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53 Cards in this Set
- Front
- Back
Pathophysiology of SCD? |
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features of SCD? |
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Findings of SCD? |
hemolysis of RBCs chronic anemia elevation of retic count |
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Hemoglobin F |
predominant at birth |
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Hemoglobin A |
normal adult hemoglobin |
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Hemoglobin FA does not does a sickling disorder |
:) |
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FAS |
FAS means the 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 |
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FAC |
FAC means the baby is a carrier of one abnormal hemoglobin gene-for hemoglobinC.This individual has benign hemoglobin C trait-i.e., is a carrier of hemoglobin C.
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FS |
This means that both of the baby's hemoglobin genes have mutations for hemoglobinS.FS is the most common hemoglobin pattern causing sickle cell disease.
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FSA |
FSA is sickle cell beta thalassemia, meaning one of the globin genes has a mutationfor S and the other has a mutation for beta thalassemia (which produces no or littlenormal hemoglobin).This pattern causes a sickling disorder, although it may behave in a milder fashionthan FS (in which both genes have the sickling mutation). From a clinicalmanagement standpoint, these patients are treated in the same manner.
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FSC |
FSC is sickle cell hemoglobin C disease: one gene has the S mutation and one genehas the mutation for hemoglobin C.This pattern causes a sickling disorder, although it may behave in a milder fashionthan FS (in which both genes have the sickling mutation). From a clinicalmanagement standpoint, these patients are treated in the same manner.
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Common procedures fo young individuals with SCD? |
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Why tonsillectomy? |
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Why cholecystectomy? |
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T/F: infants and young children with SCD are at increased risk of sepsis? Why/why not? |
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List the main goals for comprehensive visit for SCD? |
Important to cover 1. how the family deals with various complications that can occur 2. the frequency of painful or other vaso occlusive problems 3. How the family access health care 4. how the family is dealing with chronic illness |
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List four main expected complications of children with SCD? |
1. jaundice 2. anemia 3. stroke 4. respiratory problems |
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Why are there respiratory issues in children with SCD? |
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Immunizations for kids with SCD? |
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What is the inheritence pattern of SCD? |
Autosomal recessive
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Is there prenatal testing for SS? |
Yes |
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List some effects of chronic illness on growth and development? |
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Why can growth be affected by SCD? |
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Three important signs in patient with SCD? |
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What is the basis hemoglobin in SCD? |
60-90 difficult to withstand acute change |
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What might cough and breathing difficulty in child with sickle cell disease indicate? |
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What is acute chest syndrome? |
any of:
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What three things to address rapidly in patients with acute chest syndrome? |
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ACS findings on CXR? |
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CXR in pericarditis |
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CXR findings of CHF |
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CXR of Rib infarction |
pleural effusion may occur |
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CBC findings in a stress response? |
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What to consider when treating SCD pain? |
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What to encourage if patient has atelactasis? |
deep breathing often through using assistive devices |
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What is an incentive spirometer? |
To use this device, the individual places the end piece in his mouth and, as he inhales, theindicator rises. By increasing the inspiratory effort, the incentive spirometer helps to decreaseatelectasis. With a child, making this a game helps him to not think of this as "treatment."
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Should RBC transfusion be used in acute chest syndrome? |
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What are some educational challenges for kids with SCD? |
teach patient how to palpate spleen tongue blade can be marked with baseline splening size |
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ACS clinical features |
fever cough chest pain SOB decreased oxygenation |
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pericarditis features |
tachypnea fever uncommonly causes chest pain |
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CHF features |
tachypnea |
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Rib infarction features |
always suspect when child with sickle cell presents with chest pain tachypnea |
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Sepsis features |
no focal likely no chest pain |
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When to seek emergency care for patient with SCD? |
fever splenic enlargement chest pain rapid breathing increased pallor or jaundice priapism |
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Fever in sickle cell |
Fever in children with sickle cell disease is a medical emergency.It is sometimes the only sign of serious infection.
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Splenic enlargement in sickle cell |
Massive enlargement or rapid change in size can indicate splenicsequestration crisis.
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Slurred speech in sickle cell |
Slurred speech can indicate a stroke and should be rapidly evaluated.An exchange transfusion may be indicated to lower the hemoglobin-S levelto help prevent progression and to prevent recurrent stroke.
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Chest pain |
Tachypnea and chest pain may indicate acute chest syndrome, and eitherone warrants emergency evaluation.
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Rapid breathing |
Tachypnea and chest pain may indicate acute chest syndrome, and eitherone warrants emergency evaluation.
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Increased pallor |
Pallor is a typical finding from the anemia of SCD, and does not constitute anemergency.However, an increase in pallor can be caused by splenic sequestration,increased hemolysis (as discussed above) or a temporary inhibition oferythroid production (aplastic crisis).
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Increased jaundice |
Baseline jaundice is typical in sickle cell disease, so on its own is not anemergency.Marked increase in the level of jaundice may indicate an increase in thedegree of hemolysis and a need for transfusion (may be associated with aviral illness).
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Priapism |
Priapism from sickling in the penile arteries can cause permanent damage.
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What ABx to use in acute chest syndrome? |
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