miglustat


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miglustat

(mi-gloo-stat) ,

Zavesca

(trade name)

Classification

Therapeutic: none assigned
Pharmacologic: enzyme inhibitors
Pregnancy Category: X

Indications

Mild to moderate type 1 Gaucher's disease, when enzyme replacement therapy is not an option.

Action

Competitively and reversibly inhibits glucosylceramide synthase which is the initial step in the production of glycosphingolipids. The glycosphingolipid glucosylceramide accumulates in tissue in Gaucher's disease.

Therapeutic effects

Decreased production/accumulation of glycosphingolipid glucosylceramide with decreased tissue damage.

Pharmacokinetics

Absorption: Well absorbed following oral administration.
Distribution: Distributes into extravascular tissues.
Metabolism and Excretion: Not metabolized; excreted mostly unchanged in urine.
Half-life: 6–7 hr.

Time/action profile (blood levels)

ROUTEONSETPEAKDURATION
POunknown2–2.5 hr8 hr

Contraindications/Precautions

Contraindicated in: Hypersensitivity; Obstetric / Lactation: Pregnancy or lactation; Severe renal impairment (<30 mL/min).
Use Cautiously in: Mild to moderate renal impairment (dose alteration recommended if CCr <70 mL/min); Geriatric: Consider age related decrease in body mass, cardiac, renal and hepatic function, other chronic illnesses and concurrent drug therapies; Pediatric: Children <18 yr (safety not established).

Adverse Reactions/Side Effects

Central nervous system

  • headache (most frequent)

Gastrointestinal

  • abdominal pain (most frequent)
  • diarrhea (most frequent)
  • flatulence (most frequent)
  • nausea (most frequent)
  • weight loss (most frequent)
  • anorexia
  • dyspepsia

Genitourinary

  • ↓ male fertility

Hematologic

  • thrombocytopenia

Metabolic

  • weight loss (most frequent)

Neurologic

  • paresthesia
  • peripheral neuropathy
  • tremor

Interactions

Drug-Drug interaction

↑ clearance of imiglucerase (should not be used concurrently).

Route/Dosage

Oral (Adults) 100 mg three time daily at regular intervals.

Renal Impairment

Oral (Adults) CCr 50–70 mL/min- 100 mg twice daily; CCr 30–50 mL/min- 100 mg once daily.

Availability

Capsules: 100 mg

Nursing implications

Nursing assessment

  • Neurological evaluations should be performed at baseline and every 6 mo during therapy. If symptoms of peripheral neuropathy (numbness, tingling) occur, discontinuation of therapy may be considered.
  • Assess for tremor. May begin during first month of therapy and may resolve between 1–3 mo of therapy; may require discontinuation of therapy.
  • Assess for diarrhea and weight loss. Advise patients with diarrhea to avoid high carbohydrate foods.
  • Lab Test Considerations: May cause thrombocytopenia.

Potential Nursing Diagnoses

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

Implementation

  • Oral: Administer 3 times daily at regular intervals. Capsules should be swallowed whole with water. May be administered without regard to food.

Patient/Family Teaching

  • Instruct patient to take miglustat as directed at the same time each day. If a dose is missed, skip the dose and take the next capsule at the usual time. Advise patient that sharing of this medication may be dangerous.
  • Advise patient to notify healthcare professional immediately if pregnancy is planned or suspected or if breastfeeding. Instruct male patients to maintain a reliable form of contraception during and for at least 3 mo after therapy.
  • Advise patient to consult health care professional prior to taking and Rx or OTC mediations or herbal products concurrently with miglustat.
  • Instruct patient to notify healthcare professional if numbness, pain, or burning in the hands and feet occur or if tremor develops or existing tremor worsens.

Evaluation/Desired Outcomes

  • Decreases in spleen and liver volumes in patients with Gaucher's disease.

miglustat

a rarely used miscellaneous agent.
indications This drug is used to treat adults with mild to moderate type 1 Gaucher disease.
contraindications Pregnancy and known hypersensitivity to this drug prohibit its use.
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References in periodicals archive ?
Miglustat supply (DOE), Salmeterol (DOE), terbutaline (DOE), salmeterol Association (DOE / fluticasone (DOE) and Formoterol Association (DOE) / budesonide (DOE) for all service organizations Osakidetza.
An oral drug called miglustat worked in mice, but not in men.
An alternative form of therapy is substrate reduction; medication with miglustat (Zavesca) reduces the amount of glucocerebroside, allowing the patient's depleted residual glucocerebrosidase activity to cope with the reduced amount of substrate.
Sustained therapeutic effects of oral miglustat (Zavesca, Nbutyldeoxynojirimycin, OGT 918) in type I Gaucher disease.
Last month, the European Union approved a version of NB-DNJ called miglustat for Gaucher's disease, a debilitating genetic disorder.
Contract notice: Supply framework agreement miglustat (doe), salmeterol (doe), terbutaline (doe), salmeterol association (doe) / fluticasone (doe) and formoterol association (doe) / budesonide (doe for all service organizations osakidetza.
Actelion's Miglustat Proves Effective for Treating Cystic
Framework agreement with a single operator per lot for supplying drugs Bosentan (DOE), glatiramer (DOE), BCG (DOE), miglustat (DOE), Busulfan (DOE), recombinant Factor IX (DOE) and the combination of Delta -9 - Tetrahydrocannabinol and Cannabidiol.
Contract notice: Supplying drugs bosentan (doe), glatiramer (doe), bcg (doe), miglustat (doe), busulfan (doe), recombinant factor ix (doe) and the combination of delta -9 - tetrahydrocannabinol and cannabidiol.