Dosing & Uses
Dosage Forms & Strengths
capsule
- 100mg
Interstitial Cystitis
Indicated for bladder pain associated with interstitial cystitis
100 mg PO q8hr
Administration: 1 hour before or 2 hours after meals with water
Reassess every 3 Months
Sickle Cell Disease (Orphan)
Orphan indication sponsors
- Vanguard Therapeutics, Inc; Eagle Trace Dr; Half Moon Bay, CA 94019
- TRF Pharma, Inc; 863 Mitten Road; Burlingame, CA 94010
Mucopolysaccharidosis (Orphan)
Brand name: Lysosan
Orphan designation for treatment of MPS type VI
Sponsor
- Plexcera Therapeutics, LLC; 4445 North Highway A1A, Suite 241; Vero Beach, FL 32963
<16 years: Safety and efficacy not established
Adverse Effects
1-10%
Alopecia (4%)
Diarrhea (4%)
Nausea (4%)
Headache (3%)
Abdominal pain (2%)
Indigestion (2%)
Postmarketing Reports
Rectal hemorrhage
Elevated LFTs
Anemia
Leukopenia
Partial thromboplastin time increased
Prothrombin time increased
Thrombocytopenia
Optic neuritis
Retinal hemorrhage
Pigmentary changes in the retina
Warnings
Contraindications
Hypersensitivity
Cautions
Alopecia is associated with pentosan polysulfate and with heparin products; alopecia began within the first 4 weeks of treatment in clinical trials; ninety-seven percent (97%) of cases of alopecia reported were alopecia areata, limited to a single area on the scalp
Retinal pigmentary changes
- Pigmentary changes in retina, reported as pigmentary maculopathy, identified with long-term use of drug
- Although most cases occurred after 3 years of use or longer, cases have been seen with a shorter duration of use; while the etiology is unclear, cumulative dose appears to be a risk factor
- Visual symptoms in reported cases included difficulty reading, slow adjustment to low or reduced light environments, and blurred vision
- Visual consequences of pigmentary changes are not fully characterized
- Use caution in patients with retinal pigment changes from other causes in which examination findings may confound appropriate diagnosis, follow-up, and treatment
- Obtain detailed ophthalmologic history in all patients prior to starting treatment; consider testing if there is a family history of hereditary pattern dystrophy, genetic
- For patients with pre-existing ophthalmologic conditions, a comprehensive baseline retinal examination (including color fundoscopic photography, ocular coherence tomography (OCT), and auto-fluorescence imaging) is recommended prior to starting therapy
- A baseline retinal examination (including OCT and autofluorescence imaging) is suggested for all patients within six months of initiating treatment and periodically while continuing treatment; if pigmentary changes in retina develop, re-evaluate risks and benefits of continuing treatment, since these changes may be irreversible
- Follow-up retinal examinations should be continued given that retinal and vision changes may progress even after cessation of treatment
Anticoagulant effects
- The drug is a weak anticoagulant (1/15 the activity of heparin) At a daily dose of 300 mg (n=128), rectal hemorrhage was reported as an adverse event in 6.3% of patients
- Bleeding complications of ecchymosis, epistaxis, and gum hemorrhage reported
- Patients undergoing invasive procedures or having signs/symptoms of underlying coagulopathy or other increased risk of bleeding (due to other therapies such as coumarin anticoagulants, heparin, tPA, streptokinase, high dose aspirin, or nonsteroidal anti-inflammatory drugs) should be evaluated for hemorrhage.
- Patients with diseases such as aneurysms, thrombocytopenia, hemophilia, gastrointestinal ulcerations, polyps, or diverticula should be carefully evaluated before starting therapy
- Exercise caution when administering therapy in patients who have a history of heparin-induced thrombocytopenia
Pregnancy & Lactation
Pregnancy Category: B
Lactation: not known if excreted in breast milk, use caution
Pregnancy Categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available.Pharmacology
Mechanism of Action
Low molecular weight heparin-like compound; elicits weak anticoagulant (1/15 activity of heparin) and fibrinolytic effects
MOA in cystitis unknown; drug appears to attach to the bladder wall mucosa where it may act as a buffer to protect tissues from irritating substances in the urine
Pharmacokinetics
Absorption: 6%
Half-Life, Elimination: 4.8 hr
Distribution: Uroepithelium of the genitourinary tract with lesser amounts found in the liver, spleen, lung, skin, periosteum, and bone marrow
Metabolism: Spleen and liver; partial depolymerization in the kidney to a large number of metabolites
Excretion: Feces (58% as unchanged drug) Urine (6% primarily as metabolites)
Images
Patient Handout
Formulary
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