10 Flashcards
Consequences of hemolysis in SCD
chronic anemia, jaundice, predisposition to aplastic crisis, cholelithiasis, and delayed growth and sexual maturation
dactylitis
painful swelling of hands and feet
How may a young child with SCD present?
painful swelling of the hands and feet (dactylitis), pneumococcal sepsis or meningitis, severe anemia and acute splenic enlargement (splenic sequestration), acute chest syndrome, pallor, jaundice, or splenomegal
SCD presentation of older children
anemia, severe or recurrent musculoskeletal or abdominal pain, aplastic crisis, acute chest syndrome, splenomegaly or splenic sequestration, and cholelithiasis
Consequences of functional asplenia in SCD
high risk for septicemia and meningitis with pneumococci and other encapsulated bacteria
SCD, exacerbation of baseline anemia, parvovirus
aplastic crisis
substitution of valine for glutamic acid at the sixth amino acid position of the hemoglobin molecule, formation of polymers of hemoglobin when the hemoglobin becomes deoxygenated.
SCD
Hg FAS
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.
Hg FAC
FAC means the baby is a carrier of one abnormal hemoglobin gene-for hemoglobin C.
This individual has benign hemoglobin C trait-i.e., is a carrier of hemoglobin C.
Hg FS
both of the baby’s hemoglobin genes have mutations for hemoglobin S.
FS is the most common hemoglobin pattern causing sickle cell disease
Hg FSA
FSA is sickle cell beta thalassemia, meaning one of the globin genes has a mutation for S and the other has a mutation for beta thalassemia (which produces no or little normal hemoglobin).
This pattern causes a sickling disorder, although it may behave in a milder fashion than FS (in which both genes have the sickling mutation).
Hg FSC
sickle cell hemoglobin C disease: one gene has the S mutation and one gene has the mutation for hemoglobin C.
This pattern causes a sickling disorder, although it may behave in a milder fashion than FS
Are Hg FS , FSC and FSA patients essentially managed the same way?
YES
2 common procedures in pt with SCD
Tonsillectomy and cholecystectomy
Why cholecystectomy in SCD?
Bilirubin gallstones occur frequently in all patients with hemolytic anemias—including sickle cell disease—the result of increased release of hemoglobin during breakdown of abnormal red blood cells
What bugs are SCD patients susceptible to?
Encapsulated due to functional asplenia:
- Streptococcus pneumoniae
- Haemophilus influenzae type b
- Neisseria meningitidis
Why and when ppx for pt with SCD?
Until 5 years old, penicillin to protect against sepsis
Why jaundice in SCD
increased breakdown of the red blood cells,
Are SCD children at risk of stroke?
YES; 10% by the age of 15 years
What is acute chest syndrome and what to do?
Vaso-occlusion of lung parenchyma; supplemental oxygen and transfusion therapy.
How to evaluate need for ppx transfusion to protect against stroke in child with sCD?
Transcranial doppler (elevated flow = transfuse)
Why would an SCD patient be on deferasirox or deferoxamine (iron chelators)?
Protection against iron overload due to transfusions
What additional vaccines for SCD child
PCV 23 and Meningococcal at 2 and 5 years old
increased pallor, fatigue, and irritability in SCD
Splenic sequestration crisis (leaves to severe anemia and shock)
When does spleen become small and fibrotic in SCD?
4-6 years old, later if sickle trait
should you take fever in a child with SCD seriously?
YES - may be only sign of severe infection
tachypnea and chest pain in SCD
Work up for acute chest syndrome
What is aplastic crisis?
temporary inhibition of erythroid production frequently associated with viral illness, in particular Parvovirus B19. Because of the baseline rapid destruction of red blood cells and baseline lower hematocrit in patients with sickle cell disease, the brief inhibition of red blood cell production produced by the viral infection can have far greater consequences than in patients with normal red blood cell survival.
What types of processes does acute chest syndrome describe?
Pneumonia
Intrapulmonary sickling
Pulmonary fat embolism
SCD with Fever, cough, chest pain, SOB, decreased oxygenation
Acute chest syndrome
Why CHF in SCD?
Anemia
What may you seen on XR in ACS?
Multilobar infiltrates (more commonly lower and middle lobes)
Effusions
Atelectasis
CXR with Lower lobe infiltrates and cardiomegaly
CHF
Do Patients with sickle cell disease frequently demonstrate an exaggerated leukocytosis and thrombocytosis in response to stress.?
YES
What to control pain in SCD?
Intravenous fluids, NSAIDs, and intravenous narcotics (morphine or derivatives of morphine)
You start SCD patient on opioids for pain mgmt. What to monitor?
Respiratory effort
Hydration is mainstay of SCD treatment. Should you be cautious with this approach during ACS?
YES, don’t want to cause pulmonary edema
A _____ only way to directly reduce or reverse the sickling process which is the underlying cause of the acute chest syndrome
RBC transfusion
What chemotherapeutic agent has been shown to decrease the severity and frequency of the sickling disorders when administered daily to patients with more severe disease.
Hydroxyurea
