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DESCRIPTION
Cystic fibrosis (CF) is a chronic, progressive condition that primarily affects the body's respiratory and digestive systems. It is due to a gene defect that causes the body to produce abnormally thick mucus affecting the glands that produce mucus, tears, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions may plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of CF.
CF is the most common inherited illness among Americans of northern and western European ancestry, affecting some 30,000 people (adults and children). Although it occurs in people of all ethnic backgrounds, it is far more common in Caucasians (an estimated incidence of 1 in 3,000) than in African-Americans (1 in 17,000) and Asian-American children. Approximately 3,200 Caucasian babies are born each year in the United States with CF. Two-thirds of the infants born with CF will be diagnosed in the first year of life. It occurs with approximately equal frequency in men and women. One in 31 (an estimated 8 million) Americans carries an abnormal copy of the CF gene but isn't sick with the disease. These are considered "silent carriers" of the defective gene that leads to this disease. You must have two abnormal copies of the gene, one from your mother and one from your father, to have cystic fibrosis.
In the early 1950s, children born with CF died early in childhood. Today, with the help of improved medical therapies, people with CF often live into their 30s and possibly longer and have fuller, more comfortable lives.
CAUSES
The gene responsible for CF was identified in 1989 on human chromosome 7, and the defective gene encodes instructions for altering a protein that regulates the passage of salt (sodium chloride) in and out of cells of the body's endocrine glands. This defective gene transforms the protein called the cystic fibrosis transmembrane conductance regulator (CFTR) and causes it to produce thick, sticky mucus secretions too thick and too abundant for the body to excrete in the respiratory and digestive tracts, as well as the reproductive system. It also causes increased salt in sweat on the skin.
In most people with CF, the genetic instructions omit just one of the protein's 1,480 constituent amino acids, a tiny glitch, but a devastating one that affects many different glands in the body, including the pancreas, sweat glands, and glands of the digestive and respiratory systems.
The affected gene, which is inherited from a child's parents, is known as a recessive gene. All human cells (except red blood cells, eggs, and sperm) contain two copies of this gene, one inherited from each parent. CF results when both copies of the CF gene are abnormal. If one copy is abnormal and the other is normal, an individual is said to be a carrier. He or she will show no signs of CF, but is capable of passing a defective gene on to their children. Statistically, a child of two carrier parents has a 1-in-4 (25%) chance of inheriting CF; a 1-in-4 (25%) chance of being completely free of the mutant gene; and a 1-in-2 (50%) chance of being a carrier, like the parents. If only one parent has an abnormal gene and the other parent has 2 normal genes, there is a 1-in-2 (50%) chance of each child being a carrier, but it is impossible for any of these children to have the disease. If one parent has CF and the other parent is a carrier, each child has a 1-in-2 (50%) chance of being a carrier and a 1-in-2 (50%) chance of having the disease. If one parent has CF and the other has 2 normal genes, the child will be a carrier of the CF gene. About 8 to 10 million people in the US may be carriers and not know it. Because of the reproductive problems associated with CF, it is highly unlikely a child would be born between two parents with the disease. However, all children (100% chance) born to such a couple would have inherited CF.
SYMPTOMS
Symptoms of CF usually begin early in life and vary in quantity and intensity. While some children may exhibit symptoms at birth, others may not develop symptoms for weeks, months, or even years. The severity of the symptoms will vary from mild (children showing only mild digestive and respiratory problems) to severe (children displaying severe food-absorption problems and life-threatening respiratory complications). The signs and symptoms of CF may vary with age.
In some newborns the first sign may be a blockage of the intestines (meconium ileus). This occurs when the meconium (the tarry, greenish-black stools normally passed by an infant in the first few days after birth) becomes so thick it can't move through the intestines. Other signs in newborns may include a failure to grow, bulky and greasy stools (steatorrhea) and frequent respiratory infections.
The airway, gastrointestinal tract, bile ducts of the liver, ducts of the pancreas, and the male genitourinary tract all produce mucus. Cystic fibrosis alters this normally protective mucus and transforms it into a thick, abnormal excretion that obstructs airways and damages tissues. Symptoms of CF begin early in life.
