Soliris

eculizumab

(e-ku-liz-oo-mab) ,

Soliris

(trade name)

Classification

Therapeutic: hemostatic agents
Pharmacologic: complement inhibitors
Pregnancy Category: C

Indications

To reduce hemolysis in patients with paroxysmal nocturnal hemoglobinuria (PNH).To inhibit complement-mediated thrombotic microangiopathy in patients with atypical hemolytic uremic syndrome (aHUS).

Action

Eculizumab is a monoclonal antibody that binds to complement protein C5. This binding inhibits complement activation, a necessary step in the initiation of hemolysis due to PNH and the development of thrombotic microangiopathy in aHUS.

Therapeutic effects

Decreased hemolysis associated with PNH.
Reduced complement-mediated thrombotic microangiopathy in aHUS.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.
Distribution: Crosses placenta; enters breast milk.
Metabolism and Excretion: Unknown.
Half-life: 272 hr.

Time/action profile

ROUTEONSETPEAKDURATION
IVrapid end of infusion1–2 wk

Contraindications/Precautions

Contraindicated in: Unresolved serious Neisseria meninigitidis infection; Patients not vaccinated against Neisseria meninigitidis (unless risk of delaying treatment outweighs risks of developing a meningococcal infection) (vaccinate at least 2 wk prior to first dose).
Use Cautiously in: Systemic infection; Discontinuation requires monitoring for intravascular hemolysis; Obstetric: Use only if maternal benefit outweighs fetal risk; Lactation: Lactation; Pediatric: Safety and effectiveness not established for treatment of PNH.

Adverse Reactions/Side Effects

Central nervous system

  • headache (most frequent)
  • insomnia (most frequent)
  • fatigue

Cardiovascular

  • hypertension (most frequent)
  • tachycardia (most frequent)
  • peripheral edema

Ear, Eye, Nose, Throat

  • nasopharyngitis (most frequent)
  • sinusitis
  • vertigo

Respiratory

  • cough (most frequent)

Gastrointestinal

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

Musculoskeletal

  • back pain (most frequent)
  • extremity pain
  • myalgia

Hematologic

  • anemia (most frequent)
  • leukpenia (most frequent)

Miscellaneous

  • ↑ risk of meningococcal infections (life-threatening)
  • fever
  • Haemophilus influenzae infection
  • herpes simplex infection
  • influenza-like illness
  • Streptococcus pneumoniae infection

Interactions

Drug-Drug interaction

None known.

Route/Dosage

Paroxysmal Nocturnal Hemoglobinuria

Intravenous (Adults) 600 mg every 7 days for 4 doses, then 900 mg 7 days later, then 900 mg every 14 days.

Atypical Hemolytic Uremic Syndrome

Intravenous (Adults) 900 mg every 7 days for 4 doses, then 1200 mg 7 days later, then 1200 mg every 14 days.
Intravenous (Children <18 yr and ≥40 kg) 900 mg every 7 days for 4 doses, then 1200 mg 7 days later, then 1200 mg every 14 days.
Intravenous (Children <18 yr and 30–<40 kg) 600 mg every 7 days for 2 doses, then 900 mg 7 days later, then 900 mg every 14 days.
Intravenous (Children <18 yr and 20–<30 kg) 600 mg every 7 days for 2 doses, then 600 mg 7 days later, then 600 mg every 14 days.
Intravenous (Children <18 yr and 10–<20 kg) 600 mg, then 300 mg 7 days later, then 300 mg every 14 days.
Intravenous (Children <18 yr and 5–<10 kg) 300 mg, then 300 mg 7 days later, then 300 mg every 21 days.

Availability

Solution for IV administration (requires further dilution): 10 mg/mL

Nursing implications

Nursing assessment

  • Monitor for infusion reaction (anaphylaxis, infection, headache, anemia, fever) during and for at least 1 hr following infusion. If reaction occurs, solution may be slowed or stopped, if infused within 2 hr.
  • Monitor for early signs and symptoms of meningococcal infections. Evaluate immediately if infection is suspected. Consider discontinuation of therapy during treatment of serious meningococcal infections.
  • Patients who discontinue eculizumab are at increased risk for serious hemolysis (serum LDH levels > pretreatment level with either >25% absolute decrease in PNH clone size (in the absence of dilution due to transfer) in 1 wk or less, hemoglobin level <5 gm/dL, or decrease of >4 gm/dL in 1 wk or less; angina, change in mental status, 50% increase in serum creatinine level; or thrombosis. Monitor patients who discontinue therapy for at least 8 wks to detect serious hemolysis or other reactions. If serious reactions occur, consider blood transfusion (packed RBCs), or exchange transfusion if the PNH PBCs are >50% of total RBCs by flow cytometry, anticoagulation, corticosteriods, or reinstitution of eculizumab.
  • Lab Test Considerations: Serum LDH levels increase during hemolysis and may assist in monitoring effects of eculizumab, including response to discontinuation to therapy.

