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United Kingdom
Drug details for : ZAVESCA
| Drug class description : | Type 1 Gaucher Disease |
| Generic Name : | Miglustat |
| Drug description : | Each capsule contains 100 mg miglustat. Capsule, hard. White capsules with "OGT 918" printed in black on the cap and "100" printed in black on the body. |
| Presentation : | Zavesca 100 mg hard capsules |
| Indications : | Zavesca is indicated for the oral treatment of mild to moderate type 1 Gaucher disease. Zavesca may be used only in the treatment of patients for whom enzyme replacement therapy is unsuitable (Special Precautions). |
| Adult Dosage : | Therapy should be directed by physicians who are knowledgeable in the management of Gaucher disease. The recommended starting dose for the treatment of patients with type 1 Gaucher disease is 100 mg three times a day. Dose reduction to 100 mg once or twice a day may be necessary in some patients because of diarrhoea. Zavesca can be taken with or without food. Renal Impairment Pharmacokinetic data indicate increased systemic exposure to miglustat in patients with renal impairment. In patients with an adjusted creatinine clearance of 50-70 ml/min/1.73 m2, administration should commence at a dose of 100 mg twice daily. In patients with an adjusted creatinine clearance of 30-50 ml/min/1.73 m2, administration should commence at a dose of one 100 mg capsule per day. Use in patients with severe renal impairment (creatinine clearance <30 ml/min/1.73 m2) is not recommended. Hepatic Impairment Zavesca has not been evaluated in patients with hepatic impairment. |
| Child Dosage : | There is no experience with the use of Zavesca in patients under the age of 18 and over the age of 70. The use in children and adolescents is not recommended. |
| Elderly Dosage : | There is no experience with the use of Zavesca in patients under the age of 18 and over the age of 70. The use in children and adolescents is not recommended. |
| Contra Indications : | Hypersensitivity to the active substance or to any of the excipients. |
| Special Precautions : | Although no direct comparisons with Enzyme Replacement Therapy (ERT) have been performed in treatment naïve patients, it appears that it would take longer to achieve an effect with Zavesca and there is no evidence of an efficacy or safety advantage over ERT. ERT is the standard of care for patients who require treatment for type 1 Gaucher disease. The efficacy and safety of Zavesca has not been evaluated in patients with severe Gaucher disease, defined as a haemoglobin concentration below 9 g/dl or a platelet count below 50 x 109/l or active bone disease. Approximately 30% of patients have reported tremor or exacerbation of existing tremor on treatment. These tremors were described as an exaggerated physiological tremor of the hands. Tremor usually began within the first month and in many cases resolved between 1 to 3 months during treatment. Dose reduction may ameliorate the tremor usually within days but discontinuation with treatment may sometimes be required. Regular monitoring of vitamin B12 level is recommended because of the high prevalence of vitamin B12 deficiency in patients with type 1 Gaucher disease. Cases of peripheral neuropathy have been reported in patients treated with Zavesca with or without concurrent conditions such as vitamin B12deficiency and monoclonal gammopathy. All patients should undergo baseline and repeat neurological evaluation. Patients who develop symptoms such as numbness and tingling should have a careful re-assessment of risk-benefit and may require cessation of treatment. Isolated cases of cognitive dysfunction have been reported during clinical trials of Zavesca in type 1 Gaucher disease. Baseline and periodic assessment of cognitive functions is recommended in all patients on Zavesca treatment. Male patients should maintain reliable contraceptive methods whilst taking Zavesca. Studies in the rat have shown that miglustat adversely affects spermatogenesis, sperm parameters and reduces fertility. Until further information is available, it is advised that before seeking to conceive, male patients should cease Zavesca and maintain reliable contraceptive methods for 3 months thereafter. Due to limited experience, Zavesca should be used with caution in patients with renal or hepatic impairment. There is a close relationship between renal function and clearance of miglustat. Exposure of miglustat is markedly increased in patients with severe renal impairment. At present, there is insufficient clinical experience in these patients to provide dosing recommendations. Use in patients with severe renal impairment (creatinine clearance < 30 ml/min/1.73 m2) is not recommended. |
| Interactions : | Limited data suggest that co-administration of Zavesca and Cerezyme mayresult in decreased exposure to miglustat (approximately a 22% reduction in Cmax and a 14% reduction in AUC was observed in a small parallel-group study).This study also indicates that Zavesca has no or limited effect on the pharmacokinetics of Cerezyme. |
| Adverse Reactions : | Adverse events reported in clinical trials with Zavesca in 82 patients are listed in the table below by body system and frequency (very common: >1/10, common >1/100, <1/10). Most events were of mild to moderate severity. Metabolism and Nutrition Disorders: Very Common Weight loss Common Decreased appetite, weight increase Nervous System Disorders: Very Common Tremor, dizziness, headache, leg cramps Common Paraesthesia, peripheral neuropathy, cognitive dysfunction Eye Disorders: Very Common Visual disturbance Gastrointestinal Disorders: Very Common Diarrhoea, flatulence, abdominal pain, nausea, constipation, vomiting Common Dyspepsia, distension Weight loss has been observed in approximately 60% of patients. The nadir was at 12 months with a mean weight loss of 6-7% of body weight and a subsequent tendency for an increase in weight towards the baseline value. Gastrointestinal events have been observed in more than 80% of patients either at the outset of treatment, or intermittently during treatment. The majority of cases are mild and are expected to resolve spontaneously or after dose reduction. The mechanism is probably inhibition of disaccharidases in the gastrointestinal tract. The diarrhoea responds to loperamide. |
| Manufacturer : | Actelion Pharmaceuticals UK |
| Drug Availability : | (POM) |
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