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Sapropterin

Sapropterin is a synthetic form of BH4 (a natural enzyme helper) used to lower high phenylalanine levels in adults and children with BH4-responsive phenylketonuria (PKU).

Brand names KuvanJavygtorZelvysiasapropterin dihydrochloride
Drug class Phenylalanine Hydroxylase Activator
Rx Forms 2
Dr. Brian Staiger, PharmD, BCPS
Reviewed by Last reviewed Jul 10, 2026 Content updated Oct 2, 2026
🧬 Where this information comes from Click to expand

The clinical label records used on this page are FDA-filed Structured Product Labeling (SPL) retrieved through openFDA. We identified 16 clinical label sets within the page’s selected clinical scope after exact ingredient or ingredient-combination matching and applicable route/form matching. Forms, routes, brands, labelers, strengths, and NDC product-record counts are built separately from matching FDA National Drug Code Directory records. The linked DailyMed document is a representative official source for verification, not the page’s only clinical source. View the linked label on DailyMed →

✓No current FDA shortage listed · checked Oct 6, 2026 🚨Class I recall on record · 2
Sapropterin at a glance The quick version — the full detail is in the Patient Information Guide below.
Dr. Brian Staiger, PharmD, BCPS
The Pharmacist’s Take By · Clinical pharmacist · Written

Sapropterin (Kuvan) is a lab-made copy of BH4, the helper molecule the phenylalanine-clearing enzyme needs, and lowers blood phenylalanine in BH4-responsive phenylketonuria (PKU) by boosting whatever enzyme activity remains; it is a specialist medicine, on the market since about 2007 and available generically. Most people tolerate it well, with headache, a runny or stuffy nose, and sore throat the usual complaints. Stop it and get emergency care for signs of a severe allergic reaction (wheezing, trouble breathing, flushing, rash), most likely in the first few days. It works only in some people, so blood phenylalanine checks decide whether it stays, and it never replaces the diet.

Clinical pearls

  • Take it with a meal at the same time daily; tablets can be swallowed, dissolved in liquid, or stirred into soft food.
  • Report pain when swallowing, chest or stomach pain, or nausea that won't quit, since it can inflame the esophagus or stomach.
  • Tell every prescriber you take it before starting levodopa, methotrexate, valproic acid, trimethoprim, or sildenafil-type erection drugs, which need extra monitoring.
  • Blood phenylalanine can fall too low in young children, which can harm development, so their checks are more frequent.
How we write & review this content — our editorial policy →
How you get itPrescription only
Comes asOral solution, Tablet
Earliest FDA record2007
Companies listing it with the FDA10
FDA label last updatedAug 26, 2024
FDA’s strongest warning (boxed)None on the label

Patient information guide A plain-language walkthrough of Sapropterin, written from its FDA label.

What Sapropterin is used for

▾

Sapropterin is approved to lower high blood phenylalanine (Phe) levels in patients with BH4-responsive PKU (phenylketonuria). All supported products share this indication:

  • Kuvan (oral solution powder and tablets) reduces blood Phe levels in adults and children one month of age and older with hyperphenylalaninemia due to BH4-responsive PKU, used together with a phenylalanine-restricted diet
  • Javygtor (oral solution and tablets) reduces blood Phe levels in adults and children one month of age and older with BH4-responsive PKU, used together with a phenylalanine-restricted diet
  • Zelvysia (oral solution) reduces blood Phe levels in adults and children one month of age and older with BH4-responsive PKU, used together with a phenylalanine-restricted diet
  • Sapropterin dihydrochloride (oral solution powder and tablets, generic) reduces blood Phe levels in adults and children one month of age and older with BH4-responsive PKU, used together with a phenylalanine-restricted diet
  • Treatment must always accompany a phenylalanine-restricted diet — it does not replace dietary management
  • Only patients whose PKU responds to BH4 benefit; response must be confirmed through a supervised therapeutic trial, as it cannot be predicted by genetic testing alone

How Sapropterin works

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Sapropterin is a synthetic copy of BH4 (tetrahydrobiopterin), the natural cofactor — think of it as a helper molecule — that the enzyme phenylalanine hydroxylase (PAH) needs to break down the amino acid phenylalanine. In PKU, PAH activity is absent or severely reduced, so phenylalanine builds up to harmful levels. By providing extra BH4, sapropterin can switch on whatever residual PAH activity a patient still has, improving the body's ability to metabolize phenylalanine and bringing blood Phe levels down.

Not everyone with PKU has enough remaining enzyme activity to respond, which is why a trial period is required to confirm the medication is working.

