Sickle Cell Anaemia

Question 1

What is it?

Answer 1

Sickle cell anaemia is a genetic condition that causes sickle (crescent) shaped red blood cells

Autosomal recessive condition that results for synthesis of an abnormal haemoglobin chain termed HbS

Question 2

Types of crisis

Answer 2

thrombotic, ‘painful crises’
sequestration
aplastic
haemolytic

Question 3

Thrombotic Crisis

Causes

Features

Answer 3

a.k.a painful crises or vaso-occlusive crises

Precipitated by infection, dehydration and acidosis

FEATURES

• in bones (e.g. avascular necrosis of hip, hand-foot syndrome in children
• lungs: acute chest syndrome [dyspnoea, chest pain, pulmonary infiltrates, low pO2]
• brain: stroke
• penis: priapism

Question 4

Sequestration crisis features

Answer 4

sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia

Question 5

Aplastic crisis

Cause

Features

Answer 5

caused by infection with parvovirus

sudden fall in haemoglobin

Question 6

Haemolytic Crisis

What is it?

Answer 6

rare

fall in haemoglobin due an increased rate of haemolysis

Question 7

Crisis management

Answer 7

analgesia e.g. opiates

rehydrate

oxygen

consider antibiotics if evidence of infection

blood transfusion

exchange transfusion: e.g. if neurological complications

Question 8

Sickle cell anaemia management chronic

Answer 8

HYDROXYUREA: increases the HbF levels and is used in the prophylactic management of sickle cell anaemia to prevent painful episodes

NICE CKS suggest that sickle cell patients should receive the pneumococcal polysaccharide vaccine every 5 years

Question 9

Complication

Answer 9

Salmonella Osteomyeltitis