Sickle Cell Disease Flashcards

1
Q

How does sickle cell disease occur?to endo

A

Change from glutamic acid to valine at one AA site

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2
Q

frequency of sickle cell disease

A

Hgb SS occurs in 0.15% of African american newborns

8-10% of african americans are carriers

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3
Q

Pathophysiology of sickle cell disease

A

deoxygenation of mutant Hb leads to
- K effluc
- incr cell density/dehydration
- incr polymerization
sickled cells adhere to endothelial cells –> vasoconstriction –> decreased blood flow
lifespan reduced to 10-20 days, high reticulocytes, and bone marrow factory produces 3-4x normal amounts RBCs

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4
Q

clinical features of sickle cell disease

A

higher Hgb - painful episodes, acute chest syndromes, osteonecrosis, proliferative
lower Hgb - stroke, priapism, leg ulcers

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5
Q

Hand foot syndrome

A

no symptoms in 1st 6 months of life due to circulating Hgb

  • painful, symmetric swelling of the hands and feet
  • ischemic necrosis of small bones
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6
Q

Splenic sequestion

A
sudden trapping of blood within spleen
occurs in infants under 2
fever, pain, resp symptoms
death can occur in less than 30min
Treatment
-IV fluids
-splenectomy
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7
Q

Bone pain

A

chronic pain from bone infarction, sickle arthritis, and aseptic necrosis of the femur
treat with NSAIDs

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8
Q

Other problems in sickle cell disease

A

strokes
kidney damage
eye problems

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9
Q

preventative measures

A
no alcohol
no smoking
exercise regularly
8 12oz glasses of water a day
reduce stress
treat infections
wear warm clothes
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10
Q

Treatment

A

Stroke: exchange transfusion
priapism: analgesia, hydration, partial exchange transfusion
Resp distress: abx coverage, O2, transfuse
splenic sequestration: transfuse
hydrate
analgesia: ibuprofen, APAP, ketorolac, opiates
vaccines
BMT

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11
Q

Endari

A

L glutamine powder: reduces oxidant damage to RBCs
reduces severe complications of sickle cell disease
AEs: constipation, N/V, HA, abd pain, cough, pain

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12
Q

hydroxyurea

A

reduces frequency of painful crises by stimulating production of fetal Hgb
long term use of this drug might cause problems later in life
AEs: fever, sore throat, cough, congestion, bruising or bleeding, hematemesis, melena

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