Treatment Overview

Living with cholestasis can be an intensely frustrating and uncomfortable experience. The condition, defined by a reduction or stoppage of bile flow from the liver, is often characterized by a relentless, deep itch that no amount of scratching seems to relieve. This itching, known medically as pruritus, can disrupt sleep, ruin concentration, and severely impact a person’s quality of life. Beyond the physical discomfort, the accompanying fatigue and jaundice (yellowing of the skin or eyes) can be alarming.

Treatment is essential to not only alleviate this distress but also to prevent potential complications. When bile backs up, it can damage liver cells and interfere with the body’s ability to absorb essential fats and vitamins. Because cholestasis can arise from various causes, ranging from pregnancy and drug reactions to chronic liver diseases or physical blockages, treatment plans are highly specific. Medical management focuses on restoring bile flow and protecting the liver while keeping the patient comfortable (National Institute of Diabetes and Digestive and Kidney Diseases, 2023).

Overview of treatment options for Cholestasis

The therapeutic approach to cholestasis targets two main objectives: managing the underlying cause to restore bile flow and controlling the symptoms caused by the buildup of bile acids in the bloodstream. If the cholestasis is caused by a physical blockage, such as a gallstone, a procedure may be necessary. However, for metabolic or intrahepatic causes, medication is the primary form of treatment.

Physicians generally prioritize medications that modify the composition of bile to make it less toxic to the liver. Simultaneously, they employ specific drugs to target the itching, which is often the patient’s most immediate complaint. Clinical experience suggests that treating the itch aggressively early on is crucial for mental and physical well-being.

Medications used for Cholestasis

The cornerstone of treatment for many forms of cholestasis is ursodeoxycholic acid, commonly known as Ursodiol. This naturally occurring bile acid is the first-line therapy for conditions like Primary Biliary Cholangitis (PBC) and Intrahepatic Cholestasis of Pregnancy. It helps improve liver function tests and facilitates the movement of bile.

To combat the severe itching, doctors often prescribe bile acid sequestrants. Cholestyramine is a well-known medication in this class. It is a powder mixed with fluids that binds to bile acids in the intestine.

When first-line agents do not sufficiently control itching, second-line medications originally developed for other purposes may be used. Rifampin, an antibiotic, is frequently prescribed off-label for resistant pruritus. Opioid antagonists, such as naltrexone, are also utilized to block the sensation of itching in the brain. Additionally, sertraline, a type of antidepressant (SSRI), has shown efficacy in reducing itch intensity for some patients.

Because cholestasis prevents the absorption of dietary fats, patients are often prescribed supplements for fat-soluble vitamins (A, D, E, and K) to prevent deficiencies (Mayo Clinic, 2022).

How these medications work

Ursodiol works by replacing toxic, hydrophobic bile acids that harm liver cells with safer, hydrophilic ones. It also stimulates bile secretion to flush the system.

Bile acid sequestrants like cholestyramine act in the digestive tract, binding to bile acids to prevent reabsorption. This elimination via stool lowers circulating bile acid levels.

Rifampin enhances the liver’s ability to clear bile by activating detoxifying enzymes. Naltrexone blocks nervous system opioid receptors, thought to be involved in the itch sensation (pruritus) associated with liver disease (MedlinePlus, 2021).

Side effects and safety considerations

Ursodiol is generally well-tolerated, with mild side effects like diarrhea, back pain, or slight weight gain. Bile acid sequestrants such as cholestyramine can cause digestive issues (bloating, constipation, gritty taste) and must be taken hours apart from other medications as they block absorption.

Rifampin necessitates regular blood tests to monitor liver function due to the rare risk of liver stress. Naltrexone may trigger withdrawal-like symptoms in patients on opioid pain medications. Ursodiol is commonly used for pregnant women requiring specialized, baby-safe care. Patients should seek immediate care for worsening jaundice or signs of bleeding (possible Vitamin K deficiency).

Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.

References

  1. National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov
  2. Mayo Clinic. https://www.mayoclinic.org
  3. MedlinePlus. https://medlineplus.gov
  4. American Liver Foundation. https://liverfoundation.org

Medications for Cholestasis

These are drugs that have been approved by the US Food and Drug Administration (FDA), meaning they have been determined to be safe and effective for use in Cholestasis.

