Sickle cell anemia
Sickle cell anemia is 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.
Sickle cell anemia crisis
Immediate complication
A sickle cell anemia crisis is when RBCs clump together and block small blood vessels that carry blood to organs.
It causes severe pain.
What are the interventions for sickle cell anemia?
- fluids
- oxygen
- pain meds
von Willebrand's Disease
von Willebrand's disease is a genetic bleeding disorder.
It caused by missing or defective von Willebrand factor (VWF), a clotting protein.
Hemophilia
Hemophilia is 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.
Which sex gets hemophilia much more often?
Males, since it's an X-linked genetic disorder.
What are the characteristic signs and symptoms of hemophilia or von Willebrand's disease?
- Bleeding
- nose bleeds (epistaxis)
- bruising
- excessive bleeding
- joint bleeding - will report pain, tenderness, and swelling
- excessive menstrual bleeding
Hemophilia and von Willebrand's
Replace missing clotting factors
What are the immediate interventions for a child with hemophilia or von Willebrand's Disease that has an acute injury that is causing bleeding?
- 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
Hemophilia and von Willebrand's
- avoid contact sports
- wear pads and helmet when learning to walk or doing sports such as skating or bicycling
β-Thalassemia Major
β-Thalassemia Major is a genetic disorder that causes low hemoglobin levels and anemia.
Which groups is β-Thalassemia major more common in?
β-Thalassemia major is more common in those of Mediterranean descent such as:
- Italians
- Greeks
- Syrians
β-Thalassemia Major
blood transfusions
