Sickle Cell Disorders Flashcards

1
Q

___ is an oxygen-carrying molecule. A normal RBC is ___% Hb by weight.

A

Hemoglobin; 33

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2
Q

___ is a reduction in the oxygen-carrying capacity of the blood. It is considered a ___ ___ not a disease.

A

Anemia; symptom complex

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3
Q

What are some causes of anemia?

A
  1. decreased RBC production
  2. blood loss
  3. destruction of circulating RBCs
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4
Q

In patients with the sickle cell trait, how many genes for HbS do they carry? Sickle cell disease?

A

trait: 1 (heterozygous)
disease: 1 gene for each parent for HbS (homozygous)

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5
Q

__-___% of African Americans carry the sickle cell trait and ___ African Americans have sickle cell disease

A

8-10%; 1/600

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6
Q

Define sickle cell disease

A

Cellular rigidity and membrane damage cause RBCs to become irreversibly sickle-shaped due to crystallization of HbS

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7
Q

What are three sickling stimuli?

A
  1. Deoxygenated blood
  2. Decreased blood pH
  3. Dehydration
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8
Q

T/F. Patients with >80% of their Hb being HbS are very sensitive to sickling stimuli.

A

True.

Patients with 20-45% Hb as HbS are less sensitive to sickling stimuli (usually sickle cell trait patients)

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9
Q

What are the net effects of RBC sickling?

A
  1. erythrostasis
  2. increased blood viscosity
  3. reduced blood flow
  4. hypoxia
  5. RBC adhesion
  6. vascular occlusion
  7. further sickling
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10
Q

What type of patients are typically asymptomatic, less sensitive to sickling stimuli, and not at risk for adverse events during dental treatment?

A

sickle cell trait

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11
Q

Routine dental care should be given during non-___ periods.

A

crisis

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12
Q

Sickle cell patients should be given a ___-___ protocol, ___ local anesthesia, nitrous oxide sedation with at least ___% oxygen concentration, ___ +/- ___ for pain, and physician consultation prior to any surgical procedures.

A

stress-reduction; profound; 50; Tylenol +/- codeine

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13
Q

___ can trigger a crisis. How is this handled?

A

infection

remove offending sourse, I&D, systemic antibiotics
If cellulitis, consider inpatient management
adequate fluid intake to avoid dehydration

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