Cystic Fibrosis - Nutrition

 

 

Cystic Fibrosis - Nutrition

•  Cystic Fibrosis (CF) is an incurable disorder that is inherited autosomal recessively.
•  It mainly affects the respiratory system and the digestive system.
•  The body secretes an abnormally thick mucus causing an obstruction in the lungs and the pancreas.
•  The abnormal mucus secretion in the lungs leads to breathing problems and repeated respiratory infections, such as pneumonia and bronchitis.
•  In the pancreas, the mucus interferes with digestive enzymes and its ability to secrete the hormone insulin (needed to break down sugar), making it difficult to effectively digest food and absorb the nutrients.

Epidemiology

·    Affects 1 in every 3000 Caucasian babies born
·    30,000 people in the US (70,000 worldwide)
·    In Gaza Strip there are 48 cases diagnosed with CF.
·    In Jordan CF affects about 1 per 2560 newborn,
      while in Egypt it affects 1 per 4000 newborn.

·    30% of patients are adults

Cause of CF

·    CF is caused by a defect in the gene CFTR (Cystic Fibrosis Transmembrane Conductance), a protein found in cells that line the lungs, digestive tract, sweat glands, and the genitourinary system.

·     It controls the flow of chloride ions in and out of these cells.

·     In a healthy person, CFTR forms a channel in the plasma membrane allowing chloride ions to enter and leave the cells. In a person with CF, the CFTR prevents chloride from entering or leaving the cells, resulting in the thick mucus that causes the obstruction.

Symptoms of Cystic Fibrosis

·     Can very from person to person

·     They can become apparent shortly after birth or can go undetected for months or years.

·     The most common symptom is intestinal blockage in newborns (meconium ileus).

·     Others symptoms can include bulky or greasy stool, poor weight gain or abnormal weight loss.

·     Shortness of breath, wheezing, persistent cough producing a thick mucus

·     Frequent respiratory infections, such as Pseudomonas aeruginosa or abnormally salty sweat and dehydration. 

  

Diagnosing CF

·  Symptoms suggestive of CF are exhibited by the patient.

 A sweat chloride test also known as Sweat Test Measures the amount of salt in the sweat

¨   Positive test - >60 mEq/L

¨   Negative - <40 mEq/L

 

· There are various other tests used to diagnose Cystic Fibrosis such as:

          1.  DNA testing

          2.  Fecal fat Test

          3.  Upper GI and small bowel series

          4.  Measurement of pancreatic function

·  CF gene mutation is useful for diagnosing CF as well as determining C carriers

·  Pulmonary function tests (PFTs) are breathing tests that help measure lung reserve and degree of airflow obstruction

Complications

·  Pancreatic Insufficiency

 

·  Pulmonary Manifestations

 

·  Growth Complications

 

·  Insulin Dependent diabetes

 

·  Distal Intestinal Obstruction Syndrome and Constipation

 

·  Bone disease

Managing CF

· Tobramycin for lung infections caused by bacteria.

·  Decongestants, bronchodilators (drugs that open airways congested with mucus) and anti-inflammatory drugs.

·  Nutritional therapy (high-protein, high-calorie diet with vitamin supplements)

·  Pancreatic enzymes to aid in digestion

·  Chest physical therapy or postural drainage (chest or back clapping) helps to loosen and drain the mucus from the lungs.

·  Newer treatments include inhalation of a spray containing normal copies of the CF gene, which will deliver the correct copy of the CF gene into the lungs.

·  Also, protein repair therapy aims at repairing the defective CFTR protein.

Factors associated with poor nutrition

·   Increased stool losses of nutrients.

 

·  Anorexia and poor dietary intake.

   

·  Increased energy demands of the disease.

 

·  Abnormal adaptive response to malnutrition

Cystic Fibrosis - Nutrition

·  The energy needs of patients with CF have been stated as 120-150% of those required by healthy individuals of the same age, sex and size.

·  This reflects an increase in the basal metabolic rate (BMR) as a result of increase in the work of breathing. Infection and inflammation also increase the energy Demands

·   Fewer of the energy-producing nutrients are properly digested and absorbed into the body, even when treatment is optimal.

.

n  Increasing Energy Requirements

·   3 meals and 3 snacks

·   Supplemental milkshakes

·   Foods to add calories

¨  Whole milk, cheese, butter, margarine, oil

¨  Peanut butter spread

¨  Ice cream or whole milk yogurt

¨  Salad dressing and mayo

¨  Cream or half and half

¨  Cream cheese

¨  Sour cream

¨  Powdered milk

¨  At least one portion of starchy food should be offered with every meal (potatoes, bread, cereals, rice, biscuits and pasta)

Protein Requirements

·  Protein is necessary for growth and maintenance of body tissues, and proper

    functioning of the immune system.

    1.5-2 times the DRI for age

·   Most people with CF have higher-than-usual protein requirements.

·   Reference nutrient intake for protein:

·   Protein      0-3 month   4-6 month  10-12 month

·   (g/day)        12.5          12.7             14.9

 

·  Most high-protein foods are of animal origin, including milk and milk products,

    meats, poultry, fish, and eggs.

 

n   Legumes, such as peas, beans, and lentils, are also good sources of protein,

     especially when eaten with cereals.

n  High-protein, high-calori foods

·  Whole-milk products, such as yogurt,cheese, custard, and pudding.

·  Beverages made with whole milk or undiluted evaporated milk, such as

     milkshakes.

·  Nuts, nut butters (especially peanut) and halawa.

·  Meat, fish, poultry, or eggs prepared with added fat.

Fat Requirements

·  35-40% of calories

·  Fat provides more than twice as many calories as protein or carbohydrate.

