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Cystic Fibrosis MEDICAL NUTRITION THERAPY

Cystic Fibrosis MEDICAL NUTRITION THERAPY. Cystic Fibrosis Cystic Fibrosis (CF) is a chronic disease, associated with thick, sticky mucus forming in

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Page 1: Cystic Fibrosis MEDICAL NUTRITION THERAPY. Cystic Fibrosis  Cystic Fibrosis (CF) is a chronic disease, associated with thick, sticky mucus forming in

Cystic FibrosisMEDICAL NUTRITION THERAPY

Page 2: Cystic Fibrosis MEDICAL NUTRITION THERAPY. Cystic Fibrosis  Cystic Fibrosis (CF) is a chronic disease, associated with thick, sticky mucus forming in

Cystic Fibrosis

Cystic Fibrosis (CF) is a chronic disease, associated with thick, sticky mucus forming in the lungs, pancreas, and other organs. This mucus leads to clogging of major organs that is often life-threatening.

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Incidence

CF occurs in approximately 1:3500 live births.

White populations have between 2 and 5% of adults as CF carriers. This includes 10 million American adults. These individuals carry the defective gene associated with CF, however they do not experience symptoms. Consequently, most of these individuals do not know they are carriers.

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Prevalence

In the United States, there are approximately 30,000 individuals with CF.

The number of new cases each year is approximately 1000.

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Decrease in CF Cases

Studies have shown a decrease in the incidence of CF in association with increased newborn screenings for the gene.

One study examined the impact of CF carrier screening of individuals, and their relatives, undergoing in vitro fertilization. By identifying carrier couples, there was a marked decrease in new CF cases.

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Survival Rate

Behind sickle cell anemia, CF is the second most common life shortening disease. Fortunately, increased research and improved treatment has greatly improved survival. When CF was first recognized as a disease, most individuals did not survive past their first birthday.

Consequently, CF was thought to be only a pediatric disease. Since that time, 42% of individuals are surviving to or being diagnosed at 18 years or older.

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Etiology

CF is inherited in an autosomal recessive fashion. This means in order to develop CF, an individual must inherit a defective gene from each parent.

The associated gene causes a mutation in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein. More than 1400 known mutations exist for this gene.

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Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

Mutations of the gene for the CFTR result in the inability of the channel to move chloride ions outside of the cell.

The build of chloride inside the cells pulls water into cells, causing a thick sticky mucus to develop outside of the cell.

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Mutation

The most common mutation resulting in the malfunction of the CFTR is the deletion of the codon for phenylalanine at position 508. This mutation is referred to as ΔF508.

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Risk Factors

Genetics Parents who carry the mutated gene for CF have a greater likelihood

of having a child with CF.

Race CF is more common in Caucasian populations.

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Genetics

If both parents are CF carriers, there is a 25% chance that they will have a child with CF.

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Pathophysiology of CF

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Epithelial Cells & Exocrine Glands

Cystic Fibrosis mostly leads to abnormal function of epithelial cells and more specifically the exocrine glands within these epithelial tissues.

Some organs that have exocrine glands include: pancreas, lungs, breasts, skin, cervix, testicles, intestines, stomach and the mouth.

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What causes abnormal exocrine function?

Water doesn’t just follow Sodium, it follows all ions.

In CF, a mutation occurs that allows Na to diffuse across the membrane for excretion but not Cl. Cl is thus retained and less water diffuses out of the cell, leading to thicker mucus.

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Areas Affected by CF

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CF in the Lungs

Tiny cilia move mucus up through the lungs until it falls down the esophagus and is digested in the stomach.

In CF, the mucus is too thick for the cilia to function. What problems would mucostasis cause?

More frequent infections (Pneumonia)

More mucus in the lungs

Difficult breathing

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CF in the Lungs

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CF in the Pancreas What is secreted in

pancreatic juice? HCO3

CCK

Trypsinogen

Chymotrypsinogen

Pancreatic Lipase

Carboxypeptidase

Amylase

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Think about it!

What would happen if these enzymes had less water?

What would happen if these enzymes had less bicarbonate?

What would happen if there was an obstruction in the pancreatic duct?

How would these changes affect digestion?

How would these changes affect absorption?

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CF in Reproductive Organs

Vas deferens in 95% of men with CF will become obstructed due to thicker secretions

Females experience thicker secretions as well, leading to thicker mucus near the cervix. Sperm have difficulty penetrating this mucus. Women experience infertility, but to a far lesser degree.

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CF in Sweat Glands

Sweat also has a higher concentration of sodium.

