This publication is for anyone who has suffered from or may suffer from HELLP syndrome and wants to know more about the condition.

It is also for doctors and midwives involved in the care of former, current and potential sufferers. It explains the nature and origins of the syndrome, gives guidance on detection, management and aftercare and looks at the chances of recurrence in subsequent pregnancies.

HELLP is the medical term for one of the most serious complications of pre-eclampsia, in which there is a combined liver and blood clotting disorder. H stands for Haemolysis (rupture of the red blood cells); EL stands for Elevated Liver enzymes in the blood (reflecting liver damage); LP stands for Low blood levels of Platelets (specialized cells which are vital for normal clotting). HELLP is as dangerous as eclampsia (convulsions) and probably more common, although it is less easy to diagnose. Some specialists believe that HELLP may be on the increase for reasons which are not known.

HELLP syndrome may be preceded by clear signs of pre-eclampsia – most typically high blood pressure, protein in the urine and swelling of hands, feet or face. But, like eclampsia, it can also arise out of the blue without any of the classic warning signs. The typical presenting symptom is pain just below the ribs (‘epigastric pain’), sometimes accompanied by vomiting and headaches. This pain is sometimes confused with the discomfort of heartburn, a very common problem during pregnancy. But, unlike heartburn, the pain of HELLP syndrome is not burning, does not spread upwards towards the throat and is not relieved by antacid. The pain is often very severe and is associated with tenderness over the liver. It is not uncommon for women with this pain to be diagnosed as suffering from some other acute abdominal condition, typically inflammation of the gall bladder (cholecystitis).

As with eclampsia, HELLP syndrome is most likely to occur immediately after delivery – sometimes developing with devastating speed. However, it can arise at any stage during the second half of pregnancy – and some rare cases have been recorded even earlier.

HELLP syndrome may be associated with one or more of the following problems:

  • Severely disturbed blood clotting function, leading to heavy, uncontrollable bleeding, particularly after surgery;
  • Severe liver damage, which can lead to failure or even rupture of this vital organ;
  • Severe kidney problems, including kidney failure;
  • Breathing difficulties, which may be severe enough for the mother to need artificial ventilation.
  • Stroke (cerebral haemorrhage) with or without eclampsia (convulsions).

The diagnosis of HELLP syndrome can only be confirmed in hospital, and emergency admission is essential for all suspected cases. Once the syndrome is diagnosed the baby should be delivered as soon as the mother’s condition is stable, regardless of the maturity of the baby, since delivery is the only cure for this life-threatening condition. If the blood clotting system is severely disturbed it may be necessary to give transfusions of the platelets essential to clotting before delivery can take place.

It is not uncommon for the symptoms to become worse – or to develop for the first time – in the 48 hours following delivery, and treatment in an intensive care unit may be necessary. All treatment is aimed at supporting the mother’s systems which have failed (liver, kidney, lungs, clotting) until such time as they have recovered enough to cope on their own. Providing no permanent damage has occurred, the mother should enjoy a full recovery. This may take as little as a few days or as long as two to three months (not all of it spent in hospital) depending on the severity of the mother’s problems.

HELLP is a maternal problem which has no specific effects on the unborn baby. However, as with all cases of severe pre-eclampsia, the baby may suffer growth retardation and even distress as a result of the underlying cause – a shortage of maternal blood flow to the placenta. But in most cases of HELLP, delivery is for the mother’s benefit, sometimes with tragic results for babies who are too premature to survive outside the womb.

About one sufferer in every 20 will suffer a recurrence of HELLP in her next pregnancy. However, there is no way of predicting who is most likely to suffer a recurrence and no specific means of prevention, although treatment with low-dose aspirin may be recommended in cases where the syndrome developed relatively early in pregnancy – i.e. before 32 weeks (1). For optimum safety, any woman who has suffered HELLP in one pregnancy should be considered ‘at risk’ in the next pregnancy and monitored carefully throughout with a view to detecting signs of recurrence at the earliest possible stage. Former sufferers may like to consider preconception counseling with an expert to devise an appropriate ante-natal care programme for the next pregnancy (2).

Notes:

  1. Fact Sheet 1: Low-dose Aspirin for High-risk Pregnancy.
  2. Consult an Expert via APEC: list of consultants who are considered expert in the management of all aspects of pre-eclampsia and are willing to accept referrals from GPs.