MEDICAL

NUTRITION
THERAPY FOR
CYSTIC FIBROSIS
By Christa Justice, Dietetic Intern
 CF pop quiz

What is Cystic Fibrosis? (CF)

History, Symptoms, Diagnosis, Treatment

Medical Nutrition Therapy for CF

Managing Cystic Fibrosis Related Diabetes (CFRD)

OUTLINE Nutrition Care Process for CF Patients
• Nutrition Diagnosis
• Nutiriton Prescription
• Nutrition Intervention
• Monitoring/Evaluation
• Follow-up
Current CF Research Review

Conclusion
 Describe the development of Cystic
Fibrosis and Available Treatments.

Discuss the Medical Nutrition

OBJECTIV Therapy for Cystic Fibrosis.

ES Explain the Utilization of Pancreatic

Enzymes and Other Supplements.

Describe the Role of the RD with

Cystic Fibrosis.
 CF POP QUIZ!
1. Cystic Fibrosis is 2. Cystic Fibrosis is 3. The recommended
caused by? treated by? diet for CF patients
A. A defective gene A. Taking necessary A. High calorie diet
that causes vitamins B. Low fat diet
abnormalities in the B. Airway clearance C. High calorie, high
brain techniques that aim fat diet
B. A defective gene to loosen thick D. High fat diet
that leads to the mucus from the
production of an lungs
abnormal protein C. Taking pancreatic
C. Eating too much enzymes to help
salt digest food
 CF POP QUIZ!
1. Cystic Fibrosis is 2. Cystic Fibrosis is 3. The recommended
caused by? treated by? diet for CF patients
A. A defective gene A. Taking necessary A. High calorie diet
that causes vitamins B. Low fat diet
abnormalities in the B. Airway clearance C. High calorie, high
brain techniques that aim fat diet
B. A defective gene to loosen thick D. High fat diet
that leads to the mucus from the
production of an lungs
abnormal protein C. Taking pancreatic
C. Eating too much enzymes to help
salt digest food
 CF POP QUIZ!
1. Cystic Fibrosis is 2. Cystic Fibrosis is 3. The recommended
caused by? treated by? diet for CF patients
A. A defective gene A. Taking necessary A. High calorie diet
that causes vitamins B. Low fat diet
abnormalities in the B. Airway clearance C. High calorie, high
brain techniques that aim fat diet
B. A defective gene to loosen thick D. High fat diet
that leads to the mucus from the
production of an lungs
abnormal protein C. Taking pancreatic
C. Eating too much enzymes to help
salt digest food
 CF POP QUIZ!
1. Cystic Fibrosis is 2. Cystic Fibrosis is 3. The recommended
caused by? treated by? diet for CF patients
A. A defective gene A. Taking necessary A. High calorie diet
that causes vitamins B. Low fat diet
abnormalities in the B. Airway clearance C. High calorie, high
brain techniques that aim fat diet
B. A defective gene to loosen thick D. High fat diet
that leads to the mucus from the
production of an lungs
abnormal protein C. Taking pancreatic
C. Eating too much enzymes to help
salt digest food
 WHAT IS CYSTIC
FIBROSIS?
A progressive genetic disease that produces thick, sticky mucus in multiple organs
including the lungs and pancreas.(1)
Mutations to the cystic fibrosis transmembrane conductance regulator (CFTR)
[gene].1)
More than 1,700 CF causing genetic mutations identified.(2)
Quora.com
The genetic mutations cause chloride to become trapped in the epithelial cells.(2)
Mucus clogs the airways and trap germs and bacteria  infection, inflammation,
respiratory failure among other complications, increased mortality, and decline in
lung function.(2)
Most CF patients are classified as pancreatic insufficient (EPI) ~90%
https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/multimedia/cystic-fibrosis/img-20005623
 HISTORY & PREVALENCE
In 1938, Dr. Dorothy Anderson, first described the disease as “Cystic Fibrosis of the Pancreas”. (3)

1962: the predicted median survival age for CF reaches 10 years.