Potential Nursing Diagnoses

Fatigue (Indications)
Risk for infection (Adverse Reactions)

Implementation

  • Available through a restricted program only to enrolled prescribers. Call 1-888-SOLIRIS (1-888-765-4747) to enroll.
  • Vaccinate patient with meningococcal vaccine at least 2 wks prior to first dose; revaccinate according to current medical guidelines for vaccine use Children should also be vaccinated for Streptococcus pneumoniae and Haemophilus influenza type b (Hib).
  • Intravenous Administration
  • Intermittent Infusion: Withdraw required amount of eculizumab from vial into sterile syringe and transfer to an infusion bag. Diluent: Dilute with equal amount of drug volume to diluent of 0.9% NaCl, 0.45% NaCl, D5W, or LR. Final admixture is 60 mL for 300 mg, 120 mL for 600 mg dose, 180 mL for 900 mg dose, or 240 mL for 1200 mg dose. Concentration: 5 mg/mL. Gently invert bag to ensure thorough mixing; discard unused portion in vial. Prior to administration, allow admixture to adjust to room temperature; do not use microwave or water bath. Discard solutions that are discolored or contain particulate matter. Solution is stable for 24 hr at room temperature or if refrigerated.
  • Rate: Administer over 35 min via gravity feed, syringe pump, or infusion pump; total infusion time should not exceed 2 hr.

Patient/Family Teaching

  • Explain the risks and benefits of eculizumab to patient. Instruct patient to read the Medication Guide before starting therapy and with each dose in case of changes.
  • Instruct patient to obtain required meningococcal vaccination at least 2 wks prior to therapy and revaccination according to guidelines. Caution patient to report symptoms of meningococcal infection (moderate to severe headache with nausea or vomiting, headache, fever, or stiff neck or stiff back; fever of 103°F or 39.4°C or higher; fever and a rash; confusion; or severe muscle aches with flu-like symptoms; and eyes sensitive to light).
  • Advise patient to notify health care professional if pregnancy is planned or suspected.
  • Inform patient to carry Soliris Patient Safety Card describing symptoms which, if experienced, patient should seek medical evaluation, at all times.
  • Advise patient of potential for serious hemolysis if eculizumab is discontinued and of need for monitoring by health care professional for at least 8 wks following discontinuation. Caution patient to notify health care professional immediately if a large drop in red blood cell count causing anemia, confusion, chest pain, kidney problems, and blood clots occur.

Evaluation/Desired Outcomes

  • Reduced hemolysis resulting in improvements in anemia (increased hemoglobin stabilization, reduced need for RBC transfusions), less fatigue, and improved health-related quality of life.
  • Inhibition of complement-mediated thrombotic microangiopathy in patients with atypical hemolytic uremic syndrome.

Soliris

a trademark for eculizumab.
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References in periodicals archive ?
COMPANY NEWS: Shares of Alexion Pharmaceuticals (ALXN) have gained 5% after the company announced that its ALXN1210, a rare blood disorder drug being tested against Soliris, was successful in a late-stage study.
The Ministry of Health, Labour and Welfare in Japan has approved Soliris (eculizumab) as a treatment for patients with generalized myasthenia gravis who are anti-acetylcholine receptor antibody-positive and whose symptoms are difficult to control with high-dose intravenous immunoglobulin therapy or plasmapheresis, US-based Alexion Pharmaceuticals, Inc.
The subject matter is the ongoing deliveries of soliris, In the form and estimated quantity listed in annex 6 to this tender dossier.
M2 EQUITYBITES-August 23, 2017-European Commission approves Alexion Pharmaceuticals' extension of indication for Soliris
For example the HSE forks out up to [euro]400,000 for Soliris, which is used to treat rare blood disorders.
Soliris, marketed by Alexion Pharmaceuticals, is currently indicated to treat ultra-rare blood disorders, including paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome.
Hallal has played a principal role in leading the company's key growth initiatives, including the successful launches of Soliris (eculizumab) in PNH and aHUS and the building of Alexion's 50-country operating platform.
Sisters Soliris and Elbairis Gonzalez, who live on the same street as Bushes, said neighbors were worried for their safety.
Soliris Gonzalez, who live in the same street as the Bushes, said neighbours were worried for their safety.
Sisters Soliris and Elbairis Gonzalez, who live on the same street as the Bushes, said neighbors were worried for their safety.
But it emerged last night Lesley has been given a new lease of life by "orphan drug" Soliris, at a cost of PS250,000 a year.