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IRON DEFICIENCY ANEMIA

DATABASE
A. Most prevalent nutritional disorder among children in the United States; caused by lack of adequate
sources of dietary iron
1. Infant usually has iron reserve for 6 months
2. Premature infant lacks reserve
3. Children receiving only milk have no source of iron
B. Insidious onset: usually diagnosed because of an infection or chronic GI problems
C. Causes
1. Decreased intake
2. Increased destruction
3. Increased loss
D. Clinical findings
1. Pallor, weakness, tachycardia, dizziness
2. Slow motor development
3. Poor muscle tone
4. Hemoglobin level below normal for age (general rule: below 11dl)
E. Therapeutic interventions
1. Food sources rich in iron
2. Iron replacement
a. Oral iron sources
(1) Drug: ferrous sulfate - most absorbable form of iron
(2) Adverse effects: nausea, vomiting; fatalities in children who ingest enteric-coated
tablets, thinking they are candy
(3) Drug interactions: ferrous sulfate binds tetracycline and decreases absorption;
magnesium trisilicate decreases absorption of iron
b. Parenteral iron sources
(1) Drug: parenteral iron-dextran injection (Imferon)
(2) Adverse effects: tissue staining (use Z tract for intramuscular injection), fever,
lymphadenopathy, nausea, vomiting, arthralgia, urticaria, severe peripheral vascular
failure, anaphylaxis, secondary hematochromatosis

NURSING CARE OF CHILDREN WITH IRON DEFICIENCY ANEMIA


A. Assessment
1. Nutritional history
2. History of chronic infection
3. Eating habits
a. Pica
b. Ingestion of lead
4. Bowel habits/blood in stools
5. Family history of hematologic disorder
B. Analysis/Nursing Diagnoses
1. Activity intolerance related to generalized weakness
2. Altered nutrition: less than body requirements related to knowledge deficit of appropriate foods
C. Planning/Implementation
1. Prevent development of anemia
______________________________________________________________
a. Teach pregnant women the importance of their iron intake
b. Encourage feeding of iron-fortified infant formula or breastfeeding
c. Encourage feeding iron-fortified infant cereal
d. Introduce foods high in iron
2. Provide for nutrition and proper administration of supplemental iron
a. Vitamin C and hydrochloric acid aid absorption
b. Folic acid acts as a coenzyme in the formation of heme; proteins are necessary for the
synthesis of hemoglobin; ascorbic acid promotes the conversion of folic acid to folinic acid
c. Oxalates, phosphate, and caffeine decrease absorption
d. Use a straw because some liquid preparations stain teeth
e. Discolors stools; may cause gastric irritation or constipation
D. Evaluation/Outcomes
1. Child engages in appropriate activities
2. Child consumes adequate nutrients for correction of anemia
3. Parents can verbalize nutritional requirements of child

SICKLE CELL ANEMIA


DATABASE
A. Autosomal disorder affecting hemoglobin
B. Defective hemoglobin causes red blood cells to become sickle shaped and clump together under
reduced oxygen tension; initially fetal hemoglobin prevents sickling
C. Classification
1. Sickle cell anemia: homozygous for sickle cell gene
2. Sickle cell trait: heterozygous for sickle cell gene
D. Clinical findings
1. Vasoocclusive crisis (pain episode): most common and non–lifethreatening
a. Results from sickled cells obstructing blood vessels, causing occlusion, ischemia, and
potential necrosis
b. Symptoms include fever, acute abdominal pain (visceral hypoxia), hand-foot syndrome,
priapism, and
arthralgia without an exacerbation of anemia
2. Splenic sequestration crisis
a. Results from the spleen pooling large quantities of blood, which causes a precipitous drop
in blood pressure and ultimately shock
b. Acute episode occurs most commonly in children between 8 months and 5 years of age;
can result in death from anemia and cardiovascular collapse
c. Chronic manifestation is termed functional asplenia
3. Aplastic crisis: diminished red blood cell production
a. May be triggered by a viral or other infection
b. Profound anemia results due to rapid destruction of red blood cells combined with a decreased
production
4. Hyperhemolytic crisis: increased rate of red blood cell destruction
a. Characterized by anemia, jaundice, and reticulcytosis
b. Rare complication that frequently suggests a coexisting abnormality such as glucose-6-
phosphate dehydrogenase deficiency
5. Stroke: sudden and severe complication with no related illnesses
a. Sickled cells block the major blood vessels in the brain
b. Repeat strokes in 60% of children who have experienced previous one
6. Chest syndrome: clinically similiar to pneumonia
7. Overwhelming infection
a. Streptococcus pneumonia
b. Haemophilus influenzae type B
E. Therapeutic interventions
1. Prevention of sickling phenomenon
a. Adequate oxygenation
b. Adequate hydration
c. Administration of hydroxyurea to limit sickling
2. Treatment of crisis
a. Rest
b. Hydration/electrolyte replacement
c. Pain management
d. Antibiotic therapy
e. Blood products

