Sickle Cell Anaemia Flashcards
Cause of sickle cell anaemia
Autosomal recessive condition where there is an abnormal gene for beta-globin on chromosome 11
Diagnosis for sickle cell anaemia
Pregnant women at risk of being carriers are offered testing throughout pregnancy. Tested for on newborn screening heel prick test at 5 days of age
Complications of sickle cell anaemia
Anaemia, increased risk of infection, stroke, avascular necrosis, pulmonary hypertension, priapism, chronic kidney disease, sickle cell crises and acute chest syndrome
General management of sickle cell anaemia
Avoid dehydration and other triggeres of crises, ensure vaccines are up to date, antibiotic prophylaxis, hydroxycarbamide, blood transfusion for severe anaemia
What is the curative treatment
Bone marrow transplant
What does hydroxycarbamide do in sickle cell anaemia
Used to stimulate production of fetal haemoglobin which does not lead to sickling of RBCs. Protective against sickle cell crises and acute chest syndrome
What are sickle cell crises
Spectrum of acute crises related to the condition, can be mild or life threatening
What are sickle cell crises
Spectrum of acute crises related to the condition, can be mild or life threatening
Triggers for sickle cell crises
Stresses such as infection, dehydration, cold or spontaneously
Management of sickle cell crises
Supportively such as keeping warm, hydration, treating infection, analgesia (no NSAIDs), and low threshold for hospital admission
What are vaso-occlusive crises
Caused by sickle shape clogging te capillaries, causing distal ischaemia.
Associated with dehydration and raised haematocrit
Symptoms of vaso-occlusive crises
Pain, fever, priapism in men
What is splenic sequestration crisis
RBCs blocking blood flow within spleen
Consequences of splenic sequestration crisis
Acutely enlarged and painful spleen, pooling of blood into spleen causing severe anaemia and hypovolaemic shock.
What can recurrence of splenic crisis cause
Infarction and susceptibility to infections
Management of splenic crisis
Supportive with blood transfusions and fluid resus to treat anaemia and shock.
Splenectomy
What is an aplastic crisis
Situation where there is a temporary loss of creation of new blood cells - commonly triggered by parvovirus B19
What is acute chest syndrome
Fever or respiratory symptoms with new infiltrates seen on XR
Causes of acute chest syndrome
Infection such as pneumonia or bronchiolitis, or non-infective causes such as pulmonary vaso-occlusion on fat emboli
Treatment of acute chest syndrome
Abx or antivirals, blood transfusions, incentives spirometry using machine that encourages effective and deep breathing and artificial ventilation with NIV or intubation
