SHEVBA BAMARNI, JENNIFER CRUMM, WHITNEY MILLER, AND CHELSI CARDOSO

Cystic Fibrosis

WHAT IS IT?

Cystic Fibrosis

What Causes Cystic Fibrosis?

A mutation of the Cystic fibrosis transmembrane conductance regulator (CFTR): CFTR is a cAMP activated ATP gated anion channel where anions flow down the gradient pushing negatively charged ions out of the cell into the mucous layer. This negative gradient attracts sodium to exit as well, therefore causing water to make mucous more fluid.

The defective CFTR gene causes inability to move salt and water in and out of the cells, and causes uncharacteristic mucous production and salty sweat. Because of the lack of water, the mucus is very thick and sticky.

Major Issues Major Issues SymptomsSymptoms

The thick mucus obstructs the airways and glands.

The thick mucus cannot be cleared by the cilia, which can trap bacteria. This side effect is what causes chronic infections which are so common with CF.

Salty tasting skinNormal appetite but poor growth and poor weight gain

Excess mucus production

Frequent chest infections coughing, and shortness of breath

InfertilityGI instability and malabsorption

Characteristics of CF

Genetic Component

CF is an autosomal recessive disorder affecting the CFTR gene in Chromosome 7.

This means that a person must obtain two defective copies, one gene from each parent.

Most commonly occurs in Caucasians. Those who carry a defected gene but do not suffer from the disease are protected from Cholera.

Nutritional Implications of CF

Pancreatic insufficiency causes CF patients to have poor digestion, poor absorption of fat and fat-soluble vitamins, and loss of bile and bile salts.

Additionally, constant infection and deteriorating health leads to increased increased caloric intake.caloric intake.

CF patients are also at risk of osteopenia and osteoporosis because of pancreatic insufficiency, malabsorption of calcium, phosphorus, magnesium, and vitamins D and K, and the use of corticosteroids.

Nutritional Implications Continued

Anorexia due to chronic pulmonary infections.

For women, malnourishment can cause infertility because of amenorrhea.

Insulin-Dependent diabetes (10% of adults with CF)

Cirrhosis due to blockage of liver ducts (4-5% of adults with CF)

PATIENT HISTORY

Our Patient: LilyLily

Lily Johnson

14 yr old FCaucasianChief Complaint:

“I just got back from working at a camp for the past two weeks. I caught a cold, and it has gotten worse. My regular treatments were not working, and my doctor says I probably have pneumonia.”

Patient History

Onset of disease: Diagnosed with cystic fibrosis at age 6 months Since diagnosis, rather uneventful disease course Hospitalized several times for respiratory infections but otherwise maintained with outpatient therapy

Seen in the CF clinic yearly at University Hospital but receives routing medical care from her local physician

Type of Tx: Uses high-frequency chest compression vest for 1 hour twice daily.

PMH: Last hospitalization over a year ago Participates in ballet and jazz and is a cross0country runner

Typically run 3-5 miles 5-6 times a week. Has dance class three times a week

Patient History Continued

Meds: Outpatient medications include Pancrease (1-3 caps after meals); Prevacid (20 mg daily); Humabid (1/2 tablet every 12 hours); multivitamin, Proventil PRN.

Smoker: NoFamily Hx: What/Who?

Type 2 DM/grandmother (maternal) CF/great aunt (paternal, deceased)

Physical Exam

General appearance: Flushed, in no acute distress Vitals: Temperature 99.1ºF, BP 114/60 mm Hg, HR 82 bpm, RR 18 bpm

Heart: Regular rate and rhythm, heart sounds normal HEENT:

Eyes: WNL; PERRLA, fundi without lesions Ears: Clear Nose: WNL Throat: Pharynx reddened with postnasal drainage

Genitalia: Normal Neurologic: Alert and oriented Extremities: No edema Skin: Skin pale without rash Chest/lungs: Decreased breath sounds, percussion hyperresonant, rhonchi and rales present

Abdomen: Bowel sounds present, nontender

Tx Plan

Activity: Bed restDiet: Regular as toleratedLab: CBC, RPR, Chem16: I & O every shift; routine vital signs

IVF D5 @ 50 mL/hrVancomycin 10 mg/kg IV q6hCXR- EPA/LAT.Sputum cultures and gram stains

Chemistry Lab Value

Chemistry Labs Hematology LabsNormal Values

Lily’s Values

Units

Albumin 3.5-5 3.9 g/dL

Transferrin

250-380 (women)

219 mg/dL

Sodium 136-145 142 mEq/L

Potassium

3.5-5.5 3.8 mEq/L

Chloride 95-105 105 mEq/L

Magnesium

1.8-3 1.6 mg/dL

HbA1C 3.9-5.2 6.3 %

Normal Values

Lily’s Values

Units

WBC 4.8-11.8 13 x 103/mm3

HGB 12-15 (women)

11.5 g/dL

HCT 37-47 (women)

33 %

Ferritin

20-120 (women)

19 mg/mL

Interpretation of Lab Values

Hospital Course

Lily was diagnosed with acute pneumonia confirmed by CXR and sputum culture. IV antibiotics were initiated. Nutrition consult was initiated to assess current nutritional status and to ensure adequacy of current nutritional intake.

