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328 Pediatrics Exam 2 > Hematology > Flashcards

Hematology Flashcards

1
Q

What are the four major causes of Anemia?

A
  • Inadequate RBC production - nutritional deficiencies, bone marrow disorders (leukemia), medication (chemotherapy)
  • Increased RBC destruction - sickle cell anemia
  • Excessive RBC loss - hemorrhage
  • Nutritional deficiency
    • Decreased iron consumption
    • Excess milk consumption
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2
Q

How do you assess for Anemia in the lab?

A
  • Routine screening for anemia: 1 year
  • CBC with differential (Differential-classification of WBCs)
  • Hemoglobin electrophoresis - type of hemoglobin being produced
  • Reticulocyte Count
    • Immature RBCs
    • Indicates the rate and production of RBCs
  • Ferritin
    • Protein that stores iron
    • Indirectly measures iron
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3
Q

Anemia Severe and Acute manifestations?

A
  • Tachypnea
  • Tachycardia-heart beats faster to get oxygen to vital organs
  • Pallor
  • Prolonged capillary refill
  • Weak pulses
  • Systolic heart murmur
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4
Q

Anemia Chronic manifestations?

A
  • Pallor
  • Jaundice-RBC destruction-bilirubin released
  • Fatigue
  • Delayed growth
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5
Q

How is Anemia treated?

A

Treat underlying cause:

  • Nutritional intervention (Iron-rich diet)
  • Vitamins/Supplements (Oral iron supplements)
  • Transfusions

Supportive Care:

  • Oxygen
  • IV fluids
  • Rest
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6
Q

Blood transfusion nursing considerations?

A
  • Informed consent
  • Two RNs verify blood product prior to administration
  • Administer within 30 minutes of delivery
  • Filter tubing
  • Slower infusion rate the first 15 minutes
  • Max infusion time over 4 hours
  • Vitals at baseline, at 15 minutes, then per institution policy
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7
Q

What adverse effects should you monitor for during and after blood transfusions?

A
  • Fever
  • Chills
  • Pruritus
  • Dyspnea
  • Pain
  • Decreased level of consciousness
  • Change in vital signs from baseline
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8
Q

Describe the etiology of Sickle Cell Disease.

A
  • Autosomal recessive inherited blood disorder
  • Characterized by abnormal hemoglobin in the RBC (HbS).
  • Deoxygenated conditions (dehydration, high altitude, psychological stress), hemoglobin becomes a hard stiff rod.
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9
Q

Describe the etiology of Sickle Cell Disease in relation to genetics.

A
  • 25% chance will inherit two genes for sickle cell or two genes for Hemoglobin A
  • 50% chance will inherit one gene for sickle cell and one normal gene, carrier state
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10
Q

How is Sickle Cell Disease diagnosed?

A
  • Newborn screening
  • Hemoglobin electrophoresis-type of hemoglobin being produced
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11
Q

Compare RBC vs. Sickle-Shaped RBC.

A

RBC:

  • Soft, biconcave
  • Lifespan: 120 days

Sickle-Shaped RBC:

  • RBC polymerize, forming stiff rods
  • Lifespan: 20 days or less
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12
Q

Sickle Cell Disease acute complications?

A
  • Acute Pain Episodes
  • Acute Chest Syndrome
  • Infection
  • Acute Anemia
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13
Q

What are Acute Pain Episodes (sickle cell disease)?

A
  • Most common acute complication
  • Recurrent vaso-occlusion with ischemia
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14
Q

How are Acute Pain Episodes (sickle cell disease) diagnosed?

A

client’s reported pain

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15
Q

How are Acute Pain Episodes (sickle cell disease) managed?

A
  • IV opioids: morphine, hydromorphone
  • Non-steroidal anti-inflammatory agent (NSAID): ibuprofen or ketorolac
  • Integrative therapies: warm compress, massage
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16
Q

What is Acute Chest Syndrome (sickle cell disease)?

