1. Clinical Case Study: Cystic fibrosis
Carrie Draney

2. Patient Profile: KS 24 Years Old Female Caucasian Single LDS
Lives with her family

3. Past Medical History Cystic Fibrosis Pancreatic Insufficiency
Kidney Stones
PEG placement

4. KS Previous Admits Cystic fibrosis exacerbation w/ pseudomonas MRSA
Pancreatic insufficiency
Uses PERT with very meal
Nephrolithiasis

5. Weight trends: slightly variable but overall stable
PEG tube replacement
(original tube placed 1 year earlier)
Home G-tube feeding of Nutren 1.0 not tolerated
Started 2 bottles of Naked juice each night
Provides: 840 kcals w/60g protein
Weight trends: slightly variable but overall stable

6. Cystic Fibrosis Autosomal recessive disorder
Different mutations in the CFTR gene
Controls the flow of water and chloride ions
Exocrine glands are affected
Respiratory tract, sweat, salivary glands, intestines, pancreas, liver, reproductive tract
Secrete abnormally thick mucus that obstructs glands and ducts in various organs

7. Nutritional Effects Profound effect on the digestive system
85-90% patients experience pancreatic insufficiency
Maldigestion of food
Malabsorption of nutrients
Decreased bicarbonate production
Decreased bile acid reabsorption (fat malabsorption)
Meconium ileus
Thicker meconium when born causing GI blockage

8. Prognosis Once thought to be a pediatric disease
42% of cases live past 18 years of age
Median predicted survival age is close to 40

9. Present Illness
Chief complaint: Increasing shortness of breath and cough related to cystic fibrosis
Presented with: increasing cough and sputum production but w/o fevers or chills.
Complaints of weight decreasing
No GI complaints of N/V, diarrhea, or constipation

10. Physical Examination Vital Signs: saturation were 91% on room air
Normal is % on room air
Lungs: clear to auscultation and percussion
Abdomen: Bowel sounds present. PEG site looked well maintained
Metabolic: Presented with eosinophilia

11. Lab values Vitamin K: 0 undetectable Vitamin A: 100 ug/dL
Sputum cultures: Pseudomonas +
Albumin: 3.7 g/dL
Prealbumin: 18 mg/dL
HgbA1c: 5.6%
Check glucose and HgbA1c because often diabetes is related to cystic fibrosis.

12. Nutritional Status
Pt. had no complaints about food and was very knowledgeable about her nutritional needs
Complaints of minor weight loss
Current weight 48.5kg/106.9lbs
BMI 17.3kg/m2

13. Treatments- Medication
14 day treatment plan
Meropenem, tobramycin, cefazolin (Antibiotics)
Prednisone
Therapeutic Multivitamin w/ Mineral
PERT
Cholecalciferol (Vitamin D3)
Vitamin A
Zinc Gluconate
Vitamin K
Prednisone to decrease inflammation related to CF. Patient tolerated prednisone treatments.

14. Nutrition Assessment Anthropometrics Day 2 Ht: 5’ 5’’ BMI: 17.8kg/m2
Admit weight: 48.5kg/106.9lbs
Previous admit weight: 49.9kg/108.9lbs
Day 6: 49.9kg/110lbs
BMI: 17.8kg/m2
IBW: 85%

15. Biochemical Undetectable vitamin K levels Albumin: 3.7 g/dL
WNL
Prealbumin: 18 mg/dL
Measurement indicates PO meets minimal nutrition needs.

16. Nutrition focused Physical Findings
Petite
Skin unremarkable with G-tube in place

17. Estimated Needs Using Admit wt: 48.5kg Needs based on weight repletion
Kcals
Kcals/kg
Protein
Protein g/kg
mL Fluid
1ml/kcal
Based on
45-50
58-68g
2180
ABW 48.5kg

18. Nutrition Diagnosis
PES: Increased nutrient needs related to cystic fibrosis as evidenced by BMI of 17.5kg/m2 and mild weight reduction.

19. Nutrition Intervention
Regular diet w/café options
Pt. advised to eat any food from outside the hospital
MVI w/ mineral supplement
Vitamin A, Vitamin D, Vitamin K
Nocturnal TF w/ 2 bottles naked juice
Has tried other oral supplements and has had severe intolerance. Weight stayed the same when she switched from Nutren to Naked Juice and feels better.

20. Nutrition Monitoring and Evaluation
Monitor pt. PO intake
Daily wt. check using a standing scale
Obtain weekly prealbumin measurement

21. Appropriateness of Nutrition Care
KS received appropriate treatment for infections and CF exacerbation
Nutritional care was appropriate. Pt. was very aware of dietary needs for CF.
Future admits are expected

22. References
Cystic Fibrosis Foundation. About cycstic fibrosis. Fibrosis. Accessed October 21, 2015.
Mueller DH. Medical nutrition therapy for pulmonary disease. In: Mahan LK, Escott-Stump S, ed. Krause’s Food, Nutrition, & Diet Therapy. 12th ed. Philadelphia: Elsevier; 2008:
Pagana KD, Pagana TJ. Manual of Diagnostic and Laboratory Test. 5th ed. St. Louis, MO: Elsevier;2014.