Sapropterin Dihydrochloride

SAPROPTERIN DIHYDROCHLORIDE- sapropterin dihydrochloride tablet
Dr. Reddys Laboratories Inc.

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1 INDICATIONS AND USAGE

Sapropterin dihydrochloride tablets are indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). Sapropterin dihydrochloride is to be used in conjunction with a Phe-restricted diet.

2 DOSAGE AND ADMINISTRATION

2.1 Dosage

Treatment with sapropterin dihydrochloride should be directed by physicians knowledgeable in the management of PKU.

All patients with PKU who are being treated with sapropterin dihydrochloride should also be treated with a Phe-restricted diet, including dietary protein and Phe restriction.

Starting Dosage

Pediatric Patients 1 month to 6 years: The recommended starting dose of sapropterin dihydrochloride is 10 mg/kg taken once daily.

Patients 7 years and older: The recommended starting dose of sapropterin dihydrochloride is 10 to 20 mg/kg taken once daily.

Dosage Adjustment (Evaluation Period)

Existing dietary protein and Phe intake should not be modified during the evaluation period.

If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with sapropterin dihydrochloride at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of sapropterin dihydrochloride treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg per day do not show a biochemical response and treatment with sapropterin dihydrochloride should be discontinued in these patients.

If a 20 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with sapropterin dihydrochloride at 20 mg/kg per day for a period of 1 month. Blood Phe levels should be checked after 1 week of sapropterin dihydrochloride treatment and periodically during the first month. Treatment should be discontinued in patients who do not show a biochemical response (blood Phe does not decrease) after 1 month of treatment at 20 mg/kg per day [see Warnings and Precautions (5.4)].

Once responsiveness to sapropterin dihydrochloride has been established, the dosage may be adjusted within the range of 5 to 20 mg/kg per day according to biochemical response to therapy (blood Phe). Periodic blood Phe monitoring is recommended to assess blood Phe control, especially in pediatric patients [see Warnings and Precautions (5.3)].

2.2 Preparation and Administration Instructions

Take sapropterin dihydrochloride tablets orally with a meal, preferably at the same time each day [see Clinical Pharmacology (12.3)]. A missed dose should be taken as soon as possible, but two doses should not be taken on the same day.

S apropterin Dihydrochloride Tablets

Sapropterin dihydrochloride 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. Sapropterin dihydrochloride tablets may also be crushed and then mixed in a small amount of soft foods such as apple sauce or pudding.

Sapropterin Dihydrochloride Powder for Oral Solution

Patients weighing greater than 10 kg

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

Patients weighing 10 kg or less (use 100 mg packets)

For infants weighing 10 kg or less, sapropterin dihydrochloride powder for oral solutioncan be dissolved in as little as 5 mL of water or apple juice and a portion of this solutioncorresponding 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 of10 mg/kg per day. Refer to Table 2 for dosing information at 20 mg/kg per day if dosageadjustment 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) Sapropterin Dihydrochloride Powder for Oral Solution 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 sapropterin dihydrochloride per packet

Volume of water or apple juice to dissolve Sapropterin Dihydrochloride 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) Sapropterin Dihydrochloride Powder for Oral Solution 100 mg Packets* Dissolved Dilution Volume (mL) Administered Dose volume (mL) §
1 20 1 5 1
2 40 1 5 2
3 60 1 5 3
4 80 1 5 4
5 100 1 5 5
6 120 2 5 3
7 140 2 5 3.5
8 160 2 5 4
9 180 2 5 4.5
10 200 2 5 5

* Powder for oral solution provided in single use packets containing 100 mg sapropterin dihydrochloride per packet

Volume of water or apple juice to dissolve Sapropterin DihydrochloridePowder for Oral Solution.

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

3 DOSAGE FORMS AND STRENGTHS

Sapropterin dihydrochlorid tablets are for oral use. Each tablet contains 100 mg of sapropterin dihydrochloride. Tablets are round, off-white to light yellow, mottled, and debossed with “177”.

Sapropterin dihydrochloride powder for oral solution is available as a unit dose packetcontaining 100 mg of sapropterin dihydrochloride and as a unit dose packet containing500 mg of sapropterin dihydrochloride. The powder is off-white to yellow in color.

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