10

Question 1

Consequences of hemolysis in SCD

Answer 1

chronic anemia, jaundice, predisposition to aplastic crisis, cholelithiasis, and delayed growth and sexual maturation

Question 2

dactylitis

Answer 2

painful swelling of hands and feet

Question 3

How may a young child with SCD present?

Answer 3

painful swelling of the hands and feet (dactylitis), pneumococcal sepsis or meningitis, severe anemia and acute splenic enlargement (splenic sequestration), acute chest syndrome, pallor, jaundice, or splenomegal

Question 4

SCD presentation of older children

Answer 4

anemia, severe or recurrent musculoskeletal or abdominal pain, aplastic crisis, acute chest syndrome, splenomegaly or splenic sequestration, and cholelithiasis

Question 5

Consequences of functional asplenia in SCD

Answer 5

high risk for septicemia and meningitis with pneumococci and other encapsulated bacteria

Question 6

SCD, exacerbation of baseline anemia, parvovirus

Answer 6

aplastic crisis

Question 7

substitution of valine for glutamic acid at the sixth amino acid position of the hemoglobin molecule, formation of polymers of hemoglobin when the hemoglobin becomes deoxygenated.

Answer 7

SCD

Question 8

Hg FAS

Answer 8

baby is a carrier of one abnormal hemoglobin gene-for hemoglobin S.

This individual has benign sickle cell trait-i.e., is a carrier of hemoglobin S.

Question 9

Hg FAC

Answer 9

FAC means the baby is a carrier of one abnormal hemoglobin gene-for hemoglobin C.

This individual has benign hemoglobin C trait-i.e., is a carrier of hemoglobin C.

Question 10

Hg FS

Answer 10

both of the baby’s hemoglobin genes have mutations for hemoglobin S.

FS is the most common hemoglobin pattern causing sickle cell disease

Question 11

Hg FSA

Answer 11

FSA is sickle cell beta thalassemia, meaning one of the globin genes has a mutation for S and the other has a mutation for beta thalassemia (which produces no or little normal hemoglobin).

This pattern causes a sickling disorder, although it may behave in a milder fashion than FS (in which both genes have the sickling mutation).

Question 12

Hg FSC

Answer 12

sickle cell hemoglobin C disease: one gene has the S mutation and one gene has the mutation for hemoglobin C.

This pattern causes a sickling disorder, although it may behave in a milder fashion than FS

Question 13

Are Hg FS , FSC and FSA patients essentially managed the same way?

Answer 13

YES

Question 14

2 common procedures in pt with SCD

Answer 14

Tonsillectomy and cholecystectomy

Question 15

Why cholecystectomy in SCD?

Answer 15

Bilirubin gallstones occur frequently in all patients with hemolytic anemias—including sickle cell disease—the result of increased release of hemoglobin during breakdown of abnormal red blood cells

Question 16

What bugs are SCD patients susceptible to?

Answer 16

Encapsulated due to functional asplenia:

• Streptococcus pneumoniae
• Haemophilus influenzae type b
• Neisseria meningitidis

Question 17

Why and when ppx for pt with SCD?

Answer 17

Until 5 years old, penicillin to protect against sepsis

Question 18

Why jaundice in SCD

Answer 18

increased breakdown of the red blood cells,

Question 19

Are SCD children at risk of stroke?

Answer 19

YES; 10% by the age of 15 years

Question 20

What is acute chest syndrome and what to do?

Answer 20

Vaso-occlusion of lung parenchyma; supplemental oxygen and transfusion therapy.

Question 21

How to evaluate need for ppx transfusion to protect against stroke in child with sCD?

Answer 21

Transcranial doppler (elevated flow = transfuse)

Question 22

Why would an SCD patient be on deferasirox or deferoxamine (iron chelators)?

Answer 22

Protection against iron overload due to transfusions

Question 23

What additional vaccines for SCD child

Answer 23

PCV 23 and Meningococcal at 2 and 5 years old

Question 24

increased pallor, fatigue, and irritability in SCD

Answer 24

Splenic sequestration crisis (leaves to severe anemia and shock)

Question 25

When does spleen become small and fibrotic in SCD?

Answer 25

4-6 years old, later if sickle trait

Question 26

should you take fever in a child with SCD seriously?

Answer 26

YES - may be only sign of severe infection

Question 27

tachypnea and chest pain in SCD

Answer 27

Work up for acute chest syndrome

Question 28

What is aplastic crisis?

Answer 28

temporary inhibition of erythroid production frequently associated with viral illness, in particular Parvovirus B19. Because of the baseline rapid destruction of red blood cells and baseline lower hematocrit in patients with sickle cell disease, the brief inhibition of red blood cell production produced by the viral infection can have far greater consequences than in patients with normal red blood cell survival.

Question 29

What types of processes does acute chest syndrome describe?

Answer 29

Pneumonia
Intrapulmonary sickling
Pulmonary fat embolism

Question 30

SCD with Fever, cough, chest pain, SOB, decreased oxygenation

Answer 30

Acute chest syndrome

Question 31

Why CHF in SCD?

Answer 31

Anemia

Question 32

What may you seen on XR in ACS?

Answer 32

Multilobar infiltrates (more commonly lower and middle lobes)

Effusions

Atelectasis

Question 33

CXR with Lower lobe infiltrates and cardiomegaly

Answer 33

CHF

Question 34

Do Patients with sickle cell disease frequently demonstrate an exaggerated leukocytosis and thrombocytosis in response to stress.?

Answer 34

YES

Question 35

What to control pain in SCD?

Answer 35

Intravenous fluids, NSAIDs, and intravenous narcotics (morphine or derivatives of morphine)

Question 36

You start SCD patient on opioids for pain mgmt. What to monitor?

Answer 36

Respiratory effort

Question 37

Hydration is mainstay of SCD treatment. Should you be cautious with this approach during ACS?

Answer 37

YES, don’t want to cause pulmonary edema

Question 38

A _____ only way to directly reduce or reverse the sickling process which is the underlying cause of the acute chest syndrome

Answer 38

RBC transfusion

Question 39

What chemotherapeutic agent has been shown to decrease the severity and frequency of the sickling disorders when administered daily to patients with more severe disease.

Answer 39

Hydroxyurea