Talking to your doctor about NAGS deficiency - hero banner image
Talking to your doctor about NAGS deficiency - image

When and how much CARBAGLU should I take?

PROPIONIC ACIDEMIA OR METHYLMALONIC ACIDEMIA

Treatment for acute high blood ammonia levels (all ages):

  • The recommended dose for acute high blood ammonia levels in people with PA or MMA is 150 mg/kg/day for children weighing 33 pounds or less. The dose for people weighing more than 33 pounds is based on body surface area.
  • If you have problems with your kidneys, your doctor may adjust your Carbaglu dosage.
  • Your doctor will divide your or your child’s daily dose into 2 equal doses to be taken 12 hours apart.
  • Your doctor will administer other therapies in addition to CARBAGLU to lower your or your child’s high blood ammonia levels.

NAGS DEFICIENCY

A doctor who is experienced in treating metabolic disorders will start your or your child’s CARBAGLU treatment.

Treatment for acute high blood ammonia levels for NAGS deficiency (all ages):

  • The recommended dose range for acute high blood ammonia levels in people with NAGS deficiency is 100 mg/kg/ day to 250 mg/kg/day.
  • If you have problems with your kidneys, your doctor may adjust your Carbaglu dosage.
  • Your doctor will divide your or your child’s daily dose into 2 to 4 does to be taken immediately before meals or feedings.
  • Your doctor will administer other therapies in addition to CARBAGLU to lower your or your child’s high blood ammonia levels.

Maintenance therapy for NAGS deficiency (all ages):

  • The recommended dose range for maintenance therapy for chronic high blood ammonia levels is 10 mg/kg/day to 100 mg/kg/day.
  • If you have problems with your kidneys, your doctor may adjust your Carbaglu dosage.
  • Your doctor will divide your or your child’s daily dose into 2 to 4 doses to be taken immediately before meals or feedings.
  • Your doctor may also administer other therapies or a reduced protein diet to lower your or your child’s high blood ammonia levels.
  • Your doctor will adjust your or your child’s dose of CARBAGLU to target a normal blood ammonia level for your or your child’s age.

The goal of therapy is to reduce your high blood ammonia level to a level that is normal for your age. It’s very important to take CARBAGLU exactly as your doctor has prescribed to maintain a constant level of medicine in your body. This will prevent ammonia from building up in your blood and will help your doctor know if you are on the right dose of CARBAGLU.

How should I take CARBAGLU for chronic high blood ammonia due to NAGS deficiency?

Important Information:

  • CARBAGLU tablet for oral suspension must be mixed in water before taking. CARBAGLU tablets should not be mixed in any other food or liquid.
  • Do not swallow CARBAGLU tablets whole.
  • Do not crush CARBAGLU tablets.
  • Take CARBAGLU right before meals or feedings.
  • The CARBAGLU tablet and water mixture has a slightly sour taste.
  • You may need to ask your healthcare provider or pharmacist for a medicine cup to measure the correct amount of water you will need to prepare the dose of CARBAGLU.
  • The CARBAGLU tablet has 3 lines used for splitting the tablet into 4 equal parts in order to get the prescribed dose. Ask your healthcare provider if you have any questions about how to split the tablet the right way or have any questions about the prescribed dose.


There are three different ways to take CARBAGLU for chronic high blood ammonia due to NAGS deficiency based on patient age and condition:

By mouth using a cup

How to take Carbaglu ® - graphic
Children and Adults
  1. Add at least 2.5 mL of water into a small cup for each CARBAGLU tablet, or each ½ or ¼ CARBAGLU tablet, needed for the prescribed dose. For example:
    • If the prescribed dose is 2 CARBAGLU tablets, add at least 5 mL of water into the cup.
    • If the prescribed dose is 2 and a ¼ CARBAGLU tablets, add at least 7.5 mL of water into the cup.
    • If the prescribed dose is 2 and a ½ CARBAGLU tablets, add at least 7.5 mL of water into the cup.
    Ask your healthcare provider if you are not sure of how much water you should use for the prescribed dose of CARBAGLU.
  2. Place the prescribed number of CARBAGLU tablets into the water in the cup.
  3. Carefully stir the CARBAGLU tablet and water mixture in the cup to avoid spilling the mixture. CARBAGLU tablets do not dissolve completely in water.
  4. Swallow the CARBAGLU tablet and water mixture right away.
  5. Pieces of the tablet may remain in the cup. Add more water to the cup to rinse the cup and swallow the mixture right away.
  6. Repeat step 5 until there are no pieces of the tablet left in the cup.

Follow the directions above or download a guide on how to use and store CARBAGLU.

How to use and store Carbaglu ® - image

Download in English

How to use and store Carbaglu ® - image

Download in Spanish

By mouth using an oral syringe

Children
  1. Add at least 2.5 mL of water into a small cup for each CARBAGLU tablet, or each ½ or ¼ CARBAGLU tablet, needed for the prescribed dose. For example:
    • If the prescribed dose is 2 CARBAGLU tablets, add at least 5 mL of water into the cup.
    • If the prescribed dose is 2 and a ¼ CARBAGLU tablets, add at least 7.5 mL of water into the cup.
    • If the prescribed dose is 2 and a ½ CARBAGLU tablets, add at least 7.5 mL of water into the cup.
    Ask your healthcare provider if you are not sure of how much water you should use for the prescribed dose of CARBAGLU.
  2. Place the prescribed number of CARBAGLU tablets into the water in the cup.
  3. Carefully stir the CARBAGLU tablet and water mixture in the cup to avoid spilling the mixture. CARBAGLU tablets do not dissolve completely in water.
  4. Draw up all of the CARBAGLU tablet and water mixture in the cup into an oral syringe.
  5. Give your child the CARBAGLU tablet and water mixture right away by placing the tip of the oral syringe along the inner cheek of their mouth, on either the right or left side. Slowly push all the way down on the plunger to give the medicine.
  6. Pieces of the tablet may remain in the oral syringe. Refill the oral syringe with at least 1 mL to 2 mL of water, and give your child the mixture right away.
  7. Repeat step 6 until there are no pieces of the tablet left in the oral syringe.