Sapropterin dosage

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Sapropterin is taken orally once a day, with a meal, at roughly the same time each day. It is available as tablets (which can be swallowed whole, mixed into soft food, or dissolved in liquid) and as a powder for oral solution (mixed into soft food or liquid before swallowing). Dosing is based on body weight and is adjusted by the prescribing physician; younger children and older patients may start at different weight-based doses.

  • Take with food — food improves absorption, particularly with intact tablets
  • Take at the same time every day to keep blood Phe levels stable
  • If you miss a dose, take it as soon as you remember, but never double up on the same day
  • Blood phenylalanine levels must be monitored regularly, especially in children, to make sure the dose is correct and to guide dietary adjustments

Dosing details from the FDA-approved label

From the Sapropterin prescribing information (“Dosage and Administration”). Doses are set by your prescriber for your product, condition, kidney function and age — never change a dose on your own.

  • Pediatric patients 1 month to 6 years (starting dose)
    • 10 mg/kg taken once daily
    • Take with a meal, preferably at the same time each day; patients weighing 10 kg or less use 100 mg packets; must also follow a Phe-restricted diet
  • Patients 7 years and older (starting dose)
    • 10 to 20 mg/kg taken once daily
    • Take with a meal, preferably at the same time each day; must also follow a Phe-restricted diet
  • Dosage adjustment once responsiveness established
    • May be adjusted within the range of 5 to 20 mg/kg per day according to biochemical response to therapy (blood Phe)
    • Maximum: 20 mg/kg per day
    • If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day; discontinue if blood Phe does not decrease after 1 month at 20 mg/kg per day
Read the label’s full dosing text

2 DOSAGE AND ADMINISTRATION All patients with PKU who are being treated with sapropterin dihydrochloride powder for oral solution should also be treated with a Phe-restricted diet, including dietary protein and Phe restriction. ( 2.1 ) Starting Dosage Pediatric patients 1 month to 6 years : The recommended starting dose of sapropterin dihydrochloride powder for oral solution is 10 mg/kg taken once daily. ( 2.1 ) Patients 7 years and older : The recommended starting dose of sapropterin dihydrochloride powder for oral solution is 10 to 20 mg/kg taken once daily. ( 2.1 ) Dosage Adjustment Doses of sapropterin dihydrochloride powder for oral solution may be adjusted in the range of 5 to 20 mg/kg taken once daily. ( 2.1 ) Monitor blood Phe regularly, especially in pediatric patients. ( 2.1 , 5.3 ) Preparation and Administration Take with a meal. ( 2.2 ) Swallow oral solution after mixing powder in a small amount of soft foods or dissolving in recommended liquids. See full prescribing information for complete information on mixing with food or liquid. ( 2.2 ) 2.1 Dosage Treatment with sapropterin dihydrochloride powder for oral solution should be directed by physicians knowledgeable in the management of PKU. All patients with PKU who are being treated with sapropterin dihydrochloride powder for oral solution should also be treated with a Phe-restricted diet, including dietary protein and Phe restriction. Starting Dosage Pediatric Patients 1 month to 6 years : The recommended starting dose of sapropterin dihydrochloride powder for oral solution is 10 mg/kg taken once daily. Patients 7 years and older : The recommended starting dose of sapropterin dihydrochloride powder for oral solution is 10 to 20 mg/kg taken once daily. Dosage Adjustment (Evaluation Period) Existing dietary protein and Phe intake should not be modified during the evaluation period. If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with sapropterin dihydrochloride powder for oral solution at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of sapropterin dihydrochloride powder for oral solution treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day.

Excerpted — the section continues on the label. Read the full Dosage and Administration section on DailyMed →

Sapropterin side effects

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The most common side effects reported in clinical trials were generally mild:

Common side effects

  • Headache
  • Runny nose (rhinorrhea)
  • Sore or painful throat
  • Diarrhea
  • Vomiting
  • Cough
  • Nasal congestion

When to get medical help

  • Call your doctor or get emergency help if you develop signs of a severe allergic reaction (anaphylaxis): wheezing, trouble breathing, low blood pressure, flushing, nausea, or rash
  • Tell your doctor if you have stomach or throat pain, trouble swallowing, or other signs of upper GI inflammation — these can progress to serious conditions like esophagitis, gastritis, or ulcers
  • Contact your doctor if blood phenylalanine levels fall too low (hypophenylalaninemia), especially in young children — this can be harmful to development
  • If you are also taking levodopa, report any new or worsening neurological symptoms such as seizures, overstimulation, or irritability
  • Tell your doctor if you notice hyperactivity
  • Not every patient with PKU responds to sapropterin — if blood Phe levels do not fall during a trial period, your doctor will advise stopping the medication

More serious reactions have been reported and require prompt medical attention: severe allergic reactions (anaphylaxis) including breathing difficulty, low blood pressure, flushing, and rash; upper digestive tract inflammation that can progress to esophagitis, gastritis, ulcers, or bleeding; phenylalanine levels that drop too low (especially in young children at higher doses); and hyperactivity. Contact your doctor right away if you notice any of these.

