Sickle cell crisis (vaso-occlusive)MANAGEMENT

HEMATOLOGIC AND ONCOLOGIC PRESENTATIONS · 12 SECTIONS

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550 items

☷ Legend

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Cardiovascular Presentations66▾

Respiratory Presentations47▾

Neurological Presentations77▾

Gastrointestinal Presentations63▾

Genitourinary and Reproductive Presentations44▾

Infectious Disease Presentations146▾

Trauma Presentations88▾

Toxicologic Presentations67▾

Psychiatric Presentations25▾

Musculoskeletal and Rheumatologic Presentations89▾

Dermatology Presentations21▾

Environmental and Exposure Presentations26▾

Hematologic and Oncologic Presentations30▾

Acute chest syndrome Acute Hemolytic Transfusion Reaction Aplastic Anemia (Crisis)Autoimmune Hemolytic Anemia Coagulopathy Delayed Hemolytic Transfusion Reaction Disseminated Intravascular Coagulation (DIC)Essential Thrombocythemia Febrile Non-hemolytic Transfusion Reaction Graft Versus Host Disease (Acute)Hemolytic Uremic Syndrome (HUS)Hemophilia A (Bleeding Crisis)Heparin-Induced Thrombocytopenia (HIT)Hyperviscosity syndrome Immune Thrombocytopenic Purpura (ITP)Leukemoid Reaction Myelofibrosis Paroxysmal Nocturnal Hemoglobinuria Polycythemia Vera (Crisis)Pure Red Cell Aplasia Sickle Cell (Acute Chest Syndrome)Sickle Cell (Splenic Sequestration)Sickle cell crisis (vaso-occlusive)Symptomatic anemia Thrombocytopenia (severe)Thrombotic Thrombocytopenic Purpura (TTP)Transfusion-Associated Circulatory Overload (TACO)Transfusion-Related Acute Lung Injury (TRALI)Tumor lysis syndrome Von Willebrand Disease (Bleeding)

Pediatric-Specific Presentations13▾

Endocrine and Metabolic Presentations42▾

ENT and Maxillofacial Presentations21▾

Ophthalmologic Presentations15▾

Obstetric Presentations17▾

Systemic and Miscellaneous Presentations15▾

01Approach to the Critical Patient/attcp56

Triage and immediate risks

›Time-critical priorities

›High-risk flags

›Fever 38.0 C or higher

›Hypoxia

›Chest pain

›Dyspnea

›New cough

›Altered mental status

›Focal neurologic deficit

›Severe headache

›Priapism 4 hours or longer

›New unilateral limb swelling

›Pregnancy

›Immunocompromise or asplenia

›Immediate actions

›Escalate to resuscitation bay for shock or hypoxia

›If suspected acute chest syndrome, initiate oxygen and early transfusion pathway

›If suspected stroke, activate stroke protocol and urgent exchange transfusion pathway

