Two Lives, One Liver: Case Management at the Crossroads of Pregnancy and Liver Disease

INTRODUCTION

Pregnancy is a time of hope and anticipation. For some women, however, it can also bring unexpected and life-threatening complications. These complications need immediate recognition, coordinated interventions, and compassionate care. Complications can range from the persistent itching of Intrahepatic cholestasis or severe nausea and vomiting to the rapid deterioration seen in acute fatty liver of pregnancy or HELLP syndrome (Hemolysis, Elevated Liver enzymes, and Low Platelets). Every decision impacts not one life, but two. The stakes could not be higher. As case managers, these complex situations require navigating complex scenarios. Seamless multidisciplinary collaboration and advocacy that safeguards both mother and child are essential.

PREGNANCY-SPECIFIC LIVER DISORDERS

Hyperemesis gravidarum (HG) is a severe form of nausea and vomiting in pregnancy that goes beyond the “morning sickness” experienced by many women. It affects about 0.3–2% of pregnancies and typically presents in the first trimester (before 16 weeks). But symptoms may persist longer in some women. The exact cause is not fully understood, but it is thought to be related to elevated human chorionic gonadotropin (hCG) and estrogen levels, gastrointestinal motility changes in pregnancy, or possible association with Helicobacter pylori infection. Genetic and psychosocial factors may also contribute. Symptoms include inability to tolerate oral intake (food or fluids), weight loss >5% of body weight, dehydration (dry mucous membranes, tachycardia, hypotension), electrolyte abnormalities (hypokalemia, hyponatremia, metabolic alkalosis), mild elevation of liver enzymes (AST/ALT) with hyperbilirubinemia. Rare complications include Wernicke encephalopathy from thiamine deficiency (can lead to severe brain damage), Mallory-Weiss tears (tears in the esophagus caused by severe vomiting or retching which can lead to internal bleeding), or acute kidney injury. It is important to rule out other causes of nausea and vomiting in pregnancy. Other causes could be gastrointestinal disorders (hepatitis, gallstones, pancreatitis), urinary tract infections, thyroid disease (transient hyperthyroidism of hyperemesis), or pregnancy-related liver diseases (acute fatty liver of pregnancy, HELLP syndrome).

Hospitalization is necessary for nutritional support, correction of electrolyte imbalances, and IV fluids. Thiamine may be administered to prevent Wernicke encephalopathy. Psychological support is important, as HG can cause significant distress and isolation. Most women improve after the first trimester, but some women experience symptoms throughout pregnancy. With proper treatment, maternal and fetal outcomes are generally good. If untreated, HG can lead to maternal complications (malnutrition, electrolyte imbalances) and adverse fetal outcomes (low birth weight, preterm birth, small for gestational age). Hyperemesis gravidarum requires a multidisciplinary approach. Early recognition and aggressive management of dehydration, electrolytes, and nutritional needs are essential to prevent complications for both mother and baby.

Intrahepatic cholestasis of pregnancy (ICP) typically emerges in the second or third trimester when hormone levels are the highest. It is a pregnancy-specific liver disorder that affects about 0.2-2% of pregnancies. Fetal risks include stillbirth, preterm delivery, and meconium-stained amniotic fluid. Maternal prognosis is generally better. ICP often resolves spontaneously after delivery. Causes are varied. Elevated estrogen and progesterone can impair bile flow. Impaired bile acid transport leads to bile acid accumulation in the maternal serum and tissues. This causes intense itching, especially on the palms and soles which is worse at night, and sometimes mild jaundice occurs. ICP can be distinguished from dermatologic conditions as there is absence of a rash. Dark urine and pale stools may occasionally occur. Fatigue and right upper quadrant discomfort are less common symptoms. There may be genetic predisposition as well. Family history and certain ethnic groups (women of Chilean or Scandinavian descent) have a higher risk. Environmental factors also play a role. Diet and environmental changes can be triggers. Maternal prognosis is generally good as ICP resolves within days after delivery. There is some increased risk of gallstone disease and later hepatobiliary disorders. The risk of stillbirth increases significantly when the bile acids increase. Other possible complications include preterm labor and delivery, meconium-stained amniotic fluid, fetal distress, and sudden intrauterine demise. These complications sometimes occur without warning signs. Close fetal surveillance is essential. Recurrence rate in future pregnancies is 45–70%. There is no long-term maternal liver damage in most cases, although risk of later hepatobiliary disease is slightly increased.

