galsulfase

ROUTE	ONSET	PEAK	DURATION
IV	unknown	24 wk	unknown

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.

Route/Dosage ⬆ ⬇

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

Availability ⬆ ⬇

Solution for injection: 1 mg/mL

Assessment ⬆ ⬇

Assess for infusion-related reactions (fever, chills/rigors, headache, rash, mild to moderate urticaria); may occur in over half of patients. May also cause nausea, vomiting, ↑ BP, retrosternal pain, abdominal pain, malaise, and joint pain. Severe reactions include angioneurotic edema, hypotension, dyspnea, bronchospasm, respiratory distress, and apnea. May occur as late as Week 55 of treatment. Patients should receive antihistamines and antipyretics prior to administration to minimize occurrence. Slowing or temporary interruption of infusion and administration of additional antihistamines, antipyretics, and occasionally corticosteroids usually stop symptoms. Most patients are able to complete infusion. Administer subsequent infusions at a slower rate; provide additional prophylactic antihistamines and, if reaction is severe, prophylactic corticosteroids. If severe reaction occurs, discontinue infusion immediately and treat symptoms. Consider risk versus benefit before readministering galsulfase.
Anaphylaxis and serious hypersensitivity reactions have been observed in patients during and up to 24 hr after infusion. Assess for hypersensitivity reactions (anaphylaxis, shock, respiratory distress, dyspnea, bronchospasm, laryngeal edema, hypotension). If anaphylaxis or severe hypersensitivity reactions occur, immediately discontinue infusion and initiate appropriate emergency treatment (resuscitation; epinephrine; additional antihistamines, antipyretics, or corticosteroids). Consider the risks and benefits of readministering following severe hypersensitivity reactions.
Assess for sleep apnea, common in patients with MPS VI, before therapy. Administration of pretreatment antihistamines may ↑ risk. Supplemental oxygen and continuous positive airway pressure should be available during infusion.
Assess vital signs and respiratory status before administration. Administration should be delayed in patients with an acute febrile or respiratory illness.
Monitor for signs and symptoms of spinal/cervical cord compression (back pain, paralysis of limbs below level of compression, urinary and fecal incontinence). Manage symptomatically.

Implementation ⬆ ⬇

Pretreat with antihistamines with or without antipyretics 30–60 min before start of infusion.
Administer in a health care setting with appropriate medical monitoring and supportive measures (cardiopulmonary resuscitation equipment) available during administration.

IV Administration:

Intermittent Infusion: Diluent: Determine number of vials to be diluted based on patient weight; round to nearest whole vial. Remove vials from refrigerator and allow to reach room temperature. Do not use after expiration date. Do not allow vials to remain at room temperature for >24 hr before dilution. Do not heat or microwave vials. Solution is clear to slightly opalescent and colorless to pale yellow; a few translucent particles may be present. Do not administer solutions that are discolored or contain particulate matter. Withdraw and discard volume equal to galsulfase from a 250-mL bag of 0.9% NaCl (not necessary if using 100-mL bag in patients susceptible to fluid volume overload). Slowly withdraw calculated volume from galsulfase vials, using caution to avoid excessive agitation; may denature and render inactive. Do not use a filter needle. Slowly add galsulfase to 0.9% NaCl; avoid agitation. Gently rotate infusion bag to ensure distribution; do not shake. Discard unused portions. Use diluted solution immediately; storage should not exceed 48 hr from time of preparation to completion of administration. Use PVC containers and administer via PVC infusion set with an in-line low-protein-binding 0.2-micrometer filter.
Rate: Administer at a rate of 6 mL/hr for 1st hr. If infusion is well tolerated, rate may be ↑ to 80 mL/hr for next 3 hr. Administer total volume of infusion over no less than 4 hr. If 100-mL bag is used, ↓ infusion rate so total volume is infused over >4 hr. Infusion time can be extended for up to 20 hr if infusion reactions occur.
Y-Site Incompatibility: Do not administer other drugs through same IV line.

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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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇

Action ⬆ ⬇

Pharmacokinetics ⬆ ⬇

Time/Action Profile ⬆ ⬇

Contraind./Precautions ⬆ ⬇