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Information

  1. DOWN SYNDROME
    1. Introduction: Down syndrome (trisomy 21) is a chromosomal abnormality involving an extra chromosome #21 and resulting in 47 chromosomes instead of the normal 46 chromosomes. As a consequence, the child usually has varying degrees of mental retardation, characteristic facial and physical features, and other congenital anomalies. Down syndrome is the most common chromosomal disorder, occurring in approximately 1 of 800 to 1000 live births. Perinatal risk factors include advanced maternal age, especially with the first pregnancy (although average maternal age is now 25-28 years for an infant with Down's); paternal age is thought to be a related factor. Multiple causality is suspected.
    2. Assessment:
      1. Physical characteristics
        1. Brachycephalic (small, round head ) with oblique palpebral fissures (almond-shaped eyes ) and Brushfield spots (speckling of iris )—depressed nasal bridge ("saddle nose") and small, low-set ears.
        2. Mouth
          1. Small oral cavity with protruding tongue causes difficulty sucking and swallowing.
          2. Delayed eruption/misalignment of teeth.
        3. Hands
          1. Clinodactyly—incurved little finger.
          2. Simian crease—transverse palmar crease.
        4. Muscles: hypotonic ("floppy baby") with hyperextensible joints.
        5. Skin: dry, cracked.
      2. Genetic studies reveal an extra chromosome #21 ("trisomy 21").
      3. Intellectual characteristics
        1. Mental retardation—varies from severely retarded to low-average intelligence.
        2. Most fall within "trainable" range, or IQ of 36 to 51 ("moderate mental retardation").
      4. Congenital anomalies/diseases
        1. 40% to 45% have congenital heart defects: mortality highest in clients with Down syndrome and cyanotic heart disease.
        2. GI: tracheoesophageal fistula (TEF), Hirschsprung's disease.
        3. Thyroid dysfunction, especially hypothyroidism.
        4. Visual defects: cataracts, strabismus.
        5. Hearing loss.
        6. Increased incidence of leukemia.
      5. Growth and development
        1. Slow growth, especially in height.
        2. Delay in developmental milestones.
      6. Sexual development
        1. Delayed or incomplete.
        2. Women—small number have had offspring (majority have had abnormality).
        3. Men—infertile.
      7. Aging
        1. Premature aging, with shortened life expectancy.
        2. Death—generally related to respiratory complications: repeated infections, pneumonia, lung disease.
    3. Analysis/nursing diagnosis:
      1. Risk for aspiration related to hypotonia.
      2. Altered nutrition, less than body requirements, related to hypotonia or congenital anomalies.
      3. Altered growth and development related to Down syndrome.
      4. Self-care deficit related to Down syndrome.
      5. Altered family processes related to birth of an infant with a congenital defect.
      6. Knowledge deficit related to Down syndrome.
    4. Nursing care plan/implementation:
      1. Goal: prevent physical complications.
        1. Respiratory
          1. Use bulb syringe to clear nose, mouth.
          2. Vaporizer.
          3. Frequent position changes.
          4. Avoid contact with people with upper respiratory infections.
        2. Aspiration
          1. Small, more frequent feedings.
          2. Burp well during/after infant feedings.
          3. Allow sufficient time to eat.
          4. infoImagePosition after meals: head of bed elevated, right side—or on stomach, with head to side.
        3. Observe for signs and symptoms of: heart disease, constipation/GI obstruction, leukemia, thyroid dysfunction.
      2. Goal: meet nutritional needs.
        1. Suction (before meals) to clear airway.
        2. Adapt feeding techniques to meet special needs of infant/child (e.g., use long, straighthandled spoon).
        3. Monitor height and weight.
        4. As child grows, monitor caloric intake (tends toward obesity with advancing age).
        5. foodImageOffer foods high in bulk to prevent constipation related to hypotonia.
      3. Goal: promote optimal growth and development.
        1. Encourage parents to enroll infant/toddler in early stimulation program and to follow through with suggested exercises at home.
        2. Preschool/school-age: special education classes.
        3. Screen frequently, using Denver II to monitor development.
        4. Help parents focus on "normal" or positive aspects of infant/child.
        5. Help parents work toward realistic goals with their child.
      4. Goal: health teaching.
        1. Explain that tongue-thrust behavior is normal and that child should be re-fed.
        2. Before adolescence—counsel parents and child about delay in sexual development, decreased libido, marriage and family relations.
        3. In severe cases, assist parents to deal with issue of placement/institutionalization.
    5. Evaluation/outcome criteria:
      1. Physical complications are prevented.
      2. Adequate nutrition is maintained.
      3. Child attains optimal level of growth and development.
  2. ATTENTION DEFICIT–HYPERACTIVITY DISORDER (ADHD); BEHAVIORAL DISORDER (DSM-IV)
    1. Introduction: As defined by the American Psychiatric Association (APA), this diagnostic term includes a persistent pattern of inattention or hyperactivity-impulsivity. The exact cause and pathophysiology remain unknown. The major symptoms include a greatly shortened attention span and difficulty in integrating and synthesizing information. This disorder is three times more common in boys than girls, with onset before age 7; the diagnosis is based on the child's history rather than on any specific diagnostic test.
    2. Assessment:
      1. The behaviors exhibited by children with ADHD are not unusual behaviors seen in children. The behavior of children with ADHD differs from the behavior of non-ADHD children in both quality and appropriateness:
        1. Motor activity is excessive.
        2. Developmentally "younger" than chronological age.
      2. Inattention
        1. Does not pay attention to detail.
        2. Does not listen when spoken to.
        3. Does not do what he or she is told to do.
      3. Hyperactivity
        1. Fidgets and squirms excessively.
        2. Cannot sit quietly.
        3. Has difficulty playing quietly.
        4. Seems to be constantly in motion, moving or talking; always "on."
      4. Impulsiveness
        1. Blurts out answers before question is completed.
        2. Has difficulty awaiting turn. Interrupts others.
    3. Analysis/nursing diagnosis:
      1. Altered thought processes related to inattention and impulsiveness.
      2. Impaired physical mobility related to hyperactivity.
      3. Risk for injury related to impulsivity.
      4. Self-esteem disturbance related to hyperactivity and impulsivity.
      5. Knowledge deficit related to behavioral modification program, medications, and follow-up care.
    4. Nursing care plan/implementation:
      1. Goal: teach family and child about ADHD.
        1. Provide complete explanation about disorder, probable course, treatment, and prognosis.
        2. Answer questions directly, simply.
        3. Encourage family to verbalize; offer support.
      2. Goal: provide therapeutic environment using principles of behavior modification and/or psychotherapy.
        1. Reduce extraneous or distracting stimuli.
        2. Reduce stress by decreasing environmental expectations (home, school).
        3. Provide firm, consistent limits.
        4. Special education programs.
        5. Special attention to safety needs.
      3. Goal: reduce symptoms by means of prescribed medication.
        1. pillImageMedications: Ritalinand Cylert—both are CNS stimulants but have a paradoxical calming effect on the child's behavior. Tofranil and Norpramin—both are tricyclic antidepressants that ↑ action of norepinephrine and serotonin in nerve cells, but also can have paradoxical calming effect on child's behavior. Must monitor for development of tics and arrhythmias.
        2. Health teaching (child and parents).
          1. Need to take medication regularly, as ordered. Avoid taking medication late in the day because it may cause insomnia; monitor neurological and cardiac status. Assess for ↓ appetite → ↓ weight; avoid caffeine.
          2. Need for long-term administration, with probable decreased need as child nears adolescence.
      4. Goal: provide safe outlet for excess energy.
        1. Alternate planned periods of outdoor play with schoolwork or quiet indoor play.
        2. Channel energies toward safe, large-muscle activities: running track, swimming, bicycling, hiking.
    5. Evaluation/outcome criteria:
      1. Family and child verbalize understanding of "attention deficit disorders."
      2. Therapeutic environment enhances socially acceptable behavior.
      3. Medication taken regularly, with behavioral improvements noted.
      4. Excess energy directed appropriately.