Usual Adult Dose for Sickle Cell Anemia

1500 mg orally once a day

Comment:

• This drug may be given with or without hydroxyurea.

Use: For the treatment of sickle cell disease.

Usual Pediatric Dose for Sickle Cell Anemia

4 to less than 12 years:

• Weight: 10 to less than 20 kg: 600 mg orally once a day
• Weight: 20 to less than 40 kg: 900 mg orally once a day
• Weight 40 kg or greater: 1500 mg orally once a day

12 years or older: 1500 mg orally once a day

Comment:

• This drug may be given with or without hydroxyurea.

Use: For the treatment of sickle cell disease in patients 4 years or older.

Renal Dose Adjustments

Mild to severe renal impairment (CrCl 15 mL/min or greater): No adjustment recommended
ESRD: Data not available

Liver Dose Adjustments

Mild or moderate liver dysfunction (Child-Pugh A and B): No adjustment recommended.

For patients 12 years or older with severe liver dysfunction (Child-Pugh C): 1000 mg orally once a day

For patients 4 years to less than 12 years with severe liver dysfunction (Child-Pugh C):

• Weight: 10 to less than 20 kg: 300 mg orally once a day
• Weight: 20 to less than 40 kg: 600 mg orally once a day
• Weight 40 kg or greater: 900 or 1000 mg orally once a day

Dose Adjustments

Avoid Concomitant use of Strong or Moderate CYP450 3A4 Inducers; however, if use is unavoidable, voxelotor doses should be increased as follows:

12 years or older:

• If concomitant use of STRONG CYP450 3A4 Inducers is unavoidable: Increase dose of voxelotor to 2500 mg orally once a day
• If concomitant use of MODERATE CYP450 3A4 Inducers is unavoidable: Increase dose of voxelotor to 2000 mg orally once a day

4 to Less than 12 years:

• If concomitant use of STRONG CYP450 3A4 Inducers is unavoidable: Increase voxelotor dose to:
• Weight: 10 to less than 20 kg: 900 mg orally once a day
• Weight: 20 to less than 40 kg: 1500 mg orally once a day
• Weight 40 kg or greater: 2400 or 2500 mg orally once a day
• If concomitant use of MODERATE CYP450 3A4 Inducers is unavoidable: Increase voxelotor dose to:
• Weight: 10 to less than 20 kg: 900 mg orally once a day
• Weight: 20 to less than 40 kg: 1200 mg orally once a day
• Weight 40 kg or greater: 2000 or 2100 mg orally once a day

Precautions

CONTRAINDICATIONS:

• History of serious hypersensitivity reaction to the active substance or any product excipients; clinical manifestations may include generalized rash, urticaria, mild shortness of breath, mild facial swelling, and eosinophilia

Safety and efficacy have not been established in patients younger than 4 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
Oral tablets:

• Take orally once a day with or without food
• Tablets should be swallowed whole; do not cut, crush, or chew

Tablets for Oral Suspension:

• Tablets for oral suspension should be dispersed immediately before administration
• Tablet(s) should be dispersed in a cup of room temperature clear liquid (e.g., drinking water or clear soda); minimum volume of clear liquid for dispersion is 5 mL per 300 mg tablet
• Place tablet(s) in appropriate amount of clear liquid; after tablet(s)starts to disintegrate, swirl contents of cup until the tablets are dispersed; wait 1 to 5 minutes and swirl again before consuming contents of the cup
• The tablets will not completely dissolve and there will be small tablet clumps in the mixture; resuspend any residue left in the cup in more clear drink and administer; repeat until no tablet residue is left in the cup
• Do not swallow whole, cut, crush, or chew the tablets for oral suspension

Tablets for oral suspension are indicated for patients 4 to less than 12 years and may be administered to patients 12 years and older who have difficulty swallowing; use the number of tablets for oral suspension needed to achieve the recommended dose

Missed dose: If a dose is missed or not administered entirely, resume dosing the following day

General:

• This drug is approved under the accelerated approval based on increase in hemoglobin (Hb); continued approval may be contingent upon verification and description of clinical benefit in confirmatory trials.

Patient advice:

• Patients should be instructed to read the US FDA-approved patient labeling (Patient Information).
• Patients should be instructed to immediately report signs or symptoms of hypersensitivity reactions such as generalized rash, urticaria, shortness of breath, facial swelling, and eosinophilia.
• Patient should be instructed not to breastfeed and to speak with their healthcare provided if they are pregnant or planning to become pregnant.
• Patients should be instructed to tell their healthcare provider about all the medications they are taking including over the counter and herbal products.