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Flashcards in Peds Final Review - Hematologic Disorders Deck (29)
1

IRON DEFICIENCY ANEMIA

Iron deficiency anemia is when hemoglobin levels are below normal range because of the body’s inadequate supply, intake, or absorption of iron

Iron deficiency anemia is the leading hematologic disorder in children.

The need for iron is greater in children than in adults because of accelerated growth

Anemia may be caused by the following:
--Inadequate stores during fetal development

--Deficient dietary intake

--Chronic blood loss

--Poor utilization of iron by the body

2

IRON DEFICIENCY ANEMIA

Nursing Assessment

Pallor, paleness of mucous membranes

Tiredness, fatigue

Usually seen in infants 6 to 24 months old (times of growth spurt); toddlers and female adolescents most affected

Overweight “milk baby”

Dietary intake low in iron

Milk intake greater than 32 oz/day

Pica habit (eating nonfood substances)

Lab values:
--Decreased Hgb
--Low serum iron level
--Elevated total iron binding capacity (TIBC)

3

IRON DEFICIENCY ANEMIA

Nursing Diagnoses

Ineffective tissue perfusion

Activity intolerance

4

IRON DEFICIENCY ANEMIA

Nursing Interventions:

--Support child’s need to limit activities

--Provide rest periods

--Administer oral iron (ferrous sulfate) as prescribed

Teach family nutritional facts concerning iron deficiency.

Limit milk intake to less than 32oz/day

Teach about dietary sources of iron:
--Meat
--Green, leafy vegetables
--Fish
--Liver
--Whole grains
--Legumes

For infants: iron-fortified cereals and formula

Teach about appropriate nutrition for child’s age

Be aware of family’s income and cultural food preferences

Refer family to nutritionist and/or WIC (Women’s, Infants, and Children’s)

5

IRON DEFICIENCY ANEMIA

NCLEX Hint: Teach family about administration of oral iron:

Give on empty stomach (as tolerated, for better absorption)

Give with citrus juices (Vit C) for increased absorption

Use dropper or straw to avoid discoloring teeth

Teach that stools will become tarry

Teach that iron can be fatal in severe overdose; keep away from other children.

Do not give with any dairy products

6

IRON DEFICIENCY ANEMIA

NCLEX Hint: Teach family about administration of oral iron:

Give on empty stomach (as tolerated, for better absorption)

Give with citrus juices (Vit C) for increased absorption

Use dropper or straw to avoid discoloring teeth

Teach that stools will become tarry

Teach that iron can be fatal in severe overdose; keep away from other children.

Do not give with any dairy products

7

HEMOPHILIA

Inherited bleeding disorder

Transmitted by an X-linked recessive chromosome (mother is the carrier; her sons may express the disease).

A normal individual has between 50% and 200% factor activity in blood; the hemophiliac has from 0% to 25% activity

The affected individual usually is missing either factor VIII (Classic, 75% of cases) or Factor IX

8

HEMOPHILIA

Nursing Assessment

Male child: first red flag may be prolonged bleeding at the umbilical cord or injection site (vitamin K) or following circumcision

Prolonged bleeding with minor trauma

Hemarthrosis (most frequent site of bleeding)

Spontaneous bleeding into muscles and tissues (less severe cases have fewer bleeds)

Loss of motion in joints
Pain

Lab values:
--PTT is prolonged
--Factor assays less than 25%

9

HEMOPHILIA

Nursing Diagnoses:

Risk for trauma R/T

Deficient knowledge R/T

10

HEMOPHILIA

Nursing Interventions:

--Administer fresh-frozen plasma, cryoprecipitate of fresh plasma, or lyophilized (freeze-dried) concentrate as prescribed.

--Administer pain medication as prescribed (analgesics containing no aspirin).

--Follow blood precautions: risk for hepatitis

Teach child and family home care

Teach to recognize early signs of bleeding into joints

Teach local treatment for minor bleeds (pressure, splinting, ice)
Teach administration of factor replacement

Discuss dental hygiene : use soft toothbrushes

Provide protective care: give child soft toys; use padded bed rails

Have child wear MedicAlert identification

Refer family for genetic counseling

Support child and family during periods of growth and development when increased risk for bleeding occurs (e.g., learning to walk, tooth loss).