·  Conversion of fat to energy requires less oxygen than conversion of protein

    or carbohydrate to energy, putting less of a burden on the respiratory system.

·   Fats are essential for the body to properly absorb vitamins A, D, E, and K.

·  Adequate dietary fat is required fo provision of essential fatty acids.

·  Generally, individuals with CF have a low blood cholesterol level and therefore do not have to restrict the intake of saturated fat and cholesterol.

n  Vitamins Intake

·  Fat soluble vitamin deficiencies common

· The daily requirement of fat-soluble vitamins in CF is about twice the normal recommende values for age

·  Monitor overdosing

¨ Patient instruction

¨ Yearly monitoring of serum levels

·  People with CF need about 50-200 mg of vitamin E daily.

· Vitamins are absorbed most effectively when taken with fat-containing meals and PRT.

n  Salt Intake

·  People with CF lose more salt (sodium chloride) in their sweat than other individuals.

   Their need for additional salt is usually met simply by increasing intake of normal foods in order to meet caloric requirements.

·  Na requirements increased

¨ Monitor in hot weather, fever, physical exertion

¨ Sports drinks with electrolytes

 

n  Dairy Products

·  Cystic fibrosis patients tend to have less bone density. so

n  Drink at least 3cups of milk daily.

·  Take enough vitamin D and K.

·  Ensure adequate sun exposure.

·  Exercise daily to maintain bone mass

·  and help to clear the chest.

n  NSP (Fiber)

·  Children with CF should be encouraged to eat some foods which are rich in

    fiber to help maintain normal bowel function and prevent constipation.

   However, too much fiber is very filling and may reduce your child’s intake of

   high energy foods.

·  These foods also contain ‘photochemical’ which may help the body to fight infection

 

n  Water

·  Patients with CF should consume plenty

    of water.

·  Proper hydration helps to decrease the

·  viscosity of sputum.

·  Avoid caffeine

·  Encourage your child to drink 6 – 8 cups of fluid daily.

n  fruit and vegetables?

·  These foods are not high in calories, but they are valuable source fiber ,vitamins and some minerals

·  Encourage 3-5 portions daily

·  Stir frying vegetables will increase their calorie value.

·  Adding a knob of butter to vegetables increases the calories.

n  Modifying intake of regular foods

·  If caloric and nutrient requirements are to be increased, modify intake by

    following these four steps:

·  Increase portion sizes.

·  Add snacks and “mini-meals”.

·  Emphasize high-protein, high-calorie foods.

·  Add extra healthy fats- Fry foods.

 

n  Pancreatic ReplacementTherapy (PRT)

·   85-90% pancreatic insufficiency

·   (pancreatic insufficiency )very little or no pancreatic enzymes

·   enzyme requirements will differ

·   The dose of (PRT) is 1-2 capsules/kg of the average weight of the child (and not per the current weight), PLUS 20% of this dose to cover the increased basal metabolic rate.

n  Pancreatic Replacement
Therapy (PRT)

·   Then adjust the dose according to weight gain and stool character, as proper dose (PRT) promote weight gain, and prevent or minimize steatorrhoea

·   pancreatic enzymes may be inactivated by elevated gastric and duodenal acidity.

·   The use of H2 receptor antagonists or perhaps PPI should then be considered

n  Enzyme Dosing

·   Creon (microsphere)

¨  Creon 5 = 5000 units lipase/capsule

¨  Creon 10 = 10,000 units lipase/capsule

¨  Creon 20 = 20,000 units lipase/capsule

·  Pancrease MT

¨  4, 10, 16 and 20 (number x 1000 = units lipase/capsule)

·  Ultrase

¨  4500 units/capsule

·  Ultrase MT

¨  12, 18 and 20 (number x 1000 = units lipase/capsule)

n  How should pancreatic enzymes be taken?

·   Do not mix the enzymes with hot food.

·   Do not allow your child to chew the granules.

·   Distribute the required dose of Creon over 3 main meals and 2-3 snacks.

·   Do not try to dissolve enzymes in water

     or milk as they will never dissolve.

·   Fatty meals will need more enzymes, than low fat      meals.

n  How should pancreatic enzymes be taken?

·   give enzymes at the beginning and part through a meal

·   For older children who cannot swallow the capsule, open the capsules and mix the microtablets with a small amount of food.

·  Treatment of CF is not curative and must be continued throughout life.

n  Foods that do not requirePRT

n   Fruits (except avocado)

·   Vegetables, (except potatoes & beans)

·   Honey, jam, sugar

·   Jelly, candy

·  Soft drinks, tea and coffee.

n  How will I know if the amount of enzyme is correct?

·   There are several signs which may mean that the dose of enzymes is incorrect:

·   Pale, floaty stools which are difficult to flush away.

·   Increased stool frequency.

·  Abdominal pain, bloating, flatulence or wind.

·  Poor weight gain.

Summary

·    Cystic Fibrosis is an autosomal disorder that affects the lungs and the pancreas
 It is caused by a defect in the CFTR protein, resulting in obstruction due to excess mucus Patient treated with Motrin for fever and Tobramycin for Pseudomonas aeruginosa infection Receiving Pulmozyme inhalation therapy and Creon for CF

References

n  Henry J. Clinical Diagnosis and Management by Laboratory Methods. 20th ed. Philadelphia, W.B. Saunders Company, 2001.

n  Cystic Fibrosis Foundation, http://www.cff.org/home/

     Last accessed 20/01/11.

n  “65 Roses” http://www.cysticfibrosiswa.org/redrose.html

     Last accessed 20/01/11.

n  Understanding Cystic Fibrosis, http://www.hipusa.com/eTools/webmd/A-Z_Encyclopedia/cysticfibrosisbasics.htm

   Last accessed 20/01/11.