The document discusses cystic fibrosis and cystic fibrosis-related diabetes (CFRD), outlining their causes, symptoms, and treatment, including medical nutrition therapy. It also provides details of a specific 24-year-old female patient's history and condition, including her diagnosis of CFRD, and outlines her nutrition assessment, diagnosis, and prescribed nutrition interventions and monitoring.

1.  
Mary Rodavich
WVU Graduate Dietetic Intern
Sept 2012

2.  Overview of patient
 What is Cystic Fibrosis (CF)?
 What is CFRD?
 Medical Nutrition Therapy for CF
 Nutrition Assessment of Patient
o Nutrition Diagnosis
o Nutrition Prescription
o Nutrition Intervention
o Monitoring/Evaluation
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3.  24 year old female
 Current Medical History
o Cystic Fibrosis (CF)
o CFRD
o MRSA in sputum of lungs
o Pneumothorax
o Enlarged liver
o Not on lung transplant list
 Past Medical History
o 4 sinus surgeries to remove polyps
o GERD
o Port placement and collapsed lung in 2007. Mechanical ventilation
resulting in deconditioning
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4.  4 

5.  Autosomal, recessive disease
 Caused by mutation in CFTR gene
o Mutation in delta F508 gene accounts for 70% of all CF pts
 Results in thick, sticky mucus affecting:
o Respiratory System
o Digestive System
o Reproductive System
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6.  1 in 3,500 births
 Caucasian ethnicity
 Avg. life expectancy: 37 years old
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7. 7
Source: CFF Patient Registry, 2010 Annual Report

8. 8

9.  Lungs become colonized with bacteria
 Bronchiectasis develops, making infections more difficult to
treat
 Manifestations:
o Wheezing
o SOB
o Persistent cough and excessive mucus
o Repeated cases of pneumonia
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10.  Pancreatic exocrine secretions have lower bicarbonate, low
pH, and lower in volume
 Ducts become blocked and obstructed
 Impairs digestion of fat, protein, and starch
 Manifestations:
o Failure to thrive or gain weight
o Malnutrition and malabsorption
o Steattorrhea
o Abdominal discomfort, gas
o Osteoporosis
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11. Pancreatic Enzyme Effects
Lipase Lipase works with bile from the liver to break down fat
molecules.
Protease Protease breaks down proteins.
Amylase Amylase breaks down carbohydrates (starches) into
sugars. This enzyme is also found in saliva.
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12.  Variety of airway clearance techniques
 Antibiotics – Oral, IV, or aerosol
 Oral pancreatic enzyme replacement
 Lung transplantation
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13.  13 

14. Test Diagnosis
75 g Oral Glucose Tolerance Test (OGTT) 2 hr glucose > 200 mg/dL
Casual Blood Glucose > 200 mg/dL
Fasting Blood Glucose > 126 mg/dL
Hgb A1C (not recommended) > 6.5%
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Source: Brunzell et al. Managing CFRD Manual: 5th ed. CFF, 2011.

15.  Insulin therapy
 Oral diabetes agents are not as effective as insulin and are
not recommended
 Check blood sugars 3 times/day
 Moderate aerobic exercise at least 150 min/week
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16. 16
Moran A, et al. Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes: Diabetes Care. 2010. 33(12);2697-2708.

17. Sample meal plan for a 3,000 calorie diet
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Source: Brunzell et al. Managing CFRD Manual: 5th ed. CFF, 2011.

18.  18 

19. 1. 3 meals, 2-3 snacks per day
2. Pancreatic enzyme and vitamin supplementation
3. Unrestricted diet (including high fat foods and additives)
4. Supplements and nourishments
5. Encourage variety of whole grains, nuts, fruits, and veggies
6. Counseling to provide ideas for calories boosters
7. Extra salt especially during hot weather or exercise
8. Adequate calcium and fat-soluble vitamins
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20.  Calorie and protein requirements are usually 1.2 - 2 times the
DRI for age
 Factors to consider:
o Age
o Gender
o Physical activity
o Respiratory infections
o Severity of lung disease
o Severity of malabsorption
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21. Calculation from the AND Nutrition Care Manual
Step 1: Calculate BMR
Age (yrs) Females Males
10-18 12.2(kg) + 746 17.5(kg) + 651
18-30 14.7(kg) + 496 15.3(kg) + 679
30-60 8.7(kg) + 829 11.6(kg) + 879
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22. Step 2: BMR x (Activity Coefficient + Disease Coefficient)
Activity Level Activity Coefficients (AC)
Confined to bed 1.3
Sedentary 1.5
Active 1.7
Lung Disease Severity Disease Coefficients
Mild: FEV1 > 80% AC + 0
Moderate: FEV1 = 40-79% AC + 0.2
Severe: FEV1 = <40 AC + 0.3-0.5
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23. Step 3: Calculate Daily Energy Expenditure,
taking into account the degree of steatorrhea
• For pancreatic sufficient pts (including pts w/ a coefficient of fat
absorption [CFA] >93%)
• Daily energy requirement equals the daily energy expenditure
• For pancreatic insufficient pts
• Daily energy requirement equals the daily energy expenditure
time 0.93/CFA
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24.  Protein: 15-20% of calories
 Levels are increased due to malabsorption
 Fat: 35-40% of calories
 Carbs: Varies
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25. 25
Source: AND Nutrition Care Manual

