Sickle Cell Disease (1*) Flashcards
What are its complications?
• Anaemia (haemolytic) - Jaundice
• Sickle cell crises
• Acute chest syndrome
• Pulmonary HTN
• Increased risk of infection
• CKD
• Avascular necrosis
• Stroke
Sickle Cell Crises:
When can it occur?
What is the most common type of crises?
→ What occurs here?
→ What is it associated with?
→ What can it lead to?
What is Acute Chest Syndrome?
→ How is it diagnosed?
What is Splenic Sequestration Crisis?
→ What is often done in recurrent cases?
What is Aplastic Crisis?
→ What is this typically triggered by?
How are these crises managed?
➊ Spontaneously or triggered by stresses e.g. infection, dehydration, cold or significant life events
➋ Vaso-occlusive Crisis (AKA painful crisis)
→ Occlusion of vessels → Distal ischaemia, which is incredibly painful
→ Dehydration and raised haematocrit
→ Priapism
➌ Severe type of vaso-occlusive crisis – Infarction of lung parenchyma
→ Fever/respiratory symptoms + New infiltrates on CXR
➍ Blocked blood flow to spleen → Enlarged, painful spleen → Severe anaemia and Hypovolaemic shock
→ Splenectomy
➎ Temporary loss of RBC production → Anaemia
→ Parvovirus B19 (Causes slapped cheek syndrome – red rash cheeks, spotty rash on torso and limbs)
➏ Supportively as there’s no treatment – Analgesia, Abx, Warmth, Hydration
Management:
What is typically the first main thing to give these pts?
What are the other things involved in managing these pts?
Which medication can be given in special, chronic cases? How does it work?
➊ Opioid analgesia
➋ * Avoid dehydration and other triggers
* Prophylactic Abx
* Red cell transfusion
* Be up to date with vaccinations
* Bone marrow transplant
➌ Hydroxycarbamide – stimulates production of HbF
