peds final
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1. Anemia - The most common hematologic disorder of childhood
- Decrease in number of RBCs and/or hemoglobin concentration below normal
- Decreased oxygen-carrying capacity of blood
2. Therapeutic - Treat underlying cause
Management of 1) Transfusion after hemorrhage if needed
Anemia 2) Nutritional intervention for deficiency anemias
- Supportive care
1) IV fluids to replace intravascular volume
2) Oxygen
a) Assess activity level and avoid overexertion.
3) Bed rest
3. Nursing - Assessment and physical findings
Considera- - Assessment of diet (especially if lactose intolerant or low in iron)
tions—Anemia - Prepare child and family for laboratory tests
- Decrease oxygen demands (rest)
1) Signs of exertion: tachycardias, palpitations, tachypnea, dyspnea
- Prevent complications
1) Main complication is cardiac decompensation. Watch for signs of heart failure.
2) Increased risk for infection
- Anticipatory guidance and education
1) Iron-rich foods
4. Diagnostic Evalu- - CBC with differential
ation of Anemia 1) Decreased RBCs
2) Decreased Hgb and Hct
3) Hemoglobin below 10 to 11g/dl
- Specific tests to look for particular types of anemia
5. Routine Screen- - Once during infancy (9-12months)
ing for pediatric - Once during early childhood (1-5yrs)
, peds final
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patients across - Once during late childhood (5-12yrs)
childhood for - Once during adolescence (14-20yrs)
anemia (AAP rec-
ommendations)
6. Sickle Cell Ane- - A hereditary hemoglobinopathy
mia - Ethnicity
1) Occurs primarily in African Americans
a) Occurrence 1 in 375 infants born in United States
b) 1 in 12 have sickle cell trait
c) Occasionally also in people of Mediterranean descent
d) Also seen in people of South American, Arabian, and East Indian descent
7. Etiology of Sickle - Autosomal recessive disorder
Cell 1) 1 in 12 African Americans are carriers (have sickle cell trait)
2) If both parents have trait, each offspring will have 1 in 4 likelihood of having
disease
8. Pathophysiology - Partial or complete replacement of normal Hgb with abnormal hemoglobin S
of Sickle Cell (HgbS)
- Hemoglobin in the RBCs takes on an elongated ("sickle") shape
- Sickled cells are rigid and obstruct capillary blood flow
- Microscopic obstructions lead to engorgement and tissue ischemia
- Hypoxia occurs and causes sickling
- Large tissue infarctions occur
- Damaged tissues in organs lead to impaired function
9. Splenic seques- - Sickled cells block blood vessels out of the spleen. Too many RBCs in spleen
tration causes enlarged spleen (Splenomegaly)
- May require splenectomy at early age
- Results in decreased immunityLife threatening—death can occur within hours
Blood pools in the spleen
Signs
, peds final
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Profound anemia
Hypovolemia
Shock
10. Sickle Cell Crisis - Anything that increases the body's need for oxygen or alters transport of oxygen
Precipitating fac- - Trauma
tors - Infection, fever
- Physical and emotional stress
- Increased blood viscosity due to dehydration
- Hypoxia
- From high altitude, poorly pressurized airplanes, hypoventilation, vasoconstric-
tion due to cold temp/hypothermia
11. Sickle Cell Crisis - Vasoocclusive (VOC) thrombotic
types - Splenic sequestration
- Aplastic crises
12. Vasoocclusive 1) AKA painful event/ painful episode
(VOC) thrombotic 2) Most common type of crisis—very painful
3) Stasis of blood with clumping of cells in microcirculation ’ischemia ’infarction
4) Signs—fever, pain, tissue engorgement
13. Splenic seques- - Life threatening: death can occur within hours
tration - Blood pools in the spleen
- Signs
1) Profound anemia
2) Hypovolemia
3) Shock
14. Aplastic crises - Diminished production and increased destruction of RBCs
- Triggered by viral infection or depletion of folic acid
- Signs include profound anemia, pallor
, peds final
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15. Acute Chest Syn- - Similar to pneumonia. Medical Emergency.
drome - VOC or infection results in sickling in the lungs
- Chest pain, fever, cough, tachypnea, wheezing, and hypoxia
- Repeated episodes may lead to pulmonary hypertension
16. Acute Chest Syn- - Aggressive treatment of infection
drome Medical - Possibly prophylactic with oral penicillin from age 2 months to 5 years
Management - Monitor reticulocyte count regularly to evaluate bone marrow function
- Blood transfusion, if given early in crisis, may reduce ischemia
- Frequent transfusion leads to hemosiderosis (iron in tissues)
1) Treat with iron chelation
a) Parenterally: deferoxamine
b) Oral: deferasirox or deferiprone
c) Used alone or in combination
d) + Vitamin C to promote iron excretion
17. Nursing Manage- - Monitor child's growth: watch for failure to thrive
ment Sickle Cell - Minimize tissue deoxygenation: careful multisystem assessment
Anemia - Assess pain and hydration status
- Minimize Crises
- Promote supportive therapies
- Maintain HYDRATION (oral or other as needed)
- Carefully monitor for signs of shock
18. Managing Pain in - Careful assessment using developmentally appropriate pain scale
SCD - Prompt treatment of pain
- Position for comfort
- Range of motion exercises
- Warm compresses
- Rest and gentle massage
- Fear of addiction is not a reason to withhold opioids during crisis
19.