11

HEMOPHILIA

HESI/NCLEX Hint: Inherited bleeding disorders (hemophilia and sickle cell anemia) are often used to test knowledge of genetic transmission patterns.

Remember:

Autosomal recessive:
both parents must be heterozygous, or carriers of the recessive trait for the disease to be expressed in their offspring. With each pregnancy, there is a one in four chance that the infant will have the disease. However, all children of such parents can get the disease – not just 25% of them. This is the transmission pattern of sickle cell anemia, cystic fibrosis, and phenylketonuria (PKU).

X-linked recessive trait:

The trait is carried on the X chromosome; therefore, it usually affects male offspring, as in hemophilia. With each pregnancy of a woman who is a carrier, there is a 25% chance of having a child with hemophilia. If the child is male, he has a 50% chance of having hemophilia. If the child is a female, she has a 50% chance of being a carrier.

12

SICKLE CELL ANEMIA

Description: Inherited autosomal recessive disorder of red blood cells

It occurs primarily in persons of African and eastern Mediterranean descent. One in 12 persons of African ancestry is a carrier of the heterozygous gene HgbAS. There, the risk that two parents of African ancestry will have a child with sickle cell disease is 0.7%

It usually appears after 6 months of age

Hemoglobin S (HgbS) replaces all or part of the normal Hgb, which causes the red blood cells to sickle when oxygen is released into the tissues.

----Sickled cells cannot flow through capillary beds.

-----Dehydration promotes sickling.

13

SICKLE CELL ANEMIA

NCLEX Hint: Hydration is very important in the treatment of sickle cell disease because it promotes hemodilution and circulation of red cells through the blood vessels.

HgbS has a less than normal life span (less than 40 days), which leads to chronic anemia.

Tissue ischemia causes widespread pathologic changes in spleen, liver, kidney, bones, and CNS.

14

SICKLE CELL ANEMIA

NCLEX Hint: Important terms

Heterozygous gene (HgbAS) – sickle cell trait

Homozygous gene (HbSS) – sickle cell disease

Abnormal hemoglobin (HgbS) – disease and trait

15

SICKLE CELL ANEMIA

Nursing Assessment:

--Children of African descent, usually over 6 months of age

---Parents with sickle cell trait or sickle cell anemia

---Lab diagnosis: Hgb electrophoresis (differentiates trait from disease)

Frequent infections (nonfunctional spleen)

Tiredness

Chronic hemolytic anemia

Delayed physical growth

**Vaso-occlusive crisis: the classic sign
-Fever

-Severe abdominal pain

-Hand-foot syndrome
(infants); painful
edematous hands and feet

-Arthralgia

-Leg ulcers (adolescents)

-Cerebrovascular
accidents (increased risk
with dehydration)

16

SICKLE CELL ANEMIA

Nursing Diagnoses:

Ineffective Tissue Perfusion

Acute pain

Risk for infection

Deficient knowledge (crisis prevention)

17

SICKLE CELL ANEMIA

Nursing Interventions:

Teach family that to prevent crisis (hypoxia), they should:

Keep child from exercising strenuously

Keep child away from high altitudes

Avoid letting child become infected, and seek care at first sign of infection

Use prophylactic penicillin if prescribed

Keep child well hydrated

Not withhold fluids at night because enuresis is complication of both the disease and the treatment

18

SICKLE CELL ANEMIA

Nursing Interventions:

For child hospitalized with a vaso-occlusive crisis:

Administer IV fluids (one to two times maintenance levels) and electrolytes, as prescribed, to increase hydration and treat acidosis.

Monitor I&O

Administer blood products as prescribed.

Administer analgesics, including parenteral morphine for severe pain, as prescribed.

