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.

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COVID-19 guidance for children and young people with Atypical Haemolytic Uraemic Syndrome (aHUS) returning to nursery/school/college

Updated: August 2020

Below is some information relevant for children and young people returning to education in September.  As we continue to learn more about the COVID-19 virus, guidance may change over time.

In the UK, COVID-19 infection levels have significantly reduced and general lockdown measures are being relaxed. On 1st August 2020, shielding for those at high risk of severe infection stopped. Testing for COVID-19 now means outbreaks can be identified and managed quickly.  The majority of children who were previously shielding are advised to go back to school in September. The Government has issued advice for schools about measures to help reduce the spread of COVID-19.

Since previous guidance, kidney units have been collecting information on COVID-19 cases.  Reassuringly, very few UK children with kidney disease have been admitted to hospital with COVID-19.  Findings are that:

·         Most infected children have been mildly affected. There is no evidence that medicines which affect the immune system increase the risk of catching COVID-19. There is also no proof they cause a more severe infection. 

·         There have been very few children with kidney conditions on medicines affecting their immune system who have had COVID-19. Since the pandemic started Children’s Kidney doctors across the world have reported only about 100 cases. The majority were mild.

·         Other studies have not shown people on immunosuppression to be at higher risk than the general population.

·         There is much less COVID-19 infection now in the UK. Evidence is increasing that few children are severely affected by COVID-19, even those with kidney conditions.

The evidence now shows that children and young people who were previously categorised as being in an “intermediate risk” (clinically vulnerable) group are not at higher risk than other children without a kidney condition

Advice regarding returning to nursery/school/college

The government advice is that all children should attend nursery or school, not only for their educational progress and wellbeing, but for their wider development.  They say it will be mandatory for children and young people of school age to attend school from the start of the school year in September (unless there is a valid reason for not attending).

We are keen to support the safe return of children to schools. We understand many families may feel anxious about this, particularly for children who were shielding. 

Siblings should go to school or college as long as the environment has been made COVID-19 secure in line with government guidance. 

 

What will happen if there is a case of COVID-19 at nursery/school/college?

• Do not panic. Children sent home from school because of coughing or fever are just as likely to have other common respiratory viruses. A case needs to be confirmed as COVID-19 by testing, which may take a few days.

• Government advice for what schools, parents and students need to do, is online at: “What parents and carers need to know about early years providers, schools and colleges in the autumn term  

• Any student may be asked to self-isolate for 14 days by their school or college (based on advice from their local health protection teams) if they have been in close, face-to-face contact with someone who has tested positive for the virus. An outbreak is classified as 2 or more confirmed cases within 14 days, following which the local health protection team will be sent in to advise.

 

Children and young people who are on Eculizumab

Children and young people with a diagnosis of aHUS or other kidney complement disorder on Eculizumab are not considered as “higher risk” patients in relation to COVID-19.

If your child develops a fever, please remember that they are still at risk of meningococcal disease and that this will need assessing urgently by a clinician, as well as your child having a COVID-19 test.

 

Children and young people deemed “at risk” of aHUS

If you have been told that your child is at risk of aHUS (for example they are known to have a complement gene mutation such as a “Factor H” or “C3” or “CD46” mutation) but they are well, and not on treatment with Eculizumab, it is possible that COVID-19 infection could trigger an episode of aHUS just like any other viral infection. If they become unwell and do not recover quickly, or they seem more unwell than with a typical viral infection, you should contact your GP, paediatrician or paediatric nephrologist, making them aware of the aHUS alert card, as it is possible that your child may need blood tests to look for aHUS.

Who should still be regarded as higher risk?

Clinically extremely vulnerable children and young people (previously in the shielded group) include those:

·         With recent kidney transplants – first three months immediately after transplant

·         On high level of immunosuppressive medication for active disease undergoing induction treatment: those who are currently receiving or completed treatment within 6 weeks of: 

o   High dose steroids of 20 mg/day or above (or 30 mg/m2/day) AND cyclophosphamide or rituximab or other very powerful immunosuppression

Things you can do to reduce general risk:

·         It is important that your child has vaccinations that are offered, which gives some protection against other illnesses

·         Parents/carers and young people at work should discuss with employers about putting suitable arrangements in place to minimise risk.

Families should still be cautious and reduce their own risk of COVID-19 by regular hand washing, good social distancing and avoiding touching your face.

Where can I get government advice?

The four different UK nations have slightly different COVID-19 healthcare and schooling advice. Advice for clinically extremely vulnerable patients (previously shielded group) is available here:

England 

https://www.gov.uk/government/publications/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19/guidance-on-shielding-and-protecting-extremely-vulnerable-persons-from-covid-19

Scotland

https://www.gov.scot/publications/covid-shielding/

 

Wales

https://gov.wales/guidance-shielding-and-protecting-people-defined-medical-grounds-extremely-vulnerable-coronavirus-0

Northern Ireland

https://www.nidirect.gov.uk/articles/coronavirus-covid-19-pausing-shielding-extremely-vulnerable-people

 

Who to contact if you have further queries or concerns

If you would like to discuss anything in relation to this guidance, please contact the aHUS Specialist Nurses on telephone 0191 2825098 or email on ahus.nurses@nhs.net

References

1.       British Association for Paediatric Nephrology and The Renal Association (14 August 2020). Updated COVID-19 guidance for children with kidney disease on dialysis, and immunosuppression (including kidney transplants)

2.      GOV.UK “What parents and carers need to know about early years providers, schools and colleges in the Autumn term” (updated 21 Aug 2020). https://www.gov.uk/government/publications/what-parents-and-carers-need-to-know-about-early-years-providers-schools-and-colleges-during-the-coronavirus-covid-19-outbreak/what-parents-and-carers-need-to-know-about-early-years-providers-schools-and-colleges-in-the-autumn-term

 

STEC-HUS

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.