Guidelines for the management of emergencies and critical illness in pediatric and adult patients with sickle cell disease
SRLF et al. · 2025 · France
Source and licence
- Authors
- Armand Mekontso Dessap, Stephane Dauger, Mehdi Khellaf et al.
- Title
- Guidelines for the management of emergencies and critical illness in pediatric and adult patients with sickle cell disease
- Journal
- Annals of Intensive Care, 2025
- Licence
- CC BY 4.0
- Changes
- Verbatim excerpts; formatting adapted
Full text retrieved 2026-10-27 from Europe PMC · PMC12123041
Licence statement of the original publication
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Recommendations
Verbatim, with grade of recommendation or strength of consensus where given.
Admission to ICU
- Adults: Admission to ICU should probably be considered in adults with SCD who need red blood cell exchange or are at risk of an unfavourable outcome, such as those with tachypnea, acute kidney injury, or hypotensionLevel of proof: Grade 2+, Strong agreement
- Paediatrics: The experts suggest considering ICU admission for children with SCD in cases of uncontrolled hyperalgic crisis, poorly tolerated acute anemia, acute neurological event, hypoxemic respiratory distress, or any acute organ failureLevel of proof: Expert opinion, Strong agreement
Reference centers
- The experts recommend consulting a reference center for an expert opinion or arranging a transfer for adult and paediatric patients with severe acute complications of SCD or those at risk of rapid deterioration While the list of these complications is not exhaustive, it includes: - Any acute organ failure requiring admission to the ICU or HDU; - ACS, especially if requiring respiratory support (high-flow oxygen, CPAP, non-invasive or invasive ventilation) or if associated with pulmonary hypertension (tricuspid regurgitant jet velocity > 3 m/s or acute cor pulmonale); - Acute anemia with a high risk, suspicion or confirmation of delayed haemolytic transfusion reaction (DHTR); - Stroke (hemorrhagic or ischemic); - Severe hepatic VOC, sequestration, or cholestasis (with encephalopathy, prothrombin time < 50%, or total bilirubin > 15 mg/dL or 250 micromol/L); - Extensive medullary necrosis, suggested by severe VOC, potentially with neurological signs, a significant decrease in haemoglobin and platelets (typically < 100 G/L), and a marked increase in lacticodeshydrogenase (typically > 2000 IU/L); - Splenic sequestration (usually suggested by a painful increase in spleen size and a decrease of more than 2 g/dL in haemoglobin concentration); - Acute complication in a transplanted patient (kidney, liver, heart); - Acute complication in a pregnant woman; - Sepsis or septic shock; meningitis in children; - Acute priapism; - Acute complication requiring surgery; - Sudden deafness or blindnessLevel of proof: Expert opinion, Strong agreement
Pain relief
- Adults: The experts suggest immediately initiating a protocolized analgesic treatment algorithm (Fig. 1) in adults with SCD presenting with a painful crisisLevel of proof: Expert opinion, Strong agreement
- Paediatrics: A protocolized analgesic treatment algorithm (Fig. 2) should probably be initiated immediately in children with SCD presenting with a painful crisisLevel of proof: Grade 2+, Strong agreement
Oxygen saturation target
- The experts suggest maintaining a transcutaneous oxygen saturation target of 95% during VOC in adults and children with SCDLevel of proof: Expert opinion, Strong agreement
Incentive spirometry
- Incentive spirometry should probably be used to prevent the onset of ACS in adults and children with SCD who experience VOC, especially in cases of chest or back pain in adultsLevel of proof: Grade 2+, Strong agreement
Hydration
- Systematic overhydration is probably not advisable in adults and children with SCD experiencing VOC, due to the potential for serious complications and the lack of demonstrated benefitsLevel of proof: Grade 2-, Strong agreement
- Adults: The experts suggest adjusting fluid intake, whether oral or intravenous in adults with VOC, based on the patient's clinical status (hydration, respiratory), other fluid inputs (such as erythrocyte transfusions), and daily fluid requirementsLevel of proof: Expert Opinion, Strong agreement
