Sibnayal

Posology Dosing is based on age and weight. When initiating alkalising therapy, the target starting daily dose indicated below for each age group should be used and incrementally titrated to obtain the optimal dose that provides adequate metabolic acidosis control based on plasma bicarbonate levels.

5 mEq/kg/day to optimal dose 3 When switching from another alkalising therapy to Sibnayal, treatment should be initiated at the target dose used with the previous therapy (in mEq/kg/day) and titrated where necessary as described above.

The maximum dose, regardless of the age group, is either 10 mEq/kg/day or a total daily dose of 336 mEq, whichever is lower. The total daily dose should be administered in two intakes. For each individual patient, the nearest dose to the target dose should be fixed by combining whole sachets of the two available strengths.

In case of vomiting within two hours after intake, the patient should take another dose. The use of this medicinal product requires medical supervision. Mised dose If a dose is missed, it must be taken as soon as possible. However, if the next scheduled dose is due in less than six hours, the missed dose must be skipped.

Special populations Elderly No dose adjustment is required. 73 m². 73 m², the medicinal product should only be used if the potential benefits are considered to outweigh the potential risks (see Table 1). 73 m² Treatment of dRTA 45-59 • Plasma potassium levels in the normal ranges: A regular monitoring of renal function parameters and blood potassium levels is necessary at starting dose and after new dose increase or if any decrease of GFR.

4). • Elevated plasma potassium: Contraindicated ≤ 44 Contraindicated Hepatic impairment There is no need for specific target starting daily dose adjustment in patients with hepatic impairment. Paediatric population The safety and efficacy of potassium citrate/ potassium hydrogen carbonate in children below one year of age have not been established.

No data are available. Method of administration For oral use. The total daily dose is administered twice daily, typically twelve hours apart. The medicinal product must be taken orally, swallowed with a large glass of water. The full dose of granules per intake can be swallowed in several smaller portions, if necessary, but the content of each sachet must be entirely taken.

Summary of the safety profile The most frequently reported adverse reactions are abdominal pain (14 %, very common), upper abdominal pain (8 %, common) and gastrointestinal pain (2 %, common). Nausea (2 %, common) can be experienced at initiation of therapy.

Tabulated list of adverse reactions The list of adverse reactions is based on the experience in clinical trials. The frequency of adverse reactions is defined using the following convention: very common (≥1/10); common (≥1/100 to <1/10); uncommon (≥1/1 000 to <1/100); rare (≥1/10 000 to <1/1 000) and very rare (<1/10 000).

Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness: System Organ Class Frequency Adverse reaction Gastrointestinal disorders Very common Abdominal pain Common Upper abdominal pain, diarrhoea, dyspepsia, gastrointestinal disorder, gastrointestinal pain, nausea and vomiting Description of selected adverse reactions Gastrointestinal disorders Gastrointestinal pain, abdominal pain and upper abdominal pain were generally of mild or moderate intensity and resolved within 24 hours without the need to modify or stop the treatment.

All other gastro- intestinal adverse reactions (dyspepsia, vomiting, diarrhoea) were also of mild or moderate intensity, and resolved within 1 to 3 days, without modification or interruption of treatment. Paediatric population In clinical trials, although numbers were small, the safety profile was comparable in treated patients for adults (N= 16 healthy subjects and 7 dRTA patients) and paediatric population (N=27, including 10 adolescents (12-17 years old inclusive), 14 children (4-11 years old inclusive) and 3 infants (6 months – 3 years inclusive)).

Reporting of suspected adverse reactions Reporting suspected adverse reactions after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product. Healthcare professionals are asked to report any suspected adverse reactions via the national reporting system listed in Appendix V.

Hyperkalaemia and cardiotoxicity Potassium citrate/ potassium hydrogen carbonate should be used with caution in patients who have conditions pre-disposing them to hyperkalaemia, such as renal impairment, or crush syndrome, as a further rise in plasma potassium may lead to cardiac arrest.

Close monitoring of plasma potassium in patients at risk is required at starting dose and after new dose increase or in case of worsening of pre- existing disease. Then frequency is according to physicians’ criteria, but at least twice a year.

5). Gastrointestinal disorders The medicinal product should be used with caution in patients having gastro-intestinal disorders as they could affect efficacy and safety, such as malabsorption, delayed gastric emptying, diarrhoea, nausea, vomiting.

In such cases the blood bicarbonate levels should be regularly monitored and dose adjusted to maintain within normal ranges. The matrix of the granules can be found in the stools, which does not affect the efficacy or safety of the medicinal product.

73 m². 73 m², the medicinal product should only be used if the potential benefits are considered to outweigh the potential risks. 2). Special care should be taken in elderly people in whom renal function can be decreased. Potassium contents Sibnayal 8 mEq contains 308 mg of potassium per sachet.

This is to be taken into consideration if the patient has a reduced kidney function or if the patient is on a controlled potassium diet. 5 Sibnayal 24 mEq contains 924 mg of potassium per sachet. This is to be taken into consideration if the patient has a reduced kidney function or if the patient is on a controlled potassium diet.

1. 73 m². − Hyperkalaemia.