Hematology

Question 1

Describe:

Sickle cell anemia

Answer 1

A genetic disorder that changes the shape of the red blood cell from a circle to a crescent moon shape.

This causes decreased oxygen on the RBCs.

Question 2

Describe:

Sickle cell anemia crisis

(Immediate complication)

Answer 2

When RBCs clump together and block small blood vessels that carry blood to organs.

It causes severe pain.

Question 3

What are the interventions for sickle cell anemia?

Answer 3

HOP:

• Hydrate with fluids
• Oxygen
• Pain meds
• avoid visiting high altitude or cold places to avoid vasoconstriction

Question 4

Describe:

von Willebrand’s Disease

Answer 4

A genetic bleeding disorder.

It is caused by missing or defective von Willebrand factor (VWF), a clotting protein.

Question 5

Describe:

Hemophilia

Answer 5

An X-linked genetic disorder that is a group of bleeding disorders.

The most common is a clotting factor VIII or clotting factor IX deficiency.

Question 6

Which sex gets hemophilia much more often?

Answer 6

Males, since it’s an X-linked genetic disorder.

Question 7

What are the characteristic signs and symptoms of hemophilia or von Willebrand’s disease?

Answer 7

• Bleeding
• nose bleeds (epistaxis)
• bruising
• excessive bleeding
• joint bleeding - will report pain, tenderness, and swelling
• excessive menstrual bleeding

Question 8

Treatment:

Hemophilia and von Willebrand’s

Answer 8

Replace missing clotting factors.

Question 9

What are the immediate interventions for a child with hemophilia or von Willebrand’s Disease that has an acute injury that is causing bleeding?

Answer 9

• immobilize the joint
• apply ice to the injured joint
• apply pressure to the injured joint or area that is bleeding for at least 15 minutes

Question 10

Teaching:

Hemophilia and von Willebrand’s

Answer 10

• avoid contact sports
• wear pads and helmet when learning to walk or doing sports such as skating or bicycling