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MCP - Unit 3 > Sickle Cell Disease - Clinical Correlation > Flashcards

Sickle Cell Disease - Clinical Correlation Flashcards

1
Q

What is the frequency of SCD in African-American population?

A

1/400

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2
Q

What is the average lifespan for those with SCD?

A 
Females = 50
Males = 45
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3
Q

20% of SCD patients have 80% of complications. (T/F)

A

True

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4
Q

What is the most common cause of death in children with undiagnosed SCD?

A

bacterial sepsis

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5
Q

What percentage of African-Americans have the sickle cell trait (AS)?

A

8-10%

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6
Q

What is typical ratio of HbA:HbS in patients with sickle cell trait (AS)?

A

55-65% : 45-35%

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7
Q

What is the most common and most severe genetic form of SCD?

A

SS - homozygous HbS

2/3 of SCD patients

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8
Q

What percentage of African-Americans are carriers for the HbC?

A

2-3% (AC)

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9
Q

What percentage of patients with SCD are heterozygous SC?

A

25% - HbS/HbC

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10
Q

What is sickle beta thalassemia?

A

HbS trait + Beta thalassemia trait = SCD

- no/little HbA –> only HbS

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11
Q

What causes sepsis in patients with SCD?

A

vaso-occlusion of the spleen

- inhibited bacterial clearance

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12
Q

What kind of bacteria are most likely to cause sepsis in a patient with SCD?

A

encapsulated bacteria

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13
Q

2/3 of deaths are within ___time)___ of first symptoms.

A

8 hours

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14
Q

What is done to prevent sepsis in patients with SCD?

A

Prophylactic antibiotics

- Penicillin

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15
Q

What is the most common complication of SCD throughout life?

A

Painful Vaso-occlusion Episode

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16
Q

What is given to treat the pain episodes seen in patients with SCD?

A

Narcotics

  • morphine, patient controlled analgesia = best
  • respiratory depression and addiction are rare
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17
Q

What are goals of supportive care for patients with SCD?

A
  1. pain control
  2. fluids
  3. ensure oxygenation
  4. control fever

** Transfusion not usually helpful

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18
Q

What is the first diagnostic sign of SCD in an unscreened infant?

A

swollen digits

19
Q

How often does stroke develop in children with SCD?

A

10%

20
Q

How is stroke treated in patients with SCD?

A

Transfusions

  • also given as profylactic agent
  • 80% of blood replaced

Bone marrow transplant

21
Q

How often are recurrent strokes avoided in patients with SCD?

A

prophylactic transfusions about every month

22
Q

How can risk of stroke be detected in patients with SCD? What is done if risk is deemed high?

A

transcranial doppler

  • ultrasound measuring flow velocity
    • high velocity = more occluded

prophylactic blood transfusion
- prevents 80% of strokes

23
Q

What is the most common cause of death in patients over the age of 5 (including adults) who have SCD?

A

Acute Chest Syndrome

24
Q

What is given to a patient who has Acute Chest Syndrome?

A
  1. Antibiotics
    - in case it’s pneumonia
  2. Oxygenation
    - increase O2-sat.
    - – prevent the progression of sickling
  3. Incentive Spirometry
    - increase breath size
    - avoid shorter breaths due to chest pain
  4. Transfusion acutely if more severe
    - may also be given to prevent recurrence
25
Q

What is the cause of Acute Splenic Sequestration Crisis?

A

Acute vaso-occlusion of the outlet vessels from the spleen

- blood pools in spleen

26
Q

When are patients most likely to get Acute Splenic Sequestration Crisis?

A

first 5 years of life

27
Q

What is the acute treatment for Acute Splenic Sequestration Crisis?

A

Fluid resuscitation
Transfusion

*treated like shock

28
Q

What is done to prevent mortality of Acute Splenic Sequestration Crisis?

A

Teaching parents splenic palpation

  • early detection
  • listen to parents
29
Q

What studies provide pertinent genetic information to counsel parents about sickle cell disease in their future children?

A

Electrophoresis of Hb

  1. A
  2. A2
  3. F
  4. S
  5. C

Blood count

30
Q

What genotypes produce SCD?

