Information about Kuvan (Sapropterin)
Kuvan is a medication that contains sapropterin dihydrochloride, a synthetic form of the naturally occurring tetrahydrobiopterin (BH4). It is used to manage phenylketonuria (PKU), a genetic disorder that leads to the accumulation of phenylalanine in the body.
Product Highlights
- Indicated for the treatment of PKU in patients who have some residual enzyme activity, allowing for dietary flexibility and improved metabolic control.
Key Ingredient
- Sapropterin Dihydrochloride
Key Benefits
- Helps lower blood phenylalanine levels in patients with PKU.
- Allows patients to expand their dietary choices, which can improve quality of life.
- Generally well-tolerated with a favorable safety profile.
Direction of Use
- Dosing is individualized based on the patient's phenylalanine levels and clinical response; typically starts at 10 mg/kg/day.
- The powder can be mixed with soft foods or liquids; tablets should be swallowed whole.
Safety Concerns
- Common side effects may include nausea, diarrhea, and abdominal pain.
- Rarely, patients may experience allergic reactions; monitoring for symptoms is advised.
- Regular monitoring of blood phenylalanine levels is necessary to adjust dosage and ensure effectiveness.
Avoid Kuvan (Sapropterin) If
- Patients with a known allergy to sapropterin or any of its components should avoid use.
- Not recommended for patients with uncontrolled PKU or those who do not have residual enzyme activity.
- Consult a healthcare provider regarding use during pregnancy and breastfeeding, as safety data is limited.
