Paediatric multisystem inflammatory syndrome: what nurses need to know
Be alert for unusual symptoms in children as a small number of cases are identified during the pandemic
- Rare condition may be linked to coronavirus infection
- Wide range of symptoms, but some children with PIMS have symptoms common to Kawasaki disease and toxic shock syndrome
- Children with African or Caribbean heritage may be more susceptible
Most children are asymptomatic or exhibit mild symptoms from COVID-19 infection. But a small number of cases have been identified who seem to develop a significant systemic inflammatory response.
In late April, in a letter from NHS England, GPs were warned to look out for a rare but dangerous reaction in children that may be linked to the coronavirus infection. This alert said intensive care departments in London and other parts of the UK had been treating severely sick children with unusual symptoms.
Some, but not all, tested positive for COVID-19.
Paediatricians have stressed that only a relatively small number of cases have been identified, the causes are not clear, and that the risk to children is low.
What symptoms are children presenting with?
Number of children reported to have experienced the syndrome by mid-June
The Royal College of Paediatrics and Child Health (RCPCH) has published a case definition for paediatric multisystem inflammatory syndrome (PIMS) as a child presenting with persistent fever, inflammation (neutrophilia, elevated CRP and lymphopaenia) and evidence of single or multi-organ dysfunction, such as shock, cardiac, respiratory, renal, gastrointestinal or neurological disorder, with additional features.
This may include children fulfilling full or partial criteria for mucocutaneous lymph node syndrome, also known as Kawasaki disease.
Healthcare professionals should exclude any other microbial cause, although waiting for results of these investigations should not delay seeking expert advice.
Testing for COVID-19 may be positive or negative.
How many children have been affected by paediatric inflammatory multisystem syndrome?
In early May, around 20 children in the UK and a small number across Europe were thought to have had PIMS, according to the RCPCH.
Possible cases have also been seen in the United States and other countries. It is not known why some countries have not reported cases, but it may be because of a lack of recognition of the syndrome, because doctors did not write up a case description, or because the condition is extremely rare, paediatricians say.
By mid-May it was being reported that about 100 children in the UK had experienced the syndrome. By mid-June the RCPCH gave the figure as 200.
The expert group that developed the RCPCH definition stresses that it has affected an extremely small number of children in the UK and throughout the world.
An observational study of 21 children admitted to a teaching hospital in Paris, the Necker-Enfants Malades hospital, with features of a Kawasaki-like multisystem inflammatory syndrome syndrome found that 12 (57%) were of African or Caribbean heritage. Six (29%) were white and 3 (14%) were Asian.
The authors of the study, published in the BMJ, said: ‘In this study an unusually high proportion of the affected children and adolescents had gastrointestinal symptoms, Kawasaki disease shock syndrome, and were of African ancestry.’
What is the outcome of the syndrome?
Length of stay in intensive care for affected children is generally short, at three to four days, and the great majority of patients survive, according to the Paediatric Intensive Care Society (PICS).
But at least one child in the UK, a 14 year old with no underlying health conditions, is known to have died, according to an article in the Lancet.
What is Kawasaki disease?
Kawasaki disease is a condition that mainly affects children under the age of five. The characteristic symptoms are a high temperature that lasts for five days or more, with a rash, swollen glands in the neck, dry, cracked lips, red fingers or toes, and red eyes.
The cause of the condition, which can lead to serious damage to coronary arteries, is not understood. The symptoms are similar to those of an infection but, so far, no bacterial or viral cause has been identified. It is not contagious, the NHS says.
There may be a genetic element that puts some children at risk of developing it.
What is the evidence of PIMS links with COVID-19?
Many children with the syndrome had symptoms common to Kawasaki disease and toxic shock syndrome, a rare life-threatening immune reaction to certain infections. Around half the children affected in London also tested positive for COVID-19.