Monitoring and targets

›Monitoring bundle

›Vitals and reassessment cadence

›Pain reassessment every 15-30 minutes until controlled

›Continuous pulse oximetry for hypoxia or chest symptoms

›Hemodynamic and respiratory targets

›SpO2 92% or higher

›Euvolemia

Early analgesia pathway

›Analgesia first hour

›Door-to-analgesia goal

›First parenteral opioid within 30-60 minutes for severe pain

›Multimodal plan

›Opioid plus NSAID or acetaminophen when not contraindicated

Early complication screen

›Complications to rule out early

›Acute chest syndrome

›New infiltrate on CXR plus fever or respiratory symptoms

›Sepsis

›Fever plus hypotension or lactate elevation

›Stroke

›New neurologic deficit

›Splenic sequestration

›Rapid anemia plus splenomegaly and shock

›Aplastic crisis

›Severe anemia plus low reticulocyte count

›Hyperhemolysis

›Falling hemoglobin after transfusion with hemolysis markers

Consultation triggers

›Early consult criteria

›Hematology

›Transfusion decision-making

›Refractory pain requiring ICU-level analgesia

›Suspected hyperhemolysis

›ICU

›Escalating oxygen requirement

›Hemodynamic instability

›Altered mental status

›Urology

›Priapism 4 hours or longer

›Obstetrics

›Pregnancy with vaso-occlusive pain or any fetal concerns

02History/hx72

Pain episode characterization

›Episode features

›Pain pattern

›Location

›Severity and trajectory

›Typical versus atypical for patient

›Triggers

›Dehydration

›Cold exposure

›Infection symptoms

›Exertion

›Stress

›High altitude

›Home management

›Home opioid dose and timing

›NSAID or acetaminophen use

›Missed disease-modifying therapy

Complication screen

›Associated symptoms

›Respiratory

›Chest pain

›Dyspnea

›Cough

›Infectious

›Fever or chills

›Dysuria

›Sore throat

›Neurologic

›Weakness

›Speech or vision change

›Severe headache

›Hematologic

›Dark urine

›Jaundice

›Genitourinary

›Priapism

Baseline disease context

›Baseline and prior events

›Genotype

›HbSS

›HbSC

›HbS beta thalassemia

›Baseline hemoglobin

›Patient-reported baseline range

›Prior transfusion history

›Prior severe complications

›Acute chest syndrome episodes

›Stroke or TIA

›Pulmonary hypertension

›Chronic kidney disease

›Indwelling devices

›Port or central line

Medications and risks

›Medication and safety review

›Disease-modifying therapies

›Hydroxyurea adherence

›L-glutamine

›Voxelotor

›Crizanlizumab

›Anticoagulants or antiplatelets

›Bleeding risk context

›Allergies and prior opioid reactions

›Pruritus versus anaphylaxis

›Pregnancy status

›Last menstrual period

›Contraception

Social and access context

›Care plan and supports

›Existing ED pain plan

›Preferred opioid and dose

›PCA history

›Substance use risk context

›Prescription monitoring concerns

›Concurrent sedative use

›Barriers to follow-up

›Transportation

›Pharmacy access

03Physical Exam/exam40

General and vital patterns

›General assessment

›Appearance and distress

›Pain behaviors

›Toxic appearance

›Vital sign interpretation

›Fever

›Tachycardia

›Hypotension

›Tachypnea

›Hypoxia

Focused systems exam

›Cardiopulmonary

›Lung exam

›Rales or decreased breath sounds

›Work of breathing

›Cardiac exam

›New murmur

›Signs of heart failure

›Abdominal

›Splenomegaly

›Tender enlarged spleen

›Hepatomegaly and RUQ tenderness

›Hepatic sequestration concern

›Musculoskeletal and skin

›Painful areas

›Joint effusion

›Bone tenderness

›Extremity swelling

›DVT concern

›Ulcers

›Infection signs

›Neurologic

›Mental status

›New confusion

›Focal deficits

›Facial droop

›Limb weakness

PITFALLS

›Missed complications

›Normal initial CXR does not exclude evolving acute chest syndrome

›Fever may be blunted in immunocompromised or after antipyretics

›Tachycardia may reflect pain, hypovolemia, hypoxia, or sepsis

04Differential Diagnosis/ddx37

Life-threatening to exclude

›Immediate threats

›Acute chest syndrome

