Sickle cell anaemia Flashcards
What mode of inheritance is sickle cell anaemia?
Autosomal recessive
Which chain of haemoglobin does sickle cell anaemia affect?
Beta chain
What is a vaso-occlusive crisis?
Red blood cells stick together and cause ischaemia of tissues - results in pain
What is acute chest syndrome?
A vaso-occlusive crisis resulting in infarction of the lung parenchyma
What is the management of a vaso-occlusive crisis?
Strong analgesia
Oxygen
IV fluids
Treat any suspected infections
Keep warm
What is an aplastic crisis?
Where there are life threateningly low levels of reticulocytes
Characteristed by tachypnoea and tachycardia in the absence of splenomegaly
What type of haemoglobin do patients with sickle cell have?
HbS
What are the complications of sickle cell anaemia?
Increased risk of infection
CKD
Anaemia
Sickle cell crises
Acute chest syndrome
Stroke
Avascular necrosis
Priapism
Pulmonary hypertension
What can a vaso-occlusive crisis be triggered by?
Infection
Cold weather
Dehydration
Stress
What is a splenic sequestration crisis?
Sickle cells block the bloodflow to the spleen which causes an acutely large and painful spleen
What infection can trigger an aplastic crisis?
Parvovirus B19
What is the management of an acute chest crisis?`
Analgesia
Hydration (IV fluids)
Antibiotics/antivirals to treat infection
Blood transfusion for anaemia
Incentive spirometry
Oxygen or ventilation
What is incentive spirometry?
A device which encourages effective and deep breathing
What is the general management of sickle cell anaemi?
Avoid crisis triggers
Up to date vaccinations
Antibiotic prophylaxis - phenoxymethylpenicillin
Hydroxycarbamide
Blood transfusions for anaemia
Bone marrow transplant (can be curative)
What is the action of hydroxycarbamide?
Increased fetal haemoglobin (HbF)
