CLIPP 30

Question 1

SCD pathology

Answer 1

substitution of valine for glutamic acid at the sixth amino acid position of the hemoglobin molecule. This mutation leads to the formation of polymers of hemoglobin when the hemoglobin becomes deoxygenated.

These polymers lead to deformation of the red blood cell into the characteristic “sickle” cells. Sickle cells have increased adherence and block blood flow in the microvasculature, which leads to local tissue hypoxia, pain, and tissue damage.

The abnormal hemoglobin induces hemolysis of the red blood cells leading to chronic anemia with an elevation of the reticulocyte count.

Question 2

hgb nomenclature - for example, how do Hemoglobin FA vs FAS vs FSA differ

Answer 2

F listed first bc predominant kind at birth
FA = A is normal adult hgb
FAS = sickle trait, more hgb A than hgb S, will not have sickle sx?
FSA = sickle and beta thal w/ both sickle and beta thal but less hgb A than Sickle hgb. IS a disease.

Question 3

FSA vs FSC vs FAS

Answer 3

FAS - sickle triat, no sx?
FSA - sickle cell and beta thal, sx
FSC sickle cell and hgb c , sx

Question 4

2 common surgeries in sickle kids

Answer 4

tonsills - hypertrophy in waldeyers ring leads to OSA, snoring
cholecsytetomy - sickle cell dz cause bilirubin gallstones - typically do convesative management first then surgery if need, and give prbc before surgery to avoid sickle crisis. cholecystitis is serious to tx so tx if symptomatic from stones

Question 5

what med to give to sickle cell kids

Answer 5

penicillin - until age 5-6yo bc after this, little data to support use except in kids w documents sepsis and bacteremia or if spleen removed (but what about functional asplenia in kids above 5 yo?)i

Question 6

fever in sicklesr

Answer 6

Fever may be the only sign of sepsis in children with sickle cell disease and must be dealt with as a medical emergency, with rapid evaluation, blood cultures, and institution of broad-spectrum parenteral antibiotics while waiting for culture results to guide the therapy.
If cultures are negative and the patient is well, usually the child may be discharged in 48-72 hours. Remember that children with sickle cell disease also get “normal” colds and other viral illnesses.

Question 7

acute chest syn

Answer 7

The lungs are a site of occasional sickling problems.
This can occur in the form of pneumonia, because of the increased tendency to infections.
It may also occur as a result of vaso-occlusion in the lung parenchyma. This process, called acute chest syndrome, is a medical emergency requiring supplemental oxygen and transfusion therapy.

Question 8

two risks of chronic transfufsion

Answer 8

overload - tx w/ iron cheaters like deferasmionx or deferoxamine
-alloimmunzation, trranfusion rxn

Question 9

TCD studies are now recommended in __ of age to determine __

Answer 9

2-15 year olds
risk of stroke
tx w transfusion if at risk

Question 10

conjugation of vaccine

Answer 10

In a child under the age of 2 years, the immune system has a suboptimal response to purely polysaccharide vaccine. Conjugating the antigens to a protein allows an infant’s immune system to make antibodies.

Question 11

13 vs 23 pneumococal

Answer 11

13 is whats given in first year of life, but 23 is given to sickle kids at 2 and 5 yo(or 5-8yo)

Question 12

specifcla circmstances in which u should get meningicocal vaccine

Answer 12

kids w/ fx or anatomic asplenia should get this at 2yo and the booster 3-5 y later

Question 13

sickle cell inheritance is

Answer 13

AR

Question 14

sickle cell prenatal testing

Answer 14

One samples fetal blood obtained in utero;

Another involves extracting DNA from chorionic villi

Question 15

time course of spleen in sickle cell and what is splenic sequestration crisis?

Answer 15

Enlargement of the spleen is common during the first few years of life in children with sickle cell disease; massive enlargement or rapid change in size can indicate splenic sequestration crisis. This is often accompanied by other symptoms, including increased pallor, fatigue, and irritability.
This life-threatening complication occurs when blood pools in the spleen and leads to severe anemia and shock.
The spleen in sickle cell disease becomes progressively fibrotic; by the time the child is 4 to 6 years old, it is no longer palpable (children with hemoglobin SC or S beta thalassemia can have splenic enlargement into adolescence).