1970: age 16  1980: age 18  1989: age 29  2000: age 32  2020: 40-50s oldest person > 70 years
old.(3)

Historic breakthrough in 2019, the FDA approves the first triple-combination therapy,
elexacaftor/tazacaftor/ivacaftor (Trikafta™), CF patients over 12 y.o. with at least one copy of the F508del
mutation. ~90% of people with CF. Highly effective treatment for the underlying cause of the disease.(3)

More than 30,000 Americans people are living with CF (> 70,000 worldwide)(3)
SYMPTOMS OF CF 3

Persistent Frequent lung

Very salty-taste coughing (at infections Shortness of
skin times with (pneumonia or breath
phlegm) bronchitis)

Poor Frequent greasy,

growth/weight bulky stools or
Wheezing Anemia
gain (despite difficulty with
good appetite) BMs
HOW IS CF DIAGNOSED? 3
Multistep process:
Newborn screening
Sweat test (gold standard)
Genetic or carrier test
Clinical evaluation at a CF Foundation
accredited care canter
Most people are diagnosed with CF
by age 2, however, some are
diagnosed as adults
CF TREATMENT 3
Treatment plan – Requires a multidisciplinary approach, CF care team
Airway clearance techniques (ACTs)
Medications: CFTR Modulator Therapy, Nebulizers, Mucus Thinners, Antibiotics
Modulators must be taken with high fat foods (about 12 g fat)
Lung Transplant
Clinical Trials
Nutritional implications in CF: Enteric-coated pancreatic enzyme replacement therapy
(PERT), Multivitamins, EN as needed, ONS, high calorie/protein/fat diet to maintain healthy
BMI, prevent malabsorption.
Trikafta - F508del mutation
LUNG FUNCTION TEST (FEV1)
8
A spirometry test measures pulmonary
function.
Used to diagnose, detect infections, and
monitor
Breathe into a small device (spirometer) which
estimates the speeds and volume that is being
moved in and out of the lungs.
When airways are obstructed by thick mucus https://www.healthline.com/health/spirometry

produced in CF patients, it can reduce the speed

at which air can be exhaled, which reflects a
reduced forced expiratory volume (FEV in 1
second).
TRIKAFTA TRIALS 15

Trial 1: 24-week, randomized, double-blind, placebo-controlled trial, 403 patients

with one copy of the F508del mutation) – 13.8% increase in ppFEV1 from
baseline.
Trial 2: 4-week, randomized, double-blind, active-controlled trial, 107 patients
with two copies of the F508del mutations. – 10% increase in ppFEV1 from
baseline.
Other beneficial outcomes: improved sweat chloride, reduced pulmonary
exacerbations, improved BMI, safe for use.
Improved lung function, improved nutritional status, weight gain (now seeing
overweight CF patients), less need for transplant, reduced hospital
admissions/clinic.
MEDICAL NUTRITION
THERAPY FOR CF 3
High-calorie, High-fat diet
5-6 small, frequent meals and snacks
Pancreatic Enzyme Replacement Therapy (PERT), MVI and
mineral supplement
No dietary restrictions (special CF menu)
ONS with meals, not meal replacement
Education and counseling on a well-balanced diet and ways to
increase calories
Increased salt needs
15
DAILY ENERGY NEEDS
FOR CF 3
Estimated energy and protein needs are typically 1.5 to 2 times the
DRI for age compared to a non-CF individual.
Increased resting energy expenditure
Lung damage, increased work of breathing  increased calorie
needs
Fat requirements: 40% of calories
Protein requirements: 15-20% of calories
Nutrition goal: sustained weight gain or growth/maintenance
CF Foundation: Maintenance of BMI for women: at least 22 kg/m2,
for men: at least 23 kg/m2
Higher weight (BMI) = higher lung function (FEV1)
NUTRITION IN CF 14
NUTRITION IN CF 14
CF SPECIFIC VITAMINS 14
CASE
STUDY
Ms. W is a 26 YOF,
80 lb at 5’0”, with
possible new
diagnosis of CF.
Ms. W presented to
the CF clinic on
2/26/20 with SOB on
3 liters of oxygen.
 Past
Medical Past
History: Surgical
Asthma History:
None
Anemia
PATIENT
Social
Nutrition
HISTORY
history:
screening
Never
tool: MST 2
smoker, no
– weight
alcohol use,
loss (unsure)
no drug use
NUTRITION
ASSESSMENT
1 confirmed gene (F508del), other gene unknown.
Lung function measured at 16% FEV1.
Lifelong issues with abdominal cramping and occasional oily
stools.
Significant wt loss over the past year.
UBW: 125 lb, indicating a 45 lb wt loss, (36%, severe)
Initiation of PERT, suggest 1 Creon 36,000 with meals and
snacks (provides 963 units of lipase/kg/meal). Pt reports
having trouble with pill swallowing.
Stool elastase ordered
NUTRITION
ASSESSMENT CONT.
Ensure Enlive
Diet hx: Decent appetite but early satiety. 3
small meals daily. Drinks soda or juice
constantly. (H/o elevated HgbA1c (6.3%)).
• Suggest mealtime blood glucose checks and repeat HgbA1c.
• Will consider OGTT as outpatient if appropriate.

