HELLP Syndrome | What Is It?

It’s very likely you’ve heard about preeclampsia and what to watch out for during pregnancy.

However, you might not know much about HELLP syndrome during pregnancy.

HELLP syndrome is generally thought to be a very severe form or variant of preeclampsia.

Let’s take a look at what HELLP is, what causes it, and the most common symptoms.

What is HELLP syndrome?

HELLP syndrome is a rare but life-threatening condition that affects the liver, blood clotting, and blood pressure.

It usually occurs in the last trimester of pregnancy but can also happen immediately after birth.

The exact cause of HELLP syndrome is largely unknown but some experts believe it’s a very severe form of preeclampsia.

Around 10–20% of women who develop preeclampsia will also develop HELLP syndrome.

HELLP syndrome meaning

In 1982, Dr. Louis Weinstein named the condition HELLP syndrome, using the first letters from the 3 main features of the disease:

H – Hemolysis

Hemolysis is the process in which your body breaks down old or faulty red blood cells in your spleen.

For those with HELLP syndrome, however, the breakdown of red blood cells happens too soon and is too rapid.

This means there are fewer red blood cells available to carry oxygen around the body and to the baby, via the placenta, and this leads to anemia.

EL – Elevated Liver enzymes

When you have high levels of liver enzymes present in your bloodstream, this indicates your liver isn’t working as well as it should. Inflamed or damaged liver cells leak high amounts of these enzymes into your blood.

LP – Low Platelet count

Platelets are responsible for blood clotting, which prevents excessive bleeding if you have an injury.

A low platelet count increases the risk of bleeding disorders, which are dangerous during pregnancy.

When does HELLP syndrome occur?

HELLP syndrome is a rare disorder and affects less than 1% of pregnancies.

It’s most common for the syndrome to occur during the third trimester, between 26 and 40 weeks of pregnancy.

It can also happen after birth, in the postpartum period.

You’re more likely to be at risk for the syndrome if:

• You’ve experienced HELLP syndrome in a previous pregnancy
• You’ve experienced preeclampsia or eclampsia in a previous pregnancy or in the current pregnancy
• You are Caucasian
• You’re over the age of 25.

HELLP syndrome symptoms

The signs and symptoms of HELLP syndrome are very similar to the signs and symptoms of preeclampsia.

They can also be confused with other normal conditions of pregnancy, such as indigestion or heartburn.

The symptoms might seem quite normal for pregnancy, but it’s important to look at how many of the symptoms you have, and how severe they are.

It’s important you visit your doctor or emergency department if you suddenly experience several symptoms at the same time – especially if they’re severe.

The symptoms of HELLP syndrome can vary, depending on the person, but the most common ones are:

• Feeling unwell and tired
• Pain in the stomach, particularly in the upper area of the abdomen
• Nausea
• Vomiting
• Headache.

As you can see, some of these symptoms can be seen as typical during pregnancy, and others are commonly associated with having a tummy bug.

Other symptoms you might have with HELLP syndrome are:

• Sudden and excessive weight gain
• Swelling, especially around the face and hands
• Changes to vision, including blurriness or loss of vision
• Severe headaches
• Shoulder pain (pain in the right shoulder associated with liver problems)
• Pain on breathing deeply (also linked to liver problems)
• Bleeding, such as nose bleeds, that doesn’t stop as quickly as normal
• High blood pressure.

If you experience these symptoms of HELLP syndrome, contact your doctor or go to the nearest emergency department immediately.

What does HELLP pain feel like?

Pain associated with HELLP is often called epigastric pain.

It occurs under the ribs on the right or is pain referred up into the right shoulder.

The pain under your ribs might be confused with indigestion, heartburn or gall bladder pain, or even with the stomach bug going around in your toddler’s childcare center.

It’s more likely to feel sharp and specific, though, and the shoulder pain will feel like you’re being pinched. It might be painful to lie on your right side.

Indigestion and heartburn happen because of changes in your pregnancy hormones, slowing of digestion, and because there’s a baby squishing your internal organs.

You’ll feel a burning or acid reflux sensation, which is how you can differentiate it from pain associated with HELLP syndrome.

Usually, a tummy bug will feel more like cramping.

If you have pain under your ribs or shoulder on the right side, seek immediate medical attention as this is potentially a sign of liver problems.

How is HELLP syndrome diagnosed?

Your health care provider will diagnose HELLP syndrome, based on the symptoms you present with.

Early detection is very important as it will improve the chances of diagnosis and treatment, and keep you and your baby safe.

How do they test for HELLP Syndrome?

If your doctor suspects you are developing HELLP, you might need the following tests:

1. A physical exam, to check for abdominal tenderness, to see whether your liver is enlarged and whether there’s any excess swelling on your body
2. Blood tests to evaluate blood platelet count, liver enzymes, and red blood cell count. This will rule out or establish whether there are blood clotting problems
3. Your bilirubin level will be checked; bilirubin is a substance made by the breakdown of red cells
4. Your blood pressure will be checked and monitored
5. Your urine will be tested to check for abnormal proteins
6. An MRI to determine whether there’s bleeding in the liver
7. A specialized ultrasound scan to check the blood flow to the baby via the placenta
8. The ultrasonographer will also check for liver hematoma (a collection of blood caused by hemolysis and clotting disorder)
9. Blood tests to check your kidney function.