An individual may have any or all of these CF symptoms. The signs and symptoms of CF in children and young adults may include:
- Glands in the lungs and bronchial tubes secrete large quantities of thick, sticky mucus that blocks lung passages and traps harmful bacteria, giving the bacteria a perfect place to thrive. Pseudomonas aeruginosa (also common in cancer and burn patients) is the bacteria that most commonly colonizes the lungs, resulting in chronic coughing and wheezing, difficulty breathing, and recurrent lung and sinus infections, such as pneumonia, bronchitis, or other conditions resembling asthma. Once established, the bacteria remain in the lungs and are responsible for repeated outbreaks of infection.
The bacteria form their own dense structure, called a biofilm, and are immune to most current treatments. They also produce toxic proteins that can cause tissue damage and weaken the immune system. The lungs of many children with CF are inhabited or colonized by the Pseudomonas aeruginosa bacteria before they are 10 years of age.
- Thick secretions also often obstruct the release of pancreatic enzymes, preventing digestive enzymes from reaching the intestines, resulting in digestive difficulties and malabsorption problems, particularly problems with the metabolism of fats. Malnutrition may result because a lack of necessary digestive enzymes means that nutrients from foods are not properly absorbed. This in turn can cause pain after eating and, especially in young children, a failure to gain weight normally, even with an excessive appetite and delayed growth. Pancreatic enzymes need to be replaced to counter this.
- People with this disease also lose excessive amounts of salt through their sweat glands. Sweating may be profuse and the sweat itself contains abnormally high concentrations of sodium, potassium, and chloride salts. People with CF tend to have 2 to 5 times the normal amount of salt (sodium chloride) in their sweat. This may be one of the first signs parents notice because they taste the salty-tasting skin when they kiss their child.
Other signs suggestive of CF include:
- Blockage of the bowel.
- Greasy (steatorrhea), very bulky, foul smelling stools from eliminating non-digested food through the bowel.
- Thick sputum. Infants and young children tend to swallow their sputum, and parents may not be aware of it.
- Enlargement or rounding (clubbing) of the fingertips and toes (a result of poor circulation). Although clubbing eventually occurs in most people with CF, it also occurs in some people born with heart disease and other types of lung problems.
- Infertility. CF also affects the reproductive systems of both males and females. Although females with CF have normal fallopian tubes and ovaries, their thick cervical secretions may block sperm entry and prevent them from getting pregnant. Males with CF are almost always sterile because they produce relatively few or no sperm. Also, abnormally thick secretions may block the ducts that carry sperm, or the ducts may not develop properly.
- Cirrhosis of the liver due to inflammation or obstruction of the bile ducts.
- Displacement of one part of the intestine into another part of the intestine (intussusception) in children older than age 4.
- Protrusion of part of the rectum through the anus (rectal prolapse). This is often caused by stools that are difficult to pass or by frequent coughing.
DIAGNOSIS & TESTS
GENETIC TESTING: The identification of the CF gene has enabled researchers to begin developing new approaches to diagnosis and treatment of the disease. A test is now available in which cells are swabbed from the inside of the cheek and then examined for the presence of defective genes. In some cases your health care provider may perform a genetic analysis of a blood sample to confirm a diagnosis of CF. Researchers have identified more than 800 changes in a gene that when paired with another abnormal gene can result in CF. Testing is possible on approximately 30 of the most common genetic mutations. The presence of both normal and mutant CF genes indicates that the individual is a carrier. If only mutant genes are there, CF is indicated. Detection of CF in a fetus is possible through genetic testing. Although many health professionals recommend genetic testing for cystic fibrosis (as well as other genetic disorders) be offered to all expectant couples or those still planning a pregnancy, it is especially important to consider genetic testing if they have a family history of the disease. Your health care provider may suggest testing of brothers and sisters of a child with CF, even if the siblings show no symptoms. Other family members, especially first cousins, also may want to be tested. In most cases, family members can be screened with a sweat test, although in some cases genetic testing may be appropriate. Genetic testing will identify about 90% of all carriers.
ELECTROLYTE SWEAT TEST: The most widely used test for CF is the electrolyte sweat test (also called the quantitative sweat chloride test or simply "sweat test"). Developed over 40 years ago, it detects the excessive amounts of electrolytes (charged mineral salts) found on the skin of many people with CF. A health care provider would likely recommend that a sweat test be performed on a child who failed to gain weight despite adequate feeding, or who suffered from very frequent respiratory infections. CF testing is currently recommended only for those individuals with symptoms highly suggestive of the disease, or with a family history of the disorder. However, if there is any doubt about the diagnosis, the sweat test can be confirmed by performing genetic testing.