- Paediatrics: The experts suggest initially limiting fluid intake in children with VOC to 1.5L/m2/day, without exceeding 2L/m2/day (or a maximum of 3L/day)Level of proof: Expert Opinion, Strong Agreement
Lung ultrasound
- Adults: Lung ultrasound should probably be used to improve the diagnosis of ACS in adults with SCD exhibiting clinical signs suggestive of ACSLevel of proof: Grade 2+, Strong Agreement
- Paediatrics: Either lung ultrasound or standard chest X-ray should probably be used for the diagnosis of ACS in children with SCD exhibiting clinical signs suggestive of ACSLevel of proof: Grade 2+, Strong Agreement
CT scan
- Adults: The experts suggest that, after assessment of pretest clinical probability, adults with ACS undergo a CT scan to search pulmonary artery thrombosisLevel of proof: Expert opinion, Strong Agreement
- Pediatrics: The experts suggest that children admitted to the PICU with severe ACS complicated by right ventricular failure undergo a CT scan to search pulmonary artery thrombosisLevel of proof: Expert opinion, Strong Agreement
Echocardiography
- Adults: Right ventricular stress should probably be assessed with echocardiography for prognostic evaluation in adult patients with severe ACSLevel of proof: Grade 2+, Strong Agreement
- Paediatrics: The experts suggest that in children, right ventricular stress be assessed with echocardiography for prognostic evaluation in pediatric patients with severe ACSLevel of proof: Expert opinion, Strong Agreement
Systemic corticosteroids
- Systemic corticosteroid therapy should probably not be used routinely for the management of ACS in adults and children with SCDLevel of proof: Grade 2-, Strong agreement
Non-invasive ventilation
- Adults: Non-invasive ventilation should probably not be used routinely during ACS in adults with SCDLevel of proof: Grade 2-, Strong agreement
- Paediatrics: The experts suggest testing the efficacy and tolerance of non-invasive ventilation on a case-by-case basis during ACS in children with SCDLevel of proof: Expert opinion, Strong agreement
Inhaled nitric oxide
- High-dose nitric oxide should probably not be used routinely for the management of adults and children with SCD developing VOC or ACSLevel of proof: Grade 2-, Strong Agreement
Therapeutic anticoagulation
- Currently available data do not allow a recommendation to be made regarding the use of therapeutic anticoagulation in adult and pediatric patients with ACS without documented deep vein thrombosis or pulmonary artery thrombosisLevel of proof: No specific recommendation
Prophylactic anticoagulation
- Adults: Thromboprophylaxis is indicated in acutely ill medical patients, including those with ACSLevel of proof: No specific recommendation
- Paediatrics: Prophylactic anticoagulation should probably be used in children with SCD with ACS in the following cases: pubertal onset, a history of deep vein thrombosis, central venous catheterization, COVID-19, or Moya-Moya disease (after an individual assessment of the risk–benefit ratio)Level of proof: Grade 2+, Strong Agreement
Procalcitonin
- The panel makes no recommendation on procalcitonin for initiating antibiotic treatment in adult and children with ACSLevel of proof: No recommendation, Strong agreement
Antibiotics for ACS
- Adults: The experts suggest administering empirical antibiotic targeting intracellular bacteria in combination with an antibiotic targeting pyogenic bacteria in cases of severe ACS in adultsLevel of proof: Expert opinion, Strong agreement
- Paediatrics: Empirical antibiotic therapy targeting intracellular pathogens should probably be used in combination with an antibiotic targeting pyogenic bacteria in children with ACSLevel of proof: Grade 2+, Strong agreement
Intravenous beta-lactams in patients with SCD
- In adult and pediatric patients with SCD experiencing severe or difficult-to-treat infections, and without renal insufficiency, intravenous beta-lactams should probably be administered using a loading dose followed by continuous administration, or an increased number of doses per day, in order to improve their pharmacokinetics and pharmacodynamicsLevel of proof: Grade 2+, Strong agreement
