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Cystic FibrosisMEDICAL NUTRITION THERAPY
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.
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.
Prevalence
In the United States, there are approximately 30,000 individuals with CF.
The number of new cases each year is approximately 1000.
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.
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.
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.
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.
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.
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.
Genetics
If both parents are CF carriers, there is a 25% chance that they will have a child with CF.
Pathophysiology of CF
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.
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.
Areas Affected by CF
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
CF in the Lungs
CF in the Pancreas What is secreted in
pancreatic juice? HCO3
CCK
Trypsinogen
Chymotrypsinogen
Pancreatic Lipase
Carboxypeptidase
Amylase
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?
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.
CF in Sweat Glands
Sweat also has a higher concentration of sodium.
This high sodium content in sweat can be used for diagnosing CF.
Methods of Diagnosing CF
Sweat Testing
Sodium concentration measured
Considered the most reliable method.
Non-invasive
Mild pain
Low cost
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.
Chest X-Ray
•Relatively cheap method•Not useful for those with mild forms of CF•More opaque areas indicate spots where mucus is concentrated
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.
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.
Lung Function Tests
Current Medical Therapy
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
Bronchodilators Opens the airways
Mucus- thinners Most common is Dnase
Bisphosphantes To prevent brittle bones
Insulin CF can cause issues with blood sugar management
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
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
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
Physiotherapy
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
Physiotherapy
Physiotherapy: Breathing Techniques
Surgical Options and other procedures
Nasal polyp removal
Oxygen therapy
Endoscopy and lavage
Bowel surgery
Lung transplant
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
Prognosis
Median age survival: 37 years
Daily Therapy
Frequent check ups
Exercise frequently
Avoidance of sickness
Eventually may need ventilators or assistance breathing
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.
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
Nutrition Assessment Cont.
Dietary: Dietary intake
3-day fat balance
Feeding behaviors
Genetics: Any family members with Cystic
Fibrosis
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.
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
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
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
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
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.
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
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
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
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
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
PES Statement
The patient does not have adequate oral intake due to digestic insufficiency as evidenced by weight loss
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
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