Generic Name and Formulations:
Sapropterin dihydrochloride 100mg; tabs.
Indications for KUVAN:
In conjunction with a phenylalanine (Phe)-restricted diet: To reduce blood Phe levels in hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).
Adults and Children:
<1 month: consult manufacturer. Take with food at same time each day. Dissolve tab in 4–8oz water or apple juice (may crush tab) and drink within 15 mins (tab may not completely dissolve). 1 month–6yrs: Initially 10mg/kg once daily; if inadequate response may increase to 20mg/kg once daily; if inadequate response after 1 month, discontinue. ≥7yrs: 10–20mg/kg once daily; if inadequate response after 1 month, discontinue. Usual range 5–20mg/kg per day; adjust based on blood Phe levels.
Monitor blood levels of Phe after 1 week to assess effect, then periodically. Discontinue if anaphylaxis occurs. Maintain Phe-restricted diet. Monitor BP and change in neurologic status. Hepatic or renal impairment; monitor. Pregnancy (Cat.C). Nursing mothers.
Enzyme cofactor (BH4).
Caution with drugs that can inhibit folate metabolism (eg, methotrexate), PDE-5 inhibitors (eg, sildenafil, tadalafil, vardenafil), other nitric oxide donors, levodopa; monitor. May potentiate drugs that are P-gp or BCRP substrates.
Headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, nasal congestion, gastritis, hypophenylalaninemia, hyperactivity (monitor).
Tabs—120; Packet—1, 30
Sign Up for Free e-newsletters
Psychiatry Advisor Articles
- Nomophobia: The Modern-Day Pathology
- Emerging Theories in the Pathophysiology of MDD: Could the Opioid System Be Involved?
- Transdermal Nicotine Boosts Mood and Cognitive Function in Late-Life Depression
- Combination Cognitive Behavioral Therapy With Fluoxetine Effective for Adolescent Depression
- Prazosin May Be Effective as Treatment for Alcohol Use Disorder
- Text Message Reminders Improve Medication Adherence in Bipolar I Disorder
- Improving Medication Adherence in ADHD Lowers Risk for Oppositional Defiant Disorder, Conduct Disorder in Adulthood
- High Suicidality Among Children With ADHD Mediated by Family Functioning, Psychiatry Comorbidities
- Pilot Study Investigates Lithium vs Quetiapine for Bipolar Spectrum Disorder
- How Personalized Hospital Ratings May Drive Patient-Specific Care in the Digital Age