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Using L-glutamine for Sickle Cell Disease Crises

January 30, 2020
L-glutamine may reduce the frequency of pain crises and related hospitalizations, especially among those patients those unable to utilize hydroxyurea for SCD crises.

A PPM Brief

As shared in a related PPM case report on sickle cell crisis by Dr. Sota Omoigui, there is not much evidence available regarding therapies that may help to slow or interfere with the natural progression of a sickle cell crisis, which is the most common reason for hospitalization of patients with sickle cell disease (SCD). Hydroxyurea has been used for many years to reduce the frequency of such crises and, until recently, was the only medication shown to improve vaso-occlusive (VOC) in patients with the sickle cell anemia (SCA) subtype of SCD. In 2017, FDA approved L-glutamine (Endari) for patients ages 5 years and older to reduce severe complications in other genotypes of SCD as well.1,2

 

There is a lack of high-quality evidence to support the use of L-glutamine for reduction in the frequency of vaso-occlusive pain episodes in sickle cell disease patients. (Image: iStock)

To determine the state of the evidence for the use of L-glutamine in the prevention of VOC and associated pain in patients with SCD, researchers from the University of Buffalo in New York conducted a systematic review.1 After elimination of studies that did not meet inclusion criteria, only three trials remained, each under the same lead author.2-4

In the first,2 patients treated with L-glutamine exhibited an increase in levels of the pyridine nucleotides, NAD+ and its reduced form NADH, which are thought to be involved in regulating and preventing oxidative damage in red blood cells. Significant improvements were also noted in “subjective clinical outcomes” such as energy levels and levels of chronic pain. However, this 1998 pilot was a non-randomized, single center study and included only 7 patients. The second study,3 published in 2014, compared L-glutamine to placebo in reduction of sickle cell pain crises. This study was a randomized, double-blind, placebo controlled multicenter trial. It found a non-significant reduction in the number of pain crises compared with placebo. However, it did show a significant reduction in hospitalizations among the patients treated with L-glutamine. The third,4 a randomized, double-blind, placebo controlled, multicenter, 48-week trial, was published in 2018. It examined the effects of L-glutamine in reducing the incidence of pain crises in SCD patients, ultimately demonstrating a significant reduction in pain crises among patients receiving L-glutamine.

Overall, the reviewers concluded that there is a lack of high-quality evidence to support the use of L-glutamine for reduction in the frequency of VOC pain episodes. Nonetheless, the evidence that does exist, they argued, supports continued use of the medication as an option to help reduce pain crises and related hospitalizations, especially among those patients those unable to utilize hydroxyurea or in addition to hydroxyurea.

See also, new strategies for treating SCD in pediatric patients.

Last updated on: February 5, 2020
Continue Reading:
Sickle Cell Pain Crisis: Clinical Guidelines for the Use of Oxygen
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