Transfusion modality
- In the event of an acute complication warranting a transfusion in an adult or paediatric patient with SCD, the experts suggest prioritizing simple transfusion if the anemia is severe (total hemoglobin < 7 g/dL) and immediately performing an exchange transfusion (manual or automated) in other casesLevel of proof: Expert opinion, Strong agreement
- Paediatrics: In the case of an acute complication warranting a transfusion in a child with SCD, the experts suggest not to exceed a post-procedure hematocrit of 33% ± 3%Level of proof: Expert opinion, Strong agreement
Indications for blood transfusion
- The experts suggest against the systematic use of transfusion therapy for uncomplicated VOC in adult and paediatric patients with SCDLevel of proof: Expert opinion, strong agreement
- Adults: The experts suggest performing a transfusion therapy for the management of ACS in adult patients with SCD who meet severity criteria with regard to hypoxemia, severe pulmonary hypertension (tricuspid regurgitation velocity > 3 m/s or acute cor pulmonale), or any associated organ failureLevel of proof: Expert opinion, Strong agreement
- Pediatrics: The experts suggest performing a transfusion therapy for the management of ACS in pediatric patients with SCD who meet severity criteria, with regard to rapidly worsening hypoxemia, or any associated organ failureLevel of proof: Expert opinion, Strong agreement
- The experts suggest urgent transfusion therapy, ideally within two hours of symptom onset, for adult and paediatric patients with SCD showing signs of stroke. Automated exchange transfusion should be prioritized if time allows, or manual exchange if there are delays in accessing automated techniques. In cases where haemoglobin levels are below 7 g/dl, a simple transfusion is suggested while awaiting exchange transfusion to reach haemoglobin S (or S + C) below 30% with haematocrit 30 ± 3% Haemorrhagic stroke may also require exchange transfusion or simple transfusion, especially when neurosurgical procedures are indicatedLevel of proof: Expert opinion, Strong agreement
- The experts suggest transfusion therapy for persistent acute ischemic priapism in adult and paediatric patients with SCD when non-invasive and invasive treatments (aspiration and/or intracavernosal injection) fail or when priapism has been ongoing for at least four hoursLevel of proof: Expert opinion, Strong agreement
- The experts suggest transfusion therapy in cases of splenic sequestration in adult and paediatric patients with SCDLevel of proof: Expert opinion, Strong agreement
- The experts suggest transfusion therapy in the event of acute organ failureLevel of proof: Expert opinion, Strong agreement
- Transfusion therapy should probably not be performed in adult and pediatric patients with SCD presenting with Delayed Haemolytic Transfusion ReactionLevel of proof: Grade 2-, Strong Agreement
Diagnosis
- Adults: The Mekontso Dessap nomogram should probably be used to diagnose DHTR in adult patients with SCDLevel of proof: Grade 2+, Strong agreement
- Paediatrics: The experts suggest using the Mekontso Dessap nomogram to diagnose DHTR in children with SCDLevel of proof: Expert opinion, Strong agreement
- The experts suggest systematically performing a complete blood count and haemoglobin electrophoresis within 48 h following RBC transfusion in adult and pediatric patients with SCD at risk of DHTRLevel of proof: Expert opinion, Strong agreement
Prediction
- Adults: The experts suggest using the Narbey predictive score to assess the risk of DHTR before transfusion in adult patients with SCD. They suggest categorizing the risk as: i) not significant if the score is less than 8; and ii) significant if the score equals or exceeds 8, or if the patient has a history of confirmed DHTRLevel of proof: Expert opinion, Strong agreement
- Paediatrics: The Narbey predictive score should probably not be used in children with SCD to assess the risk of DHTRLevel of proof: Grade 2-, Strong agreement
Priapism