A
  • Pulmonary vascular vaso-occlusion
  • New infiltrate on chest imaging
  • Plus 2 of the following
    • Chest pain
    • Decreased oxygen saturation
    • Tachypnea
    • Fever
17
Q

What causes Acute Chest Syndrome (sickle cell disease)?

A
  • Infection
  • Acute pain episode
18
Q

How is Acute Chest Syndrome (sickle cell disease) managed?

A
  • Frequent assessment
  • Chest imaging
  • Oxygenation
  • IV Fluids
  • Antibiotics
  • Analgesia (NSAIDs & Opioids)
  • Blood transfusion if indicated
  • Blood cultures CBCD Reticulocyte count
  • Incentive spirometry
19
Q

What is Infection (sickle cell disease)?

A

Risk for pneumonia, sepsis, and osteomyelitis

20
Q

How does infection affect the spleen?

A
  • Spleen plays a role in preventing infection
  • Filtering abnormal RBCs causes spleen to not function properly
21
Q

How does Anemia affect RBCs?

A
  • Shorter RBC lifespan
  • Reduced oxygen carrying capacity
22
Q

What immunizations are used to manage Sickle Cell Disease?

A
  • Pneumococcal
  • Meningococcal
  • Haemophilus influenzae type B (Hib)
23
Q

How is Penicillin prophylaxis used to manage Sickle Cell Disease?

A
  • All children with SCD from 2 months to 5 years of age
  • Daily dose
24
Q

How is Hydroxyurea used to manage Sickle Cell Disease?

A
  • Increases hemoglobin F
  • HbF reduces HbS polymerization
  • Decrease risk:
    • ACS
    • Acute pain
25
Q

What are some lifestyle modifications that can be used to manage Sickle Cell Disease?

A

Avoid:

  • Dehydration
  • Cold temperatures
  • High altitudes
26
Q

Describe Sickle Cell Disease (CF) in relation to Structural Racism.

A
  • Sickle Cell Disease and Cystic Fibrosis (CF)
    • CF affects one third fewer Americans than SCD, but receives 7-11 times the research funding
      • Unequal development of medications
      • National network of specialty clinics
  • Sickle Cell Disease
    • Fewer centers available
    • Centers less likely to be utilized
27
Q

Describe Sickle Cell Disease (patients with SCD) in relation to Structural Racism.

A
  • Patients with SCD
    • Marginalized and dismissed while seeking medical care for life-threatening complications
    • Stigmatized as drug-seeking and exaggerating their pain
      • Inadequate treatment and increased suffering
    • Often avoid medical care because of the perceived racial stigma
28
Q

Describe Sickle Cell Disease (healthcare professionals) in relation to Structural Racism.

A
  • Open-minded
  • Self-aware
  • Recognize own implicit biases
  • Treat patients equitably, regardless of race
  • Report any unequitable treatment
29
Q

What are the different types of Hemophilia?

A
  • X-linked genetic disorder
  • Hemophilia A
  • Hemophilia B
30
Q

Describe X-linked genetic disorder.

A
  • Characterized by a deficiency or absence of clotting factors
  • Prolonged bleeding
31
Q

Describe Hemophilia A.

A
  • Factor VIII deficiency
  • Treat with Factor VIII
  • Desmopressin
    • Desmopressin - increases Factor VIII and von Willebrand factor
32
Q

Describe Hemophilia B.

A
  • Factor IX deficiency
  • Treat with Factor IX
  • Desmopressin
33
Q

Hemophilia manifestations?

A

Bleeding:

  • With or without trauma
  • Common sites:
    • Joint
    • Muscle
    • Mouth
    • GI sites
    • Hematuria
34
Q

How is Hemophilia managed?

A
  • Prophylactic: Factor VIII or IX
  • Hemophilia A: Emicizumab administered subq every 4 weeks
  • Hold pressure after all punctures
  • Avoid contact sports
35
Q

Hemophilia acute injury nursing interventions?

A
  • Acute injury- Administration of Factor VIII or IX
    • Never delay factor administration
    • RICE (rest, ice, compression, elevation)

328 Pediatrics Exam 2 (7 decks)

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