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

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What is Cystic Fibrosis?

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.

Signs and Symptoms?

Symptoms of Cystic Fibrosis (2)In

new

born

s • Delayed growth

• Failure to gain weight normally

• No bowel movements within first 48 hours of life

• Salty tasting skin

Gast

roin

test

inal • Severe

constipation

• Increased gas

• Bloating• Nausea or

loss of appetite

• Stools that contain fat

Resp

irato

ry • 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

Possible Nutrition Therapy goals?

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/medicine/divisions/digestive-health/nutrition-support-team/nutrition-articles/GoodinArticle.pdf

4. http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=treatments-and-drugs

5. http://www.lung.org/assets/documents/publications/solddc-chapters/cf.pdf