What Is Endari Used For?
Endari reduces painful episodes (crises) in adults and children 5 years and older with sickle cell disease.
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
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.
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:
ConstipationNauseaHeadacheStomach painCoughPain in the arms or legsBack 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 throatSigns of a pancreas problem (pancreatitis), including severe stomach pain, back pain, nausea, or vomitingFeeling like you need to have a bowel movement often or rectal irritationVomiting blood or vomit that looks like coffee groundsBlack, tarry, or bloody stoolsChest pain or pressure
Dosage: How Much Endari Should I Take?
Modifications
The following modifications (changes) should be kept in mind when using Endari:
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).
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.
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.
Precautions
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.
If someone collapses or isn’t breathing after taking Endari, call 911 immediately.
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 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): OralAdakveo (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.
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.
