HELLP syndrome is a rare complication of pregnancy that primarily occurs in the third trimester as a type of preeclampsia. Preeclampsia and related disorders such as HELLP syndrome and eclampsia are characterized by high blood pressure that can lead to seizure, stroke, and multiple organ failure. In rare cases it can cause the death of the mother and/or the baby.
The acronym HELLP stands for Hemolysis (a breakdown of red blood cells that leads to a low red cell count); Elevated Liver enzyme levels (which indicate poor liver function); and Low Platelet count, which hinders the natural clotting ability of blood. Although these abnormalities can be detected through routine prenatal blood testing, they can easily be missed unless your doctor is specifically looking for them.
HELLP is rare, affecting only 0.2 to 0.6 percent of pregnancies. Or to look at it another way: preeclampsia is present in only about 6 percent of all pregnancies, and fewer than 10 percent of those pregnancies are affected by HELLP.
Early diagnosis is complicated because blood tests that are used to diagnose HELLP syndrome are not part of routine prenatal testing. Although researchers are looking into ways to diagnose HELLP syndrome early, there are currently no clear-cut diagnostic indicators and no way to predict which mothers are at risk for serious complications.
Delivery of your baby is the only effective treatment for HELLP syndrome, although excellent medical care of HELLP can prolong the pregnancy, which improves the baby’s chances for a good outcome.
HELLP Syndrome Symptoms
The symptoms of HELLP syndrome are vague and can easily be confused with those of several other medical conditions, including viral illnesses. In more than half of all cases, symptoms appear before the 37th week of pregnancy, but the syndrome can occur as early as the 20th week, and can also develop in the weeks after delivery. Symptoms may include:
- General malaise (a feeling of being unwell)
- Pain and tenderness in the upper right side of the abdomen
- Vision changes, such as blurred vision
- Edema (swelling)
All these symptoms can be confused with the normal symptoms of pregnancy. It is essential to pay close attention to your health and well being throughout your pregnancy, particularly during the third trimester and especially if you already have preeclampsia or are at risk for it. Should you notice any unusual symptoms or a sudden change in how you are feeling, contact your doctor immediately.
Causes of HELLP Syndrome
As yet there is no proven explanation for why HELLP syndrome or pre-eclampsia develops, but there are some good hypotheses, and some animal models are getting close to providing an answer.
It appears that abnormalities in early pregnancy placental development can affect the formation of blood vessels to the placenta, resulting in less than optimal blood flow to the placenta. Later in pregnancy, the body starts to increase the blood pressure in order to try to deliver more blood to the placenta and the baby. This leads to the high blood pressure component of pre-eclampsia.
These abnormal blood vessels also allow fragments of placental tissue to enter the mother’s circulation. The creates an immune response that causes the mother’s blood vessels to become “leaky,” allowing fluid and blood proteins (mainly albumin) to leak from the circulation into the urine (via the kidneys) or into the tissues (leading to swelling typically in the feet and legs).
This combination leads to the classic hallmark diagnosis of pre-eclampsia, which is high blood pressure with protein in the urine, and usually peripheral swelling (a newer definition of pre-eclampsia does not require swelling). HELLP syndrome is a more severe type of pre-eclampsia with abnormal blood chemistries due to swelling of the liver (leading to liver damage) and blood clotting problems, leading to lower platelet counts.
HELLP syndrome coexists with preeclampsia in 70 to 80 percent of cases, and a previous HELLP pregnancy is associated with an increased risk of HELLP and preeclampsia in subsequent pregnancies (European Journal of Obstetrics & Gynecology and Reproductive Biology, 2013). This suggests that both conditions may have a similar cause.
HELLP Syndrome Diagnosis
Early diagnosis of HELLP syndrome remains a challenge. The condition is more often identified in pregnant women who have already been diagnosed with preeclampsia because doctors are well aware of the association between the two. Conditions that can present similarly to HELLP syndrome include:
- Flu or other viral illness
- Gallbladder disease
- Idiopathic thrombocytopenic purpura
- Thrombotic thrombocytopenic purpura
If your practitioner suspects HELLP syndrome, he or she will perform a complete physical examination that includes blood testing. The findings may reveal abdominal tenderness, an enlarged liver, high blood pressure, and leg swelling. Results of liver function tests may show high levels of liver enzymes, and your platelet count may be low.
Other tests, such as ultrasonography and computerized tomography scans, may be performed as needed. These tests may show abnormalities in blood flow or internal bleeding.
The health of your baby will be evaluated by a variety of means, including biophysical testing, ultrasound examination, a fetal non-stress test, and fetal movement evaluation. Your doctor will make a diagnosis on the basis of multiple factors and will discuss your options with you once the diagnosis has been made.
HELLP Syndrome Treatment
The best way to stop HELLP Syndrome from continuing or worsening is by giving birth. Depending on how far along the expectant mother is in her pregnancy, doctors may consider inducing labor early, even if this means the baby will be born prematurely. With pregnancies that have not yet progressed to the 34th week, doctors will try to evaluate the baby’s lung function to see how well he or she would handle the delivery.
If early delivery is deemed unsafe, or if the pregnancy is still too far away from the due date, treatment may include:
- Best rest, sometimes in the patient’s home but usually in the hospital (depending on the severity of the condition)
- Corticosteroids (help baby’s lungs develop quicker)
- Magnesium Sulfate (prevents seizures)
- Blood pressure medication
- Blood transfusion if platelet count is too low
- Fetal monitoring and tests
In many cases a C-section delivery will be considered.
HELLP Syndrome Prevention
For women who are at risk for HELLP syndrome, particularly those with preeclampsia and those who have had HELLP in a previous pregnancy, close monitoring during the current pregnancy may be able to identify any problems early and prevent serious complications. Recent data shows that daily baby aspirin in a subsequent pregnancy can reduce the risk of repeat HELLP syndrome or severe pre-eclampsia by about a third.
Learning more about HELLP syndrome will enable you to prevent it from becoming life-threatening by being aware of the syndrome’s symptoms and notifying your practitioner immediately if any such symptoms develop.
HELLP Syndrome Risks and Complications
If this condition is left untreated or undiagnosed, or if it is misdiagnosed for too long, it can result in life-threatening complications for both mom and baby. About 25 percent of women develop serious pregnancy complications if HELLP syndrome is not treated early enough. Some of the complications that can arise from HELLP syndrome include:
- Placental abruption
- Pulmonary edema (a buildup of fluid in the lungs)
- Respiratory failure in mother
- Acute renal failure
- Respiratory failure in baby (from being born extremely premature)
- Intrauterine Growth Restriction (fetal IUGR)
- Ruptured liver hematoma
- Permanent liver damage
- Death of mother
- Death of baby
If the condition is severe enough and treatment is not prompt, HELLP syndrome can be fatal to the baby, and possibly to the mother as well. The mortality rate in cases of HELLP syndrome is 10–60 percent for infants and 1.1 percent for mothers.
When to Call Your Doctor
If you haven’t been diagnosed with HELLP Syndrome, but have symptoms or are at risk for developing it, you should definitely call your doctor immediately to begin testing. Early detection is important in the majority of cases of HELLP Syndrome.