Sickle Cell Disease

Question 1

Is sickle cell hereditary?

Answer 1

Yes

Question 2

How does sickle cell affect a person?

Answer 2

Sickle cell affects the haemoglobin within the red blood cells. The red blood cells form an irregular sickle shape when exposed to a variety of factors, which increases the chances of occluding the microvasculature, leading to tissue ischaemia, and red cell breakdown, causing anaemia.

Factors causing this shape inclue hypoxia, extreme temperature, dehyration, infection, pregnancy, surgery and many others.

Question 3

What is acute chest syndrome?

Answer 3

A life threatening complication of painful crises. It is the leading cause of death amongst sickle cell patients.
Often precipitated by a chest infection, with crackles at the bases of the lungs, sometimes ascending rapidly to the whole lung fields.
Symptoms include SOB, hypoxia, tachypnoea and tachycardia.
The hypoxia responds poorly to inhaled O2.

• Medical emergency to nearest receiving hospital *

Question 4

Describe the painful episodes associated with SCD.

Answer 4

The painful crises are due to vaso-occlusion in the small blood vessels, often the small capillaries in the long bones.
Vaso-occlusion can result in damage to the organs and tissues.

Patients have a history of crises, know what medications work for them, and often have a treatment plan in place.

Question 5

Suspect STROKE with:

Answer 5

Any change in consciousness, seizure, severe headache, loss of movement in a limb, loss of speech and treat accordingly as per our guidelines.