Use warm compresses (not ice)

Administer prescribed antibiotics to treat infection

19

SICKLE CELL ANEMIA

Nursing Interventions:

Administer pneumococcal vaccine, meningococcal vaccine, and Hib vaccine as prescribed.

Administer hepatitis B vaccine as prescribed (for child at risk because of transfusions).

Refer family for genetic counseling

Support child and family experiencing chronic disease.

20

SICKLE CELL ANEMIA

NCLEX Hint:

Supplemental iron is not given to clients with sickle cell anemia.

The anemia is not caused by iron deficiency.

Folic acid is given orally to stimulate RBC synthesis.

21

SICKLE CELL ANEMIA

NCLEX Hint:

Supplemental iron is not given to clients with sickle cell anemia.

The anemia is not caused by iron deficiency.

Folic acid is given orally to stimulate RBC synthesis.

22

ACUTE LYMPHOCYTIC LEUKEMIA

Cancer of the blood-forming organs

Acute lymphocytic leukemia accounts for about 80% of childhood leukemia

It is noted for the presence of lymphoblasts (immature lymphocytes), which replace normal cells in the bone marrow

Blast cells are also seen in the peripheral blood

23

ACUTE LYMPHOCYTIC LEUKEMIA

Acute lymphocytic leukemia is classified according to whether it involves:

T lymphocytes

B lymphocytes

Null cells (neither T cells nor B cells)

24

ACUTE LYMPHOCYTIC LEUKEMIA

More than 75% of children with acute lymphocytic leukemia have the null cell type, which has the best prognosis

The signs and symptoms of leukemia result from the replacement of normal cells by leukemic cells in the bone marrow and extramedullary sites.

Definitive diagnosis is made by bone marrow aspiration.

25

ACUTE LYMPHOCYTIC LEUKEMIA

Treatment has four phases:

Induction
Sanctuary
Consolidation
Maintenance

26

ACUTE LYMPHOCYTIC LEUKEMIA

Nursing Assessment:

Pallor, tiredness, weakness, lethargy due to anemia

Petechia, bleeding, bruising due to thrombocytopenia

Infection, fever due to neutropenia

Bone joint pain due to leukemic infiltration of bone marrow

Enlarged lymph nodes; hepatosplenomegaly

Headache and vomiting (signs of CNS involvement)

Anorexia, weight loss

Lab data: bone marrow aspiration that reveals 80 % -- 90% immature blast cells

27

ACUTE LYMPHOCYTIC LEUKEMIA

Nursing Diagnoses:

Risk for infection

Fear

Deficient knowledge R/T

28

ACUTE LYMPHOCYTIC LEUKEMIA

Nursing Interventions:

-Recommend private room

-Reverse isolation if prescribed

-Provide child with age-appropriate explanations for diagnostic tests, treatments, and nursing care.

-Examine child for infection of skin, needle-stick sites, dental problems

-Administer blood products as prescribed

-Administer antineoplastic chemotherapy as prescribed

-Monitor for side effects of chemotherapeutic agents

-Provide care directed toward managing side effects and toxic effects of antineoplastic agents.

-Administer anti-emetics as prescribed

Monitor for signs of infection

Monitor for signs of bleeding

Monitor for cumulative toxic effects of drugs: hepatic toxicity, cardiac toxicity, renal toxicity, and neurotoxicity

Provide oral hygiene

Provide small, appealing meals; increase calories and protein; refer to nutritionist

Promote self-esteem and positive body image if child has alopecia, severe weight loss, or other disturbance in body image

Provide care to prevent infection

Provide emotional support for family in crisis.

Encourage family’s and child’s input and control in determining plans and treatment.

Monitor fluid balance

29

ACUTE LYMPHOCYTIC LEUKEMIA

NCLEX Hint: Prednisone is frequently used in combination with antineoplastic drugs to reduce the mitosis of lymphocytes.

Allopurinol, a xanthine oxidase inhibitor, is also administered to prevent renal damage caused by uric acid buildup and cellular lysis.

NCLEX Hint: Have epinephrine and oxygen readily available to treat anaphylaxis when administering L-asparaginase.