Week 9-sickle Cell

Question 1

Patho

Answer 1

Hemoglobin a replaced with hemoglobin s

If both parents have trait 1/4 chance child gets disease

Question 2

Signs and synptoms

Answer 2

Labs-S shaped hemoglobin
Swelling
Fever
Pain

Question 3

Nursing assessments

Answer 3

Pain assessment
Sickle cell crisis assessment
Cardio and neuro assessment

Question 4

This that can trigger blood loss

Answer 4

Menses, potential GI lose

Can worsen sickle cell symptoms

Question 5

Manifestations of sickle cell

Answer 5

Vassooclusive crisis
Acute chest syndrome
Sequestration crisis
Cerebrovascular accident
Emotional and behavioral problems linked to long hospitalization

Question 6

When does child start showing sickle cell symptoms

Answer 6

4-6 months

Fetal hemoglobin resists sickling and suppress symptoms

Question 7

Sickle cell crisis

Answer 7

Acute exacerbation that varies in frequency and severity

Question 8

Types of sickle cell crisis

Answer 8

Vaso-occlusive thrombotic
Splenic sequestration

Question 9

VOC

Answer 9

Most common-very painful
Stasis of blood with clumping of cells in microcirculation ->ischemia->infarction

Question 10

Signs if VOC

Answer 10

Fever
Pain
Tissue engorgement

Question 11

Splenic Sequestration

Answer 11

Life threat-death in hours
Blood pools in spleen

Question 12

Signs of splenic sequestration

Answer 12

Profound Anemia, hypovolemia, shock

Question 13

Do sickle cell patients get chronic skin ulcers

Answer 13

Yes

Question 14

Testing for sickle cell

Answer 14

Hemoglobin electrophoresis-quantifies percent of types of HGB S and HGB A

Sickledex-used with fingerstick blood gets results in 3 mins to determine presence of HGB S

Question 15

Complications of sickle cell

Answer 15

Hypoxia
Ischemia
Infection
CVA
Anemia
Renal failure
Heart failure

Question 16

Nursing interventions

Answer 16

Pain management
Fatigue management
Infection prevention
Pain meds
Hydration(Iv or oral fluids)
Antibiotics
02 if sat less than or equal to 92

Monitor child’s growth for failure to thrive
Multisystem assessment
Assess pain
Observe for inflammation or infection
Monitor for shock

Question 17

Polycythemia

Answer 17

Increased RBC volume

Secondary polyc.
-too much erythropoietin bc of lower oxygen, cyanotic heart disease, nonpatholical conditions or Neoplasms

Question 18

Medical management

Answer 18

Not needed if condition is mild
Treat underlying cause
Therapeutic phlebotomist
Prophylactic antibiotics
Monitor reticulocyte count to evaluate bone marrow function
Blood transfusion can be given early in crisis to reduce ischemia

Question 19

What does frequent transfusions cause

Answer 19

Hemosiderisis- iron in tissue
Treat with iron chelation(feroxanine) and vitamin C for iron excretion