NICE recommends Ravulizumab for the treatment of atypical haemolytic uraemic syndrome
The National Renal Complement Therapeutics centre is pleased to hear today’s announcement that NICE has recommended ravulizumab (Ultomiris®) for funding for the treatment of atypical haemolytic uraemic syndrome.
Ravulizumab is a long-acting complement C5 inhibitor that acts in the same way as eculizumab. Whereas eculizumab infusions are required every 2 weeks, ravulizumab infusions are only required every 8 weeks.
In the light of this announcement, we will be contacting patients and their local clinicians to discuss whether switching to ravulizumab is the best choice for them. Initiation of this process is currently envisaged to begin in early autumn 2021.
Important information about COVID-19
Guidance from the National Renal Complement Therapeutics Centre regarding vaccination against Covid-19 for aHUS patients
Organisations across kidney medicine have welcomed the arrival of effective Covid-19 vaccines.
Whilst the Covid-19 vaccine trials were not performed on patients with aHUS specifically, given previous experience with other vaccinations, there is no evidence to suggest that any of the available vaccinations for Covid-19 would not be safe and protective for our patients.
Any small risk associated with the vaccination is likely to be significantly outweighed by the potential benefits against Covid-19
- We would recommend that all aHUS patients including those with a kidney transplant on Eculizumab are vaccinated against Covid-19
- If you have been told that you are at risk of developing aHUS (due to a complement mutation) we would recommend that you are vaccinated against Covid-19 *
* In this situation, there is a theoretical risk of a vaccine precipitating aHUS, and if you develop symptoms lasting more than a few days after the vaccination, blood tests to include: U&E, FBC and LDH should be performed (this is the same information on the “at risk” cards we produce). We believe that the benefits of a vaccine outweigh any theoretical risk. If any of these results are abnormal, patients should refer to the National aHUS Service website for advice (www.atypicalhus.co.uk or telephone 0191 282 0385).
Children/young people under 16 years
The Covid-19 vaccine trials have not yet studied the vaccine in children under 16 years old and therefore the vaccine is not currently licensed for patients under the age of 16 years. Because of this, we cannot recommend that children with aHUS or receiving Eculizumab treatment receive a Covid-19 vaccination at this time. Reassuringly, serious complications of Covid-19 infection are very rare in children, even in those with underlying health conditions or those receiving treatments that affect the immune system such as Eculizumab.
The advice given in this document is general guidance for those with aHUS. We cannot advise regarding any other health conditions patients might have. For this, you would need to seek specialist advice where appropriate, or discuss with the healthcare professional giving the vaccine.
The MHRA has provided guidance on the small number of individuals who should not receive this vaccination: https://www.gov.uk/government/news/second-covid-19-vaccine-authorised-by-medicines-regulator
- Government identified priorities for vaccination(https://www.gov.uk/coronavirus)
- Kidney Care UK – Coronavirus (Covid-19) guidance for patients with kidney disease https://www.kidneycareuk.org/news-and-campaigns/coronavirus-advice/#research
- Renal Association – Frequently asked questions letter https://renal.org/sites/renal.org/files/Patient%E2%80%99s%20Frequently%20Asked%20Questions%20letter%20on%20COVID-19%20Vaccines%20-%20December%202020.pdf
- COVID-19 vaccination for adult patients with kidney disease: a position statement from the UK renal community – 4 December 2020
- MHRA, Information for Healthcare Professionals on Pfizer/BioNTech COVID-19 vaccine (updated 8 December 2020)
- Briefing document on COVID-19 vaccination in the renal community – 24th December 2020
Atypical Haemolytic Uraemic Syndrome (aHUS) & Eculizumab Specialists
NRCTC specialises in Atypical Haemolytic Uraemic Syndrome (aHUS), Eculizumab, C3 Glomerulopathy (C3G) and MPGN.
Members of our team have been instrumental in research into pathogenic mechanisms of aHUS that has revealed the important role of complement.
Approximately 50% of patients with aHUS have an underlying complement abnormality. Eculizumab is a complement inhibitor that was recommended by NICE in the treatment of aHUS in England.
Eculizumab is currently funded by NHS England through the national aHUS service based within the National Renal Complement Therapeutics Service at the Newcastle upon Tyne Hospitals NHS Foundation Trust.
COVID-19 and aHUS/Eculizumab
Patients with aHUS receiving eculizumab should continue to receive their eculizumab during the current COVID-19 pandemic
As of 22/03/2020 the UK government has updated its advice for extremely vulnerable patients (available here).
- For aHUS patients on Eculizumab with a renal transplant the government advice is clear – implement shielding i.e. staying at home and avoiding any face-to-face contact for a period of at least 12 weeks.
- For aHUS patients on Eculizumab (not transplanted), shielding may be appropriate depending on your general health, age (>70yrs) and stage of kidney function (CKD stages 3, 4 or 5) which is best assessed by your primary nephrologist and we are happy to assist them in this decision (for further information click here.)
As a minimum all aHUS patients on Eculizumab should be particularly stringent in following social distancing measures (please click here for the government link).
Patients on eculizumab remain at risk of meningococcal sepsis and should continue on their prophylactic antibiotics and seek medical advice (presenting with your meningococcal risk card) if they believe they have symptoms (summarised here) suggestive of meningococcal sepsis
Patients in the Stopping Eculizumab Treatment Safely (SETSaHUS) should continue with standard monitoring as described in the SETSaHUS information pack
If individuals have previously been told by a medical professional that they are at risk of developing aHUS, standard advice about seeking medical advice with their “aHUS at-risk” card applies if they are unwell and think that they have symptoms of aHUS
There is currently no evidence suggesting eculizumab worsens COVID19 however this is a rapidly evolving field. Many patients on Eculizumab will have co-existent chronic kidney disease, renal transplants and other health conditions necessitating the above measures.
Summer is the high season for infections by bacteria of which some strains can cause haemolytic uremic syndrome. To inform patients about this type of HUS, particularly those who were referred to NRCTC to investigate for atypical HUS and were found to have STEC causing their symptoms, we have created a short video to summarise the cause of STEC HUS, its symptom and prognosis.