Sapropterin warnings and precautions

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  • Severe allergic reactions (anaphylaxis): Reactions including difficulty breathing, low blood pressure, and rash have occurred, usually within the first few days of starting treatment. Stop the medication immediately and get emergency medical care. Continue your phenylalanine-restricted diet even after stopping.
  • Upper GI inflammation: Sapropterin can irritate the lining of the esophagus and stomach, potentially leading to ulcers or bleeding. Report stomach pain, chest pain, or pain when swallowing to your doctor promptly.
  • Phenylalanine levels falling too low (hypophenylalaninemia): This is a particular risk in children under 7 years at higher doses. Levels that are too low can harm growth and development. Frequent blood monitoring is essential.
  • Not all patients respond: Response cannot be predicted by genetic testing. A supervised therapeutic trial is required. If blood Phe does not decrease, the medication should be stopped.
  • Levodopa interaction: Seizures and neurological side effects have been reported when sapropterin was taken with levodopa. Close monitoring is required for anyone on both drugs.
  • Hyperactivity: Two cases have been reported post-marketing, including one associated with accidental overdose. Monitor for this effect.

Sapropterin interactions

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Three clinically important drug interactions are recognized with sapropterin:

  • Levodopa: Sapropterin may increase levels of tyrosine, a precursor to levodopa, and neurological events (seizures, overstimulation, irritability) have been reported when both drugs are used together. Patients on levodopa need close neurological monitoring.
  • Folate-synthesis inhibitors (e.g., methotrexate, valproic acid, phenobarbital, trimethoprim): These drugs can reduce the body's own BH4 levels, potentially raising phenylalanine. More frequent blood Phe monitoring is recommended, and a higher sapropterin dose may be needed.
  • PDE-5 inhibitors (e.g., sildenafil, vardenafil, tadalafil): Both sapropterin and these medications can relax blood vessels and lower blood pressure. Blood pressure should be monitored when both are used together.

Interactions listed in the FDA-approved label

From the Sapropterin prescribing information (“Drug Interactions”): what the manufacturer studied or reported to the FDA.

  • Levodopa: Sapropterin dihydrochloride may increase the availability of tyrosine, a precursor of levodopa; neurologic events were reported post marketing in patients receiving sapropterin and levodopa concomitantly for a non-PKU indication. Monitor patients for a change in neurologic status.
  • Inhibitors of folate synthesis (e.g., methotrexate, valproic acid, phenobarbital, trimethoprim): These drugs may decrease the bioavailability of endogenous BH4 by inhibiting dihydrofolate reductase, and this reduction in net BH4 levels may increase Phe levels. Consider monitoring blood Phe levels more frequently during concomitant administration; an increased dosage of sapropterin dihydrochloride may be necessary to achieve a biochemical response.
  • Drugs affecting nitric oxide-mediated vasorelaxation (e.g., PDE-5 inhibitors such as sildenafil, vardenafil, or tadalafil): Both sapropterin dihydrochloride and PDE-5 inhibitors may induce vasorelaxation, so a reduction in blood pressure could occur; the combined use has not been evaluated in humans. Monitor blood pressure.
Read the label’s full interactions text

7 DRUG INTERACTIONS Table 4 includes drugs with clinically important drug interactions when administered with sapropterin dihydrochloride and instructions for preventing or managing them. Table 4: Clinically Relevant Drug Interactions Levodopa Clinical Impact Sapropterin dihydrochloride may increase the availability of tyrosine, a precursor of levodopa. Neurologic events were reported post marketing in patients receiving sapropterin and levodopa concomitantly for a non- PKU indication [see Warnings and Precautions ( 5.5 )] Intervention Monitor patients for a change in neurologic status. Inhibitors of Folate Synthesis (e.g., methotrexate, valproic acid, phenobarbital, trimethoprim) Clinical Impact In vitro and in vivo nonclinical data suggest that drugs that inhibit folate synthesis may decrease the bioavailability of endogenous BH4 by inhibiting the enzyme dihydrofolate reductase, which is involved in the recycling (regeneration) of BH4. This reduction in net BH4 levels may increase Phe levels. Intervention Consider monitoring blood Phe levels more frequently during concomitant administration. An increased dosage of sapropterin dihydrochloride tablets may be necessary to achieve a biochemical response. Drugs Affecting Nitric Oxide-Mediated Vasorelaxation (e.g., PDE-5 inhibitors such as sildenafil, vardenafil, or tadalafil) Clinical Impact Both sapropterin dihydrochloride and PDE-5 inhibitors may induce vasorelaxation. A reduction in blood pressure could occur; however, the combined use of these medications has not been evaluated in humans. Intervention Monitor blood pressure. • Inhibitors of Folate Synthesis (e.g., methotrexate, valproic acid, phenobarbital, trimethoprim) : Can decrease endogenous BH4 levels; monitor blood Phe levels more frequently and adjust sapropterin dihydrochloride tablets dosage as needed. ( 7 ) • Drugs Affecting Nitric Oxide-Mediated Vasorelaxation (e.g., PDE-5 inhibitors) : Potential for vasorelaxation; monitor blood pressure. ( 7 )

