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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 _____

A

Asplenia

25
Q

Any fever in a child caused by SCD is treated as what?

A

Medical emergency

26
Q

What drugs are used to treat the fever caused by SCD?

A

3rd generation cephalosporins (ceftriaxone)

27
Q

If mycoplasma pneumonia is suspected, what should be added on?

A

Macrolide

28
Q

What can be considered in acutely ill children with a fever?

A

Vancomycin

29
Q

What is acute chest syndrome caused by?

A

Vaso-occlusion in the pulmonary vasculature

30
Q

How is acute chest syndrome defined?

A

Pulmonary infiltrate on chest xray
+ one or more of the following:
1. Fever
2. Tachycardia
3. Chest pain
4. Dyspnea
5. Hypoxia

31
Q

What are 3 treatments for ACS?

A
  1. Supplemental oxygen
  2. Bronchodilators
  3. Empiric antibiotics
32
Q

What antibiotics can be used for the treatment of ACS?

A
  1. Cephalosporins
  2. Macrolide
33
Q

What should you use in caution with ACS?

A

Opioid analgesics and IV fluids

34
Q

When can RBC transfusions be used in ACS?

A

Life threatening conditions (severe ACS or multi system organ failure)

35
Q

What has markedly decreased the incidence of invasive pneumococcal disease?

A

Penicillin

36
Q

If there is a penicillin allergy then what can be given?

A

Erythromycin twice daily

37
Q

All infants with SCD should receive what vaccine series?

A

Pneumococcal (PCV13)

38
Q

What type of vaccine needs to be given after age 2 years?

A

PPV23

39
Q

What vaccine should be administered to patients with SCD who are 2 months or older?

A

Meningococcal

40
Q

Children with SCD should also receive what other vaccine?

A

Annual influenza vaccine

41
Q

What is used in SCD because of its ability to increase fatal hemoglobin?

A

Hydroxyurea

42
Q

What is the only established preventive pharmacologic agent for pediatric patients with recurrent vast-occlusive episodes?

A

Hydroxyurea

43
Q

What does hydroxyurea increase?

A

HbF

44
Q

What is required before discontinuing Hydroxyurea?

A

6 month trial of the max tolerated dose

45
Q

What are 5 common ADEs caused by Hydroxyurea?

A
  1. Myelosuppression
  2. Skin and nail changes
  3. GI disturbances
  4. Hair loss/ thinning
  5. Leg ulcers
46
Q

What should patients and families that are elected to start Hydroxyurea be counseled on?

A

Adherence to the dosage regimen

Oncology Exam 3 (6 decks)

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