Usual Adult Dose for Sickle Cell Anemia

Initial dose: 5 mg/kg via IV infusion every 2 weeks for 2 doses, then
Maintenance dose: 5 mg/kg via IV infusion every 4 weeks

Comments:

• This drug may be given with or without hydroxyurea.
• Use patient's actual body weight for dose calculation.

Usual Pediatric Dose for Sickle Cell Anemia

16 years or older:
Initial dose: 5 mg/kg via IV infusion every 2 weeks for 2 doses, then
Maintenance dose: 5 mg/kg via IV infusion every 4 weeks

Comments:

• This drug may be given with or without hydroxyurea.
• Use patient's actual body weight for dose calculation.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

Management of Infusion Related Reactions:
MILD TO MODERATE infusion-related reaction:

• Temporarily interrupt or slow the rate of infusion
• Initiate symptomatic treatment (e.g., acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), opioids, antihistamines, IV fluids, and/or oxygen therapy)
• For subsequent infusions, consider premedication and/or reduced infusion rate

• Discontinue infusion
• Institute medical care
• Consider permanent discontinuation

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 16 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Administer by IV infusion over 30 minutes
• This drug should be prepared and administered by a healthcare professional
• IV line should contain a sterile, nonpyrogenic 0.2-micron inline filter
• After administration, flush line with at least 25 mL or NS or D5W

• If administered within 2 weeks after missed dose, continue dosing according to original schedule
• If administered more than 2 weeks after missed dose, continue dosing every 4 weeks thereafter

• Stable at temperatures up to 25C (77F) for up to 4.5 hours from initial piercing of first vial to completion of infusion, OR
• Refrigerated (2C to 8C [36F to 46F]) for no more than 24 hours from initial piercing of first vial to completion of infusion (this includes storage of diluted solution and time to warm up to room temperature)

• Store and transport refrigerated at 2C to 8C (36F to 46F) in the original carton to protect from light
• Do not shake; do not freeze

• Bring vials to room temperature for a maximum of 4 hours prior to start of preparation (piercing the first vial)
• Drug is to be diluted in NS or D5W to a total volume of 100 mL prior to administration; infusion bags should be made of polyvinyl chloride, polyethylene, or polypropylene
• From 100 mL bag of NS or D5W, remove volume of NS or D5W that is equal to volume of drug to be added; once removed, add volume of drug to infusion bag, volume of drug should not exceed 96 mL
• Gently invert infusion bag to mix diluted solution; do not shake
• Administer prepared solution as soon as possible not exceeding storage requirements listed above

• This drug is the first targeted therapy approved for sickle cell disease that specifically inhibits selectin, a substance that contributes to cells sticking together and leads to vaso-occlusive crisis.
• Efficacy in clinical trials was based on patients having fewer health care visits for vaso-occlusive crisis annually (median annual rate of 1.63 visits), compared to patients who received a placebo (median annual rate of 2.98 visits).

• Monitor for signs and symptoms of infusion-related reactions.

• 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 infusion-related reactions.
• Patient should inform healthcare provider that they are receiving this drug prior to any blood tests used to measure platelet counts.

Frequently asked questions

• What type of drug is Adakveo and how does it work?
• How is Adakveo administered?