sodium phenylacetate/sodium benzoate

Urea cycle disorders (UCDs) are rare, genetic conditions that are passed down from members of your family. UCDs affect about 1 in 35,000 births in the U.S. They happen when proteins that turn ammonia into a waste product called urea either don't work correctly or are missing. This leads to a toxic buildup of ammonia in the blood. Symptoms can begin to appear shortly after birth and can include poor appetite, vomiting, irritability, and low energy. If left untreated, UCDs can cause coma and even death. Providers can check for UCDs through blood work. Treatments include medications that help lower ammonia levels in the blood.

Ammonul (sodium phenylacetate / sodium benzoate) is typically given in a healthcare setting by a provider. It should only be given through a tube inserted into a large vein in the neck, upper chest, or groin. This is known as a central line. It's different from a peripheral line, which is usually inserted into a small vein in the hand or arm. Ammonul (sodium phenylacetate / sodium benzoate) should never be given through a peripheral line, because it can cause burns. The dose of Ammonul (sodium phenylacetate / sodium benzoate) that you receive is determined by your healthcare provider based on your weight or body size. You'll typically receive an initial dose that's given over one and a half to two hours. Then, you'll receive another dose that's given over a longer period of time (usually 24 hours). This dose is continued until your ammonia levels are back within normal range or when you're able to tolerate food and medications taken by mouth.

The most common side effects of Ammonul (sodium phenylacetate / sodium benzoate) are vomiting, high blood sugar, low potassium, involuntary muscle contractions, and problems with thinking.

Yes, Ammonul (sodium phenylacetate / sodium benzoate) can cause damage to the brain, especially if taken for a long period of time or if doses need to be repeated often. This might result in tiredness, lightheadedness, headaches, confusion, hearing loss, and problems with memory. These side effects usually go away once you stop receiving the medication. Ammonul (sodium phenylacetate / sodium benzoate) might also cause too much sodium to build up in your blood. This can cause your body to hold onto water and lead to swelling, which might result in high blood pressure and worsening heart problems. People with heart or kidney problems are especially at risk for this side effect. Your provider will monitor your labs while you're receiving treatment with Ammonul (sodium phenylacetate / sodium benzoate) to make sure it's safe for you.