Peds Final Review - Hematologic Disorders Flashcards
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
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)
IRON DEFICIENCY ANEMIA
Nursing Diagnoses
Ineffective tissue perfusion
Activity intolerance
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)
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
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
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
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%
HEMOPHILIA
Nursing Diagnoses:
Risk for trauma R/T
Deficient knowledge R/T
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).
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.
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.
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.
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
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)
SICKLE CELL ANEMIA
Nursing Diagnoses:
Ineffective Tissue Perfusion
Acute pain
Risk for infection
Deficient knowledge (crisis prevention)
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
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
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.
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.
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.
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
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)
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.
ACUTE LYMPHOCYTIC LEUKEMIA
Treatment has four phases:
Induction
Sanctuary
Consolidation
Maintenance
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
ACUTE LYMPHOCYTIC LEUKEMIA
Nursing Diagnoses:
Risk for infection
Fear
Deficient knowledge R/T
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
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.
