How to Take RAVICTI

Voiceover:

Welcome to the RAVICTI® (glycerol phenylbutyrate) Oral Liquid instructional dosing video.

In this video you will see step-by-step instructions for the oral administration of RAVICTI for you or your child.

Approved Uses for RAVICTI® (glycerol phenylbutyrate) Oral Liquid
RAVICTI is a prescription medicine used in adults and children 2 months of age and older for long-term management of high levels of ammonia in the blood (hyperammonemia) caused by a condition called a urea cycle disorder (UCD). RAVICTI must be used along with a low-protein diet and, in some cases, dietary supplements. RAVICTI should only be used if the UCD cannot be managed with a low-protein diet and dietary supplements alone.

RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crises) in people with UCDs as they may require rapidly acting medication.
It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.
It is not known if RAVICTI is safe and effective for children younger than 2 months.

This video is intended to complement the instructions you have already received from your healthcare provider. Always take RAVICTI exactly as directed by your doctor.

Do not mix RAVICTI with formula or any other liquids.

Now, let's get started.

Your RAVICTI shipment includes: a 25-mL bottle of RAVICTI, a blue AdaptaCapTM Bottle Adapter, and an oral dosing syringe.

When dosing RAVICTI, be sure to use the oral dosing syringe provided with the product.

First, remove the childproof cap on the RAVICTI bottle. To do that, push down on the cap while twisting it to the left.

Then, attach the blue AdaptaCap™ Bottle Adapter onto the bottle. Put it on top of the bottle and twist it to the right.

Make sure it is secured tightly on the bottle.

While holding the RAVICTI bottle securely, place the tip of the oral dosing syringe into the AdaptaCapTM Bottle Adapter.

Turn the bottle upside down with the oral dosing syringe still inserted.

Now, you're ready to fill the oral dosing syringe with RAVICTI.

Get the medicine into the oral dosing syringe by pulling the plunger back slowly. Pulling slowly will help avoid large air bubbles from forming in the medicine.

Pull the plunger back until the first line of the black rubber stopper is even with the dosing mark. The dosing marks are on the barrel of the oral dosing syringe. Match the dosing mark with the amount of medicine prescribed by your doctor.

Then, turn the bottle upright again.

If there are large air bubbles, push the medicine back into the bottle. Do this slowly.

Then, draw up the medicine again.

Once you have your oral dosing syringe ready, place it into the mouth. Push the plunger to squirt the medicine directly into the mouth.

Make sure all of the medicine is pushed out of the oral dosing syringe and swallowed.

Put the cap on the oral dosing syringe.

Drink liquid or eat some food right after taking RAVICTI.

This helps ensure that all of the medicine is swallowed.

Close the tab on the AdaptaCap™ Bottle Adapter.

Make sure it's closed tightly.

You can use 1 oral dosing syringe and 1 blue AdaptaCap™ Bottle Adapter for all doses each day. You can also choose to use a new oral dosing syringe with each dose. Do not rinse the AdaptaCap™ Bottle Adapter or oral dosing syringe between doses.

After the last dose of the day, dispose of the oral dosing syringe.

If you take too much RAVICTI, call your doctor or go to the nearest hospital emergency room right away.

You should consult your physician if you have missed a dose of RAVICTI.

Do not mix RAVICTI with formula or any other liquids.

Detailed Important Safety Information

Who should not take RAVICTI:
Children younger than 2 months should not take RAVICTI because they may not be able to digest it. Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have symptoms of an allergic reaction, such as wheezing, shortness of breath, cough, low blood pressure, flushing, nausea, or rash while taking RAVICTI.

RAVICTI may cause serious side effects:
The breakdown of RAVICTI produces the byproduct phenylacetate, which may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI: sleepiness; lightheadedness; change in taste; problems with hearing; confusion; problems with memory; worsening of numbness, tingling, or burning in your hands or feet; headache, tiredness; nausea; or vomiting.

What are the possible side effects of RAVICTI?
The most common side effects of RAVICTI in adults include diarrhea, gas, headache, nausea, vomiting, tiredness, decreased appetite, and dizziness.

The most common side effects of RAVICTI in children ages 2 to 17 years include stomach pain, nausea, vomiting, diarrhea, decreased appetite, and headache.

The most common side effects of RAVICTI in children ages 2 months to younger than 2 years include decreased level of a type of white blood cell, vomiting, diarrhea, fever, decreased appetite, cough, nasal congestion, runny nose, and rash.

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects.

Before you take RAVICTI:
Tell your doctor if you have liver or kidney problems, pancreas or bowel (intestine) problems, or any other medical conditions. Tell your doctor if you are pregnant or plan to become pregnant. It is not known if RAVICTI will harm your unborn baby. Tell your doctor if you are breastfeeding or plan to breastfeed. It is not known if RAVICTI passes into your breast milk. Breastfeeding is not recommended during treatment with RAVICTI. Talk to your doctor about the best way to feed your baby if you take RAVICTI.

Talk to your doctor about participating in a UCD registry. The purpose of this registry is to collect information about people with UCDs to improve care. For more information about the registry program, call 1-855-823-2595 or visit www.ucdregistry.com.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

This information is not comprehensive. To learn more, talk to your healthcare provider or pharmacist. The FDA-approved product labeling, including the Medication Guide, can be found at Ravicti.com.

Voiceover:

Welcome to the RAVICTI® (glycerol phenylbutyrate) Oral Liquid instructional dosing video.

In this video, you will see step-by-step instructions for administration of RAVICTI if you or your child have a nasogastric or gastrostomy [ga-‘sträs-tə-mē] feeding tube.