Found 5 Approved Drugs for Cholestasis

Ursodiol

Brand Names
Urso 250, Reltone, Urso

Ursodiol

Brand Names
Urso 250, Reltone, Urso
Form: Tablet, Capsule
Method of administration: Oral
FDA approval date: December 31, 1987
Classification: Bile Acid
Ursodiol capsules are indicated for patients with radiolucent, noncalcified gallbladder stones <20 mm in greatest diameter in whom elective cholecystectomy would be undertaken except for the presence of increased surgical risk due to systemic disease, advanced age, idiosyncratic reaction to general anesthesia, or for those patients who refuse surgery. Safety of use of ursodiol beyond 24 months is not established. Ursodiol capsules are indicated for the prevention of gallstone formation in obese patients experiencing rapid weight loss.

Cholestyramine

Brand Names
Choleystyramine, Prevalite, Questran

Cholestyramine

Brand Names
Choleystyramine, Prevalite, Questran
Form: Powder
Method of administration: Oral
FDA approval date: February 01, 1996
Classification: Bile Acid Sequestrant
1) Prevalite ® (cholestyramine for oral suspension, USP) powder is indicated as adjunctive therapy to diet for the reduction of elevated serum cholesterol in patients with primary hypercholesterolemia (elevated low density lipoprotein [LDL] cholesterol) who do not respond adequately to diet. Prevalite ® (cholestyramine for oral suspension, USP) powder may be useful to lower LDL cholesterol in patients who also have hypertriglyceridemia, but it is not indicated where hypertriglyceridemia is the abnormality of most concern. Therapy with lipid-altering agents should be a component of multiple risk factor intervention in those individuals at significantly increased risk for atherosclerotic vascular disease due to hypercholesterolemia. Treatment should begin and continue with dietary therapy specific for the type of hyperlipoproteinemia determined prior to initiation of drug therapy. Excess body weight may be an important factor and caloric restriction for weight normalization should be addressed prior to drug therapy in the overweight. Prior to initiating therapy with cholestyramine resin, secondary causes of hypercholesterolemia (e.g., poorly controlled diabetes mellitus, hypothyroidism, nephrotic syndrome, dysproteinemias, obstructive liver disease, other drug therapy, alcoholism), should be excluded and a lipid profile performed to assess Total Cholesterol, HDL-C and triglycerides (TG). For individuals with TG less than 400 mg/dL.

Bylvay

Generic Name
Odevixibat

Bylvay

Generic Name
Odevixibat
Form: Capsule
Method of administration: Oral
FDA approval date: July 20, 2021
Classification: Ileal Bile Acid Transporter Inhibitor
BYLVAY is an ileal bile acid transporter (IBAT) inhibitor indicated for: Progressive Familial Intrahepatic Cholestasis (PFIC) the treatment of pruritus in patients 3 months of age and older with progressive familial intrahepatic cholestasis (PFIC).

Livmarli

Generic Name
Maralixibat

Livmarli

Generic Name
Maralixibat
Form: Tablet, Solution
Method of administration: Oral
FDA approval date: September 29, 2021
Classification: Ileal Bile Acid Transporter Inhibitor
LIVMARLI is an ileal bile acid transporter (IBAT) inhibitor indicated for: the treatment of cholestatic pruritus in patients 3 months of age and older with Alagille syndrome (ALGS).

Syprine

Generic Name
Trientine

Syprine

Generic Name
Trientine
Form: Capsule
Method of administration: Oral
FDA approval date: November 08, 1985
Classification: Copper Chelator
Trientine hydrochloride is indicated in the treatment of patients with Wilson's disease who are intolerant of penicillamine. Clinical experience with trientine hydrochloride is limited and alternate dosing regimens have not been well-characterized; all endpoints in determining an individual patient's dose have not been well defined. Trientine hydrochloride and penicillamine cannot be considered interchangeable. Trientine hydrochloride should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects. Unlike penicillamine, trientine hydrochloride is not recommended in cystinuria or rheumatoid arthritis. The absence of a sulfhydryl moiety renders it incapable of binding cystine and, therefore, it is of no use in cystinuria. In 15 patients with rheumatoid arthritis, trientine hydrochloride was reported not to be effective in improving any clinical or biochemical parameter after 12 weeks of treatment. Trientine hydrochloride is not indicated for treatment of biliary cirrhosis.
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