Follow the directions above or download a guide on how to use and store CARBAGLU.

How to use and store Carbaglu ® - image

Download in English

How to use and store Carbaglu ® - image

Download in Spanish

Through a nasogastric (NG) tube or gastrostomy tube (G-tube)

How to take Carbaglu ® - graphic expanded
How to take Carbaglu ® - graphic expanded
Children and Adults
  1. Add at least 2.5 mL of water into a small cup for each CARBAGLU tablet, or each ½ or ¼ CARBAGLU tablet, needed for the prescribed dose. For example:
    • If the prescribed dose is 2 CARBAGLU tablets, add at least 5 mL of water into the cup.
    • If the prescribed dose is 2 and a ¼ CARBAGLU tablets, add at least 7.5 mL of water into the cup.
    • If the prescribed dose is 2 and a ½ CARBAGLU tablet, add at least 7.5 mL of water into the cup.
    Ask your healthcare provider if you are not sure of how much water you should use for the prescribed dose of CARBAGLU.
  2. Place the prescribed number of CARBAGLU tablets into the water in the cup.
  3. Carefully stir the CARBAGLU tablet and water mixture in the cup to avoid spilling the mixture. CARBAGLU tablets do not dissolve completely in water.
  4. Draw up all of the CARBAGLU tablet and water mixture in the cup into a catheter-tip syringe.
  5. Connect the catheter-tip syringe to the NG tube or G-tube.
  6. Give the CARBAGLU tablet and water mixture through the NG tube or G-tube right away.
  7. Pieces of the tablet may remain in the catheter-tip syringe or NG tube or G-tube.
  8. Refill the catheter-tip syringe with 1 mL to 2 mL of water and flush the NG tube or G-tube right away.
  9. Repeat step 8 until there are no pieces of the tablet left in the catheter-tip syringe or NG tube or G-tube.

Follow the directions above or download a guide on how to use and store CARBAGLU.

How to use and store Carbaglu ® - image

Download in English

How to use and store Carbaglu ® - image

Download in Spanish

How should I store CARBAGLU?

  • Before opening, store CARBAGLU in a refrigerator between 36°F to 46°F (2°C to 8°C) in the container it comes in.

  • After opening, store CARBAGLU at room temperature between 59°F to 86°F (15°C to 30°C). Do not store opened CARBAGLU in a refrigerator.

  • Keep CARBAGLU tablets in a tightly closed container to protect the tablets from moisture.

  • Write the date the CARBAGLU tablet container is opened on the container label. Throw away any unused tablets one month after opening the tablet container. Do not use CARBAGLU tablets after the expiration date on the tablet container.

    Keep CARBAGLU and all medicines out of the reach of children.

How do I get my CARBAGLU prescription for chronic NAGS deficiency?

You cannot get CARBAGLU from a local retail pharmacy. Instead, your doctor will fax your CARBAGLU prescription and other information to a specialty pharmacy called Accredo.

  • Once your prescription is verified and all paperwork is completed, Accredo will mail your CARBAGLU medication to your home in a temperature-controlled container.

  • This initial process may take some time, depending on your insurance coverage and other factors. Representatives at Accredo are committed to helping you through the process so that you can get your medicine as quickly as possible.

  • Accredo will also send prescription refills to your home. It is very important that you return all phone calls from your doctor and from Accredo to make sure you receive your CARBAGLU refills when you need them.

  • It’s very important to take CARBAGLU exactly as your doctor has prescribed to maintain a constant level of medicine in your body. This will prevent ammonia from building up in your blood.

     

    For more information about Accredo, visit www.accredo.com or call 1-888-454-8860.

  • Treatment with CARBAGLU

    If taken correctly, as prescribed by your doctor, CARBAGLU will help return your high ammonia levels to normal.

    Learn more about CARBAGLU

  • Patient Support

    Get access to financial and other support for CARBAGLU.

    Access CARBAGLU resources

INDICATIONS AND USAGE

Carbaglu® (carglumic acid) tablets for oral suspension 200mg is a prescription drug used in all ages to help treat the following:

  • CARBAGLU is used to supplement standard of care treatment for acute high blood ammonia levels (hyperammonemia) due to the lack or shortage of NAGS enzyme activity.
  • CARBAGLU is used to maintain normal blood ammonia levels due to the lack or shortage of NAGS enzyme activity.
  • CARBAGLU is used to supplement standard of care treatment for acute high blood ammonia levels due to propionic acidemia (PA) or methylmalonic acidemia (MMA).

IMPORTANT SAFETY INFORMATION

  • Contraindications: None.
  • NAGS deficiency: Most common side effects in ≥13% of patients are: vomiting, abdominal pain, fever, inflammation of the tonsils, reduced red cells in the blood, diarrhea, ear infection, infections, inflammation of the throat and nasal passages, hemoglobin decreased in red blood cells, and headache.
  • PA and MMA: Most common side effects in ≥5% of patients are: lower than normal white blood cells, reduced red cells in the blood, vomiting, abnormal levels of minerals in the body, decreased appetite, low blood sugar levels, lack of energy/unresponsiveness, brain disease that alters brain structure or function, and inflammation of the pancreas/increase in a type of protein made by the pancreas that helps the body digest fats.
  • To report SUSPECTED SIDE EFFECTS, contact Recordati Rare Diseases Inc. at 1-888-575-8344, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
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