How does Endari work?

Many sickle cell disease complications occur due to damaged red blood cells. Endari works by helping reduce damage to red blood cells.

Endari (L-Glutamine) - Oral: Uses, Side Effects, Dosages

What Is Endari?

Endari (l-glutamine) is a prescription medication that reduces painful episodes (crises) in adults and children with sickle cell disease. Endari belongs to a group of medications called amino acids. It works by helping reduce damage to red blood cells. Endari is available as a powder you mix with a beverage or soft food before consuming.

Drug Facts

Generic Name: L-glutamine

Brand Name(s): Endari

Drug Availability: Prescription

Therapeutic Classification: Amino acid

Available Generically: No

Controlled Substance: No

Administration Route: Oral

Active Ingredient: L-glutamine

Dosage Form(s): Oral powder

What Is Endari Used For?

Endari reduces painful episodes (crises) in adults and children 5 years and older with sickle cell disease.

Sickle cell disease is an inherited genetic disorder that affects approximately 100,000 people in the United States. Sickle cell disease alters hemoglobin—the oxygen-carrying molecule on red blood cells. This change can cause red blood cells to rupture or be sickle-shaped, leading to painful episodes and other complications associated with sickle cell disease.

How to Take Endari

Endari is typically taken two times per day. Endari comes as powder packets that you mix with a beverage or soft food. Your dose may require one or multiple packets.

Before taking Endari, you will need to mix the appropriate number of packets with 8 ounces (oz) of liquid (such as water, milk, or apple juice) or 4 to 6 ounces of soft food (such as yogurt or applesauce). Be sure the food or liquid is cold or at room temperature. Do not use hot food or beverages for mixing.

Use a spoon to mix Endari with the liquid or food. Don't worry if your dose of Endari doesn't dissolve completely—this will not impact its effects.

Drink or eat the mixture as soon as you've prepared your dose. Do not save it for later use.

Storage

Endari should be stored at room temperature, but keep your prescription away from direct sunlight. Keep Endari and all of your medicines in a safe location, out of the reach of children and pets.

How Long Does Endari Take to Work?

Endari reduces the frequency of painful episodes over time. In clinical studies, participants had fewer painful episodes and hospitalizations during 48 weeks than participants who received a placebo (powder with no active medication). The time it takes Endari to work may be different for everyone. Be sure to check with your healthcare provider or pharmacist for any questions.

What Are the Side Effects of Endari?

This is not a complete list of side effects and others may occur. A healthcare provider can advise you on side effects. If you experience other effects, contact your pharmacist or a healthcare provider. You may report side effects to the Food and Drug Administration (FDA) at fda.gov/medwatch or 800-FDA-1088.

Common Side Effects

You may experience side effects while taking Endari. Let your healthcare provider know if you develop any side effects that bother you or don't go away.

Common side effects include:

Constipation
Nausea
Headache
Stomach pain
Cough
Pain in the arms or legs
Back pain

Severe Side Effects

Rarely, Endari can cause serious side effects. Contact your healthcare provider immediately if you develop any signs of a severe reaction. Call 911 if your symptoms feel life-threatening.

Serious side effects and their symptoms include:

Signs of an allergic reaction, such as a rash, hives, difficulty breathing, or swelling of the face, mouth, lips, tongue, or throat
Signs of a pancreas problem (pancreatitis), including severe stomach pain, back pain, nausea, or vomiting
Feeling like you need to have a bowel movement often or rectal irritation
Vomiting blood or vomit that looks like coffee grounds
Black, tarry, or bloody stools
Chest pain or pressure

Report Side Effects

Endari may cause other side effects. Call your healthcare provider if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your provider may send a report to the FDA's MedWatch Adverse Event Reporting Program or by phone (800-332-1088).

(800) 332-1088

Dosage: How Much Endari Should I Take?

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

For oral dosage form (powder for oral solution):
- For short bowel syndrome:
  - Adults—30 grams (g) per day in divided doses (5 g taken 6 times a day) for up to 16 weeks. Taken with meals or snacks, 2 to 3 hours apart while awake.
  - Children—Use and dose must be determined by your doctor.
For oral dosage form (oral powder):
- For sickle cell disease:
  - Adults and children 5 years of age and older and weighs greater than 65 kilograms (kg)—15 grams (g) per dose (3 packets per dose) 2 times a day or 30 g per day (6 packets per day).
  - Adults and children 5 years of age and older and weighs 30 to 65 kg—10 g per dose (2 packets per dose) 2 times a day or 20 g per day (4 packets per day).
  - Children 5 years of age and older and weighs less than 30 kg—5 g per dose (1 packet per dose) 2 times a day or 10 g per day (2 packets per day).
  - Children younger than 5 years of age—Use and dose must be determined by your doctor.

