Sickle Cell Anaemia

Question 1

What is sickle cell anaemia, what does it predispose to, and which group of people is it most common in?

Answer 1

1. Autosomal recessive genetic disorder where red blood cells are abnormally shaped making them more easily destructible.
2. Vaso-occlusion and haemolytic anaemia
3. Sub-Saharan Africa and Afro-Caribbean

Question 2

What is this a presentation of?
Painful swollen hands and feet, symptoms of anaemia, failure to thrive, jaundice secondary to haemolysis, splenomegaly in young children, gallstones from second decade of life.

Answer 2

Sickle cell anaemia

Question 3

What are the triggers for vaso-occlusive crisis to avoid in sickle cell anaemia?

Answer 3

Cold, dehydration, infection, hypoxia, stress, alcohol.

Question 4

What is this describing?
Sickle red blood cell become stuck capillaries leading to ischemia of organs. Pain, swollen joints or dactylitis, neurological symptoms may occur.

Answer 4

Vaso-occlusive event due to sickle cell anaemia.

Question 5

What is the treatment for a vaso-occlusive event due to sickle cell anaemia?

Answer 5

Oxygen, analgesia, hydration, warmth.

Question 6

What is this describing?
Vaso-occlusion of pulmonary vessels, common cause of death in sickle cell disease, triggered by pulmonary infection, hypoxia worsens sickling. Chest pain,
tachypnoea, dyspnoea, new sputum production.

Answer 6

Acute chest syndrome due to sickle cell anaemia

Question 7

What is the treatment for acute chest syndrome due to sickle cell anaemia?

Answer 7

1. Oxygen, analgesia, hydration, warmth.

2. Antibiotics, consider exchange transfusion.

Question 8

What is this describing?

Cooling of sickle cells in spleen, sudden splenic enlargement and decrease in haemoglobin, hypovolemia.

Answer 8

Splenic sequestration due to sickle cell anaemia

Question 9

What is the treatment for splenic sequestration due to sickle cell anaemia?

Answer 9

1. Supportive

2. Blood transfusion often needed

Question 10

What is this describing?
Severe anaemia secondary to bone marrow failure due to sickle cell anaemia. Precipitated by parvovirus B19, resolves spontaneously. Features of severe anaemia.

Answer 10

Aplastic anaemia due to sickle cell anaemia

Question 11

What is the treatment for aplastic anaemia due to sickle cell anaemia?

Answer 11

Supportive, blood transfusion.

Question 12

How is sickle cell anaemia diagnosed?

Answer 12

1. Antenatal diagnosis if parents are known carriers
2. Neonatal screening programme
3. FBC - normocytic anaemia and high reticulocyte count
4. Blood film - Sickle cells
5. Haemoglobin electrophoresis confirms presence of sickle haemoglobin

Question 13

What is the management for sickle cell anaemia?

Answer 13

1. Folic acid due to high turnover of red blood cells
2. Prophylactic penicillin V
3. Full immunisations and pneumococcal vaccine
4. Hydroxyurea to increase foetal haemoglobin and reduce complications

Question 14

What are the complications of sickle cell anaemia?

Answer 14

Salmonella osteomyelitis, gallstones, priapism, stroke, renal failure, iron overload secondary to recurrent transfusions.