To conduct this painless test, a health care provider gives the child a medications that stimulates the production of sweat. During the procedure a small amount of odorless sweat-producing chemical is applied to a small area on the arms or legs. An electrode attached to the area stimulates a very weak electrical current, causing a tingling or warm feeling. Then he or she applies a gauze pad to a patch of skin (typically on the forearm) and wraps it in plastic. After 30 minutes or so, the plastic is removed and the collected sweat is analyzed in a laboratory. The sweat test is usually performed twice. A consistently high level of salt indicates CF. This test doesn't show whether someone has a mild or severe case of the disease, however, and it can't predict how well someone with CF will do.
IMMUNOREACTIVE TRYPSINOGEN TEST (IRT): The sweat test can be difficult to administer to newborns because they do not have active sweat glands and do not produce enough sweat for a reliable diagnosis in the first month of life. For newborns, an immunoreactive trypsinogen test (IRT) may be used instead. This test involves drawing blood 2 to 3 days after birth and analyzing it for a specific protein called trypsinogen. A positive IRT may be confirmed at a later date with the sweat test (when the infant is several months old).
HELPFUL RELATED LINKS:
AMA (Medical Science) ACOG & Cystic Fibrosis
RISK INCREASES WITH
Risk for developing cystic fibrosis increases with presence of the CF genes in both parents. If you have CF in your family history you (and your partner) should be tested for the presence of defective CF genes before you plan to have children. CF occurs mainly in Caucasian individuals of Northern European ancestry (1 in 29). It is relatively rare in Hispanic (1 in 46), African-American (1 in 62) and Asian-Americans (1 in 90).
COMPLICATIONS
The most frequent complications of CF are chronic respiratory infections, including pneumonia, bronchitis and bronchiectasis (an abnormal dilation of the walls of the bronchial tubes that make it more difficult to clear your airways).
Respiratory infections are common because thick mucus blocks the airways and provides a perfect breeding ground for bacteria. The most common infective agent in people with CF is Pseudomonas aeruginosa, a bacterium that can cause increased inflammation of the respiratory tract. Although antibiotics can decrease the frequency and severity of attacks, the bacterial are never completely eradicated from the airways and the lungs.
People with CF may also develop collapsed lung, a condition in which lung air leaks into the chest cavity (pneumothorax), bleeding from the lungs (hemoptysis) or respiratory failure. Because of lung disease, the right ventricle of the heart may also fail (cor pulmonale). Ultimately complications from lung problems may prove fatal to many people with CF.
Cystic fibrosis makes you prone to chronic diarrhea and severe nutritional deficiencies. That's because thick secretions obstruct the ducts in the pancreas, preventing enzymes that digest fats and proteins from reaching your intestines. These secretions also prevent your body from absorbing the fat-soluble vitamins A, D, E, and K.
CF affects the pancreas and because the pancreas controls the level of sugar in your blood, about 7% of people with CF may develop type 1 diabetes (formerly called juvenile or insulin-dependent diabetes). In addition, the bile duct, the duct that carries bile from your liver and gallbladder to your small intestine, may become blocked and inflamed, leading to liver problems.
CF also affects the reproductive system. Because thick secretions often block the tube connecting the testes and prostate gland (vas deferens), many men with CF are infertile. But certain fertility methods and surgical procedures may sometimes make it possible for men with CF to become fathers. Although women with CF may be less fertile than other women are, it's possible to conceive and to have successful pregnancies. Discuss with your midwife or health care provider the options and weigh carefully the possible risks. In addition, using oral contraceptives can sometimes aggravate certain symptoms of CF. Discuss you birth control options with your health care provider.
TREATMENT
Children with CF are living longer today, largely because of advances in antibiotic treatment and improved nutritional management. Treatment focuses on both the respiratory and digestive components of the disease.
Recent media reports on the beneficial effects of the spice Turmeric (actually, its pigment curcumin) in treating CF should not be taken to indicate that this is a cure. It is true that in animals tests conducted at Yale University, curcumin achieved beneficial results. Curcumin is a weak calcium-pump inhibitor and this enhances the energy transport mechanism of the cell, which appears to be defective in patients with CF. At this point, researchers caution that taking curcumin pills as a supplement, along with the existing CF drug therapies, has not been tested and could pose hazards. A phase I safety trial is being conducted. Research is still in the preliminary stages.
