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PNI > Sickle Cell Disease > Flashcards

Sickle Cell Disease Flashcards

1
Q

genetic disorder resulting from abnormal Hemoglobin chains

A

sickle cell disease

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2
Q

who is SCD prevalent in

A

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

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3
Q

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

A

hydration

promotes hemodilution and circulation of RBC through blood vessels

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4
Q

what do you give sickle cell pt to stimulate RBC synthesis

A

folic acid

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5
Q

normal Hgb values

A

M: 14-18
W: 12-16

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6
Q

normal RBC lifespan

A

100-120 days

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7
Q

sickle cell TRAIT

A

hterozygous (HgbAS)

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8
Q

sickle cell disease

A

homozygous gene (HbSS)

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9
Q

disease and trait

A

abnormal Hgb (HgbS)

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10
Q

describe Hemoglobin S (HgbS)

A
  • replaces all or part of normal Hgb
  • causes RBCs to sickle when O2 is released into tissues
  • has less than normal life span
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11
Q

HgbS lifespan

A

fewer than 40 days

leads to chronic anemia

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12
Q

3 problems with HgbS

A

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

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13
Q

tissue ischemia causes…

A

widespread pathological changes:

spleen, liver, kidney, bone, CNS

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14
Q

is supplemental iron given for SCD anemia?

A

NO as anemia is NOT d/t iron deficiency

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15
Q

s/s of SCD

A
  • frequent infections d/t nonfunctional spleen
  • tiredness
  • chronic hemolytic anemia
  • delayed physical growth
  • vasooclusive crisis
  • leg ulcers
  • CVA (increased risk with dehydration)
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16
Q

s/s of vasooclusive crisis

A
  • fever
  • severe abd pain
  • hand, foot, mouth (infants)
  • painful, edematuous hand/feet
  • arthralgia (joint pain)
17
Q

treatment of vasooclusive crisis

A
  • 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)
18
Q

nursing interventions

A
  • 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
19
Q

how to prevent crisis or hypoxia

A
  • 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)

PNI (11 decks)

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