Factors Associated With Mechanical Ventilation Use in Children With Sickle Cell Disease and Acute Chest Syndrome

Abstract Acute chest syndrome (ACS) is the most common cause of death during hospitalization of adults with sickle cell disease (SCD). ACS includes symptoms referable to the chest and a new infiltrate on chest X-ray. Adults over age 20 years have more symptoms of the disease and are at increased risk of early death compared to children. ACS may be the presenting diagnosis for a patient with SCD, but equally as often, develops while the patient has a painful vascular occlusive crisis. Notably, 35% of SCD patients have a normal lung exam upon presentation to the hospital. Previous research studies indicate that nearly three-fourths of SCD patients who die present during painful crises in an extremity, and about 50% conclusively by autopsy died of massive fat embolism syndrome (FES). Unfortunately, definitive diagnostic tests with rapid turn-around for FES and other acute vascular occlusive lung events do not exist. Earlier identification of the danger that this event may be evolving can be life saving. Clinicians who adhere to the strict definition of ACS may prematurely dismiss the likelihood of a subsequent fatal event. This alarming rate of adverse events may represent a “pre-chest syndrome” prodromal phase of ACS. Arterial hypoxemia syndrome (AHS) or pre-chest syndrome is defined as any sign or symptom referable to the chest, an oxygen saturation (Sp02) of <94% by direct pulse oximeter or a Pa02 <80% by arterial blood gas on room air plus a clear chest X-ray with or without fever. AHS may be a warning sign of an ultimately fatal event if earlier interventions are not done in a timely manner. A secondary data analysis was performed utilizing 500 health records of SCD patients from 1960 to 2004. Prior to 2003, we averaged 2 to 3 ICU admissions per month for ACS with about 20% requiring mechanical ventilation. This study sought to gain insight on 45 years of experience in the treatment of SCD, particularly “pre-chest syndrome.” The primary aims of the study were to devise treatment protocols to reduce ICU admissions and the need for mechanical ventilation in SCD patients presenting with AHS. Retrospective analysis suggests that earlier blood exchanges for patients with SCD may substantially reduce ICU admissions and the need for mechanical ventilation in patients presenting with AHS, compared with patients receiving standard supportive care. Examination of computerized hospital records of 437 sickle cell hospital admissions from January 2003 to March 2005 revealed 3 ICU and 2 step-down unit admissions. During this time period, there were 101 chest syndrome occurrences, of which 2 died. Both patients required mechanical ventilation and underwent red cell apheresis to reduce hemoglobin S to <30%. One patient was admitted due to major trauma from a motor vehicle accident. Death was due to multi-organ failure. The medical condition of the second patient improved. This patient was discharged home in stable condition but died, unexpectedly, 48 hours at home of a massive pulmonary embolus. A protocol has been developed to prospectively evaluate our aims.

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Pulse oximetry and factors associated with hemoglobin oxygen desaturation in children with sickle cell disease

Blood ◽

10.1182/blood.v81.12.3422.bloodjournal81123422 ◽

1993 ◽

Vol 81 (12) ◽

pp. 3422-3427 ◽

Cited By ~ 1

Author(s):

WR Rackoff ◽

N Kunkel ◽

JH Silber ◽

T Asakura ◽

K Ohene-Frempong

Keyword(s):

Sickle Cell Disease ◽

Pulse Oximetry ◽

Sickle Cell ◽

Arterial Oxygen Saturation ◽

Oxygen Desaturation ◽

Arterial Oxygen ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Factors Associated ◽

History Of

The observation of low transcutaneous arterial oxygen saturation (SaO2) in otherwise well sickle cell patients has lead to questions about the interpretation of pulse oximetry values in these patients. We undertook a prospective study of children with sickle cell disease to (1) determine the prevalence of, and factors associated with, low transcutaneous SaO2 in clinically well patients, (2) develop an algorithm for the use of pulse oximetry in acutely ill patients, and (3) assess the accuracy of pulse oximetry in these patients. Eighty-six clinically well children with hemoglobin (Hb) SS had a lower mean transcutaneous SaO2 than 22 Hb SC patients and 10 control subjects (95.6% v 99.1% v 99.0%, respectively; p < .001). In Hb SS patients, a history of acute chest syndrome and age greater than 5 years were associated with lower transcutaneous SaO2 (mean 93.8% for those with a history of acute chest syndrome v 97.8% for those without a history of acute chest syndrome, and 94.0% for patients = 5 years old v 97.2% for those < or = 5 years old; P < .001). These associations were not seen in Hb SC patients. During acute illness, Hb SS patients with acute chest syndrome had transcutaneous SaO2 values that were less than 96% and at least 3 points lower than measurements made when they were well. A nomogram was designed to aid in the interpretation of transcutaneous SaO2 in acutely ill Hb SS patients when a comparison value is not available. The accuracy of pulse oximetry was shown by the correlation between SaO2 measured by pulse oximetry and calculated by using the patient's oxygen dissociation curve and PaO2 (r = .97). This study provides evidence that Hb oxygen desaturation is not a universal finding among children with sickle cell disease and identifies factors associated with Hb oxygen desaturation. We conclude that pulse oximetry may be useful to assess whether progressive pulmonary dysfunction begins at an early age in Hb SS patients, and to assess acutely ill patients for the presence of hypoxemia associated with acute chest syndrome.

