Golodirsen

Indications

Golodirsen is used for: Duchenne Muscular Dystrophy

Adult Dose

Duchenne Muscular Dystrophy Indicated for Duchenne muscular dystrophy (DMD) in patients who have a confirmed mutation of the dystrophin gene that is amenable to exon 53 skipping 30 mg/kg IV qWeek

Child Dose

Renal Dose

Renal impairment Renal clearance is reduced in non-DMD adults with renal impairment, based on estimated glomerular filtration rate (eGFR) calculated using the Modification of Diet and Renal Disease (MDRD) equation No specific dosage adjustment can be recommended for patients with DMD who have renal impairment based on eGFR Closely monitor patients with known renal function impairment during treatment

Administration

Contra Indications

Precautions

Hypersensitivity Hypersensitivity reactions, including rash, pyrexia, pruritus, urticaria, dermatitis, and skin exfoliation, reported, some requiring treatment If a hypersensitivity reaction occurs, institute appropriate medical treatment and consider slowing the infusion or interrupting therapy Renal toxicity Renal toxicity was observed in animals Although renal toxicity was not observed in the clinical studies, renal toxicity, including potentially fatal glomerulonephritis, has been observed after administration of some antisense oligonucleotides Monitor renal function during therapy

Pregnancy-Lactation

Pregnancy No human or animal data are available to assess use during pregnancy Lactation No human or animal data are available to assess effects on milk production, presence in milk, or effects on the breastfed infant

Interactions

Adverse Effects

Side effects of Golodirsen : >10% Headache (41%) Pyrexia (41%) Fall (29%) Abdominal pain (27%) Nasopharyngitis (27%) Cough (27%) Vomiting (27%) Nausea (20%) 1-10% >5% Administration site pain Back pain Pain Diarrhea Dizziness Ligament sprain Contusion Influenza Oropharyngeal pain Rhinitis Skin abrasion Ear infection Seasonal allergy Tachycardia Catheter site-related reaction Constipation Fracture Frequency Not Defined Hypersensitivity

Mechanism of Action

Binds to exon 53 of dystrophin pre-mRNA, resulting in exclusion of this exon during mRNA processing in patients with genetic mutations that are amenable to exon 53 skipping Exon 53 skipping is intended to allow for production of an internally truncated dystrophin protein in patients with genetic mutations that are amenable to exon 53 skipping