 Sickle cell disease (SCD), an inherited disorder due to
homozygosity for the abnormal
hemoglobin, hemoglobin S (HbS).
 Hemoglobin S (HbS), results from the substitution of a
valine for glutamic acid as the sixth amino acid of the
beta globin chain, which produces a hemoglobin
tetramer (alpha2/beta S2) that is poorly soluble when
2. Characteristic Sickle Shaped RBCs
3. Major Clinical Menifestations
 Vaso-oclusive Crises
 Episodes of ischemic pain (i.e., painful crises)
 Ischemic malfunction or frank infarction in the
central nervous system,
 Chronic Anemia
 Acute severe anemia
 There are three settings in which an acute fall in
hemoglobin concentration may be superimposed upon the
1.Splenic sequestration crisis
5. Vaso-occlusive crisis & its effects
 Acute painful episodes
 Multi organ failure
 Effect on growth and
 Psychosocial effects
 Bone ischemia &
 Cardiac –MI
 Effects on pregnancy
6. Acute Chest Syndrome
The acute chest syndrome (ACS) is the most common form
of acute pulmonary disease in patients with
SCD, occurring in almost one-half of patients.
It is the most frequently reported cause of death in
adults, and is a risk factor for early mortality.
7. Definition of Acute Chest
Presence of a new pulmonary infiltrate, not due to
 Involving at least one complete lung segment
 Chest pain
 Temperature >38.5ºC
 Tachypnea, wheezing, or cough
Causes can be listed as:
1. Unknown cause
2. Pulmonary infarction
4.Chlamydia pneumoniae infection
5.Mycoplasma pneumoniae infection
9. Clinical findings
Patients with ACS present with
 chest pain
 extremity pain,
 nonproductive cough
Examination of the chest may reveal local tenderness
over the ribs or sternum; findings of pulmonary
consolidation may also be noted.
10. Common laboratory findings
Thrombocytopenia or thrombocytosis
Falling hemoglobin concentration
Elevations in lactate dehydrogenase
High Bilirubin levels
11. Diagnosis of Acute Chest
No current laboratory or radiographic finding permits the
differentiation of ACS from other acute pulmonary
manifestations of SCD, including pneumonia and
The finding of pulmonary infiltrate should be treated as
infectious pneumonia (assume both are present) until
12. Pulmonary infarction due to PE or ACS?
This differentiation remains problematic.
 Lack of evidence for DVT
 Abnormal ventilation-perfusion scans
 Inability to safely perform contrast studies in
these patients because of the possible
association with further sickling.
13. Investigations for ACS
Steady state Hb% & Hb electrophoresis
CBC, Retics & peripheral film
Urine microscopy & cultures
Blood group & antibody screening
ABGs & Pulse oxymetry
14. Acute Management
Clinical severity of Acute Chest Syndrome is broad.
Close monitoring of progressive pulmonary changes and
escalating severity because the clinical status of these
patients can quickly deteriorate if the underlying
pulmonary insult is not reversed.
15. Therapeutic Interventions
 The goals of therapy are
 To correct underlying factors that contribute
to deoxygenation of hemoglobin including
dehydration & Infections
 To control pain
 To support the patient’s respiratory &
16. Fluid Management
If dehydration is present, it should be corrected as
hypovolemia can contribute to increase sickling.
 Once it is corrected, euvolemia should be
 Overhydration or rapid hydration should be avoided
 Weights should be monitored daily along with
intake/output for assessment of the fluid status and
management of the patient
Broad spectrum empiric coverage with
 a third generation cephalosporin (eg, cefotaxime or
ceftriaxone) for common bacterial coverage and
 a macrolide (eg, azithromycin or erythromycin) for
coverage of atypical organisms (such as mycoplasma and
should be initiated immediately on admission.
19. Respiratory support
Oxygen supplementation should be provided to maintain
arterial oxygen saturation ≥ 92 percent.
Incentive spirometry, preferably supervised by a clinical
worker, should be employed at least every two hours to
prevent atelectasis from hypoventilation.
20. Patients with poor respiratory effort or rising
Use of positive pressure ventilation devices such as
nasal mask continuous positive airway pressure
(CPAP) or bilevel positive airway pressure (BiPAP)
may be useful.
Patients with respiratory failure and acute
respiratory distress syndrome
Conventional or high-frequency oscillatory mechanical
ventilation can be used.
21. Other respiratory interventions
 Inhaled nitric oxide
 Extra corporeal membrane oxygenation
 Bronchoalveolar lavage
(Bronchoscopy with bronchoalveolar lavage (BAL), usually reserved for
patients with severe or progressive infiltrates, provides both diagnostic and
therapeutic benefits. Bronchial samples can be examined for lipid content in
alveolar macrophage as evidence for pulmonary fat embolism and also sent
for culture. In intubated patients, bronchoscopy with suction and removal of
bronchial casts has been reported to improve patient ventilation)
In patients with ACS, transfusion therapy should be
considered early in the course of the disease.
 Simple Transfusion
 Exchange Transfusion
23. Simple Transfusion
The goal of simple transfusion is to increase the
hematocrit (Hct) to 30 percent or hemoglobin (Hgb) to 11
 To improve oxygenation
 For accentuated anemia
 For patients with clinical or radiological progression
of disease but not impending respiratory failure.
 For patients in whom exchange transfusion will be