The liver cells produce bile that passes through ducts in the liver and reaches the gallbladder. Bile plays an important role in proper digestion of fat and fat-soluble vitamins. In some people, the liver cannot produce bile or bile does not flow to the gallbladder and duodenum. This happens when you develop a condition called cholestatic liver disease, which is characterized by the degeneration of liver tissue, formation of scar tissue, and chronic inflammation. The effects of this condition can be quite serious, and if left untreated, it leads to hepatic insufficiency, cirrhosis, and liver transplantation.

Types of Cholestatic Liver Diseases

Primary Biliary Cirrhosis (PBC)

This refers to the impairment of intrahepatic bile ducts. This rare disease affects 40 out of every 100000 people, and women are at greater risk of developing this disease. Although it is called primary biliary cirrhosis, it is worth pointing out that cirrhosis only occurs in the later stages of the disease.

• Symptoms: The symptoms take time to develop, and a generalized fatigue is usually the very first symptom of PBC. You may also experience itching and pruritus. Many patients do not experience any symptoms at all, especially during the early stage. Later-stage symptoms may include darkening of the urine, yellowing of the skin and eyes, and collections of cholesterol around the eyes and in the skin.
• Causes: The real causes are still unknown, but experts associate it to some environmental and genetic factors. Experts are of the view that it is an autoimmune disease.
• Treatment: Your doctor will check the presence of anti-mitochondrial antibodies to make a diagnosis. Once confirmed, they will treat this cholestatic liver disease with ursodeoxycholic acid (UDCA), which is the only medication available and is effective in 60% of patients. The rest have a high risk of developing liver insufficiency and cirrhosis. They require liver transplantation.

Primary Sclerosing Cholangitis (PSC)

This affects extra and intra-hepatic bile ducts and causes inflammation in the bile ducts. This causes narrowing of the bile ducts, which leads to a buildup of bile in the liver. The buildup causes damage to liver cells and results in cancer or cirrhosis.

• Symptoms: Abdominal pain and pruritus are the earliest symptoms. Most patients stay asymptomatic during the early stage, but their liver function tests show abnormalities. Later-stage symptoms include right upper abdominal pain, jaundice, and chills and fever.
• Causes: Just like PBC, the causes of PSC are also unknown but it is considered an autoimmune disease that progresses very slowly.
• Treatments: Quite like PBC, UDCA treatment proves beneficial in most patients but there is no therapy available for PSC that reduces the need for liver transplantation in patients.

Cholestasis of Pregnancy

Cholestasis refers to a condition that affects the flow of bile from the liver. Many women develop this condition during pregnancy. Here is a bit more about this cholestatic liver disease.

Symptoms

The condition develops in late pregnancy and causes severe itching on the feet and hand. You may experience itching on other body parts as well, which becomes worse at night. Other symptoms associated with cholestasis of pregnancy include nausea, yellowing of the skin and eyes, and loss of appetite. While the condition can make you extremely uncomfortable, it does not pose any long-term risk to an expectant mother. It can be dangerous for a developing baby though and early delivery may be recommended in this case.

Treatments

Your doctor will give prescription medications and suggest other treatment options mainly to soothe intense itching. They may give you medication ursodiol that helps decrease the level of bile in your blood that in turn will relieve itching. It also reduces complications for a developing baby. Soaking itchy area in lukewarm water may also help.

Complications Preventions

Your doctor will monitor your pregnancy and growth of your baby quite closely because a cholestatic liver disease can cause serious complications for a growing baby. Your doctor may take different measures depending on your condition. For instance:

• They will order biophysical profile scores and non-stress tests to keep an eye on your baby's health. A non-stress test helps determine how many times your baby moves within a set amount of time. This also tells about an increase in their heart rate with movement. On the other hand, the biophysical profile provides complete information about fetal muscle tone, the volume of amniotic fluid, and fetal activity.
• They may suggest early induction of labor even when your prenatal tests do not show any abnormality. This happens if you are already 37 weeks pregnant. Early delivery is the best option to prevent fetal death.

Cholestasis in Newborns

The condition refers to the failure of bilirubin excretion that leads to a buildup of bilirubin in the blood. This also causes the level of bile salt to come down in the GI tract. With insufficient bile in the GI tract, it becomes difficult for your baby to absorb fat and fat-soluble vitamins. This may result in inadequate nutrition, vitamin deficiency, and growth failure.

Symptoms

This cholestatic liver disease affects infants during the first couple of weeks of their lives. Your infant will develop jaundice and have acholic stools, dark urine, and hepatomegaly. Chronic pruritus is also a common symptom and usually indicates a deficiency of fat-soluble vitamins. If there is an underlying disorder, it may result in dilated abdominal veins and ascites that may also lead to the development of portal hypertension. Esophageal varices may cause upper GI bleeding in some cases.

Treatments

The treatment depends on the underlying cause. Healthcare providers usually stick to nutritional therapy when there is no specific therapy available for the underlying cause. Nutritional therapy may include supplements of vitamins K, E, D, and A. Formula-fed infants may benefit from using a formula high in medium-chain triglycerides. Infants may also need more than 130 calories/kg a day. Ursodeoxycholic acid in doses of 10-15mg/kg once a day may also help relieve itching.