Sickle Cell Acute Chest Syndrome

Treat ACS as present when a patient with sickle cell disease develops a new pulmonary infiltrate involving at least one complete lung segment consistent with alveolar consolidation, excluding atelectasis, plus chest pain, temperature above 38.5°C, tachypnea, wheezing or rales, or cough. A practical emergency definition also includes a new chest-imaging radiodensity with respiratory symptoms and/or fever. cdn clinicaltrials+1cdn clinicaltrials[PDF] 14 February 2025 Re: Cover Letter for ClinicalTrials.gov ...PubMedHigh risk and low prevalence diseases: Acute chest syndrome in sickle cell disease - PubMed

Obtain pulse oximetry, focused respiratory examination, and chest radiography at presentation. Chest radiography is the customary first-line imaging test; consider chest CT when the diagnosis remains uncertain or further imaging is needed after the initial radiograph. Lung ultrasound is a promising adjunct but does not replace clinical reassessment in a deteriorating patient. PubMedPubMedHigh risk and low prevalence diseases: Acute chest syndrome in sickle cell disease - PubMed

Admit patients with ACS. Obtain early hematology input and involve intensive care for increasing oxygen requirement, worsening work of breathing, refractory hypoxemia, extensive bilateral infiltrates, or rapid clinical decline. ACS is a leading cause of ICU admission in adults with sickle cell disease and may progress to respiratory failure requiring mechanical ventilation in 20% of adult patients. ScienceDirect+1ScienceDirectThe many facets of cardiopulmonary complications in sickle cell disease - ScienceDirectPubMedHigh risk and low prevalence diseases: Acute chest syndrome in sickle cell disease - PubMed

Obtain a chest radiograph in every patient with suspected ACS and compare it with prior imaging when available. A negative or limited initial study should not end evaluation when symptoms are evolving; repeat clinical assessment and pursue CT when additional imaging is required to clarify the pulmonary process. PubMedPubMedHigh risk and low prevalence diseases: Acute chest syndrome in sickle cell disease - PubMed

Order a type and crossmatch at admission because transfusion may be required as ACS progresses. In severe ACS, involve hematology and intensive care while transfusion planning is underway rather than reserving consultation until respiratory failure occurs. PubMedPubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Use the clinical pattern to direct immediate parallel evaluation. Fever and a new infiltrate support infectious pneumonia requiring antibacterial coverage; wheeze or a suspected reactive-airway component supports nebulized therapy; chest, back, or abdominal pain and opioid exposure increase the need for lung expansion measures and careful analgesic reassessment. ASH+2ASHHow I treat acute chest syndrome in children with sickle cell diseasecdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Provide supplemental oxygen and target an oxygen saturation of at least 95%. Failure of hypoxemia to correct with supplemental oxygen should prompt urgent escalation toward exchange transfusion and critical-care management rather than continued observation on the ward. cdn clinicaltrials+1cdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Treat pain sufficiently to permit deep breathing, coughing, mobilization, and use of incentive spirometry. Acetaminophen, NSAIDs, and opioids may be used as needed within the patient's established pain plan; in pediatric vaso-occlusive events, scheduled acetaminophen and an NSAID alongside opioids are recommended. Prevent opioid-related constipation with polyethylene glycol 3350 when opioids are used. cdn clinicaltrials+1cdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Use incentive spirometry and respiratory therapy early. One ACS protocol recommends 10 incentive-spirometry breaths every 2 hours while awake; for pain episodes involving the chest, back, or abdomen, maintain upright posture and encourage ambulation as soon as feasible to reduce pulmonary complications. cdn clinicaltrials+1cdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Use oral hydration when tolerated and limit IV fluids to maintenance-level total fluid intake rather than hyperhydration. Excess hydration has been associated with increased ACS risk, while ACS management reviews support modest hydration as part of initial treatment. ASH+1ASHHow I treat acute chest syndrome in children with sickle cell diseasePubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Start broad-spectrum antibacterial treatment for ACS and tailor subsequent therapy to the suspected community-acquired pulmonary pathogens and clinical course. Macrolide antibiotics are included in pediatric ACS management, reflecting the recognized role of atypical respiratory bacteria. ASH+2ASHHow I treat acute chest syndrome in children with sickle cell diseasecdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Crossmatch early and consider simple red cell transfusion as part of ACS management, particularly when anemia accompanies the pulmonary event. Reviews describe supplemental oxygen, modest hydration, antibiotics, and often simple transfusion as the core early regimen for pediatric ACS. ASH+1ASHHow I treat acute chest syndrome in children with sickle cell diseasePubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

Use partial or automated red cell exchange when respiratory disease is escalating despite initial measures. Specific triggers include extensive bilateral pulmonary disease, hypoxemia not corrected with supplemental oxygen, and rapid clinical deterioration. In a patient with mild anemia, defined in pediatric guidance as hemoglobin greater than 9 g/dL, but worsening respiratory status, partial exchange transfusion is preferred over relying on simple transfusion. ASH+1ASHHow I treat acute chest syndrome in children with sickle cell diseasecdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...

Automated red cell exchange rapidly removes sickled erythrocytes while replacing donor cells and can reduce hemoglobin S without the cumulative iron burden of chronic simple transfusion. Severe ACS is an established indication; coordinate the procedure with hematology, transfusion medicine, and critical care according to local apheresis capability. Wolters Kluwer+2Wolters KluwerA Study of Red Cell Exchange in Patients of Sickle Cell... : Global Journal of Transfusion Medicinecdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

If ventilatory failure develops, use lung-protective mechanical ventilation and continue urgent transfusion-based reduction of sickling burden. Do not treat mechanical ventilation as a substitute for exchange-transfusion assessment in rapidly progressive ACS. Wiley+2WileyAcute chest syndrome from sickle cell disease successfully ...cdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedHigh risk and low prevalence diseases: Acute chest syndrome in sickle cell disease - PubMed

After stabilization, review hydroxyurea use and adherence with the patient's sickle cell clinician. Hydroxyurea has demonstrated effectiveness in preventing recurrent ACS in adults and children; recurrent ACS despite disease-modifying therapy may also lead to consideration of chronic transfusion strategies or hematopoietic stem cell transplantation in appropriately selected patients. Wolters KluwerWolters KluwerStandard management of sickle cell disease complications ☆ : Hematology/Oncology and Stem Cell Therapy

Provide a discharge plan that specifies when to return for fever, cough, chest pain, dyspnea, or worsening pain, and ensure hematology follow-up. For pain management after discharge, provide appropriate prescriptions and education rather than allowing gaps in analgesic access that can promote repeated emergency presentations. PubMedPubMedAcute complications in children with sickle cell disease: Prevention and management - PMC

For patients with recurrent pulmonary events, reassess modifiable contributors identified during the admission, including inadequate pain control, lack of incentive spirometry during pain episodes, fluid excess, and untreated reactive-airway symptoms. cdn clinicaltrials+1cdn clinicaltrials[PDF] Feasibility Study of Unfractionated Heparin in Acute Chest ...PubMedAcute complications in children with sickle cell disease: Prevention and management - PMC