ICP is benign for the mother, but poses serious risks for the fetus. Early recognition, medication management, close monitoring, and delivery planning are essential to reduce adverse outcomes.

Acute fatty liver (AFLP) is rare but potentially fatal. It is a potentially life-threatening obstetric emergency which typically occurs in the third trimester but may also appear in the early postpartum period. Symptoms include nausea, jaundice, hypoglycemia, and coagulopathy. Mother mortality rate can be as high as 18%, while fetal mortality rate can reach 23%. Treatment is immediate delivery. The disorder is associated with an inherited defect in fetal mitochondrial fatty acid oxidation. The maternal liver, already under metabolic stress in late pregnancy, cannot adequately process the metabolites which accumulate and lead to hepatocellular dysfunction. The result is impaired liver function, hypoglycemia, and metabolic decompensation. AFLP occurs more often in male fetuses, when there is a history of AFLP in a prior pregnancy, or familial fatty acid oxidation disorders. Symptoms are often nonspecific at onset and may mimic pre-eclampsia or HELLP syndrome. Symptoms may include nausea, vomiting, anorexia, malaise, epigastric or right upper quadrant abdominal pain. The symptoms typically progress rapidly to include jaundice, pruritus, polydipsia/polyuria from diabetes insipidus, confusion, or encephalopathy. Signs of severe disease include hypoglycemia, ascites, coagulopathy with bleeding, or renal dysfunction. Maternal complications include hepatic failure, disseminated intravascular coagulation (DIC), renal failure, sepsis, hypoglycemia, and hemorrhage. Fetal complications include intrauterine death, prematurity, hypoglycemia, and metabolic disorders. Maternal mortality is 2–18% and fetal mortality is 7–23%. Immediate delivery once the mother is stabilized is necessary regardless of gestational age. With prompt diagnosis and delivery, survival can improve dramatically. Liver function typically improves within days after delivery, but complications can persist. Most women have complete recovery of liver function. Both mother and infant should have genetic counseling to be evaluated for fatty acid oxidation disorders. Recurrence in future pregnancies is possible but rare, unless the fetus again carries a fatty acid oxidation disorder. Long-term maternal health outcomes are generally favorable, but infants with inherited fatty acid oxidation defects may require lifelong management.

CASE STUDY

A 30 year-old woman, gravida 3 para 1, presented at 34 weeks’ gestation with intense itching on her palms and soles. Routine labs showed mildly elevated liver enzymes, and her bile acid test returned significantly above normal limits. Recognizing the potential seriousness, the case manager immediately coordinated a hepatology consultation, arranged for weekly fetal surveillance, and educated the patient about warning signs to watch for at home. At 37 weeks, induction was initiated, and she delivered a healthy infant. Her symptoms resolved within days postpartum. Without early recognition and rapid coordination, the outcome for both mother and child could have been very different.

