1. Patient: Lily Johnson Case study by Alexa Angelo
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)
In newborns
Delayed growth
Failure to gain weight normally
No bowel movements within first 48 hours of life
Salty tasting skin
Gastrointestinal
Severe constipation
Increased gas
Bloating
Nausea or loss of appetite
Stools that contain fat
Respiratory
Coughing
Increased mucus in lungs and sinuses
Fatigue
Nasal congestion
Recurring pneumonia

7. Diagnosis (2) Tests include: Sweat chloride test
Blood test to confirm mutation of CFTR gene
Sputum cultures to test for any infections typical 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:
Age
Vitamin A (IU)
Vitamin E (IU)
Vitamin D (IU)
Vitamin K (IU)
>8
10,000

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
3. ine/divisions/digestive-health/nutrition-support-team/nutrition- articles/GoodinArticle.pdf
4. fibrosis/DS00287/DSECTION=treatments-and-drugs
5. chapters/cf.pdf