Perinatal Specialists may encounter clients who manifest symptoms with an ultimate diagnosis of HELLP Syndrome. HELLP Syndrome is a very severe variation of pre-eclampsia and can affect as many as 15% of expectant mothers.

HELLP is an acronym for Hemolysis (injury to or shredding of red blood cells), Elevated Liver enzymes, and Low Platelet count.

Signs and Symptoms

HELLP syndrome basically begins with a client having flu-like symptoms as early as the middle of the second trimester to after the baby is born (about the first week postpartum). Blood pressure and proteinuria, cardinal signs of impending pre-eclampsia, may or may not be present.

Hemolysis may produce symptoms similar to those of anemia or hepatitis.

Elevated Liver Enzymes: HELLP syndrome impairs liver function; levels may be elevated or may not be affected. These enzymes include aspartate aminotranaminase (AST) and alanine aminotransaminase (ALT)

Right Upper quadrant pain is the result of the obstruction of blood flow in the liver. There may also be hyperbilirubinemia, thus making a misdiagnosis of hepatitis sometimes likely. Other symptoms may include:

• Nausea
• Vomiting
• Headache
• Despondency
• Edema
• Bleeding gums (this is rare)
• Shortness of breath (due to pulmonary edema)
• Decreased placental perfusion
• IUGR (Intrauterine growth retardation of fetus)
• Maternal cerebral edema causing headaches and visual disturbances
• DIC (Disseminated Intravascular Coagulation - a disorder where anticoagulation and procoagulation effects exist at the same time causing an array of physical complications)

HELLP syndrome may be misdiagnosed as hepatitis, gallbladder disease, idiopathic thrombocytopenic purpura, or thrombotic thrombocytopenic purpura.

Management

Delivery of the baby is essential because liver function (and possibly renal function) rapidly deteriorates in this condition. Cesarean section is often the means of delivery as induction for vaginal delivery may be hazardous to the mother. General anesthesia may be the anesthesia of choice due to the severity of the syndrome and the need for quick action. High neonatal death or complication is associated with HELLP syndrome usually because of prematurity, when the syndrome manifests early in the pregnancy. Hemorrhage may occur within the mother's liver. Permanent liver damage may occur if delivery of the baby is delayed. The most common primary cause of maternal death was intracranial hemorrhage or stroke. Other causes may include cardiopulmonary arrest, respiratory failure, hepatic hemorrhage, hypoxic ischemic encephalopathy, and disseminated intravascular coagulation. Recent studies evaluating the use of high-dose corticosteroids in patients with HELLP syndrome have suggested that maternal morbidity and mortality could be significantly reduced using this therapy.

References:

Pritchard, J., MacDonald, P., and Gant, N. Williams Obstetrics. Appleton-Century-Crofts: Norwalk, Connecticut, 17th Edition, 1985.

Reeder, S., Martin, L., and Koniak-Griffin, D. Maternity Nursing: Family, Newborn and Women's Health Care. Lippincott: Philadelphia & New York. 18th Edition, 1997.

Internet Resources:

Most resources on an internet search of HELLP Syndrome will include many sites from mother's who have experienced HELLP Syndrome.

You might find the American Academy of Family Physician's article on HELLP Syndrome helpful as well as the HELLP Syndrome Society Website.