The symptoms of CF are normally controlled with a number of different drugs. Antibiotics are used to combat the infections to which people with CF are prone, especially infection with Pseudomonas aeruginosa, a type of microbe that is attracted to the sticky mucus in the lungs.
Pancrelipase (also sold under a variety of brand names, including Viokase) is a prescription product containing a combination of digestive enzymes that is often prescribed for people with CF and other pancreatic insufficiencies.
Many people also take anti-inflammatory drugs such as ibuprofen (Advil, Nuprin, and others), naproxen (Naprosyn, Aleve), or prednisone (Deltasone and others).
A small handheld device called the Flutter helps people with CF to dislodge mucus from the airways. It can replace conventional physiotherapy.
The drug amiloride (Midamor, Moduretic), which is used as an adjunct to treatment with some diuretic drugs, is being tested as a treatment for CF. It is believed to thin lung secretions by blocking sodium uptake by lung cells.
DNAase (Dornase) is a naturally occurring enzyme that breaks down DNA molecules. Part of the reason the mucus that clogs the airways of people with CF is so thick and sticky is that it contains large molecules of DNA released by white blood cells as they die fighting chronic bacterial infection. This makes the mucus even denser and more difficult to expel. Breaking down the DNA molecules helps to thin the mucus.
Levels of 2 fatty acids - arachidonic acid (AA) and docosahexaenoic acid (DHA) - have been found to be out of balance in people with CF. This lipid imbalance is most noticeable in the lungs, pancreas, and intestine, all of the areas most affected by cystic fibrosis. Research in this area seems promising.
When the antibiotic tobramycin (Nebcin) is administered by aerosol spray, it has proved to be more effective in reaching infected lung tissue than when administered through the traditional intravenous route.
Love levels of Vitamin E and selenium have been linked to cystic fibrosis and cancer.
For more information about cystic fibrosis, see the links below under "Related Links" or contact the Cystic Fibrosis Foundation.
MANAGEMENT OF RESPIRATORY PROBLEMS
The focus of respiratory management is prevention of infection.
BRONCHIAL AIRWAY DRAINAGE. People with CF need a way to physically remove thick mucus from their lungs. Daily chest physiotherapy (CPT), also known as percussion and postural drainage, helps to loosen lung secretions and stimulates coughing. To perform CPT, the person is put into a position that helps drainage, and then all areas of the lungs are "percussed" by clapping on the person's back with a cupped hand. Family members can perform CPT on small children, while older children and young adults can learn to do it themselves. CPT is usually performed twice a day, though it may be done more frequently when the person has an active lung infection. Regular exercise also helps to loosen and move secretions.
ANTIBIOTICS. Antibiotics are crucial to treating CF lung infections (primarily Pseudomonas aeruginosa, a type of microbe that is attracted to the sticky mucus in the lungs). The newer antibiotics may be more effective to fight the bacteria that cause lung infections in people with CF. These may be administered in a vein (intravenous) and orally and have made antibiotic therapy available on an outpatient basis. Aerosol mist antibiotics can be inhaled. In late 1997, the FDA approved the use of an inhaled antibiotic called TOBI (Tobramycin Solution for Inhalation). The benefits of TOBI are that it gets directly to the site of a lung infection. Its development is expected to lead to more aerosol antibiotics for people with CF.
MUCUS THINNING DRUGS. DNA, the basic material in your white blood cells, is released when these cells attack bacteria in your airways, making the mucus in the airways even thicker. Another substance undergoing testing as a potential treatment for CF is deoxyribonuclease (DNase), a protein administered in aerosol form to help thin secretions and clear the lungs by fragmenting DNA. This makes the mucus thinner and easier to cough up. Studies suggest that its use may significantly reduce the risk of death and shorten hospital stays for people with CF. In 1994, researchers developed a mucus-thinning drug called Pulmozyme (dornase alfa) Inhalation Solution. Pulmozyme is a highly purified solution of recombinant human deoxyribonuclease I (rhDNase), an enzyme which selectively cleaves DNA. It is administered by inhalation of an aerosol mist produced by a compressed air driven nebulizer system. It is the first new drug treatment specifically for CF in 30 years. In tests, Pulmozyme cut the number of respiratory infections and increased lung function. Side effects of Pulmozyme may include chest irritation and sore throat. The drug amiloride (Midamor, Moduretic), which is used as an adjunct to treatment with some diuretic drugs, is being tested as a treatment for CF. It is believed to thin lung secretions by blocking sodium uptake by lung cells.
