Sickle Cell

Question 1

What is sickle cell disease?

Answer 1

Sickle cell mutliorgan disorder
Chronic vasular disease
Ongoing vaso-occlusion
Self perpetuating cycle of vaso-occlusion and vaso-occlusive crises (VOCs)

Question 2

How does sickle cell affect the CNS?

Answer 2

•Acute Pain is the number one cause of hospital admissions4
•Cerebrovascular accident
–Transient ischemic attack occurs in ~4%5
–Overt stroke occurs in ~16%5
–Silent cerebral infarction occurs in ~20% of children6
–Screen for vascular disease and stroke starting at age 2 years and continuing until at least age 16 years7
•Retinopathy occurs in ~23%5
•Seizures occur in ~12%5

Question 3

How does SCD affect muscoskeletal?

Answer 3

• Bone pain crisis (~30%) is the most common complication of vaso-occlusive pain, and can lead to bone or bone marrow infarction and osteonecrosis8,9
  
  • Avascular necrosis: 21%1

Question 4

How does SCD affect cardiopulmonary ?

Answer 4

• Acute chest syndrome(ACS) occurs in ~75%5
  
  • Pulmonary hypertensionoccurs in ~20%6
  • Left-sided heart diseaseoccurs in ~13%6

Question 5

How does SCD affect reticuloendothelial?

Answer 5

Splenic sequestration, which occurs in ~50% of patients who survive their first episode and can worsen anemia

Question 6

How does SCD affect Gastrointestinala and urogential?

Answer 6

Gastrointestinal
•Cholelithiasis, acute cholecystitis, biliary sludge, and acute choledocholithiasis are common7
•Gallstones occur in ~75%7
Urogenital
•Renal dysfunction begins at an early age, and ~30% experience chronic renal failure6
•Priapism occurs in ~35% of men1

Question 7

What are social impacts of SCD?

Answer 7

Can experience PTSD, axiety, depression
Missed work/school/education
Stay at home os lose social interactions, not feeling like fit in
Overt and/or silent stroke may affect cognitive function e.g. memory impairment, communication skills Hospitalisation
ER visits

Question 8

What are other impacts of SCD and how many are affected?

Answer 8

SCD (African Caribbean): sepsis, organ failure, vomit, temperature 
Diarrhoea vomiting fever 
15,000 in UK 
Pain and wait 
Staff shortages so not getting care

Question 9

What are some symptoms of SCD?

Answer 9

• Anemia
• Repeated infections
• Periodic episodes of pain
• Frequent hospitalisation

Question 10

What is the blood like in SCD?

Answer 10

Sickle shaped cells not flexible and so stick to vessel walls causing blockage and so oxygen unable to reach nearby tissues
Affects haemoglobin
Cells break down prematurely
High bp

Question 11

What are causes of SCD?

Answer 11

• Mutation in HBB gene

- beta globin mutation HbS (at least one beta globin subunits replaced with HbS for sickle cell both are replaced

Question 12

What is there bone crisis in SCD?

Answer 12

• Bone marrow ischemia or infraction of synovium
• with acute bone pair crisis, fever, leukocytosis and warmth and tenderness around affected joints
• Vaso-occlusive bone pain (when circulation of blood vessels obstructed causing iscoemic injuries

Question 13

Why is there visual loss in SCD?

Answer 13

• Blockage in small blood vessels in inner lining of retina of the eyes
• deprive eye of oxygen
• Sickle retinopathy

Question 14

Why is there a stroke in SCD?

Answer 14

• Blockage of arteries in brain - overtime damage lining and affect big ones
• Form collaterals to compensate but these are fryable, leaky susceptible to damage and not as good as the OG - they “by[pass” area of narrowing and help to restore blood flow

Question 15

What is ischaemia?

Answer 15

an inadequate blood supply to an organ or part of the body, especially the heart muscles.

Question 16

What is infraction?

Answer 16

obstruction of the blood supply to an organ or region of tissue, typically by a thrombus or embolus, causing local death of the tissue.