Sapropterin Dihydrochloride Oral tablet

SAPROPTERIN (SAP roe TER in) treats high phenylalanine levels in people with phenylketonuria (PKU). It works by helping your body break down phenylalanine. This prevents it from building up in the blood. Changes to diet are often combined with this medication.

Take this medication by mouth with food. Follow the directions on the prescription label. Tablets may be swallowed whole or you may dissolve tablets in 4 to 8 ounces (120 to 240 mL) of water or apple juice. Drink within 15 minutes of mixing. To make tablets dissolve faster, you may crush or stir them. Tablets may not dissolve completely, and you may see small pieces floating in the glass. If small pieces remain in the glass after taking the medication, add more water or apple juice to the glass and drink it to make sure you get all of the medication. Tablets may also be crushed and then mixed in a small amount of apple sauce or pudding. Take your medication at regular intervals. Do not take it more often than directed. Do not stop taking except on your care team's advice.

Talk to your care team about the use of this medication in children. While this medication may be prescribed for children as young as 1 month for selected conditions, precautions do apply.

Side effects that you should report to your care team as soon as possible:

• Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
• Hyperactivity—restlessness, fidgeting, excessive talking
• Inflammation of the esophagus or stomach—loss of appetite, throat pain, pain or trouble swallowing, heartburn, nausea, vomiting, dry cough, bloody or black, tar-like stools

Side effects that usually do not require medical attention (report to your care team if they continue or are bothersome):

• Cough
• Diarrhea
• Headache
• Runny or stuffy nose
• Sore throat
• Vomiting

Keep out of the reach of children and pets.

Store at room temperature between 20 and 25 degrees C (68 and 77 degrees F). Keep this medication in the original container. Keep the container tightly closed. Get rid of any unused medication after the expiration date.

To get rid of medications that are no longer needed or have expired:

• Take the medication to a medication take-back program. Check with your pharmacy or law enforcement to find a location.
• If you cannot return the medication, check the label or package insert to see if the medication should be thrown out in the garbage or flushed down the toilet. If you are not sure, ask your care team. If it is safe to put it in the trash, empty the medication out of the container. Mix the medication with cat litter, dirt, coffee grounds, or other unwanted substance. Seal the mixture in a bag or container. Put it in the trash.

They need to know if you have any of these conditions:

• Eating disorder, such as anorexia or bulimia
• Fever
• Infection
• Kidney disease
• Liver disease
• Seizures
• An unusual or allergic reaction to sapropterin, other medications, foods, dyes, or preservatives
• Pregnant or trying to get pregnant
• Breastfeeding

• Avanafil
• Levodopa
• Methotrexate
• Pemetrexed
• Pyrimethamine
• Sildenafil
• Tadalafil
• Vardenafil

If you miss a dose, take it as soon as you can. If it is almost time for your next dose, take only that dose. Do not take double or extra doses.

Your condition will be monitored carefully while you are receiving this medication. Visit your care team for regular check-ups and for blood tests. It may take up to 4 weeks for your body to respond to this medication.

Tell your care team if your symptoms do not start to get better or if they get worse.

You will need to be on a special diet while you are taking this medication. Talk with your care team before making any changes to your diet.