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After reading the article you should be able to:

1. Identify symptoms of hemolysis, elevated enzymes, and low platelets (HELLP) syndrome.
2. Describe the difference between moderate and severe classifications.
3. Discuss nursing and medical interventions for a HELLP patient who presents in a crisis.

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By DIANNA J. HAGL-FENTON, RN, BSN

DIANNA HAGL-FENTON is the perinatal educator at Metropolitan Methodist Hospital, Women’s Pavilion, San Antonio, TX. The author has no financial relationships to disclose. EDITOR:Kathleen Moore, RN, BS. She has no financial relationships to disclose.

Your ability to recognize this critical condition could spare your pregnant patients and their babies a host of complications?and in some cases, save their lives.

The night shift on our labor and delivery unit was coming to a close when one of our obstetricians called to tell us that a 17-year-old in her 37th week of pregnancy was on her way in. The young woman had phoned her doctor that morning with complaints of stomachache, headache, and "not feeling well."

At her last prenatal checkup just a few days before, the patient had been "fine," with no signs of labor. "I think she may just have GI upset," the doctor informed me. "Please place her on the fetal monitors, do an assessment, and call me at home."

Within 10 minutes, the patient—I'll call her Maria King—arrived, moving gingerly and holding her right side. Small beads of sweat trickled down her face, brow, and upper lip. I escorted her to the closest labor room, thinking she must be in labor, but her appearance suggested something worse. She looked sick, and her expression was one of despair. I had seen that look before.

As I accompanied Ms. King to the bathroom, she began to cry. She was obviously in physical pain. When I asked if she was having contractions, she said she wasn't sure, this being her first pregnancy. I helped her into bed and then checked her urine sample for protein; it measured 4+.

While I attached the monitor, I asked about her present pregnancy and medical history. Pointing to her right upper abdomen, she explained that the pain had begun at about 1 am, waking her from sleep, and had been getting worse ever since. She rated the pain as 8 /10.

I quickly checked her vital signs, which were elevated: BP 208/127 mm Hg, pulse 128, respirations 28, and temperature 99.8?F (37.7?C). Her abdomen was tender to touch. Her lower extremities were swollen, with 2+ pitting edema, and her reflexes were abnormal at 3+, with two beats of clonus in each leg. The pelvic exam revealed a cervix that was thick, high, posterior, and closed, and the monitor showed no contractions. My patient was not in labor; she was in trouble.

Pain does not always spell labor

Ms. King's hypertension and proteinuria pointed to preeclampsia, but her abdominal pain meant the possibility of something more: HELLP syndrome. (The acronym stands for hemolysis, elevated liver enzymes, and low platelets.) The syndrome, which occurs in 0.17% – 0.85% of pregnancies,¹ is marked by a 35% – 50% fall in the mother's platelet count over a 24-hour period, with platelets decreasing at an average rate of 40,000/µL per day.² (In normal pregnancies, the platelet count remains above 150,000/µL.) Maternal liver enzyme levels rise—particularly the aminotransaminases, aspartate aminotransferase (AST) and alanine aminotransferase (ALT), and total serum lactate dehydrogenase (LDH)—indicating hepatic dysfunction and hemolysis. Severe right epigastric pain or upper abdominal pain signifies sudden, rapid progression of the disease.

The triad of abnormalities that constitute HELLP puts the mother at risk for liver hemorrhage and rupture, as well as a list of other complications, including anemia, disseminated intravascular coagulation (DIC), pulmonary edema, hepatic and renal failure, and stroke.² HELLP is also a threat to the baby, as it increases the likelihood of placental separation, premature birth, and other problems, such as respiratory distress syndrome.²

Most experts consider the HELLP syndrome a variant or subset of preeclampsia, or a severe form of the condition. It most commonly occurs in the third trimester; however, it can develop earlier in the pregnancy or in the first 48 hours postpartum.