If you can swallow liquids, it is recommended you take RAVICTI by mouth.

Approved Uses for RAVICTI® (glycerol phenylbutyrate) Oral Liquid
RAVICTI is a prescription medicine used in adults and children 2 months of age and older for long-term management of high levels of ammonia in the blood (hyperammonemia) caused by a condition called a urea cycle disorder (UCD). RAVICTI must be used along with a low-protein diet and, in some cases, dietary supplements. RAVICTI should only be used if the UCD cannot be managed with a low-protein diet and dietary supplements alone.

RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crises) in people with UCDs as they may require rapidly acting medication.
It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.
It is not known if RAVICTI is safe and effective for children younger than 2 months.

This video is intended to complement the instructions you have already received from your healthcare provider. Always take RAVICTI exactly as directed by your doctor.

Do not mix RAVICTI with formula or any other liquids.

Now, let's get started.

Your RAVICTI shipment includes:

a 25-mL bottle of RAVICTI, a blue AdaptaCapTM Bottle Adapter, and an oral dosing syringe.

When dosing RAVICTI, be sure to use the oral dosing syringe provided with the product.

First, remove the childproof cap on the RAVICTI bottle. To do that, push down on the cap while twisting it to the left.

Then, attach the blue AdaptaCapTM Bottle Adapter onto the bottle. Put it on top of the bottle and twist it to the right.

Make sure it is secured tightly on the bottle.

While holding the RAVICTI bottle securely, place the tip of the oral dosing syringe into the AdaptaCapTM Bottle Adapter.

Turn the bottle upside down with the oral dosing syringe still inserted.

Now, you're ready to fill the oral dosing syringe with RAVICTI.

Get the medicine into the oral dosing syringe by pulling the plunger back slowly. Pulling slowly will help avoid large air bubbles from forming in the medicine.

Pull the plunger back until the first line of the black rubber stopper is even with the dosing mark. The dosing marks are on the barrel of the oral dosing syringe. Match the dosing mark with the amount prescribed by your doctor.

Then, turn the bottle upright again.

If there are large air bubbles, push the medicine back into the bottle. Do this slowly.

Then, draw up the medicine again.

Next, place the tip of the oral dosing syringe into the smaller port of the feeding tube.

The smaller port of the nasogastric or gastrostomy feeding tube is used for medicine.

Ask your doctor if you’re not sure which port to use.

Using the plunger of the oral dosing syringe, slowly and carefully push RAVICTI into the feeding tube.

Make sure all of the medicine is pushed out of the oral dosing syringe.

After you have pushed all of the RAVICTI into the feeding tube, remove the oral dosing syringe, put the cap on, and set it aside.

Close the tab on the AdaptaCapTM Bottle Adapter.

Make sure it's closed tightly.

Use a separate large syringe to flush water into the feeding tube. A large syringe can be found in the box containing your feeding tube.

Make sure all of the water or formula drains through the feeding tube.

You can use 1 oral dosing syringe and 1 blue AdaptaCap™ Bottle Adapter for all doses each day. You can also choose to use a new oral dosing syringe with each dose. Do not rinse the AdaptaCap™ Bottle Adapter or oral dosing syringe between doses.

After the last dose of the day, dispose of the oral dosing syringe.

If you take too much RAVICTI, call your doctor or go to the nearest hospital emergency room right away.

You should consult your physician if you have missed a dose of RAVICTI.

Do not mix RAVICTI with formula or any other liquids.

Detailed Important Safety Information

Who should not take RAVICTI:
Children younger than 2 months should not take RAVICTI because they may not be able to digest it. Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have symptoms of an allergic reaction, such as wheezing, shortness of breath, cough, low blood pressure, flushing, nausea, or rash while taking RAVICTI.

RAVICTI may cause serious side effects:
The breakdown of RAVICTI produces the byproduct phenylacetate, which may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI: sleepiness; lightheadedness; change in taste; problems with hearing; confusion; problems with memory; worsening of numbness, tingling, or burning in your hands or feet; headache, tiredness; nausea; or vomiting.

What are the possible side effects of RAVICTI?
The most common side effects of RAVICTI in adults include diarrhea, gas, headache, nausea, vomiting, tiredness, decreased appetite, and dizziness.

The most common side effects of RAVICTI in children ages 2 to 17 years include stomach pain, nausea, vomiting, diarrhea, decreased appetite, and headache.

The most common side effects of RAVICTI in children ages 2 months to younger than 2 years include decreased level of a type of white blood cell, vomiting, diarrhea, fever, decreased appetite, cough, nasal congestion, runny nose, and rash.

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects.

Before you take RAVICTI:
Tell your doctor if you have liver or kidney problems, pancreas or bowel (intestine) problems, or any other medical conditions. Tell your doctor if you are pregnant or plan to become pregnant. It is not known if RAVICTI will harm your unborn baby. Tell your doctor if you are breastfeeding or plan to breastfeed. It is not known if RAVICTI passes into your breast milk. Breastfeeding is not recommended during treatment with RAVICTI. Talk to your doctor about the best way to feed your baby if you take RAVICTI.

Talk to your doctor about participating in a UCD registry. The purpose of this registry is to collect information about people with UCDs to improve care. For more information about the registry program, call 1-855-823-2595 or visit www.ucdregistry.com.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

This information is not comprehensive. To learn more, talk to your healthcare provider or pharmacist. The FDA-approved product labeling, including the Medication Guide, can be found at Ravicti.com.