The itching started at the end of the first trimester. And it wasn’t just any itch. This was “plague level,” said Sarah Strand, 40, an associate professor at Loyola Marymount University in Los Angeles, who was pregnant with twins last year.
“It’s like you laid down in a mosquito cave,” Strand said. It was particularly bad at night when Strand would scratch in her sleep, waking to find her sheets bloody.
She called her doctor’s office and a nurse advised her to see a dermatologist, but Strand found no relief.
Toward the end of her pregnancy, she said, the itch was so bad she felt she was “going insane.” By the time she reached 34 weeks, she had begun researching her symptoms, desperate for relief. It was then that she discovered a liver disease called intrahepatic cholestasis of pregnancy.
Cholestasis slows or stops the flow of bile from the liver. Normally, the gallbladder, a small organ under the liver, releases bile into the small intestine to help break down and absorb fats during digestion. When the flow of bile is impeded, bile acids can build up in the liver and leak into the bloodstream.
Studies have found that the more bile acids build up in the bloodstream, the higher the risk of stillbirth. Other risks of cholestasis include fetal distress, preterm delivery and maternal haemorrhage. And if a mother has cholestasis, her baby is at higher risk of passing the first stool, or meconium, in the womb, which can be dangerous if a baby inhales it into the lungs.
“I was freaking out,” Strand said.
Strand took a blood test to see if her liver enzymes were elevated, a sign her liver cells were damaged. The test delivered shocking results: She had more than 30 times the normal levels.
A bile acid test, considered the most sensitive and reliable test for cholestasis, also came back severely elevated. Her doctor told her they were the highest numbers she had ever seen, Strand said.
When women are diagnosed with cholestasis, most obstetricians consider the optimal timing of delivery to be around 37 weeks, but in this case, her doctor recommended that she deliver immediately.
At 34 weeks and 6 days, her twin girls were born via C-section. And in less than 24 hours, Strand’s itching disappeared.
Cholestasis is relatively rare and can present differently among different women, so it isn’t necessarily the first thing doctors or nurses think of when a patient complains about itching. To further complicate things, the main symptom, itching, can mimic other problems like PUPPP (pruritic urticarial papules and plaques of pregnancy), eczema or the general itchiness that pregnant women experience as their skin stretches and their blood volume and hormone levels start to change.
“This has flown under the radar for so many decades,” said Dr Jonathan K. Mays, M.D., the director of obstetrics and maternal-fetal medicine at NYC Health + Hospitals/Metropolitan and an associate professor at New York Medical College.
If it’s not caught in time, the outcome can be devastating.
Jessica Tamez, 23, who lives in Quincy, Fla., about 25 miles northwest of Tallahassee, started itching in her third trimester. She called her doctor’s office in October, the month before her baby was due, and a nurse told her to take Benadryl.
“I took so much Benadryl that I couldn’t even really drive myself places. It was the only way I wouldn’t scratch myself raw,” she said. “I thought I was crazy.”
She also had other telltale signs of cholestasis: dark urine and pain in the upper right quadrant of her abdomen.
“I felt silly thinking about going to labour and delivery complaining about itching, so I just took more Benadryl,” she said. “I thought they were never going to take me seriously.”
When she arrived for her regularly scheduled appointment the following week, her doctor listened to her symptoms and ordered a blood test, which she took the following day.
But on the night the blood test was performed, she realized that her baby, a girl, wasn’t moving as much as she usually did. The next morning, she wasn’t feeling any movement. An ultrasound revealed that her daughter was in distress.
Minutes after the ultrasound, her doctor ordered an emergency C-section, but it was too late.
“They tried to resuscitate her and couldn’t,” Tamez said. Her daughter, whom she named Elena, had died.
Her doctor told her that he believed the cause of death was cholestasis because Tamez’s bile acids were 12 times the normal amount at the end of her pregnancy and her daughter had meconium aspiration.
Even if the disease is caught right away, careful monitoring will not necessarily prevent stillbirth. Though cholestasis may cause itching for weeks in the mother, babies can decline rapidly and without warning, said Dr Frederick Friedman, M.D., the director of obstetrics for the Mount Sinai Health System.
Unlike in some chronic conditions that are monitored during pregnancy, such as hypertensive disorders, fetal death from cholestasis tends to be harder to predict, Dr Friedman said.
It’s not entirely clear why some babies die and others don’t, but stillbirths are generally thought to result from sudden stress to the baby’s cells, Dr Friedman said, “akin to disconnecting the power cord to a machine.”
“Luckily, that outcome is rare,” he said. “There’s still a roughly 95 per cent chance the baby is going to be fine.”
The origins of the disease remain a mystery. And the prevalence of cholestasis can vary widely — in the United States, studies suggest it ranges from 0.32 to 5.6 percent of the population depending on one’s ethnicity and genetic makeup. It occurs more frequently among Latina women in the United States, as well as those from some Scandinavian, South Asian and South American countries.
Cholestasis is typically characterized by intense itching on the palms and soles of the feet, dark urine, pain in the upper right quadrant of the abdomen and elevated liver enzymes and bile acid levels. But not all women experience these symptoms.
Tamez never showed elevated liver enzymes, though her bile acid levels were very high. And although Strand is of Swedish descent and had a twin pregnancy, which both raised her risk of contracting the disease, she never had dark urine or abdominal pain and her itching wasn’t localized to her hands or feet.
Cholestasis research is still an emerging field, but Dr Mays has been examining it for decades after his hospital had three stillbirths over the course of two years in the early 2000s. Over the last 15 years, he has compiled a database of more than 500 women diagnosed with the disease at his hospital in East Harlem, which serves a large population of Latinas.
“We are averaging between 30 and 40 patients per year with the diagnosis,” said Dr Mays, whose protocol requires asking women at 28 weeks if they’re having any itching, then proactively testing women for cholestasis if they develop symptoms. By testing and delivering the babies as soon as their lungs are mature, usually around 37 weeks, Dr Mays said he has reduced infant mortality in his hospital by fivefold.
Strand is pregnant again and in her second trimester. She was diagnosed with cholestasis again, which is common for people who have had it before. This time around she is seeing a liver specialist, who prescribed ursodeoxycholic acid to lower her bile acid levels and recommended the over-the-counter supplement SAMe (S-adenosyl-L-methionine) to control the itching.
“I’m not scratching in my sleep anymore,” she said.
She advised women who suspect cholestasis to alert their doctors quickly.
“I just kind of wanted to be a good patient and be tough through all this,” she said of her first pregnancy, “so I didn’t really push as hard as I should have.”
“Itching During Pregnancy? It Might Signal a Bigger Problem” by Christina Caron © 2020 The New York Times Company
Christina Caron is a parenting reporter at The New York Times.
This story was originally published on 17 April 2020 in NYT Parenting.