Read the full Drug Interactions section on DailyMed →

Not a complete list. The label covers the interactions the manufacturer studied or reported, and our own interaction database does not cover every medicine either. Bring your full medication list, including vitamins and herbal products, to your pharmacist.

🌿17 supplements and herbs with documented Sapropterin interactions, ranked by severity Check Sapropterin against a supplement or herb →

Before taking Sapropterin

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  • Do not take sapropterin if you have previously had a severe allergic reaction (anaphylaxis) to it
  • Pregnancy: Uncontrolled high phenylalanine during pregnancy is associated with serious fetal harm, including neurological and cardiac defects. Blood phenylalanine should be carefully controlled before conception and throughout pregnancy. Discuss your treatment plan with your doctor if you are pregnant or planning to become pregnant.
  • Breastfeeding: Limited data are available. Discuss the benefits and risks with your doctor before breastfeeding while on sapropterin.
  • Young children (under 7 years): Higher doses carry an increased risk of phenylalanine dropping too low. Frequent blood monitoring is especially important in this age group.
  • Neonates (newborns): Safety and efficacy have not been established in newborns.
  • Adults 65 and older: This age group was not included in PKU clinical trials; it is not known if they respond differently.
  • Tell your doctor about all medications you take, especially levodopa, methotrexate, valproic acid, phenobarbital, trimethoprim, or any PDE-5 inhibitor
  • Sapropterin must always be used alongside a phenylalanine-restricted diet

Sapropterin overdose

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Two unintentional overdoses with sapropterin have been reported. One adult who took nearly twice the intended dose experienced mild headache and dizziness that resolved within an hour without treatment. One child who received more than twice the intended daily dose developed hyperactivity, which resolved when the dose was corrected. In a research study, a very high single dose given to healthy adults caused upper abdominal pain and dizziness, and a dose-dependent shortening of the QT interval (a heart rhythm measurement) was noted, though no serious reactions occurred. If you think you or someone else has taken too much sapropterin, contact your doctor right away.

What Sapropterin does in the body

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In patients whose PKU responds to BH4, blood phenylalanine levels begin to fall within 24 hours of the first dose of sapropterin, though the full effect may take up to a month. A single daily dose is sufficient to keep blood Phe levels stable over a 24-hour period, and levels do not spike significantly after meals. Higher doses produce greater reductions in blood Phe levels, demonstrating a clear dose-response relationship, though individual responses vary considerably from patient to patient.

How your body processes Sapropterin

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Sapropterin is absorbed from the gut, and food meaningfully increases how much the body absorbs — particularly with intact tablets taken with a meal. The body processes and clears sapropterin relatively quickly, with an average half-life (the time for blood levels to fall by half) of about 6.7 hours in PKU patients. Body weight is the main factor that affects how quickly the drug is cleared, which is why dosing is based on weight. Pharmacokinetic data in patients older than 49 years are not available.

How to store Sapropterin

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Store KUVAN tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) . Protect from moisture. Store KUVAN for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) .

Written from the FDA-approved label with AI and reviewed by our pharmacy team. How we use AI →

17

Supplements & herbs that interact with Sapropterin

17 supplements and herbal products have documented interactions with Sapropterin in the licensed clinical database we use. Most are manageable — but your pharmacist should know about everything you take, including vitamins and herbals.

Severity mix
  • Major · 1
  • Moderate · 7
  • Minor · 9
ACTIVATED CHARCOAL Major COFFEE CHARCOAL Moderate EUROPEAN MANDRAKE Moderate GLUCOMANNAN Moderate GUAR GUM Moderate GUM ARABIC Moderate SLIPPERY ELM Moderate TRAGACANTH Moderate +9 more
Every supplement checked against Sapropterin — ranked by severity The full interaction hub: 1 major · 7 moderate · 9 minor · every one linked to its full report. Check Sapropterin against your exact supplements

Educational information from a licensed clinical database (Natural Medicines) — see our data sources & update cadence. Not medical advice: an interaction being documented does not mean it will happen to you; do not start or stop medications or supplements without professional guidance.