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1. Anemia - The most common hematologic disorder of childhood
- Decrease in number of RBCs and/or hemoglobin concentration below normal
- Decreased oxygen-carrying capacity of blood
2. Therapeutic - Treat underlying cause
Management of 1) Transfusion after hemorrhage if needed
Anemia 2) Nutritional intervention for deficiency anemias
- Supportive care
1) IV fluids to replace intravascular volume
2) Oxygen
a) Assess activity level and avoid overexertion.
3) Bed rest
3. Nursing - Assessment and physical findings
Considera- - Assessment of diet (especially if lactose intolerant or low in iron)
tions—Anemia - Prepare child and family for laboratory tests
- Decrease oxygen demands (rest)
1) Signs of exertion: tachycardias, palpitations, tachypnea, dyspnea
- Prevent complications
1) Main complication is cardiac decompensation. Watch for signs of heart failure.
2) Increased risk for infection
- Anticipatory guidance and education
1) Iron-rich foods
4. Diagnostic Evalu- - CBC with differential
ation of Anemia 1) Decreased RBCs
2) Decreased Hgb and Hct
3) Hemoglobin below 10 to 11g/dl
- Specific tests to look for particular types of anemia
5. Routine Screen- - Once during infancy (9-12months)
ing for pediatric - Once during early childhood (1-5yrs)
, peds final
Study online at https://quizlet.com/_h0n18k
patients across - Once during late childhood (5-12yrs)
childhood for - Once during adolescence (14-20yrs)
anemia (AAP rec-
ommendations)
6. Sickle Cell Ane- - A hereditary hemoglobinopathy
mia - Ethnicity
1) Occurs primarily in African Americans
a) Occurrence 1 in 375 infants born in United States
b) 1 in 12 have sickle cell trait
c) Occasionally also in people of Mediterranean descent
d) Also seen in people of South American, Arabian, and East Indian descent
7. Etiology of Sickle - Autosomal recessive disorder
Cell 1) 1 in 12 African Americans are carriers (have sickle cell trait)
2) If both parents have trait, each offspring will have 1 in 4 likelihood of having
disease
8. Pathophysiology - Partial or complete replacement of normal Hgb with abnormal hemoglobin S
of Sickle Cell (HgbS)
- Hemoglobin in the RBCs takes on an elongated ("sickle") shape
- Sickled cells are rigid and obstruct capillary blood flow
- Microscopic obstructions lead to engorgement and tissue ischemia
- Hypoxia occurs and causes sickling
- Large tissue infarctions occur
- Damaged tissues in organs lead to impaired function
9. Splenic seques- - Sickled cells block blood vessels out of the spleen. Too many RBCs in spleen
tration causes enlarged spleen (Splenomegaly)
- May require splenectomy at early age
- Results in decreased immunityLife threatening—death can occur within hours
Blood pools in the spleen
Signs
, peds final
Study online at https://quizlet.com/_h0n18k
Profound anemia
Hypovolemia
Shock
10. Sickle Cell Crisis - Anything that increases the body's need for oxygen or alters transport of oxygen
Precipitating fac- - Trauma
tors - Infection, fever
- Physical and emotional stress
- Increased blood viscosity due to dehydration
- Hypoxia
- From high altitude, poorly pressurized airplanes, hypoventilation, vasoconstric-
tion due to cold temp/hypothermia
11. Sickle Cell Crisis - Vasoocclusive (VOC) thrombotic
types - Splenic sequestration
- Aplastic crises
12. Vasoocclusive 1) AKA painful event/ painful episode
(VOC) thrombotic 2) Most common type of crisis—very painful
3) Stasis of blood with clumping of cells in microcirculation ’ischemia ’infarction
4) Signs—fever, pain, tissue engorgement
13. Splenic seques- - Life threatening: death can occur within hours
tration - Blood pools in the spleen
- Signs
1) Profound anemia
2) Hypovolemia
3) Shock
14. Aplastic crises - Diminished production and increased destruction of RBCs
- Triggered by viral infection or depletion of folic acid
- Signs include profound anemia, pallor
, peds final
Study online at https://quizlet.com/_h0n18k
15. Acute Chest Syn- - Similar to pneumonia. Medical Emergency.
drome - VOC or infection results in sickling in the lungs
- Chest pain, fever, cough, tachypnea, wheezing, and hypoxia
- Repeated episodes may lead to pulmonary hypertension
16. Acute Chest Syn- - Aggressive treatment of infection
drome Medical - Possibly prophylactic with oral penicillin from age 2 months to 5 years
Management - Monitor reticulocyte count regularly to evaluate bone marrow function
- Blood transfusion, if given early in crisis, may reduce ischemia
- Frequent transfusion leads to hemosiderosis (iron in tissues)
1) Treat with iron chelation
a) Parenterally: deferoxamine
b) Oral: deferasirox or deferiprone
c) Used alone or in combination
d) + Vitamin C to promote iron excretion
17. Nursing Manage- - Monitor child's growth: watch for failure to thrive
ment Sickle Cell - Minimize tissue deoxygenation: careful multisystem assessment
Anemia - Assess pain and hydration status
- Minimize Crises
- Promote supportive therapies
- Maintain HYDRATION (oral or other as needed)
- Carefully monitor for signs of shock
18. Managing Pain in - Careful assessment using developmentally appropriate pain scale
SCD - Prompt treatment of pain
- Position for comfort
- Range of motion exercises
- Warm compresses
- Rest and gentle massage
- Fear of addiction is not a reason to withhold opioids during crisis
19.
