HELLP in pregnancy: Take your symptoms seriously!

Lisa, a Scottish woman living in Abu Dhabi, was in the middle of what seemed like a normal pregnancy. Her blood pressure was normal. And at her regular checkups, everything else seemed to be progressing normally as well.

But at around 20 weeks in, Lisa received a single sign that something could be awry: the fetus was smaller than it should have been at that stage. Lisa's doctor said they would keep an eye on it, but that it was nothing to worry about.

Four weeks later, Lisa started feeling what she thought was heartburn. A few days later, the sensation was still there, so she went to the doctor.

They ran some tests, including urine and blood pressure, and sent Lisa home. Everything looked fine, the doctor said.

But the pain grew over the course of the next 24 hours, eventually becoming so intense that Lisa knew she needed to go to the emergency room.

There, after more tests, Lisa was rushed to a larger regional hospital. Upon arrival, she had a seizure. They wheeled her into surgery to deliver the baby.

Two days later, she woke up to her child, a baby boy named Angus, and news that while she was asleep, her liver had ruptured — a very rare complication of HELLP syndrome, which has a 50% survival rate for mothers. HELLP syndrome stands for hemolysis, elevated liver enzymes and low platelet count.

Their babies are at risk as well: Born between 23 and 24 weeks, babies are so small and fragile they often do not survive. Lisa's baby, Agnus, lived a mere seven days.

"When my dad took me to [the emergency room] on that Monday night at 8 p.m., I did not think that was ever going to be the outcome, that Angus was going to be born," said Lisa. "I don't know what I thought they were going to do, but I certainly didn't think it was going to be that."

Subtle signs of HELLP

Stories such as Lisa's are emblematic of the danger when HELLP strikes.

HELLP is generally characterized by doctors as a severe form of preeclampsia, said Brian Brocato, physician and professor of maternal fetal medicine at the University of Alabama.

Although many women who develop HELLP have symptoms of preeclampsia, such as high blood pressure early on in their pregnancy, a number of women do not have such symptoms. HELLP frequently seems to come out of the blue, as it did with Lisa.

"A woman's not going to know always via symptoms that her platelet count has dropped, her liver function is elevated," said Brocato, outlining the classic signs of HELLP syndrome. "The [extreme] symptoms of pain up on their right side or bleeding from the gums … may not happen until laboratory [results] are very abnormal."

Doctors need to be aware of this, said Brocato. They need to understand that common pregnancy symptoms like heartburn, increased swelling, headaches and slightly raised blood pressure are not necessarily signs of preeclampsia or HELLP, but they could be and they require extra surveillance.

"A lot of patients — and I will even say [health care] providers — will blow off very subtle symptoms because they could be normal pregnancy symptoms," said Brocato.

The vast majority of pregnancies are "normal" and healthy and people "may lose some awareness" of rare complications like HELLP, especially when the woman seems to have had a healthy pregnancy up to that point.

DW spoke with two other women who described similar experiences with HELLP.

Sandra told us the majority of her pregnancy had been healthy, but that she delivered her baby five weeks early after developing HELLP. It was just a day after a routine checkup and blood tests had indicated everything was fine.

Another woman, Katie, developed HELLP syndrome in her third pregnancy. Her first two pregnancies had been healthy, and although she did have high blood pressure during her third, HELLP came on rapidly and with little warning, resulting in the delivery of her baby at 28 weeks.

The World Health Organization defines preterm as babies born alive before 37 weeks of pregnancy are completed. In the US, a full-term pregnancy is after 39 weeks.

HELLP: Things 'we don't understand'

HELLP syndrome involves hemolysis, elevated liver enzymes and low platelet count.

Hemolysis means red blood cells are breaking down, while elevated liver enzymes indicate an injury to the liver.

"We don't completely understand why each of those things happen," said Brocato, adding that doctors suspect it's a consequence of the process of preeclampsia.

Preeclampsia classically involves elevated blood pressure, which is caused by the constriction of blood vessels. This can decrease blood flow to the liver and contribute to a lower platelet count.

Current research indicates that preeclampsia and HELLP both stem from complications in the development of the placenta. That's likely why Angus, Lisa's baby, was measuring small at 20 weeks, said Brocato. "We often see growth restriction and preeclampsia go hand in hand."

Hindered development of the placenta will at some point show itself as a complication of the pregnancy, said Brocato, explaining that in most cases, this presents as preeclampsia that develops near a mother's due date. In these cases, a healthy baby can typically be delivered.

"But there's a subset of women, and we're talking about things that we don't understand, that are going to develop that severe disease very quickly, or very early in pregnancy. And I don't have a reason [why]," said Brocato. This can include women who do not appear to have any risk factors for preeclampsia.

Doctors also don't fully understand why a small percentage of women develop HELLP syndrome or severe preeclampsia after delivering the baby, when the placenta has been removed.

Brocato said this is particularly dangerous because after birth, women are not monitored nearly as closely as during their pregnancies, and signs of preeclampsia can be missed.

"We may see someone once or twice a week, right before the baby is born. And then the baby is born and we may not see them for several weeks … no one is watching," said Brocato.

It's critical, he said, that women and their health care providers keep an eye on any potential symptoms of preeclampsia that develop after birth, including headaches or increased swelling.

Surviving HELLP depends on where you live

HELLP is a rare pregnancy complication. It's very hard to measure how common it is globally, said Peter von Dadelszen, professor of global women's health at King's College London, because in many countries there is no word in the local language for preeclampsia or HELLP.

Risk factors include: people with underlying hypertension prior to pregnancy, a family history, such as a mother who experienced high blood pressure in pregnancy, diabetes, obesity, past cases of preeclampsia, infertility treatments and autoimmune conditions, such as lupus.

Both experts, Brocato and von Dadelszen, said Black women seem more likely to develop preeclampsia.

In general, research shows that around 3% to 8% of pregnant women will experience preeclampsia. But that can vary based on a woman's location.

Brocato, who works in the state of Alabama — which has the United States' sixth highest poverty rate, sixth highest obesity rate and the third highest rate of hypertension — said the figure there is closer to 20%.

Of those women, said Brocato, around 5% of the preeclampsia patients he sees develop HELLP.

Some estimates suggest HELLP has a mortality rate of 25%. But von Dadelszen said HELLP death rates are also place-dependent.

"I've not had a maternal death in my practice in almost 40 years. That's probably just because I'm lucky rather than good, and I've seen lots of HELLP syndrome. So in high income countries, if you have HELLP syndrome and you go to [the hospital], I can almost guarantee you survive," von Dadelszen said.

"If you pitch up with HELLP in a district hospital in rural Africa, that's a less certain outcome."