Introduction
 Sickle cell disease (SCD), an inherited disorder due to
homozygosity for the abnormal
hemoglobin, hemoglobi...
Characteristic Sickle Shaped RBCs
Major Clinical Menifestations
 Anemia
 Vaso-oclusive Crises
 Episodes of ischemic pain (i.e., painful crises)
 Ischemi...
Anemia
 Chronic Anemia
 Acute severe anemia
 There are three settings in which an acute fall in
hemoglobin concentratio...
Vaso-occlusive crisis & its effects
 Acute painful episodes
 Multi organ failure
 Effect on growth and

development
 P...
Acute Chest Syndrome
The acute chest syndrome (ACS) is the most common form
of acute pulmonary disease in patients with
SC...
Definition of Acute Chest
Syndrome


Presence of a new pulmonary infiltrate, not due to
atelectasis,
 Involving at least...
Etiology
Causes can be listed as:

1. Unknown cause
2. Pulmonary infarction
3.Fat embolism
4.Chlamydia pneumoniae infectio...
Clinical findings
Patients with ACS present with
 fever
 chest pain
 extremity pain,
 dyspnea
 nonproductive cough

E...
Common laboratory findings








Leukocytosis
Thrombocytopenia or thrombocytosis
Falling hemoglobin concentration
...
Diagnosis of Acute Chest
Syndrome
No current laboratory or radiographic finding permits the
differentiation of ACS from ot...
Pulmonary infarction due to PE or ACS?
This differentiation remains problematic.

 Lack of evidence for DVT
 Abnormal ve...
Investigations for ACS
1.
2.
3.
4.
5.
6.
7.
8.

Steady state Hb% & Hb electrophoresis
CBC, Retics & peripheral film
Blood ...
Acute Management
Clinical severity of Acute Chest Syndrome is broad.
Close monitoring of progressive pulmonary changes and...
Therapeutic Interventions
 The goals of therapy are
 To correct underlying factors that contribute

to deoxygenation of ...
Fluid Management


If dehydration is present, it should be corrected as
hypovolemia can contribute to increase sickling.
...
Infections
Broad spectrum empiric coverage with
 a third generation cephalosporin (eg, cefotaxime or
ceftriaxone) for com...
Pain control
Opiates (Morphine, Diamorphine, Pathedine)
Ketorolac
Tramadol
Epidural Analgesia
Methylprednisolone
Poloxamer...
Respiratory support
Oxygen supplementation should be provided to maintain
arterial oxygen saturation ≥ 92 percent.
Incenti...
Patients with poor respiratory effort or rising
oxygen requirements
Use of positive pressure ventilation devices such as
n...
Other respiratory interventions
 Inhaled nitric oxide
 Extra corporeal membrane oxygenation
 Bronchoalveolar lavage
(Br...
Transfusion
In patients with ACS, transfusion therapy should be
considered early in the course of the disease.

 Simple T...
Simple Transfusion
The goal of simple transfusion is to increase the
hematocrit (Hct) to 30 percent or hemoglobin (Hgb) to...
Exchange Transfusion






Neurological involvement
Lung involvement (PaO2<9kPa with FiO2>60%)
Rapidly falling haemogl...
Complications
Neurologic events may complicate the course of
ACS, particularly when patients have severe pulmonary
disease...
Sickle Cell Disease and ICU
Sickle Cell Disease and ICU
Sickle Cell Disease and ICU
Sickle Cell Disease and ICU
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Sickle Cell Disease and ICU

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Sickle Cell Disease and ICU

  1. 1. Introduction  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 deoxygenated.
  2. 2. Characteristic Sickle Shaped RBCs
  3. 3. Major Clinical Menifestations  Anemia  Vaso-oclusive Crises  Episodes of ischemic pain (i.e., painful crises)  Ischemic malfunction or frank infarction in the spleen, central nervous system, bones, liver, kidneys, retina & lungs.
  4. 4. Anemia  Chronic Anemia  Acute severe anemia  There are three settings in which an acute fall in hemoglobin concentration may be superimposed upon the chronic anemia 1.Splenic sequestration crisis 2.Aplastic crisis 3.Hyperhemolytic crisis
  5. 5. Vaso-occlusive crisis & its effects  Acute painful episodes  Multi organ failure  Effect on growth and development  Psychosocial effects  Infection  CVA  Bone ischemia & infarction  Cardiac –MI  Dermatological---Leg ulcer  Hepatobiliary  Pulmonary  Renal  Retinopathy  Effects on pregnancy  Priaprism
  6. 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. 7. Definition of Acute Chest Syndrome  Presence of a new pulmonary infiltrate, not due to atelectasis,  Involving at least one complete lung segment  Chest pain  Temperature >38.5ºC  Tachypnea, wheezing, or cough
  8. 8. Etiology Causes can be listed as: 1. Unknown cause 2. Pulmonary infarction 3.Fat embolism 4.Chlamydia pneumoniae infection 5.Mycoplasma pneumoniae infection 6.Viral infection 7.Mixed infections 8.Other pathogens
  9. 9. Clinical findings Patients with ACS present with  fever  chest pain  extremity pain,  dyspnea  nonproductive cough Examination of the chest may reveal local tenderness over the ribs or sternum; findings of pulmonary consolidation may also be noted.
  10. 10. Common laboratory findings       Leukocytosis Thrombocytopenia or thrombocytosis Falling hemoglobin concentration Elevations in lactate dehydrogenase High Bilirubin levels Chest Radiographs
  11. 11. Diagnosis of Acute Chest Syndrome No current laboratory or radiographic finding permits the differentiation of ACS from other acute pulmonary manifestations of SCD, including pneumonia and infarction. The finding of pulmonary infiltrate should be treated as infectious pneumonia (assume both are present) until proven otherwise.
  12. 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. 13. Investigations for ACS 1. 2. 3. 4. 5. 6. 7. 8. Steady state Hb% & Hb electrophoresis CBC, Retics & peripheral film Blood Cultures Urine microscopy & cultures CXR U&E Blood group & antibody screening ABGs & Pulse oxymetry
  14. 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. 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 & haemodynamic status
  16. 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 maintained.  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
  17. 17. Infections 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 chlamydia) should be initiated immediately on admission.
  18. 18. Pain control Opiates (Morphine, Diamorphine, Pathedine) Ketorolac Tramadol Epidural Analgesia Methylprednisolone Poloxamer 188
  19. 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. 20. Patients with poor respiratory effort or rising oxygen requirements 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. 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)
  22. 22. Transfusion In patients with ACS, transfusion therapy should be considered early in the course of the disease.  Simple Transfusion  Exchange Transfusion
  23. 23. Simple Transfusion The goal of simple transfusion is to increase the hematocrit (Hct) to 30 percent or hemoglobin (Hgb) to 11 g/dL.  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 delayed.
  24. 24. Exchange Transfusion     Neurological involvement Lung involvement (PaO2<9kPa with FiO2>60%) Rapidly falling haemoglobin Priaprism
  25. 25. Complications Neurologic events may complicate the course of ACS, particularly when patients have severe pulmonary disease and/or respiratory failure, including;  Reversible posterior leukoencephalopathy syndrome  Silent cerebral infarcts  Acute necrotizing encephalitis

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