Sapropterin: pharmacist answers to common questions

Quick, plain-English answers to the questions patients ask most about Sapropterin — the kind of thing our pharmacy team would talk through with you at the counter.

What exactly does sapropterin do, and why do I still have to follow a special diet?

▾
Sapropterin gives your body more of a helper molecule called BH4 that the enzyme responsible for breaking down phenylalanine needs to work. If you have BH4-responsive PKU, this can get that enzyme working better and lower your phenylalanine levels. But it doesn't completely fix the enzyme, so you still need the low-phenylalanine diet to keep things in a safe range. Think of the medication and the diet as a team — you need both.

How will I know if sapropterin is actually working for me?

▾
Your doctor will check your blood phenylalanine levels regularly after you start the medication. If the drug is working, your levels should start to drop within the first 24 hours, though it can take up to a month to see the full effect. Not everyone with PKU responds to sapropterin — in fact, that can't be predicted by genetic testing alone. If your blood Phe doesn't come down during the trial period, your doctor will likely advise stopping it.

What side effects are most common, and when should I call my doctor?

▾
Most people who experience side effects notice things like headache, a runny nose, sore throat, diarrhea, vomiting, cough, or nasal congestion — generally manageable and similar to cold symptoms. Call your doctor right away if you develop signs of a serious allergic reaction like wheezing, trouble breathing, low blood pressure, flushing, or a rash — these can happen within the first few days of starting the medication. Also let your doctor know promptly if you have stomach pain, chest pain, or pain when swallowing, as these could signal irritation or inflammation in your esophagus or stomach.

Does it matter whether I take sapropterin with food or on an empty stomach?

▾
Yes, food matters. Taking sapropterin with a meal — especially if you're taking the intact tablet form — helps your body absorb more of the medication. The label recommends taking it with a meal, at roughly the same time every day. If you miss a dose, take it as soon as you remember, but don't take two doses in one day to make up for it.

I'm pregnant (or trying to get pregnant) — is it safe to take sapropterin?

▾
This is a really important conversation to have with your doctor. What's very clear is that having uncontrolled high phenylalanine levels during pregnancy is seriously harmful to the baby, increasing the risk of neurological, heart, and growth problems. Keeping your Phe levels in a safe range before and during pregnancy is essential. Available pregnancy registry data don't show a clear pattern of birth defects from sapropterin itself, but data are limited. Your doctor will help you weigh the risks and benefits and monitor your Phe levels closely throughout.

Are there any medications I should avoid while taking sapropterin?

▾
There are a few important ones to know about. If you take levodopa (used for Parkinson's disease), combining it with sapropterin has been linked to seizures and neurological side effects in some patients, so your doctor will want to monitor you closely. Certain drugs including methotrexate, valproic acid, phenobarbital, and trimethoprim can reduce your body's natural BH4 levels and may raise your phenylalanine — tell your doctor if you take any of these. Medications for erectile dysfunction or pulmonary hypertension like sildenafil, vardenafil, or tadalafil can also lower blood pressure when combined with sapropterin, so blood pressure monitoring may be recommended.

Is Sapropterin available over the counter?

▾
No. Sapropterin is a prescription medicine: every Sapropterin product listed with the FDA is prescription only, so it is not sold over the counter. Your prescriber decides whether it is appropriate for you, and your pharmacist dispenses it.

When was Sapropterin first available?

▾
The earliest FDA record we hold for Sapropterin is from 2007: its first approval in Drugs@FDA or its earliest product listing in the NDC Directory, whichever is older. Many older medicines were in use before their first formal FDA approval, so this is the earliest date on record, not necessarily when Sapropterin was first used.

Who makes Sapropterin?

▾
10 companies currently list Sapropterin products in the FDA's NDC Directory, including Dr. Reddys Laboratories Inc, BioMarin Pharmaceutical Inc., Camber Pharmaceuticals, Inc.. That count includes repackagers and distributors as well as manufacturers (companies that sell only the raw ingredient are not counted), so fewer companies actually make it; the label on your bottle names the one that supplied yours.

Answers drafted from the FDA-approved label with AI and reviewed by our pharmacy team. How we use AI →

More about Sapropterin on HelloPharmacist

📊Sapropterin supplement interactions17 checked — ranked by severity 🌿Drug & Supplement CheckerSapropterin vs. vitamins & herbs 💬Ask a PharmacistFree answer, usually < 24h 🔎NDC & pricing lookupFind Sapropterin NDCs & costs 🅰️Browse Drugs A–ZLook up any medication
Detailed data & references for Sapropterin Forms & strengths, pricing, billing codes, manufacturers, safety reports and sourcing — the data behind the page. Most people can stop here. Each section below opens on a tap.

