Written by By Jemela Williams
I’ve been asked this question on more than one occasion when I mention the medications that I’m currently on for the management of my Sickle Cell Anemia. What is Endari? So I’m here to answer this question amongst others such as, what Endari is for, what it’s supposed to do, how do you use it, side effects and more. I’ll also include how my Sickle Cell SS has been affected personally since beginning the medication.
Endari is the first FDA approved drug for the treatment of Sickle Cell Anemia and Sickle Cell Thalassemia since Hydroxyurea, making it the first approved treatment in almost 20 years, approved in 2017. It’s manufactured by Emmaus Life Sciences. The medical name is Endari, but it is L-Glutamine. L-Glutamine is an amino acid. Glutamine is naturally produced within our bodies, mainly in our lungs. Endari increases the amount of Glutamine in our bodies, essentially helping the sickled red blood cells to attain the flexibility they need to flow more freely through the blood vessels.
The purpose of Endari is to reduce the acute complications of Sickle Cell Disease, like painful Sickle Cell crises, for adults and younger pediatric aged children, 5 years of age and up. It’s been shown to increase the time in between crises and to reduce hospitalizations due to crises.
Endari is packaged as a powder that you mix with liquids. You can mix it with cold or room temperature liquids, such as water, milk or juice, but you cannot mix it with hot liquids such as hot tea or coffee. You can also mix it with some foods, preferably yogurt or applesauce. Some people do say that it has a weird taste, but in my opinion, it doesn’t. I believe that if you mix it or shake it up, really well, whether it be water or juice, you won’t taste a thing, but obviously everyone’s taste is different. I suggest mixing it first with juice or something else flavorful to mask the taste and as you become used to it, then you can replace the juice with water. I’ve found that in doing so, it’s helped me to remember to drink much more water than I usually do.
The dosage of Endari is by body weight, and you should consume the full dose of it immediately after mixing it. There’s three different doses, 1, 2, or 3 packets daily. So 5 grams, 10 grams and 15 grams, or powder per day. When mixing it with liquid, it should be 8 oz. of liquid and 4 to 6 oz of applesauce or yogurt, when mixing the Endari with foods. It’s recommended that you take your Endari twice per day, morning and evening.
I have been on Hydroxyurea for over 10 years now and in February of 2019, my Doctors started me on Endari as well. I can honestly say that it has indeed done wonders for my hemoglobin levels. My hemoglobin is normally between 7 to 8.7, no higher. Before I started Endari in February, I’d had a doctors appointment and my hemoglobin was 8.7. After starting it and later attending my next appointment in March, my HBG was at 9.2. Both my NP and I were excited because it’s never gotten up to 9, ever before, even while being on Hydroxyurea.
My hemoglobin dropped back down in April 2019, all the way to 6, because I had a Sickle Cell Crisis at the start of that month. Doctors who weren’t familiar with me, were adamant that I needed a blood transfusion. Instead I insisted on being given my daily scheduled meds, including Endari.
The medication was so new that it wasn’t in their systems so my Mom brought my prescription box in from home. After starting back on my Endari, along with my other meds, my HGB started rising again to normal levels and I was able to be discharged within a week.
Since starting Endari, my HGB levels have gone up and fluctuated between 8.7 and up to 9.4, that is without any incidence of blood transfusions. At my appointment in November, it was up to 10, this December it has gone back to 9.4, so still very good. My bilirubin has also dropped below normal and my NP says she can see the difference as there has been no jaundice in my eyes as of late. So since February, I’ve seen some good changes to my health after starting Endari.
Patients in the Emmaus Life Sciences’ clinical trials for Endari, took it while still on Hydroxyurea and still received blood transfusions while on Endari as well. Endari is a pharmaceutical grade of L-glutamine and is not something you can get at a health or supplement store, it is only available through specialty pharmacies with a prescription.
There have been no studies of the use of Endari in women who are pregnant or in women who are breastfeeding. So please talk to your doctor if you are pregnant or thinking about becoming pregnant or if you are breastfeeding.
The most common side effects in clinical studies were constipation, nausea, headache, pain in the stomach area, cough, pain in the hands or feet, back pain, and chest pain. During the clinical trial of Endari, there were some side effects that led to a stop in treatment. There was one case of each of the following side effects, Overactive spleen (an organ that helps filter blood), stomach pain, indigestion, burning sensation and hot flash. Please let your doctors know if you experience any other negative side effects listed here, or otherwise.
I’m not a medical professional so I cannot ultimately recommend any medication to you, but I do highly suggest that you speak to your Physician, about Endari and find out if it is right for you. Always consult your physician before starting any new medications.
I feel as someone with a chronic illness, it’s important for us to stay up to date on any and all possible treatments available for Sickle Cell Disease.
ENDARI is a trademark of Emmaus Medical, Inc.