The use of extracorporeal membrane oxygenation in pediatric patients with sickle cell disease

Abstract

Previous reports have described the use of extracorporeal membrane oxygenation (ECMO) for acute chest syndrome of sickle cell disease (SCD). However, there have been no reports of venoarterial (VA) ECMO for cardiac dysfunction in patients with SCD. We describe a patient with SCD and life-threatening cardiogenic shock who was successfully treated with VA ECMO. Furthermore, SCD patients have unique comorbidities that warrant particular consideration when utilizing ECMO. We discuss these considerations and review the documented experience with ECMO for pediatric SCD patients from the Extracorporeal Life Support Organization (ELSO) registry. From 1990 until 2012, 52% of the 65 pediatric patients with SCD placed on ECMO survived, with 85% of those receiving venovenous (VV) ECMO surviving and 43% of those receiving VA ECMO surviving. However, significant complications, such as bleeding, neurological injury and kidney injury, also occurred with both VV and VA ECMO. Ten percent of SCD patients receiving VA ECMO experienced either a cerebral infarct or hemorrhage; our patient suffered a cerebrovascular accident while on ECMO, though she survived with good neurologic outcome. To our knowledge, this is the first report of a pediatric patient with SCD and cardiogenic shock successfully managed with VA ECMO. In conjunction with the ELSO registry review, this case report suggests that, while VA ECMO can be successfully used in patients with SCD and severe cardiovascular dysfunction, clinicians should also be aware of the potential for serious complications in this high-risk population.

Guideline on the management of acute chest syndrome in sickle cell disease

“…The majority of patients can be managed with conventional mechanical ventilation. There are occasional case reports of the use of high frequency oscillatory ventilation (Wratney et al , ) and using systems such as extracorporeal membrane oxygenation (Gillett et al , ; Pelidis et al , ; Kuo et al , ). It is impossible to make recommendations based on these historical case reports, particularly as the techniques involved have progressed in the time since the reports were published, and they do not reflect current practice.…”

Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review

“…[65][66][67] We recommend that in states of hypotension and shock in patients with SCD, vasoactive agents would be beneficial. 68,69 Myocardial injury or infarctions due to fat embolism in patients with SCD have been reported. 70 The management of these patients is exchange transfusion or multiple red blood cell transfusion.…”

“…70 Extracorporeal membrane oxygenation has also shown to be beneficial in patients with SCD having ACS. 68,69 It is the opinion of the authors of this article that in patients with SCD having ACS or severe VOCs unalleviated with high doses of analgesics, clinicians are to have a high index of suspicion for FES. We recommend the early application of a scoring system to detect patients who may be having FES.…”

Particularités de l’ECMO pour le syndrome de détresse respiratoire aiguë en pédiatrie

“…En effet, un certain nombre d'affections héréditaires ou acquises ont bénéficié ces dernières années de grands progrès permettant d'envisager une survie et une qualité de vie avec atteinte de l'âge adulte, ce qui n'était pas le cas auparavant. Ainsi, l'ECMO est proposé dans des SDRA chez des enfants immunodéprimés, des patients drépanocytoses homozygotes, des insuffisances respiratoires chroniques, des cardiopathies congénitales… [35][36][37].…”