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History of HELLP Syndrome

HELLP syndrome, which is a unique variant of preeclampsia (toxemia), was named by Louis Weinstein in 1982 after its characteristics:

H (hemolysis, which is the breaking down of red blood cells),
EL (elevated liver enzymes) and
LP (low platelet count).

It can be fatal to both the mother and the baby. by. HELLP Syndrome occurs in tandem with preeclampsia, but because HELLP Syndrome's symptoms may happen before preeclampsia's three findings (high blood pressure, protein in the urine, and swelling), they may be misdiagnosed as symptoms of gastritis, disseminated intravascular coagulation (DIC), acute hepatitis, gall bladder disease, and other conditions. As a result, the mother may not get the right treatment, leaving both mother and baby that much more at risk.

Who is at risk of getting HELLP Syndrome?

Five to seven percent of all pregnant women in the United States develop preeclampsia and between two and 12 percent go on to suffer from HELLP Syndrome. Best estimates are that HELLP Syndrome occurs in one per 150 live births (about one half of one percent), with a maternal mortality rate of 3.50 percent.

These numbers will vary with attention to the mothers care. If preeclampsia is diagnosed early and the baby is delivered, HELLP may not develop. The rate of HELLP and the mortality will then be lower than stated. Just how often does HELLP Syndrome happen? The exact number is unknown, because doctors may or may not catch it and have a difference of opinion as to what exact lab values constitute when a woman develops HELLP. If the diagnosis of preeclampsia was delayed or it was managed too conservatively, a woman's likelihood of developing HELLP Syndrome is even higher.

According to statistics obtained from a central database maintained by the National Center for Health Statistics for the year 2000, there were 4,065,674 live births in the United States. If one half of one percent of these births were to result in HELLP Syndrome, that means approximately twenty thousand women per year will develop HELLP.

Risk of Getting HELLP in Future Pregnancies

Studies vary on their estimates of HELLP occurring again. One study (Sibai, et al, 1995) places the risk at 5%. Another one (Sullivan, et al, 1994) finds it to be between 19% and 27%.

Both studies agree that HELLP mothers have increased risk (40-50%) for pregnancy-related complications in general with any future pregnancies. Some of these complications might include (but aren't limited to) gestational diabetes, high maternal blood pressure (that doesn't lead to HELLP Syndrome), and premature birth of the baby.

Study sources:

 -Sibai BM, Ramadan MK, Chari RS, Friedman SA. Pregnancies complicated by HELLP Syndrome: Subsequent pregnancy outcome and long-term prognosis. American Journal of Obstetrics and Gynecology 172: 125-9, 1995.-Sullivan CA, Magann EF, Perry KG, Roberts WE, Blake PG, Martin JN. The recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP) in subsequent gestations. American Journal of Obstetrics and Gynecology 171: 940-3, 1994

Babies Born from HELLP Syndrome

The good news is, in the majority of cases, the babies born to mothers with HELLP Syndrome do remarkably well. In a survey of babies born to mothers with HELLP Syndrome at WVU Children's Hospital, the most important thing is the size of the baby.

If the baby weighs over 1000 grams (approx. 2 lbs.), at birth, his or her survival rate and length of hospital stay is similar to non-HELLP babies of comparable sizes, and there doesn't seem to be many long-term adverse outcome. West Virginia University is studying long-term medical and developmental outcome of these babies.

If the baby weighs than 1000 grams at delivery, the news is not so good. Several studies have suggested longer hospital stays and more chance of needing ventilator care. Data from WVU Children's Hospital suggests that these small babies have a decreased chance of survival compared to other babies of the same size. Unfortunately, right now doctors can't predict the scope of the medical problems that these small babies encounter. Research into the effects of HELLP Syndrome on the newborn is ongoing at WVU and elsewhere.

How likely is the baby to die from HELLP Syndrome? Dr. Baha Sibai M.D., a perinatologist from the University of Tennessee in Memphis, notes that the perinatal mortality from HELLP Syndrome, 'ranges from 7.7 to 60 percent.' Most of these deaths are attributed to abruption of the placenta (placenta prematurely separating), intrauterine asphyxia (fetus not getting enough oxygen), and extreme prematurity.

Symptoms of HELLP Syndrome

The physical symptoms of HELLP Syndrome may seem at first like other pregnancy-induced high blood pressure conditions (such as preeclampsia).
Signs for a pregnant woman to look for include one or all of the following:

  • headache
  • nausea/vomiting
  • epigastric (stomach) tenderness and right upper quadrant pain (from liver distention)

These symptoms may or may not be present:

  • severe headache
  • bleeding
  • visual disturbances
  • swelling
  • high blood pressure
  • protein in the urine

The most common reasons for the mother to die are liver rupture or stroke, (cerebral edema or cerebral hemorrhage). These can be prevented if it's caught in time! If you or someone you know has any of these symptoms, please see a doctor immediately!

The only definitive treatment for women with HELLP Syndrome is delivery, regardless of how far along in the pregnancy the woman is.



Last Update: May 4, 2009


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