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Oncology Exam 3 > Sickle Cell Disease > Flashcards

Sickle Cell Disease Flashcards

1
Q

What is the most common inherited RBC disorder?

A

Sickle Cell anemia

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2
Q

What manner is SCD inherited in?

A

Autosomal recessive

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3
Q

What 2 inherited genes can cause SCD?

A

Two abnormal hemoglobin genes

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4
Q

What is the normal hemoglobin gene that is expressed in children and adults?

A

Hemoglobin A

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5
Q

What is the mutated version if hemoglobin A?

A

Hemoglobin S

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6
Q

What gets replaced that causes the altered form in SCD?

A

Glutamine to Valine

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7
Q

What 2 things do sickled blood cells cause?

A
  1. Vaso-Occlusion
  2. Decreased oxygen delivery
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8
Q

What do newborns RBCs mostly contain?

A

Fetal hemoglobin (HbF)

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9
Q

In children with SCD, the production of what type of hemoglobin begins to occur?

A

Hemoglobin S

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10
Q

What is the median life expectancy for SCD?

A

58 years

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11
Q

What patient population is SCD the most common in?

A

African Americans

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12
Q

When do the first symptoms of SCD appear?

A

Around 5 months of age

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13
Q

What are 5 symptoms that can be seen with SCD?

A
  1. Pain
  2. Anemia
  3. Growth delays
  4. Stroke
  5. Infections
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14
Q

What is the most common complication of SCD?

A

Pain

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15
Q

What 5 things can cause vaso-occlusive crisis?

A
  1. Infection
  2. Emotional stress
  3. Cold
  4. Wind
  5. High altitude
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16
Q

What are common locations of vaso-occlusive crisis?

A
  1. Lower back
  2. Chest
  3. Legs
  4. Arms
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17
Q

VOC usually appears in a ______ pattern

A

Systemic

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18
Q

What are 3 components of therapy for VOC?

A
  1. IV fluids
  2. NSAIDs
  3. Opioid analgesics
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19
Q

When should treatment begin for VOC?

A

Within 30 minutes of arrival or within 60 mins of registration

20
Q

Patients with VOC may be incorrectly identified as those with what?

A

Drug seeking behavior

21
Q

What is used for mild to moderate pain caused by VOC?

A
  1. NSAIDs
  2. Acetaminophen
22
Q

For severe pain caused by VOC, what can be used?

A

Opioid analgesics

23
Q

What routes should be avoided when giving opioid analgesics?

A

IM and transdermals

24
Q

Patients with SCD are at risk for infections with encapsulated organisms caused by _____

25
Any fever in a child caused by SCD is treated as what?
Medical emergency
26
What drugs are used to treat the fever caused by SCD?
3rd generation cephalosporins (ceftriaxone)
27
If mycoplasma pneumonia is suspected, what should be added on?
Macrolide
28
What can be considered in acutely ill children with a fever?
Vancomycin
29
What is acute chest syndrome caused by?
Vaso-occlusion in the pulmonary vasculature
30
How is acute chest syndrome defined?
Pulmonary infiltrate on chest xray + one or more of the following: 1. Fever 2. Tachycardia 3. Chest pain 4. Dyspnea 5. Hypoxia
31
What are 3 treatments for ACS?
1. Supplemental oxygen 2. Bronchodilators 3. Empiric antibiotics
32
What antibiotics can be used for the treatment of ACS?
1. Cephalosporins 2. Macrolide
33
What should you use in caution with ACS?
Opioid analgesics and IV fluids
34
When can RBC transfusions be used in ACS?
Life threatening conditions (severe ACS or multi system organ failure)
35
What has markedly decreased the incidence of invasive pneumococcal disease?
Penicillin
36
If there is a penicillin allergy then what can be given?
Erythromycin twice daily
37
All infants with SCD should receive what vaccine series?
Pneumococcal (PCV13)
38
What type of vaccine needs to be given after age 2 years?
PPV23
39
What vaccine should be administered to patients with SCD who are 2 months or older?
Meningococcal
40
Children with SCD should also receive what other vaccine?
Annual influenza vaccine
41
What is used in SCD because of its ability to increase fatal hemoglobin?
Hydroxyurea
42
What is the only established preventive pharmacologic agent for pediatric patients with recurrent vast-occlusive episodes?
Hydroxyurea
43
What does hydroxyurea increase?
HbF
44
What is required before discontinuing Hydroxyurea?
6 month trial of the max tolerated dose
45
What are 5 common ADEs caused by Hydroxyurea?
1. Myelosuppression 2. Skin and nail changes 3. GI disturbances 4. Hair loss/ thinning 5. Leg ulcers
46
What should patients and families that are elected to start Hydroxyurea be counseled on?
Adherence to the dosage regimen

Oncology Exam 3 (6 decks)

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