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What Is It and What Causes It?

Reyes Syndrome is a rare and often fatal illness that develops rapidly and can be a complication of the flu or chicken pox in children. It can also cause severe brain damage.

Reyes Syndrome has been linked to the use of aspirin in the treatment of these viral infections. It is highly recommended that aspirin should not be given to children 19 years of age and under who are suffering from the flu or chicken pox.

There appears to be no danger in giving aspirin to otherwise healthy children for simple pain or minor cuts or other injuries.

What Are the Symptoms?

The symptoms include sudden, severe vomiting, violent headaches, hyperactive or combative behavior, personality and behavior changes in those who appear to be recovering from a viral infection. The symptoms progress to listlessness, sleeping constantly, and possibly convulsions and coma.

If these symptoms are present, parents are advised to seek immediate medical attention.

How Is It Treated?

Treatment of Reyes Syndrome requires hospitalization.

* What is Reye’s Syndrome?

Reye’s syndrome is an acute disorder which affects children when they seem to be recovering from a viral illness like ‘flu’, chicken-pox or diarrhoea.

The child has a change in his/her personality or becomes drowsy or unconscious and develops frequent or persistent vomiting.

Unless diagnosed and treated successfully, death or severe disability may result. Abnormal accumulations of fat develop in the liver and some other organs of the body. There is swelling of the brain which can cause it injury.

The disease affects children from infancy onwards. Although it does occur in teenagers and adults, it is rarely recognized after 19 years of age. Children of both sexes and all races can be affected.

*What Causes Reye’s Syndrome?

The cause is unknown; but a number of studies suggest a link between the development of Reye’s syndrome and the use of aspirin to treat the viral illness which precedes it. These studies do not prove that aspirin causes Reye’s syndrome but may aggravate it. Other chemical substances including insecticides and emulsifiers have also been implicated but the evidence that these are involved is controversial.

Because there are alternatives, there is no need to use aspirin for feverish illnesses in childhood. Since 1986, aspirin preparations have been labeled with differing warnings. The latest advice from the Medicine Control Agency, issued in April 2002, is as follows:-

Carton/Label

DO NOT GIVE TO CHILDREN UNDER 12 YEARS, AND AVOID UP TO AND INCLUDING 15 YEARS OF AGE IF FEVERISH

Patient Information Leaflet

THERE IS A POSSIBLE ASSOCIATION BETWEEN ASPIRIN AND REYE’S SYNDROME WHEN GIVEN TO CHILDREN WITH A FEVER. REYE’S SYNDROME IS A VERY RARE DISEASE WHICH CAN BE FATAL. FOR THIS REASON ASPIRIN SHOULD NOT BE GIVEN TO CHILDREN UNDER 12 YEARS AND SHOULD BE AVOIDED UP TO AND INCLUDING 15 YEARS OF AGE IF FEVERISH.

*What are Reye-like illnesses?

Many rare genetic (inherited) disorders of the body chemistry (metabolic disorders) are now known to cause an illness exactly like Reye’s syndrome. The early treatment of these “Reye-like illnesses” is similar to that of Reye’s syndrome but some require special drugs or diets for treatment and to prevent recurrence of illness. Special laboratory tests are required to make certain that such disorders are excluded in all children with Reye’s syndrome. Furthermore other children in the family may also have the genetic disorder in a latent form. (Reye-like syndromes).

*What are the early stages of Reye’s syndrome?

Persistent or continuous vomiting and/or signs of brain swelling:

Listlessness

Loss of energy

Drowsiness

Personality changes:

Irritability

Aggressive behaviour

Disorientation:

Confusion

Irrational behaviour

Delirium, Convulsions

Reye’s syndrome should be suspected if a child displays all or part of this pattern of symptoms a few days after the onset of viral illness such as ‘flu’ or chicken-pox. Fever is not usually present. Many diseases have similar features. Medical staff who do not have experience of Reye’s syndrome may initially suspect encephalitis, meningitis, poisoning, mental illness or even drug abuse. In infants the symptoms of Reye’s syndrome may not follow a typical pattern; for example vomiting is less common. However, it is in infants that the “Reye-like illnesses” are most likely to occur.

*What should be done if symptoms of Reye’s Syndrome develop?

Advice to parents

Consult your general practitioner immediately, a child’s life may depend on obtaining emergency treatment in the nearest children’s unit.

Statistics indicate a better chance of survival when Reye’s syndrome is diagnosed and treated in its earliest stages. The later the diagnosis, the more advanced the coma and the chances of survival and full recover are greatly reduced.

Reye’s syndrome can successfully be managed with a good outcome if diagnosed early and meticulously treated.

*Are there lasting effects?

Survival is related to the severity of the brain swelling. Some children recover completely. Others may sustain slight to severe permanent brain damage. All children surviving Reye’s syndrome require follow up, with developmental assessment. (After Reye’s Syndrome).

*What can I do if my child has a fever and I cannot use aspirin?

Fever is a normal response to infection. It may actually help your child to fight off viral illness. If you feel you must do something, there are a number of non-drug actions which may be taken:

Excess covering and clothes should be removed. Sponge or bathe the child (especially head, neck and face) with cool water and allow this to evaporate. This will often induce sleep. Keep the room at a moderate temperature. Encourage the child to drink liquids such as water or non-fizzy drinks.

If the fever does not respond to these measures, obtain advice from your doctor.

Children under 12 months of age who have fever should not be treated without medical advice.

*Is Reye’s Syndrome a common disease?

Reye’s syndrome was first described in 1963 by an Australian pathologist, R. Douglas Reye, MD. Since then it has been recognized in many parts of the world. It used to occur in minor epidemics, which in some countries (most notably the USA) were associated with influenza epidemics.

Although the exact incidence of the disease in the UK and Ireland is not known, a surveillance scheme depending on voluntary reporting of cases by British and Irish pediatricians has been in operation since 1981. This has shown a dramatic fall from nearly a hundred reported cases in 1984 to only two in 2000.

It is likely that there is some under-reporting and under-diagnosis, but this downward trend has also been seen in the USA and is attributed to reduction in use of aspirin in children since the public warnings in 1986. Nevertheless, there may be a resurgence, especially if there is a large ‘flu’ epidemic or the aspirin warning is ignored as time goes by. It is thus essential to retain high diagnostic awareness of Reye’s syndrome and Reye-like illnesses among clinicians.

Remember

Reye’s syndrome can appear soon after a viral infection, especially a flu-like illness or chicken-pox.

Early signs of Reye’s syndrome are continuous vomiting, listlessness, drowsiness, irritability, confusion, irrational behaviour or a convulsion.

The GP should be phoned immediately.

In patients with the signs and symptoms described above, abnormal liver function tests, including raised blood ammonia strongly suggest a diagnosis of Reye’s syndrome or a “Reye-like” inherited metabolic disorder.

Inherited metabolic disorders can present with a “Reye-like” illness especially in children under 4 years. It is essential to diagnose them because of implications for genetic counseling, specific treatment and investigation of other children in the family.

Medicines can mask symptoms. Therefore, if a child develops any of the symptoms of Reye’s syndrome, the child should not be given anti-nausea or fever-reducing medicine.

Do not give aspirin to children under 12 years except on medical advice and avoid in children aged up to and including 15 years if feverish.

Reye’s syndrome can rarely occur in adults.

Early diagnosis and appropriate treatment is vital.