Adult Dosing

Nephropathic Cystinosis

>12 yrs and >110 lbs (Cystagon)

• Initial dose: 1/4 to 1/6 of the maintenance dose in divided doses increased gradually over 4-6 wks to maintenance dose
• Maintenance dose: 500 mg PO qid

• Start therapy once the diagnosis is confirmed
• Target levels of leukocyte cystine should be <1 nmol/1/2 cystine/mg protein 5-6 hrs after administration

Nephropathic Cystinosis

>6 yrs (Procysbi)

• Initial dose: 1/4 to 1/6 of the maintenance dose in divided doses increased gradually over 4-6 wks to maintenance dose
• Maintenance dose: 1.3 gram/m²/day PO divided q12hr; may increase to 1.95 g/m²/day

Note:

• Increase dose if WBC cystine level remains higher than the target, or cysteamine concentration has not been achieved
• Take at least 2 hr after and at least 30 minutes before eating

Pediatric Dosing

Nephropathic Cystinosis

<12 yrs

• Initial dose: 1/4 to 1/6 of the maintenance dose in divided doses increased gradually over 4-6 wks to maintenance dose
• Maintenance dose: 1.3 g/m²/day PO divided qid

Note:

• Capsules should not be administered to children < 6 yrs of age due to the risk of aspiration. It can be given by sprinkling the capsule contents over food

• Management of nephropathic cystinosis

• Hypersensitivity to any of the ingredients of the product or penicillamine

• N/A

Renal Dose Adjustment

• Renal impairment: Use cautiously; dose adjustments not defined

Hepatic Dose Adjustment

• Hepatic impairment: Dose adjustments not defined

See Supplemental Patient Information

• If a skin rash develops, withhold therapy until the rash resolves; restart therapy at lower dose under close supervision and then titrate slowly to therapeutic dose. Do not re-administer therapy in case of multiforme bullosa or toxic epidermal necrolysis
• Patient should be carefully examined and the dose adjusted as required if CNS symptoms such as seizures, lethargy, somnolence, depression, and encephalopathy occur
• Patients receiving therapy have reported bleeding and gastrointestinal ulceration as well as gastrointestinal tract symptoms including nausea, vomiting, anorexia and abdominal pain
• Inform patients about the signs and symptoms of serious GI toxicity
• There have been reports of reversible leukopenia and abnormal liver function studies; monitor CBC and LFTs
• Monitor leukocyte cystine measurements 5-6 hrs after dose administration, then q3 months or more frequently if patients are transferred from phosphocysteamine solutions or cysteamine hydrochloride; target levels of leukocyte cystine should be <1 nmol/1/2 cystine/mg protein
• Patients treated with high doses of cysteamine reported serious skin lesions, skin striae, bone lesions, leg pain and joint hyperextension. Skin and bones of patients receiving cysteamine should be monitored routinely; reduce the dosage if skin or bone abnormalities appear
• Benign intracranial hypertension (pseudotumor cerebri; PTC) and/or papilledema has been reported in patients receiving the therapy, monitor carefully for signs and symptoms of PTC, headache, tinnitus, dizziness, nausea, diplopia, blurry vision, loss of vision, pain behind the eye or pain with eye moveme
• Ehler-Danlos syndrome has been reported in patients treated with high doses of immediate-release cysteamine bitartrate or other cysteamine salts

Cautions: Use cautiously in

• Hypertension
• Renal impairment
• Pseudotumor cerebri (PTC)
• GI bleeding

Supplemental Patient Information

• Advise patients to report symptoms such as headache, tinnitus, dizziness, nausea, diplopia, blurry vision, loss of vision, pain behind the eye or pain with eye movement, and signs of gastrointestinal ulceration and bleeding
• Warn patients against performing hazardous activities until the effects of drug on mental performance are known

Pregnancy Category:C

Breastfeeding: Safety unknown; is excreted in breastmilk of lactating rats. Because of the potential for possible serious adverse reactions in nursing infants, a decision should be made whether to discontinue nursing or to discontinue the drug, taking into account the importance of the drug to the mother.

• CNS: Somnolence, encephalopathy, headache, seizures, ataxia, confusion, tremor, hyperkinesia, dizziness, jitteriness
• CV: Hypertension
• PSYCHIATRY: Nervousness, abnormal thinking, depression, emotional lability, hallucinations, nightmares
• GI: Nausea, vomiting, bad breath, abdominal pain, dyspepsia, constipation, gastroenteritis, duodenitis, gastrointestinal ulceration, bleeding, diarrhea, anorexia
• EENT: Decreased hearing, loss of vision, blurry vision, tinnitus, pain with eye movement
• HEMA: Anemia, leukopenia
• LABTESTS: Abnormal liver function, anemia, leukopenia
• DERM: Rash, erythema multiforme bullosa, epidermal necrolysis, urticaria
• GU: Interstitial nephritis, renal failure
• MISC: Fever, lethargy, dehydration

• Cystine-depleting agent; prevents the accumulation of intracellular cystine by favoring conversion to cysteine and cysteine compounds that are able to leave the intracellular space
• Metabolism: Unknown
• Excretion: Unknown
• Half-life: Unknown

US Trade Name(s)

• Cystagon
• Procysbi

US Availability

Cystagon

• CAPS: 50, 150 mg

Procysbi

• Delayed release CAPS: 25, 75 mg

Canadian Trade Name(s)

• N/A

Canadian Availability

• N/A

UK Trade Name(s)

• Cystagon

UK Availability

Cystagon

• CAPS: 50, 150 mg

Australian Trade Name(s)

• Cystagon

Australian Availability

Cystagon

• CAPS: 50, 150 mg

Urologic

Other Urologics

Cystine-depleting Agent

Drug Name: Cystagon 150 MG Oral Capsule

Ingredient(s): Cysteamine

Imprint: CYSTAGON;150;MYLAN

Color(s): White

Shape: Capsule

Size (mm): 22.00

Score: 1

Inactive Ingredient(s): ammonium hydroxide / black iron oxide / colloidal silicon dioxide / croscarmellose sodium / gelatin / magnesium stearate / microcrystalline cellulose / pharmaceutical glaze / pregelatinized starch / propylene glycol / silicon dioxide / sodium lauryl sulfate / titanium dioxide

Drug Label Author:
Mylan Pharmaceuticals Inc.

DEA Schedule:
Non-Scheduled

Drug Name: Cystagon 50 MG Oral Capsule

Ingredient(s): Cysteamine

Imprint: CYSTA;50;MYLAN

Color(s): White

Shape: Capsule

Size (mm): 16.00

Score: 1

Inactive Ingredient(s): ammonium hydroxide / black iron oxide / colloidal silicon dioxide / croscarmellose sodium / gelatin / magnesium stearate / microcrystalline cellulose / pharmaceutical glaze / pregelatinized starch / propylene glycol / silicon dioxide / sodium lauryl sulfate / titanium dioxide

Drug Label Author:
Mylan Pharmaceuticals Inc.

DEA Schedule:
Non-Scheduled