Sapropterin forms and strengths (how it comes)

Sapropterin is available in 2 forms. Different forms and brands can have different approved uses, doses, schedules, and directions. Follow the instructions for your exact product, and do not switch forms without guidance from your prescriber or pharmacist.

Need a version that does not list lactose, a gluten source, a dye or gelatin? Inactive ingredients differ by manufacturer: see Sapropterin inactive ingredients by manufacturer.

Oral solution

By mouth
Brands Javygtor Kuvan Sapropterin dihydrochloride Zelvysia
How this form is used
What it may be used for
  • Javygtor oral solution is used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
  • Kuvan oral solution powder is used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
  • Zelvysia oral solution is used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
  • Sapropterin dihydrochloride powder for oral solution is used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
Important instructions
  • Mix the powder in a small amount of soft food or dissolve it in the recommended liquid before swallowing; follow the full preparation instructions in the prescribing information for your specific product
  • Take with a meal, preferably at the same time each day
  • If you miss a dose, take it as soon as possible — but do not take two doses on the same day
  • Zelvysia, Kuvan, Javygtor, and sapropterin dihydrochloride powder for oral solution should all be administered under the direction of a physician experienced in managing PKU
Available strengths 2 strengths
100 mg 500 mg
26 FDA-listed product records ⓘ

Tablet

By mouth
Brands Javygtor Kuvan Sapropterin Dihydrochloride
How this form is used
What it may be used for
  • Javygtor tablets are used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
  • Kuvan tablets are used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
  • Sapropterin dihydrochloride tablets are used to reduce blood phenylalanine levels in adults and children one month of age and older with BH4-responsive PKU, alongside a phenylalanine-restricted diet
Important instructions
  • Tablets may be swallowed whole, mixed into a small amount of soft food, or dissolved in a recommended liquid before swallowing
  • Taking intact tablets with food increases absorption compared to fasted conditions — follow your prescriber's specific guidance for your product
  • Take once daily with a meal, preferably at the same time each day
  • If you miss a dose, take it as soon as possible — but do not take two doses on the same day
Available strengths 1 strength
100 mg
15 FDA-listed product records ⓘ

Sapropterin on the U.S. market: products, makers and brands

How Sapropterin appears in the FDA’s National Drug Code Directory — including its dosage forms, strengths, brand names, manufacturers, and FDA-listed product records. These are directory records, not sales or prescription volume.

10 companies list 24 Sapropterin product records with the FDA, mostly as oral solution, tablet; the earliest marketing or approval year on record is 2007. Brand names on the directory include Kuvan, Javygtor, Zelvysia and 1 more; the rest are generics.

2
Dose forms ⓘ
10
Labelers (makers, repackagers, distributors)
4
Brand names
24
FDA-listed product records ⓘ
2007
Earliest FDA record ⓘ

FDA-listed product records by dose form (tablet variants merged) — FDA National Drug Code Directory. Record counts are not sales or prescription volume.

Also listed with the FDA, not a patient dose form: Powder (10) — bulk drug substance sold to compounders/manufacturers and multi-item kits. These aren’t counted in the dose-form total above.

Dr. Reddys Laboratories Inc17%
BioMarin Pharmaceutical Inc.13%
Camber Pharmaceuticals, Inc.13%
NorthStar RxLLC13%
Par Health USA, LLC13%
Aucta Pharmaceuticals, Inc.8%
Other makers25%

Share of FDA-listed product records by manufacturer — FDA National Drug Code Directory. Record counts are not sales or prescription volume.

How widely Sapropterin is used (Medicaid data)

A general measure of how commonly Sapropterin is prescribed, based on Medicaid — one of the largest public drug programs. The figure below is prescriptions filled in Q1–Q4 2025, summed across every form and brand of the drug.

Think of this as a proxy for how widely Sapropterin is used, drawn from freely available public data. Medicaid is just one program — these numbers don’t include Medicare, other government coverage, or commercial and cash-pay prescriptions, so real-world use is higher than what’s shown here.

6,060
Medicaid prescriptions filled (Q1–Q4 2025)
$31.8M gross Medicaid reimbursement (before mandatory rebates — net cost is lower)

How commonly is Sapropterin prescribed? Of the 1,601 medications we measure, Sapropterin ranks #1,015 by Medicaid prescriptions — more than 37% of them.

