Ucedane

Ucedane can help to eliminate greatly increased ammonia levels in plasma (increased content of ammonia in the blood). Ammonia is particularly harmful to the brain and, in severe cases, can lead to a reduced level of consciousness and coma. High ammonia values (hyperammonemia) may be due to

a deficiency of a specific liver enzyme, N-acetyl glutamate synthase. Patients with this rare disease are unable to eliminate nitrogen waste products that form after consuming protein. The disease persists throughout the affected patient’s lifetime and the need for this treatment is therefore lifelong.
isovaleric acidemia, methylmalonic acidemia, and propionic acidemia. Patients suffering from any of these diseases need treatment during the hyperammonemia crisis.

What you need to know before you take Ucedane

Do not take Ucedane

if you are allergic to carglumic acid or any of the other ingredients of this medicine (listed in section 6),
if you are breastfeeding.

Warnings and precautions

Talk to your doctor or pharmacist before taking Ucedane.

Ucedane treatment should be initiated under the supervision of a physician experienced in the treatment of metabolic disorders.

Your doctor will evaluate how you respond to treatment with carglumic acid before starting any long-term treatment.

The dose should be adjusted individually to maintain normal plasma levels of ammonia.

Your doctor will possibly prescribe the addition of the amino acid arginine or limit your intake of protein.

To monitor your condition and your treatment, your doctor may need to examine you regularly about your liver, kidneys, heart, and blood.

Other medicines and Ucedane

Tell your doctor or pharmacist if you are using, have recently used, or might be using other medicines.

Ucedane with food and drink

Ucedane should be taken by mouth before a meal or feeding.

The tablets should be dissolved in at least 5 to 10 ml of water and taken immediately.

Pregnancy and breastfeeding

The effects of Ucedane on pregnancy and the fetus are not known. If you are pregnant, think you may be pregnant, or are planning to have a baby, consult your doctor before using this medicine.

The excretion of carglumic acid into breast milk has not been studied in women. However, since the presence of carglumic acid in the milk of lactating rats has been shown, with the risk harmful effects on the fed pups, you must not breastfeed your baby if you are taking Ucedane.

Driving ability and use of machinery

Effects on the ability to drive motor vehicles and use machines are not known.

Ucedane contains sodium

This medicine contains less than 1 mmol sodium (23 mg) per maximum daily dose, i.e. it is almost “sodium-free”.

How to take Ucedane

Always take this medicine as directed by your doctor. Ask your doctor or pharmacist if you are unsure.

The initial daily dose is usually 100 mg per kilogram of body weight, up to a maximum of 250 mg per kilogram of body weight (for example, if you weigh 10 kg, you should take 1 g per day or 5 tablets of 200 mg). In the long term, the daily dose usually varies between 10 mg and 100 mg per kilogram of body weight for patients who are deficient in N-acetyl glutamate synthase.

Your doctor will determine the appropriate dose for you to maintain normal blood ammonia levels.

Ucedane should ONLY be given by mouth or by gastric tube (with a syringe, if necessary).

If the patient is in a hyperammonemia coma , Ucedane is given by rapidly pushing one dose through a syringe via the feeding tube inserted.

If you have taken too much Ucedane

Side effects such as tachycardia (increased heart rate), profuse sweating, increased bronchial secretion, increased body temperature and restlessness may occur. Consult your doctor or pharmacist.

If you forget to take Ucedane

Do not take double doses to make up for missed doses.

If you stop taking Ucedane

Do not stop taking Ucedane without telling your doctor.

If you have any further questions about this medicine, contact your doctor or pharmacist.

Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.

Common side effects ( may affect up to 1 in 10 people):

increased sweating

Uncommon side effects ( may affect up to 1 in 100 people):

bradycardia (decreased heart rate)
diarrhea
fever
increased transaminases (liver enzymes)
vomiting

Side effects with no known frequency (frequency cannot be calculated from available data):

rash

How to store Ucedane

Keep this medicine out of sight and reach of children.

Use before the expiry date stated on the blister pack and carton. The expiration date is the last day of the specified month.

No special storage instructions.

Medicines must not be thrown into the drain or among the household waste. Ask the pharmacist how to dispose of medicines that are no longer used. These measures will help to protect the environment.

Contents of the packaging and other information

Contents declaration

The active substance is carglumic acid. Each dispersible tablet contains 200 mg of carglumic acid.
Other ingredients are microcrystalline cellulose, anhydrous colloidal silicon dioxide, sodium stearyl fumarate (see section 2 “Ucedane contains sodium”), mannitol, povidone K28, crospovidone type B.

Appearance and package sizes of the medicine

Ucedane dispersible tablets are rod-shaped, white, and biconvex with three scores on both sides and engraved with “L/L/L/L” on one side.

The tablet is approximately 17 mm long and 6 mm wide.

The tablet can be divided into four equal doses.

The tablets are available in aluminum/aluminum blister packs in a carton.

There are 12 or 60 dispersible tablets in each pack.

Not all pack sizes may be marketed.

Marketing Authorisation Holder

Eurocept International BV

Trap goose 5

1244 RL Ankeveen

Netherlands

Manufacturer