Glossary of sickle cell disease

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Hemoglobin A (HgA) is replaced by ? in sickle cell disease.
(HgS)abnormal sickled Hemoglobin
The 4 most common diseases related to sickle cell disease in the US are?
Sickle cell anemia, Sickle cell C disease, Sickle cell hemoglobin E disease, Sickle cell thalassemia disease.
This, the most common form, is said to the result of selective protection afforded trait carriers against one type of malaria.
Sickle Cell Anemia
HgS is on an autosome & is always detectable(when present) and dominant. People who have both HgA & HgS are considered?
to be carriers of the sickle cell trait
If both parents have the sickle cell trait there is a ___% chance for each preg to have SCA.
The HgS is usually not apparent until later in infancy because of ___ that is given by the mother in utero.
HbF- fetal hemoglobin & it takes the place of the HbS so there is less to cause problems.
The HbF will last about ___ and then the infant may begin to show s/s of SCA.
about a year
SCA is considered a disease of A.obstruction/destruction C.destruction/instruction
Obstruction & destruction
The sickle shaped cells get all tangled up and cause vaso-occlusion. Results are 3 major issues.
Hypoxia/Ischemia/Infarction (cell death)
The sequence of events following occlusion to organs are:123
1.Stasis with enlargement 2.Infarction w/ischemia & destruction 3.Replacement with fibrous tissue (scarring)
The most acute symptom of SCA is?
exacerbation called "crisis"
The 5 types of SCA crisis are?
Vaso-occlusive, Sequestration, Aplastic, Hyperhemolytic, Chest Syndrome (like pneumonia)
Lehann said to remember that the blood can filter on through, its just tangled up. So it's not considered a ?
Clot- it is blood & it is an occlusion but not a clot
Which is the main or #1 crisis & the more often cause of going to ER w/SCA?
The FIRST symptom in kids is usually?
Pain in the extremities
The syndrome noted for symmetrical swelling of hands/feet, painful joints, abdominal pain & you may think they have appendicitis
Hand and Foot syndrome (clinical name is dactylitis)
vaso-occlusion where? 1.causing vision chgs & stroke 2.episodes of pulmonary disease/pneumonia
3.jaundice/hepatic coma
1.Cerebrum 2.Acute chest syndrome 3.liver
VO crisis where? if: 1.hematuria 2.priapism
1.kidneys 2.genitals
Crisis of pooling of large amounts of blood in the liver & spleen, <blood volume, poss shock, poss spleenectomy
Sequestration crisis
Dont produce RBC's, severe anemia, Hgb is low
Aplastic crisis
accelerated rate of destruction of RBC's- faster than you can make them, anemia, jaundice
Hyperhemolytic crisis
Most frequent problem occuring w/SCA
vaso-occlusive pain asstd w/vaso-occlusive crisis
SCA diagnostics include
Hgb electrophoresis-newborns b/c presence of HgF;Blood smear shows sickled cells
If not diagnosed in infancy its usually dx when
young child following a URI or GI infection
Physical exam; growth chart can indicate
slow growth, puberty delayed- will eventually catch up- but hard time through middle/high school
Common findings-
tachycardia, murmurs, pallor, scleral icterus, jaundice, hepatosplenomegaly
If SCA gets hypoxia what will happen
hypoxia=metabolic acidosis=sickling=severe crisis

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