Document ID: chunk:federal_register_of_legislation:F2025C00124:clause:3_1:p86
Version: federal_register_of_legislation:F2025C00124
Segment Type: clause
Provision Reference: sch 3 cl 1 (pt 86/476)
Character Range: 1329679–1336484

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.
                                                                                                                                            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 are present at the time of the initial organ impairment.
                                                                                                                                            Serial haematological results (every 3 months while the patient is receiving treatment) must be provided with every subsequent application for treatment.
                                                                                                                                            The authority application must be in writing and must include all of the following:
                                                                                                                                            (1) A completed authority prescription form(s);
                                                                                                                                            (2) A completed authority application form relevant to the indication and treatment phase (the latest version is located on the website specified in the Administrative Advice);
                                                                                                                                            (3) A detailed cover letter from the prescriber;
                                                                                                                                            (4) A measurement of body weight at the time of application;
                                                                                                                                            (5) The result of ADAMTS-13 activity on a blood sample taken prior to plasma exchange or infusion; the date and time that the sample for the ADAMTS-13 assay was collected, and the dates and times of any plasma exchanges or infusions that were undertaken in the 2 weeks prior to collection of the ADAMTS-13 assay;
                                                                                                                                            (6) In the case that a sample for ADAMTS-13 assay was not collected prior to plasma exchange or infusion, measurement of ADAMTS-13 activity