Sickle cell

Question 1

How long is the lifespan of a normal RBC?

Answer 1

90-120 days

Question 2

Which statement about how sickle cell anemia is passed to offspring is CORRECT?
A. This disease is an x-linked recessive disease.
B. Sickle cell anemia is an autosomal dominant disease.
C. This condition is an autosomal recessive disease.
D. Sickle cell anemia is rarely passed to offspring and is an autosomal x-linked dominant disease.

Answer 2

C

SCA is an autosomal recessive disease in that the offspring must receive TWO hemoglobin S genes (one for each parent).

Question 3

How long is the lifespan of a sick cell RBC?

Answer 3

10-20 days

Question 4

What is the cure for sickle cell disease?

Answer 4

Bone marrow/stem cell transplant can cure sickle cell disease.

Question 5

Which type of hemoglobin is present in a patient who has sickle cell anemia?
A. Hemoglobin AA
B. Hemoglobin AS
C. Hemoglobin SS 
C. Hemoglobin AC

Answer 5

C

Question 6

A 25 year-old pregnant female and her partner both have sickle cell trait. What is the percentage that their offspring will develop sickle cell anemia?
A. 50%
B. 25% 
C. 75%
D. 100%

Answer 6

B

Question 7

You're assisting a physician with sickle cell anemia screening. As the nurse you know that which patient population listed below is at risk for sickle cell disease?
A. Native Americans
B. African-Americans 
C. Pacific Islanders
D. Latino

Answer 7

B

Question 8

A 14 year-old female has sickle cell anemia. Which factors below can increase the patient’s risk for developing sickle cell crisis? Select all that apply:
A. Shellfish
B. Infection 
C. Dehydration 
D. Hypoxia 
E. Low altitudes
F. Hemorrhage 
G. Strenuous exercise

Answer 8

The answers are B, C, D, F and G.

Question 9

During an outpatient well visit with a patient who has sickle cell anemia, you make it PRIORITY to assess the patient's?
A. hemoglobin A1C level
B. heart rate
C. reflexes
D. vaccination history

Answer 9

D
Patients will sickle cell anemia are at risk for infection because of spleen compromise. The patient should be up-to-date with the flu, pneumococcal, and meningococcal vaccines.

Question 10

An 18 year-old male is taking Hydroxyurea for treatment of sickle cell anemia. Which options below indicate this medication is working successfully? Select all that apply:
A. The patient needs fewer blood transfusions.
B. The patient experiences diuresis.
C. The patient experiences an increase in fetal hemoglobin (Hbg F).
D. The patient experiences a decrease in hemoglobin S.

Answer 10

A, C

Question 11

A mother brings in her 8 month-old child to the ER. The mother reports the baby has recently started being extremely fussy, has a fever, and swelling in the hands and feet. The child is diagnosed with sickle cell disease. As the nurse you know that the swelling in the hands and feet in the infant is termed?
A. Dactylitis 
B. Erythromelaglia
C. Dyshidrotia
D. Phalitis

Answer 11

A

Dactylitis (also called hand-foot syndrome) occurs mainly in infants who are newly diagnosed with sickle cell anemia.

Question 12

A 12-year-old girl with sickle cell disease has
pain in her right arm. An x-ray of the right upper
extremity shows bony lesions consistent with
osteomyelitis. Which of the following is the most
likely causal organism?
(A) Clostridium septicum
(B) Enterococcus faecalis
(C) Listeria monocytogenes
(D) Streptococcus pneumoniae
(E) Pseudomonas aeruginosa
(F) Haemopilus influenzae
(G) Neisseria meningitidis

Answer 12

We are worried about encapsulated organisms:

D, F, G

Question 13

When administering chronic blood transfusions to SCD patient; the goal Hgb level should be no higher than \_\_\_g/dl?
A.) 11
B.) 12
C.) 10
D.) 9

Answer 13

C

Question 14

How does hydroxyurea work?
A.) Inhibits the sickling of RBCs
B.) Inhibit HgbS replication
C.) Reduce vascular occlusion of RBCs
D.) Stimulate the production of HgbF

Answer 14

D

Question 15

What monitoring is required for hydroxyurea?
A.) Potassium 
B.) CPK levels
C.) Seizures 
D.) CBC with differential

Answer 15

D

Question 16

Which supplementation is recommended with hydroxyurea?
A.) Vitamin E
B.) Folic acid
C.) Niacin 
D.) Thamine

Answer 16

B

Question 17

Hydroxyurea has which of the following BBWs? Select all?
A.) Malignacy 
B.) Myelosupression
C.) Liver Cirrosis 
D.) Bleeding

Answer 17

A, B

Question 18

Who is hydroxyurea indicated for? Select all?

A.) Adults with >3 moderate to severe pain crisis per year
B.) Patients with severe or recurrent acute chest syndrome
C.) Chronic symptomatic anemia or disability
D.) Cholecystitis secondary to sickle cell disease

Answer 18

A, B, C

Should be considered in all children > 9 months of age regardless of disease severity

Question 19

When should hydroxyurea be held?

Answer 19

If toxicity occurs (ANC < 2000, platelets < 80) - hold until bone marrow recovers, then restart at a dose 5 mg/kg/day lower.

Question 20

What are the draw backs to deferoxamine?

Answer 20

It is not available orally, requires prolonged infusions over 8-12 hours, significant toxicities compared to oral chelating agents (Exjade, Jadenu, Ferriprox).

Question 21

Which antibiotic is used prophylactically that reduces the mortality associated with invasive pneumoncoccal infection in young children?
A.) Ceftrixone 
B.) Penicillin 
C.) Amoxicillin/Clavulanic acid
D.) Cefuroxime

Answer 21

Prophylactic penicillin (PCN)- started at age 2 months and continued minimally until age 5 (if PCN allergic you can use erythromycin 10 mg/kg po BID).

Question 22

How to manage mild to moderate pain management in SCD? Select all?
A.) Rest
B.) Fluids
C.) Application of warm compresses applied to affected areas
D.) Steroids
E.) NSAIDs/APAP
F.) Application of cold compresses applied to affected areas

Answer 22

A, B, C, E

Question 23

How to manage severe pain in SCD?

Answer 23

Opioids; many patients will need a PCA pump

Question 24

Which statement best describes the effects of sickle cell disease​ (SCD) on​ RBCs?

A.) Sickling dramatically decreases the life span of affected RBCs.
B.) The cellular membrane of a sickled RBC becomes tough and durable.
C.) Sickled RBCs may cause ischemia along with intense vascular dilation.

Answer 24

A

Question 25

The nurse is caring for a​ 58-year-old Marc​ McRyan, who is diagnosed with sickle cell disease​ (SCD). Mr.​ McRyan's healthcare provider plans to use pharmacologic therapy to stimulate his production of fetal hemoglobin. Which therapy should the nurse anticipate will be ordered by Mr.​ McRyan's healthcare​ provider?
A.) Hydroxyurea
B.) Oxygen
C.) Penicillin
D.) IV iron

Answer 25

A

Question 26

The nurse is assessing the client with sickle cell disease​ (SCD) for signs and symptoms of acute chest syndrome. Which assessment finding is most reflective of a manifestation of acute chest​ syndrome?
A.) Urine output ​> 0.5​ mL/kg/hr
B.) Absence of adventitious breath sounds
C.) Increased white blood cell count
D.) Oxygen saturation of ​>​ 90%

Answer 26

C
Manifestations of acute chest syndrome include chest​ pain, adventitious breath​ sounds, pulmonary​ infiltrates, and increased white blood cell​ (WBC) count.