The severity of HELLP syndrome is measured according to the blood platelet count of the mother.

It is divided into three categories, according to a system called the Mississippi classification:

• Class I (severe thrombocytopenia): platelets under 50,000/mm3
• Class II (moderate thrombocytopenia): platelets between 50,000 and 100,000/mm3
• Class III (AST > 40 IU/L, mild thrombocytopenia): platelets between 100,000 and 150,000/mm3

HELLP syndrome treatment

Treatment for HELLP depends on how many weeks pregnant you are and the severity of the condition. Birth of the baby is the best way to prevent the condition from becoming worse and stopping further complications.

However, if your symptoms are mild, or if you’re less than 34 weeks pregnant, your doctor might recommend:

• Corticosteroid medication, to prepare your baby’s lungs in case preterm birth is needed
• Blood transfusions, to treat low platelet levels and anemia
• Treatment to prevent seizures
• Medication to control blood pressure.

What happens if you have HELLP syndrome?

While you are having treatment, there will be ongoing monitoring of your red blood cells, platelets, and liver enzyme levels.

Your baby will also be closely monitored, to ensure he is coping and developing.

This means you’ll stay in hospital, as most of the monitoring will happen every day.

If your care provider decides your condition is becoming more severe and you need to give birth, you will have a plan for the best course of action.

Previously a c-section was the only option but the American Pregnancy Association now recommends women should try for labor, as those with HELLP syndrome have a higher risk of blood clotting problems after surgical birth.

The methods of induction of labor will depend on how close you are to giving birth and the severity of your symptoms.

You will need constant monitoring throughout labor and will probably be given a set timeframe for giving birth before assistance will become necessary.

If your baby is preterm, he will be cared for in a neonatal intensive care unit or special care nursery, depending on how early he is and whether the condition has affected him.

Mortality rate of HELLP syndrome

In the scientific literature, it has been suggested the mortality rate of HELLP syndrome is as high as 30%.

This is quite high but remember, HELLP syndrome occurs in less than 1% of all pregnancies.

HELLP syndrome is a severe and life-threatening disorder and can cause several other complications if not treated.

It can lead to:

• A blood clotting disorder called disseminated intravascular coagulation (DIC). This leads to heavy bleeding that is difficult to control, due to the inability of the blood to clot
• Breathing problems, caused by fluid buildup in the lungs (also called pulmonary edema)
• Kidney failure and fluid retention
• Liver rupture or liver failure
• Placental abruption, where the placenta separates from the wall of the uterus before birth
• Stroke
• Death.

Will HELLP syndrome affect the baby?

There are several birth injuries and complications associated with HELLP syndrome. The risks of these occurring, however, are reduced with prompt diagnosis and treatment.

Most of the complications occur due to placental abruption, poor placenta function, and extremely premature birth.

How long does it take to recover from HELLP syndrome?

It can take several days to recover from the symptoms of HELLP syndrome. If you have complications, it can take weeks to recover.

You will also need time to recover from giving birth and possibly from having a c-section.

Research shows the long-term effects of HELLP include depression and chronic hypertension.

It’s really important to seek additional support and after-care if you experience HELLP syndrome.

As a sudden and life-threatening disorder that occurs at the happiest but also the most vulnerable time in your life, the effects can be long-lasting and frightening.

It will make your postpartum recovery much longer, and you and your partner will need a higher level of support.

Can HELLP syndrome happen twice?

Women who have a history of HELLP syndrome are more at risk for it recurring in future pregnancies.

The good news is the condition is likely to be less severe if you have it again, and there is a reduced risk of complications.

It also means you are more able to prepare for and recognize the recurrence.

Can I have another baby after HELLP syndrome?

This is a really personal and individual decision that only you and your partner can make.

You can have another baby and, although you’re at risk of developing HELLP again, research shows there’s less chance of adverse outcomes in a second or subsequent pregnancy.

Can HELLP syndrome be prevented?

There’s no way to prevent developing HELLP syndrome. The best thing you can do is to be at your healthiest before and during pregnancy and to watch out for early signs of the condition.

These are ways you can be proactive:

• Be at your healthiest before getting pregnant
• Have a healthy diet and exercise during pregnancy
• See your care provider regularly during pregnancy
• Be aware of, and let your care provider know about, any risk factors for HELLP – such as high blood pressure, previous high-risk pregnancies, or family history of HELLP, preeclampsia, etc.
• Be familiar with the warning signs of HELLP and preeclampsia, and contact your healthcare provider immediately if you experience them
• Trust your instincts; if things don’t feel right, see your doctor.