BRONCHODILATORS. Use of medications such as albuterol (Proventil, Ventolin) may help keep the airways in the lungs open by helping to clear thick bronchial secretions.
The future of CF treatment may lie in gene therapy, offering the best hope for a lifesaving treatment that attacks the cause of CF, rather than just treating the symptoms. Since 1989, when the defective CF gene was discovered, researchers have learned how to make copies of the normal gene and use it to correct defective cells. They must now find a way to correct a significant number of defective cells in individuals with CF. In the laboratory, normal CF genes have been successfully introduced into cells from people with CF. Experiments in rats have indicated that replacing the defective CF genes with normal genes in just 10 percent of the lung lining cells improves lung function. However, because the genes in the cells of the reproductive system are unaffected by this procedure, the defect can still be passed on to offspring. Several clinical studies are under way to refine gene delivery. Research continues, and much work remains before an actual cure for this complex disease is found.
MANAGEMENT OF DIGESTIVE PROBLEMS
People with CF must eat a well-balanced, high-calorie, high-protein diet. Their reduced absorption of nutrients often means that as children, they must consume up to 150% of the daily recommended allowances to meet their growth needs. Multivitamins and supplements of the fat-soluble vitamins (A, D, E, and K) are also important. To enhance digestion, people need to take supplemental pancreatic enzymes before every meal and snack and extra enzymes to help digest high-fat foods. Those with severe digestive impairment may require supplemental feedings either with a feeding tube or by vein.
HERBS
Alfalfa extract supplies vitamin K and necessary minerals, which are often deficient with cystic fibrosis due to absorption problems. It is also a good source of chlorophyll.
Boswellia, Bromelain, Cayenne, Ginger, and Peppermint can aid in reducing inflammation.
Expectorant herbs such as Cayenne, Elecampane, Garlic, Horehound, Hyssop, and Mullein may be effective in helping to clear some congestion.
Clear Lungs from RidgeCrest Herbals is a Chinese herbal formula that is highly recommended for this condition.
Eucalyptus, Garlic, Onion, Tea Tree Oil, and Thyme have natural antiseptic properties and fight infection.
Echinacea, Licorice, and Siberian Ginseng are good for building up the immune system. Caution: If overused, licorice can elevate blood pressure. Do not use this herb on a daily basis for more than 7 days in a row. Avoid it completely if you have hypoglycemia, high blood pressure, or a heart disorder.
Lung Tonic from Herbs, Etc., is a combination of many organic herbs designed to support the lungs.
Other herbs beneficial for cystic fibrosis include Echinacea, Ginger, Goldenseal, and Yarrow tea.
HOLISTIC RECOMMENDATIONS
Eat a diet consisting of 75% raw fruits and vegetables, and raw nuts and seeds.
Make sure your intake of calories, protein, and other nutrients is adequate. People with CF require as much as 50% more of many nutrients than normal. Take supplements to provide required enzymes.
Include in the diet foods that are high in germanium, such as garlic, shiitake mushrooms, and onions. Germanium helps to improve tissue oxygenation at the cellular level.
During hot weather, drink plenty of fluids and increase your salt intake.
Do not eat foods that stimulate secretions by the mucous membranes. Cooked and processed foods cause excess mucus buildup and drain the body of energy. These foods are harder to digest. Do not eat animal products, dairy products, processed foods, sugar, or white flour products.
When you must take antibiotics, take acidophilus to replace "friendly" bacteria.
Low levels of selenium and vitamin E have been linked to cystic fibrosis and cancer. Be sure to include these supplements in your nutritional plan.
NUTRIENTS Supplement Suggested Dosage Comments Very Important Pancreatin As directed on label. Take with meals. Needed for protein digestion. Proteolytic Enzymes As directed on label. Take between meals or an empty stomach. Aids in controlling infection, helps digestion, and thins the mucous secretions of the lungs.
MetabolicZyme (Hypoallergenic), Allergy Research Group / Nutricology, 900 Tabs,
Plant Enzymes, NOW Foods, Vegetarian, 120 VCaps,
Enzymes, All Complete, 250 mg, 90 CapsVitamin A 50,000 IU daily. If you are pregnant, do not exceed 10,000 IU daily. Aids in the maintenance and repair of epithelial tissue, which makes up the mucous membrane. Needed for tissue repair and boosts immune function. Use emulsion form for better absorption and greater safety at higher doses.
