Cystic Fibrosis

A Case Study of Cystic Fibrosis in an adolescent patient

Childrens National Medical Center Annie Gallagher University of Maryland College Park Dietetic Intern

Cystic Fibrosis
Autosomal recessive disorder Causes a defect in the chloride ion exchange resulting in impaired ion transport Resulting in thick mucous secretions throughout the body Most commonly affects
Lungs Pancreas

Most common genetic disorder in Caucasians

Cystic Fibrosis
Diagnosis
Sweat Test
Infants to 6 months 40 59 mmol/L CF may be possible 60 mmol/L CF likely to be diagnosed

Pancreatic Insufficiency
Thick mucus secretions cause a decrease in pancreatic enzymes Diagnosis
Fecal ElastaseTest Ug Elastace/g stool
< 200 Insufficiency Pt needs Pancreatic Enzymes 200 500 Potential for Insufficiency >500 - Functioning Pancrease

Treatment
Supplement Pancreatic Enzymes Break down macronutrients for increased absorption and utilization

Clinical Indications
Chronic Pulmonary Function Malnutrition Pancreatic Insufficiency CF Related Diabetes Mellitus Intestinal Obstruction Syndrome Fat soluble vitamin deficiencies

Cierra
16 year old female with Cystic Fibrosis Neonatal Meconium Ileus Volvulus 2010 with subsequent bowel removal and short gut
Began G-Tube feeds

Exploratory Laparotomy 2/2014 revealed dilated loops Admitted 3/12/2014 due to declining PFTs

Initial Assessment
Food Intake 4 meals per day with 2-3 snacks Nightly G-Tube feeds
Peptamen Jr 30 cal/oz ,100 ml/hr, 9 pm 7 am Providing a total of 1000 ml, 20 kcal/kg, approximately 44% estimated needs

Stooling 2x/day Supplements

Miralax 17gm/ day mixed into applejuice AQUADEK 1 tablet 2x/day

Normal Intake
Tube feed turned off between 4 & 5 a.m. Breakfast - 9 a.m. - Peanut Butter and Jelly Sandwich with whole milk Lunch - 12 p.m. - French Fries, Chicken Sandwich with Ketchup Snack - 2:30 p.m. - Pasta with ground beef flavored with a soy/ teriyaki sauce with garlic and onions Snack - 4/5 p.m. - chips Dinner - 6-8 p.m. - whatever mom cooks - usually pasta, chicken, kale, shrimp avocados Snack - 9-10 p.m. - Barbeque Sauce Sandwich

Pancreatic Enzyme Regimen

Creon 24,000 24,000 lipase units per capsules 2 capsules to end tube feed 3 capsules with meals 2 capsules with snacks 6 capsules to begin tube feed

Providing 1437 units lipase/meal

Estimated Nutrition Needs

Kcals/kg

46 kcal/kg
WHO equation with an activity factor of 1.7 Grams Protein/kg

2.0 g/kg
15-20% of patient's total caloric mL/day to meet maintenance fluid needs

2100 ml/day, 42 ml/kg/d

Holiday Segar Equation

Nutrition Related Medications

Medication AQUADEK Miralax Vitamin D3 Lactobacillus Acidophillus & Bulgaricus Creon 24,000 1 tablet 17 gm powder 500 iu 2 tablets Units BID Daily Daily Daily When

24,000 lipase units per capsules

Albuterol Bubesonide Fluticasone Nasal

2.5 mg inhalant 0.5 mg inhalant 2 sprays

2 capsules to end tube feed 3 capsules with meals 2 capsules with snacks 6 capsules to begin tube feed QID QID Daily

Lab Values
Sodium Potassium Chloride Glucose BUN Calcium Phosphorous Magnesium Vitamin D Vitamin A Vitamin E 3/17 133 4.0 98 125 21 9.0 4.4 2.3 3/12 138 3.8 104 92 22 8.7 3.9 1.8 15 31 2.5 3/11 136 3.9 100 27 24 8.9 4.3 1.9 27 2.6

Growth
10th 25th Percentile

25th 50th Percentile

Growth

50th 75th Percentile

Nutrition Diagnosis
NI 1.2 Energy Expenditure Increased related to cystic fibrosis as evidenced by h/o pts po intake unable to meet nutrient needs. Nutrition Risk
Complex I

Cierras Supplements
AQUADEK chewable tablets
18,167 IU Vitamin A 800 IU Vitamin D 700 mcgVitamin K 12 mcg Vitamin B12 75 mcg Selenium 30 milligrams Vitamin E.

Scandishakes - 580 calories

220mg Na 389mg Cl, 369mg Ca 365mg Phos 55mg Mg 580mg K

Miralax provides polyethylene glycol

Diet Order
High Calorie, High Protein, High Fat Tube Feed: Peptamen Jr 30cal/oz, 100 mL/hr, 9 p.m. - 7 a.m. 20 kcal/kg Scandishakes BID

Goals
Pt will be able to meet caloric needs PO
Met by incorporating 100-200 kcal to meals and snacks

Decrease nightly feeds per patient request Continue follow-up in CF clinic

High Cal/ High Protein Diet Scandishakes Nightly TF (PRN) Creon 24,000: 3 caps with meals, 2 caps with snacks, 6 caps before tube feed, 2 caps after tube feed 1437 units lipase/meal Monitor Stools Monitor Vitamin A, D, E, K

References
The Academy of Nutrition and Dietetics. Nutrition Care Manual Cystic Fibrosis. 2013. Accessed 23 November 2013
Parsons H, Beaudry P, Dumas A, et al. Energy Needs and Growth in Children with Cystic Fibrosis. Journal of Pediatric Gastroenterology and Nutrition. 1983; 2: 44-49. Pedreira CC, Robert RGD, Dalton V, et al. Association of Body Composition and Lung Function in Children with Cystic Fibrosis. Pediatric Pulmonology. 2005; 39: 276-280. Cystic Fibrosis Foundation. 2010. Accessed 18 March 2014. < http://www.cff.org/> Gifford, Heather. Nutrition Management of the Cystic Fibrosis Patient. The Academy of Nutrition and Dietetics. 2009.