NURSING CARE OF CHILDREN WITH SICKLE CELL ANEMIA


A. Assessment
1. Vital signs
2. Neurologic signs
3. Vision/hearing
______________________________________________________________
4. Location and intensity of pain
B. Analysis/Nursing Diagnoses
1. Body image disturbance related to:
a. Retarded growth and maturation
b. Limited activity tolerance
c. Chronic illness
2. Fear related to:
a. Unfamiliar environment
b. Separation from support system
3. Pain related to tissue ischemia
4. Altered tissue perfusion (cardiovascular) related to decreased oxygen tension
C. Planning/Implementation
1. Prevent crisis
a. Avoid infection, dehydration, and other conditions causing strain on body, which
precipitates a crisis; prophylactic use of pneumococcal, meningococcal, and Haemophilus flu
vaccines
b. Avoid hypoxia: treat respiratory tract infections immediately
c. Avoid dehydration
(1) May cause a rapid thrombus formation
(2) Daily fluid intake should be calculated according to body weight (130 to 200 ml per
kilogram [2 to 3 oz per pound])
(3) During crisis, fluid needs to be increased, especially if the child is febrile
2. During crisis provide for:
a. Adequate hydration (may need IV therapy)
b. Proper positioning, careful handling
c. Exercise as tolerated (immobility promotes thrombus formation and respiratory problems)
d. Adequate ventilation
e. Control of pain; use narcotics; schedule to prevent pain
f. Blood transfusions for severe anemia
3. Provide for genetic counseling
a. Disorder mostly of blacks; can be found in Mediterranean people
b. Parents need to know the risk of having other children with trait or disease
c. If both parents are carriers, each pregnancy has 25% chance of producing a child with the
disease
d. Screen young children for the disorder, since clinical manifestations usually do not appear
before 6 months of age
D. Evaluation/Outcomes
1. Child reports minimal pain
2. Child verbalizes feelings about disease process
3. Child demonstrates positive body image
4. Child does not exhibit signs of sickling

