Don’t Sweat It:

Basics of CF Nutrition

Presenters:

Erin Hribernik, MS, RD, CNSC

Umme Vahanvaty, MS, RD, CSP

Learning Objectives

1. Discuss the physiology of cystic fibrosis and its nutrition

related complications

2. Calculate the estimated nutrition needs and dosage of

enzyme replacement therapy for children with cystic

fibrosis

3. Describe the nutrition interventions for the

management of cystic fibrosis in children

• Cystic fibrosis is a progressive, recessive genetic disease that is caused by

a mutation in the cystic fibrosis transmembrane conductance regulator

(CFTR) gene.

Cystic Fibrosis

CF

Incidence Carrier

Frequency

Caucasians 1 in 3,300 1 in 29

Hispanics 1 in 9,500 1 in 49

African-Americans 1 in 15,300 1 in 62

Asian-Americans 1 in 32,100 1 in 90

Genetic Testing for Cystic Fibrosis. NIH Consensus Development Conference, 1997.

According to the Cystic Fibrosis Foundation Patient Registry, in the

United States:

• More than 30,000 people are living with cystic fibrosis (more than 70,000

worldwide).

• Approximately 1,000 new cases of CF are diagnosed each year.

• More than 75 percent of people with CF are diagnosed by age 2.

• More than half of the CF population is age 18 or older.

Incidence and Prevalence

Diagnosis

Diagnosing CF is a multistep process.

A complete diagnostic evaluation for CF should

include:

• a genetic or carrier test (“Gold Standard”),

• a sweat chloride test,

• a fecal elastase test,

• and a clinical evaluation at a CF Foundation-

accredited care center

Elevated sweat chloride levels are diagnostic of CF

Diagnosis

Fecal elastase (EL1) is a diagnostic test for pancreatic function in people with

Cystic Fibrosis, since it remains intact during its intestinal transition and its

concentration reflects the secretory capacity of the pancreas with 100%

sensitivity.

Diagnosis

People with CF can have a variety of

symptoms, including:

Very salty-tasting skin

Persistent coughing, at times with

phlegm

Frequent lung infections including

pneumonia or bronchitis

Wheezing or shortness of breath

Poor growth or weight gain in spite of a good

appetite

Frequent greasy, bulky stools or difficulty with

bowel movements

Symptoms of CF

CFTR Function

Normal CF

• CFTR dysfunction begins a cascade leading to structural damage in

the lungs

• Progressive lung disease is the leading cause of CF morbidity and

mortality.

• The cascade can result in infection, inflammation, and damage

Progressive Lung Damage

• A similar cascade occurs in the pancreas, leading to organ damage

• Damage to the pancreas is multi-factorial, driven primarily by CFTR

dysfunction

Clinical Manifestations of CF

Patients require care from CF multidisciplinary teams and their complications

change over time

•Such complications include:

•Monitoring for these complications can help detect their emergence and

progression

•Other less prevalent complications may also occur such as pancreatitis in 10-

15% of pancreatic-sufficient patients

CF Related Complications

• Pancreas:

• Thickened secretions prevent digestive enzymes from reaching the intestine

• Auto-digestion of the pancreas occurs

• CF Related Diabetes can result from pancreatic destruction

• Liver:

• Thickened biliary secretions cause cirrhosis

• Severe liver disease present in ~5%-6% of CF patients

• Some may require a liver transplant

• Intestinal tract:

• Thick secretions in digestive tract cause build up of stool leading to constipation and gut inflammation

• Distal Intestinal Obstruction Syndrome (DIOS) is a severe complication of CF constipation

The CF Gut

• Exocrine Pancreatic insufficiency is present in

85% of people with CF

• Absent pancreatic digestive enzymes cause

malabsorption, malnutrition and growth failure

• Fats are most poorly digested, however protein

digestion likely affected as well

• ↑ Fat in stools and abdominal pain

• Fat soluble vitamin deficiency

Exocrine Pancreatic Insufficiency (EPI)

Pancreatic Sufficiency

• About 15% of people with CF do have pancreatic

function

• Acute and chronic pancreatitis is much more

common – Occurs in about 10-15% of patients who are pancreatic sufficient

– Managed by avoiding trigger foods (often foods that are high

fat)

– May still require non-enteric or enteric coated enzymes if

chronic

Endocrine Pancreatic Insufficiency

• Endocrine Pancreatic insufficiency causes Cystic

Fibrosis-Related Diabetes (CFRD).

