In the body, sodium phenylbutyrate is converted to a chemical that assists the kidneys in eliminating waste products. These waste products may generate ammonia, which is harmful if it accumulates in the blood.
Sodium phenylbutyrate is used to treat urea cycle problems in individuals who lack certain liver enzymes required for the normal elimination of waste products. Sodium phenylbutyrate prevents an ammonia buildup in the blood.
Additionally, sodium phenylbutyrate may be used for uses not covered in this prescription guide.
Warnings
Seek immediate medical assistance if you see any symptoms of ammonia accumulation, such as mood or behavior changes, memory loss, difficulty thinking, muscular twitches, back-and-forth eye movement, vomiting, growing weakness, slurred speech, convulsions (fits), or fainting.
Sodium phenylbutyrate will not alleviate the acute symptoms of a buildup of ammonia.
Prior to using this medication
If you are allergic to sodium phenylbutyrate, you should not use it. Sodium phenylbutyrate will not alleviate the symptoms of an ammonia buildup immediately.
To ensure sodium phenylbutyrate is safe for you, inform your doctor if you have any of the following:
- cardiac congestive failure;
- elevated blood pressure;
- serious renal disease;
- enlargement or fluid retention; or
- or another seizure problem
FDA pregnancy category C. It is unknown whether sodium phenylbutyrate is harmful to an unborn child. Inform your doctor if you are pregnant or want to become pregnant during treatment with this medication.
It is unknown whether sodium phenylbutyrate enters breast milk or if it might be harmful to a breastfeeding infant. Inform your physician if you are breastfeeding.
Do not provide this medication to a kid weighing less than 44 pounds unless instructed to do so by your doctor.
How should sodium phenylbutyrate be administered?
Follow all instructions on the label of your medication. Do not use this medication in bigger or lower doses or for a longer duration than prescribed.
Consume this medication with every meal. Divide your complete daily dosage across each of your meals.
Before ingesting, sodium phenylbutyrate powder should be combined with meals.
Before combining the powder with food, gently shake it.
Regular use of sodium phenylbutyrate will provide the most benefit. Refill your prescription before you run out of medication entirely. This medication may need to be used for the rest of your life.
If you experience any indications of infection, such as fever, chills, redness, swelling, body pains, or weakness, contact your doctor immediately. Even when using sodium phenylbutyrate, an infection might cause your health to spiral out of control.
While using sodium phenylbutyrate, regular blood tests may be necessary.
Sodium phenylbutyrate is simply one component of a therapy plan that may include involve nutrition, dialysis, and other drugs. Adhere strictly to your doctor’s directions.
Continue taking this medication even if you feel OK. Your illness may not manifest any symptoms. If the urea cycle problem is not treated, an accumulation of ammonia may lead to life-threatening adverse effects.
Temperature-controlled storage away from moisture and heat. Close the bottle firmly when not in use.
What is the effect of missing a dose?
Take the dosage as soon as you realize you missed it. If it is nearly time for your next scheduled dosage, skip the missed dose. Do not take more medication to compensate for a missing dose.
Ensure that Pure PBA is taken with meals.
What happens if I overdose?
Seek immediate medical treatment or reach the Poison Help line.
What should I avoid when using phenylbutyrate sodium?
Follow your doctor’s advice about any food, drink, or exercise limitations.
Sodium phenylbutyrate adverse reactions
Obtain immediate medical attention if you exhibit the following symptoms of an allergic reaction: hives; difficulty breathing; and swelling of the face, lips, tongue, or throat.
Even when treated, urea cycle abnormalities may lead to a dangerous buildup of ammonia in the blood. Seek immediate medical assistance if you see any symptoms of ammonia accumulation, such as mood or behavior changes, memory loss, difficulty thinking, muscular twitches, back-and-forth eye movement, vomiting, growing weakness, slurred speech, convulsions (fits), or fainting.
Phenylbutyrate sodium may induce severe adverse effects. Contact your doctor immediately if you have:
- excessive sleepiness and disorientation;
- edema, fast weight gain;
- a sense of dizziness, as though one would pass out;
or
- pale complexion, shortness of breath, and fast heart rate.
Common sodium phenylbutyrate adverse effects may include:
- skipped periods, alterations to your usual cycle;
- lack of hunger;
- migraine;
or
- strange or unpleasant flavor in the tongue.
This is not an exhaustive list of possible adverse effects; more may arise. Consult your physician for guidance on side effects.
Sodium phenylbutyrate adverse reactions (additional detail)
Dosage details for sodium phenylbutyrate
Usual Dose for Adults with Urea Cycle Disorders:
Administer orally with each meal or feeding (three to six times daily):
Less than 20 kilograms: 450 to 600 mg/kg/day
-Weight 20 kg or More: 9.9 to 13 g/m2 per day
Comments:
-Treatment should include dietary protein limitation and, in certain instances, necessary amino acid supplementation.
supplementation.
Uses:
-Adjunctive treatment in the chronic management of urea cycle disorders (UCDs) characterized by deficits in carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase.
-Used to treat neonatal-onset deficit (full enzyme deficiency, appearing within the first 28 days of birth) and late-onset illness (partial enzymatic deficiency, presenting beyond the first month of life) with a history of hyperammonemic encephalopathy.
Normal pediatric dose for disorders of the urea cycle:
Administer orally with each meal or feeding (three to six times daily):
Less than 20 kilograms: 450 to 600 mg/kg/day
-Weight 20 kg or More: 9.9 to 13 g/m2 per day
Comments:
-Treatment should include dietary protein limitation and, in certain instances, necessary amino acid supplementation.
supplementation.
-Patients weighing less than 20 kg should not take tablets.
Uses:
-Adjunctive treatment for the chronic management of UCDs associated with deficits in carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase.
-Used to treat neonatal-onset deficit (full enzyme deficiency, appearing within the first 28 days of birth) and late-onset illness (partial enzymatic deficiency, presenting beyond the first month of life) with a history of hyperammonemic encephalopathy.
Detailed information on sodium phenylbutyrate dose
What other medications interact with sodium phenylbutyrate?
Inform your doctor of any medications you take, including those you begin or discontinue taking during your sodium phenylbutyrate therapy, especially:
- divalproex (Depakote);
- haloperidol (Haldol); or
- valproic acid (Depakene, Stavzor).
This list is insufficient. Other pharmaceuticals, including prescription and over-the-counter medications, vitamins, and herbal items, may interact with sodium phenylbutyrate. Not every potential drug interaction is included in this medication guide.
