1) What is Pheburane®?
P=PALATABLE
P=PELLETS
Medunik USA offers Pheburane® through its UNIK Support Program. Please visit the Support section to learn more and to download the Enrollment Form to get your patient(s) started on Pheburane®.
Pheburane® coated pellets can be taken 2 different ways with food consumption (meal or snack):
* Administration of Pheburane® oral pellets with other foods has not been studied and is not recommended. Additionally, administration with soft food is only recommended in patients old enough to consume soft foods.
Yes, patients do require monitoring during treatment.
Increased exposure to phenylacetate, the major metabolite of Pheburane®, may be associated with neurotoxicity in patients with UCDs. If symptoms of vomiting, nausea, headache, somnolence or confusion are present in the absence of high ammonia levels, consider reducing the dose of Pheburane®.
Renal excretion of phenylacetylglutamine may induce urinary loss of potassium. Monitor serum potassium during therapy, and initiate appropriate treatment when necessary.
In order to decide if administration of Pheburane® is appropriate in patients with diseases that involve edema, calculate the total amount of sodium patients will be exposed to, based on their weight or body surface area.
Administration via gastrotomy or nasogastric tubes has not been evaluated.
Pheburane® is indicated as adjunctive therapy to standard of care, which includes dietary management, for the chronic management of adult and pediatric patients with urea cycle disorders (UCDs), involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC) or argininosuccinic acid synthetase (AS).
Limitations of Use
Episodes of acute hyperammonemia may occur in patients while on Pheburane®. Pheburane® is not indicated for the treatment of acute hyperammonemia, which can be a life-threatening medical emergency that requires rapid acting interventions to reduce plasma ammonia levels. Read more
Pheburane® is indicated as adjunctive therapy to standard of care, which includes dietary management, for the chronic management of adult and pediatric patients with urea cycle disorders (UCDs).
Limitations of Use
Pheburane® is not indicated for the treatment of acute hyperammonemia. Read more
INDICATION
Pheburane® is indicated as adjunctive therapy to standard of care, which includes dietary management, for the chronic management of adult and pediatric patients with urea cycle disorders (UCDs), involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC) or argininosuccinic acid synthetase (AS).
Limitations of Use
Episodes of acute hyperammonemia may occur in patients while on Pheburane®. Pheburane® is not indicated for the treatment of acute hyperammonemia, which can be a life-threatening medical emergency that requires rapid acting interventions to reduce plasma ammonia levels.
WARNINGS AND PRECAUTIONS
Neurotoxicity of Phenylacetate
Increased exposure to phenylacetate, the major metabolite of Pheburane®, may be associated with neurotoxicity in patients with UCDs. If symptoms of vomiting, nausea, headache, somnolence or confusion are present in the absence of high ammonia levels, consider reducing the dose of Pheburane®.
Hypokalemia
Renal excretion of phenylacetylglutamine may induce urinary loss of potassium. Monitor serum potassium during therapy, and initiate appropriate treatment when necessary.
Conditions Associated with Edema
In order to decide if administration of Pheburane® is appropriate in patients with diseases that involve edema, calculate the total amount of sodium patients will be exposed to, based on their weight or body surface area. If a patient develops new-onset edema or worsening edema while on treatment, discontinue administration of Pheburane® and initiate appropriate therapy.
Diabetes Mellitus, Hereditary Fructose Intolerance, Glucose-Galactose Malabsorption or Sucrase-Isomaltase Insufficiency
Avoid use of Pheburane® in patients with rare hereditary problems of fructose intolerance, glucose-galactose malabsorption or sucrose-isomaltase insufficiency.
ADVERSE REACTIONS
The most common adverse reactions associated with the use of Pheburane® (incidence > 3%) are menstrual dysfunction, decreased appetite, body odor and bad taste or taste aversion.
DRUG INTERACTIONS
OVERDOSAGE
Overdoses of Pheburane® exceeding ten-fold the maximum recommended dosage may produce emesis, CNS depression, metabolic acidosis with or without respiratory alkalosis, hypernatremia, hypokalemia, and hypophosphatemia. Symptoms of overdose overlap with those of acute hyperammonemia. If overdose occurs, discontinue Pheburane®, monitor plasma phenylacetate and ammonia levels closely, and institute appropriate emergency management.
To report suspected adverse reactions, contact Medunik USA at 1-844-884-5520 or [email protected].
Please read the Full Prescribing Information.References: