Cystic Fibrosis
Patient: Lily Johnson
Case study by Alexa Angelo
Lily Johnson
•
14 year old
Caucasian female
•
Diagnosed with
Cystic Fibrosis when
she was 6 months old
•
Very active girl and
participates in dance
class three times a
week and runs cross
country for her high
school (usually 5 to 7
miles a day, 3 to 4
days per week)
•
Hospitalized because
she caught a cold
that her medications
could not cure; may
have pneumonia
What is Cystic Fibrosis? (1)
 Caused by a mutation of the cystic fibrosis
transmembrane conductance regulator (CFTR)
 The abnormal mutation of CFTR causes thick viscous
mucous secretions that eventually lead to obstruction
of the glands and ducts of various organ systems,
including the respiratory tract, the gastrointestinal
tract, the liver, the genitourinary system, and the
sweat glands.
• Severe
constipation
• Increased
gas
• Bloating
• Nausea or
loss of
appetite
• Stools that
contain fat
Respiratory
• Delayed
growth
• Failure to
gain weight
normally
• No bowel
movements
within first 48
hours of life
• Salty tasting
skin
Gastrointestinal
In newborns
Symptoms of Cystic Fibrosis (2)
• Coughing
• Increased
mucus in
lungs and
sinuses
• Fatigue
• Nasal
congestion
• Recurring
pneumonia
Diagnosis (2)
Tests include:
•
Sweat chloride test
•
Blood test to confirm mutation of CFTR gene
•
Sputum cultures to test for any infections typical to CF
•
Pancreatic function tests
•
Pulmonary function tests
•
DNA-based test to detect genetic variation that causes CF
Treatment for CF (2)
 Antibiotics
 Inhaled medicines
 DNAase enzyme therapy
 High concentration of salt solutions
 Flu and PPV vaccines annually
 Possible lung transplant
 Oxygen therapy
 Percussion nest, manual chest percussion, A-capella, and
TheraPEP device
Medical Nutrition Therapy (1)
 Lily has a very low BMI of 17, and is a very active child
 This shows her increase for energy requirements : RDA 110% to
150% for a steady weight gain
 Fat consumption is increased (35% to 40% total Kcal)
 Protein consumption (15% to 20% total Kcal)
 CHO needs to be distributed evenly throughout the day to
prevent CHO loading because CF patients are more likely to
develop Type 2 DM
 Administer Multivitamin with the following vitamin amounts:
Age
Vitamin A (IU)
Vitamin E (IU)
Vitamin D (IU)
Vitamin K (IU)
>8
10,000
200-400
400-800
0.3-0.5
Specific Macronutrient Goals
for Lily
 Total energy requirements: 3,000 kcal
 FAT: 3,000 x .40 = 1,200 kcal from fat
 1,200/9 = 133 g fat/day
 Protein: 3,000 x .15 = 450 kcal from protein
 450/ 4 = 113 g protein/day
 CHO: 3,000 x .45= 1,350 kcal/day
 1,350/4 = 338 g CHO/day
Overall Plan of Action
 Increase Lily’s energy intake by adding additional kcal in
the form of in-between meal snacks with nutrient-dense
foods particularly high in fats
 Fortified beverages or pudding may be beneficial
 Vitamin supplementation for the following nutrients:
Vitamins A, D, E and K, iron and zinc
 Monitor weight and lab values
 Make sure diet is high in salt, because CF patients are at risk
for hyponatremia
Prognosis (2)
 Most children with CF live fairly healthy lives until they
reach adulthood.
 The lung disease eventually worsens until they are
disabled
 A person with CF has an average life span to
approximately 37 years
 Death is usually caused by lung complications
References
 1. Nelms, M. N. (2011). Nutrition therapy and pathophysiology
(2nd ed.). Belmont, CA: Wadsworth, Cengage Learning.
 2. Cystic fibrosis - PubMed Health. National Center for
Biotechnology Information. Retrieved November 10, 2012, from
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/
 3.
http://www.medicine.virginia.edu/clinical/departments/medic
ine/divisions/digestive-health/nutrition-support-team/nutritionarticles/GoodinArticle.pdf
 4. http://www.mayoclinic.com/health/cysticfibrosis/DS00287/DSECTION=treatments-and-drugs
 5.
http://www.lung.org/assets/documents/publications/solddcchapters/cf.pdf
