HELLP Syndrome 101

Understanding HELLP syndrome, the uncommon, but very serious pregnancy complication.

Yesterday we wrote about Dr. Desiree Bley’s horrible week, which featured a tragic case of severe HELLP syndrome. Today, we’re breaking the disease down to understand this uncommon, but very serious pregnancy complication.

HELLP is an acronym whose letters mean:

• H — hemolysis (the breakdown of red blood cells)
• EL — elevated liver enzymes
• LP — low platelet count (which is involved in blood clotting factors)

It’s among the scariest conditions healthcare providers and pregnant women will face, partly because it has so few predicting symptoms and no absolute specific diagnostic tests. Nobody knows what causes it and therefore, we can’t predict which women will get it. It occurs in approximately 1–2 pregnancies out of 1000 and in about 10–20% of pregnant women with severe preeclampsia or eclampsia. It’s a variant of these diseases, but most women with preeclampsia won’t go on to develop HELLP syndrome. It usually occurs at less than 37 weeks gestation, but can occur later and up to four weeks after delivery. The Preeclampsia Foundation estimates that as many as 48,000 women per year will develop HELLP syndrome in the US. If it’s not caught and treated in time, it can result in death of both mother and baby.

How do doctors know when their patient is developing HELLP?

The Preeclampsia Foundation says, “In some patients who are developing HELLP syndrome the primary preeclampsia indicators of high blood pressure and protein in the urine may not be present, and its symptoms can be mistaken for gastritis, flu, acute hepatitis, gall bladder disease, or other conditions. While some of these other conditions may also be present, there is no evidence they are related.”

What are the symptoms?

The symptoms that are sometimes reported by women and their doctors as associated with HELLP are these:

• Fatigue or feeling unwell
• Fluid retention and excess weight gain
• Headache
• Nausea and vomiting that continues to get worse
• Pain in the upper right part of the abdomen
• Blurry vision
• Nosebleed or other bleeding that won’t stop easily (rare)
• Seizures or convulsions (rare)

How is it diagnosed?

HELLP is diagnosed based on symptoms and lab tests. If blood tests show an abnormal red blood cell count, elevated liver enzymes and low platelets, even without the presence of other symptoms showing up in the mother, HELLP will be suspected.

What are the complications?

In mothers, doctors are worried about hemorrhage, seizures and these conditions:

• Disseminated intravascular coagulation (DIC) — a clotting disorder that leads to excess bleeding (hemorrhage)
• Fluid in the lungs (pulmonary edema)
• Kidney failure
• Liver hemorrhage and failure
• Separation of the placenta from the uterine wall (placental abruption)

As serious as HELLP syndrome is, most women in America who have access to prenatal and emergency medical care will recover from this disease. The American Pregnancy Association says that the maternal mortality rate is about 1.1% with HELLP.

In babies, complications depend on how early they’re delivered. If they must be delivered extremely prematurely in order to save their mother’s life, survival depends on their birth weight, development of their vital organs and the availability of NICU support.

How is it treated?

The best cure for HELLP syndrome is immediate delivery of the baby.

• If the baby is extremely premature, however, and if mom is relatively stable, doctors may try to buy some time by hospitalizing mom and treating her with antihypertensive medication, anti-seizure medication and corticosteroids to help her baby’s lungs mature in anticipation of a premature delivery.
• If baby is old enough to be delivered, mom may require induction of labor or a C-section. Depending on her lab values and level of medical stability, she may also require blood transfusions, blood pressure medication, anti-seizure medications and treatment in the intensive care unit.

While we can’t predict who will get HELLP, improving your overall health before getting pregnant, getting regular prenatal care and being aware of the symptoms may reduce your odds of developing complications. If you’re feeling a little “punk,” as Dr. Bley’s patient reported before she was overwhelmed by HELLP Syndrome, let your healthcare provider know ASAP.

Photo by Alice Proujansky/Every Mother Counts