Sapropterin Dihydrochloride, Oral

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KEY POINTS

• This medicine is taken by mouth to treat phenylketonuria. It is used along with a special diet. Take it exactly as directed.
• Keep all appointments for tests to see how this medicine affects you.
• This medicine may cause unwanted side effects. Tell your healthcare provider if you have any side effects that are serious, continue, or get worse.
• Tell all healthcare providers who treat you about all the prescription medicines, nonprescription medicines, supplements, natural remedies, and vitamins that you take.

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What are other names for this medicine?

Type of medicine: enzyme cofactor

Generic and brand names: sapropterin dihydrochloride, oral; Kuvan

What is this medicine used for?

This medicine is taken by mouth to treat phenylketonuria (PKU). It is used along with a special diet.

This medicine may be used to treat other conditions as determined by your healthcare provider.

What should my healthcare provider know before I take this medicine?

Before taking this medicine, tell your healthcare provider if you have ever had:

• An allergic reaction to any medicine
• Anorexia or other eating disorder
• Liver or kidney problems

Also tell your provider if you have a fever or infection of any kind.

Females of childbearing age: Talk with your healthcare provider if you are pregnant or plan to become pregnant. It is not known whether this medicine will harm an unborn baby. Do not breast-feed while taking this medicine without your healthcare provider's approval.

How do I take it?

Check the label on the medicine for directions about your specific dose. Take this medicine exactly as your healthcare provider prescribes. Do not take more or less than prescribed. Do not stop taking this medicine without your healthcare provider's approval. Your dose of this medicine may need to be changed several times to find what works best for you.

Check with your healthcare provider before using this medicine in children under age 1 month.

This medicine is usually taken one time a day. Take this medicine with a meal, at the same time each day.

This medicine comes as a tablet or in a packet that contain a powder for an oral solution. You may swallow the tablets whole or dissolve them in 4 to 8 ounces of water or apple juice. Take the mixture within 15 minutes of mixing. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, you can stir or crush them. If you still see small pieces of the tablet after you drink the medicine, add more water or apple juice and drink it to make sure that you take all of your medicine. You may also crush the tablets and mix in a small amount of soft food, such as apple sauce or pudding before taking.

You may dissolve the powder for oral solution in 4 to 8 ounces water, apple juice, applesauce, or pudding. Mix thoroughly. Take the mixture within 30 minutes after mixing.

What if I miss a dose?

If you miss a dose, take it as soon as you remember unless it is almost time for the next scheduled dose. In that case, skip the missed dose and take the next one as directed. Do not take double doses. Do not take 2 doses in one day. If you are not sure of what to do if you miss a dose, or if you miss more than one dose, contact your healthcare provider.

What if I overdose?

If you or anyone else has intentionally taken too much of this medicine, call 911 or go to the emergency room right away. If you pass out, have seizures, weakness or confusion, or have trouble breathing, call 911. If you think that you or anyone else may have taken too much of this medicine, call the poison control center. Do this even if there are no signs of discomfort or poisoning. The poison control center number is 800-222-1222.

Symptoms of an acute overdose may include: headache, dizziness, stomach pain.

What should I watch out for?

While taking this medicine, it is important to follow your provider's instructions about diet. Do not make changes in your diet unless your healthcare provider approves.

You will need to have blood tests regularly to see how this medicine affects you. Keep all appointments for these tests.

If you have a fever, or if you are sick, your healthcare provider may change your dose of this medicine. Talk with your provider about this.

If you need emergency care, surgery, lab tests, or dental work, tell the healthcare provider or dentist you are taking this medicine.

What are the possible side effects?

Along with its needed effects, your medicine may cause some unwanted side effects. Some side effects may be very serious. Some side effects may go away as your body adjusts to the medicine. Tell your healthcare provider if you have any side effects that continue or get worse.

Life-threatening (Report these to your healthcare provider right away. If you cannot reach your healthcare provider right away, get emergency medical care or call 911 for help.): Allergic reaction (hives; itching; rash; trouble breathing; chest pain or tightness in your chest; swelling of your lips, tongue, and throat).

Serious (Report these to your healthcare provider right away.): Severe stomach pain, nausea, or vomiting; flushing; severe dizziness or fainting; blood in your bowel movements or vomit; black or tarry bowel movements restlessness; chest pain; seizures; irritability; trouble breathing.

Other: Headache, diarrhea, mild nausea or vomiting, mild stomach pain, sore throat, runny or stuffy nose.

What products might interact with this medicine?

When you take this medicine with other medicines, it can change the way this or any of the other medicines work. Nonprescription medicines, vitamins, natural remedies, and certain foods may also interact. Using these products together might cause harmful side effects. Talk to your healthcare provider if you are taking:

• Erectile dysfunction medicines such as avanafil (Stendra), sildenafil (Viagra), tadalafil (Cialis), and vardenafil (Levitra, Staxyn)
• Levodopa/carbidopa (Duopa, Rytary, Sinemet)
• Medicines to treat pulmonary hypertension such as sildenafil (Revatio) and tadalafil (Adcirca)
• Methotrexate (Otrexup, Rasuvo, Rheumatrex, Trexall)
• Pralatrexate (Folotyn)

It is very important to follow the diet prescribed by your healthcare provider.

If you are not sure if your medicines might interact, ask your pharmacist or healthcare provider. Keep a list of all your medicines with you. List all the prescription medicines, nonprescription medicines, supplements, natural remedies, and vitamins that you take. Be sure that you tell all healthcare providers who treat you about all the products you are taking.

How should I store this medicine?

Store this medicine at room temperature. Keep the container tightly closed. Do not open the powder packet until you are ready to use it. Protect it from heat, high humidity, and bright light.

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This advisory includes selected information only and may not include all side effects of this medicine or interactions with other medicines. Ask your healthcare provider or pharmacist for more information or if you have any questions.

Ask your pharmacist for the best way to dispose of outdated medicine or medicine you have not used. Do not throw medicine in the trash.

Keep all medicines out of the reach of children.

Do not share medicines with other people.