HELLP usually accompanies other signs and symptoms of preeclampsia. But in some cases, hypertension and proteinuria—the hallmarks of preeclampsia—are mild or absent, at least initially.³ Patients with HELLP typically present with one or more of the following: malaise, epigastric or right upper abdominal pain or tenderness, nausea and vomiting, headache, visual problems, or exhaustion. Other signs of preeclampsia or eclampsia, such as edema, hyperreflexia, or seizures, may be present.

Researchers don't know definitively what causes HELLP. However, recent studies suggest that proteins from the placenta may damage liver cells, causing an acute inflammatory condition and disruption of the normal immune process.³

Anticipate the worst and act fast

Given the rapidly progressive and life-threatening nature of HELLP, suspect the syndrome whenever a pregnant patient reports epigastric or upper abdominal pain or tenderness.³ Consider it, too, when a woman develops thrombocytopenia of new onset during pregnancy, particularly in the third trimester or early postpartum period.³

Ms. King's right upper quadrant pain was highly suspicious of HELLP, so I knew I'd have to act fast. I phoned the obstetrician with my assessment and called for assistance from my colleagues. Together, we established IV access and drew blood for the following tests: CBC with platelets, chemistry profile with liver enzymes, PT/PTT, fibrinogen, and bleeding time. In addition, the physician ordered an ultrasound to check for the presence of a liver hematoma, bleeding, fetal wellbeing, and placental positioning.

We also obtained consent for delivery of the baby—the only true "cure" for HELLP. Since the syndrome is triggered by pregnancy, delivery of the fetus is necessary for the mother's recovery to begin.³ Suggested indications for delivery in patients with preeclampsia and HELLP are as follows: platelet count < 100,000/µL; progressive deterioration in hepatic or renal function; headaches, visual changes, epigastric pain, nausea, or vomiting, if persistent and severe; gestational age of 38 weeks; suspected placental abruption; severe fetal growth restriction; oligohydramnios; or other nonreassuring results on fetal testing.⁴

In some cases of HELLP, delivery is postponed to allow the fetus to mature, and the condition is managed more conservatively. This decision is based on the condition of the mother and fetus and the baby's gestational age. When delivery is delayed because of the baby's age, the mother typically receives a course of corticosteroids to promote fetal lung maturation. The mother's lab values may also improve with the administration of corticosteroids, so you may see it ordered for this purpose as well, either before or after delivery.^3,4

No matter how HELLP is managed, ongoing assessment is essential. Recognizing that, we continued to monitor both mother and baby while we awaited the initial lab results. We treated Ms. King's pain with meperidine hydrochloride (Demerol) and administered magnesium sulfate to relax her central nervous system in order to reduce the likelihood of seizures and lower her blood pressure slightly. We gave a 4-gram IV bolus of the magnesium sulfate, and then 2 grams per hour by IV infusion. (Ms. King later received labetalol [Normodyne, Trandate] to lower her BP further.)

In the meantime, the lab results came in, revealing a platelet count of 72,000/µL and greatly elevated liver enzyme levels. We considered this moderate, or Class II, HELLP. For more information on diagnosing and classifying HELLP, see the box on page 25.

Delivering the baby, planning for discharge

Although HELLP syndrome doesn't always preclude a vaginal delivery, Ms. King's obstetrician delivered the baby by C-section within a few hours of the patient's admission. The doctor based her decision on the patient's presenting lab results, the gestational age of the baby (37 weeks), and the fact that Ms. King was not in labor and was in considerable pain.

A CT scan performed several hours after the surgery showed a slight hemorrhage in Ms. King's liver, which explained her right-sided abdominal pain. The patient stayed in the hospital for several more days while she recovered.

Since lab abnormalities in HELLP don't begin to resolve until a couple of days postpartum, we continued to keep a close eye on Ms. King. The hemorrhage resolved, and the patient and infant fared well from then on, with no further problems related to HELLP.