Vitamin A, 10,000 IU, 100% Natural, Nature's Way, 100 SoftgelsPlus
Natural Carotenoid Complex With Beta Carotene25,000 IU daily. Potent antioxidant and precursor of vitamin A.
Beta Carotene (Natural Dunaliella Salina), Nature's Way, 100% Natural, 25,000 IU, 100 Softgels,
Multi-Carotene Antioxidant, Nature's Way, 60 SoftgelsVitamin B Complex
Plus Extra
Vitamin B-2 (Riboflavin)100 mg 3 times daily with meals. Amounts of individual vitamins in a complex will vary. Riboflavin: 50 mg 3 times daily. Aids in digestion, healing, and tissue repair.
Ultimate B (Vitamin B Complex), Nature's Secret, 60 Tabs,
Vitamin B-100 Complex, w/ Coenzyme B-2, Nature's Way, 631 mg, 100 CapsVitamin B-12 1,000-2,000 mcg daily on an empty stomach. Needed for proper digestion and assimilation of nutrients, including iron. Use a lozenge, spray, or sublingual form.
Vitamin B-12 Complex Liquid, NOW Foods, 2 fl. oz.Vitamin C With Bioflavonoids 3,000-6,000 mg daily, in divided doses. Boosts immune function and tissue repair.
Vitamin C Liquid w/ Rose Hips & Bioflavonoids, Kosher, Natural Citrus Flavor, Dynamic Health, 1000 mg, 16 fl. oz.,
Ester C With Bioflavonoids, Nature's Way, 1000 mg, 90 Tabs,
Vitamin C 1000 With Bioflavonoids, Nature's Way, 100% Natural, 1000 mg, 250 VCaps,
The Right C, Nature's Way, 1000 mg, 120 TabsVitamin K
Or AlfalfaVitamin K: 100 mcg. Alfalfa: As direction on label. Fat-soluble vitamins are not absorbed well in this disorder. Alfalfa supplies vitamin K and necessary minerals, which is often deficient in those with this disorder. A Good source of chlorophyll. Needed for proper digestion.
Chlorophyll With Alfalfa Powder, NOW Foods, 100 mg, 90 Caps,
Full Spectrum Vitamin K, Allergy Research Group / Nutricology, 90 SoftGels,
Alfalfa, NOW Foods, 650 mg, (10 Grain), 500 Tabs.Important Essential Fatty Acids
(Primrose Oil)As directed on label. Relieves inflammation.
Ultimate Oil, Essential Fatty Acids Supplement, Nature's Secret, 90 SoftGels,
Barlean's Primrose Oil, With Gamma Linolenic Acid (GLA), 1300 mg, 120 SoftGelsProtein Supplement As directed on label. Needed for healing. Use protein from a vegetable source or a free form amino acid complex.
Protein Supplements & Products, Assorted types from soy, rice, or whey. Obtain and use the specific type for your particular dietary needs.Free-Form Amino Acid Complex As directed on label. Take on an empty stomach. Supplies protein in a form that is rapidly absorbed and assimilated. Needed for healing
AMINO 1000 Complete, NOW Foods, 120 Caps,
Branched Chain Amino Acid Powder, NOW Foods, 12 oz.,
Amino 1500, Chewable Blend of 20 Amino Acids, Predigested, NOW Foods, 150 Tabs,
Branched Chain Amino Acids, Free Form, 120 CapsVitamin E 400-1,000 IU daily. Start with 100-200 IU daily and slowly increase to 400-1,000 IU daily. An antioxidant necessary for tissue repair. Emulsion form is recommended because it provides for easier assimilation and greater safety at high doses.
Ester E Natural Vitamin E, California Natural, 400 IU, 60 Softgels,
Vitamin E, 400 IU, 100% Natural, NOW Foods, 100 Gels,
Vitamin E-1000, NOW Foods, 1000 IU, 100 Gels,
Vitamin E, d-alpha-tocopherol, 400 IU, 100 Softgels
Zinc 50 mg daily.
For Lozenges: 1 15-mg lozenge 5 times daily. Do not exceed a total of 100 mg daily from all supplements.Needed for immune function and healing of tissue. Zinc gluconate lozenges for best absorption.
