Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso‐occlusive pain: A single institution experience

Carden, Marcus A.; Patil, Prabhumallikarjun; Ahmad, Mekaal E.; Lam, Wilbur A.; Joiner, Clinton H.; Morris, Claudia R.

doi:10.1002/pbc.26742

Cited by 12 publications

(17 citation statements)

References 6 publications

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“…6,7 Vaso-occlusive pain crisis (VOC) is associated with high use of health care resources, 6,8,9 yet there is no effective treatment that targets the pathophysiologic mechanism of acute VOC; symptomatic relief with hydration and analgesics is currently the only available treatment modality. [10][11][12] Nitric oxide (NO), enzymatically produced from L-arginine, is a potent vasodilator and exerts multiple effects on vascular and circulating blood cells, including the inhibition of platelet aggregation, downregulation of adhesion molecules, and modulation of ischemiareperfusion injury, 13 all pathways adversely affected during VOC. 14 Recently a specific metabolic signature was identified in SCA-VOC that differs from steady-state involving altered arginine-NO metabolism.…”

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confidence: 99%

Randomized control trial of oral arginine therapy for children with sickle cell anemia hospitalized for pain in Nigeria

et al. 2020

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Low arginine bioavailability is associated with vaso‐occlusive painful crisis (VOC) severity in sickle cell anemia (SCA) and predicts need for pediatric hospitalization. Intravenous arginine therapy has opioid‐sparing effects and was found to significantly decrease pain scores in children hospitalized with SCA‐VOC in a phase‐two randomized placebo‐controlled trial (RCT). Efficacy of oral arginine is unknown. Our objective was to determine the safety and efficacy of oral arginine therapy in Nigerian children with SCA. A double‐blind RCT of oral L‐arginine‐hydrochloride (100 mg/kg TID) was conducted in children with SCA‐VOC, aged 5‐17 years, hospitalized at two Nigerian sites. The primary outcome measure was analgesic usage, quantified by difference in the mean Analgesic Medication Quantification Scale (MQS). Secondary outcomes included daily pain scores, time‐to‐crisis‐resolution and length‐of‐hospital‐stay. An intention‐to‐treat analysis was performed. Sixty‐eight children (age 5‐17 years, mean 10.6 ± 0.4 years; 56% male), were randomized to receive L‐arginine (35 patients) or placebo (33 patients). The mean total MQS for the arginine group was 73.4 (95% CI, 62.4‐84.3) vs 120.0 (96.7‐143.3) for placebo (P < .001). The mean rate of decline in worst pain scores was faster in the arginine arm vs placebo (1.50 [1.23‐1.77] vs 1.09 [0.94‐1.24] point/d, P = .009). Children receiving arginine had a shorter time‐to‐crisis‐resolution (P = .02), shorter hospital‐stay (P = .002) and experienced no serious adverse event. Pain control was more rapid, total analgesic requirement was significantly reduced, and most notably, time‐to‐crisis‐resolution and length‐of‐hospital‐stay were shorter in children with SCA‐VOC receiving arginine vs placebo. Given the established safety and low cost, oral arginine is a promising adjuvant therapy for SCA‐VOC management.

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confidence: 99%

Randomized control trial of oral arginine therapy for children with sickle cell anemia hospitalized for pain in Nigeria

et al. 2020

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“…The IVFs (bolus and/or maintenance) were used in 84% of patients. 15,16 However, some practitioners discourage its use due to concerns that patients with kidney dysfunction, a common finding in sickle cell disease, may not effectively excrete its high sodium, hyperosmolar load. Mean triage pain scores were similar between groups, but improvement in pain scores from presentation-to-ED-disposition was smaller in the NSB group (2.2 vs 3.0, P = .03), while admission rates were higher (71% vs 59%, P = .01).…”

Section: Funding Informationmentioning

confidence: 99%

“…seek emergency department (ED) care. 16 Here we investigate the use of NSB on a larger scale. [3][4][5] However, evidence for utilizing IVF in euvolemic patients or guiding the clinicians' choice for IVFs is lacking, [3][4][5] has changed little over the last decade, [6][7][8] and was not adequately addressed by recent National Heart, Lung, and Blood Institute (NHLBI) guidelines on SCD management.…”