Where Sapropterin ranks among the drugs we measure · Q1–Q4 2025 · neighbors ±5
Total prescriptions — #1,015 of 1,601
#1,010 Pemetrexed Disodium 6,336 Rx #1,011 Riociguat 6,299 Rx #1,012 Voriconazole 6,277 Rx #1,013 Technetium Tc-99M 6,225 Rx #1,014 Amoxicillin / Omeprazo... 6,073 Rx #1,015 Sapropterin 6,060 Rx #1,016 Urea 6,045 Rx #1,017 Abaloparatide Injectio... 6,041 Rx #1,018 Tobramycin Injection 6,028 Rx #1,019 Zolmitriptan Nasal Spr... 5,993 Rx #1,020 Meperidine Injection 5,961 Rx

Utilization by Sapropterin product

Pick a product to see its own Medicaid numbers. Each chip is one clinical product — a specific strength & dosage form as defined by RxNorm — with brand-name and generic versions combined.

The 100 mg powder for oral solution is the most-dispensed Sapropterin product — about 45% of these Medicaid prescriptions.

sapropterin dihydrochloride 100 MG Powder for Oral Solution Most dispensed

2,728 Medicaid prescriptions (Q1–Q4 2025) 45% of Sapropterin prescriptions shown $6M gross reimbursement (before rebates) ≈ $2,196 per prescription (gross)

Summed across the 4 of 19 listed NDC products with Medicaid activity — every brand and generic of this exact strength & form. RxNorm 1111018

sapropterin dihydrochloride 500 MG Powder for Oral Solution

2,587 Medicaid prescriptions (Q1–Q4 2025) 43% of Sapropterin prescriptions shown $19M gross reimbursement (before rebates) ≈ $7,354 per prescription (gross)

Summed across the 4 of 17 listed NDC products with Medicaid activity — every brand and generic of this exact strength & form. RxNorm 1653217

sapropterin dihydrochloride 100 MG Oral Tablet

745 Medicaid prescriptions (Q1–Q4 2025) 12% of Sapropterin prescriptions shown $6.8M gross reimbursement (before rebates) ≈ $9,098 per prescription (gross)

Summed across the 4 of 12 listed NDC products with Medicaid activity — every brand and generic of this exact strength & form. RxNorm 1486687

Shares are of the Medicaid prescriptions shown here. Product grouping follows RxNorm (the National Library of Medicine’s drug terminology), so “one product” means one strength & dosage form regardless of manufacturer. Dollar figures are gross Medicaid reimbursement before mandatory rebates — rebates (often large, especially for brand-name drugs) are confidential, so actual net cost to Medicaid is lower than shown.

Source: Medicaid State Drug Utilization Data (CMS), aggregated by generic ingredient — summed across every NDC (all brands, strengths, salt forms and dosage forms) of Sapropterin, and across all states and both fee-for-service and managed-care claims. A general popularity signal; it excludes Medicare, commercial insurance and cash prescriptions, so it is not total U.S. use. Based on the 12 of 48 listed Sapropterin NDCs with Medicaid activity.

Compare Sapropterin products

A representative set of FDA-listed product records for Sapropterin — across manufacturers, strengths, and dosage forms, grouped by form with each product’s manufacturer and how the FDA approved it. The same medicine may be made and packaged by different companies, so a single drug can have many directory entries. (It’s also why a refill can look different — a new shape, color, or box — even though it contains the same medication.)

Showing 24 of 24 products — FDA National Drug Code Directory. See every product, package size and price: browse all 24 Sapropterin NDCs →

Sapropterin recall history

A recall is when a specific batch of a medicine is pulled from the market — usually a manufacturing issue, not a problem with the drug itself. Class I is most serious, Class III least.

The FDA’s enforcement reports list 2 Sapropterin recalls since July 2021. Both are still ongoing or began in the last six months. A recall names specific lots from one company, so check the lot number on your own bottle rather than assuming every product is affected; your pharmacist can check whether your supply came from an affected lot.

Ongoing Class III Nov 22, 2024 · Dr. Reddy's Laboratories, Inc.
Failed Impurities/Degradation Specifications
  • Javygtor (sapropterin dihydrochloride) Tablets 100mg, 120-count bottle, Rx Only, Distributor: Dr. Reddy's Laboratories Inc., Princeton, NJ 08540 Made in India, NDC 43598-096-04. Lots: Lot #: T2300653, Exp 01/31/2025; T2303956, T2303750, Exp 06/30/2025; T2304190, T2304987, Exp 08/31/2025; T2302026, Exp 03/31/2025; T2302526, Exp 05/31/2025. FDA record D-0117-2025 →
Ongoing Class I Apr 8, 2024 · Dr. Reddy's Laboratories, Inc.
Sub-potent Drug; powder discoloration associated with decreased potency
  • Javygtor (sapropterin dihydrochloride) Powder for Oral Solution 100mg, 30 individual packets per carton, Rx Only, Distributor: Dr. Reddy's Laboratories Inc., Princeton, NJ 08540 Made in India, NDC 43… Lots: Lot #: T2202812, Exp. 07/31/2025; T2204053, Exp. 10/31/2025; T2300975, T2300976, Exp. 02/28/2026; T2304356, Exp. 08/31/2026. FDA record D-0485-2024 →

Source: FDA enforcement (recall) reports, via openFDA; our copy was last updated Sep 24, 2026.

