alglucosidase

alglucosidase

(al-gloo-ko-side-ase) ,

Lumizyme

(trade name),

Myozyme

(trade name)

Classification

Therapeutic: replacement enzyme
Pregnancy Category: B

Indications

Myozyme: Replacement enzyme in infantile-onset Pompe disease (alpha glucosidase (GAA) deficiency).Lumizyme: Replacement enzyme in late-onset (non-infantile) Pompe disease in patients without evidence of cardiac hypertrophy.

Action

Replaces alpha-glucosidase. Without this enzyme, glycogen accumulates in tissues including cardiac and skeletal muscles and hepatic tissues, leading to the development of cardiomyopathy, progressive muscle weakness, and impairment of respiratory function.

Therapeutic effects

Improved survival with delayed need for ventilatory support.
Improved lung function and exercise capacity.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.
Distribution: Unknown.
Metabolism and Excretion: Unknown.
Half-life: 2.3–2.5 hr.

Time/action profile

ROUTEONSETPEAKDURATION
IVunknownend of infusion2 wk

Contraindications/Precautions

Contraindicated in: None known.
Use Cautiously in: Acute underlying illness (↑ risk of infusion reactions); Obstetric: Use only if clearly needed; Lactation: Lactation; Pediatric: Safety not established in children <1 mo or >3.5 yr (Myozyme) or <8 yr (Lumizyme).

Adverse Reactions/Side Effects

Cardiovascular

  • bradycardia (most frequent)
  • tachycardia (most frequent)

Respiratory

  • respiratory distress failure
  • cough (most frequent)
  • ↓ oxygen saturation (most frequent)
  • tachypnea

Ear, Eye, Nose, Throat

  • blurred vision
  • vertigo

Gastrointestinal

  • constipation (most frequent)
  • diarrhea (most frequent)
  • reflux (most frequent)
  • vomiting (most frequent)

Dermatologic

  • necrotizing skin lesions (life-threatening)
  • flushing (most frequent)
  • rash (most frequent)
  • dermatitis (most frequent)
  • urticaria (most frequent)

Hematologic

  • anemia (most frequent)
  • lymphadenopathy

Fluid and Electrolyte

  • edema

Miscellaneous

  • allergic reactions including anaphylaxis (life-threatening)
  • infusion reactions (life-threatening)
  • fever (most frequent)

Interactions

Drug-Drug interaction

None noted.

Route/Dosage

Intravenous (Children 1 mo–3.5 yr) Myozyme—20 mg/kg every 2 wk.
Intravenous (Adults and Children ≥8 yr) Lumizyme—20 mg/kg every 2 wk.

Availability

Lyophilized powder for IV administration (requires reconstitution): 50 mg/vial

Nursing implications

Nursing assessment

  • Observe for signs and symptoms of anaphylaxis (rash, pruritus, laryngeal edema, wheezing). Keep epinephrine, an antihistamine, corticosteroids, and resuscitation equipment close by in case of anaphylactic reaction.
  • Monitor for infusion-related reactions (headache, fever, tachycardia, cough, cyanosis, rash, erythema, urticaria, hypotension. Most reactions are managed with antihistamines and/or corticosteroids prior to or during infusions, slowing rate of infusion, and/or early discontinuation if reaction is serious. Infusion reactions may occur any time during or up to 2 hr after infusion and are more likely with higher infusion rates.
  • Monitor cardiorespiratory status continuously during therapy. May cause acute cardiorespiratory failure requiring intubation and inotropic support.
  • Lab Test Considerations: Monitor liver enzymes prior to and periodically during therapy.

Potential Nursing Diagnoses

Ineffective tissue perfusion (Indications)

