Peds-Hematological

Question 1

What complications does sickle cell disease (SCD) cause?

Answer 1

tissue ischemia, infarcts, and end organ damage?

Question 2

What organs are primarily affected by sickle cell anemia’s end organ damage?

Answer 2

Lungs, brain and spleen

Question 3

What is the life span of a RBC with SCD vs normally?

Answer 3

With SCD the RBC lasts 12 days vs normally lasting 120 days

Question 4

In an infant with SCD, when does the disease begin to show symptoms?

Answer 4

4-6 months

Question 5

What are symptoms of SCD?

Answer 5

Fatigue, Jaundice, Breathlessness, Hand Swelling, Back Pain, Joint Pain and Foot Swelling

Question 6

What lab result is indicative of SCD?

Answer 6

Elevated iron levels

Question 7

What medication will a child with SCD be on for much of their early childhood?

Answer 7

Penicillin

Question 8

What vaccine would a infant with SCD disease be given?

Answer 8

Pneumoccal polyvalent vaccine; at 2, 4, 6 and 12-15 months

Question 9

What level should HgbS be maintained at?

Answer 9

30% or lower (that is the goal)

Question 10

What are common causes of sickle cell crisis?

Answer 10

Infection, dehydration, hypoxia, trauma and stress

Question 11

What are the 3 forms of Sickle cell crisis?

Answer 11

1. Vaso-occlusive
2. Acute Sequestration
3. Aplastic

Question 12

What temperature in a SCD child requires immediate medical evaluation? Why?

Answer 12

101.3; because of the risk for infection

Question 13

What is “dactylitis”?

Answer 13

Swelling of the hands and feet

Question 14

What is a “priaprism”?

Answer 14

Persistent, painful erection

Question 15

What is chelation-therapy?

Answer 15

Administration of agents that remove heavy metals from the body

Question 16

What is Erythrocytapheresis?

Answer 16

A procedure where red blood cells are separated from whole blood. Whole blood is extracted from the patient, the red blood cells are separated, and the remaining blood is returned to circulation.