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Cystic Fibrosis

Cystic Fibrosis . Patient: Lily Johnson Case study by Alexa Angelo. Lily Johnson . 14 year old Caucasian female Diagnosed with Cystic Fibrosis when she was 6 months old

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Cystic Fibrosis

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  1. Cystic Fibrosis Patient: Lily Johnson Case study by Alexa Angelo

  2. Lily Johnson 14 year old Caucasian female Diagnosed with Cystic Fibrosis when she was 6 months old Very active girl and participates in dance class three times a week and runs cross country for her high school (usually 5 to 7 miles a day, 3 to 4 days per week) Hospitalized because she caught a cold that her medications could not cure; may have pneumonia

  3. What is Cystic Fibrosis?

  4. What is Cystic Fibrosis? (1) • Caused by a mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) • The abnormal mutation of CFTR causes thick viscous mucous secretions that eventually lead to obstruction of the glands and ducts of various organ systems, including the respiratory tract, the gastrointestinal tract, the liver, the genitourinary system, and the sweat glands.

  5. Signs and Symptoms?

  6. Symptoms of Cystic Fibrosis (2)

  7. Diagnosis (2) Tests include: • Sweat chloride test • Blood test to confirm mutation of CFTR gene • Sputum cultures to test for any infectionstypical to CF • Pancreatic function tests • Pulmonary function tests • DNA-based test to detect genetic variation that causes CF

  8. Treatment for CF (2) • Antibiotics • Inhaled medicines • DNAase enzyme therapy • High concentration of salt solutions • Flu and PPV vaccines annually • Possible lung transplant • Oxygen therapy • Percussion nest, manual chest percussion, A-capella, and TheraPEP device

  9. Possible Nutrition Therapy goals?

  10. Medical Nutrition Therapy (1) • Lily has a very low BMI of 17, and is a very active child • This shows her increase for energy requirements : RDA 110% to 150% for a steady weight gain • Fat consumption is increased (35% to 40% total Kcal) • Protein consumption (15% to 20% total Kcal) • CHO needs to be distributed evenly throughout the day to prevent CHO loading because CF patients are more likely to develop Type 2 DM • Administer Multivitamin with the following vitamin amounts:

  11. Specific Macronutrient Goals for Lily • Total energy requirements: 3,000 kcal • FAT: 3,000 x .40 = 1,200 kcal from fat • 1,200/9 = 133 g fat/day • Protein: 3,000 x .15 = 450 kcal from protein • 450/ 4 = 113 g protein/day • CHO: 3,000 x .45= 1,350 kcal/day • 1,350/4 = 338 g CHO/day

  12. Overall Plan of Action • Increase Lily’s energy intake by adding additional kcal in the form of in-between meal snacks with nutrient-dense foods particularly high in fats • Fortified beverages or pudding may be beneficial • Vitamin supplementation for the following nutrients: Vitamins A, D, E and K, iron and zinc • Monitor weight and lab values • Make sure diet is high in salt, because CF patients are at risk for hyponatremia

  13. Prognosis (2) • Most children with CF live fairly healthy lives until they reach adulthood. • The lung disease eventually worsens until they are disabled • A person with CF has an average life span to approximately 37 years • Death is usually caused by lung complications

  14. References • 1. Nelms, M. N. (2011). Nutrition therapy and pathophysiology (2nd ed.). Belmont, CA: Wadsworth, Cengage Learning. • 2. Cystic fibrosis - PubMed Health. National Center for Biotechnology Information. Retrieved November 10, 2012, from http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/ • 3. http://www.medicine.virginia.edu/clinical/departments/medicine/divisions/digestive-health/nutrition-support-team/nutrition-articles/GoodinArticle.pdf • 4. http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=treatments-and-drugs • 5. http://www.lung.org/assets/documents/publications/solddc-chapters/cf.pdf

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