LEUKEMIA
DATABASE
A. The most common type of childhood cancer; prognosis is improving
B. Peak incidence: 2 to 6 years of age
C. Malignant neoplasm of blood-forming organs
D. In children, overproduction of immature leukocytes: blast-cell or stem-cell leukemia
E. Classification
1. Acute lymphocytic: about 85% of incidence; better prognosis than for myelogenous
2. Acute myelogenous or acute nonlymphoid: about 10% incidence; poorer prognosis
______________________________________________________________
3. Others: 5% incidence
F. Clinical findings
1. Caused by overproduction of immature nonfunctional cells
2. Anemia: pallor, weakness, irritability
3. Infection: fever
4. Tendency toward bleeding: petechiae and bleeding into joints
5. Pain in joints caused by seepage of serous fluid
6. Tendency toward easy fracture of bones
7. Enlargement of spleen, liver, lymph glands
8. Abdominal pain and anorexia resulting in weight loss
9. Necrosis and bleeding of gums and other mucous membranes
10. Later symptoms: CNS involvement and frank hemorrhage
G. Therapeutic interventions
1. Induce remission by chemotherapy (see Pharmacology related to neoplastic disorders)
a. Prednisone: steroid
b. Vincristine: plant alkaloid
c. Methotrexate: folic acid antagonist
d. L-asparaginase: enzyme
e. 6-mercaptopurine: purine antagonist
f. Cyclophosphamide: alkylating agent
g. Doxorubicin hydrochloride: cytotoxic antibiotic
2. Prevent CNS involvement by use of irradiation and intrathecal methotrexate, because leukemic cells
invade the brain, but most antileukemic drugs do not pass the blood-brain barrier
3. Transfusions to replace and provide needed blood factors such as red blood cells, platelets, and
white blood cells
4. Bone marrow transplantation
NURSING CARE OF CHILDREN WITH LEUKEMIA
A. Assessment
1. Hematologic status
a. Anemia
b. Thrombocytopenia
c. Neutropenia
2. Activity level
3. Exposure to infectious diseases
4. Complications of therapy/disease process
B. Analysis/Nursing Diagnoses
1. Activity intolerance related to:
a. Anemia
b. Reduced energy and fatigue
2. Body image disturbance related to:
a. Loss of hair
b. Moon face
c. Debilitation
3. Altered family processes related to situational crisis (child with life-threatening disease)
4. Fear related to:
a. Diagnostic tests
b. Procedures
5. Anticipatory grieving related to perceived potential loss of child
6. Risk for infection related to:
a. Decreased immune response
b. Use of chemotherapy
7. Risk for injury (including hemorrhage) related to:
a. Decreased strength and endurance
b. Pain and discomfort
c. Decreased platelets
8. Altered nutrition: less than body requirements related to loss of appetite
9. Pain related to physiologic effect of neoplasia and treatment
10. Impaired physical mobility related to:
a. Decreased strength and endurance
b. Pain and discomfort
c. Neuromuscular impairment
11. Risk for impaired skin integrity related to:
a. Immobility
b. Administration of antimetabolites
c. Disease process
C. Planning/Implementation
1. Encourage adjustment to chronic illness; stress need for normal life-style
2. Deal with the child's idea of death: discussion should be appropriate to level of understanding
a. Preschooler: concept that death is reversible; greatest fear is separation
______________________________________________________________
b. Child 6 to 9 years of age: concept that death is personified; a person actually comes and
removes the child
c. Child over 9 years of age: adult concept of death as irreversible and inevitable
3. Be alert for and attempt to support the child experiencing side effects of drugs
a. Cytoxan: severe nausea, vomiting, cystitis, and alopecia
b. Vincristine: constipation, alopecia, neurotoxicity
c. Methotrexate: oral and rectal ulcers
d. Corticosteroids: mood swings and fluid retension
4. Prevent infection by hand washing; avoid contact with people who have active infections and
avoid crowded places
5. Handle the child carefully because of pain and hemorrhage; administer analgesics for pain
6. Provide gentle oral hygiene; soft, bland foods; increased liquids
7. Provide for frequent rest periods, quiet play
D. Evaluation/Outcomes
1. Child participates in developmental, age-appropriate activities
2. Child repeats information accurately
3. Child exhibits no physiologic signs of pain
4. Child and family demonstrate understanding of procedures
5. Family and child discuss fears, concerns, and needs
6. Child is not exposed to infectious diseases
7. Child does not exhibit signs of bleeding/injury
8. Child ambulates without difficulty
9. Child consumes adequate calories for growth
10. Child expresses feelings about altered body image
11. Child does not exhibit signs of infection

HEMOPHILIA
DATABASE
A. Defect in clotting mechanism of blood
B. Genetic disorder; X-linked recessive transmission
C. Usually occurs in males; females are carriers but do not have the disease
D. Classification
1. Factor VIII deficiency (classic hemophilia): hemophilia A
2. Factor IX deficiency (Christmas disease): hemophilia B
E. Clinical findings
1. Prolonged bleeding from any wound
2. Bleeding into the joints (hemarthrosis), resulting in pain, deformity, and retarded growth
3. Intracranial hemorrhage
4. Severity of bleeding
a. Mild
(1) Factor VIII activity of 5% to 50%
(2) Bleeding with severe trauma or surgery
b. Moderate
(1) Factor VIII activity of 1% to 5%
(2) Bleeding with trauma
c. Severe
(1) Factor VIII activity of 1%
(2) Spontaneous bleeding without trauma
5. Anemia
F. Therapeutic interventions
______________________________________________________________
1. Control of bleeding
2. Prevention of bleeding with use of factor replacement
a. Drugs that replace deficient coagulation factors
(1) Factor VIII concentrate from recombinant DNA
(2) Factor IX complex contains factors II, VII, IX, X (concentrated)
b. Adjunctive measures
(1) Aminocaproic acid (Amicar): inhibits the enzyme that destroys formed fibrin and
increases fibrinogen activity in clot formation
(2) Fibrinogen: maintains plasma fibrinogen levels required for clotting materials
(3) Thrombin: supplies physiologic levels of natural material at superficial bleeding
sites to control bleeding

NURSING CARE OF CHILDREN WITH HEMOPHILIA


A. Assessment
1. Parent/child knowledge of disease process and injury prevention
2. Joint bleeding
3. Mobility of joints
B. Analysis/Nursing Diagnoses
1. Body image disturbance related to:
a. Perception of self as different
b. Inability to participate in selected activities
2. Altered family processes related to situational crisis (child with a chronic illness)
3. Risk for infection related to frequent transfusions
4. Risk for injury (hemorrhage) related to deficient blood clotting
5. Knowledge deficit related to:
a. Disease process
b. Home management
c. Activity limitations
6. Impaired mobility related to effects of hemorrhages into joints and other tissues
7. Pain related to bleeding into joints/tissues
C. Planning/Implementation
1. Instruct the child and parents in the treatment of bleeding, especially of joints
a. Immobilization of the area
b. Compression of the area
c. Elevation of the body part
d. Application of cool compresses
2. Provide for appropriate activity that lessens the chance of trauma, which is often difficult
because boys are so physically active
3. Select safe toys and inform parents to safe-proof house to minimize injuries; secure throw rugs
4. Avoid use of aspirin or ibuprofen
5. Control joint pain so the child uses extremities to prevent muscle atrophy
6. Provide counseling, because disease is genetic and parents need assistance
7. Encourage parents to treat the child as normally as possible, avoiding overprotection or
overpermissiveness
SKELETAL MALFORMATIONS
CLUB FOOT

DATABASE
A. Foot has been twisted out of normal shape or position
B. Most common type: talipes equinovarus: foot is fixed in plantar flexion (downward) and deviated
medially (inward) C. Clinical findings
1. Deformity is readily apparent at birth
2. Deformity may be rigid or flexible
D. Therapeutic interventions
1. Treatment is most successful when started early in infancy because delay causes muscles and
bones of legs to develop abnormally, with shortening of tendons
2. Nonsurgical treatment: gentle, repeated manipulation of the foot with casting; done every few
days for 1 to 2 weeks then at 1- to 2-week intervals
3. Surgical treatment: done if nonsurgical treatment not effective
a. Tight ligaments released
b. Tendons lengthened or transplanted
4. Follow-up care of the client
a. Extended medical supervision is required because there is a tendency for this deformity to
recur (considered cured when the child is able to wear normal shoes and walk properly)
b. Care emphasizes muscle reeducation (by manipulation) and proper walking
c. Heels and soles of braces or shoes prescribed following correction must be kept in repair
d. Corrective shoes may have sole and heel lifts on lateral border to maintain proper position

