2. INTRODUCTION
ā¢ Sickle Cell Syndromes are structural
haemoglobinopathies
ā¢ Mutations alter the amino acid sequence
of a globin chain (i.e. sixth amino acid
Glutamate ļ Valine)
ā¢ Cause alteration in physiological
properties of variant haemoglobin and
produce characteristic clinical
abnormalities
7. CLINICAL MANIFESTATIONS OF
SICKLE CELL ANAEMIA
Clinical
Manifestations of
Sickle Cell
Anaemia
Due to Haemolysis
Anaemia ,
Jaundice and Gall
Stones
Due to
Microvascular
Occlusion
Stroke, Pain crisis,
Acute Chest
Syndrome, Hand-
foot Syndrome
Osteonecrosis ,
Occlusion of retinal
vessels, Priapism,
Chronic leg ulcers
11. MANAGEMENT OF ACUTE PAINFUL
CRISIS
ā¢ Vigorous hydration and thorough evaluation for underlying cause (such as
infection )
ā¢ Aggressive analgesia should be given
ā¢ Morphine- for severe pain.Dose: 0.1-0.15 mg/kg every 6-8 hr
ā¢ Ketorolac-for bone pain. Dose-30-60 mg initial dose then15-30 mg every 6-8
hr
ā¢ NO- can be used to provide short term pain relief
ā¢ Blood transfusion should be reserved for extreme cases
12. MANAGEMENT OF ACUTE CHEST
SYNDROME
ā¢ Medical emergency requiring management in ICU
ā¢ Continuous monitoring of hydration is essential to avoid development of
pulmonary edema
ā¢ Vigorous oxygen therapy for protection of arterial oxygen saturation
ā¢ Blood transfusion should be given to maintain a hematocrit of >30
ā¢ Emergency exchange transfusion if arterial saturation drops to <90%
13. MANAGEMENT OF CASES
SUFFERING FROM SEVERE DISEASE
ā¢ Use of Hydroxyurea
ā¢ Blood Transfusion
ā¢ Bone marrow Transplantation
14. HYDROXYUREA
ā¢ Mainstay of therapy for patients with severe symptoms
ā¢ Mechanism of Action :
ļ¼Increases fetal hemoglobin(HbF).
ļ¼Beneficial effects on RBC hydration and vascular wall adherence.
ļ¼Suppression of the granulocytes and reticulocytes.
ā¢ Dose:10-30 mg/kg per day.
15. BLOOD TRANSFUSION
ā¢ Simple Transfusion:
ļ¼Indications of simple transfusion-
ļ¶ Splenic sequestration
ļ¶ Aplastic crisis
ļ¶ Acute chest syndrome
ā¢ Exchange Transfusion:
ļ¼Indicated in children who have suffered from cerebrovascular accident , to
reduce the risk of stroke in future
16. ADDITIONAL PROPHYLACTIC
MEASURES
ļ¼Children should be vaccinated against capsulated organisms like
pneumococcus, meningococcus , H.influenza-B, Hepatitis B and seasonal
influenza.
ļ¼Regular slit lamp examination to monitor retinopathy.
ļ¼Antibiotic prophylaxis for splenectomized patient during dental or invasive
procedures.
ļ¼Vigorous oral hydration during periods of extreme exercise,exposure to hot
and cold,emotional stress or infection.