FAMILY CASE STUDY

PRESENTATION
By: Kate Morton
MEET MY CASE STUDY PATIENT
10 years old
Active soccer &
basketball player
Alabama fan
Big AND little
brother
Cystic Fibrosis
patient
ROLL TIDE
 ANTHROPOMETRICS
Ht: 138cm 38th%ile ( z- score: -0.39)
Wt: 34.9kg 59th%ile (z score: 0.22)
BMI: 18.2kg/m2 72nd%ile (z score: 0.60)
ESTIMATED CALORIE NEEDS

Daily Recommended Intake (DRI) x Highest Physical Activity

Level =

2800 kcals/ day

Or
Estimated Energy Requirements(EER) x 1.2-2 ( CF Needs) =

1,777 3,000
WHY SO MANY CALORIES?

Increased work of breathing = Increased energy

needs
Increased malabsorption
Increased energy nutrients help guard against
infection
GROWTH CHART
MEDICAL HISTORY
Cystic Fibrosis
CF Hepatic Disease
Asthma
Sinusitis (chronic)
Meconium Ileus
Portal Hypertension
Small Bowel Obstruction
Short Gut due to bowel resection
CYSTIC FIBROSIS - PRIMARY
What is it?
A genetic disorder that primarily affects the
exocrine glands

What does that mean?

The epithelial cells produce a thick mucus that
leads to blockage of pancreatic ducts in the
intestines and bronchi.
MECONIUM ILEUS
What is it?
A bowel blockage in the intestine of newborns

Is this related to CF?

YES
18% of children with CF have Meconium Ileus
98% of full term infants who have Meconium
Ileus have CF

("Diagnosis: Presentations: Meconium Ileus")

CF HEPATIC DISEASE - CFLD
30% of patients with CF well develop CFLD
The small bile ducts of the liver become
damaged first
Leading to further damage and destruction of
the liver

(Kobelska-Dubiel, Klincewicz, & Cichy, 2014)

PORTAL HYPERTENSION
8% of children who have CFLD will develop
portal hypertension

("Liver cirrhosis and portal hypertension in cystic

fibrosis.")
 CURRENT MEDICATIONS
Medication Dosage / Instructions Reason
Pulmozyme 1 mg/ml Sol, Nebulize 1 vial daily improve lung function in people with cystic
fibrosis by thinning pulmonary secretions and
reducing the risk of respiratory tract infections.
Albuterol Sulfate 2.5 mg/ml Nebulize 1 vial every 4 Asthma
hours as needed
Ventolin Inhale 2 puffs every 4 hours as bronchospasms / asthma
needed

Singulair 5 mg chew rake one by mouth at allergies

night

Vitamin D 1000 tabs, take 2 by mouth Decreased absorption of fat soluable vitamins
(cholecalciferol) everyday due to CF

Azithromycin 250mg tabs take 1 by mouth MWF Antibiotic

Gentamicin 40mg/ml, Nebulize vial 2x daily Antibiotic

every other month

Proair Inhale 4 puffs every 4 hours Treatment and prevention of bronchospasms

Advair 2 puffs inhaled 2x per day with Asthma treatment

aero chamber
NUTRITION RELATED MEDICATIONS
Medication Instructions Reason

Vitamin D (Cholecalciferol) take 2 by mouth every day Decreased absorption of fat soluble
1000 unit tabs vitamins due to CF

Creon 24000 unit Give 3 capsules with meals and pancreatic insufficiency
2 with snacks

Actigall 300mg capsule take 1 capsule by mouth 2x A bile acid naturally found in the human
daily body that helps prevent gallstones

Miralax Powder Mix 1 capful with 8oz of juice or Treatment of constipation/ stool softener
water daily

Prevacid 15 mg Take 1 tablet by mouth daily Antacid to combat GERD

Aquadeks Take 1 capsule by mouth daily Cystic Fibrosis multivitamin that helps
replace fat soluble vitamins that may be
mal absorbed due to CF

Pediasure Liquid Drink 8oz 3x/ day Nutritional supplement to support healthy
weight gain
VIDEO ON LIVING WITH CF
CULTURAL HISTORY
Both Mom & Dad were born and raised in Israel
They moved to the US together 30 years ago
The family is catholic and attends church
regularly
All 5 children attend catholic school
HOW CULTURE IMPACTS CARE
Over all the family chooses to strictly follow
doctors orders to air on the side of caution
 Benefits:

CULTURAL HISTORY
Helps with
gas
Increases
appetite
Helps with
asthma
symptoms
FROM A PARENTS POINT OF VIEW
INSURANCE & ASSISTANCE
CRS
Blue Cross
Point of Sale coverage
DISASTER PLAN EXAMPLE TORNADO
How will Therapies be done?
How will medication be delivered?
How will nutritional needs be met?
Where will the family safe place be?
WORKS CITED
Diagnosis: Presentations: Meconium Illeus. (n.d.). Retrieved December
05, 2016, from http://www.hopkinscf.org/what-is-
cf/diagnosis/presentations/meconium-illeus/

Kobelska-Dubiel, N., Klincewicz, B., & Cichy, W. (2014). Liver disease in

cystic fibrosis. Retrieved December 05, 2016, from
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4110359/

Liver cirrhosis and portal hypertension in cystic fibrosis. (n.d.). Retrieved

December 05, 2016, from https://www.ncbi.nlm.nih.gov/pubmed/14501614