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PROCYSBI is a brand name for Cysteamine, supplied as a capsule (delayed release). The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: AND CLINICAL USE ..............................................................................3 CONTRAINDICATIONS ...................................................................................................3 WARNINGS AND PRECAUTIONS…
Verbatim from this product's HC label. Tap a section to expand.
0%). 1%) In Study 03, one serious adverse event (SAE), abdominal discomfort in a patient receiving treatment with PROCYSBI, was considered drug-related. In Study 04, six SAEs were assessed as drug-related: vomiting (two SAEs), renal failure, constipation, diarrhea and acute gastroenteritis.
7%). Clinical Trial Adverse Drug Reactions Because clinical trials are conducted under very specific conditions, the adverse reaction rates observed in the clinical trials may not reflect the rates observed in practice and should not be compared to the rates in the clinical trials of another drug.
Adverse drug reaction information from clinical trials is useful for identifying drug-related adverse events and for approximating rates. 19 grams/m2 per day. All patients were transitioned from immediate-release cysteamine bitartrate to PROCYSBI.
Forty-three patients, aged 7 to 24 years, received PROCYSBI in an 9-week, open- label, randomized sequence, cross-over trial comparing 3 weeks of treatment with PROCYSBI to 3 weeks of treatment with immediate-release cysteamine bitartrate (Study 03).
Forty of 43 patients continued PROCYSBI treatment in an open-label, uncontrolled extension trial (36 patients were treated with PROCYSBI for longer than 2 years of which 20 patients were treated for longer than 5 years) (Study 04).
An additional 19 patients (6 patients with a renal transplant, and 13 patients aged 2 to 6 years) were enrolled directly into this trial (12 patients were treated with PROCYSBI for longer than 2 years of which 9 patients were treated longer than 5 years).
6%) in Study 03 experienced one or more treatment-emergent adverse events (TEAEs) that were assessed as treatment-related. ADRs reported with a frequency of ≥ 1% are shown in Table 1. In the long-term extension trial, Study 04, 37 patients (63%) experienced one or more TEAEs which were assessed as treatment-related.
ADRs reported with a frequency of ≥ 1% are shown in Table 2. ADRs are consistent with those reported in Study 03 and with those previously described for immediate-release cysteamine bitartrate. ADRs within the subpopulations of patients ≤ 6 years of age (n=13) and in the renal transplant recipients (n=6) suggest a similar safety profile to that observed in patients from Study 03 (n=40).
General Ehlers-Danlos-like Syndrome:
Skin and bone lesions that resemble clinical features of Ehlers- Danlos syndrome have been reported in patients treated with high doses of immediate-release cysteamine bitartrate or other cysteamine salts. These include purplish hemorrhagic lesions (which have been described as molluscoid pseudotumors), skin striae, bone lesions (including osteopenia, compression fractures, scoliosis and genu valgum), leg pain and joint hyperextension.
One patient on immediate-release cysteamine bitartrate with serious skin lesions subsequently died of acute cerebral ischemia with marked vasculopathy. Monitor patients for development of skin or bone lesions and interrupt PROCYSBI dosing if patients develop these lesions.
PROCYSBI may be restarted at a lower dose under close supervision, then slowly increased to the appropriate therapeutic dose. Gastrointestinal Gastrointestinal (GI) ulceration and bleeding have been reported in patients receiving immediate- release cysteamine bitartrate.
GI tract symptoms including nausea, vomiting, anorexia and abdominal pain, sometimes severe, have been associated with PROCYSBI. Physicians should remain alert for signs of ulceration and bleeding and should inform patients and/or guardians about the signs and symptoms of serious GI toxicity and what steps to take if they occur.
If severe GI tract symptoms develop, consider decreasing the dose of PROCYSBI. Strictures of the ileo-caecum and large bowel (fibrosing colonopathy) was first described in cystic fibrosis patients who were given high doses of pancreatic enzymes in the form of tablets with an enteric coating of methacrylic acid- ethyl acrylate copolymer, one of the excipients in PROCYSBI.
As a precaution, unusual abdominal symptoms or changes in abdominal symptoms should be medically assessed to exclude the possibility of fibrosing colonopathy. Hepatic PROCYSBI has not been studied in patients with hepatic impairment.
Not medical advice. Always read the patient information leaflet and follow your prescriber or pharmacist.
Other brands of Cysteamine in Canada.