›ICD-10 D57.01 or D57.211 or D57.411

›Ischemic stroke or intracranial hemorrhage

›ICD-10 I63.- or I61.-

›Sepsis

›ICD-10 A41.-

›Splenic sequestration

›ICD-10 D57.02 or D57.212 or D57.412

›Pulmonary embolism

›ICD-10 I26.-

›Meningitis

›ICD-10 G00.-

Sickle-related mimics and overlaps

›Overlapping sickle complications

›Aplastic crisis

›Parvovirus B19 association

›Hyperhemolysis syndrome

›Post-transfusion hemoglobin drop

›Osteomyelitis

›Salmonella association in sickle cell

›Septic arthritis

›Single hot swollen joint

›Renal papillary necrosis

›Hematuria and flank pain

›Priapism

›Ischemic risk with prolonged duration

Non-sickle causes of pain

›Alternative pain etiologies

›Appendicitis

›ICD-10 K35.-

›Cholecystitis or biliary colic

›ICD-10 K81.- or K80.-

›Acute coronary syndrome

›ICD-10 I21.-

›Pneumonia

›ICD-10 J18.-

›Kidney stone

›ICD-10 N20.-

05Laboratory Tests/lt41

Core evaluation

›Core labs

›Complete blood count with differential

›Hemoglobin trend versus baseline

›Leukocytosis context with pain versus infection

›Reticulocyte count

›Low reticulocytes with severe anemia suggests aplastic crisis

›Basic metabolic panel

›Creatinine baseline and acute kidney injury

›Potassium abnormalities with hemolysis or renal dysfunction

›Liver panel

›Total bilirubin and direct bilirubin

›AST and ALT for hepatic injury

Hemolysis and transfusion planning

›Hemolysis and transfusion set

›LDH

›Hemolysis marker trend

›Haptoglobin

›Low values support hemolysis

›Type and screen

›Antibody history for alloimmunization risk

›Extended phenotype or genotyping availability

›Rh and Kell matching strategy when transfusing

Infection evaluation

›Infection workup when indicated

›Blood cultures

›Fever or sepsis physiology

›Urinalysis and urine culture

›Dysuria or flank pain

›Respiratory viral testing

›Cough or URI symptoms

›Lactate mmol/L

›Sepsis physiology or shock

Pregnancy and other adjuncts

›Adjunct labs

›Beta-hCG

›Pregnancy status

›Creatine kinase

›Severe myalgias or prolonged immobilization

›Troponin

›Chest pain with ACS concern

PITFALLS

›Lab interpretation traps

›Hemoglobin may be near baseline in vaso-occlusion despite severe pain

›Leukocytosis may reflect stress response rather than infection

›Reticulocyte count interpretation requires hemoglobin context

06Diagnostic Tests/dt40

Scoring Systems

›Bedside risk tools

›Pain response tracking

›Pain score trend after each analgesic step

›Functional markers

›Ambulation ability

›Oral intake ability

›Early warning scoring

›NEWS2 or local deterioration score

›Escalation thresholds per institutional protocol

MRI

›MRI indications

›Suspected stroke with nondiagnostic CT

›Diffusion-weighted imaging for early ischemia

›Suspected osteomyelitis when plain films nondiagnostic

›Marrow edema patterns

›CT use cases

›CT head

›New focal neurologic deficit

›Altered mental status without explanation

›CT pulmonary angiography

›PE concern with hypoxia or pleuritic chest pain

›CT abdomen and pelvis

›Severe abdominal pain atypical for patient

›Concern for appendicitis or other surgical abdomen

Ultrasound

›Ultrasound applications

›Lung ultrasound

›B-lines or consolidation supporting acute chest syndrome or pneumonia

›Lower extremity venous ultrasound

›Unilateral leg swelling for DVT

›RUQ ultrasound

›Gallstones or cholecystitis

›Testicular ultrasound

›Testicular torsion mimic in groin pain presentations

Plain radiography and ECG

›High-yield basic tests

›Chest X-ray

›Any chest symptoms

›Fever without source

›Baseline for evolving acute chest syndrome

›ECG

›Chest pain

›Significant tachycardia

07Disposition/dispo37

Admission and higher level of care

›Admission criteria

›Uncontrolled pain

›Persistent severe pain after ED parenteral opioid escalation

›Need for PCA or continuous infusion strategy

›Complication concern

›Hypoxia or new oxygen requirement