– CFRD is a unique type of diabetes.

– It is extremely common in people with CF especially

as they get older.

– CFRD is found in 35% of adults aged 20-29 and 43%

for those over 30 years old.

CFRD

CFRD has features of both type 1 and type 2

Diabetes. – Like type 1 they have insulin deficiency related to scarring of

the pancreas.

– Like type 2 they have insulin resistance related to

inflammation, increased cortisol levels and chronic use of

steroids.

– Like Type 1 they can be treated only with insulin but they never

have elevated ketone levels

– Unlike type 2 they do not have weight loss, other macro

vascular complications of diabetes like heart disease a

hypertension

CFRD

Diagnosis of CFRD – Oral Glucose Tolerance tests- fasting >126mg/dL or 2hr PP is

200mg/dL or more

– Fasting glucose levels- >126mg/dL

– Random blood glucose levels- > 200mg/dL-need to follow with

an OGTT

– Hemoglobin A 1c (HbA1c) -> 6.5% + OGTT

CFRD

• Management of CFRD – Insulin, insulin, insulin

– Regular blood sugar testing

– High calorie/high protein diet

– Use of high calorie oral

supplements

Bone Health

• Causes and risk factors of low bone density – Malabsorption

– Low body weight

– Lack of exercise

– Low vitamin D/calcium intake

– Chronic infection

– Long term steroid use

• Recommendations – Baseline DEXA scan at 10 yrs

– Screening every 1-2 yrs thereafter pending results

– Diet education – Vitamin D and calcium

– Supplements

– Weight bearing exercise

– Referral to Endocrine for Bisphosphonate Therapy

Nutrition Goals for CF

Optimal weight gain and linear growth velocities for

age

The CF Foundation recommends: Weight/length > 50%ile

BMI/age > 50%ile and < 85%ile

BMI > 22 kg/m2 for adult women

BMI > 23 kg/m2 for adult men

Research has shown best long term maintenance of lung

function (FEV1) with these BMI goals

Cystic Fibrosis Patient Registry Annual Data Report 2010. Cystic Fibrosis Foundation.

FEV1 vs. BMI in CF

Nutrition Assessment

• Research has shown that estimated nutrient needs for children

with CF are 110-200% higher than the normal, healthy population

• Energy needs:

– Pancreatic insufficiency: 110-130+ kcal/kg (infants) or DRI x 1.5-2.0 (3yrs+)

– Pancreatic sufficiency: 90-110+ kcal/kg (infants) or DRI x 1.2-1.5 (3yrs+)

• Protein: 2-3 gm/kg (infants) or 1.5-2.0 gm/kg (2 yrs+)

• Fluid: Holliday Segar equation + extra fluid during physical

exertion

Source: Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, Perspectives in Practice: Evidence-based practice

recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: Results of a

systematic review. Journal of the American Dietetic Association. 2008; 108:832-839.

Annual Nutrition Labs

• Fat soluble vitamins – A, D, E, K

• Retinol, 25OH D, Alpha-tocopherol, and PT

• Minerals – Iron panel, zinc

• Screening for CF related Diabetes – Oral Glucose Tolerance Test

• 2 hour result: Normal <149 mg/dl, IGT 150-199 mg/dl, CFRD > 200 mg/dl

– HgbA1c

Diet Interventions

• High calorie

• High protein

• High salt

• High fiber

• Provide with daily calorie goal

• Education on consistent use of calorie boosters

• Recipes for high calorie shakes

• Hydration and salt supplementation

• 3 day food records

Salt Supplementation

• Infants – 0-6 months: 1/8 tsp added to formula or breast milk

daily • Advised to measure out at beginning of day and added to

feeds throughout the day

– 6-12 months: ¼ tsp added to formula or pureed foods daily

• Toddlers, Children and Adolescents – No salt restriction

– Salty snacks

– Added salt to meals

– 12 oz Gatorade or powerade + 1/8 tsp extra salt

Exocrine Pancreatic insufficiency

Enzymes

• Enteric coated:

– Coating prevents breakdown of enzymes before reaching small intestine • Creon

• Zenpep

• Pertzye

• Pancreaze

• Non-enteric coated:

– Should be given with PPI • Viokace

Enzymes

Pancreatic Enzyme Replacement Therapy (PERT)