Modifications

The following modifications (changes) should be kept in mind when using Endari:

Pregnancy: Studies on the effect of Endari on pregnancy are lacking. We don't know enough about the safety and effectiveness of Endari in pregnant people and their unborn fetuses. Talk with your healthcare provider if you are or plan to become pregnant, and discuss the benefits and risks of taking Endari during your pregnancy.

Breastfeeding: Studies on the effect of Endari on breastfeeding are lacking. Therefore, we don't know enough about the safety of Endari on nursing babies. Talk with your healthcare provider if you plan to breastfeed. Discuss the benefits and risks of taking Endari while nursing and the different ways to feed your baby.

Adults over 65: Clinical studies haven't included a large enough number of people in this age group to see whether they respond differently from younger adults.

Children: Endari is approved to treat children with sickle cell disease 5 years and older. However, the safety and effectiveness of Endari in children under 5 have not been evaluated.

Missed Dose

If you accidentally forgot your Endari dose, take it as soon as you remember. If it's already close to your next scheduled dose, then skip the missed dose and take the following dose at your next scheduled dosing time. Don't try to double up to make up for the missed dose.

Try to find ways to help yourself remember to take your medication routinely. If you miss too many doses, Endari might be less effective at preventing painful episodes.

Overdose: What Happens If I Take Too Much Endari?

There is limited information available about the effects of an Endari overdose.

If you think you're experiencing an overdose or life-threatening symptoms, seek immediate medical attention.

What Happens If I Overdose on Endari?

If you think you or someone else may have overdosed on Endari, call a healthcare provider or the Poison Control Center (800-222-1222).

If someone collapses or isn't breathing after taking Endari, call 911 immediately.

911

Precautions

It is very important that your doctor check your progress at regular visits to make sure that this medicine is working properly. Blood tests may be needed to check for unwanted effects.

Check with your doctor right away if you have pain or tenderness in the upper stomach, pale stools, dark urine, loss of appetite, nausea, vomiting, or yellow eyes or skin. These could be symptoms of a serious liver problem.

Do not take other medicines unless they have been discussed with your doctor. This includes prescription or nonprescription (over-the-counter [OTC]) medicines and herbal or vitamin supplements.

What Are Reasons I Shouldn’t Take Endari?

Do not take Endari if you are allergic to l-glutamine or any other ingredient in Endari.

What Other Medications Interact With Endari?

Use caution when taking Endari with the following medication:

Lactulose

For more detailed information about medication interactions with Endari, talk with your pharmacist or healthcare provider.

What Medications Are Similar?

Endari is the only amino acid used to decrease sickle cell acute pain episodes. Other medications used to decrease painful episodes but work differently than Endari include:

Hydrea (hydroxyurea): Oral
Adakveo (crizanlizumab): Once-monthly intravenous (IV) infusion

Given its effectiveness, healthcare providers typically prefer hydroxyurea to treat children and adults with sickle cell disease. However, some people cannot tolerate hydroxyurea or continue to have frequent painful episodes. In these cases, other medications such as Endari or Adakveo may be tried or combined with hydroxyurea. Deciding on your treatment will depend on your age, other health conditions, and preferred route of administration.

Frequently Asked Questions

What is Endari used for?

Endari is used to reduce the frequency of acute pain episodes in adults and children 5 years and older with sickle cell disease.
How does Endari work?

Many sickle cell disease complications occur due to damaged red blood cells. Endari works by helping reduce damage to red blood cells.
What are the side effects of Endari?

The most common side effects of Endari include constipation, nausea, headache, stomach pain, and cough.

How Can I Stay Healthy While Taking Endari?

If you're taking Endari, chances are sickle cell disease has been negatively affecting your quality of life. You may have tried different approaches or treatments. While living with sickle cell disease does have its challenges, there are ways to help improve your quality of life. Refer below for some general tips to support your health:

Take sickle cell disease-related medications as recommended by your healthcare provider.
Consider keeping a diary to become more aware of your triggers.
Avoid dehydration. Stay well hydrated during exercise and travel.
Maintain a healthy, balanced diet.
Consider support groups or working with a mental health professional. Many people with sickle cell disease may struggle with depression or anxiety, so seeking help and getting the care you need are essential.

Medical Disclaimer

Verywell Health's drug information is meant for educational purposes only and is not intended as a replacement for medical advice, diagnosis, or treatment from a healthcare provider. Consult your healthcare provider before taking any new medication(s). IBM Watson Micromedex provides some of the drug content, as indicated on the page.

3 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

DailyMed. Endari - glutamine powder, for solution.
Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019;14:263-292. doi:10.1146/annurev-pathmechdis-012418-012838
Kapoor S, Little JA, Pecker LH. Advances in the treatment of sickle cell disease. Mayo Clin Proc. 2018;93(12):1810-1824. doi:10.1016/j.mayocp.2018.08.001

By Christina Varvatsis, PharmD
Christina Varvatsis is a hospital pharmacist and freelance medical writer. She is passionate about helping individuals make informed healthcare choices by understanding the benefits and risks of their treatment options.

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