CLIPP 30: 2 year old with Sickle Cell

Question 1

What three hemoglobin patterns are associated with a sickling disease on the newborn screen?

Answer 1

FS
FSA
FSC

Question 2

Two most common surgeries in children with sickle cell disease

Answer 2

Cholecystectomy- gallstones form from hemolysis

Tonsillectomy- lymphoid tissue swells and causes swallowing difficulty

Question 3

What three infections are prophylaxed in young children with sickle cell?

Answer 3

Strep pneumo
HiB
Neisseria Meningitidis

Question 4

What is the risk of stroke in sickle cell children?

Answer 4

10% by 15 years old

Question 5

What is acute chest syndrome?

Answer 5

Vaso-occlusion of the lung parenchyma

It is a medical emergency requiring O2 support and transfusion therapy

Question 6

What two vaccines should 2 year olds with sickle cell receive?

Answer 6

23 valent pneumonia

meningitis

Question 7

Inheritance pattern of sickle cell?

Answer 7

autosomal recessive

Question 8

What causes impaired growth in kids with sickle cell?

Answer 8

Chronic anemia
Poor nutrition
Painful crises
Endocrine dysfunction
Poor pulmonary function

Question 9

What is a splenic sequestration crisis?

Answer 9

Blood pools in the spleen leading to hypotension and shock

Question 10

How should you treat a SS child with a fever?

Answer 10

Medical emergency
Children with anatomic or functional asplenia should also receive the meningococcal conjugate vaccine at
age 2 years.
Unless they have a milder genotype than Gerardo’s Hgb SS, most children with sickle cell will have
functional asplenia. They will receive a booster dose 3-5 years later.only sign of a serious infection

Question 11

Three causes of acute chest syndrome?

Answer 11

Pneumonia
Intrapulmonary sickling
Pulmonary fat embolism

Question 12

How long is penicillin continued in ss kids?

Answer 12

until aged 5-6

Question 13

How are children with sickle cell protected against pneumonia

Answer 13

Prevnar 13 within the first year of life

Booster with 23 valent at ages 2 and 5

Question 14

How are children with ss disease protected against meneingitis?

Answer 14

Children with anatomic or functional asplenia should also receive the meningococcal conjugate vaccine at
age 2 years.
Unless they have a milder genotype than Gerardo’s Hgb SS, most children with sickle cell will have
functional asplenia. They will receive a booster dose 3-5 years later.

Question 15

Two methods for prenatal testing?

Answer 15

One samples fetal blood obtained in utero

Another involves extracting DNA from chorionic villi

Question 16

Baseline hemoglobins for SS kids?

Answer 16

between 6-9

Question 17

Cxray findings for acs in ss kids?

Answer 17

Multilobar infiltrates (more commonly lower and middle lobes)
Effusions
Atelectasis
It is always difficult to distinguish whether an infiltrate in the lung of a child with sickle cell disease
represents an infectious process (pneumonia) or atelectasis from infarction or pulmonary fat
embolus

Question 18

What findings are seen on CBC in response to stress?

Answer 18

thrombocytopenia and leukocytosis

Question 19

What causes priapism in kids?

Answer 19

Sickling in the penile arteries

Question 20

When are we careful about fluid administration in kids in sickle cell crisis?

Answer 20

With ACS - need to avoid pulmonary edema