The timing of these clusters of cases during a pandemic means that it has been suggested the syndrome is linked to COVID-19. The World Health Organization says ‘initial hypotheses’ are that this syndrome may be related to COVID-19 based on initial laboratory testing showing positive serology in a majority of patients.
But clinicians are clear that it is still not known if COVID-19 is connected.
What does the RCPCH say?
Early recognition of the syndrome by paediatricians and specialist referral, including to critical care, is essential, according to the college.
All stable children should be discussed as soon as possible with specialist services to ensure prompt treatment as deterioration can be rapid. There should be a low threshold for referral to paediatric intensive care using normal pathways.
Affected children may require paediatric intensive care and input from paediatric infectious diseases, cardiology, and rheumatology.
Paediatrician and RCPCH registrar Mike Linney says: ‘There is no one sign or symptom which alone would indicate that a child has the condition. Clinicians can use the definition to look for a pattern of combined symptoms.
‘All of these children were extremely unwell, with features suggestive of sepsis, such as a persistently high temperature coupled with rapid breathing, cold hands and feet and sleepiness. The other symptoms varied greatly in the different cases.’
What does the RCN say?
RCN professional lead for children and young people Fiona Smith says more research is needed to understand the syndrome. ‘The new syndrome thankfully appears to be extremely rare so far.'
'We urgently need more research about the condition, and in particular the strength of its association with COVID-19, whose antibodies have been found in the bloodstream of many of the children presenting with the syndrome.
‘Surveillance studies are taking place which should increase our understanding of the condition. In the meantime, staff who suspect the presence of the syndrome in children should discuss with their multidisciplinary team, including the senior nurse, any concerns they have so that supportive and reporting measures can be instigated.’
What should children’s nurses be doing?
Carli Whittaker, PICS vice president, sister at the paediatric critical care unit at Nottingham Children’s Hospital and director of clinical skills at the University of Nottingham, says nurses should keep themselves informed of the latest information on the syndrome.
‘Children’s nurses should keep up to date with the advice that has been circulated by the RCPCH and PICS, and be familiar with the RCPCH case definition,’ she says.
‘These children require close observations and hourly paediatric early warning scores until they have been stable for at least 12 hours. There is ongoing collaboration to share knowledge and provide guidance.’
What clinical advice is there?
The RCPCH and PICS have published management guidance based on the experience of clinicians so far. It sets out tests that should be undertaken and recommends monitoring and treatment approaches.
PICS says the essential basis for management of the condition remains high quality general paediatric intensive care.
A multidisciplinary team approach is encouraged to ensure treatment decisions are coordinated. This should engage colleagues from cardiology, infectious diseases, and rheumatology/immunology, depending on local circumstances.
What should concerned parents do?
Advice for parents concerned about the health of their child has not changed, the NHS says.
NHS national clinical director for children and young people Simon Kenny says: ‘To be clear to any worried parents out there, this remains rare, and the advice remains the same as ever: if you are worried about your child for whatever reason, contact NHS 111 or your family doctor for urgent advice, or 999 in an emergency, and if a professional tells you to go to hospital, please do so immediately.’
The RCPCH has produced guidance for families on PIMS It states there is a wide range of symptoms (such as a rash or abdominal symptoms) and children with PIMS can be affected very differently. It stresses that the most important thing is that any child who is seriously unwell needs to be treated quickly – whatever the illness.
What is being done about the syndrome?
Studies are now under way in the UK to gain a better understanding of the syndrome, who it affects and what causes it.
A British Paediatric Surveillance Unit study, funded by Public Health England, will ask paediatricians to report all cases that have features of the inflammation syndrome, and to report all cases of Kawasaki disease and toxic shock syndrome.
- RCPCH: Guidance: Paediatric multisystem inflammatory syndrome temporally associated with COVID-19
- Paediatric Intensive Care Society: Paediatric Inflammatory Multi-system Syndrome – temporally associated with SARS-CoV 2 (PIMS-TS): Critical Care guidance
- Hyperinflammatory shock in children during COVID-19 pandemic