- Adults: In cases of persistent, painful erection in an adult with SCD suggesting acute ischemic priapism, experts recommend promptly initiating non-invasive measures (such as massages, physical exertion, and possibly oral alpha-adrenergic agonists). If priapism lasts for more than an hour, an intracavernosal injection of alpha-adrenergic agonists should be considered, with a repeat injection after 20 min if the first is ineffective, the objective being to minimize complications and long-term sequelaeLevel of proof: Expert opinion, Strong agreement
- Adults: For ischemic priapism persisting for four hours or more in adults with SCD, experts recommend aspirating cavernosal blood, followed by an intracavernous injection of alpha-adrenergic agonists. These steps should be repeated multiple times before considering surgical intervention, the objective being to minimize complications and long-term sequelaeLevel of proof: Expert opinion, Strong agreement
- Paediatrics: Experts suggest performing an intracavernous injection of alpha-adrenergic agonists and draining the corpora cavernosa in a child with SCD who has been experiencing acute priapism for more than an hourLevel of proof: Expert opinion, Strong agreement
First area: referral of patients
- R1.1Adults: Admission to ICU should probably be considered in adults with SCD who need red blood cell exchange or are at risk of an unfavourable outcome, such as those with tachypnea, acute kidney injury, or arterial hypotension.Grade 2+, strong agreement
- R1.2Pediatrics: The experts suggest considering ICU admission for children with SCD in cases of uncontrolled hyperalgic crisis, poorly tolerated acute anemia, acute neurological event, hypoxemic respiratory distress, or any acute organ failure.Expert opinion, strong agreement
Anti-nociceptive
- Not recommended as first-line. Experts suggest using NSAIDs on an individualized basis, especially considering suspected triggers and the patient's history (good usual therapeutic response, renal insufficiency, etc.)Grade: GRADE 2-; Agreement: Strong
- Recommended immediately if severe pain (VAS ≥ 60 mm) or refractory to strong non-opioid analgesics Experts suggest prioritizing the administration of strong opioids intravenously using the PCA method earlyClass: Strong opioids**; Grade: Expert opinion; Agreement: Strong
- Recommended for severe pain (VAS ≥ 60 mm) without immediate vein accessGrade: Expert opinion; Agreement: Strong
- Recommended when high doses of strong opioids are needed (≥1 mg/kg oral morphine equivalent over a 4-h period) Experts suggest the following usage modality: a slow 0.25 mg/kg IV bolus followed by a continuous infusion between 0.1 and 0.25 mg/kg/hGrade: Expert opinion; Agreement: Strong
- Not recommended as a first-line treatment Reserved for painful proceduresGrade: Expert opinion; Agreement: Strong
- Recommended for neuropathic pain elements and localized painClass: Modulator of transmission and peripheral sensitization; Grade: Expert opinion; Agreement: Strong
Second area: VOC
- R4Oxygen saturation target
- R5Incentive spirometry
Third area: ACS
- R8.1Adults: The experts suggest that, after assessment of pretest clinical probability, adults with ACS undergo a CT scan to search for pulmonary artery thrombosis.Expert opinion, Strong Agreement
- R8.2Pediatrics: The experts suggest that children admitted to the PICU with severe ACS complicated by right ventricular failure undergo a CT scan to search for pulmonary artery thrombosis.Expert opinion, Strong Agreement
- R9.2Pediatrics: The experts suggest that in children, right ventricular stress be assessed with echocardiography for prognostic evaluation in pediatric patients with severe ACSExpert opinion, Strong Agreement
- R10Systemic corticosteroids
- R11Non-invasive ventilation
- R12Inhaled nitric oxide
- High-dose nitric oxide should probably not be used routinely for the management of adult and children with SCD developing VOC or ACS.Grade 2-, Strong Agreement
- R13Therapeutic anticoagulation
- R14Prophylactic anticoagulation
- R14.1Adults: Thromboprophylaxis is indicated in acutely ill medical patients, including those with ACS. (No specific recommendation)