NURSING CARE OF INFANTS AND CHILDREN WITH CLUBFOOT


A. Assessment
1. Parental understanding of treatment regimen
2. Skin and circulation of affected limb
B. Analysis/Nursing Diagnoses
1. Risk for injury related to knowledge deficit and use of corrective devices
2. Risk for impaired skin integrity related to use of corrective devices
______________________________________________________________
C. Planning/Implementation
1. Observe toes for signs of circulatory impairment; make sure toes are visible at the end of the
cast
2. Watch for signs of weakness and wear of the cast, especially if the child is allowed to walk on it
3. Teach parents all the necessary care and emphasize the need for follow-up, which may be
prolonged
D. Evaluation/Outcomes
1. Parents can demonstrate home care
2. Skin remains intact
CONGENITAL HIP DYSPLASIA –DATABASE

A. Imperfect development of hip - can affect femoral head, acetabulum, or both


B. Head of the femur does not lie deep enough within the acetabulum and slips out on movement
C. Occurs in females seven times more often than in males
D. Classification
1. Acetabular dysplasia
a. Mildest form
b. Femoral head remains in acetabulum
2. Subluxation
a. Most common form
b. Femoral head partially displaced
3. Dislocation
a. Femoral head not in contact with acetabulum
b. Displaced posteriorly and superiorly
E. Clinical findings
1. Limitation in abduction of leg on the affected side
2. Asymmetry of gluteal, popliteal, and thigh folds
3. Audible click when abducting and externally rotating the hip on the affected side: Ortolani's
sign
4. Apparent shortening of the femur: Galeazzi's sign
5. Waddling gait and lordosis when the child begins to walk
F. Therapeutic interventions
1. Directed toward enlarging and deepening the acetabulum by placing the head of the femur
within the acetabulum and applying constant pressure
2. Proper positioning: legs slightly flexed and abducted
a. Pavlik harness
b. Frejka pillow: a pillow splint that maintains abduction of the legs
c. Bryant's traction
d. Spica cast: from the waist to below the knees
e. Brace
3. Surgical intervention such as open reduction with casting

Nursing Care of Infants and Children with Congenital Hip Dysplasia


A. Assessment
1. Limb shorter on affected side
2. Positive Ortolani's test (hip click)
3. Restricted abduction of hip on affected side
B. Analysis/Nursing Diagnoses
1. Altered family processes related to having a child with a physical defect
2. Altered growth and development related to immobilization
3. Impaired physical mobility related to immobilizing device
4. Risk for injury related to:
a. Corrective device
b. Immobility
C. Planning/Implementation
1. Respiratory problems: hypostatic pneumonia
a. Change position frequently from back to stomach; raise head of mattress rather than
head to prevent flexion of neck
b. Teach parents postural drainage and exercises for child, such as blowing bubbles to
increase lung expansion
c. Encourage parents to seek immediate medical care if the child develops congestion or
cough
2. Infection and excoriation of skin
a. Observe for circulation to toes, pedal pulses, and blanching
b. Do not let the child put small toys or food inside cast
c. Use gauze strips inside cast as a scratcher
d. Alert parents to signs of infection, such as odor
e. Protect cast edges with adhesive tape or waterproof material, especially around perineum
f. Use diapers and plastic lining to minimize soiling of cast by feces and urine
3. Constipation from immobility
______________________________________________________________
a. Teach parents to observe for straining on defecation and constipation
b. Increase fluids and fiber to prevent constipation
4. Nutrition
a. Provide small, frequent meals because of inflexibility of cast around waist (a window may
be made over the abdominal area to allow for expansion with meals)
b. Adjust calorie intake, because less energy expenditure can lead to obesity
5. Transportation and positioning
a. Use wagon or stroller with back flat or mechanic's creeper
b. Protect child from falling when positioned
c. Never pick up child by the bar between the legs of the cast (use two people to provide
adequate body support if necessary)
6. Meet emotional needs
a. Use touch as much as possible; small children can be picked up and cuddled
b. Stimulate and provide for play activities appropriate to age
7. Provide parents with help and support
a. Give written instructions
b. Schedule routine home visits with telephone counseling available
c. Stress need for follow-up care because treatment may be prolonged
d. Prepare parents for the possible use of an abduction brace after the cast is removed
D. Evaluation/Outcomes
1. Child can move about and control environment
2. Child remains free of injury
3. Parents can discuss special needs and demonstrate home care
4. Child is able to regain prior movement (crawling/walking) when device is removed
SCOLIOSIS
DATABASE