Suggest MVI BID. Will collect vitamin levels

as OP and optimize supplementation as needed.
ANTHROPOMETRICS
Height: 1.524 m (5’)
Weight: 36.1 kg (80 lb 9.6 oz)
UBW: 125 lb (CBW is 64% of UBW)
Body mass index: 15.55 kg/m2
IBW: 45.5 kg
IBW for CF: 51 kg, CF Foundation
recommends BMI of 22 or greater for females
 • GI: Last BM 2/26
• Occasional loose stools
GI • Constant abdominal cramping

GI AND
SKIN
• Appears intact
Skin
Component Value
WBC 7.4
HGB 8.8 (L)
HCT 30.5 (L)
MCV 88.2
PLT
Glucose
184
73
LAB
BUN 9 VALUES
NA 141
No current results for: HgbA1c; 6.3%
K 4.1 on 2/29/19
Cl 101 No current results for: Iron, TIBC,
ferritin; iron 24 ug/dL (L), TIBC 421
CO2 35 (H)
ug/dL (WNL), Ferritin < 10 ng/mL (L)
Creatinine 0.58 on 2/25/19
Theragran (MVI) BID

PERT: Creon 36,000 units 1

capsule TID
MEDICATI
PRN: miralax, glycolax,
zofran
ONS

IV: none
 Etiology: Chronic Illness (CI)

Nutrient Intake CI: Mild, less than or equal to

75% of estimated needs for greater than or
MALNUTR equal to 5 days

ITION Weight Loss CI: Severe, (36%, 45 lbs)

EVIDENC (>20% over 1 year)

E Subcutaneous Fat Loss: Severe, dark circles

under eyes; arm – very little to no skinfold fat

Muscle Loss: Severe, temple – depression;

interosseous – depression; squared shoulders;
prominently protruding acromion process
 Fluid Accumulation: None

MALNUTR
ITION Reduced Grip Strength: Not
EVIDENC assessed
E Malnutrition Evaluation: Meets
criteria for SEVERE protein-
calorie malnutrition
ESTIMATE 2300-2800 calories/day
D (45-55 kcal/kg IBW 45.5 kg)
NUTRITIO
NAL
NEEDS 77-128 g protein/day
(1.5-2.5 g pro/kg IBW 45.5 kg)

Nutrition Prescription:
Regular diet-no restriction and
Ensure Enlive x1/day
 Diet Education Needs: Diet education
indicated and patient agreeable

Adequacy: Not meeting calorie needs or

protein needs

Tolerance: Tolerating PO

Nutrition Care Level: High per clinical

https://hopkinscf.org/clinical-care/nutrition/
judgement

Goal: Patient will meet 75-100% of

estimated needs by follow up
PES STATEMENTS

Impaired nutrient utilization

Increased protein and calorie
related to compromised GI
needs related to cystic fibrosis
function as evidenced by
with chronic lung infection and
pancreatic insufficiency and s/s
increased work of breathing and
of malabsorption; oily stool,
malabsorption as evidenced by
abdominal pain, met criteria for
CFF guidelines for care, 46 lb
severe protein calorie
wt loss in 1 year (36%, severe).
malnutrition.
INTERVENTIO
N
General/healthful diet
Commercial beverage (Ensure
Enlive)
MVI/mineral supplement therapy
Prescription medication (PERT)
Collaboration with other providers
Survival information and
recommended modifications
Total energy intake

Total protein intake

Modified diet MONITORI

Liquid supplement
NG/
EVALUATIO
Weight change
N
Ability to recall nutrition goals

Level of knowledge and self management

RD RECOMMENDATIONS FOR
PHYSICIAN/PLAN OF CARE
Continue CF therapeutic diet; unlimited ONS x1/day
portions
RD following per clinical nutrition protocol
Collect fecal elastase
Pt meets criteria for SEVERE protein
Initiate PERT: 1 Creon 36,000 with all meals calorie malnutrition
and snacks, provide 962 units of lipase/kg/meal
Instructed pt to open Creon capsule and place
beads on spoonful of applesauce (not pudding),
due to trouble swallowing pills
MVI BID
Bowel regimen: Miralax prn
Pre meal accuchecks/HgbA1c collection
Visited pt at bedside, surrounded by
multiple sputum cups. Noted stool
elastase collected and sent off. Pt
reports using Creon 36, 1 capsule
per meal.

