Ward Class

An Online Resource Site For Student Nurses

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Ward Class is an online resource for student nurses. Created with the aim of helping student nurses cope with the demands of nursing school and clinicals, it offers free downloads, notes, sample NCPs, sample drug studies, study aids, news and updates, and practical tips to its users.

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The December 2012 PRC Nursing Board Exam Result is already available here at Ward Class and at Pinoy R.N.




Respiratory Disorders: Cystic Fibrosis

  • a disease in which there is a generalized dysfunction of the exocrine glands
  • mucus secretions of the body, particularly in the lungs and pancreas, have difficulty flowing through gland ducts
  • also characterized by a marked electrolyte change-->sweat and saliva have high levels of sodium chloride
  • an autosomal recessive trait, caused by an abnormality in the long arm of chromosome 7-->results in an inability to transport small molecules across cell membranes-->leads to dehydration of the epithelial cells in the airway and pancreas
  • most common lethal genetic disease among Caucasians in United Sates and Europe
Assessment Findings
  • Extent of Organ Involvement
    • Pancreas-->85% of clients with cystic fibrosis (CF) have pancreatic involvement
      • ducts are obstructed leading to fibrosis and atrophy of pancreas and eventual little or no release of pancreatic enzymes (lipase, amylase, trypsin)
      • malabsorption of fats and proteins due to absence of enzymes
      • steatorrhea-->foul-smelling, greasy stools
      • failure to thrive due to loss of nutrients and inability absorb fat-soluble vitamins (A, D,E,K)
    • Respiratory Tract-->99.9% of clients with CF have respiratory involvement
      • increased production of secretions causes airway obstruction
      • pulmonary congestion leads to cor pulmonale
      • death occurs by drowning in own secretions
    • Reproductive System
      • males are sterile
      • increased mucus in vaginal tract of females make conception difficult
      • pregnancy increases stress on respiratory system of females with CF
    • Liver
      • One-third of clients with CF develop portal hypertension/cirrhosis
  • Signs and Symptoms
    • Pancreas
      1. growth failure
      2. large, foul-smelling, fatty stools (steatorrhea)
      3. meconeum ileus-->blockage of intestines in newborns, usually first sign
      4. rectal prolapse--often caused by stools that are difficult to pass or frequent coughing
      5. voracious appetite
      6. protruding abdomen (caused by bulk of feces in intestines) and thin extremities and buttocks
      7. symptoms of ADEK deficiency (night blindness, dry skin, rickets, he,olysis of RBCs, bleeding tendencies, and fractures)
      8. anemia
      9. diagnostic tests:
        • duodenal contents contain little or no trypsin
        • increased fecal fat in stool specimen
    • Respiratory System
      1. signs of respiratory distress (nasal flaring, open-mouthed breathing, wheezing, use of accessory muscles when breathing)
      2. frequent productive cough
      3. frequent pseudomonas infections
      4. nasal polyps
      5. barrel chest-->due to air trapping
      6. clubbing of fingers
      7. activity intolerance
      8. diagnostic tests
        • chest x-ray-->atelectasis (collapse of lung tissue), infiltration, emphysemic changes
        • abnormal pulmonary function studies
        • ABG reveals respiratory acidosis
    • Electrolytes
      1. hyponatremia in hot weather
      2. salty taste to sweat-->due to high levels of NACL in sweat
      3. diagnostic tests: pilocarpine iontophoresis sweat test reveals elevated NACL (2-5x higher than normal)
Medical Management
  1. bronchodilators-->medications such as albuterol, delivered by an inhaler or a nebulizer, helps keep airway patent.
  2. antibiotics-->given for frequent Pseudoman infections
  3. chest physioterapy.
  4. oral enzymes and better nutrition-->Pancrease, Cotazym, Viokase-->given with meals and snacks
  5. diet modifications-->high-calorie, high-protein, low-fat diet for older children; predigested formula for infants
  6. ibuprofen (Advil, Motrin, others) may slow lung deterioration in some children with cystic fibrosis
Surgical Management

Lung transplantation for severe breathing problems, life-threatening pulmonary complications or increasing resistance to antibiotics used to treat lung infections.

Nursing Management
  1. administer pancreatic enzymes with meals as ordered-->do not mix until ready to use ; best mixed with applesauce
  2. administer antibiotics, expectorants, and mucolytics as ordered
  3. avoid cough suppressants and antihistamines-->respiratory goal for patients with CF is to cough up secretions
  4. provide diet high in calories, protein, but normal in fat; no empty calories
  5. add salt to meals esp. during summer; give salty snacks such as pretzels
  6. provide vitamins ADEK in water-soluble form; other vitamins may be doubled as ordered
  7. encourage coughing and breathing exercises
  8. provide percussion and drainage at least 4 times a day
  9. provide aerosol treatments as ordered (nebulizers, mist tent)
  10. provide client teachings and discharge planning concerning:
    • counseling
      • for parents of child with CF, focus on CF being genetic disorder so it may be passed on to any future children
      • for older clients: focus on marriage and intimacy issues; provide resources on fertility evaluation, genetic, and financial counseling
    • promotion of client's independence
    • avoidance of environmental triggers-->cigarette smoke, pollution, sources of infections
    • availability of support groups and community agencies
    • education for clients -->focus on home education during hospitalization or home recovery; give appropriate referrals to concerned agencies
Prognosis

Cystic Fibrosis is fatal in early life but with early detection and treatments, 50% of infants with CF live past 28 years of age. With lung transplantation, full life expectancy has increased.

Prevention

Cystic Fibrosis is not preventable.

Note: Although Cystic Fibrosis affects the lungs, pancreas, and liver, most reference books categorize it as a respiratory disorder.

Related Link:
Sample NCP for Cystic Fibrosis


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