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In the spotlight: Diagnosing meningococcal disease in the first 12 hours

A new study identifies key early symptoms that could increase cases spotted in initial GP consultations. Study leaders Dr Matthew Thompson and Dr Anthony Harnden ­ GPs themselves ­ explain

A new study identifies key early symptoms that could increase cases spotted in initial GP consultations. Study leaders Dr Matthew Thompson and Dr Anthony Harnden ­ GPs themselves ­ explain

Missing a diagnosis of meningococcal disease can have devastating consequences. But the challenge facing GPs is immense. GPs must identify those children who may have meningococcal disease, from the vast majority who have just self-limiting infections.

In some children, the diagnosis of possible meningococcal disease is obvious: this is the febrile child with a petechial or even grossly haemorrhagic rash, who is looking generally unwell. However, for the majority of children, making the diagnosis in general practice is far more difficult.

Our study found the classic symptoms of rash, neck pain and photophobia did not appear until 13 to 15 hours and yet most parents bring their child to the GP within six to 12 hours of the illness starting (see box overleaf).Because of this up to 50 per cent of children presenting to GPs with meningococcal disease are not initially recognised and so re-present later to their GP or A&E.

Middle stage (six to 12 hours)

One of our key findings was that three-quarters of children presenting with meningococcal disease in the middle stage (six to 12 hours) will have one or more of three symptoms of sepsis: leg pains, cold extremities or abnormal skin colour.

These features appear to be less common in self-limiting infections.

Leg pain: The leg pain in meningococcal disease is often so severe that the child will be reluctant to stand up ­ this occurs in 53-62 per cent of children aged over five, and in 31 per cent of those aged one to four years. The pathophysiology of the leg pain in meningococcal disease is not usually due to septic arthritis, but may be a response to inflammatory mediators released from muscles during rigors.

Cold extremities and pallor: Children will have cold hands and feet despite the fever. As well as pallor, other abnormal skin colouration to look for include mottled or cyanosed.

Cold extremities and pallor represent deterioration in the peripheral circulation. Measuring capillary refill time and possibly pulse rate or blood pressure might give more objective measures of early shock.

Other 'middle stage' symptoms: These include drowsiness and breathing difficulty that is laboured and/or rapid. Remember, though, that breathing difficulty can be a sign of respiratory infection as well as early sepsis of course, and is another potential red herring.

A quarter of parents we interviewed said their child had respiratory tract infection symptoms (eg cold, cough,) before developing meningococcal disease.

The GP 'instinct'

As well as symptoms and signs described, GPs often use other information to help in their assessment of a febrile child. GPs often have a 'gut feeling' about a child ­ they are probably subconsciously recognising a child who looks generally unwell, not smiling or playing, not interacting with the environment, looking very pale, floppy in tone, etc.

Knowledge of the family and their consultation pattern is also important ­ the parent who rarely brings a child in for the usual cough and cold, who is now very worried, needs to be taken seriously. This can help when triaging children with febrile illnesses over the phone. In general it is far better to offer a face-to-face consultation, especially if the child's symptoms appear different to common self-limiting infections.

Remember, too, that it is important to review in a reasonable timeframe febrile children who are deteriorating, even if they have already been seen for the same illness.Meningococcal disease develops very rapidly in children; most will be in hospital within 24 hours of the first symptoms starting. GPs should take very seriously the child who became unwell that day and seems to be deteriorating, rather than the child who has had a febrile illness for many days ­ meningococcal disease is less likely in the latter group.

If the GP has concerns about a child with non-specific features such as fever, poor feeding or irritability who seems to be deteriorating, then they should schedule a repeat consultation for four to six hours ­ not the next day. Our data confirms that symptoms progress rapidly in a matter of hours and so parents should be able to re-consult their GP if the child's conditions worsens.

Earlier stages (four to six hours)

At this stage in the disease children may have very little, if anything, to distinguish them from children with common infections, for example gastroenteritis and URTI. Their symptoms include:

  • fever ­ almost all have raised temperature
  • generally miserable or irritable
  • diarrhoea and vomiting may occur
  • sore throat, runny nose, cough
  • headache in older children.

There are generally few indications at this stage that the child may have meningococcal disease. Hence the importance of 'safety netting' for parents of febrile children ­ continued deterioration in a child seen earlier that day may well warrant a follow-up consultation within a few hours. Using paracetamol or ibuprofen to reduce fever and settle a fussy child is unlikely to mask impending sepsis or meningitis. The fever may reduce slightly, but the child will continue to deteriorate.

Later stages (>12 hours)

As stated before, the haemorrhagic rash occurs only after about 12 hours from the illness starting, when the child develops what we know as the 'classic' features of meningococcal disease.

The haemorrhagic rash is only present in 42 per cent of infants, and up to 70 per cent of older children.Its absence, especially in infants, is not necessarily very reassuring. Furthermore, the rash may not be the classic petechial or grossly haemorrhagic when it begins ­ it may be macular or papular and easily confused with the myriad other causes of rash in children.

Again, it is only at this later stage that the typical features of meningism occur ­ photophobia, neck pain or stiffness, and occasionally bulging fontanelle in infants. Deteriorating mental status will present as confusion and floppy tone in infants. Nearly 10 per cent of children will be unconscious and 10 per cent may have a seizure before admission. By this stage in the illness, children are generally recognised as being obviously extremely unwell, and urgent referral initiated.

Treatment

If you suspect meningococcal disease, it is critical to arrange urgent paediatric hospital assessment. Calling 999, and talking with the hospital on-take team (or A&E) about your concerns, are important.

The current Department of Health recommendation is to administer parenteral benzylpenicillin for children in whom you suspect meningococcal disease. Evidence for this is somewhat conflicting in the medical literature, as there is theoretical risk of accelerating deterioration by endotoxin release without appropriate management of shock. The other priority is to get the child to hospital as quickly as possible.

But in situations where the hospital is more than an hour or so travelling time away, it would be ideal to also start IV fluids.This will help to treat the septicaemic shock that is an important cause of death in these children. However, starting IVs in young children can be extremely difficult.

This paper was the first systematic study of how children and adolescents with meningococcal disease present before hospital admission. There have been very few primary care-based studies looking at how the disease presents. Most 'textbook' descriptions of meningococcal disease are based on hospital-case series, when the disease may be far more advanced and obvious.

Method

Parents were interviewed and GP records examined of 448 children with meningococcal disease in the UK. This gave them hour-by-hour information on what clinical features these children had before hospital admission. The children ranged in age from 0 to 16.

Our results

In the first four to six hours of the illness starting, most children had only non-specific symptoms (nausea, vomiting, fever, irritable, miserable). The classic features of the haemorrhagic rash, neck stiffness, photophobia, impaired level of consciousness occurred at 13-22 hours. Some 72 per cent of children had one or more of severe leg pains, cold hands or feet, and abnormally pale skin. These 'warning signs' of sepsis developed at about eight hours into the illness, and earlier than the 'classic' features.

By familiarising themselves with the new checklist of symptoms, GPs could reduce the proportion of cases missed at first consultation from a half to a quarter.

Matthew Thompson is a clinical lecturer in the department of primary health care, University of Oxford ­ he also works as a GP a in OxfordCompeting interests None declaredAnthony Harnden is a senior lecturer in the department of primary health care, University of Oxford ­ he is a GP principal in Wheatley, Oxford. Competing interests: None declared

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