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PROCYSBI® cysteamine delayed-release capsules Page 9 of 42 Seventeen cysteamine-naïve patients in Study 08 (fifteen patients between the ages of 1 and 5 years, one 9-year old and one 22-year old) received PROCYSBI in an open-label clinical trial.
Adverse reactions occurring in at least 2 patients (>10%) were: breath odor (n=4) and vomiting (n=4). Adverse events which occurred in >10% of patients are shown in Table 3. Abnormal Hematologic and Clinical Chemistry Findings There were no changes observed in laboratory tests results for PROCYSBI during the clinical trials beyond that expected with nephropathic cystinosis.
3) 0 (0) Note: A patient is counted once if he/she reported one or more events. Percentages are based on the number of patients in the safety population within each treatment group. 0. a Defined as treatment emergent adverse events that have been assessed by the study Investigator to be possibly or probably related to study treatment.
b Use of gastric acid reducing medications, including proton pump inhibitors, was allowed during treatment with immediate-release cysteamine bitartrate but restricted to intolerable gastric upset during PROCYSBI treatment. 7%) Infections […]
Closer monitoring of the WBC cystine levels is recommended in these patients.
Monitoring and Laboratory Tests:
White blood cell (WBC) cystine levels WBC cystine levels should be routinely monitored to assess the effect of PROCYSBI treatment on intracellular cystine depletion. Refer to the assay-specific therapeutic target for cystine depletion provided by individual testing laboratories.
0 nmol ½ cystine/mg protein. g. granulocyte method) have different treatment targets. PROCYSBI® cysteamine delayed-release capsules Page 5 of 42 Obtain blood samples for WBC cystine concentration measurement at drug trough (as close to 30 minutes post dosing as possible).
See DOSAGE AND ADMINISTRATION. In addition, it is important to accurately record the time of the last dose, the actual dose consumed, and the time the blood sample was taken. The recommended frequency of monitoring WBC cystine concentration is as follows: • Cysteamine-naïve patients 1 year to less than 6 years: Obtain measurement two weeks after initiation of PROCYSBI treatment and continue monitoring during dosage titration period until the therapeutic target WBC cystine concentration is achieved.
Once the therapeutic target is achieved, continue monitoring monthly for 3 months, then quarterly for 1 year, and then twice yearly, at a minimum. • Cysteamine-naive patients greater than 6 years: Obtain measurement every two to four weeks while titrating the dose of PROCYSBI until reaching the maintenance PROCYSBI dose (see Table 5, DOSAGE AND ADMINISTRATION for maintenance doses), then monthly for 3 months, quarterly for 1 year, and twice-yearly thereafter, at a minimum.
• Patients switching from immediate-release cysteamine to PROCYSBI: Obtain measurement every two weeks while titrating the dose of PROCYSBI, quarterly for 6 months, and twice yearly thereafter, at a minimum. More frequent monitoring of WBC cystine concentration is recommended when drugs that increase the gastric pH are introduced and when dose adjustments occur.
See DRUG INTERACTIONS Because the measured WBC cystine concentration depends on the assays used for cystine and total protein content, individual patient sample concentration values from different assays and laboratories may not be interchangeable.
Consideration of assay results must be made with knowledge of the specific assays used. Therefore, communication should be maintained with the laboratory performing the assay. Leukopenia Cysteamine, as an immediate-release formulation, has been associated with reversible leukopenia.
Monitor WBC counts. If WBC levels remain abnormally decreased, consider decreasing the dose or discontinuing PROCYSBI until values revert to normal. Alkaline Phosphatase Cysteamine, as an immediate-release formulation, has been associated with elevated alkaline phosphatase levels.
Monitor alkaline phosphate levels. If values remain elevated, consider decreasing the dose or discontinuing PROCYSBI until values revert to normal. PROCYSBI® cysteamine delayed-release capsules Page 6 of 42 Neurologic Central nervous system (CNS) symptoms such as seizures, lethargy, somnolence, depression, and encephalopathy have been associated with immediate-release cysteamine bitartrate.
Carefully evaluate and monitor patients who develop CNS symptoms. Interrupt medication or adjust the dose as necessary for patients with severe symptoms or with symptoms that persist or progress. Benign intracranial hypertension (pseudotumor cerebri; PTC) and/or papilledema have been reported in patients receiving immediate-release cysteamine bitartrate treatment.
Physicians should monitor patients for signs and symptoms of PTC, including headache, tinnitus, dizziness, nausea, diplopia, blurry vision, loss of vision, pain behind the eye or pain with eye movement. If signs/symptoms persist, interrupt dosing or decrease the dose and refer the patient to an […]