›New infiltrate on CXR

›Fever with uncertain source

›Hemodynamic instability

›New neurologic symptoms

›Acute kidney injury

›High-risk social context

›Inability to obtain medications

›No safe follow-up

›ICU criteria

›Escalating respiratory support

›High-flow oxygen requirement

›Rising work of breathing

›Exchange transfusion pathway activation

›Suspected stroke

›Severe acute chest syndrome

Discharge and observation

›Discharge criteria

›Pain control

›Controlled on oral regimen

›Ambulating or baseline mobility

›Physiologic stability

›SpO2 92% or higher on room air

›No fever or clear outpatient plan for fever source

›Complication exclusion

›No chest symptoms

›No neurologic symptoms

›Follow-up plan

›Hematology or primary care follow-up within 24-72 hours when feasible

›Observation unit criteria

›Intermediate pathway

›Needs serial analgesia reassessment

›Mild dehydration requiring monitored fluids

08Treatment/t128

Analgesia strategy

›Multimodal analgesia

›Opioid pathway for severe pain

›Morphine IV 0.1 mg/kg

›Repeat 0.05-0.1 mg/kg every 15-30 minutes to effect

›Typical adult bolus 4-8 mg IV

›Hydromorphone IV 0.015 mg/kg

›Repeat 0.0075-0.015 mg/kg every 15-30 minutes to effect

›Typical adult bolus 0.5-1 mg IV

›Fentanyl IV 0.5-1 mcg/kg

›Repeat every 5-10 minutes for rapid titration

›Useful option with renal impairment

›PCA option for admitted patients

›Morphine PCA example

›Demand dose 1 mg

›Lockout 6-10 minutes

›Basal infusion per protocol for opioid-tolerant patients

›Hydromorphone PCA example

›Demand dose 0.2 mg

›Lockout 6-10 minutes

›Basal infusion per protocol for opioid-tolerant patients

›Non-opioid adjuncts

›Ketorolac IV 15 mg

›Every 6 hours as needed

›Maximum duration 5 days

›Ibuprofen PO 400-600 mg

›Every 6-8 hours as needed

›Avoid in acute kidney injury

›Acetaminophen PO or IV 650-1000 mg

›Every 6 hours as needed

›Maximum 3000 mg per day for many adults

›Refractory pain adjuncts

›Ketamine low-dose infusion

›Initiate 0.1-0.3 mg/kg/hour

›Titrate by 0.05-0.1 mg/kg/hour every 30-60 minutes

›Stop for emergence reaction or intolerable dizziness

›Lidocaine infusion

›Consider per institutional protocol

›Contraindications with severe heart block or seizure risk

›Pruritus and nausea management

›Ondansetron PO or IV 4-8 mg

›Every 8 hours as needed

›Diphenhydramine PO or IV 25-50 mg

›Prefer non-sedating strategies when possible

Fluids and oxygen

›Supportive care

›Fluids

›Oral hydration preferred when tolerating PO

›IV isotonic crystalloid for dehydration

›250-500 mL boluses with reassessment

›Avoid routine aggressive overhydration

›Oxygen

›Supplemental oxygen for hypoxia

›Target SpO2 92% or higher

›No routine oxygen for normal saturation

›Temperature and comfort

›Warm environment

›Avoid cold exposure triggers

Acute chest syndrome pathway

›Acute chest syndrome management

›Respiratory support

›Oxygen escalation to maintain SpO2 92% or higher

›Incentive spirometry

›10 breaths every 2 hours while awake

›Antibiotics when suspected

›Ceftriaxone IV 2 g daily

›Plus azithromycin IV or PO 500 mg daily

›Alternative for beta-lactam allergy

›Levofloxacin IV or PO 750 mg daily

›Bronchodilator trial

›Wheeze or known asthma

›Transfusion strategy

›Simple transfusion

›Symptomatic anemia or hemoglobin significantly below baseline

›Exchange transfusion

›Severe hypoxia

›Rapid progression

›Multilobar infiltrates

Stroke and neurologic emergency pathway

›Stroke management

›Neuroimaging

›Immediate CT head

›MRI if available and nondiagnostic CT with persistent concern

›Transfusion

›Exchange transfusion for suspected ischemic stroke

›Target HbS fraction per hematology protocol

›Antithrombotics

›Per stroke team after hemorrhage exclusion

Transfusion principles

›RBC transfusion decision-making

›General targets

›Avoid post-transfusion hemoglobin above 100 g/L in HbSS to reduce hyperviscosity risk