• Based on units of lipase/kg/meal

• > 4 years of age: Begin with 500 units lipase/kg/meal

• May increase up to 2,500 units lipase/kg/meal or 10,000 lipase/kg/day

• Based on units of lipase/grams of fat eaten

• Infant formula or Breast milk: 2,000-4,000 units lipase/120mL

• Other solids and liquids: 500-4,000 units lipase/gm fat eaten

PERT

• Enzyme capsules are taken by mouth with every fat containing

meal, snack, drink and GT feed

• For GT feeds:

• If able to PO

• enzymes provided at beginning and end of GT feeds (and

occasionally mid feed)

• If NPO

• Whole beads may be bolused through GT in nectar thick

medium IF GT is a 16-18 french size

• Beads may be crushed and added to open feeding bag

system

• Beads may be dissolved in bicarbonate solution and

provided Q 3-4 hours during feeds

PERT Dosage Calculation

Calculate the enzyme dosage required for a

6 year old who weighs 25kg and is on Creon

12000

PERT Calculation

Meal dosing: 500-2500 U lipase per kg per meal

1500 U lipase per kg x

25 patient weight in kg

= 37500 total U lipase per meal / u lipase/enzyme

= 3.1 # enzyme capsules per meal

We would round that off to 3 capsules per meal

PERT Calculation

Snack dosing: ½ meal dose per snack

750 U lipase per kg x

25 patient weight in kg

= 18750 total U lipase per meal / u lipase/enzyme

= 1.5 # enzyme capsules per meal

We would round that off to 1-2 capsules per snack depending on the kind of snack

PERT Calculation

Oral Supplement Dosing: 1,000-4,000 U lipase per g of fat

Pediasure – 9gm of fat per bottle/pack

9 total gm of fat from formula

x 2000 U lipase per gm of fat

= 18000 U lipase per total formula intake

/ 12000 U lipase/enzyme

= 1.5 # of enzyme capsules to cover the formula

We would round that off to 2 capsules per can of formula.

(Multiply 12000 X 2= 24000 /9gm of fat= 2666 U lipase/gm of fat)

PERT calculation

Average daily dose: less than 10,000 U lipase per

kg (weight) per day

3 # enzymes per meal x 3 meals per day

+ 1 # enzymes per snack x 2 # snacks per day

+ 4 # enzymes supplement

= 15 total number enzymes per day

x 12000 U lipase per capsule

= 180000 U lipase per day / kg (weight)

= 7200 U lipase per kg per day

Stool Assessment

• Frequency

• Consistency

• Loose vs. solid

• Volume

• Buoyancy

• Color

• Pale/Yellow/orange =

malabsorption

• Presence of grease

• Smell

• Foul smelling =

malabsorption

Vitamins

• Intake dosage of fat soluble vitamins is larger due to malabsorption

• Fat soluble vitamins have to be provided in a water miscible form

• Several companies make vitamins specially meant for use in conditions where malabsorption occurs

Vitamins

Vitamin dosage is usually based on Annual labs

and repeat labs Typical dosage

– Liquid MVI- 0.5-2ml/day

– Chewable- 1-4/d

– Softgels – 1-2/d

Vitamins

• AquADEK

• MVW Complete Formulation

• DEKAs

• Libertas

• Choiceful

• May be taken with or between

meals

• Examples: Boost Kid

Essentials, Pediasure, Boost,

Ensure, Scandishake,

Carnation Instant Breakfast

• Must be taken with enzymes

• May provide 240 – 580

additional calories per serving

Oral Nutritional Supplements

Appetite Stimulants

• Cyproheptadine (Periactin) – Oral antihistamine with beneficial “side effect” of appetite

stimulation

– Other side effects: drowsiness

• Dronabinol (Marinol) – Active ingredient: Synthetic delta-9-THC

– Controlled substance

– Possible side effects: dizziness, euphoria, somnolence

• Megestrol acetate (Megace) – Corticosteroid, very effective appetite stimulant

– Abdominal adiposity

– Bone Density

Tube Feeding

• Gastrostomy Tube (G-

tube)

