Unit 22

Question 1

What is s/s of sickle cell anemia?

Answer 1

Pain
CVA (stroke)
Avascular necrosis (shoulder pain)
Splenic sequestration (affects immunity)
Hematuria
Abdominal pain
Slowed sexual maturation

Question 2

What is sequestration crisis concerning sickle cell anemia?

Answer 2

Pooling of blood, hepatomegaly, splenomegaly, and circulatory collapse

Question 3

What is a vasoocclusive crisis concerning sickle cell anemia?

Answer 3

Episodes of pain
Extremities, chest, liver, kidney, abdomen, and brain can all be affected.

Question 4

What is management of sickle cell anemia?

Answer 4

Rest
O2 if needed
Correct imbalances of electrolytes
Hydration
Pain management
Blood transfusion - exchange transfusion
Antibiotics - due to spleen dysfunction
Education on physical activity (no contact sports) and monitor temperature closely

Question 5

What is s/s of hemophilia?

Answer 5

Easily bruising
Prolonged bleeding
GI bleeds
Hemarthrosis - joint bleeding (characterized by pain, stiff and warm; usually in knee, ankle, or elbow)
Hematoma

Question 6

What are management of hemophilia?

Answer 6

Replace missing clotting factors by transfusion
Desmopressin (increases clotting factor 8)
Corticosteroids (manages symptoms)
NSAID (avoid aspirin, helps with join pain, watch platelet lvls)

Question 7

What are s/s of spina bifida?

Answer 7

Paralysis
Clump of hair
Dimpling
Cyst

Question 8

What are the problems with spina bifida?

Answer 8

Hydrocephalus (fluid by brain)
Paralysis
Nervous system complications
Bladder and bowel control difficulties
Learning disabilities
Social and sexual issues
Latex allergy (kiwi, avocado, banana)

Question 9

What is the management of care for spina bifida?

Answer 9

Surgical closure within 24-72 hours after birth
Prone position
Warmer, no diaper
Sterile, moist, nonadherent dressing
Watch for infection

Question 10

How can you prevent spina bifida?

Answer 10

0.4 Folic acid by women
Avoid AEDs and trimethoprim

Question 11

What is the nursing care during a seizure?

Answer 11

Protect from injury
Airway
Suction
Side-lying
Don’t restrain
Remain with
Note onset, time, and characteristics

Question 12

What is the nursing care for after a seizure?

Answer 12

Side-lying
O2 and suctioning
Assess for injuries
Reorient them
Maintain precautions
Assessment (speech, CNS functioning, vomiting)
Remain with child
NPO until fully awake
Document
Rest