Often misdiagnosed at first, HELLP syndrome occurs in one to two out of every 1,000 pregnancies and can lead to serious complications if not treated early. The acronym HELLP stands for Hemolysis, Elevated Liver enzymes and Low Platelet count.

While some researchers believe HELLP syndrome is a variant of preeclampsia, it can also occur independent of preeclampsia. The cause of HELLP syndrome remains unknown.

Many women exhibit symptoms that lead to diagnosis of HELLP syndrome:

Of course, some of these symptoms are normal for any pregnant woman during the third trimester. Blood tests can help determine if symptoms are a result of HELLP syndrome. However, not all women are symptomatic, and the disease can present without any forewarning.

When Susan Shay’s waters began to leak a week and a half before her due date, she had no idea she was in labor. The San Francisco mom had planned to labor at home for as long as possible, but because of the leakage she went to the hospital. Doctors did blood tests immediately and found a low platelet count and high liver enzymes.

“My platelet count was incredibly low,” said Shay, “the lowest they told me they’d ever seen at the hospital, and my liver enzymes were the highest they’d ever seen.”

While at first the doctors believed Shay might have preeclampsia, the numbers did not improve after several hours, and it became clear that this was HELLP syndrome.

HELLP generally develops in the third trimester, usually before the 37th week of pregnancy, although it can also present soon after childbirth. Many women have high blood pressure before developing HELLP syndrome. This was not the case for Shay. “I actually have low blood pressure,” she stressed. “I had no symptoms at all. I was 38.5 weeks pregnant. Three days before I gave birth, my blood pressure was totally normal. I hadn’t had blood tests for weeks, so I don’t know about those levels, but I wasn’t feeling any differently.” Doctors told her that most women in her situation would have been puffed up like a balloon from edema, but she didn’t experience any swelling. In fact, she displayed none of the typical symptoms of the syndrome and has no idea what caused its onset.

The cause of HELLP syndrome continues to elude medical professionals. Researchers have found that women over 25 years of age, and especially Caucasians, are more likely to develop HELLP syndrome. The risk is also higher for women who have already had children or who have had problematic pregnancies in the past.

Because the cause is unknown, there is really no way to prevent the onset of HELLP syndrome. It’s a good idea to see a healthcare professional throughout pregnancy and discuss any symptoms that arise.

Although magnesium sulfate can help prevent seizures in women with HELLP syndrome, the ultimate treatment for HELLP is to give birth. For Shay, this required a pitocin drip after six hours when her labor was not progressing. “It was becoming urgent to have this baby,” she said, “not for the baby’s sake, but for my sake. The baby would have been fine. It was for me.”

Even after birth, the syndrome may not disappear immediately. “Delivery is what cures this,” noted Shay, “but I continued to have blood tests still every six hours because my numbers weren’t improving. The first 24 hours after giving birth, I was so thirsty! I was not allowed to have more than one pitcher of liquid the entire day.” Because of HELLP syndrome’s effects on the liver, it is critical for doctors to ensure the kidneys are functioning correctly and that the woman doesn’t experience severe swelling.

HELLP syndrome can endanger a baby as well as its mother, primarily because its onset may require inducing labor, even if the pregnancy hasn’t reached full term. Some research has shown that bed rest, fluids and close observation can help manage the syndrome in pregnancies less than 32 weeks, allowing for the fetus to develop further before childbirth becomes necessary. Other treatment options are also available, including steroids, but if levels aren’t stabilized induction may be necessary.

A baby’s survival rate depends on its level of development at birth. If the pregnancy is far enough along, the baby may have no complications at all. This was true for Shay’s baby. “She was jaundiced and had trouble nursing, but she was also born 10 days early. We don’t know if it was because of HELLP, my IV fluids or her being born early. Who knows? They know very little about this syndrome.”

HELLP syndrome recurs in 19 - 27 percent of subsequent pregnancies. The risk of preeclampsia in future pregnancies can be as high as 43 percent. Usually HELLP syndrome is less severe the second time, and it tends to develop later in the pregnancy.

For Shay, the possibility of experiencing HELLP syndrome again is frightening. However, she feels confident that with extra monitoring everything will work out just fine. She knows her next birth experience will be different as a result of having had HELLP. “The instant I’m in labor, I have to go to the hospital. I don’t have the option of waiting it out.”

Sources:

"HELLP syndrome - PubMed Health." National Center for Biotechnology Information. Web. 10 Aug. 2011. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001892

“HELLP syndrome and your pregnancy.” American Family Physician. Web. 10 Aug. 2011.
http://www.aafp.org/afp/990901ap/990901b.html

"HELLP Syndrome: Recognition and Perinatal Management - September 1, 1999 - American Academy of Family Physicians." Home Page -- American Academy of Family Physicians. Web. 10 Aug. 2011. http://www.aafp.org/afp/990901ap/829.html

Shay, Susan. Telephone interview. 10 Aug. 2011.

Reviewed August 11, 2011
by Michele Blacksberg R.N.
Edited by Jody Smith