Zinc Ionic Mineral Supplement, Fully Absorbable, 100 +/- ppm, 16 fl. oz.,
Colloidal Silver & Zinc Lozenges, Silva Solution, 90 Lozenges,
Zinc Lozenges W/ Echinacea & Vitamin C, Nature's Way, 23 mg, 60 Lozenges,
Zinc Ionic Mineral Supplement, Fully Absorbable, 100 +/- ppm, 16 fl. oz.,
Zinc (Chelated), 100% Natural, Nature's Way, 30 mg, 100 CapsHelpful Coenzyme Q-10 100 mg daily. Acts as an immunostimulant.
CoQ-10, 100% Pure Powder, Vegetarian, NOW Foods, 1 oz.,
CoQ-10, Liquid, NOW Foods, 100 mg/teaspoon, 4 oz.,
CoQ-10, Liquid, W/Trace Minerals, 50 mg/teaspoon, 4 fl. oz.Coenzyme A
Coenzyme A TechnologiesAs directed on label. Supports the immune system's detoxification of many dangerous substances.
Pantethine (Coenzyme A Precursor), Highly Active Form of Vitamin B-5, NOW Foods, 300 mg, 60 Softgels,Copper 3 mg daily. Low levels have been linked to cystic fibrosis.
Copper Ionic Mineral Supplement, Fully Absorbable, 50 +/- ppm, 16 fl. oz.,Selenium 200 mcg daily. Do not exceed a total daily intake of 800 mcg daily from all sources, including diet. If you are pregnant, do not exceed 40 mcg daily. Low levels of selenium h ave been linked to cystic fibrosis. Caution: Do not take supplemental selenium if you are pregnant or have heart, kidney, or liver disease.
Selenium Supplement, Yeast Free, NOW Foods, 200 mcg, 180 Caps,
Selenium Ionic Mineral Supplement, Fully Absorbable, 50 +/- ppm, 16 fl. oz.,
Ionic Selenium, Trace Minerals, 300 mcg, 2 fl. oz.,
Selenium, 100% Natural, Nature's way, 200 mcg, 100 CapsOr
"Green Drinks"
Fresh Wheatgrass Juice
Such as Kyo-Green from WakunagaAs directed on label. Concentrated barley and wheatgrass juice; contains nutrients needed for healing. To supply minerals and chlorophyll needed to control infection. Use liquid or tablet form. Also prepare fresh "green drinks" from green leafy vegetables.
L-Cysteine
And
L-Methionine500 mg twice daily, on an empty stomach. Take with water or juice. Do not take with milk. Take with 50 mg vitamin B-6 and 100 mg vitamin C for better absorption. Needed for repair of lung tissue and to protect the liver.
L-Cysteine Structural Support W/Vitamin B-6 & C, Vegetarian, NOW Foods, 500 mg, 100 Caps,
L-Methionine, NOW Foods, Plus B-6, 500 mg / 10 mg, 100 CapsLipoic Acid As directed on label. Helps the pancreas to function properly and controls the metabolism of sugar.
Alpha Lipoic Acid, NOW Foods, 250 mg, 60 CapsMethylsulfonylmethane (MSM) As directed on label. Clinical tests have proven that this supplement combats lung damage.
MSM Supplement (Methylsulfonylmethane), 1000 mg, 120 Caps Pure Lignisul W/ Free 4 oz. Lotion,
MSM Liquid (Methylsulfonylmethane), Pure Lignisul, 16 fl. oz.Pycnogenol As directed on label. A powerful antioxidant that also protects the lungs.
Pycnogenol W/ Vitamin E, 100% Natural, Nature's Way, 50 mg, 30 TabsRaw Pancreas
And
Raw Spleen
And
Raw Thymus Glandulars500 mg each twice daily. To stimulate proper immune function and to relieve inflammation.
Pancreas (Beef), Organic Glandular, Hypoallergenic, Allergy Research Group / Nutricology, 90 Caps,
Thymus Organic Glandular, Hypoallergenic, Allergy Research Group / Nutricology, 75 Caps,
Immuno-Gland Plex, Organic Glandular (Adrenal, Pancreas, Thymus, Spleen), 125 mg each gland, Hypoallergenic, Allergy Research Group / Nutricology, 60 CapsVitamin D 400 IU daily. Aids in protecting the lungs.
