Sickle Cell Disease

Front 1

Hemoglobin A (HgA) is replaced by ? in sickle cell disease.

Back 1

(HgS)abnormal sickled Hemoglobin

Front 2

The 4 most common diseases related to sickle cell disease in the US are?

Back 2

Sickle cell anemia, Sickle cell C disease, Sickle cell hemoglobin E disease, Sickle cell thalassemia disease.

Front 3

This, the most common form, is said to the result of selective protection afforded trait carriers against one type of malaria.

Back 3

Sickle Cell Anemia

Front 4

HgS is on an autosome & is always detectable(when present) and dominant. People who have both HgA & HgS are considered?

Back 4

to be carriers of the sickle cell trait

Front 5

If both parents have the sickle cell trait there is a ___% chance for each preg to have SCA.

Back 5

25%

Front 6

The HgS is usually not apparent until later in infancy because of ___ that is given by the mother in utero.

Back 6

HbF- fetal hemoglobin & it takes the place of the HbS so there is less to cause problems.

Front 7

The HbF will last about ___ and then the infant may begin to show s/s of SCA.

Back 7

about a year

Front 8

SCA is considered a disease of A.obstruction/destruction B.construction/destruction C.destruction/instruction

Back 8

Obstruction & destruction

Front 9

The sickle shaped cells get all tangled up and cause vaso-occlusion. Results are 3 major issues.

Back 9

Hypoxia/Ischemia/Infarction (cell death)

Front 10

The sequence of events following occlusion to organs are:123

Back 10

1.Stasis with enlargement 2.Infarction w/ischemia & destruction 3.Replacement with fibrous tissue (scarring)

Front 11

The most acute symptom of SCA is?

Back 11

exacerbation called "crisis"

Front 12

The 5 types of SCA crisis are?

Back 12

Vaso-occlusive, Sequestration, Aplastic, Hyperhemolytic, Chest Syndrome (like pneumonia)

Front 13

Lehann said to remember that the blood can filter on through, its just tangled up. So it's not considered a ?

Back 13

Clot- it is blood & it is an occlusion but not a clot

Front 14

Which is the main or #1 crisis & the more often cause of going to ER w/SCA?

Back 14

Vaso-Occlusive

Front 15

The FIRST symptom in kids is usually?

Back 15

Pain in the extremities

Front 16

The syndrome noted for symmetrical swelling of hands/feet, painful joints, abdominal pain & you may think they have appendicitis

Back 16

Hand and Foot syndrome (clinical name is dactylitis)

Front 17

vaso-occlusion where? 1.causing vision chgs & stroke 2.episodes of pulmonary disease/pneumonia
3.jaundice/hepatic coma

Back 17

1.Cerebrum 2.Acute chest syndrome 3.liver

Front 18

VO crisis where? if: 1.hematuria 2.priapism

Back 18

1.kidneys 2.genitals

Front 19

Crisis of pooling of large amounts of blood in the liver & spleen, <blood volume, poss shock, poss spleenectomy

Back 19

Sequestration crisis

Front 20

Dont produce RBC's, severe anemia, Hgb is low

Back 20

Aplastic crisis

Front 21

accelerated rate of destruction of RBC's- faster than you can make them, anemia, jaundice

Back 21

Hyperhemolytic crisis

Front 22

Most frequent problem occuring w/SCA

Back 22

vaso-occlusive pain asstd w/vaso-occlusive crisis

Front 23

Lehann said to remember that the blood can filter on through, its just tangled up. So it's not considered a ?

Back 23

Clot- it is blood & it is an occlusion but not a clot

Front 24

Which is the main or #1 crisis & the more often cause of going to ER w/SCA?

Back 24

Vaso-Occlusive

Front 25

The FIRST symptom in kids is usually?

Back 25

Pain in the extremities

Front 26

The syndrome noted for symmetrical swelling of hands/feet, painful joints, abdominal pain & you may think they have appendicitis

Back 26

Hand and Foot syndrome (clinical name is dactylitis)

Front 27

vaso-occlusion where? 1.causing vision chgs & stroke 2.episodes of pulmonary disease/pneumonia
3.jaundice/hepatic coma

Back 27

1.Cerebrum 2.Acute chest syndrome 3.liver

Front 28

SCA diagnostics include

Back 28

Hgb electrophoresis-newborns b/c presence of HgF;Blood smear shows sickled cells

Front 29

If not diagnosed in infancy its usually dx when

Back 29

young child following a URI or GI infection

Front 30

Physical exam; growth chart can indicate

Back 30

slow growth, puberty delayed- will eventually catch up- but hard time through middle/high school

Front 31

Common findings-

Back 31

tachycardia, murmurs, pallor, scleral icterus, jaundice, hepatosplenomegaly

Front 32

If SCA gets hypoxia what will happen

Back 32

hypoxia=metabolic acidosis=sickling=severe crisis