l‐glutamine was approved by the U.S. Food and Drug Administration (FDA) for sickle cell disease (SCD) in 2017. A vaso‐occlusive crisis (VOC) occurs in persons with SCD and is associated with acute pain episodes. This systematic review summarizes the evidence for l‐glutamine in the prevention of VOC and associated pain in patients with SCD. Medline, Embase, and International Pharmaceutical Abstracts were searched for records reporting on l‐glutamine use in persons with SCD. Eligibility criteria identified primary reports of investigations conducted in humans who were administered l‐glutamine, reported on outcomes related to VOC or associated pain, published in English, and were available as full text. All relevant efficacy, safety, participant demographic data, and study method characteristics were extracted and documented. Risk‐of‐bias assessments were conducted using the Risk of Bias in Non‐Randomized Studies‐of Interventions (ROBINS‐I) tool and the revised Cochrane risk‐of‐bias tool for randomized studies. Three studies assessing the effect of exogenous l‐glutamine administration in patients with SCD met eligibility criteria: one prospective nonrandomized controlled study and two prospective randomized controlled trials. Rate of VOC and related hospitalizations were reduced in patients receiving l‐glutamine, although some conflicting results were noted between studies. l‐glutamine was generally well tolerated. Limitations of one or more of the eligible studies included small sample size, nonblinding, and study groups that differed at baseline. l‐glutamine has limited high‐quality evidence supporting its use. Although l‐glutamine is FDA approved for the prevention of frequent episodes of VOC pain, only one randomized controlled trial has strong evidence to support this indication. Based on the results of a systematic review, l‐glutamine may be considered for patients unable to receive hydroxyurea or in addition to hydroxyurea for reduction in VOC and associated pain.

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INTRODUCTION: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)/Berdon syndrome is a rare disorder characterized by a distended non-obstructed bladder, microcolon, and decreased intestinal peristalsis with average life expectancy less than 6 months. We present case of a patient with MMIHS. CASE DESCRIPTION/METHODS: A 23-year-old female with MMIHS presented to the emergency department for abdominal pain and inability to tolerate oral or feeds through gastrostomy tube for 3 weeks. She had extensive surgical history since birth with LADD’s procedure due to intestine malrotation, exploratory laparotomy and subtotal colectomy for volvulous, colectomy with end ileostomy, Bilroth I, gastrostomy tube, and multiple nephrostomy tube for recurrent urinary infection. Patient had similar recurrent symptoms thought to be due to small intestinal bacterial overgrown and recurrent urinary tract infection. It appeared with each hospitalization her ability to tolerate oral intake declined to a lower baseline. At home patient was on Cisapride 14ml four times daily. During hospitalization lab work and imaging was non-contributory other than urine culture positive for Klebsiella. A small bowel follow through showed delayed gastric empting. Due to inability to tolerate oral intake or feeds patient was started on total parenteral nutrition (TPN). Esophagogastroduodenoscopy showed esophagitis, moderate chronic active gastritis with widely patent gastrojejunostomy anastomosis and jejunal limb. Her symptoms were likely due to progression of hypomotility syndrome. Cisapride was increased by 14%, under direction of outpatient pediatric gastroenterologist, with good response. However, due to prolonged QTc on electrocardiogram, upon discharge she was changed to Lubipristone. DISCUSSION: Our case demonstrates a complex presentation of a rare but fatal intestinal myopathy with most patients not surviving past infancy with even fewer cases surviving long term. Complications such as perforation, sepsis, malnutrition, multi-organ failure and pseudo-obstruction is not well understood in these patients and needs to be ruled out at each hospitalization. A multidisciplinary approach is needed for long term survival of patients with MMIHS. Main goals of treatment include providing a bladder-emptying strategy and adequate nutrition. In our case study, we demonstrate off label use of Cisapride and Lubiprostone for dysmotility. Close long term follow-up is necessary with multidisciplinary approach for long term survival.

Introduction: Limited guidance has been provided for opioid de-escalation when managing pain associated with vaso-occlusive crisis (VOC) in hospitalized patients with sickle cell disease (SCD). Therefore, a pain management protocol was developed for use in this population and piloted at a tertiary academic medical center.Objective: To assess implementation of a newly developed pain management protocol for use in hospitalized patients with a VOC.Methods: This was a retrospective, single-center, pre-post cohort study of SCD patients admitted with a VOC between June 1, 2018 to June 1, 2019 (pre-protocol) and December 31, 2019 to May 12, 2020 (post-protocol). The primary outcome was a reduction in intravenous (IV) short-acting opioid utilization as defined by the proportion of hospitalized days where the route of administration for short-acting opioids was oral rather than IV for ≥75% of doses given. Secondary outcomes included the proportion of hospitalized days where ≥50% and ≥25% of the short-acting opioid doses were administered by mouth. Opioid use (in morphine milligram equivalents [MME]) was also compared between groups.Results: A total of 96 admissions in the pre-protocol group and 29 admissions in the post-protocol group were included in the study. There was a statistically significant increase in the proportion of hospitalized days where ≥75% of the short-acting opioid doses were administered by mouth rather than IV in the post-protocol group compared with the pre-protocol group (0% vs 8.3%, P = .0114). Intravenous opioid use and total opioid use in the post-protocol group was lower compared with the pre-protocol group. Conclusion:Implementation of a pain management protocol for use in hospitalized SCD patients admitted with VOC was associated with an increase in the use of oral short-acting opioids and reduced overall opioid use.