HELLP Syndrome

HELLP syndrome is a severe form of preeclampsia, and the one habit that matters is this: do not use the blood pressure cuff to rule it out.

What the letters mean

HELLP stands for Hemolysis, Elevated Liver enzymes, and Low Platelets. The same vessel injury that drives preeclampsia damages the lining of the small vessels. Red blood cells are sheared apart as they squeeze through, which is the hemolysis. Platelets are consumed at those injured surfaces, which is the low platelet count. Fibrin deposited in the liver injures liver cells and stretches the capsule around the organ, which raises the liver enzymes and produces the pain that usually brings the patient in.

How it actually presents

The classic presentation is not a high blood pressure. It is right upper quadrant or epigastric pain, with nausea, vomiting, and a general feeling of being unwell for a few days. That picture is routinely mistaken for gastroenteritis, gallbladder disease, or influenza, and the pregnant patient is sent home. A meaningful share of patients with HELLP are normotensive, and a meaningful share have no protein in the urine. Headache, visual changes, jaundice, easy bruising, and bleeding gums also appear. Pain that refers up to the right shoulder is blood collecting under the liver capsule.

The laboratory triad

The diagnosis is made in the laboratory. Hemolysis shows up as schistocytes or burr cells on the peripheral smear, a rising LDH, a low haptoglobin, and a rising bilirubin. Liver injury shows up as AST and ALT at least twice the upper limit of normal. And the platelet count falls below 100,000. The nurse trends the platelet count rather than reading it once, and watches the fibrinogen, because a falling fibrinogen signals disseminated intravascular coagulation.

Treatment

Delivery is the only definitive treatment. HELLP does not resolve while the pregnancy continues, so transfusions and medications only hold the line until birth. Magnesium sulfate is given for seizure prophylaxis, and it is started even when the blood pressure is normal, because HELLP can still progress to eclampsia. It runs about 24 hours after delivery. Betamethasone is given before 34 weeks for fetal lung maturity. Labetalol or hydralazine are added only if the blood pressure climbs into the severe range. When the platelet count is low, an epidural or spinal is unsafe because of the risk of a hematoma around the spinal cord, so general anesthesia may be needed for a cesarean birth.

Nursing care assumes the patient bleeds

Give medications intravenously, never intramuscularly, because an injection site bleeds into the muscle. Avoid deep abdominal palpation, because pressure on a swollen liver can rupture a hematoma under its capsule. Hold prolonged pressure after every venipuncture, use a soft toothbrush and an electric razor, and keep needle sticks to a minimum. Maintain seizure precautions and a low-stimulation room, monitor mother and fetus continuously, keep strict intake and output, and have blood products available.

Report now

Escalate immediately for sudden severe right upper quadrant pain, for pain that radiates to the right shoulder, and for a sudden drop in blood pressure with a rising pulse. Together those point to a ruptured subcapsular liver hematoma, which is a surgical emergency. Also report oozing from intravenous sites or gums, a falling platelet count with rising liver enzymes, a seizure, new shortness of breath, and a urine output under 30 milliliters per hour.

After discharge

HELLP can begin after the patient goes home. New onset is most common 3 to 7 days after birth, and the risk lasts up to 6 weeks, including in women whose pregnancies were uncomplicated. Teach the patient to go to the emergency department for upper abdominal or shoulder pain, and to report a severe headache, visual changes, new nausea and vomiting, or unusual bruising and bleeding. The patient should keep postpartum blood pressure and laboratory follow-up, tell every future provider about the HELLP history, and never dismiss feeling unwell as normal recovery.