- R16.1Adults: The experts suggest administering an empirical antibiotic targeting intracellular bacteria in combination with an antibiotic targeting pyogenic bacteria in cases of severe ACS in adults.Expert opinion, Strong agreement
- R17Intravenous beta-lactams in patients with SCD
Fourth area: transfusion therapy
- R18Transfusion modality
- R18.1Adults & paediatrics: In the event of an acute complication warranting a transfusion in an adult or pediatric patient with SCD, the experts suggest prioritizing simple transfusion if the anemia is severe (total haemoglobin < 7 g/dL) and immediately performing an exchange transfusion (manual or automated) in other cases.Expert opinion, Strong agreement
- R18.2Paediatrics: In case of an acute complication warranting a transfusion in a child with SCD, the experts suggest not to exceed a post-procedure hematocrit of 33 ± 3%.Expert opinion, Strong agreement
- R19Indications for blood transfusion
- R19.2Adults: The experts suggest performing a transfusion therapy for the management of ACS in adult patients with SCD who meet severity criteria with regard to hypoxemia (need for oxygen therapy to achieve transcutaneous oxygen saturation of 95%), severe pulmonary hypertension (tricuspid regurgitation velocity > 3 m/s or acute cor pulmonale), or any associated organ failure.Expert opinion, Strong agreement
- R19.3Paediatrics: The experts suggest performing a transfusion therapy for the management of ACS in pediatric patients with SCD who meet severity criteria, with regard to rapidly worsening hypoxemia, or any associated organ failure.Expert opinion, Strong agreement
- R19.6Splenic sequestration
- R19.8Delayed haemolytic transfusion reaction (DHTR)
- R20.3The experts suggest systematically performing a complete blood count and haemoglobin electrophoresis within 48 h following RBC transfusion in adult and paediatric patients with SCD at risk of DHTR.Expert opinion, Strong agreement
Abstract
Forty-two questions were evaluated concerning management of emergencies and critical illnesses in paediatric and adult patients with sickle cell disease. The assessment covered the following areas: patient referral, vaso-occlusive crisis, acute chest syndrome, transfusion therapy, and priapism. The patient referral category included guidelines for admission to intensive care unit and management at specialized reference centers. The vaso-occlusive crisis topic encompassed pain management, hydration, incentive spirometry, and target oxygen saturation levels. For acute chest syndrome, the focus areas included imaging techniques such as lung ultrasound, computed tomography scans, and echocardiography; treatment with systemic corticosteroids; non-invasive ventilation; prophylactic and therapeutic anticoagulation; and procalcitonin and antibiotic therapy. The section on transfusion therapy addressed indications and methods of transfusion, as well as the diagnosis and prediction of delayed hemolytic transfusion reactions. A total of 45 recommendations were proposed, including 14 specific to adults, 13 specific to pediatrics, and 18 applicable to both adults and children, along with three therapeutic algorithms. The Grade of Recommendation Assessment, Development, and Evaluation (GRADE) methodology was adhered to throughout the process. Sixteen recommendations were based on a low level of evidence (GRADE 2+ or 2−), while 26 were based on evidence that could not be classified under the GRADE system and were therefore considered expert opinions. Finally, for three aspects of sickle cell disease management, the experts concluded that no reliable recommendations could be made based on the current state of knowledge. The recommendations and therapeutic algorithms received strong agreement from the experts.
Supplementary Information: The online version contains supplementary material available at 10.1186/s13613-025-01479-3.
Contents of the original publication
The section headings of the original.
- Introduction
- Methods
- Results
- First area: referral of patients
- Second area: VOC
- Third area: ACS
- Fourth area: transfusion therapy
- Fifth area: priapism
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