Sickle cell anaemia Flashcards
What is sickle cell anaemia?
An autosomal recessive condition where the red blood cells are shaped like sickle and are fragile and easily destroyed.
You are likely to survive malaria if you have sickle cell trait or be a carrier.
How to diagnose Sickle cell?
Pregnant woman are carriers are offered testing during pregnancy
New born babies do a heel prick test
Complication of sickle cell?
Anaemia
increase risk infection
Stroke
Avascular necrosis in large joints
Pulmonary hypertension
Painful erections
Chronic kidney disease
Sickle cell crisis
Acute chest syndrome
General management of sickle cell?
Avoid dehydration and other triggers of crisis
Ensure vaccines up to date
Antibiotics act as prophylaxis to fight infection
Hydroxycarbamide stops sickle cell RBC
Blood transfusion
Bone marrow transplant
What is sickle cell crisis?
Are reactions to sickle cell triggered by stress, dehydration + cold or significant life events.
You can manage by:
Treating infections
Keeping warm
Penile aspiration
Keeping hydrated
NSAIDS
What is Vaso-occlusive crisis (painful crisis) ?
This is where capillaries are clogged due to sickle RBC causing distal ischemia
In men it cause painful random erections which can be treated by aspiration of blood from penis
What is splenic sequestration crisis?
Where RBC block blood flow to the spleen, causing an enlarged painful spleen.
Its treated with blood transfusion + if happens a lot a splenectomy is done.
What is Aplastic crisis?
This is where there is temporary loss of creation of RBC, This is triggered by infection with parovirus B19
It leads to anaemia, however resolves itself in a week.
What is Acute chest syndrome?
Diagnosis requires:
Fever or respiratory symptoms
New infiltrates seen on chest X-Ray
Has high mortality so need good treatment and managment:
Antibiotics or antiviral for infections
Blood transfusion for anaemia
