Ctexli (chenodiol) is a bile acid medication that’s used to treat adults with a rare genetic condition called cerebrotendinous xanthomatosis (CTX). It replaces a missing bile acid in the body to help get rid of harmful substances that build up from the condition. The dose is taken by mouth three times daily, with or without food. Common side effects include diarrhea, headache, high blood pressure, and muscle weakness. Ctexli (chenodiol) also has a risk of liver damage, so look out for signs like yellowing of the skin or eyes, dark urine, or stomach pain.
Cerebrotendinous xanthomatosis (CTX) in adults
Ctexli (chenodiol) is a lab-made bile acid. It helps people with CTX who don’t make enough chenodiol, a natural bile acid. Without enough chenodiol, harmful substances can build up in the body and cause damage to many organs, like the brain and tendons.
Ctexli (chenodiol) gives the body extra chenodiol. This helps lower the amount of harmful substances that build up in people with CTX.
Source: FDA
Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.
Check with your doctor immediately if any of the following side effects occur:
Incidence not known
Black, tarry stools
chills
painful or difficult urination
shortness of breath
sores, ulcers, or white spots on the lips or in the mouth
swollen glands
unusual bleeding or bruising
unusual tiredness or weakness
Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:
More common
Less common
Abdominal or stomach pain
acid or sour stomach
belching
bloated
cramps
difficulty having a bowel movement (stool)
excess air or gas in the stomach or intestines
full feeling
indigestion
loss of appetite
pain in the chest below the breastbone
pain or discomfort in chest, upper stomach, or throat
passing gas
stomach discomfort or upset
Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.
Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.
The only FDA-approved treatment for CTX
Helps lower the level of harmful substances (like cholestanol) in people with CTX
May harm the liver
Need at least yearly blood tests to check your liver during treatment
Not approved for children, since we don’t know if it’s safe or if it works well for them
Take Ctexli (chenodiol) three times a day, with or without food. To help you remember, try taking each dose with your meals (breakfast, lunch, and dinner). Setting phone reminders or using a pill organizer with time slots can help you stay on schedule. This helps keep your condition under control.
Keep taking Ctexli (chenodiol) every day, even if you start to feel better. The medication helps keep the levels of harmful substances stay low in the body. Problems from CTX might come back if you stop taking it. Don’t stop taking Ctexli (chenodiol) unless your care team tells you to.
Make sure you swallow the Ctexli (chenodiol) tablet whole with water. If you have trouble swallowing pills, ask your prescriber or pharmacist about your options.
Avoid taking aluminum-based antacids with Ctexli (chenodiol). Also don’t take bile acid sequestrants at the same time. Examples include cholestyramine (Prevalite), colesevelam (Welchol), and colestipol (Colestid). These medications can prevent Ctexli (chenodiol) from working properly.
If you take warfarin (Coumadin), tell the care team managing your blood thinner that you’re also taking Ctexli (chenodiol). These medications can interact and raise your risk of bleeding. Once you start Ctexli (chenodiol), the team will check your INR more often to see if your warfarin dose will need to be adjusted.
Store Ctexli (chenodiol) at room temperature and keep it away from moisture and heat. Don’t keep it in the bathroom.
Ctexli (chenodiol) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Risk factors: History of liver problems | Bile duct problems | Trouble processing certain bile acids (lithocholic acid)
Ctexli (chenodiol) can damage the liver in some people. The risk might be higher if you already have liver or bile duct problems.
Before you start Ctexli (chenodiol), your prescriber will order blood tests to check your liver for any signs of stress or damage. These tests are repeated at least once a year, or more often if needed, to make sure your liver is healthy. If your liver enzyme and bilirubin levels are high, you may need to stop the medication until they return to normal. If these problems keep coming back, you may need to stop Ctexli (chenodiol) for good.
While taking Ctexli (chenodiol), watch out for warning signs that may mean your liver isn’t working properly. These include yellowing of the skin or eyes, dark urine, nausea, and tiredness. If you notice any of these symptoms, stop taking Ctexli (chenodiol) and call your care team right away. If your symptoms are severe or get worse quickly, go to the emergency room or call 911.
| Dosage | Quantity | Price as low as | Price per unit |
|---|---|---|---|
| 250mg | 90 tablets | $56023.54 | $622.48 |
The typical dose is 250 mg by mouth three times daily.
Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.
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