›Indications for simple transfusion

›Symptomatic anemia

›Perioperative optimization

›Mild to moderate acute chest syndrome

›Indications for exchange transfusion

›Stroke or TIA concern

›Severe acute chest syndrome

›Multiorgan failure syndrome

›Alloimmunization mitigation

›Extended phenotype-matched units when feasible

›Early hematology involvement with complex antibody history

Infection and fever

›Fever management

›Sepsis evaluation and antibiotics when indicated

›Early broad-spectrum antibiotics for sepsis physiology

›Source-directed antibiotics for suspected pneumonia or UTI

›Asplenia risk

›Lower threshold for bacteremia evaluation

VTE prophylaxis and mobility

›Thrombosis prevention

›Pharmacologic prophylaxis for admitted adults unless contraindicated

›Enoxaparin SC 40 mg daily

›Renal adjustment per protocol

›Early mobilization

›As tolerated when pain controlled

Priapism

›Priapism pathway

›Initial measures

›Analgesia

›Hydration

›Oxygen for hypoxia

›Urology escalation

›Ischemic priapism 4 hours or longer

›Aspiration and intracavernosal phenylephrine per urology protocol

Avoidance and safety

›High-risk therapies to avoid

›Meperidine

›Neurotoxic metabolite and seizure risk

›Routine systemic corticosteroids for uncomplicated pain crisis

›Rebound pain and readmission risk

›Opioid safety

›Sedation monitoring

›Hold escalation for respiratory depression

›Naloxone availability

›IV 0.04 mg titrated for opioid-induced respiratory depression

09Special Populations/sp25

Pregnancy

›Pregnancy considerations

›Maternal-fetal risks

›Higher acute chest syndrome risk

›Higher VTE risk

›Medication considerations

›NSAID avoidance in later pregnancy per obstetric guidance

›Opioid use with monitoring for oversedation

›Disposition bias toward admission

›Lower threshold with fever, hypoxia, or reduced fetal movement

Geriatric

›Older adult considerations

›Comorbidity burden

›Chronic kidney disease and NSAID risk

›Heart failure and fluid sensitivity

›Medication sensitivity

›Lower opioid starting doses and slower titration

›Delirium risk with sedatives and antihistamines

Pediatrics

›Pediatric considerations

›Weight-based dosing

›Morphine IV 0.05-0.1 mg/kg

›Hydromorphone IV 0.01-0.015 mg/kg

›Splenic sequestration vigilance

›Rapid pallor and lethargy

›Enlarging spleen on exam

›Acute chest syndrome monitoring

›Lower threshold for admission with fever or cough

10Background/b30

Epidemiology

›Epidemiology overview

›Disease burden

›High ED utilization and recurrent admissions in severe genotypes

›Vaso-occlusive pain as most common acute presentation

›Risk patterns

›Increased crisis frequency with dehydration and infection

›Higher complication risk in HbSS and HbS beta zero thalassemia

Pathophysiology

›Pathophysiology essentials

›Vaso-occlusion mechanism

›Sickling under deoxygenation and acidosis

›Endothelial activation and adhesion

›Microvascular obstruction leading to ischemic pain

›Hemolysis consequences

›Nitric oxide scavenging and vasoconstriction

›Pulmonary hypertension risk

›Acute chest syndrome mechanism

›Infection trigger

›Fat embolism trigger

›Hypoventilation from pain and atelectasis

Therapeutic Considerations

›Treatment rationale

›Early opioid analgesia

›Prevents pain amplification and facilitates ventilation

›Multimodal analgesia

›Opioid-sparing and improved functional outcomes

›Judicious fluids

›Dehydration correction without pulmonary edema risk

›Incentive spirometry

›Atelectasis prevention and acute chest syndrome risk reduction

›Transfusion strategy

›Oxygen delivery improvement and HbS reduction when severe complications occur

11Patient Discharge Instructions/pdi27

copy discharge instructions

›Home plan

›Pain control plan

›Take prescribed pain medicines exactly as directed

›Use scheduled acetaminophen if recommended

›Use NSAID only if approved for you

›Hydration and rest

›Drink fluids regularly

›Avoid cold exposure

›Breathing exercises

›Incentive spirometer if provided

›Deep breathing and gentle walking as tolerated

›Follow-up

›Contact hematology or primary care within 1-3 days

›Refill planning to avoid running out of pain medicine

›Return to ED now for red flags

›Fever 38.0 C or higher

›Chest pain

›Trouble breathing

›Oxygen level low if you monitor it

›New cough

›Severe headache

›Weakness, numbness, speech trouble, or vision change

›Fainting or severe dizziness

›Uncontrolled pain despite home plan

›Priapism 4 hours or longer

›New one-sided leg swelling

›Pregnancy with decreased fetal movement or vaginal bleeding

12References/r17

Guidelines and core sources

›Guideline set

›NHLBI Evidence-Based Management of Sickle Cell Disease

›Acute pain episode management framework

›Transfusion indications for acute complications

›American Society of Hematology sickle cell guidelines

›Acute pain management recommendations

›Transfusion support recommendations

›British Society for Haematology sickle cell guidance

›Acute chest syndrome management

›Transfusion and exchange transfusion pathways

Evidence grading conventions

›Evidence labeling used in this document

›Class I recommendation

›Strong benefit and general agreement

›Class IIa recommendation

›Moderate benefit and supportive evidence

›Class IIb recommendation

›Possible benefit with limited evidence

Learn More: Sickle cell crisis (vaso-occlusive)→

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Reviewed by SymptomDx Medical Team · Last reviewed June 16, 2026

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