Treating aHUS

Plasma Exchange/Infusion

  • Your physician may carry out a procedure known as plasma exchange. The purpose of this is to remove plasma from your body that contains the faulty proteins and replace it with fresh plasma containing non-faulty proteins.

  • This procedure normally lasts a few hours and you will need a line to be inserted into one of your larger blood vessels for it to be carried out.

 

Dialysis

  • aHUS can cause severe damage to the kidneys and dialysis may be required to do the job of the kidneys.

  • Like plasma exchange, this will also take a few hours and will also require a line to be inserted into your blood vessel.

  • It is not unusual to need more than one session of dialysis and in a small number of cases this may continue for a few weeks or months.

  • In some instances, the damage to the kidneys does not get better and dialysis may be required in the longer term.

 

Eculizumab

  • Eculizumab (Soliris©) is the only medication currently licensed for use in aHUS.

  • It works by blocking the action of complement and therefore preventing it from damaging the body’s own cells.

  • Eculizumab is given as an infusion that usually lasts around 45 minutes, longer if the infusion is for a child.

  • After the first doses have been given, the regular long-term dose is every two weeks.

  • Usually Eculizumab is given for the remainder of your life but in the near future there will be a clinical trial which will assess when it is safe to stop Eculizumab infusions and what monitoring should be done when the infusions stop.

  • If you are interested in finding out about whether it would be safe for you to stop receiving Eculizumab infusions then you should speak to your local physician.

 

YOU SHOULD NOT STOP RECEIVING YOUR ECULIZUMAB INFUSIONS WITHOUT FIRST OF ALL DISCUSSING THIS WITH YOUR DOCTOR.

 

Long-term monitoring on Eculizumab

  • When you are receiving Eculizumab treatment your physician will carry out some regular tests to ensure that the drug is working and that there is no evidence of the disease returning. 

  • These will include:-

    • Full blood countto look at the level of platelets and Haemoglobin in your blood

    • Urea & Electrolytes – to assess your kidney function

    • LDH – this should return to normal levels once the disease is no longer active or has been suppressed by Eculizumab

    • Urine Protein-Creatinine Ratio (PCR) – measures the amount of protein being excreted in urine which can be an indicator of renal damage

    • Complement studies – to ensure that Eculizumab is suppressing your complement activity.

 

Transplantation

  • Patients with end-stage kidney disease resulting from aHUS will usually be assessed for their suitability for a kidney transplant.

  • Unfortunately aHUS can occur in a transplanted kidney as the faulty complement proteins are still produced by the liver.

  • Eculizumab can be given to prevent the recurrence of aHUS in the transplanted kidney.