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Oncology > Sickle cell disease > Flashcards

Sickle cell disease Flashcards

1
Q

How is sickle cell disease inherited?

A

autosomal recessive; two abnormal Hgb genes

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2
Q

What is the mutated version of the Hgb gene?

A

Hemoglobin S

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3
Q

What is the normal version of the Hgb gene?

A

Hemoglobin A

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4
Q

What is the cause of the mutated HbS gene?

A

glutamic acid is replaced with valine

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5
Q

How do sickle cells affect blood flow?

A
  1. vaso- occlusion
  2. decreased oxygen delivery to tissues
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6
Q

What type of hemoglobin do newborns have?

A

fetal hemoglobin (HcF)

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7
Q

When do symptoms of sickle cell disease present?

A

5 months of age after HbF decreases and HbS levels increase

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8
Q

What are the symptoms of sickle cell disease?

A
  1. pain
  2. anemia
  3. growth delays
  4. stroke
  5. infections due to blocked spleen
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9
Q

What are acute complications of sickle cell disease?

A
  1. vaso- occlusive crisis
  2. fever
  3. acute chest syndrome
  4. anemia
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10
Q

What are the chronic complications of sickle cell disease?

A

infection prophylaxis
hydroxyurea

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11
Q

What is the most common complication of vaso-occlusive crisis?

A

impeded blood flow –> pain

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12
Q

What can a vaso-occlusive crisis be caused by?

A

infection
emotional stress
exposure to cold, wind, or high-altitude

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13
Q

Where and how does pain usually present?

A

lower back, chest, legs/arms ina symmetric pattern

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14
Q

What are treatments for vaso-occlusive crisis?

A
  1. IV fluids
  2. NSAIDs
  3. opioid analgesics
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15
Q

How soon should treatment begin for a vaso-occlusive crisis?

A

30 min of triage- 60 min of registration

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16
Q

What is used for mild-moderate pain?

A

anti-inflammatory meds (NSAIDs or acetaminophen)

17
Q

What is used for severe pain?

A

Oral opioid analgesics

18
Q

What routes of opioid analgesic should NOT be used?

A

IM
transdermal

19
Q

What kind of infections are patients with SCD at higher risk for?

A

encapsulated organisms caused by functional asplenia

20
Q

When is a fever treated as a medical emergency?

A

any fever (orla >/= 38.3) in a child with SCD

21
Q

What bugs need to be covered for fever in SCD?

A

s. pneumonia
GM - enteric organisms
possibly mycoplasma pneumonia

22
Q

What agents are used to treat fever is SCD?

A

3rd gen cephalosporins (ceftriaxone)
+/- macrolide (mycoplasma pneumoniae)
+/- vancomycin (acutely ill)

23
Q

What is the cause of acute chest syndrome?

A

vaso-occlusion of pulmonary vasculature

24
Q

How is acute chest syndrome diagnosed?

A

new pulmonary infiltrate on chest x ray
AND ONE OF THE FOLLOWING
Fever/ tachypnea/ chest pain/ dyspnea/ hypoxia

25
Q

What is the treatment for acute chest syndrome?

A
  1. supplemental oxygen
  2. bronchodilators
  3. empiric ABX (cephalosporin and macrolide)
26
Q

Why should opioids and IV fluids be used with caution in acute chest syndrome?

A

concern for analgesia-induced hypoventilation and pulmonary edema

27
Q

When are blood transfusions recommended?

A

life-threatening complications:
symptomatic severe acute chest syndrome
multisystem organ failure
medium or high-risk surgical procedures

28
Q

When should blood transfusions NOT be used?

A

asymptomatic anemia
uncomplicated pain crisis

29
Q

What agent is used for infection prophylaxis to decrease the incidence of pneumococcal disease?

A

Penicillin
Erythromycin (allergy)

30
Q

What vaccines should children get to prevent infection?

A
  1. PCV 13 (newborns)
  2. meningococcal conjugate vaccine (2 months and older)
  3. annual influenza vaccine
31
Q

Why is Hydroxyurea used in SCD?

A

increases FHb, reduces hospitalization end-organ damage, and stroke

32
Q

What role does Hydroxyurea have in SCD?

A

prevent pharmacologic agent for pediatric patients with recurrent vaso-occlusive episodes

33
Q

How long of a trial of the maximum tolerated dose is required before discontinuing treatment?

A

6 months

34
Q

How long can hydroxyurea be taken if they have clinical response?

A

indefinitely

35
Q

What are common adverse effects of Hydroxyurea?

A
  1. myelosuppression (mild)
  2. skin/ nail changes
  3. GI disturbances
  4. hair loss/ thinning
  5. leg ulcers
36
Q

What are the serious adverse effects of Hydroxyurea?

A
  1. growth delays
  2. infertility
  3. possible secondary leukemias or other cancers
37
Q

At what age should Hydroxyurea begin OFFERED if asymptomatic?

A

9 y/o

38
Q

What does the therapy of Hydroxyurea effectiveness depend on

A

adherence to the dosage regimen

Oncology (22 decks)

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