Sickle Cell

Question 1

What is the pathophysiology of SCD?

Answer 1

There is a substitution for the amino acid VALINE for GLUTAMIC acid
When RBCs are deoxygenated, it crystallizes and changes the shape to a crescent

Question 2

Pooling of blood in liver or spleen causing hep or splenomegaly, decrease in hub and hgt, and can lead to shock because of the decrease in circulating blood volume

Answer 2

Sequestration (spleen or liver)

Question 3

Decrease RBC production causing profound anemia, can be caused by parvovirus

Answer 3

Aplastic

Question 4

Increased rate of RBC destruction causing anemia, jaundice, and an increase in reticulocytes

Answer 4

Hyperhemolytic

Question 5

Chest pain, fever, pneumonia like cough, anemia

Answer 5

Acute Chest Syndrome

Question 6

Excessive need for folic acid and vitamin B12

Answer 6

Megaloblastic

Question 7

Stroke

Answer 7

CVA

Question 8

How is pain managed?

Answer 8

PCA using either Morphine or Dilaudid as well as IV Toreador (NSAID). Transitioned to methadone PO. Warm soaks are also helpful

Question 9

What are the side effects of the medications?

Answer 9

Constipation, itching, nausea, sleepiness, and decreased urination

Question 10

Why Penicillin?

Answer 10

Penicillin because of functional aspleenia. Infection is the leading cause of death in children under 5, with SCD

Question 11

What does Hydroxyurea do?

Answer 11

Reactivates fetal hub increasing oxygenation of RBC

Question 12

Immunizations

Answer 12

No immunizations are contraindicated, but may wait till after a crisis. Additional needed: (1) Hib-MenCY
(2) pneumococcal vaccine

Question 13

5th Disease / Blood Transfusion

Answer 13

Connection between them. Parvovirus can cause aplastic crisis (decrease RBC production causing profound anemia)

Question 14

Why do you administer DESFERAL while receiving a blood transfusion?

Answer 14

It is a chelation agent that will bind with excess iron. Patient should be notified that urine will turn orange and can cause hearing loss

Question 15

Orange urine / Possible hearing loss

Answer 15

Desferal (given while receiving a blood transfusion)

Question 16

Testing

Answer 16

Can test baby in utero with amniocentesis and chorionic villi

Question 17

Chances of having SCD

Answer 17

Autosomal recessive - 25% chance each pregnancy

Question 18

Long term complications from SCD

Answer 18

Renal, retinopathy, CHF, liver failure, functional asplenia, avascular necrosis of hips and shoulders, chronic hemolytic anemia, poor wound healing, and CVA

Question 19

Is there a cure?

Answer 19

Yes. Stem cell transplant

Question 20

SCD - discharge education

Answer 20

Importance of hydration
Notify care provider if fever above 101.5
Avoid extreme cold
Pain management
Signs of splenic sequestration crisis and respiratory disease, importance of continuing meds and immunizations