agalsidase beta

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agalsidase beta

Fabrazyme, Fibrazyme

Pharmacologic class: Homodimeric glycoprotein

Therapeutic class: Recombinant human alpha-galactosidase enzyme

Pregnancy risk category B

Action

Provides exogenous source of alpha-galactosidase A (which is deficient in Fabry disease) and reduces deposits of globotriaosylceramide in kidney and other body tissues

Availability

Powder for reconstitution: for injection, lyophilized: 5 mg, 35 mg

Indications and dosages

Fabry disease

Adults: 1 mg/kg I.V. q 2 weeks. Infuse no faster than 0.25 mg/minute; if tolerated, increase rate by 0.05 to 0.08 mg/minute in subsequent infusions.

Contraindications

None

Precautions

Use cautiously in:

• advanced Fabry disease, cardiac dysfunction

• pregnant or breastfeeding patients

• children.

Administration

• Premedicate with antipyretics, as prescribed.

• To reconstitute, slowly inject 7.2 ml of sterile water for injection into vial; then roll and tilt vial gently to mix drug.

• Don't shake drug, and don't use filter needles.

• Dilute reconstituted solution with normal saline injection to a final volume of 500 ml.

• Infuse through separate I.V. line; don't mix with other drugs.

Adverse reactions

CNS: anxiety, depression, dizziness, paresthesias

CV: dependent edema, chest pain, cardiomegaly

EENT: rhinitis, sinusitis, laryngitis, pharyngitis

GI: nausea, dyspepsia

GU: testicular pain

Musculoskeletal: arthrosis, bone pain

Respiratory: bronchitis, bronchospasm

Skin: pallor

Other: pain, allergic reactions, infusion reactions (hypertension, chest tightness, dyspnea, fever, rigors, hypotension, abdominal pain, pruritus, myalgia, headache, urticaria)

Interactions

Drug-drug. Amiodarone, chloroquine, gentamicin, monobenzone: inhibition of intracellular agalsidase activity

Patient monitoring

• Watch closely for signs and symptoms of allergic or infusion reaction.

• Monitor vital signs and fluid intake and output. Stay alert for dependent edema, blood pressure changes, and chest pain.

• Measure temperature. Watch for signs and symptoms of infection (particularly EENT and respiratory infections).

• Evaluate patient's mood. Report significant anxiety or depression.

Patient teaching

Teach patient to recognize and immediately report signs and symptoms of allergic or infusion reaction.

• Caution patient to avoid driving and other hazardous activities until he knows how drug affects mood, balance, and blood pressure.

• Advise patient to report signs and symptoms of infection (particularly EENT and respiratory infections).

• Inform patient that drug can cause depression and anxiety. Instruct him to notify prescriber if these effects occur.

• As appropriate, review all other significant and life-threatening adverse reactions and interactions, especially those related to the drugs mentioned above.

agalsidase beta

(a-gal-si-dase) ,

Fabrazyme

(trade name)

Classification

Therapeutic: replacement enzyme
Pharmacologic: enzymes
Pregnancy Category: B

Indications

Fabry disease.

Action

Replaces a deficient enzyme.

Therapeutic effects

Prevents damage from accumulation of globotriaosylceramide in capillary endothelium.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.
Distribution: Widely distributed.
Metabolism and Excretion: Unknown.
Half-life: 45–102 min.

Time/action profile

ROUTEONSETPEAKDURATION
IVunknownend of infusion2 wk

Contraindications/Precautions

Contraindicated in: None noted.
Use Cautiously in: Compromised cardiac function (may have ↑ risk of infusion reactions); Obstetric / Lactation / Pediatric: Safety not established.

Adverse Reactions/Side Effects

Central nervous system

  • anxiety
  • depression
  • dizziness
  • headache

Respiratory

  • bronchospasm

Cardiovascular

  • edema (most frequent)
  • cardiomegaly
  • hypotension (most frequent)
  • hypertension

Gastrointestinal

  • dyspepsia
  • nausea

Genitourinary

  • testicular pain

Dermatologic

  • pallor

Musculoskeletal

  • skeletal pain (most frequent)
  • arthrosis

Miscellaneous

  • infusion reactions
  • rigors (most frequent)
  • fever

Interactions

Drug-Drug interaction

None noted.

Route/Dosage

Intravenous (Adults) 1 mg/kg every 2 wk.