A
  1. SS
  2. SC
  3. S+beta thalassemia
31
Q

What topics can be covered in genetic counseling about SCD?

A

Diagnosis via amniocentesis

Preimplantation genetics

Harvesting placental cord blood
- safest possible transplant to cure affected sibling

32
Q

What is included in the typical therapy for patients with SCD?

A

Mainly supportive

  1. hydration
  2. pain Rx
  3. oxygenation
  4. RBC transfusions
33
Q

What is the only effective drug that modifies SCD?

A

Hydroxurea

34
Q

What are the effects of hydroxyurea in patients with SCD?

A

Pathogenesis –> increases HbF

Symptoms

  • decreases pain episodes and acute chest syndrome by 50%
  • decreases mortality
35
Q

What is the only possibility of curing SCD?

A

Bone Marrow Transplant

- requires HLA-identical sibling

36
Q

What percent of families have an HLA identical donor for bone marrow transplant for SCD?

A

14%

37
Q

What is the mortality rate of bone marrow transplant for treatment of SCD?

A

~5%

* reduced with use of placental cord blood

38
Q

Who are the candidates for transplant in the US?

A
  1. CVA (stroke)
  2. high risk for CVA (transcranial doppler)
  3. Frequent debilitating painful episodes
  4. Recurrent and severe acute chest syndrome
39
Q

A 7 year old boy with homozygous sickle cell anemia was fine until this morning when he could not get out of the bathtub himself. His mother noticed weakness of his left arm and leg and that his face looked funny.

You should:

a. See the child in clinic the next day
b. Have the mother observe the child for 2 hours for improvement
c. Bring the child in for hydroxyurea
d. Do a RBC exchange transfusion

A

d. Do a RBC transfusion

The child is likely having a stroke. This is a medical emergency, so RBC transfusion should be done urgently to prevent further damage.

Hydroxyurea can help prevent strokes but is NOT acute management.

40
Q

The mother of a 4 year old with sickle cell disease wants information concerning possible bone arrow transplantation. The child has had no serious complications. There are 2 half siblings.

You should:

a. Do HLA testing on the half siblings and patient HLA test the siblings, parents, and patient.
b. Tell her a bone marrow transplant is impossible
c. Ask whether she is planning to become pregnant again.

A

c. Ask whether she is planning to become pregnant again.

Half siblings are very unlikely to be appropriate donors. Future newborns, if born to the same parents, have 25% chance of being best donor: an HLA identical sibling’s umbilical cord blood (assuming the sibling doe not have sickle cell disease).

41
Q

A 37 year old man with SS disease was well until this morning when he developed fever of 102 and began to have some difficulty breathing.

  • Temp = 101.8
  • RR = 65
  • PR = 150
  • O2 sat. = 84%

What do you do?

A

(Acute Chest Syndrome)

Decreasing oxygen saturation indicates patient needs supportive care:

  • supplemental oxygen
  • antibiotics
  • incentive spirometry

Also needs preventive care
- RBC transfusion to try to prevent further pulmonary damage

42
Q

A 32 year old woman with hemoglobin SC disease comes to the ER complaining of severe and diffuse pain in both lower extremities. She has a history of possibly abusing narcotics in the past. You find no abnormalities on exam except that she appears uncomfortable.

What do you do?

A

Even with the concern about narcotic abuse, you need to initially provide narcotics for severe pain. You cannot chance leaving real pain untreated. It is better to give appropriate pain medication and then observe the patient and more accurately assess how to proceed with additional pain medication.

43
Q

A 37 year old woman with sickle cell anemia presents to you in the ER with excruciating chest and abdominal pain. You give 10 mg of morphine IV and she has minimal relief after 15 minutes.

Do you:

a. Wait 15 min to reach peak morphine effect
b. Wait because of concern of decreased respiratory effort due to morphine
c. Give acetaminophen
d. Give more morphine
e. Give ibuprofen

A

d. Give more morphine

This is a difficult situation, the reality is that you do what is basic for a physician - relieve pain. If the first dose was appropriate, carefully but surely proved more while carefully assessing the patient. We more often see inadequate pain relief causing problems as opposed to morphine induced respiratory depression.

MCP - Unit 3 (2 decks)

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