What does kuvan do for PKU?

What does kuvan do for PKU?

KUVAN adds more BH4 and stimulates the PAH enzyme to process Phe in people with PKU. The PAH enzyme begins to convert Phe to tyrosine, and helps to keep your blood Phe levels low.

What is Kuvan used to treat?

KUVAN® (sapropterin dihydrochloride) Tablets for Oral Use and Powder for Oral Solution are prescription medicines used to lower blood Phe levels in adults and children over one month of age with a certain type of Phenylketonuria (PKU). KUVAN is to be used along with a Phe-restricted diet.

How do you take Kuvan?

Taking KUVAN Powder 500 mg Is as Easy as 1-2-3 KUVAN Powder dissolves completely in 15 seconds in water, apple juice, or a small amount of soft food such as applesauce or pudding.

Who manufactures Kuvan?

2013.157. KUVAN [package insert]. Novato, CA: BioMarin Pharmaceutical Inc; 2016.

What can you eat on a PKU diet?

The diet for PKU consists of a phenylalanine-free medical formula and carefully measured amounts of fruits, vegetables, bread, pasta, and cereals. Many people who follow a low phenylalanine (phe) food pattern eat special low protein breads and pastas.

What happens if someone with PKU eat protein?

A dangerous buildup of phenylalanine can develop when a person with PKU eats protein-rich foods, such as milk, cheese, nuts or meat, and even grains such as bread and pasta, or eats aspartame, an artificial sweetener. This buildup of phenylalanine results in damage to nerve cells in the brain.

What is the cost of Kuvan?

BioMarin said that Kuvan would cost an average of $57,000 a year. It would be less than that for small children but could be more than $200,000 for large adults getting the highest dose. The company, based in Novato, Calif., said it expected insurers to pay for the drug.

What can you eat if you have PKU?

Can you eat potatoes with PKU?

Vegetables: Starchy vegetables like white, sweet, purple potatoes (and fries), winter squash, corn, and peas should be eaten sparingly. Most other fresh or frozen vegetables are low in phenylalanine with the exception of the ones on the non-compliant list.

When do you take Kuvan tablets and powder?

KUVAN Tablets and Powder contain the same blood Phe-lowering medication. Both dosage forms are taken once a day (at the same time each day) with food—preferably your largest meal of the day. Don’t be concerned if you see small, undissolved pieces floating on top.

How many mg of sapropterin is in a tablet of Kuvan?

Each tablet contains 100 mg of sapropterin dihydrochloride. Tablets are round, off-white to light yellow, mottled, and debossed with “177”. Kuvan powder for oral solution is available as a unit dose packet containing 100 mg of sapropterin dihydrochloride and as a unit dose packet containing 500 mg of sapropterin dihydrochloride.

Which is the active ingredient in the drug Kuvan?

Kuvan (sapropterin dihydrochloride) is an orally administered Phenylalanine Hydroxylase activator (or PAH activator). Sapropterin dihydrochloride, the active pharmaceutical ingredient in Kuvan, is a synthetic preparation of the dihydrochloride salt of naturally occurring tetrahydrobiopterin (BH4).

How can you tell if Kuvan is working for You?

It is not possible to know whether KUVAN will work for you until you start taking it. Your clinic team will determine whether or not KUVAN is working by checking to see if your blood Phe level drops while you are on a low-Phe diet. Will KUVAN continue to work over time? Yes.