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Pulse oximetry and factors associated with hemoglobin oxygen desaturation in children with sickle cell disease

Blood ◽

10.1182/blood.v81.12.3422.3422 ◽

1993 ◽

Vol 81 (12) ◽

pp. 3422-3427 ◽

Cited By ~ 66

Author(s):

WR Rackoff ◽

N Kunkel ◽

JH Silber ◽

T Asakura ◽

K Ohene-Frempong

Keyword(s):

Sickle Cell Disease ◽

Pulse Oximetry ◽

Sickle Cell ◽

Arterial Oxygen Saturation ◽

Oxygen Desaturation ◽

Arterial Oxygen ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Factors Associated ◽

History Of

Abstract The observation of low transcutaneous arterial oxygen saturation (SaO2) in otherwise well sickle cell patients has lead to questions about the interpretation of pulse oximetry values in these patients. We undertook a prospective study of children with sickle cell disease to (1) determine the prevalence of, and factors associated with, low transcutaneous SaO2 in clinically well patients, (2) develop an algorithm for the use of pulse oximetry in acutely ill patients, and (3) assess the accuracy of pulse oximetry in these patients. Eighty-six clinically well children with hemoglobin (Hb) SS had a lower mean transcutaneous SaO2 than 22 Hb SC patients and 10 control subjects (95.6% v 99.1% v 99.0%, respectively; p < .001). In Hb SS patients, a history of acute chest syndrome and age greater than 5 years were associated with lower transcutaneous SaO2 (mean 93.8% for those with a history of acute chest syndrome v 97.8% for those without a history of acute chest syndrome, and 94.0% for patients = 5 years old v 97.2% for those < or = 5 years old; P < .001). These associations were not seen in Hb SC patients. During acute illness, Hb SS patients with acute chest syndrome had transcutaneous SaO2 values that were less than 96% and at least 3 points lower than measurements made when they were well. A nomogram was designed to aid in the interpretation of transcutaneous SaO2 in acutely ill Hb SS patients when a comparison value is not available. The accuracy of pulse oximetry was shown by the correlation between SaO2 measured by pulse oximetry and calculated by using the patient's oxygen dissociation curve and PaO2 (r = .97). This study provides evidence that Hb oxygen desaturation is not a universal finding among children with sickle cell disease and identifies factors associated with Hb oxygen desaturation. We conclude that pulse oximetry may be useful to assess whether progressive pulmonary dysfunction begins at an early age in Hb SS patients, and to assess acutely ill patients for the presence of hypoxemia associated with acute chest syndrome.

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Nationwide retrospective study of critically ill adults with sickle cell disease in France

Scientific Reports ◽

10.1038/s41598-021-02437-2 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Maïté Agbakou ◽

Armand Mekontso-Dessap ◽

Morgane Pere ◽

Guillaume Voiriot ◽

Muriel Picard ◽

...

Keyword(s):

Retrospective Study ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Adverse Outcome ◽

Adverse Outcomes ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Factors Associated ◽

Icu Admission ◽

Arterial Blood

AbstractLittle is known about patients with sickle cell disease (SCD) who require intensive care unit (ICU) admission. The goals of this study were to assess outcomes in patients admitted to the ICU for acute complications of SCD and to identify factors associated with adverse outcomes. This multicenter retrospective study included consecutive adults with SCD admitted to one of 17 participating ICUs. An adverse outcome was defined as death or a need for life-sustaining therapies (non-invasive or invasive ventilation, vasoactive drugs, renal replacement therapy, and/or extracorporeal membrane oxygenation). Factors associated with adverse outcomes were identified by mixed multivariable logistic regression. We included 488 patients admitted in 2015–2017. The main reasons for ICU admission were acute chest syndrome (47.5%) and severely painful vaso-occlusive event (21.3%). Sixteen (3.3%) patients died in the ICU, mainly of multi-organ failure following a painful vaso-occlusive event or sepsis. An adverse outcome occurred in 81 (16.6%; 95% confidence interval [95% CI], 13.3%–19.9%) patients. Independent factors associated with adverse outcomes were low mean arterial blood pressure (adjusted odds ratio [aOR], 0.98; 95% CI 0.95–0.99; p = 0.027), faster respiratory rate (aOR, 1.09; 95% CI 1.05–1.14; p < 0.0001), higher haemoglobin level (aOR, 1.22; 95% CI 1.01–1.48; p = 0.038), impaired creatinine clearance at ICU admission (aOR, 0.98; 95% CI 0.97–0.98; p < 0.0001), and red blood cell exchange before ICU admission (aOR, 5.16; 95% CI 1.16–22.94; p = 0.031). Patients with SCD have a substantial risk of adverse outcomes if they require ICU admission. Early ICU admission should be encouraged in patients who develop abnormal physiological parameters.