Swallowing difficulty  placing

beads on applesauce as previously
FOLLOW
instructed, going well. Stools are
more solid. Encouraged use of
UP –
miralax as needed (prn) 2/28/20
Edu  Reviewed physiology of
enzymes, pancreatic function, and
GI symptoms. Pt asked appropriate
questions and was engaged.
FOLLOW UP – 2/28/20 CONT.
No longer experiencing abdominal cramping after meals. No BM yet today,
encouraged patient to request miralax this evening if no BM later.
Increased appetite and feeling very hungry. Arranged TID snacks (small meals).
Tolerating Ensure Enlive with meals.
Conferred with pulmonary team, awaiting sputum cultures to optimize abx
regimen. Spoke with CF Pharmacist to arrange bedside availability of Creon and
update MAR.
Blood glucose < 200, no intervention indicated at this time.
• Will complete OGTT when well as OP F/U.
Goal: Progressing
Learner(s): patient

Barriers: new possible dx, other new meds

Cultural/Language Modifications: none NUTRITIO

Readiness: planning, action
N
EDUCATIO
Method: verbal discussion N
Topics: CF diet, PERT, GI symptom management, high fat,
high protein, high calorie diet

Understand/Compliance: fair to good compliance

anticipated – states understanding, will need reinforcement
Pt seen with MD during CF team
interdisciplinary rounds. Appetite at
baseline. Taking Creon 1 cap/meal but
has not noticed much of a difference.

At home, eats mostly fast food/a lot of

fried food followed by cramping,
sometimes greasy stool. Currently
FOLLOW
eating lower fat foods available at
hospital.
UP – 3/3/20
Discussed how PERT may help with
digestion, weight gain, as well as
reduce cramping. Encouraged to finish
remainder of Creon at home/continue
current regimen until fecal elastase test
is resulted (still pending).
3 stools in <24 hours, looser than normal Pt biggest
concern right now is how to get off continuous
oxygen.

Reiterated MD’s recommendations for a sweat test

followed by genetic sequencing for definitive
diagnosis of CF. FOLLOW
Encouraged PO intake of high fat, high protein foods.
Reviewed CF menu to find healthy, acceptable choices.
UP – 3/3/20
Discussed that optimizing nutrition status will help with
stabilizing overall health.
CONT.
Encouraged close F/U in CF clinic and to reach out
with any questions via OP MyChart. Will continue to
follow in OP clinic.
CURREN
T CF
CLINICA
L
RESEAR
CH
REVIEW
 TANGPRICHA ET AL.
VITAMIN D FOR THE IMMUNE SYSTEM
IN CF 7
Multicenter double-blind, randomized, placebo-controlled clinical trial, assessed the
risk for pulmonary exacerbations of CF associated with Vitamin D deficiency.

Single high-dose bolus vitamin D3 – 250,000 IU and placebo within 72 hours of hospital
admit, maintenance treatment – 50,000 IU every other week start after 3 mo. of random
assignment. Given to 91 adult participants with CF during pulmonary exacerbation.

Early studies suggest a potential benefit for lung function, innate immunity, and
reduced inflammation with vitamin D.
TANGPRICHA ET AL. RESULTS
7

Vitamin D3 supplementation given at start of

pulmonary exacerbation did not alter the time till the
next pulmonary exacerbation or mortality after 1 year.
• No effect on lung function or plasma cathelicidin concentrations.

This study did not support Vitamin D3

supplementation for treatment in acute pulmonary
exacerbation of CF.
SIMONEAU ET AL.
VITAMIN D2 OR D3 SUPPLEMENT? 9

Non-blinded randomized controlled 8-week trial.

Compared two different vitamin D replacement methods for CF

patients ages 6-21 who were vitamin D deficient or insufficient.

Treatment groups: 50,000 IU ergocalciferol (D2, oil) twice

weekly OR 50,000 IU cholecalciferol (D3, powder) weekly.
SIMONEAU ET AL. RESULTS 9

No significant
difference in markers:
BMI, FEV1, IgG, IgE,
Significant reduction and CRP (vitamin D3
in PTH with D3 group trended toward greater
– possible benefit to increase in FEV1 and
No difference between bone health. reduction in IgE.)
outcomes – similar
mean increase in
25(OH)D. 2/3 patients
achieved vitamin D
sufficiency.
Overall, either regimen is effective. However, vitamin D3 is more readily absorbed in that the same
results were seen with double the dose of vitamin D2.
STARK ET AL. WEB-BASED
INTERVENTION 10
Usability and Pilot trial (BeInCharge.org (BIC))
10 –week behavior plus nutrition intervention

Barriers to face-to-face
interventions: cost, time, distance
from providers
10 mothers of children with CF ages 4-9 yr.
• Children with CF ages 2-20 years should maintain a BMI > 50th
percentile
• More than half fail to meet this recommendation.
STARK ET AL. RESULTS 10

Average 0.67 kg weight gain from baseline

Increased caloric intake (clinically Children receiving BIC able to
significant) achieve caloric goal of 140% EER.

