1. The client/caregiver can define cystic fibrosis.
    1. It is an inherited disease that affects breathing and digestion.
    2. Cystic fibrosis affects the movement of sodium (salt) into and out of certain cells.
    3. This malfunction results in formation of thick, sticky mucus.
    4. It affects multiple systems of the body, such as
      1. Lungs (most common)
      2. Pancreas (also common)
      3. Gastrointestinal tract
      4. Salivary glands
      5. Reproductive tract
    5. It is usually diagnosed in early infancy or childhood. Cystic fibrosis rarely occurs in blacks and Asians.
  2. The client/caregiver can recognize signs and symptoms.
    1. Coughing or wheezing
    2. Repeated lung infections
    3. Shortness of breath
    4. Greasy, foul-smelling, pale stools
    5. Excessive appetite but poor weight gain and growth
    6. Intestinal blockage in newborns
    7. Rectal prolapse
    8. Salty sweat, tears, and saliva
    9. Infertility
    10. Clubbing of the fingers and toes
  3. The client/caregiver can list measures to manage cystic fibrosis.
    1. Prevent infection.
      1. Avoid persons with respiratory infections.
      2. Take antibiotics as needed.
      3. Obtain immunizations as recommended.
    2. Provide respiratory care.
      1. Be aware of postural drainage and percussion (respiratory care treatments).
      2. Take oxygen as needed.
      3. Use nebulizer treatments.
      4. Take medications as ordered.
      5. Do breathing exercises.
      6. Avoid known irritants such as smoke and air pollution.
    3. Provide a well-balanced diet.
      1. Eat food that is high in calories and protein but low in fat.
      2. Increase fluids.
      3. Eat salty foods, and add salt to food at the table as tolerated.
      4. Take pancreatic enzyme supplements. Take supplements with foods especially carbohydrates.
      5. Discuss the use of vitamins A, D, E, and K and zinc with physician.
    4. Do daily exercise with rest periods. Use respiratory treatments before exercise. Modify exercise on warm days, and drink plenty of fluids.
    5. Keep follow-up appointments.
    6. Provide good oral hygiene.
  4. The client/caregiver can list possible complications.
    1. Pneumothorax
    2. Cor pulmonale
    3. Dehydration
    4. Salt depletion
    5. Rectal prolapse
    6. Damage to the eye
    7. Osteoporosis

Resources

March of Dimeswww.marchofdimes.com
Cystic Fibrosis Foundation800-344-4823www.cff.org/
National Heart, Lung, and Blood Institute Health Information301-592-8573www.nhlbi.nih.gov/health/infoctr/index.htm
Support groups
Dietician
Counseling

References

Ackley, B. J., & Ladwig, G. B. (2006). Nursing diagnosis handbook: A guide to planning care. St. Louis: Mosby Inc.Canobbio, M. M. (2006). Mosby’s handbook of patient teaching.St. Louis: Mosby Inc.Lutz, C., & Przytulski, K. (2001). Nutrition and diet therapy. Philadelphia: F. A. Davis Company.Muscari, M. E. (2005). Pediatric nursing. Philadelphia: Lippincott Williams & Wilkins.Novak, J. C., & Broom, B. L. (1999). Maternal and child health nursing. St. Louis: Mosby, Inc.

Credits

Client Teaching Guides for Home Health Care, 2nd ed.© 2008 Jones and Bartlett Publishers, Inc.www.jbpub.com

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