Elaprase

idursulfase

(i-dur-sul-fase) ,

Elaprase

(trade name)

Classification

Therapeutic: replacement enzyme
Pharmacologic: enzymes
Pregnancy Category: C

Indications

Treatment of Mucopolysaccharidosis II (MPS II, Hunter syndrome).

Action

Replaces an enzyme not present in Hunter syndrome. Absence of the enzyme results in cellular accumulation of aminoglycans, organomegaly and organ dysfunction.

Therapeutic effects

Improved walking capacity.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.
Distribution: Unknown.
Metabolism and Excretion: unknownnown.
Half-life: 44–48 min.

Time/action profile

ROUTEONSETPEAKDURATION
IVrapidend of infusionunknown

Contraindications/Precautions

Contraindicated in: No known contraindications.
Use Cautiously in: Compromised respiratory function/acute respiratory disease (increased risk of infusion reactions); Severe genetic mutations (↑ risk of serious adverse reactions)Patients at risk for fluid overload, acute respiratory disorder, cardiac or respiratory illness (↑ risk of compromised cardiac or respiratory function) Obstetric: Use in pregnancy only if clearly needed; Obstetric: Use cautiously in lactation; Pediatric: Children <16 mo (safety not established).

Adverse Reactions/Side Effects

Central nervous system

  • headache (most frequent)
  • malaise

Cardiovascular

  • hypertension

Gastrointestinal

  • dyspepsia

Dermatologic

  • cutaneous reactions (most frequent)

Local

  • infusion site swelling

Musculoskeletal

  • arthralgia (most frequent)

Miscellaneous

  • anaphylaxis (life-threatening)
  • fever (most frequent)

Interactions

Drug-Drug interaction

None noted.

Route/Dosage

Intravenous (Adults and Children ≥5 yr) 0.5 mg/kg weekly

Availability

Solution for IV infusion (requires dilution): 2 mg/mL

Nursing implications

Nursing assessment

  • Observe for signs and symptoms of anaphylaxis (rash, pruritus, laryngeal edema, wheezing) during and for 24 hrs after infusion. Keep epinephrine, an antihistamine, corticosteroids, and resuscitation equipment close by in case of anaphylactic reaction.
  • Monitor for infusion-related reactions (headache, fever, rash, pruritus, erythema, urticaria, hypertension. 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.

Potential Nursing Diagnoses

Impaired physical mobility (Indications)

Implementation

  • Patients with compromised respiratory function or acute respiratory disease are at increased risk of life-threatening infusion reactions. Consider delaying infusion in patients with concomitant acute respiratory and/or febrile illness.
  • Intermittent Infusion: Determine number of vials required for the dose ordered. If number of vials includes a fraction, round up to next whole number. Vials are for single use; discard remaining medication. Dilute each vial in 100 mL of 0.9% NaCl. Use an infusion set with a 0.2 micrometer filter for administration. Mix gently; do not shake solution. Solution is clear to slightly opalescent; do not administer solutions that are discolored or contain particulate matter. Solution may be stored in refrigerator for up to 48 hr. Discard solution of not administered or refrigerated within 8 hrs.
  • Rate: Administer at 8 mL/hr for first 15 min. If well tolerated, may increase by 8 mL/hr at 15 min intervals to administer total volume within desired time. Do not exceed 100 mL/hr. May slow, temporarily stop, or discontinue for that visit if infusion reactions occur. Administer total infusion over 1–3 hrs. May require longer times if infusion reactions occur; should not exceed 8 hrs.
  • Y-Site Incompatibility: Do not administer other medications or solutions in same tubing as idursulfase.

Patient/Family Teaching

  • Explain purpose of infusion to patient and parents.
  • Advise female patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.
  • Inform patient that a Hunter Outcome Survey was established to evaluate long term treatments. For information, visit www.elaprase.com or call 1–866–888–0660.

Evaluation/Desired Outcomes

  • Improved walking capacity in patients with Hunter syndrome.

Elaprase

a trademark for idursulfase.
Mentioned in ?
References in periodicals archive ?
Contract award notice: Delivery of elaprase to the independent public health care center in gostyn
During her career, Barbier has made significant contributions to several approved products such as Elaprase (Hunter syndrome), Aubagio (multiple sclerosis) and Firazyr (hereditary angioedema), and has led several investigational new drug applications for new chemical entities.
host disease Calaspargase pegol acute lymphoblastic leukemia Cinryze antibody mediated rejection Gattex short bowel syndrome SHP555 chronic constipation SHP620 cytomegalovirus infection Vonvendi Von Willebrand disease EARLY RESEARCH PROJECTS DRUG INDICATION SHP611 MLD SHP622 Friedreich's ataxia SHP 623 neuromyelitis optica SHP631 neurocognitive decline associated with Hunter syndrome SHP655 hereditary thrombotic thrombocytopenic purpura SHP656 hemophilia A TOP SELLING DRUGS DRUG INDICATION 2016 SALES (+/-%) Vyvanse ADHD $2,014 17% Hemophilia hematology $1,789 n/a Immunoglobulin immunology $1,144 n/a Therapies Lialda ulcerative colitis $792 16% Cinryze hereditary angioedema $680 10% Elaprase Hunter syndrome $589 7%
Shire said that product sales growth was generated from across its portfolio, particularly VYVANSE (up 27% to USD805m), ADDERALL XR (up 48% to USD533m), REPLAGAL (up 35% to USD475m), ELAPRASE (up 15% to USD465m), LIALDA/MEZAVANT (up 27% to USD372m) and VPRIV (up 79% to USD256m).
Therefore, comparison of the incidence of antibodies to Oncaspar[R] with the incidence of antibodies to other products may be misleading' Elaprase The data reflect the percentage of patients (idursulfase) whose test results were positive for antibodies to idursulfase in specific assays, and are highly dependent on the sensitivity and specificity of these assays.
Elaprase, which costs about 375,000 dollars a year, holds the second spot on Forbes' list, reports News.
But already mum Elaine Quinn, from High Heaton, is noticing small changes in the 10-year-old and it is hoped the drug, Elaprase, could go on to change his life.
The drug for Hunter Syndrome sufferers called Elaprase will be available by Christmas.
Room at the Top - More Treatments for Hunter Syndrome Needed Despite Elaprase Breakthrough, New Study Finds
The new Co-Pay Assistance Program will take effect immediately, and will apply to eligible ELAPRASE (idursulfase) patients and VPRIV patients in the US, the company added.
Tests have shown Elaprase could help minimise the effects of the incurable illness.