Nichole Nilson had a killer headache and severe abdominal pain 32 weeks into a "wonderful" pregnancy and was told it was probably her gallbladder acting up. When AnneMarie Pilkington developed what could be signs of toxemia, her doctor told her the condition never occurs in a second pregnancy and not to worry so much. Dana Tracy's "perfect" pregnancy came to a screeching halt when abdominal pain became uncontrollable. After being checked for gall stones, she went home to suffer for two more days.
Nathan Hodgson made two trips to the drug store to pick up increasingly powerful indigestion remedies for his pregnant wife, Ellen, just days before her baby was due, again after an uneventful pregnancy.
Tammy Thunell was told her swollen hands and feet were normal.
Eventually, each woman was diagnosed with a syndrome that's a subset of preeclampsia, a medical emergency that can be solved only by delivering the baby.
It's called HELLP syndrome, a nickname coined by Dr. Louis Weinstein in 1982 because of its characteristics. The letters stand for Hemolysis, which is the breakdown of red blood cells, Elevated Liver enzymes and Low Platelet count, which negatively affects blood clotting.
Despite the moniker, it's anything but helpful. It can pose a life-and-death emergency for baby and mom, regardless of when it occurs in the pregnancy. And although some practitioners say it may be possible to delay up to 72 hours to allow very premature babies to gain lung-building benefit from steroids, prompt delivery is a must. So HELLP babies are sometimes very premature and sometimes full-term.
"It's nice if the baby is older than 30 weeks (gestation)," says retired Dr. Anthon E. Anderson, who treated many cases of HELLP during his 31 years as an OB/GYN, "but whenever it happens, you have to act."
Pilkington was worried because she'd seen firsthand the dangers of preeclampsia and HELLP. When her sister, Cindy Wagstaff, was 28 weeks pregnant, she went to the ER with a severe migraine. Even though a few hours later she had an emergency C-section, she nearly died. And her son, Stephen, who weighed only 1 pound 14 ounces at birth, was in the intensive care unit for three months and developed cerebral palsy from a blood clot. Later, Wagstaff had two more children and two more cases of HELLP, although early intervention spared those babies any long-term effects. Pilkington knew it could happen in a second pregnancy or in an even later pregnancy. She changed doctors.
The list of misdiagnoses is long. Women have also been told they have gastritis, disseminated intravascular coagulation, acute hepatitis and even flu.
Ellen Hodgson never had the classic symptoms of elevated blood pressure and swelling. Still, she needed an emergency C-section. Daughter Peri arrived in December 1998, fully developed and healthy. Hodgson has had two babies since, both healthy, with no sign of the syndrome, she says, although they were deliberately taken a week early to avoid late-onset HELLP.
"You really have to be on your toes if someone has had it with a first pregnancy," Anderson says. "If there's toxemia again in subsequent pregnancies, it's very likely."
The good news is that most pregnant women won't ever experience HELLP. While well-characterized eclampsia (also called toxemia) occurs in about 3 percent of pregnancies, HELLP accounts for only about 5 percent of those cases, according to Dr. Michael Varner, professor of maternal-fetal medicine at the University of Utah. The national HELLP Syndrome Society estimates about 27,000 women experienced the syndrome in 2000. It's hard to get a clear count, though, because it is often missed, and there's some debate about which lab values actually mean HELLP.
Varner notes that obstetricians are generally familiar with the condition and family practitioners are not far behind. Nevertheless, every couple of years a pregnant woman dies because it was not properly diagnosed, he says.
The symptoms and where a woman seeks care for them may make the difference, since the complaint may not seem to be related to the pregnancy. A woman goes to an emergency room with a killer migraine, for instance, and a doctor unfamiliar with HELLP never considers it. Plus, trademark symptoms like high blood pressure and protein in the urine may be mild or delayed, further complicating diagnosis.
By the time gall stones were ruled out and Tracy was diagnosed a couple of days later, her body was shutting down. Her blood wasn't clotting. Son Ryker, taken by emerency C-section, was small 4 pounds 5 ounces but fine. His mom still has her kidneys tested periodically, but she has recovered well. And when she got pregnant a second time, she had some toxemia, but she was able to deliver Regan in 2001 without major complications or C-section.
Thunell ignored the "don't worry" advice and kept complaining about the swelling. Her routine appointment was bumped up a few days, and she left it not in her car, but in an air ambulance headed to a hospital that could deal with her dire situation.
It's hard to pinpoint the cause. Preeclampsia itself is rather mysterious. Varner says that's called a "disease of theories about what causes it." Even less is known about the less common HELLP.
With HELLP, there's no guesswork, though, about the cure. Delivery and quickly.
About 2 percent of moms die from HELLP syndrome, the society says, while between 7 percent and 15 percent of the babies don't survive. Fully one-fourth of women suffer serious complications including kidney failure and liver rupture or fluid in the lungs. It's unclear how often the syndrome occurs in subsequent pregnancies, with estimates ranging from 5 percent to 27 percent. All the studies find that the HELLP mothers have increased risk for obstetric complications in general with subsequent pregnancies and those with pre-term severe preeclampsia and HELLP have an elevated long-term risk for cardiovascular disease.
Although early delivery is risky for a baby, the baby does "as well or better" than it would inside the womb at that point, Varner says. And mom is always better off.
Time factors help. Preeclampsia is more likely to develop the closer a woman gets to her due date. And late onset of a complication obviously helps baby's chance of thriving. "Where it is most dangerous is when it's in women remote from their due dates," Varner says. Delivering a baby "real early is a bad thing no matter how you look at it, and we have to make some pretty unpleasant choices."
HELLP syndrome babies typically do very well if they weigh more than two pounds at birth, according to the society, their outcomes similar to those of non-HELLP babies of comparable size. For those who are smaller, outcomes vary greatly. Many deaths are the result of the placenta prematurely separating because of the syndrome or intrauterine asphyxia, where the baby dies from lack of oxygen. And extreme prematurity also kills simply because the baby isn't developed enough to survive.
Those anxious to increase recognition of HELLP say they battle misconceptions. Pilkington was told, for instance, that she couldn't have HELLP or preeclampsia in a second pregnancy; it's the provenance of a first-timer. That's wrong, although preeclampsia is more common in a first pregnancy. HELLP doesn't seem to care how many pregnancies a woman has had.
Varner says women with any new onset of symptoms during pregnancy, including abdominal pain, shortness of breath, headaches or visual symptoms should see their care provider, although it could be something else.
Anderson says HELLP is clearly a response of the mother's body to the pregnancy, but why it reacts as it does is unclear. Whether it's due to excessive hormones or something else is simply a mystery. Infants developing in the womb are "basically parasites," he says, who do what they need to increase circulation and survive. They are known to produce substances that raise mom's blood pressure in order to increase the circulation that sustains them. Is that part of it? It's unknown.
When the baby is delivered, Anderson says, the mom's recovery is usually "almost miraculous."