Acute Chest Syndrome

A life-threatening complication of sickle cell

disease.
The most common cause of premature death

in patients with sickle cell disease.

Definition; causes; risks

A new pulmonary infiltrate with some combination

of fever, chest pain, and signs and symptoms of

pulmonary diseases such as tachypnea, cough,
and dyspnea.
The cause of most cases of acute chest syndrome

is unknown, but infection (both bacteria and

viruses) and pulmonary infarction are believed to
play the most important roles.
Risk factors include: increasing age, asthma,

smoking, recent surgery, chronic hypoxia,

SS disease, and other genetic factors.

Signs and Symptoms

Fever, cough, chest pain, shortness of breath, chills,

wheezing, and hemoptysis.

Children are more likely to present with fever and cough.
Adults are more likely to present with chest pain,

shortness of breath, productive cough, hypoxia, and

hemoptysis.
Chest, rib, and extremity pain are more common in

adults.

Radiography
61% of cases are not suspected prior to the chest

x-ray results.
ACS and associated infiltrates may not appear until

2-3 days after initial presentation.

Acute chest syndrome frequently develops after

admission for uncomplicated vaso-occlusive crisis.

Incentive spirometry can be used to prevent acute

chest.
Chest x-ray underestimates the degree of pulmonary

involvement.

Hematologic Findings
Typically, hemoglobin drops 0.7 g/dl
Typically, white blood cell counts increases by 69%,

accompanied by increased reticulocyte count.

These changes do not distinguish this complication

from simple vaso-occlusive crisis.

70% of the patients are hypoxemic during the course

of the illness (oxygen saturation by pulse oximetry of

less than 90%), but may not be hypoxic at the time
of initial presentation.

Treatment: Acute Chest Syndrome

Monitor oxygenation with pulse oximetry;

give supplemental oxygen to maintain SpO2 95%

Pain management
Empiric antibiotic coverage
Consult hematology
Blood transfusion of exchange transfusion may be needed

in as many as of cases

Treatment: Pain and Hydration

Pain management using adequate opiate analgesia

helps to prevent hypoventilation

Intercostal nerve blocks should be considered if

chest pain is marked and sedation limits opiate use.

Overhydration or excessive opiate use may worsen

acute chest syndrome.

Patient-controlled analgesia may help to limit sedation

from opiate analgesia.

Transfusion and Antibiotics

Consult Hematology for possible transfusion
Give simple blood transfusion (10 mL/kg red blood cells) to improve oxygen

carrying capacity to people with symptomatic ACS and a hemoglobin

concentration 1 gm/dl below baseline. If baseline hemoglobin is 9 gm/dl
or higher, simple blood transfusion may not be required.
Perform urgent exchange transfusion with consultation from hematology/

critical care and/or apheresis specialist in people with ACS whose oxygen
saturation is below 90 percent despite supplemental oxygen, and/or with
increasing respiratory distress, progressive pulmonary infiltrates, decline
in hemoglobin concentration, or rapid progression of symptoms.
For antibiotic coverage, use a third or fourth generation cephalosporin

plus a macrolide IV. Alter coverage depending on local bacterial patterns,

resistance and allergy history.

Complications
Pulmonary embolism / deep vein thrombosis
Acute pulmonary hypertension
Respiratory failure
Neurologic events including stroke
Sepsis
Obstructive and restrictive pulmonary dysfunction
Acute kidney injury
Overall the death rate is 3%, but the death rate is

9% in adult patients.

Summary
ACS is life-threatening
Patients complain of chest pain and pulmonary symptoms,

but the syndrome may follow a vaso-occlusive pain crisis.

Patients are treated with oxygen, careful pain

management, careful hydration, antibiotics, and blood

transfusion as needed to reverse hypoxia.
Patients should be carefully monitored and treated for

any complications that arise.

Post Test
1. Which of the following statement concerning Acute Chest Syndrome
are false:
A. Risk factors include among other factors: increasing age, asthma,
smoking, recent surgery, chronic hypoxia, and
SS
disease.
B. Acute chest syndrome is defined as: a new pulmonary infiltrate with
some combination of fever, chest pain, hypoxia, and
signs
and symptoms of pulmonary diseases such as tachypnea, cough,
and dyspnea.
C. The cause of acute chest syndrome is usually bacterial pneumonia
and should be treated as a simple pneumonia.
D. Acute chest syndrome frequently develops after hospital admission
for uncomplicated vaso-occlusive crisis.

Post Test
2. Which of the following statements concerning Acute Chest
Syndrome is true:
A. Infiltrates on chest x-ray are always present at the time of admission
to the hospital.
B. Over hydration or excessive opiate use is rarely a problem in
this disorder.
C. Exchange transfusion is indicated if any of the following are

present: 1. saturation is below 90 percent despite supplemental oxygen,

2. increasing respiratory distress, 3. progressive pulmonary infiltrates, 4.
decline in hemoglobin concentration, or rapid progression of symptoms.
D. The death rate for this disorder is less than 1%.

Post Test Answers

1. The answer is C. The cause of acute chest syndrome is usually

bacterial pneumonia and should be treated as a simple pneumonia.

This is the only false statement.
While antibiotics are part of the treatment of acute chest syndrome,
bacterial pathogens are frequently not recovered from cultures,
and unlike the treatment for simple pneumonia, transfusion is frequently
needed and can be life saving.
2. The correct answer is C, which lists the correct indications for

exchange transfusion for the patient with acute chest syndrome.

Answer A is not correct because infiltrates may not appear until 2-3
days after initial presentation and admission to the hospital.
Answer B is not correct because over-hydration or excessive opiate
use has been shown to worsen the course of patients with acute chest
syndrome by increasing hypoxia.
Answer D is not correct because the death rate is 3% in all patients
including children, but the death rate is 9% in adult patients.