It was more than 40 years ago, but ob-gyn Louis Weinstein, MD, vividly remembers the night he sat at the beside of a young Navajo woman suffering from a mysterious condition related to her pregnancy.
Hers was the first maternal death that Dr. Weinstein had experienced. The misery he felt at being unable to prevent it would result in his identifying and naming, for the first time, HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome. HELLP syndrome is a form of severe preeclampsia that affects about 45,000 pregnant patients annually in the U.S., according to the Preeclampsia Foundation.
The patient’s tragic death also inspired Dr. Weinstein to do decades of research on the condition, which had been previously misdiagnosed or unrecognized. It felt as though the patient “chose me, and the disease chose me. ... I did not choose them.”
That awful night with the patient who tragically died of then-unclassified HELLP syndrome also confirmed Dr. Weinstein’s belief in the “powers of observation” by any physician or health professional, young or old. Such observations, even “when least expected, can have profound ripple effects,” Dr. Weinstein said, “and this event supports that notion.”
The woman at the center of the discovery of HELLP syndrome came into Dr. Weinstein’s life in 1980.
Dr. Weinstein, a former chair of the AMA Senior Physicians Section governing council, was at the time the first maternal fetal medicine fellow at the University of Arizona in Tucson when he received a phone call from an Indian Health Service physician at a nearby Navajo reservation.
He was told about a woman in her 20s, pregnant with her first child and approximately 29 weeks’ gestation, who had been feeling ill for about a week with right upper quadrant pain, nausea and vomiting. The transferring physician reported to Dr. Weinstein the woman’s “markedly abnormal” laboratory results, which included elevated liver enzymes, low platelet and hematocrit counts, and glucose levels of 25 mg/dl.
She was not bleeding, nor was she in the mental state one might expect with severe hypoglycemia, and Dr. Weinstein assumed the laboratory results were in error. He repeated the tests, only to discover that they were, indeed, accurate.
“It really didn't make any sense,” he said, remembering that even his mentors, whom he reached out to for help, had not seen anything like it. “She was lucid, oriented. … She was not hypertensive. She did not have proteinuria. It wasn't by any means severe hypertension.”
After an ultrasound determined the woman’s fetus was anencephalic, Dr. Weinstein induced labor and she delivered a baby who died within minutes. But the woman’s condition degraded even further. She continued to hemolyze, he said, and there was marked hyperbilirubinemia. Even with glucose infusion, he said, her glucose level never rose above 50 mg/dl, a factor he would later determine to be a major marker for mortality from HELLP syndrome.
About a day after she delivered, the woman died, remaining lucid to within 10 or 20 minutes prior to death.
“I sat with her the whole time,” said Dr. Weinstein, feeling frustrated and confused. The postmortem examination yielded few answers.
“It showed significant disruption to the cells of the pancreas and the liver, but it didn't give us any reason why she died,” Dr. Weinstein said. She had petechial hemorrhaging, but that wouldn’t have killed her. “She didn't have what we would call a hemorrhagic stroke. None of us could figure out why she expired. It was awful.”
Compelled to investigate, Dr. Weinstein asked all the physicians practicing obstetrics in Arizona to send him pregnant patients with similar findings. He would treat almost 30 such women, and in 1982, he published an article in the American Journal of Obstetrics and Gynecology detailing the condition they shared.
The HELLP acronym he arrived at by way of asking himself what those with the syndrome most needed. Unfortunately, their symptoms were often overlooked, he said. After reviewing a plethora of cases, the most common preparation given to all of those who died of the disease, he said, is an antacid, used for ‘heartburn,’ which was the mistaken cause for the right upper quadrant pain.
“The key to the treatment of any patient with suspected HELLP syndrome is simply recognition,” Dr. Weinstein later wrote in the American Journal of Obstetrics and Gynecology in 2005.
Over the years, he has done more research and education on HELLP syndrome, and understanding of the disease has progressed. He is the past Bowers Professor and Chair in the ob-gyn department at the Sidney Kimmel Medical College at Thomas Jefferson University in Philadelphia.
Hypertensive disorders of pregnancy, including HELLP syndrome, “are among the leading causes of maternal morbidity and mortality in this country,” according to a U.S. Preventive Services Task Force (USPSTF) recommendation statement published in JAMA® in September.
Since 1993, the rate of hypertensive disorders of pregnancy has roughly doubled to over 1,000 cases for every 10,000 deliveries, the USPSTF’s statement says, noting that such disorders “were responsible for 6.8% of pregnancy-related deaths in the U.S. during 2014 to 2017.” The USPSTF recommends screening for hypertensive disorders of pregnancy with blood-pressure measurements at each prenatal visit, which is consistent with its 2017 statement on preeclampsia screening.
Dr. Weinstein said HELLP syndrome appears to be a complex immunological response to the pregnancy, and he’s certain that if the young woman who inspired his work arrived at a hospital today, she would have an excellent chance at surviving.
“The recognition is far superior,” he said, and she would have been given large amounts of glucose, transfusions and, in the only true therapy for HELLP syndrome, “she would be delivered in an expeditious manner.”
“I would have liked the opportunity to do better,” he said.
Dr. Weinstein now volunteers at the Barrier Island Free Medical Clinic in Johns Island, South Carolina, where he sees gynecological patients from the area who do not have medical insurance. All of their treatment is free.
He also continues to speak and write about HELLP syndrome and frequently hears from physicians and patients about their experiences with the syndrome.
Dr. Weinstein said that the United States’ high maternal mortality rates show that a great deal of work remains to improve prenatal and postpartum health in this country, with a major emphasis needing to be placed on pre-conception counseling, but advances in diagnosis and management are markedly improving. All physicians and other health professionals need to always listen to patients and use their observation skills, he said.
Learn more about AMA advocacy to improve maternal health.