HELLP syndrome is characterized by Hemolysis, Elevated Liver enzymes, and Low Platelets. HELLP develops in 0.5-0.9% of pregnancies. It often occurs in the third trimester and is associated with severe preeclampsia, but it can occur within 48 hours of delivery. Risk factors include maternal age over 25 years, multiparity, Caucasian race, history of preeclampsia or HELLP in prior pregnancy, and chronic hypertension. The onset of HELLP is often insidious and nonspecific. Common symptoms include right upper quadrant or epigastric pain (secondary to liver swelling or subcapsular hematoma), nausea and vomiting, malaise, fatigue, headache, visual disturbances (less common but overlap with preeclampsia), hypertension, and proteinuria. Maternal complications include disseminated intravascular coagulation (DIC), placental abruption, acute renal failure, pulmonary edema, subcapsular liver hematoma or rupture (rare but catastrophic), stroke, or intracerebral hemorrhage. Maternal mortality is estimated at 1–3%. Fetal risks include preterm delivery, intrauterine growth restriction (IUGR), and placental abruption. Perinatal mortality rate is 7–20% which is largely due to prematurity and placental insufficiency. Delivery is recommended if the pregnancy is at least 34 weeks or if maternal/fetal condition is deteriorating. Most women recover fully after delivery with lab abnormalities resolving within days. Recurrence risk in subsequent pregnancies is 3–27% Recurrence rate is higher if HELLP occurred early in gestation. Women with HELLP or severe preeclampsia have an increased lifetime risk of cardiovascular disease.

Some women enter pregnancy with chronic liver disease such as hepatitis B or C, autoimmune hepatitis, cirrhosis, or Wilson disease. In hepatitis B, maternal management includes screening early in pregnancy, monitoring viral load, initiating antiviral therapy when indicated, and ensuring the newborn receives hepatitis B immunoglobulin and vaccination within 12 hours of birth. Autoimmune hepatitis may require careful adjustment of immunosuppressive therapy to maintain maternal disease control while reducing fetal risk.

ROLE OF THE CASE MANAGER

Case managers stand at the intersection of science, advocacy, and compassion. Early identification is vital, and case managers are often the first ones to recognize the symptoms or abnormal blood values. Psychosocial support is essential, as high-risk pregnancies involving liver disease can provoke anxiety, uncertainty, and grief. Case managers link families with counseling, support groups, and community resources. Coordinating multidisciplinary care for the mother and fetus is a vital case management intervention. Other essential case management interventions include coordinating frequent prenatal visits and maternal-fetal medicine consultations, facilitating adherence to medications, monitoring for complications, arranging fetal surveillance, and helping plan timely delivery. By providing education and psychosocial support, the case manager can help the mother manage anxiety related to fetal risks. In the postpartum period, case managers facilitate follow-up of maternal and infant recovery, manage any ongoing chronic liver conditions, and confirm that infants receive appropriate vaccinations and developmental surveillance.

Case managers play an important role in assisting with SDoH. Women in rural or underserved regions may have limited access to a liver specialist, obstetrician, and prenatal care. This can significantly impact timely diagnosis and positive outcomes. The case manager may be their only link to securing specialized care. Transportation to a major healthcare center to receive specialized care may be another issue that case managers need to assess and address. Language barriers may prevent pregnant mothers from reporting complications or symptoms which can lead to unwanted outcomes. By providing personalized, compassionate care, case managers can address many perinatal issues and positively influence outcomes. Advocating for the mother and fetus is a major part of the case manager’s role.

SUMMARY

Pregnancy-specific liver disorders remind us that the journey to motherhood is not always straightforward. What begins as excitement and hope can suddenly shift into a race against time when symptoms like itching, jaundice, severe vomiting, or unexplained pain point to potentially life-threatening complications. These conditions pose unique risks for both mother and fetus and need early recognition, rapid intervention, and seamless collaboration across disciplines.

At the crossroads of pregnancy and liver disease, case managers safeguard two lives simultaneously, ensuring that interventions are timely, compassionate, and patient-centered. The challenge lies not only in coordinating complex medical care, but also in providing education, advocacy, and psychosocial support. The ability to bridge clinical expertise with advocacy underscores the indispensable role of case management in improving maternal and fetal outcomes. Case management can mean the difference between tragedy and survival, fear and reassurance, isolation and support.

Image credit: ISTOCK.COM/MI-VIRI