in the preceding 14 days; AND
                                                                                                                                            Patient must have clinical features of active organ damage or impairment; AND
                                                                                                                                            Patient must not receive more than 2 weeks of treatment under this restriction.
                                                                                                                                            Must be treated by a prescriber who is either: (i) a haematologist, (ii) a nephrologist; OR
                                                                                                                                            Must be treated by a medical practitioner who has consulted at least one of the above mentioned specialist types, with agreement reached that the patient should be treated with this pharmaceutical benefit on this occasion; AND
                                                                                                                                            Patient must be undergoing treatment with one C5 inhibitor therapy only at any given time.
                                                                                                                                            This drug is not PBS-subsidised if it is prescribed to an in-patient in a public hospital setting.
                                                                                                                                            Evidence of active and progressing TMA is defined by the following:
                                                                                                                                            (1) A platelet count of less than 150x10^9/L; and evidence of at least two of the following:
                                                                                                                                            (i) presence of schistocytes on blood film;
                                                                                                                                            (ii) low or absent haptoglobin;
                                                                                                                                            (iii) lactate dehydrogenase (LDH) above normal range; or
                                                                                                                                            (2) In recipients of a kidney transplant for end-stage kidney disease due to aHUS, a kidney biopsy confirming TMA; and
                                                                                                                                            (3) Evidence of at least one of the following clinical features of active TMA-related organ damage or impairment is defined as below:
                                                                                                                                            (a) kidney impairment as demonstrated by one or more of the following:
                                                                                                                                            (i) a decline in estimated Glomerular Filtration Rate (eGFR) of greater than 20% in a patient who has pre-existing kidney impairment;
                                                                                                                                            (ii) a serum creatinine (sCr) of greater than the upper limit of normal (ULN) in a patient who has no history of pre-existing kidney impairment;
                                                                                                                                            (iii) a sCr of greater than the age-appropriate ULN in paediatric patients;
                                                                                                                                            (iv) a renal biopsy consistent with aHUS;
                                                                                                                                            (b) onset of TMA-related neurological impairment;
                                                                                                                                            (c) onset of TMA-related cardiac impairment;
                                                                                                                                            (d) onset of TMA-related gastrointestinal impairment;
                                                                                                                                            (e) onset of TMA-related pulmonary impairment.
                                                                                                                                            Claims of non-renal TMA-related organ damage should be made at the point of application for