galsulfase

Contraindicated in:

None.

Use Cautiously in:

Febrile or respiratory illness;
OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk;
Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
Pedi: Children <5 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: malaise

Derm: facial edema

EENT: conjunctivitis, corneal opacification, ear pain

GI: gastroenteritis, abdominal pain

MS: areflexia

Neuro: SPINAL/CERVICAL CORD COMPRESSION

Resp: dyspnea

Misc: HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS), infusion reactions, rigors

Interactions ⬆ ⬇

Drug-drug:

None noted.

Availability ⬆ ⬇

Solution for injection: 1 mg/mL

Route/Dosage ⬆ ⬇

IV (Adults and Children >5 yr): 1 mg/kg once weekly.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Replaces a deficient enzyme in MPS IV. Without replacement, glycoaminoglycans accumulate, resulting in cell, organ, and tissue dysfunction.

Therapeutic effects:

Improved walking and stair climbing.

Classifications ⬆ ⬇

Therapeutic Classification: replacement enzyme

Pharmacologic Classification: enzymes

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Unknown.

Half-Life: 9 min (after one wk of treatment; 26 min after 24 wk of treatment).

Time/Action Profile ⬆ ⬇

(improved exercise parameters)

ROUTE	ONSET	PEAK	DURATION
IV	unknown	24 wk	unknown

Patient/Family Teaching ⬆ ⬇

Explain purpose and side effects of medication to patient. Advise patient to read Patient Information before starting therapy. Advise patient and/or caregiver to notify a health care professional if a dose is missed.
Advise patient and/or caregiver to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Advise patient and/or caregiver to notify health care professional immediately if signs or symptoms of a hypersensitivity or infusion reaction occur during infusion or if signs or symptoms of cardiac or respiratory decompensation occur during or following an infusion. Inform patients using supplemental oxygen or continuous positive airway pressure during sleep to have these treatments readily available during infusion or extreme drowsiness/sleep induced by antihistamine use.
Rep: Advise women of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. Pregnancy can exacerbate preexisting clinical manifestations of MPS and lead to adverse pregnancy outcomes for both mother and fetus.

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Indications ⬇

Contraind./Precautions ⬆ ⬇