Nutrition Assessment

Typical Daily Intake

Greasy foods give her diarrhea She has had nutrition therapy in the past Her mother and grandmother provide food for her Supposed to take a multivitamin every day but frequently misses

Breakfast: Rarely eats

Lunch: 3 tbsp extra crunchy peanut butter or 2 oz ham & 2 oz Swiss cheese sandwich, 2-3 oz chips, 1 orange or other piece of fruit, and water

Dinner: 5-6 oz of chicken, pork, or beef- usually grilled or baked, 1-2 c of raw vegetables on lettuce, ¼ c of ranch dressing, 1 c pasta, potatoes, or rice- usually with 1-2 tbsp margarine, and water (Dinner really depends on whether she eats at home or at a friend’s house.)

24 Hour Recall

Total Energy Intake: Consumed: 1532 kcals1532 kcals Needed ~3550 kcals

Fat: Consumed: 71 g or 71 g or 639 kcals639 kcals

Needed: 1240-1420 kcals

Protein: Consumed: 48 g or 48 g or 192 kcals192 kcals

Needed 530-710 kcals

Breakfast: nothing

Lunch : 2 oz hot dog on bun, 1 ½ c macaroni and cheese (Kraft box variety made with 2% milk)

Dinner: 5 oz Salisbury steak with ¼ c gravy, few bites of green beans, 1 roll with tbsp margarine, grape juice about 2 c

Anthropometric Information

Lily’s Height: 5 ft. 5 in. => 165.1 cmLily’s Weight: 102 lbs. => 46.4 kgLily’s UBW: 110-115 lbs. (3 months ago)

BMI = kg/m2 = 46.4/ (1.651)2 = 17.0

%UBW = % Wt Change = { (UBW-ABW)/UBW } x 100 = { (115-102)/115} x 100 = 11.3%

 Time           Significant           Severe  1 week              1-2%                  >2%  1 month              5%                    >5%  3 months          7.5%                   >7.5%  6 months           10%                    >10%

Energy Requirements

CF patient receiving enzyme therapy must consume 120-150% calories for a healthy child of the same age and sex

Typical daily energy requirement for a 14 year old, female based on DRI: 2,368 kcals2,368 kcals

Because Lily is very active with ballet and jazz classes and running 3-4 miles 5-6 times per week, she should consume 150% calories

Estimated energy requirement for Lily: 2,368 kcals x 1.5 = 3,552 3,552 kcalskcals

Macronutrient Requirements

Protein energy requirement for a CF patient is 15-20% of total kcals = 532.8 to 710.4 kcals532.8 to 710.4 kcals

Fat energy requirement for a CF patient is 35-40% of total kcals = 1243.2 to 1420.8 kcals1243.2 to 1420.8 kcals

Carbohydrate energy requirement for a CF patient is unrestricted so the remaining calories would come from carbohydrates and should be distributed throughout the day

Micronutrient Requirements

Lily is at risk for electrolyte imbalances.When sodium intake becomes inadequate, lethargy, vomiting, and dehydration may occur.

14 year old females require 1500 mg/day of both sodium and chlorine.

Recommendations for Daily Vitamin Supplementation for Adolescents (> 8 years) with Cystic Fibrosis:. Vitamin A: 10,000 IU Vitamin E: 200-400 IU Vitamin D: 400-800 IU Vitamin K: 0.3-0.5 IU

CF patients should also supplement iron and zinc

Medication Function of Medication

Nutritional Implications

Pancrease Helps to digest proteins, starches, & fats.

Needs to be taken so that she may better absorb nutrients.

Prilosec Helps alleviate heartburn.

Need to be taken to avoid heartburn while food is being digested.

Humabid Helps breakup phlegm and alleviate cough.

Needs to be taken to help decrease coughing due to excess phlegm.

Proventil Increases air flow to lungs

Needs to be taken after physical activity to increase air flow into the lungs.

multivitamin Supplies vitamins that aren’t consumed in diet.

Needs to be taken because she isn’t absorbing as many nutrients from foods as she should.

Pancrease Information

Pancrease is used to treat pancreatic enzyme deficiency and it taken to help with digestion of proteins, starches, and fats.

Children over the age of 4 have a recommended dose of 500 lipase units/kg per meal.

As children grow older, they require less lipase units per kilogram body weight because they tend to ingest less fat per kilogram and body weight.

Nutrition Therapy

Nutrition Care Process

Clinical Domain• Impaired nutrient utilization (NC-2.1)

• Altered nutrition-related laboratory values (NC-2.2)

• Food-medication interaction (NC-2.3)

• Underweight (NC-3.1)• Involuntary weight loss (NC-3.2)

Factors Impacting Success

Busy lifestyleLack of knowledge with pancreatic enzyme medication

PES Statement

Involuntary weight loss related to inadequate energy intake as evidenced by thirteen pounds of weight loss within three months. Goals: Cessation of weight loss and eventually promote weight gain.

Interventions: Consume 35%-40% of total kcals from fat per day; plan meals and snacks ahead of time; educate on appropriate doses of pancreatic enzyme medication (take with every meal and snack)

PES Statement

Increased iron needs related to inadequate iron intake as evidenced by transferrin (219 mg/dL), ferritin (19 mg/mL), hemoglobin (11.5 g/dL), and hematocrit (33 %) values below the normal range. Goals: Increase transferrin levels to be within 250-380 mg/dL, ferritin levels within 20-120 mg/mL, hemoglobin levels within 12-15 g/dL, and hematocrit levels within 37-47%

Intervention: Recommend an iron supplement (with a vitamin C source for better absorption); increase intake of high iron foods (beef, beef liver, lima beans, kidney beans, lentils, dark green vegetables, and enriched and fortified cereals)

Questions?