Implementation

  • Lumizyme is available only through a restricted distribution program called the LUMIZYME ACE Program due to the potential risk of rapid disease progression in Pompe disease patients less than 8 yrs of age. Only prescribers and healthcare facilities enrolled in the program may prescribe, dispense or administer Lumizyme. Lumizyme may be administered only to patients who are enrolled in and meet all the conditions of the Lumizyme ACE Program. To enroll in the Lumizyme ACE Program call 1-800-745-4447.
  • Intravenous Administration
  • Intermittent Infusion: Determine number of vials required for the dose ordered. If number of vials includes a fraction, round up to next whole number. Allow vials to reach room temperature before reconstitution, approximately 30 min. Reconstitute by slowly injecting 10.3 mL of Sterile Water for Injection to inside wall of each vial. Avoid forceful impact of water on powder to avoid foaming. Tilt and roll each vial gently. Do not invert, swirl, or shake. Protect solution from light. Each vial contains 5 mg/mL with a total extractable dose of 50 mg/10 mL. Solution is clear and may occasionally contain white strands or translucent fibers; do not administer solutions that are discolored or contain particulate matter. Vials are for single use; discard remaining medication. Diluent: Dilute each vial in 100 mL of 0.9% NaCl immediately after reconstitution. Concentration: 0.5–4 mg/mL. Slowly withdraw reconstituted solution from each vial. Remove airspace from infusion bag to minimize particle formation due to sensitivity of medication to air. Add reconstituted solution slowly and directly into 0.9% NaCl solution, not into airspace in bag. Gently invert or massage to mix; do not shake. Use a 0.2 micrometer low-protein binding in-line filter for administration. Administer immediately. Solution may be stored in refrigerator for up to 24hr.
  • Rate: Administer over 4 hr. Using an infusion pump, administer initially at a rate of 1 mg/kg/hr. ↑ rate to 2 mg/kg/hr every 30 min, after tolerance to medication is established, until a maximum rate of 7 mg/kg/hr is reached. Monitor vital signs with each dose ↑. If stable, administer at 7 mg/kg/hr until infusion is completed. Slow or temporarily stop infusion if infusion reactions occur.

Patient/Family Teaching

  • Inform patient that a registry for patients with Pompe disease was established to evaluate long term treatments. Women of childbearing potential are also encouraged to register. For information, visit www.pomperegistry.com or call 1-800-745-4447.

Evaluation/Desired Outcomes

  • Improved survival with delayed need for ventilatory support in patients with Pompe disease.
  • Improved lung function and exercise capacity.
References in periodicals archive ?
duvoglustat hydrochloride + alglucosidase alfa - Drug Profile 50
The Phase III trial is a randomized, multi-center, multi-national, double-blinded study to compare the efficacy and safety of repeated bi-weekly infusions of neoGAA and alglucosidase alfa in treatment-naive patients with late-onset Pompe disease.
COMET is a Phase three randomised, multi-centre, multi-national, double-blinded study to compare the efficacy and safety of repeated bi-weekly infusions of neoGAA and alglucosidase alfa in treatment-naive patients with late-onset Pompe disease.
Contract notice: Delivery of the drug alglucosidase alpha 800 packs.
Clinical outcomes after long-term treatment with alglucosidase alfa in infants and children with advanced Pompe disease.
Pregnant women treated with alglucosidase alfa (Myozyme) for Pompe disease can enroll in the Pompe Registry (800-745-4447 x 15500 / www.
Since the end of 2012, enzyme replacement therapy (ERT) (as alglucosidase alfa) has been registered with the Medicines Control Council in South Africa for use in PD patients.
Mecanismo Doencaalvo Nome do farmaco de acao Fabry Betagalsidase TRE Fabry Alfagalsidase TRE Fabry AT1001 Chaperona Gaucher Velaglucerase alfa (glicocerebrosidase TRE produzida em celulas humanas) Gaucher Glicocerebrosidase produzida em TRE celulas vegetais Gaucher Miglustate ISS Gaucher Genz-112638 ISS Gaucher AT2101 Chaperona MPS I Laronidase TRE MPS II Idursulfase TRE MPS VI Galsulfase TRE Niemann-Pick B Esfingomielinase recombinante TRE Niemann-Pick C Miglustate ISS Pompe Alglucosidase alfa([alpha]-glicosidase TRE acida produzida em celulas CHO) Pompe [alfa]-glicosidase acida recombinante TRE produzida a partir do leite de coelhos transgenicos Tay-Sachs (tardia) Miglustate ISS Tay-Sachs (tardia) Pirimetamina Chaperona Registro Doencaalvo Fase de desenvolvimento Anvisa?
In May 2007 Genzyme began providing alglucosidase alfa free-of-charge to patients in the United States through a program known as the Alglucosidase Alfa Temporary Access Program (ATAP).
According to the company, NeoGAA is an investigational second-generation alglucosidase alfa enzyme replacement therapy that has been specifically designed for enhanced receptor targeting and enzyme uptake through greater affinity for the M6P receptors on muscle cells, with the aim of enhancing glycogen clearance and improving on the clinical efficacy achieved with alglucosidase alfa.
Tenders are invited for Inj Alglucosidase Alfa 50 Mg(Myozyme)
Included in the classifications are three products that will not be discussed: estradiol valerate/dienogest (Natazia; X), a combination oral contraceptive; alglucosidase alfa (Lumizyme; B), a metabolic agent for Pompe disease that is nearly identical to Myozyme (same generic name); and a formulation of botulinum toxin type A (Xeomin; C).