Sickle Cell

Question 1

Inheritance pattern of Sickle Cell Disease

Answer 1

Autosomal Recessive

Question 2

What is the most common ethnic origin of Sickle Cell Disease

Answer 2

African origin

Question 3

Pathology of Sickle Cell Anaemia

Answer 3

HbS polymerises when deoxygenates causing RBC to deform
Producing sickle cells which cannot carry O2, are fragile and haemolyse quickly
Also blocking small vessels (vaso-occlusion)

Question 4

Once a RBC is sickle is this reversible

Answer 4

No

Question 5

What is the commonest presentation of sickle cell anaemia

Answer 5

Pain

Question 6

Ix for Sickle Cell anaemia

Answer 6

Screen antenatally
Hb electrophoresis
Blood film

Question 7

Blood film appearance of Sickle Cell disease

Answer 7

Abnormal Sickle shaped cells

Question 8

Painful crisis management in Sickle Cell Diease

Answer 8

Opiates

<30 mins presentation

Question 9

Chest crisis management in Sickle Cell Disease

Answer 9

Resp. support 
Abx. 
IV fluids
Analgesia 
Transfusion

Question 10

Describe sequestration crisis

Answer 10

Spleen (infarction, atrophy, hyposplenic, immunocompromised)

Causes severe anemia dn shock

Question 11

Rx for sequestration crisis

Answer 11

Urgent transfusion required

Question 12

Which virus is associated with aplastic crisis

Answer 12

Parovirus B19 (86%) cases

Question 13

What is aplastic crisis

Answer 13

Sudden shut down of RBC production

Question 14

Rx for aplastic crisis

Answer 14

Usually self limiting
<2 weeks
Transfusion may be needed

Question 15

Which chains are affected in Sickle Cell anaemia

Answer 15

Beta chains

Resulting in 2 beta (sickle) chains

Question 16

What causes painful vaso-occlusive crises

Answer 16

The abnormal blood cells blocking/occluding small vessels

Question 17

Clinical features of chest crisis

Answer 17

Chest pain
Fever
Worsening hypoxia
Infiltrates on CXR

Question 18

Clinical features of Sickle Cell Anaemia

Answer 18

Fever
Pneumonia like syndrome 
Bone pain 
Visual floaters
Tachypnoea 
Failure to thrive
Pallor 
Jaundice
Tachycardia
Lethargy

Question 19

Chronic management of Sickle Cell Disease

Answer 19

Keep up to date with immunisations 
Antibiotics 
Blood transfusion
DMARD 
Bone marrow Tx

Question 20

Why are sickle cell patients more prone to infection

Answer 20

Due to hyposplenism

Question 21

What types of crisis can occur in Sickle Cell Disease

Answer 21

Vaso-occlusive
Aplastic
Sequestration
Haemolytic

Question 22

What is clinical sign of sickle cell anaemia seen in the hands

Answer 22

Dactylitis

Question 23

Complications of Sickle Cell Disease

Answer 23

Stroke
Increased risk infection 
Cholelithiasis 
Avascular necrosis 
Ophthalmology problems

Question 24

What does Sickle Cell trait confer protection against

Answer 24

Malaria