Improved access to nutrition intervention and web-based

behavior through BIC appears to be helpful by increasing
calorie intake and sustained weight gain.
MANAGING CF RELATED
DIABETES (CFRD) 5
Similar to both type 1 and type 2 diabetes
Symptoms: possibly asymptomatic, polyuria, polydipsia, tiredness, weight
loss, unable to gain weight, and loss of lung function
Diagnosis of CFRD:
• FBG of over > 126 mg/dL
• OGTT 2 –hour glucose > 200 mg/dL
• Random BG > 200 mg/dL.

Hemoglobin A1c not recommended - often falsely low. OGTT is

recommended for further testing accuracy.
Treatment: insulin therapy, check BGs 3x/day, oral diabetes agents not
recommended.
TERLIESNER ET AL. 11
CFRD  GROWTH FAILURE AND DECLINE IN
LUNG FUNCTION
Retrospective case-control study from January 1990 to
December 2014

32 CF patients with or without CFRD

Worsening glucose metabolism  increased bacterial

growth in lungs  airway infections  reduces lung
function and life expectancy
TERLIESNER ET AL. RESULTS &
CONCLUSION 11

Reduced growth/weight gain before and after diabetes diagnosis

(decline in height) in children and adolescents.

Early preventative actions in order to delay onset of diabetes 

monitor growth and weight.

CFRD development  decline in lung function (FVC and FEV1).

ENTERAL FEEDING FOR CF 12
NG tube or G-tube
Typically nocturnal feedings via pump to maintain quality of life
Calorie-dense formulas
PERT
Typically taken orally prior to feeding
RELiZORB

NG-tube complications: dislodged with coughing, nasal irritation or clog

G-tube complications: post-insertional pain, leakage or infection
Overnight feeding may cause GERD or vomiting
EN feeding can be highly effective in improving weight gain/growth, respiratory
function, and overall well-being of a person with CF
SHIMMIN ET AL. ENTERAL
FEEDING FOR CF 12
Evidence-based research support EN feeding excludes RCTs despite being
commonly used as treatment and nutrition intervention in CF clinics.

Excluded studies (44 total) from this review reported beneficial effects
of EN including increased energy intake, significant weight gain and
growth.

Reduced infections/hospital admissions, slowed lung function decline or

improved lung function, increased physical activity, well-tolerated with
few adverse effects (mild N/V/D/abdominal pain/GERD)
STEVENS ET AL. RELIZORB
ABSORPTION AND SAFETY 13
Multicenter, 90-day open-label study, 36 subjects
(median age 13.8, BMI 17.7, 6.2 yrs use of overnight EN)

In-line digestive enzyme cartridge – hydrolyzes fat in enteral

formula “medical device”
 RELiZORB with overnight EN – may have long-term therapeutic benefits
 Change over time in RBC uptake of DHA and EPA, GI symptoms, Clinical
and anthropometric parameters.
STEVENS ET AL. RESULTS 13

Improved fat Increased Omega-3 FA levels

absorption  plasma level of (systemic marker of
increased RBC fat absorption) 
levels of DHA+EPA. No improve pulmonary,
DHA+EPA, unanticipated inflammatory
improved omega- adverse events. status, and
6/omega-3 ratio. anthropometric data
in CF patients.
 Table 2: Changes in DHA, EPA, DHA+EPA, and omega-6/omega-3 ratio in plasma
Baseline, n=36 Day 30, n=36 Day 60, n=36 Day 90, n=36
DHA, ug/mL 50.33 (34.1) 102.64 (39.3) 96.77 (36.4) 102.36 (36.3)
EPA, ug/mL 22.41 (21.1) 65.34 (45.9) 64.01 (49.6) 64.09 (41.5)
Total DHA+EPA, 72.73 (52.1) 167.99 (82.5) 160.78 (83.2) 166.46 (73.7)
ug/mL
Omega-6/omega- 11.52 (4.3) 5.31 (2.5) 5.63 (3.3) 5.23 (3.1)
3 ratio