This high sodium content in sweat can be used for diagnosing CF.

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Methods of Diagnosing CF

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Sweat Testing

Sodium concentration measured

Considered the most reliable method.

Non-invasive

Mild pain

Low cost

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Genetic Testing

Cells are collected from a patient to determine their genotype.

A patient’s genetic code is compared to known mutations of the CFTR gene on chromosome-7.

More costly method of diagnosing.

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Chest X-Ray

•Relatively cheap method•Not useful for those with mild forms of CF•More opaque areas indicate spots where mucus is concentrated

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Sinus X-Ray

Low cost

Opaque areas indicate

thick mucus

In this picture you can see that there is an excessive amount of mucus in the right maxillary sinus, but not on the left.

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Lung Function Tests

FEV1 – Forced expiratory volume in 1 second. Volume of air the can be forcefully exhaled in one second. Usually decreased in CF patients.

FEF – Forced expiratory flow. The rate of air released during the middle of an expiration. Usually decreased in CF patients.

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Lung Function Tests

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Current Medical Therapy

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Current Medical Therapy

Medicines: treats the problems associated with disease Pancreatic enzymes

Due to common pancreatic insufficiency

Most common Pancrease

Taken with each meal

Antibiotics

To avoid common infections

Anti-inflamatories

Ibuprofen

Corticosteroids

Tissues can become inflamed

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Bronchodilators Opens the airways

Mucus- thinners Most common is Dnase

Bisphosphantes To prevent brittle bones

Insulin CF can cause issues with blood sugar management

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Alternative Therapies

Antioxidants Some research indicates that it is beneficial to increase antioxidants

Probiotics

Acupuncture Helps with chest wall pain

Herbal Supplements Boswellia

Anti inflammatory

Not supported by research

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Cochrane Review

http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD002201.pub4/full Supplement use of omega-3 fatty acid to treat CF Results:

Unclear as to whether or not supplements are helpful Individuals with CF taking supplements so increase pancreatic enzyme intake Larger and longer studies need to decided if this is helpful for CF patients

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Physiotherapy

Exercise Maintains health and ability to clear airways

Both aerobic and anaerobic recommended

Good posture Easier to breathe and clear airways

Postural Drainage Lying in certain positions while caregiver vibrates section of the lungs

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Physiotherapy

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Physiotherapy

Devices Handhelds can create vibration in the lungs to aid clearance in daily

therapy

The flutter

RC cornet

Acapella

High frequency chest wall oscillation (HFCWO) an electric air compressor connects to an inflatable jacket (vest) to

vibrate your chest

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Physiotherapy

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Physiotherapy: Breathing Techniques

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Surgical Options and other procedures

Nasal polyp removal

Oxygen therapy

Endoscopy and lavage

Bowel surgery

Lung transplant

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Check ups

Every 3 months

Physical exam Check for nasal polyps

Pulmonary function testing

Review of medications and compliance

Nutrition assessment

Additional tests as needed Vitamin levels

Liver function

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Prognosis

Median age survival: 37 years

Daily Therapy

Frequent check ups

Exercise frequently

Avoidance of sickness

Eventually may need ventilators or assistance breathing

Page 42: Cystic Fibrosis MEDICAL NUTRITION THERAPY. Cystic Fibrosis  Cystic Fibrosis (CF) is a chronic disease, associated with thick, sticky mucus forming in

http://youtu.be/itQns51NKOo

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MNT for Cystic Fibrosis

Goal of MNT for CF: The aim of MNT should be to

control maldigestion and malabsorption.

Provide adequate nutrients to promote growth and weight

Support Pulmonary functions

Prevent deficiencies

All MNT should be individualized for each patient to include medications, and other forms of treatment.

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Nutrition Assessment

Anthropometrics: Height

Weight

Head circumference

Mid-arm circumference

Triceps skinfold

Biochemical: Complete Blood Count

Serum Reitnal Value

Serum α-tocopheral

Albumin

Electrolyte and acid-base status

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Nutrition Assessment Cont.

Dietary: Dietary intake

3-day fat balance

Feeding behaviors

Genetics: Any family members with Cystic

Fibrosis

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Energy Requirements

Energy Requirements are very individual, and change throughout life.

Needs based on gender, age, BMR, physical activity, respiratory infection, severity of lung disease, and severity of malabsorption

Higher energy expenditure due to the increase effector of breathing.

Shouldn’t decrease physical activity but rather increase energy needs for physically active CF patients

Special equation to estimate energy needs if laboratory methods aren’t available.

