H.E.L.L.P. Syndrome

HELLP syndrome is a rare and potentially life-threatening complication that can occur in pregnancy. It is a collection of symptoms that can affect women who have pre-eclampsia or eclampsia, causing liver damage and problems with blood clotting. HELLP syndrome was identified in 1982 and is characterised by:

  • H aemolysis, meaning the breakdown of red blood cells
  • E L (elevated liver) enzymes, meaning possible liver damage
  • LP (low platelet) count, meaning that blood does not clot normally and bleeding may be prolonged

HELLP syndrome affects less than 1% of all pregnancies, and up to 20% of cases of severe pre-eclampsia. It often presents after pre-eclampsia has already been diagnosed. In around 80% of cases, HELLP syndrome occurs well before the pregnancy reaches full-term.

Signs and symptoms

The main symptoms of HELLP syndrome are:

  • Pain in the upper abdomen, including pain after eating
  • Upper abdominal tenderness, particularly on the right-hand side
  • Nausea and vomiting, which may be getting progressively worse
  • Pain in the shoulder or pain when breathing deeply
  • Headache
  • Eyesight problems and visual disturbance
  • Jaundice
  • Hepatomegaly, meaning enlargement of the liver
  • Easy or unexplained bruising
  • High blood pressure
  • Protein in the urine
  • Swelling

Diagnosis and complications

If HELLP syndrome is suspected it needs to be diagnosed or ruled out as quickly as possible. Diagnosis is made using liver function tests, and a full blood count should be taken to look at haemoglobin levels and platelet count.

If HELLP syndrome is not diagnosed properly and managed effectively, there are serious complications that occur in around 25% of cases. These include haemorrhage or rupture of the liver, and in rare cases a liver transplant may be required if the damage is severe. HELLP syndrome can also lead to retinal detachment – meaning that the tissue lining the inside of the eye is coming away from the blood vessels that keep it healthy. Other complications include intraventricular haemorrhage – meaning bleeding in the brain, and hydrocephalus – meaning a build-up of fluid inside the skull that causes the brain to swell. HELLP syndrome can be fatal if left untreated.

Stillbirth rates resulting from HELLP syndrome are higher than the statistics relating to pre-eclampsia and eclampsia, and will usually be caused by placental abruption – when the placenta becomes separated from the uterus; or placental failure with intrauterine asphyxia – meaning that baby is deprived of oxygen. Neonatal deaths in babies caused by HELLP syndrome usually result from prematurity.

 

Treatment and prognosis

The primary treatment for a woman who has HELLP syndrome is to deliver her baby as quickly as possible, even if this means that baby will be born prematurely.  A transfusion of blood products such as platelets or red blood cells may be needed.

Treatment with steroids – called corticosteroid therapy – may be given to help to mature the baby’s lungs prior to delivery, and a study from Missisippi reported that this can carry health benefits for the mother as well. Survival rates of babies born to women diagnosed with HELLP syndrome vary according to their birth weight and gestational age.

A woman with a history of pre-eclampsia or HELLP syndrome will be at an increased risk of developing the same complications in future pregnancies. HELLP syndrome is thought to recur in anything up to 20% of cases, although the statistics vary greatly by population. Each individual case is unique but in general, any woman who has a history of serious complications will need extra monitoring under the care of an obstetrician, so that the right care and treatment can be given if needed.

 

For more information on any of these issues, please contact The Pre-Eclampsia Foundation for help and support.

 

Sources:
www.preeclampsia.org
www.ncbi.nlm.nih.gov
www.patient.co.uk

 

by Helena, mum to Amalia and Luca

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