REliZORB used for 90 days was associated with a lower ratio of omega-
6/omega-3  key marker of inflammation 13

Plasma concentrations of total DHA and EPA measured by ultra-high-performance liquid

chromatography
Total DHA+EPA calculated by adding concentrations of total DHA and EPA.
DHA = docosahexaenoic acid; EPA = eicosapentaenoic acid
 GI symptoms decreased in the RELiZORB group throughout the study.
 23 patients (59.0%) at baseline compared to 12 patients (30.8%), on day 90 post-
treatment period, reported any symptom 13
CONCLUSION
Emphasis on diet and nutrition – integral part of CF treatment and
management.
RDs play a huge role in preventing fat malabsorption with PERT,
preventing nutrient deficiencies, maintaining adequate body weight and
overall wellness.
Cystic Fibrosis Foundation is an excellent resource for keeping up to
date on the recent advancements in treatment methods and nutrition care
protocols. (15)
It is an exciting era for CF care with emerging CFTR modulator
therapies (Trikafta) showing promising benefits in majority of
individuals living with CF.
May present new challenges related to CF patients living longer and
becoming more susceptible to nutrition related conditions such as
CFRD, heart disease, obesity, etc. (15)
QUESTIONS?
Contact Info:
Christa Justice
Email: Justice.christa@yahoo.com
Cell: 281-881-3696
REFERENCES
1. Mary Rozga, PhD, RDN; Deepa Handu, PhD, RDN. Nutrition Care for Patients with Cystic Fibrosis:
An Evidence Analysis Center Scoping Review. Journal of the Academy of Nutrition and Dietetics 2019.
Accessed February 25, 2020.
2. Zachari Breeding, MS, RDN, LDN, FAND. Cystic Fibrosis Corner: Update on CFTR Modulator
Therapy. Volume 38-2. Accessed February 25, 2020.
3. https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/
4. https://www.pancan.org/facing-pancreatic-cancer/living-with-pancreatic-cancer/diet-and-nutrition/panc
reatic-enzymes/
5. Managing Cystic Fibrosis-Related Diabetes (CFRD). An Instruction Guide for Patients and Families.
6th Edition. Brunzell C., Hardin D., Kogler A., Moran A., Schindler T. 2015 Cystic Fibrosis Foundation.
6. Carr V., Breeding Z. An In-Depth Review of the Implications of Vitamin D in Cystic Fibrosis. Volume
37-2. MNPG. Accessed February 28, 2020.
7. Tangpricha V, Lukemire J, Chen Y, et al. Vitamin D for the Immune System in Cystic Fibrosis (DISC):
A double-blind, multicenter, randomized, placebo-controlled clinical trial. Am J Clin Nutr.
2019;109(3):554-565.
REFERENCES CONT.
8. https://cysticfibrosisnewstoday.com/cystic-fibrosis-diagnosis/spirometry-test/#
9. Simoneau T, Sawicki GS, Milliren CE, Feldman HA, Gordon CM. A randomized controlled trial of vitamin D replacement
strategies in pediatric CF patients. J Cyst Fibros. 2016;15(2):234-241.
10. Stark LJ, Opipari-Arrigan L, Filigno SS, et al. Web-based intervention for nutritional management in cystic fibrosis:
Development, usability, and pilot trial. J Pediatr Psychol. 2016;41(5):510-521.
11. Terliesner N, Vogel M, Steighardt A, et al. Cystic-fibrosis related-diabetes (CFRD) is preceded by and associated
with growth failure and deteriorating lung function. J Pediatr Endocrinol Metab. 2017;30(8):815-821.
doi:10.1515/jpem-2017-0005
12. Shimmin D, Lowdon J, Remmington T. Enteral tube feeding for cystic fibrosis. Cochrane Database Syst Rev. 2019;2019(7).
13. Stevens J, Wyatt C, Brown P, Patel D, Grujic D, Freedman SD. Absorption and safety with sustained use of RELiZORB
evaluation (ASSURE) study in patients with cystic fibrosis receiving enteral feeding. J Pediatr Gastroenterol Nutr.
2018;67(4):527-532.
14. Altman K, McDonald CM, Michel SH, Maguiness K. Nutrition in cystic fibrosis: From the past to the present and into the
future. Pediatr Pulmonol. 2019;54(S3):S56-S73. doi:10.1002/ppul.24521
15. https://www.fda.gov/drugs/drug-approvals-and-databases/drug-trials-snapshots-trikafta