Before her discharge, in addition to our standard post-C-section instructions, we provided Ms. King with written information on the signs and symptoms of preeclampsia and HELLP, to keep on hand in case of future pregnancies. A woman with a history of HELLP has an increased risk (perhaps as high as 19%) of developing the syndrome with a subsequent pregnancy; her risk of developing some form of pregnancy-related hypertension during a future gestation is even higher.²

When identified and addressed promptly, HELLP syndrome is often manageable. When misdiagnosed or not detected, it's more likely to lead to serious complications. Knowing the symptoms and laboratory abnormalities that signal HELLP increases the chances that you'll recognize the syndrome when it presents itself, and manage it appropriately. Fortunately for Ms. King and her baby, we were able to intervene before it was too late.

REFERENCES

1. Yucesoy, G., Ozkan, S., et al. (2005). Maternal and perinatal outcome in pregnancies complicated with hypertensive disorder of pregnancy: A seven-year experience of a tertiary care center. Arch Gynecol Obstet, 273(1), 43.

2. Martin, J. N., Jr., Rose, C. H., & Briery, C. M. (2006). Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol, 195(4), 914.

3. Martin, J. N., Jr., Magann, E. F., & Isler, C. M. (2003). HELLP syndrome: The scope of disease and treatment. In M. A. Belfort, S. Thornton, & F. R. Saade (Eds.), Hypertension in pregnancy (pp. 141–188). Oxford: Marcel Dekker.

4. National High Blood Pressure Education Program Working Group. (2000). Report of the National High Blood Pressure Education Program Working Group on High Blood Pressure in Pregnancy. Am J Obstet Gynecol, 183(1), S1.

Signs and symptoms

In addition to laboratory abnormalities, a woman with HELLP is likely to present with one or more of the following:

• Malaise
• Epigastric or upper abdominal pain or tenderness
• Headache
• Nausea/vomiting
• Visual problems
• Exhaustion
• Hypertension (may be mild)
• Proteinuria (may be mild)
• Edema
• Hyperreflexia
• Seizures

Diagnosing and classifying HELLP

There is no universally agreed-upon set of laboratory criteria for the diagnosis and classification of HELLP syndrome. Generally speaking, a diagnosis of HELLP is indicated when a pregnant woman has a platelet count that's below 100,000 – 150,000/µL along with elevated levels of the liver enzymes AST or ALT and LDH. (Other lab studies, such as serum haptoglobin, serum indirect bilirubin, and a peripheral blood smear, help determine the presence of hemolysis.)

HELLP syndrome may be classified as full or partial, based on the number of lab abnormalities present, or as Class I, II, or III, based on platelet count and liver enzyme levels. Two systems currently exist. The Tennessee classification system combines patients in Classes I and II and denotes "complete" HELLP syndrome for moderate to severe thrombocytopenia with platelets ≤ 100,000/µL and hepatic dysfunction with AST ≥ 70 IU/L and LDH ≥ 600 IU/L or bilirubin ≥ 1.2 mg/dL. Patients with some but not all of these characteristics are said to have "partial" HELLP syndrome. The Mississippi classification system uses the patient's lowest platelet count as the primary determinant. A patient platelet count < 50,000/µL, LDH levels> 600 IU/L, and AST and/or ALT levels> 70 IU/L constitute Stage I, or severe, HELLP. The moderate (Stage II) classification is used when LDH and AST and/or ALT levels are similar to those of Stage I but the platelet count is between 50,000/µL and 100,000/µL. A platelet count between 100,000/µL and 150,000/µL, LDH levels> 600 IU/L, and AST and/or ALT levels> 40 IU/L constitute Stage III, or mild, HELLP.

Sources: 1. Martin, J. N., Jr., Magann, E. F., & Isler, C. M. (2003). HELLP syndrome: The scope of disease and treatment. In M. A. Belfort, S. Thornton, & F. R. Saade (eds.), Hypertension in pregnancy (141–188). Oxford: Marcel Dekker. 2. Martin, J. N., Jr., Rose, C. H., & Briery, C. M. (2006). Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol, 195(4), 914.