Mary Rodavich

WVU Graduate Dietetic Intern

Sept 2012

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

2

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

3

4

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

5

1 in 3,500 births

Caucasian ethnicity

Avg. life expectancy: 37 years old

6

7

Source: CFF Patient Registry, 2010 Annual Report

8

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

9

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

10

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.

11

Variety of airway clearance techniques

Antibiotics – Oral, IV, or aerosol

Oral pancreatic enzyme replacement

Lung transplantation

12

13

14

Source: Brunzell et al. Managing CFRD Manual: 5th ed. CFF, 2011.

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%

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

15

16

Moran A, et al. Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes: Diabetes Care. 2010. 33(12);2697-2708.

Sample meal plan for a 3,000 calorie diet

17

Source: Brunzell et al. Managing CFRD Manual: 5th ed. CFF, 2011.

18

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

19

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

20

Calculation from the AND Nutrition Care Manual

Step 1: Calculate BMR

21

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

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

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

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

23

Protein: 15-20% of calories

Levels are increased due to malabsorption

Fat: 35-40% of calories

Carbs: Varies

24

25

Source: AND Nutrition Care Manual

Age Vitamin A

(IU/d)

Vitamin E

(IU/d)

Vitamin D

(IU/d)

Vitamin K

(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

26

Source: AND Nutrition Care Manual

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

27

Sources: AND Nutr. Care Manual, Krause’s Food & Nutr. Therapy

The nutritional status of CF patients have been improving

since 1990…but more needs to be done

28

Source: CFF Patient Registry, 2010 Annual Report

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

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

increases air flow to the lungs.

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 --

32

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.

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

Pt consumes 1-2 Scandishakes daily

Provides 600 cals in 8 oz of whole milk

34

35

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.

36