A. Lateral curvature of the spine usually associated with a rotary deformity that eventually causes
cosmetic and physiologic alterations in the spine, chest, and pelvis
B. Cause in 70% of cases is "idiopathic"; probably transmitted as an autosomal dominant trait with
incomplete penetrance
C. Most common spinal deformity
D. More frequent in adolescent girls during growth spurt
E. Classification
1. Nonstructural scoliosis: curve is flexible and corrects by bending
2. Structural scoliosis: curve fails to straighten on side-bending: characterized by changes in
the spine and its supporting structures
F. Clinical findings
1. Prominence of one hip
2. Deformity of the rib cage
3. Prominence of one scapula
4. Difference in shoulder or scapular height
5. Curve in the vertebral spinous process alignment
6. Breasts appear unequal in size
7. Other clues
a. Clothes do not fit right
b. Skirt hems are uneven
G. Therapeutic interventions
1. Screening for scoliosis
2. Diagnosis: confirmed by x-ray examination
3. Interventions depend on severity of the curvature
a. Exercise such as swimming can be used in nonstructural scoliosis
b. Mild to moderate curvature
(1) Braces
(a) Milwaukee brace, an individually adapted steel and leather brace that
extends from a chin cup and neck pads to the pelvis, where lumbar pads
rest on the hips
(b) Low profile or underarm brace
(2) Treatment
(a) Worn 23 hours a day
(b) The child is gradually weaned from the brace over a 1- to 2-year period
(c) Brace then worn only at night until the spine is mature
(d) Electrical stimulation to the convex side of the curvature may prevent
progression of the scoliosis
c. More severe curves usually require surgery: techniques consist of spinal realignment
and straightening by way of external or internal fixation and instrumentation combined
with bony fusion (arthrodesis) of the realigned spine
(1) Harrington rods
(2) Luque segmental instrumentation
(3) Dwyer instrumentation
(4) Texas Scottish Rite Hospital (TSRH) system
d. Most severe scoliotic curvatures require traction devices and exercises for a time
before spinal fusion to provide partial correction and more flexibility

NURSING CARE OF ADOLESCENTS WITH SCOLIOSIS


A. Assessment
1. Have the child stand erect, clothed only in underpants (and bra if older girl) and observe from
behind; note asymmetry of the shoulders and hips
2. Have the child bend forward so the back is parallel with the floor; observe from the side, noting
asymmetry or prominence of the rib
B. Analysis/Nursing Diagnoses
1. Body image disturbance related to:
a. Perceived alteration in body structure
b. Altered appearance when using supportive devices
2. Altered family processes related to situational crisis (child with a structural defect)
3. Altered growth and development related to disease process
4. Risk for injury related to use of supportive devices
5. Knowledge deficit related to disorder
6. Impaired physical mobility related to use of supportive devices
7. Risk for impaired skin integrity related to:
______________________________________________________________
a. Presence of brace
b. Use of electrical stimulation
8. Pain related to medical/surgical therapies
C. Planning/Implementation
1. Check spinal alignment
2. Reinforce and clarify explanations provided by the orthopedist in regard to:
a. Appliance
b. Plan of care
c. Activities allowed or restricted
d. Child's and parents' responsibilities in therapy
3. Examine skin surfaces in contact with the brace or electrical stimulator for signs of irritation;
implement corrective action to treat or prevent skin breakdown
4. Help in selection of the appropriate wearing apparel to wear over the brace to minimize altered
appearance and footwear to maintain proper balance
5. Prepare for surgery if required
D. Evaluation/Outcomes
1. Family members demonstrate appropriate care and support of child
2. Child demonstrates proper use of brace
3. Parent/child verbalize understanding of disorder and treatment plan
4. Child verbalizes feelings and concerns
5. Skin does not break down
6. Child engages in activities appropriate to limitations and developmental level
7. Child reports minimal pain

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