Availability

Lyophilized powder for injection: 5 mg/mL in 5– and 35–mL vials

Nursing implications

Nursing assessment

  • Assess for infusion reactions (fever, rigors, chest tightness, hypertension, hypotension, pruritus, myalgia, dyspnea, urticaria, abdominal pain, headache). Pre-treat patients with antipyretics. If an infusion reaction occurs, regardless of pre-treatment, decrease infusion rate, temporarily stop the infusion, and/or administer additional antipyretics, antihistamines, and/or oral corticosteroids. Infusion reactions decline in frequency with continued use; serious reactions may still occur with extended of therapy.
  • Monitor cardiac function during treatment in patients with previously compromised cardiac function.

Potential Nursing Diagnoses

Deficient knowledge, related to medication regimen (Patient/Family Teaching)

Implementation

  • Intravenous Administration
  • Administer antipyretics prior to infusion.
  • Intermittent Infusion: Allow refrigerated agalsidase and diluent to reach room temperature prior to reconstitution (30 min). Determine number of 35 mg and 5 mg vials needed for dose. Reconstitute each vial by slowly injecting 7.2 mL and 1.1 mL of Sterile Water for Injection into each 35 mg vial and 5 mg vial, respectively. Inject down the inside wall of vial. Roll and tilt each vial gently. Solution is clear and colorless; do not administer solutions that are discolored or contain particulate matter. Diluent: Remove an equal volume of 0.9% NaCl from a 500 mL infusion bag, then dilute further with 0.9% NaCl for a final volume of 500 mL. Inject reconstituted agalsidase directly into 0.9% NaCl, do not inject into air space. Discard unused portion of vial. Gently invert bag to mix solution, avoid vigorous shaking or agitation. Diluted solution may be filtered through an in-line low protein-binding 0.2 micrometer filter during administration. Use reconstituted and diluted solutions immediately or within 24 hrs..
  • Rate: Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr). Infusion rate may be slowed if infusion reaction occurs. After tolerance to rate is well established, infusion may be increased in increments of 0.05–0.08 mg/min (increments of 3–5 mg/hr)each subsequent infusion.
  • Y-Site Incompatibility: Do not infuse agalsidase in the same IV line with other products.

Patient/Family Teaching

  • Inform patients that a Registry has been established to better understand Fabry disease and to monitor the effects of agalsidase. Encourage patients to participate; participation is voluntary and may be long term. For information visit www.fabryregistry.com or call 800–745–4447.

Evaluation/Desired Outcomes

  • Reduction of globotriaosylceramide deposition in capillary endothelium of the kidney and certain other cell types.
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References in periodicals archive ?
Galafold (migalastat) 123 mg capsules is an oral pharmacological chaperone of alpha-Galactosidase A (alpha-Gal A) for the treatment of Fabry disease in adults who have amenable GLA variants.
Alpha-galactosidase is a digestive enzyme that breaks down the carbohydrates in beans into simpler sugars to make them easier to digest.
Combat this problem with a supplement containing the enzyme alpha-galactosidase, or you know, just eat your broccoli at home.
Fabry disease is an inherited metabolic disease caused by mutations in the GLA gene, which result in a deficiency of the enzyme alpha-galactosidase A (?-Gal A).
(14) observed no correlation between the enzyme activities in DBS and in leukocytes for the whole population because of the corrected calculation of enzyme activity regarding protein levels in leucocytes and the inhibition of alpha-galactosidase B isozyme activity by N-acetyl-D-galactosamine in DBS samples but not inhibition of isozyme in leucocyte samples (14).
Eng, "Alpha-galactosidase A deficiency: fabry disease," in The Metabolic and Molecular Basis of Inherited Disease, C.
Anderson-Fabry disease (AFD) is an X-linked disease of lysosomal metabolism resulting in attenuated activity or absence of the enzyme alpha-galactosidase A (a-Gal A).
1.86-21.9 [micro]mol/L/h); alpha-galactosidase, as control enzyme, was within normal limits (8.13 [micro]mol/L/h; n.v.
Fabry disease is caused by a genetic mutation at q22 of the X chromosome, and it results in a deficiency of the alpha-galactosidase A enzyme.
We found statistically significant correlations between increased resistance to 5-fluorocytosine and higher activity of naphthyl phosphohydrolase and lipase; increased resistance to miconazole and higher activity of beta-glucosidase; increased resistance to fluconazole and higher activity of alpha-glucosidase, alpha-galactosidase, and fucosidase.
He also takes an enzyme blend of amylase, cellulase, lactase, glucoamylase, alpha-galactosidase and invertase.
Desnick, and etal., "Fabry: disease: enzymatic diagnosis of hemizygotes and heterozygotes alpha-galactosidase activities in plasma, serum, urine, and leukocytes," Journal of Laboratory and Clinical Medicine, vol.