Hematologic Dysfunction

Question 1

Anemia

Answer 1

• Most common hematologic disorder of childhood
• Dcr in # of RBCs or Hgb concentration, or both
• Dcrd oxygen-carrying capacity of blood

Question 2

Anemias - Morphology

Answer 2

Characteristic changes in RBC size, shape, or color, or a combination of these

Question 3

Anemias - Diagnositc Evaluation

Answer 3

Sometimes defined as Hgb less than 10 or 11 g/dL

Question 4

Anemias - Consequences

Answer 4

• Hemodilution
• Incrd cardiac circulation & turbulence
• Murmur
• Growth restriction

Question 5

Anemias - Supportive Care

Answer 5

• IV fluids to replace intravascular volume
• Oxygen therapy
• Bed rest

Question 6

Iron Deficiency Anemia

Answer 6

• Caused by inadequate supply of dietary iron
• Generally preventable
  > iron-fortified cereals & formulas for infants
  > special needs of prematuer infants
  > adolescents at risk bc of rapid growth & poor eating habits

Question 7

Iron Deficiency Anemia - Therapeutic Management

Answer 7

Incr in the amount of iron the child receives

Question 8

Iron Deficiency Anemia - Nursing Care Management

Answer 8

• Diet
• Iron supplementation

Question 9

Sickle Cell Anemia

Answer 9

• Autosomal recessive disorder
• Clinical Features
  > obstruction caused by sickled RBCs
  > incrd RBC destruction
  > the vascular inflammation
  > abnormal adhesion, entanglement, & meshing of rigid sickle-shaped cells
  > local hypoxia
  > cellular death

Question 10

Sickle Cell Disease - Diagnosis

Answer 10

Universal screening of newborns in US

Question 11

Sickle Cell Disease - Management

Answer 11

• Prevent sickling
• Rest & minimize energy expenditure
• Hydration
• Electrolyte replacement
• Analgesia
• Blood replacement
• Antibiotics

Question 12

Sickle Cell Disease - Nursing Care

Answer 12

• No cure
  > except possible bone marrow transplant
• Supportive care/prevention of sickling episodes
• Frequent bacterial infections bc of immunocompromise
• Bacterial infection: leading cause of death in yooung child w/ SCD
• Usual life span: into 5th decade
• Education!

Question 13

Sickel Cell Disease - Therapeutic Management

Answer 13

• Treat the medical emergencies of sickle cell crisis
  > rest to minimize energy loss
  > hydration through oral or IV
  > electrolyte replacement
  > analgesia for pain (tend to be undermedicated)
  > blood replacement for anemia
  > antibiotics for infection
• Possible prophylactic antibiotics for 2 months - 5 years
• Monitoring of reticulocyte count regularly to evaluate bone marrow function
• Blood transfusion: if given early in crisis, may reduce ischemia

Question 14

Hemophilia

Answer 14

• A group of hereditary bleeding disorders tht result from deficiencies of specific clotting factors
• X-linked recessive
• Identification of a specific factor deficiency allows for definitive treatment

Question 15

Hemophilia A

Answer 15

• “Classic hemophilia”
• Deficiency of factor VIII

Question 16

Hemophilia B

Answer 16

• Also known as “Christmas disease”
• Deficiency of factor IX

Question 17

Von Willebrand Disease

Answer 17

Deficiency of Von Willebrand factor & factor VIII

Question 18

Hemophilia - Diagnostic Evaluation

Answer 18

• Hx of bleeding episodes
  > overt prolonged bleeding
  > hemarthrosis
  > ecchymosis
• X-linked inheritance
• Lab findings
  > low lvls of factor VIII or IX, prolonged partial thromboplastin (PT) time
  > Normal: platelet count, parathormone lvl, & fibrinogen lvl

Question 19

Hemophilia - Care Management

Answer 19

• Prevent bleeding
  > safe environment
  > dental hygiene
• Recognize & control bleeding
  > RICE
• Prevent crippling effects of bleeding
• Support family & home care
  > genetic counseling
• Replace missing clotting factors
  > aggressive replacement therpay w/ factor concentrate
  > home infusion
• Mild-moderate hemophilia; pts live near-normal lives
• control of symptoms & limiting joint damage improve quality of life

Question 20

Von Willebrand’s Disease

Answer 20

• Inherited disorder
• Child has a defect/deficiency in von Willebrand factor protein (helps w/ clotting)

Question 21

Von Willebrand’s Disease - Assessment

Answer 21

• Epistaxis
• Gum bleeding
• Easy bruising
• Excessive menstrual bleeding

Question 22

Von Willebrand’s Disease - Nursing Interventions

Answer 22

• Similar to hemophilia (RICE)
• Admin clotting factors
• Support

Question 23

B-Thalassemia Major

Answer 23

• Autosomal recessive disorder
  > both parents have to carriers
• Common in Mediterranean, African, S. E asain
• Reduced production of 1 of the Beta-globin chains in synthesis of hemoglobin

Question 24

B-Thalassemia Major - Assessment

Answer 24

• Anemia
• Hepatosplenomegaly
• Growth restriction
• Chipmunk facies

Question 25

B-Thalassemia Major - Nursing Interventions

Answer 25

- **Blood admin** - **Monitor for iron overload** > hemochromatosis - **Education**

Question 26

Lead Poisoning

Answer 26

- **Most common btwn 6 months - 6 years** > peaks around 2-3yrs - **Risk screening starting at 6 months** - **Blood lead level greater than 5mcg/dL requires intervention**

Question 27

Lead Poisoning - Treatment

Answer 27

- **Separate child from lead source** > if higher than 45mcg/dL, pharm treatment necessary > Chelation therapy - **Prevention**

Question 28

Epistaxis

Answer 28

- **Isolated & transiet espistaxis is common in childhood** - **Recurrent or severe episodes may indicate underlying disease** > vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von willebrand & hemophilia)

Question 29

Epistaxis - Care Management

Answer 29

- **Remain calm** - **Bleeding usually stops w/in 10 mins after nasal pressure** - **Sit up & lean forward** - **Apply pressure to soft lower part of nose** - **Evaluate further if bleeding continues**