Sickle Cell Disease

Question 1

genetic disorder resulting from abnormal Hemoglobin chains

Answer 1

sickle cell disease

Question 2

who is SCD prevalent in

Answer 2

8% of African Americans carry the disease
<1% African Americans have it
also in Indian, Mediterranean and Middle Eastern

Question 3

what is one of the important treatments during a sickle cell disease

Answer 3

hydration

promotes hemodilution and circulation of RBC through blood vessels

Question 4

what do you give sickle cell pt to stimulate RBC synthesis

Answer 4

folic acid

Question 5

normal Hgb values

Answer 5

M: 14-18
W: 12-16

Question 6

normal RBC lifespan

Answer 6

100-120 days

Question 7

sickle cell TRAIT

Answer 7

hterozygous (HgbAS)

Question 8

sickle cell disease

Answer 8

homozygous gene (HbSS)

Question 9

disease and trait

Answer 9

abnormal Hgb (HgbS)

Question 10

describe Hemoglobin S (HgbS)

Answer 10

• replaces all or part of normal Hgb
• causes RBCs to sickle when O2 is released into tissues
• has less than normal life span

Question 11

HgbS lifespan

Answer 11

fewer than 40 days

leads to chronic anemia

Question 12

3 problems with HgbS

Answer 12

1) sickled cells cannot flow through capillary bed
2) dehydration promotes sickling
3) increased sickling episodes occur with cold (cold causes constriction of vessels)

Question 13

tissue ischemia causes…

Answer 13

widespread pathological changes:

spleen, liver, kidney, bone, CNS

Question 14

is supplemental iron given for SCD anemia?

Answer 14

NO as anemia is NOT d/t iron deficiency

Question 15

s/s of SCD

Answer 15

• frequent infections d/t nonfunctional spleen
• tiredness
• chronic hemolytic anemia
• delayed physical growth
• vasooclusive crisis
• leg ulcers
• CVA (increased risk with dehydration)

Question 16

s/s of vasooclusive crisis

Answer 16

• fever
• severe abd pain
• hand, foot, mouth (infants)
• painful, edematuous hand/feet
• arthralgia (joint pain)

Question 17

treatment of vasooclusive crisis

Answer 17

• administer IV fluids and electrolytes (increased hydration)
• monitor I&Os
• give blood
• give morphine and analgesics
• give abx to treat infection
• give WARM compress (NOT ice)

Question 18

nursing interventions

Answer 18

• teach family to prevent crisis/hypoxia
• administer pneumococal vaccine, meningococcal vaccine, and Hib vaccine
• administer Hep B vaccine as at risk d/t transfusions
• genetic counsel refereal
• support pt and family as chronic disease

Question 19

how to prevent crisis or hypoxia

Answer 19

• do not exercise strenuously
• stay away from high altitudes
• avoid infections and treat quickly
• use prophylactic penicillin
• keep hydrated
• do NOT withhold fluids at night (enuresis is common SE)