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Pediatrics > Sickle Cell > Flashcards

Sickle Cell Flashcards

1
Q

Comprehensive Clinics

A

Nurses, NPs, doctors, social workers, counselors

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2
Q

Sickle Cell Disease Pathophys

A

substitution of valine for glutamic acid at 6th AA position on hemoglobin

  • formation of polymers when hemoglobin becomes deoxygenated
  • deformed (sickled) RBCs –> block blood flow in microvasculature –> local tissue hypoxia, pain, tissue damage
  • hemolysis –> chronic anemia
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3
Q

Sickle cell traits

A

FA - normal
FS - both alleles are S –> sickle cell disease
FSC - one allele S, one allele C –> sickle cell disease
FAS - carrier of sickle cell
FSA - beta-thalassemia

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4
Q

Common procedures for SCD kids

A
  1. Tonsillectomy - lymphoidal tissue hypertrophy –> obstructive sleep apnea
  2. Cholecystectomy - bilirubin gallstones from hemolytic anemia
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5
Q

Risk of sepsis in kids with SCD

A

infants and kids with SCD at greater risk of sepsis

  • decreased splenic function -> encapsulated organisms (s. pneumo, Hib, N. meningitidis)
  • prophylactic penicillin greatly decreases risk (stop around 5-6 y.o)
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6
Q

Goals for comprehensive SCD appointment

A
  1. How is family doing/dealing with disease?
  2. Frequency of painful/vaso-occlusive episodes?
  3. Frequency of fevers?
  4. Access to healthcare?
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7
Q

Expected complications of SCD

A
  1. Jaundice - hemolysis of RBCs
  2. Anemia - can be result of recent infection, hypersplenism
  3. Stroke - increased risk (evaluate with doppler)
  4. Respiratory problems - lungs frequent site for sickling, vaso-occlusion of lung parenchyma –> acute chest syndrome (EMERGENCY = oxygen supplementation and transfusion)
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8
Q

Immunizations for SCD patients

A

Hib vaccine
Pneumococcal vaccine
Meningococcal vaccine
Influenza vaccine

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9
Q

Inheritance of SCD

A

Autosomal recessive - each parent must have 1 allele

- knowing newborn screen is important to know whether or not to start prophylactic penicillin

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10
Q

Prenatal testing for SCD

A

Can do it, some testing in development

  • samples of blood obtained in utero
  • extracting DNA from chorionic villi
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11
Q

Effects of chronic illness on growth and development

A
  • can affect academic progress

- socialization and self-esteem

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12
Q

Growth in SCD

A
  • impairment of growth in SCD is common
  • chronic anemia
  • poor nutrition
  • painful crises
  • endorine dysfunction
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13
Q

Important PE for SCD

A
  1. Splenic Enlargement - common in first 2 years of life (make sure it isn’t massive)
  2. Sclera - jaundice from RBC hemolysis
  3. Neuro exam - evaluate for potential stroke
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14
Q

Baseline CBC in SCD

A

common to have baseline Hgb from 6-9 range

- lower the baseline = lower threshold for acute changes

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15
Q

When to seek care with SCD

A 
FEVER - emergency
Splenic enlargement 
Slurred Speech - stroke
Chest pain - EMERGENCY
Rapid breathing - EMERGENCY
Pallor and jaundice
Priapism - permanent damage possible
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16
Q

Aplastic crisis

A

temporary inhibition of erythroid production caused by viral illness (parvovirus)

17
Q

Acute Chest Syndrome

A

child with SCD presenting with fever, cough, difficulty breathing, low sats, chest pain
- PNA, fat embolism, intrapulmonary sickling

18
Q

DDx for chest pain, tachypnea and fever in SCD

A
  1. Acute Chest Syndrome
  2. Pericarditis
  3. CHF
  4. Rib infarction - vaso-occlusive crisis
  5. Sepsis
19
Q

Acute Chest Syndrome CXR

A

bilateral lung infiltrates
atelectasis - pain can exacerbate this by not taking good breaths
cardiomegaly - from baseline in SCD
possible effusions

20
Q

CBC findings is stress response in SCD

A

SCD patients frequently show exaggerated leukocytosis and thrombocytosis
- don’t definitively help diagnose sepsis or ACS

21
Q

Treatment for pain crises

A 
IV fluids (vigorously rehydrate as it can contribute to sickling), NSAIDs, narcotics (remember they are a respiratory depressant --> further exacerbate atelectasis)
- possible PCA
22
Q

Atelectasis

A

partially collapsed lung due to obstruction

  • serious risk for kids with ACS
  • critical to do deep breathing and assist devices (incentive spirometer)
23
Q

RBC transfusion for ACS

A

only way to directly reduce # of sickling RBCs
*exchange transfusion - best wat
Use if: fall in Hgb below baseline, increased RR, worsening chest symptoms, declining O2 sats, infiltrates on CXR

24
Q

ABx in acute chest syndrome

A

Broad IV Abx started
- Viruses, Chlamydia, Mycoplasma, Bacteria
3rd generation ceph + macrolide

Pediatrics (36 decks)

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