what you need to know about acute chest syndrome by susan hernandez, rn, cnn, bsn, and g. elaine...

What You Need to Know What You Need to Know About Acute Chest About Acute Chest

SyndromeSyndromeBy Susan Hernandez, RN, CNN, BSN,By Susan Hernandez, RN, CNN, BSN,

and G. Elaine Patterson, RN-C, EdD, MA, Med, FPN-Cand G. Elaine Patterson, RN-C, EdD, MA, Med, FPN-C

Nursing2009,Nursing2009, June 2009 June 2009Online: www.nursingcenter.comOnline: www.nursingcenter.com

© 2009 by Lippincott Williams & Wilkins. All world rights reserved.© 2009 by Lippincott Williams & Wilkins. All world rights reserved.

Acute chest syndrome (ACS)Acute chest syndrome (ACS)

Potentially life-threatening Potentially life-threatening complication of sickle-cell diseasecomplication of sickle-cell disease

Can lead to respiratory failureCan lead to respiratory failure

ACS is the leading cause of death ACS is the leading cause of death among patients with sickle-cell among patients with sickle-cell diseasedisease

Acute chest syndrome (ACS)Acute chest syndrome (ACS)

Sickle-cell disease affects 80,000 Sickle-cell disease affects 80,000 AmericansAmericans

Inherited disorderInherited disorder

Seen in equatorial African descent, Seen in equatorial African descent, Mediterranean, East Indian, Middle Mediterranean, East Indian, Middle Eastern lineageEastern lineage

Background of sickle-cell Background of sickle-cell diseasedisease

Hemoglobin is oxygen-carrying protein in RBCs

Normal adult hemoglobin is designated hemoglobin A. A patient with sickle-cell disease has abnormal hemoglobin (designated hemoglobin S), alone or in combination with other abnormal hemoglobin (typically hemoglobin C)


Homozygous form of sickle-cell disease (hemoglobin SS, or sickle-cell anemia) is most severe, accounts for about 65% of cases

Other types are sickle-cell thalassemia and sickle-cell disease with hemoglobin SC


Signs and symptoms of sickle-cell disease are caused by elongated and rigid hemoglobin S

Abnormal RBCs cause vascular occlusions, creating a cycle of more deoxygenation, sickling, and sluggish blood flow. This ultimately leads to ischemia and infarction in distal organs


Abnormal hemoglobin also means RBC has shorter life (16 days vs. 120 for normal); leads to chronic intravascular and extravascular hemolysis

30 years ago, life expectancy was 14 years; now patients are living into 40s and 50s. Acute complications experienced by adult patients include vaso-occlusive crisis, ACS, renal failure

Vaso-occlusive crisisVaso-occlusive crisis

Low oxygen tension causes red blood Low oxygen tension causes red blood cells (RBCs) to lose their round shapecells (RBCs) to lose their round shape

RBCs adhere to each other and the RBCs adhere to each other and the endotheliumendothelium

Causes pain, edema, fever, tissue Causes pain, edema, fever, tissue ischemiaischemia

Vaso-occlusive crisisVaso-occlusive crisis

Can be triggered by cold, excessive Can be triggered by cold, excessive physical exertion, late pregnancy, physical exertion, late pregnancy, infection, dehydration, emotional or infection, dehydration, emotional or mental stressmental stress

Many patients hospitalized with vaso-Many patients hospitalized with vaso-occlusive crisis develop ACSocclusive crisis develop ACS

ACS definedACS defined

Acute complicationAcute complication

New pulmonary infiltrate on chest X-New pulmonary infiltrate on chest X-rayray

Accompanied by at least one other Accompanied by at least one other new sign or symptom: fever, chest new sign or symptom: fever, chest pain, coughing, wheezing, tachypneapain, coughing, wheezing, tachypnea

Possible causes of ACSPossible causes of ACS

Fat embolism - more common in Fat embolism - more common in adults, diagnosis confirmed with adults, diagnosis confirmed with bronchoscopy, can progress to ARDSbronchoscopy, can progress to ARDS

Infection - Infection - Chlamydia pneumoniaeChlamydia pneumoniae, , Mycoplasma pneumoniaeMycoplasma pneumoniae

Atelectasis - secondary to Atelectasis - secondary to hypoventilation and poor respiratory hypoventilation and poor respiratory effort with opioid useeffort with opioid use

DiagnosticsDiagnostics

Chest X-ray is cornerstone of Chest X-ray is cornerstone of diagnosisdiagnosis

Hemoglobin levelsHemoglobin levels

White blood cell countWhite blood cell count

SpOSpO22

Caring for patients with ACSCaring for patients with ACS

Improving oxygenation is first priority; Improving oxygenation is first priority; supplemental oxygen may be given supplemental oxygen may be given (incentive spirometry, nebulizer treatments)(incentive spirometry, nebulizer treatments)

Administer opioids as ordered for pain; be Administer opioids as ordered for pain; be careful of hindering respiratory effortcareful of hindering respiratory effort

Continue to assess respiratory, neurologic, Continue to assess respiratory, neurologic, and oversedation statusand oversedation status

Caring for patients with ACSCaring for patients with ACS

Administer antibiotics as orderedAdminister antibiotics as ordered

Administer I.V. fluids to reverse Administer I.V. fluids to reverse dehydration and decrease blood dehydration and decrease blood viscosityviscosity

Monitor intake and output to prevent Monitor intake and output to prevent fluid overload, which can worsen fluid overload, which can worsen pulmonary statuspulmonary status

Treatment with hydroxyureaTreatment with hydroxyurea

In patients with three or more In patients with three or more episodes of ACS or acute painful episodes of ACS or acute painful sickle cell crisis in the previous yearsickle cell crisis in the previous year

Used long-term to treat adults with Used long-term to treat adults with moderate to severe sickle cell diseasemoderate to severe sickle cell disease

Has cytotoxic effects on RBCsHas cytotoxic effects on RBCs


Reduces WBCs and platelets, which Reduces WBCs and platelets, which reduces vascular injury, incidence of ACSreduces vascular injury, incidence of ACS

Blood counts monitored every 2 weeks Blood counts monitored every 2 weeks to establish optimal dose; decreases to establish optimal dose; decreases pain, increases hemoglobin, provides pain, increases hemoglobin, provides patient well-beingpatient well-being

Monitor for bone marrow suppressionMonitor for bone marrow suppression


All patients should be on reliable All patients should be on reliable contraception during therapycontraception during therapy

If patient’s condition continues to If patient’s condition continues to deteriorate, may need mechanical deteriorate, may need mechanical ventilation and RBC exchange ventilation and RBC exchange therapy; done in ICUtherapy; done in ICU

what you need to know about acute chest syndrome by susan hernandez, rn, cnn, bsn, and g. elaine...

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