RxNorm Concept Web — From Ingredient to Every Form and Strength

This page starts with one ingredient concept (IN). The branches below show its dose-form concepts (SCDF), then the available clinical drug and strength concepts (SCD). Combination products remain separate concepts with their own pages.

Ingredient (IN) Sapropterin RxCUI 753340 2 dose forms · 3 strengths
  1. Dose form (SCDF) Oral Tablet RxCUI 1102010 In current FDA listings
    Clinical drug / strength (SCD) dihydrochloride 100 MG RxCUI 1486687

Look up RxCUI 753340 in the RxCUI Atlas →

RxNorm is the U.S. National Library of Medicine’s normalized drug vocabulary. Its concept hierarchy can include forms that are not currently marketed; the status on each branch compares that concept with current FDA listings. RxCUIs identify vocabulary concepts, not individual packages or manufacturers.

Sapropterin brand names

Sapropterin is sold under 4 brand names in the FDA directory — Kuvan, Javygtor, Zelvysia, sapropterin dihydrochloride. A brand and its generic contain the same active ingredient; the brand name belongs to one company's product.

Kuvan Javygtor Zelvysia sapropterin dihydrochloride

Who makes Sapropterin: manufacturers and labelers

10 companies list Sapropterin products with the FDA. By number of product records, the largest are Dr. Reddys Laboratories Inc, BioMarin Pharmaceutical Inc., Camber Pharmaceuticals, Inc.. Labelers include manufacturers and repackagers; the product in your bottle depends on which one your pharmacy stocks.

Dr. Reddys Laboratories Inc 4 BioMarin Pharmaceutical Inc. 3 Camber Pharmaceuticals, Inc. 3 NorthStar RxLLC 3 Par Health USA, LLC 3 Aucta Pharmaceuticals, Inc. 2 Micro Labs Limited 2 Trupharma, Llc 2 Aurobindo Pharma Limited 1 Hikma Pharmaceuticals USA Inc. 1

Sapropterin drug class (RxNorm)

Sapropterin belongs to the Phenylalanine Hydroxylase Activator class.

Pharmacologic class Phenylalanine Hydroxylase Activator
Drug family (ATC) Various alimentary tract and metabolism products
How it works P-Glycoprotein Inhibitors, Breast Cancer Resistance Protein Inhibitors, Phenylalanine Hydroxylase Activators

Classified by RxNorm RxClass — the U.S. National Library of Medicine's standardized drug-classification service (Established Pharmacologic Class from the FDA, the ATC drug family from the WHO, and mechanism of action). Reference only, not medical advice.

Sources for this Sapropterin page

Core label, product, RxNorm, safety, and utilization data on this page come from the sources identified below. AI-written, editorial, and licensed sections are labeled separately.

📄
FDA-filed drug labels (SPL) Uses, dosing, warnings & side effects from SPL records retrieved through openFDA; the linked representative set can be verified on DailyMed. ✓ Linked label effective Aug 26, 2024
🏭
Forms, brands & labelersAggregated from ingredient-matched FDA National Drug Code Directory product records for Sapropterin.
📈
Real-world reportsThe FDA Adverse Event Reporting System (FAERS), served through openFDA.
🚨
Recalls & shortagesopenFDA drug-enforcement reports and the FDA drug-shortage database.
🧾 Clinical label set — FDA-filed SPL labels retained for Sapropterin after exact ingredient or ingredient-combination matching and applicable route/form matching
Clinical labels in scope
16
Finished products in the current FDA directory
24
Companies listing them (makers, repackagers, distributors)
10
FDA labeler codes behind them ⓘ
10
Linked representative label ⓘ
BioMarin Pharmaceutical Inc.
Linked label effective
Aug 26, 2024
Composite sections available
23
Linked SPL Set ID
af38711e-8873-4790-a92d-4d583e23fb89
Linked SPL Document ID
c4286a54-6e01-4444-94a0-943411790e1d
Open the linked representative label on DailyMed ↗

How this page is built

HelloPharmacist combines public FDA and NLM records and does not author the underlying clinical facts. The plain-language sections are written with AI from the FDA-approved label text and reviewed by our pharmacy team before publication. BioMarin Pharmaceutical Inc. is the representative DailyMed label linked for verification and dates. This is general education, not medical advice: always talk to your doctor or pharmacist about your own medicines. How we build and review drug pages →