Children: High energy, moderate fat, and PERT

Adults: High energy to maintain weight.

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Macronutrients

Fat Needs 35-40% of total Calories, or more

as tolerated

Include sources of EFAs in diet

Monitor for EFA deficiency in regular blood lipid levels

Malabsorption of fat may lead to fat-soluble vitamins deficiency

Monitor of fat intolerance in the stool

Protein Needs 15-20% of total Calories

Protein needs generally met if adequate energy needs are met.

CHO Needs• Needs change as

disease progresses

• CF patients might become lactose intolerant

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Micronutrients-Vitamins

Water-soluble vitamins adequately absorbed

Vitamin A- low serum levels, and high hepatic store. Suggests impaired mobilization and transport.

Vitamin D- decreased bone mineralization

Vitamin E- hemolytic anemia and abnormal neurological findings

Vitamin K- antibiotic use and liver disease

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Micronutrients-Minerals

Sodium- Increased need

Increase loss in sweat

Adequate amounts generally met with normal diet.

Supplement Na+ when: breastfed, fever, hot weather, and physical exertion.

S/S: lethargy, vomiting, and dehydration

Calcium

Decrease bone mineralization

Zinc

Growth and healing

Magnesium

Iron

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Pancreatic Enzyme Replacement Therapy

First step to correct malabsorption and maldigestion

Able to withstand acidic environment of the stomach

Released in the duodenum

Breaks down CHO, Fats, and Protein

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Pancreatic Enzyme Replacement Therapy

How to determine an adequate amount to give:

Dosage depends on: degree of pancreatic insufficiencies, the amount and content of food.

Should try to control GI symptoms (steatorrhea) and promote growth

Fecal elastase, fecal fat, or nitrogen balance studies.

Fecal elastase: is an enzyme secreted by the pancreas used to digest protein and is involved in hydrolysis of peptide bonds.

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Feeding Strategies

Infants Breast feeding is encouraged

Pancreatic insufficiency give enzymes either mixed with food or directly into infants mouth

Formula fed: standard formula (20-27kcal/oz) with enzyme supplementation

High calorie formula may be necessary

Children and Adults Positive eating behaviors

Enjoyable meal times

Larger portions and extra snacks

Nutrient dense food

Adding supplements to food

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Food Strategies

Enteral Nutrition Unable to meet needs orally

Continuous infusion

Elemental and nonelemental formulas used

Parental Nutrition Only used for short term

After a gastrointestinal surgery

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Case Study: LJ

Age:14

High School Student

Female

Caucasion

Ballet, Jazz, and Cross-country

Anthropometrics

5’ 5”

3 Months ago: 110-115 lbs

Currently: 102

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Case Study: LJ

Biochemical

Transferrin- 219 (250-380)

Magnesium- 1.6 (1.8-3)

HbA1C- 6.3 (3.9-5.2)

WBC- 13 (4.8-11.8)

HGB- 11.5 (12-15)

HCT-33 (37-47)

Ferritin- 19 (20-120)

Clinical

Pharynx red with postnasal drainage

Pale Skin

Decreased breath sounds

Percussion hyper resonant

Rhonchi and rales present

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Case Study: LJ

Usual Diet

Rarely eats breakfast

Lunch: pb or ham and swiss sandwich, 2-3 oz chips, orange or other fruit, water

Dinner: 5-6 oz grilled or baked meat,1-2 c raw veggies on lettuce w/ ¼ c ranch, 1 c pasta, potatoes, or rice, usually with 1-2 T margarine, water

Depends on whether at home or at a friend’s

Babysits a lot

Often is away from home for meal times

Working at a camp

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PES Statement

The patient does not have adequate oral intake due to digestic insufficiency as evidenced by weight loss

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Case Study: LJ

BMI: 17 (18.5-24.9)

IBW: 125 lbs 13 lbs under

Calculated intake EER(act)

(135.3-(30.8xage))+PAx((10xwt)+(934xht))

2522 kcal

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Sample Diet

Breakfast: 1 slice of toast with margarine, 1 fruit, 1 c milk

Lunch: Sandwich- 2 slices bread, 3 oz lunch meat, 2 oz cheese, lettuce, tomato, mayonnaise. 1 fruit, 1 cup fruit juice

Dinner: Beef Stroganoff-4 oz beef, 1 cup egg noodles, mushroom cream sauce. 2 cup salad with ¼ cup ranch, 1 cup milk

Try not to eat any “diet” items

Encourage breakfast