Skip to Main Content

++

INTRODUCTION

++

galsulfase (gal-sul-fase)

Naglazyme

Classification

Therapeutic: replacement enzyme

Pharmacologic: enzymes

++
Indications
++

Mucopolysaccharidosis VI (MPS IV).

++
Action
++

Replaces a deficient enzyme in MPS IV. Without replacement, glycosaminoglycans accumulate resulting in cell, organ, and tissue dysfunction. Therapeutic Effects: Improved walking and stair climbing.

++
Adverse Reactions/Side Effects
++

CV: malaise. EENT: conjunctivitis, corneal opacification, ear pain. Resp: dyspnea. CV: chest pain, ↑ blood pressure. Derm: facial edema. GI: gastroenteritis, abdominal pain. MS: areflexia. Misc: INFUSION REACTIONS, rigors.

++

PHYSICAL THERAPY IMPLICATIONS

++
Examination and Evaluation
++

  • Be alert for allergy-like responses (wheezing, laryngeal edema, urticaria, other skin reactions) that occur during and after administration (infusion reactions). Report these responses to the physician or nursing staff immediately.

  • Assess ambulation on level surfaces and during stair climbing. Document any changes in functional ability to help document whether drug therapy is effective in maintaining or improving motor function.

  • Monitor any changes in muscle tone or deep tendon reflexes. Report increased muscle contractions or decreased reflex activity.

  • Assess blood pressure (BP) and compare to normal values (See Appendix F). Report a sustained increase in BP (hypertension) or any chest pain or difficult, labored breathing.

  • Monitor and report any vision problems, eye inflammation, or ear pain.

++
Interventions
++

  • Implement therapeutic exercises (resistive training, coordination exercises, gait training) to complement the effects of drug therapy and help achieve optimal function.

++
Patient/Client-Related Instruction
++

  • Instruct patient and family/caregivers to report other troublesome side effects such as severe or prolonged malaise, facial swelling, or GI problems (gastroenteritis, abdominal pain).

++
Pharmacokinetics
++

Absorption: IV administration results in complete bioavailability.

++

Distribution: Widely distributed.

++

Metabolism and Excretion: Unknown.

++

Half-life: 9 min (after 1 wk of treatment, 26 min (after 24 wk of treatment).

++

Table Graphic Jump Location
Favorite Table | Download (.pdf) | Print

TIME/ACTION PROFILE (improve exercise parameters)

ROUTE ONSET PEAK DURATION
IV unknown 24 wk unknown

++
Contraindications/Precautions
++

Contraindicated in: None.

++

Use Cautiously in: Febrile or respiratory illness; Pedi: Children <5 yr (safety not established); OB: Pregnancy or lactation (safety being evaluated).

++
Interactions
++

Drug-Drug: None noted.

++
Route/Dosage
++

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

++
Availability
++

Solution for IV administration (diluted prior to use): 5 mg/5 mL in 5-mL vials.

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.