26. Age Vitamin A Vitamin E Vitamin D Vitamin K
(IU/d) (IU/d) (IU/d) (IU/d)
0-12 mos 1,500 40-50 400 At least 0.3 mg
1-3 yrs 5,000 80-150 400-800
4-8 yrs 5,000-10,000 100-200 400-800
>8 yrs 10,000 200-400 400-800
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Source: AND Nutrition Care Manual

27.  If initial nutrition interventions are not effective and
BMI<19, consider enteral feedings
 Can be NG tube, G-tube, or J-tube
o Usually nocturnal feedings
 Caloric-dense formulas, greater than 1 kcal/mL
 Enzyme replacement:
o If nocturnal feedings – enzymes taken orally before feeding and
once or twice during the night
o Enzyme powder can also be added directly to the formula
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Sources: AND Nutr. Care Manual, Krause’s Food & Nutr. Therapy

28.  The nutritional status of CF patients have been improving
since 1990…but more needs to be done
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Source: CFF Patient Registry, 2010 Annual Report

29.  29 

30. Lab Value
Height 5 ft.
Weight 97 lbs
BMI 18.9 (normal)
% IBW 97%
PO2, arterial 44 mmHg
O2 saturation 84%
Glucose 118 mg/dL
Hgb A1C 8.2%
Albumin 3.1 gm/dL
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31. Medication Name Reason
Creon 2400 Pancreatic enzymes
24,000 USP units of lipase; 76,000 USP units of protease;
120,000 USP units of amylase
Azithromycin, Vancomycin Oral antibiotics
Tobramycin Inhaled antibiotic
Lantus, Novolog Insulin for CFRD
Aquadeks Multivitamin that improves absorption of fat-soluble
vitamins
Protonix Decreases stomach acid
Pulmozyme (by inhalation) Thins and clears mucus
Albuterol (by inhalation) Bronchodilator that relaxes muscles in the airways and
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increases air flow to the lungs.

32. Est. Energy Needs 60-80 kcal/kg 2640-3520 kcal/d
Est. Protein Needs 2-3 g/kg 88-132 g protein/d
Est. Fluid Needs Per MD --
 Current Diet Order: Pediatric, High Calorie, High Protein
 Spoke with pt. Discussed home regimen. Stated
Endocrinology adjusted insulin last month and her blood
sugars are now ranging 80-150 mg/dL.
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33. Diagnosis: Altered nutrition-related laboratory values related to
CFRD as evidenced by glucose: 118 mg/dL, Hgb A1C: 8.2%.
Goals:
Adequate intake
Maintain po intake >75% diet
Maintain wt
Stabilization of blood glucose levels
Intervention: Encourage po intake, Monitor wt, labs, and po
intake
Monitoring: Low/Moderate risk. Follow-up in 5-7 days. 33

34.  Pt consumes 1-2 Scandishakes daily
 Provides 600 cals in 8 oz of whole milk
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35. 35

36.  AND Nutrition Care Manual
 Cystic Fibrosis Foundation: www.cff.org
 Mahan LK, Escott-Stump S. Krause’s Food and Nutrition Therapy: 12th
ed. 2008.
 Brunzell C, Hardin DA, Moran A, Schindler T. Managing CFRD Manual,
An Instruction Guide for Patients and Families: 5th ed. CFF, 2011.
 Moran A, et al. Clinical Care Guidelines for Cystic Fibrosis–Related
Diabetes: A position statement of the American Diabetes Association
and a clinical practice guideline of the Cystic Fibrosis Foundation,
endorsed by the Pediatric Endocrine Society. Diabetes Care. Dec 2010.
33(12);2697-2708.
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