• Tube inserted into abdomen to

deliver nutrition directly to the

stomach

• Feeds are provided either as

boluses during the day or more

commonly, supplemental

overnight feeds

• Enzymes must be provided with

GT feeds

Relizorb Cartridge

• Lipase containing cartridge hydrolyzes fat in formula during

administration

• Potential Benefits: • Improved weight gain and growth, improvement in BMI/age

• Improved tolerance of GT feeds

• Decreased incidence of nausea, vomiting, bloating and fullness after

feeds

• Many patients report ability to eat breakfast in the morning

• Improved adherence to GT feeds

When to consider GT Placement…

– Downward trend in BMI/age and height/age

– Eating a high calorie diet +oral supplements to meet

caloric goals but still no improvement in BMI/age

– Unable or unwilling to eat enough to meet caloric

goals

– Stress around meal times affecting relationships

– Ability and willingness of the family to care for the

GT

– Transplant

Practice Case Study

• Medical History – 6yo female, African American descent

– Diagnosed at 2.5 months old, presented with syncope and

cyanosis

• Sweat Cl: 99mEq/L

– EPI

• Fecal elastase: <15mg/g

– Rectal prolaspe repair in 2013, recurrence in 2015

– GT placement in 2014

– 1 admission for abd pain and constipation

• Current diagnoses: CF, PI, MSSA+, GERD, chronic

constipation, vitamin D deficiency, chronic rhinitis, poor

weight gain

Practice Case Study

• Nutritional Assessment - Anthropometrics

Practice Case Study

• Nutritional Assessment – Growth Charts

Practice Case Study

Practice Case Study

• Nutritional Assessment - Labs

Practice Case Study

• Nutritional Assessment - Medications

Practice Case Study

• Nutritional Assessment – Estimated needs (wt: 17.9kg)

– Energy: 1637-2183 kcal/d (DRI x 1.5-2.0)

– Protein: ~27 g/d (1.5 gm/kg)

– Fluid: 1395+ml (Holliday Segar method)

• Intake

– Overnight feeds: 4 cans Peptamen Jr 1.5 @ 95 ml/hr via GT

• 1500kcal, 45gm PRO, 68gm FAT, 772ml

– PO supplements: 1-2 cans PediaSure

• 240 kcal/can, 7gm PRO, 9gm FAT, 200 ml fluid

– Eats 2-3 meals per day + 2-3 snacks, sometimes skips breakfast

– Preferred foods: • spaghetti, pizza, lasagna, tacos, beans, rice, chicken potpie, macaroni and cheese, mashed potatoes, sweet potatoes, tomatoes,

green beans, okra, corn, carrots, bell peppers, onions, strawberries, grapes, watermelon, and bananas

– 4-6 cups of other fluids (water, juice, Gatorade, milk)

• Stool Assessment

– 1-2 BMs, soft, sinking

• Enzymes: Creon 12,000

• Wt: 17.9kg – 3 capsules with meals

• 2011 u lipase/kg/meal

– 2 capsules with snacks • 1340 u lipase/kg/snack

– 2 capsules with 1 PediaSure (9gm fat) • 2667 u lipase/gm fat

– 6 capsules at beginning and 6 capsules at the end with GT feeds of 4 cans of Peptamen Jr 1.5 (68 gm fat) • 2117 u lipase/gm fat

– **6 capsules at beginning of GT feeds + 1 Relizorb cartridge

Practice Case Study

References

• Lüth S, Teyssen S, Forssmann K, Kölbel C, Krummenauer F, Singer MV. Fecal elastase-1

determination: 'gold standard' of indirect pancreatic function tests? Scand J

Gastroenterol. 2001 Oct;36(10):1092-9.

• Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, 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. Diabetes Care. 2010; Dec; 33(12):

2697-2708.

• Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, Perspectives in Practice:

Evidence-based practice recommendations for nutrition-related management of children and

adults with cystic fibrosis and pancreatic insufficiency: Results of a systematic review. Journal

of the American Dietetic Association. 2008; 108:832-839.

• S.J. Schwarzenberg, S.E. Hempstead, C.M. McDonald, S.W. Powers, J. Wooldridge, S. Blair, et

al. Enteral tube feeding for individuals with cystic fibrosis: cystic fibrosis foundation evidence-

informed guidelines. J Cyst Fibros, 15 (2016), pp. 724-735

• D. Turck, C.P. Braegger, C. Colombo, D. Declercq, A. Morton, R. Pancheva, et al. ESPEN-

ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic

fibrosis. Clin Nutr, 35 (3) (Jun 2016), pp. 557-577

• www.CFF.org

Questions?