Special Warning : אזהרת שימוש

4.4   Special warnings and precautions for use
CYSTAGON therapy must be initiated promptly after confirmation of the diagnosis of nephropathic cystinosis to achieve maximum benefit.

Nephropathic cystinosis must have been diagnosed by both clinical signs and biochemical investigations (leucocyte cystine measurements).

Cases of Ehlers-Danlos like syndrome and vascular disorders on elbows have been reported in children treated with high doses of different cysteamine preparations (cysteamine chlorhydrate or cystamine or cysteamine bitartrate) mostly above the maximal dose 1.95 g/m2/day. These skin lesions were associated with vascular proliferation, skin striae and bone lesions.
It is therefore recommended to monitor regularly skin and to consider X-ray examinations of the bone as necessary. Self-examination of the skin by the patient or the parents should also be advised. If any similar skin or bone abnormalities appear, it is recommended to decrease the dose of CYSTAGON.
The use of doses higher than 1.95g/m2/day is not recommended (see sections 4.2 and 4.8).

Monitoring of blood cell count is recommended on a regular basis.

Oral cysteamine has not been shown to prevent eye deposition of cystine crystals. Therefore, where cysteamine ophthalmic solution is used for that purpose, its usage should continue.

In contrast to phosphocysteamine, CYSTAGON does not contain phosphate. Most patients will already be receiving phosphate supplements and the dose of these may need to be altered when CYSTAGON is substituted for phosphocysteamine.

Intact CYSTAGON hard capsules should not be administered to children under the age of approximately 6 years due to risk of aspiration (see section 4.2).

Effects on Driving

4.7   Effects on ability to drive and use machines

CYSTAGON has minor or moderate influence on the ability to drive and use machines CYSTAGON may cause drowsiness. When starting therapy, patients should not engage in potentially hazardous activities until the effects of the medicinal product on each individual are known.