Brineura

Brineura is used to slow the loss of the ability to walk in children with a form of Batten disease. This medication is administered by a healthcare professional.

Brineura Overview

Reviewed: May 4, 2017
Updated: 

Brineura is a prescription medication used to slow the loss of ambulation (ability to walk) in children 3 years of age and older with ceroid lipofuscinosis neuronal disease (CLN2), a form of Batten disease (a fatal nervous system disorder). 

Brineura belongs to a group of drugs called hydrolytic lysosomal N-terminal tripeptidyl peptidases. These help by replacing tripeptidyl peptidase 1 (TPP1), an enzyme patients with Batten disease are deficient in.

This medication comes in infusion form and is administered into the cerebrospinal fluid (CSF) via surgically implanted reservoir and catheter in the head (intraventricular access device), followed by intraventricular electrolyte injection, for approximately 4.5 hours.

Common side effects of Brineura include fever, heart electrical activity abnormalities including slow heart rate (bradycardia), hypersensitivity, decrease or increase in CSF protein, and vomiting.

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Uses of Brineura

Brineura is a prescription medication used to slow the loss of ambulation (ability to walk) in children 3 years of age and older with ceroid lipofuscinosis neuronal disease (CLN2), a form of Batten disease. CLN2 is a rare inherited nervous system disorder that affects essential motor skills, such as sitting and walking. 

This medication may be prescribed for other uses. Ask your doctor or pharmacist for more information.

Cerliponase alfa

For more information on this medication choose from the list of selections below.

Side Effects of Brineura

Serious side effects have been reported with Brineura. See the “Brineura Precautions” section.

Common side effects of Brineura include the following:

  • fever
  • heart electrical activity abnormalities including slow heart rate (bradycardia)
  • hypersensitivity
  • decrease or increase in CSF protein
  • vomiting
  • seizures
  • hematoma (abnormal collection of blood outside of a blood vessel)
  • headache
  • irritability
  • increased CSF white blood cell count (pleocytosis)
  • device-related infection
  • feeling jittery
  • low blood pressure

This is not a complete list of Brineura side effects. Ask your doctor or pharmacist for more information.

Tell your doctor if your child has any side effects that do not go away.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Brineura Interactions

No drug interactions have been studied by the manufacturer. However, you should tell your doctor about all the medicines your child takes including prescription and non-prescription medicines, vitamins, and herbal supplements. Not all drug interactions are known or reported and new drug interactions are continually being reported.

Brineura Precautions

Serious side effects have been reported with Brineura including the following:

  • Device‑Related Complications. Your child's healthcare provider will inspect your child's scalp to ensure the intraventricular access device is not compromised prior to each infusion. CSF samples should routinely be sent by your doctor for testing to detect device infections. Brineura cannot be used if there are signs of device leakage or infection.
  • Heart problems. Low blood pressure and/or slow heart rate may occur during and following the infusion of Brineura. Electrocardiogram (ECG) monitoring is performed during infusion in patients with a history of slow heart rate, conduction disorder, or with structural heart disease. Tell your healthcare provider right away if your child has some or all of the following symptoms of heart problems.
    • fatigue
    • lightheadedness
    • dizziness
    • nausea
    • blurred vision
  • Serious infusion reactions (including anaphylaxis or serum sickness-like reactions). Tell your doctor or get medical help right away if your child gets any of these symptoms during or after an infusion with Brineuro:
    • fever
    • vomiting
    • irritability
    • hives (red itchy welts) or rash
    • itching
    • shortness of breath, difficulty breathing, or wheezing
    • weakness
    • dizziness or feel faint
    • palpitations (feel like your heart is racing or fluttering)
    • chest pain

Brineura is contraindicated in patients with:

  • acute intraventricular access device-related complications (e.g., leakage, device failure, or device-related infection).
  • ventriculoperitoneal shunts (a medical device that relieves pressure on the brain caused by fluid accumulation).

Brineura Food Interactions

Medications can interact with certain foods. In some cases, this may be harmful and your doctor may advise you to avoid certain foods. In the case of Brineura, there are no specific foods that must be excluded from the diet when receiving this medication.

Inform MD

Before your child takes this medication, tell your doctor about all of your child's medical conditions. Especially tell your doctor if your child:

  • is allergic to Brineuro or to any of its ingredients
  • has or has had shunts used to drain extra fluid around the brain
  • has heart problems such as bradycardia (slow heart rate), conduction disorder, or structural heart disease

Tell your doctor about all the medicines your child takes including prescription and non-prescription medicines, vitamins, and herbal supplements.

Brineura and Pregnancy

No studies have been done in animals, and no well-controlled studies have been done in pregnant women. Brineuro should be given during pregnancy only if clearly needed.

Brineura and Lactation

It is not known if Brineuro crosses into human milk. Because many medications can cross into human milk and because of the possibility for serious adverse reactions in nursing infants with use of this medication, a choice should be made whether to stop nursing or stop the use of this medication. Your doctor and you will decide if the benefits outweigh the risk of using Brineuro.

Brineura Usage

Brineuro should be taken exactly as prescribed.

This medication is available in an injectable form to be infused directly into the cerebrospinal fluid (CSF) via surgically implanted reservoir and catheter in the head (intraventricular access device), followed by intraventricular electrolyte injection, by a healthcare professional.

The intraventricular access device must be implanted prior to the first infusion. It is recommended that the first dose is administered at least 5 to 7 days after device implantation. 

Aseptic technique must be strictly observed during preparation and administration of Brineuro.

Brineura is intended to be administered with the B Braun Perfusor® Space Infusion Pump System. The essential performance requirements for this syringe pump used to deliver Brineura are as follows: 

  • Delivery rate of 2.5 mL/hr with delivery accuracy of +/- 1 mL/hr
  • Compatible with 20 mL syringes provided in the Administration Kit for use with Brineura
  • Occlusion alarm setting to ≤ 281 mm Hg

Brineura and the intraventricular electrolytes are administered using the provided Administration Kit.

Brineura Dosage

This medication should be taken exactly as prescribed by your doctor. 

The dose your doctor recommends may be based on the following:

  • the condition being treated
  • other medical conditions your child has
  • other medications your child is taking
  • how your child responds to this medication
  • your child's weight

The recommended dose of Brineura in pediatric patients 3 years of age and older is 300 mg administered once every other week by intraventricular infusion, followed by electrolytes infusion.

Brineura Overdose

Brineuro is administered by a healthcare provider in a medical setting; it is unlikely that an overdose will occur. However, if overdose is suspected, seek emergency medical attention.