2.3 Instructions for UseKuvan Tablets

Kuvan tablets may be swallowed either as whole tablets or dissolved in 120 to 240 mL of water or apple juice and taken orally within 15 minutes of dissolution. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, tablets may be stirred or crushed. The tablets may not dissolve completely. Patients may see small pieces floating on top of the water or apple juice. This is normal and safe for patients to swallow. If after drinking the medicine patients still see pieces of the tablet in the container, more water or apple juice can be added to make sure all of the medicine is consumed. Kuvan tablets may also be crushed and then mixed in a small amount of soft foods such as apple sauce or pudding.

Kuvan Powder for Oral Solution

Kuvan powder for oral solution should be dissolved in 120 to 240 mL of water or apple juice and taken orally within 30 minutes of dissolution. Kuvan powder for oral solution may also be stirred in a small amount of soft foods such as apple sauce or pudding.  Empty the contents of the packet(s) in water, apple juice, or a small amount of soft foods and mix thoroughly. The powder should dissolve completely.

For infants weighing 10 kg or less, Kuvan can be dissolved in as little as 5 mL of water or apple juice and a portion of this solution corresponding to a 10 mg/kg dose may be administered orally via an oral dosing syringe. Table 1 provides dosing information for infants at the recommended starting dose of 10 mg/kg per day. Refer to Table 2 for dosing information at 20 mg/kg per day if dosage adjustment is needed.

Table 1: 10 mg/kg per day Dosing Table for Infants Weighing 10 kg or less

Patient

Weight (kg)
 Starting Dose: 10 mg/kg per day*
 
Dose (mg)
 # Kuvan

100 mg Packets
Dissolved†
 Dilution Volume
(mL)‡
 Administered

Dose volume
(mL)§
 
  1
 10
 1
 10
  1
 
  2
 20
 1
 10
  2
 
  3
 30
 1
 10
  3
 
  4
 40
 1
 10
  4
 
  5
 50
 1
 10
  5
 
  6
 60
 1
 5
  3
 
  7
 70
 1
 5
   3.5
 
  8
 80
 1
 5
  4
 
  9
 90
 1
 5
 4.5
 
10
 100
 1
 5
 5
 
*Starting dose for infants is 10 mg/kg per day. Dosing information for 20 mg/kg per day is provided in Table 2.

† Powder for oral solution provided in single use packets containing 100 mg Kuvan per packet

‡ Volume of water or apple juice to dissolve Kuvan Powder for Oral Solution.

§Discard remainder of mixture after volume to be administered is drawn.

Table 2: 20 mg/kg per day Dosing Table for Infants Weighing 10 kg or less

Patient

Weight (kg)
 20 mg/kg per day
 
Dose (mg)
 # Kuvan 100 mg  Packets*
Dissolved
 Dilution Volume
(mL)†
 Administered
Dose volume

(mL)§
 
  1
   20
 1