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ECMO Therapy in Acute Chest Syndrome for Patients with Sickle Cell Disease: a Case Report and Literature Review

SN Comprehensive Clinical Medicine ◽

10.1007/s42399-021-00987-0 ◽

2021 ◽

Author(s):

Soi Avgeridou ◽

Ilija Djordjevic ◽

Anton Sabashnikov ◽

Kaveh Eghbalzadeh ◽

Laura Suhr ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Scientific Data ◽

Acute Chest Syndrome ◽

Data Sets ◽

Limited Data ◽

Cell Disease ◽

Life Saving ◽

Institutional Experience ◽

Ecmo Therapy

AbstractExtracorporeal membrane oxygenation (ECMO) plays an important role as a life-saving tool for patients with therapy-refractory cardio-respiratory failure. Especially, for rare and infrequent indications, scientific data is scarce. The conducted paper focuses primarily on our institutional experience with a 19-year-old patient suffering an acute chest syndrome, a pathognomonic pulmonary condition presented by patients with sickle cell disease. After implementation of awake ECMO therapy, the patient was successfully weaned off support and discharged home 22 days after initiation of the extracorporeal circulation. In addition to limited data and current literature, further and larger data sets are necessary to determine the outcome after ECMO therapy for this rare indication.

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Streptococcus pneumoniae and Its Virulence Factors H2O2 and Pneumolysin Are Potent Mediators of the Acute Chest Syndrome in Sickle Cell Disease

Toxins ◽

10.3390/toxins13020157 ◽

2021 ◽

Vol 13 (2) ◽

pp. 157

Author(s):

Joyce Gonzales ◽

Trinad Chakraborty ◽

Maritza Romero ◽

Mobarak Abu Mraheil ◽

Abdullah Kutlar ◽

...

Keyword(s):

Streptococcus Pneumoniae ◽

Sickle Cell Disease ◽

Virulence Factors ◽

Sickle Cell ◽

Biological Activities ◽

Acute Chest Syndrome ◽

Antibiotics Resistance ◽

Cell Disease ◽

Antibiotic Drug ◽

Tract Infections

Sickle cell disease (SCD) is one of the most common autosomal recessive disorders in the world. Due to functional asplenia, a dysfunctional antibody response, antibiotic drug resistance and poor response to immunization, SCD patients have impaired immunity. A leading cause of hospitalization and death in SCD patients is the acute chest syndrome (ACS). This complication is especially manifested upon infection of SCD patients with Streptococcus pneumoniae (Spn)—a facultative anaerobic Gram-positive bacterium that causes lower respiratory tract infections. Spn has developed increased rates of antibiotics resistance and is particularly virulent in SCD patients. The primary defense against Spn is the generation of reactive oxygen species (ROS) during the oxidative burst of neutrophils and macrophages. Paradoxically, Spn itself produces high levels of the ROS hydrogen peroxide (H2O2) as a virulence strategy. Apart from H2O2, Spn also secretes another virulence factor, i.e., the pore-forming exotoxin pneumolysin (PLY), a potent mediator of lung injury in patients with pneumonia in general and particularly in those with SCD. PLY is released early on in infection either by autolysis or bacterial lysis following the treatment with antibiotics and has a broad range of biological activities. This review will discuss recent findings on the role of pneumococci in ACS pathogenesis and on strategies to counteract the devastating effects of its virulence factors on the lungs in SCD patients.

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To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

Transfusion Medicine and Hemotherapy ◽

10.1159/000512644 ◽

2021 ◽

pp. 1-5

Author(s):

Justin E. Juskewitch ◽

Craig D. Tauscher ◽

Sheila K. Moldenhauer ◽

Jennifer E. Schieber ◽

Eapen K. Jacob ◽

...

Keyword(s):

Pregnant Woman ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Pregnancy Termination ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Childbearing Age ◽

Procedural Complications ◽

History Of ◽

Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

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