Sickle Cell Anemia Case OSCE

The Case Mostly will involve history taking and may ask about further management plans for a Sickle Cell patient.

Sickle Cell Anemia is an autosomal recessive disorder due to missense mutation at the 6th position of beta globin chain where Glutamate replaces valine (MCEM Part A).

Remember these patients will present in the ED with varied complaints like chest pain, bone pain, seizures, septicemia, etctetra. Hence, it becomes essential for us to know what are these emergencies possible in these patients, so that we could target our history to look for clues.

Vaso-occlusive crisis:
Brain: Seizure, Stroke (Children), Bleeds in adults, Coma
Bone: Pains in humerus, tibia, femur (Avascular Necrosis), Vertebra
Acute Chest syndrome +/- fever,
Pain abdomen: Splenic Sequestration
Priapism
Skin Ulcers

Hemotological crisis:
Especially recent viral infection (Parvovirus B19)

Infectious Crisis: Capsulated organisms like Streptococci, meningococci, Hemophilus, Klebsiella, and Salmonella Typhi murium (MCEM Part A)

(Before we move ahead, I wanted to give some pearls not directly related to this topic, but, just for our learning.

1. Stroke in Kids, think of sickle cell
2. Stroke in Young female, think of SLE Lupus anticoagulant.
3. Parvovirus B19: Remember Slapped Cheek appearance in pediatrics with erythema infectiosum
4. Osteomyelitis in Sickle Cell patients, provide cover for Salmonella)

The precipitating factors for any of the above are:
Stress: Emotional, Physical.... So think of infections, trauma, dehydration, hypoxia
Alcohol

So a Standard approach would be,

Introduction
Asking for pain and offering analgesia outright
Asking Vitals and correcting them if inappropriate (May need IV hydration)

Asking history of presenting complaints

SOCRATES Associated Symptoms
Similar event in past, and what they did (most patients will tell you what works best for them)
Histroy of Precipitant: Dehydration, infection, alcohol
Complications: Crisis
Family history
Past medical and surgical history
VACCINATIONS against Influenza and Pneumonia
Medications

When asked about management:
Investigations should include FBC, Reticulocyte Count (increased upto 12 - 15%), CXR, Septic Screen
Treatment:
IV Analgesia
IV Hydration
Hemotologist review
Admit

When do we do Blood transfusion: If Hb Less than 8, or the symptoms do not subside in spite of adequate analgesia and hydration. May consider autotransfusion)