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confidence: 99%

“…13,14 Use of NSB during the treatment of sickle cell disease-related VOE in the ED is common. 15,16 However, some practitioners discourage its use due to concerns that patients with kidney dysfunction, a common finding in sickle cell disease, may not effectively excrete its high sodium, hyperosmolar load. In large volumes, normal saline can lead to hyperchloremic metabolic acidosis.…”

Section: Funding Informationmentioning

confidence: 99%

“…We also found that the use of NSB was associated with worse pain control. 16 Here we investigate the use of NSB on a larger scale.…”

Section: Funding Informationmentioning

confidence: 99%

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Normal saline bolus use in pediatric emergency departments is associated with poorer pain control in children with sickle cell anemia and vaso‐occlusive pain

et al. 2019

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Vaso‐occlusive pain events (VOE) are the leading cause of emergency department (ED) visits in sickle cell anemia (SCA). This study assessed the variability in use of intravenous fluids (IVFs), and the association of normal saline bolus (NSB), on pain and other clinical outcomes in children with SCA, presenting to pediatric emergency departments (PED) with VOE. Four‐hundred charts of children age 3‐21 years with SCA/VOE receiving parenteral opioids at 20 high‐volume PEDs were evaluated in a retrospective study. Data on type and amount of IVFs used were collected. Patients were divided into two groups: those who received NSB and those who did not. The association of NSB use on change in pain scores and admission rates was evaluated. Among 400 children studied, 261 (65%) received a NSB. Mean age was 13.8 ± 4.9 years; 46% were male; 92% had hemoglobin‐SS. The IVFs (bolus and/or maintenance) were used in 84% of patients. Eight different types of IVFs were utilized and IVF volume administered varied widely. Mean triage pain scores were similar between groups, but improvement in pain scores from presentation‐to‐ED‐disposition was smaller in the NSB group (2.2 vs 3.0, P = .03), while admission rates were higher (71% vs 59%, P = .01). Use of NSB remained associated with poorer final pain scores and worse change in pain scores in our multivariable model. In conclusion, wide variations in practice utilizing IVFs are common. NSB is given to >50% of children with SCA/VOE, but is associated with poorer pain control; a controlled prospective trial is needed to determine causality.

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Novel hydration and nutritional strategies for sickle cell disease

Purnell

Rayborn

2020

eJHaem

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Introduction Sickle cell disease and sickle cell trait affect over 300 million people worldwide. Vaso‐occlusive crises (VOCs) are the most common reason that these patients seek medical care. Objectives Recently, a newly identified “trigger” (involving glucose and electrolytes) for a mechanism of abnormal actin polymerization may offer further understanding with regard to the sequence of events that cascade to complications such as VOCs in those with sickle cell disease (SCD) and as well as those with sickle cell trait. Methods A literature review to identify the current standard of care guidelines for hydration and nutritional strategies during VOCs in patients with SCD and sickle cell trait was conducted in PubMed, OVID, and Google Scholar. Results This review suggested that current rationales for hydration and nutritional strategies for these patients during periods of crisis are generally based on consensus and have remained largely undefined to date. Conclusion This new trigger, along with this literature review, suggests investigations related to serum glucose and cation (electrolyte) levels may help define novel strategies for the development of protocols/standard of care with regard to intravenous and oral hydration/nutritional guidelines in these patients during both clinical and perioperative management periods.

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Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso‐occlusive pain: A single institution experience

Cited by 12 publications

References 6 publications

Randomized control trial of oral arginine therapy for children with sickle cell anemia hospitalized for pain in Nigeria

Randomized control trial of oral arginine therapy for children with sickle cell anemia hospitalized for pain in Nigeria

Normal saline bolus use in pediatric emergency departments is associated with poorer pain control in children with sickle cell anemia and vaso‐occlusive pain

Novel hydration and nutritional strategies for sickle cell disease

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