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)