Question 16

3 key things to examine in sickle kid

Answer 16

spleen, neuro (look for stroke sx), sclera

Question 17

kids w/ scd frequently have hemoglobins between and __ for heart size

Answer 17

6-9, cardiomeg

Question 18

fever in scd

Answer 18

medical emergency - could be only or first sign of sepsis

Question 19

slurred pseech in scd

Answer 19

could be sign of stroke- transfuse to decrease amt of hgb s to prevent progression

Question 20

tacyhpnea and CP in scd

Answer 20

could mean acute chest

Question 21

pallor in scd

Answer 21

could mean aplastic crisis, splenic sequestration, incr hemolysis

Question 22

scd skin

Answer 22

baseline jaundice - but incr levels of jaundice can mean more hemolysis ad a need for transfusion, can be assoc w/ viral stuff

Question 23

pripism

Answer 23

can happen in sickling in penile arteries and can be permanent

Question 24

in scd, acute chest syn is the term for __

Answer 24

plum fat embolism, pna, or intrapulm sickling–>occlusion of plum vasculature - must assess pain, pulmo/resp complaints and chance of infection in these pt

Question 25

spleen palp 3 vs 2 cm below left costal margin

Answer 25

3 means splenomeg

Question 26

ACS sx (5)

Answer 26

Fever
Cough
Chest pain (this can lead to difficulty with expansion of the lower lung and result in atelectasis)
Shortness of breath
Decreased oxygenatio

Question 27

in scd, infiltrates can happen in…

and effusions can happen in…

Answer 27

infiltrates - acs, chf, pericarditits

effusions - acs, rib infarct, pericarditis

Question 28

wbc and plateltes in scd w/ stress

Answer 28

leukocytosis, thrombocytosi (vs will be lower in aplastic crisis)

Question 29

faces pain scale

Answer 29

typically not under 4yo but sickle kids under 4 yo may understand it, can also use parent judgmeent

Question 30

hallmark of scd

Answer 30

Painful crisis with or without fever or pulmonary event is one of the hallmarks of sickle cell disease. Intravenous fluids, NSAIDs, and intravenous narcotics (morphine or derivatives of morphine) are frequently required to control the pain. It is important to know that pain is often underdiagnosed and undertreated in the pediatric population, including patients with sickle cell disease.

Patient-controlled anesthesia (PCA) can be an excellent approach to the use of narcotics in these patients, restoring the locus of control over pain to the patient.
Patients on PCA receive a baseline continuous infusion of narcotic analgesics and can also receive small boluses on demand using a handheld button.
If narcotics are used, ensure that your patient is carefully monitored for respiratory depression.

Question 31

fluid in ACS

Answer 31

can give bolus right away but later do maintenance to make up for tachypena/insensible loss - more than maintenance can flood them (or 1.5 maint???)

Question 32

atelectasis

Answer 32

A collapsed or airless segment of lung due to obstruction with distal collapse of the alveoli.

Atelectasis is a serious risk in a child with chest pain. It can exacerbate acute chest syndrome, causing it even to become life-threatening.

Therefore, it is critical to encourage deep breathing, often through the use of assistive devices.

Question 33

incentive spirometry

Answer 33

increase insrpiation efforts to lower atelectasis

Question 34

trasnsfusion in ACS

Answer 34

An RBC transfusion is the only way to directly reduce or reverse the sickling process which is the underlying cause of the acute chest syndrome. Indications for RBC transfusion in ACS are not precise, but if a child experiences one of the following, a transfusion of packed red cells is recommended:

A fall in hemoglobin from baseline
Increasing respiratory rate
Worsening chest symptoms
Declining O2 sats
Progressive infiltrates on chest x-ray 
It should be noted that the vast majority of sickle cell patients with acute chest syndrome will have one or more of these findings.

Exchange transfusion (erythrocytapheresis) should be reserved for especially severe disease and/or hypoxemia not corrected by oxygen therapy. For more on erythrocytapheresis, see the Expert.

Question 35

erythracytapheresis

Answer 35

An automated method of doing an exchange transfusion.
The best way to rapidly lower the hemoglobin S level and rapidly raise the hemoglobin.
This method would be used with severe respiratory distress, failing pulmonary function, or acute onset of stroke.
In acute chest syndrome, increasing the hemoglobin alone is usually effective to treat this life-threatening condition.

Question 36

abx in acute chest

Answer 36

third-generation cephalosporin and a macrolide antibiotic. However, the overall rate of positive cultures was < 10%.
-hard to distinguish between cvaso occlusion or fat embolism or actual infection

Question 37

most common reasons for admission in sickle cell

Answer 37

Osteomyelitis and painful vaso-occlusive crises represent the most common reasons for admission for sickle cell disease patients. Rib infarction may lead to a picture similar to ACS as the pain can lead to hypoventilation, which may result in atelectasis and the characteristic radiographic findings of ACS. However, no radiographic findings are present in this patient. As a result, rib infarction is the most likely etiology.

Question 38

prevnar vs pneumovax

Answer 38

prevnar = what all kids get
pneumovax = the 23 valent for sc kids (pneumococcal polysach vaccine)