Document ID: chunk:federal_register_of_legislation:F2023L01745:schedule:1:p3
Version: federal_register_of_legislation:F2023L01745
Segment Type: schedule
Provision Reference: sch 1 (pt 3/42)
Character Range: 8988–13061

dose  0
             C14476 C14530 C14531 C14746 C14747 C14748 C14749         1 dose  2

    [15]       Schedule 2, entry for Ustekinumab
         insert in numerical order in the column headed "Circumstances": C14758 C14787 C14801
    [16]       Schedule 3, entry for Eculizumab
       (a)           omit:
   C6626     Atypical haemolytic uraemic syndrome (aHUS)                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                  Compliance with Written Authority Required procedures
             Initial treatment
             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 4 weeks of treatment under this restriction.
             Must be treated by a paediatric nephrologist, a nephrologist, a paediatric haematologist or a haematologist, or, must be in consultation with a paediatric nephrologist, a nephrologist, a paediatric haematologist or a haematologist.
             Evidence of active and progressing TMA is defined by the following:
             (1) a platelet count of less than 150x10^9/L; and evidence of 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 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; and/or
             (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; or
             (iii) a sCr of greater than the age‑appropriate ULN in paediatric patients; or
             (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 initial PBS‑subsidised eculizumab (where possible), and should be supported by objective clinical measures. The prescriber's cover letter should establish that the observed organ damage is directly linked to active and progressing TMA, particularly when indirect causes such as severe thrombocytopenia, hypertension and acute renal failure