Vitamin D, Nature's Way, 100% Natural, 400 IU, 100 Caps
CYSTIC FIBROSIS DISEASE SUPPLEMENTS
Information, products and supplements for Cystic Fibrosis, a chronic, inherited disease that causes mucus in the body to become thick, dry and sticky.
Alive! Whole Food Energizer Multi-Vitamin & Mineral With Naturally Occurring Iron (No Iron Added), Nature's Way, 909 Tabs
No other supplement contains more life-giving nutrients than Nature's Way ALIVE Multi Vitamin. ALIVE Multi Vitamin is better absorbed into your blood stream because its tablets disintegrate up to 5X faster than other leading brands.Alfa Max Alfalfa Extract, Nature's Way, 525 mg, 100 Caps
Nature's Way Alfa-Max Alfalfa Extract capsules is a 10x extract of fresh green Alfalfa leaves.Branched Chain Amino Acids, Free Form, 120 Caps
The branched chain amino acids (BCAA's) leucine, isoleucine and valine are three of the eight essential amino acids the human body must receive in food.Calcium Ionic Mineral Supplement, Fully Absorbable, 700 +/- ppm, 16 fl. oz.
WaterOz Ionic Calcium is a pure liquid Calcium supplement. Calcium is one of the most essential of the major biochemical elements needed in human nutrition. It is needed in every organ of the body, including the brain. Calcium is called the "knitter" because it promotes healing or knitting everywhere in the body. It is valuable for tone, power, strength, longevity, vitality, and endurance, healing of wounds, counter-acting acids, and helping regulate metabolism.Cayenne & Garlic Supplement, Nature's Way, 100 Caps
Nature's Way Cayenne and Garlic Combo gives the benefit of cayenne herb which is that it has an invigorating warming effect and the serum cholesterol lowering and Garlic bulb to support healthy circulation. Cayenne & Garlic Supplement contains Certi-FIERY Cayenne (40,000 Heat Units). Cayenne is combined with Garlic to help maintain good health.Copper Ionic Mineral Supplement, Fully Absorbable, 50 +/- ppm, 16 fl. oz.
WaterOz Ionic Copper is a pure liquid Copper supplement. Copper influences protein metabolism and general healing, improves vitamin c oxidation and is integral in the formation of RNA.Digestive Enzymes, 750 mg, 100 Caps
Since cooking destroys many of the digestive enzymes in food, taking plant fiber-based digestive enzymes aids in digesting even the heaviest meal.Echinacea Root Tincture, 100% Organic, 2 fl. oz.
Echinacea Root is also known as Purple Coneflower and considered very valuable by the North American Indians.Essential Fatty Acids For Baby & Children, Organic, 8 fl. oz.
Excellent for Infants and Children - All aspects of a child's physical, emotional and mental growth and function are dependent upon EFAs. Essential Fatty Acids are called essential because they are required for so many important functions in the body and brain.Horehound Tincture, 100% Organic, 2 fl. oz.
Horehound herb is often used to treat respiratory disorders and has 350 years of history as a soothing expectorant.Hyssop Essential Oil, NOW Foods, 100% Pure, 1 fl. oz.
This essential oil has a pleasant mild fragrance which is useful in aromatherapy. Hyssop oil can also be used to make a chest rub by diluting 10 drops in a 1 oz base of almond oil or olive oil.Instant Energy B-12, NOW Foods, 2000 mcg, 75 Packets
NOW® Vitamin B-12 Instant Energy is the perfect way to keep alert and energized, regardless of the situation. These convenient, fast-acting packets are perfect when traveling or for convenience.Lactobacillus Acidophilus Supplement, Non-Dairy, 100 Caps
Lactobacillus Acidophilus Supplement taken as a dietary supplement may help to detoxify and to rebuild a balanced intestinal flora.Multi-Vitamin Without Iron, Nature's Way, 100 Caps
The vitamins and minerals in this Multi Vitamin without Iron play many important roles in the body: antioxidants to protect fats, cells and DNA, coenzyme precursors for energy production and metabolism, and cofactors for hormones and enzymes which regulate body processes.Protein From Rice Powder, Dairy Free, Vegetarian / Vegan, Hypoallergenic, Chocolate, 1 lb 6.9 oz.
A great tasting vegetable protein made from non-genetically modified California grown brown rice. Free of the common food allergens from soy, milk, egg, wheat and yeast.Protein From Rice Powder, Dairy Free, Vegetarian / Vegan, Hypoallergenic, Mixed Berry, 1 lb 5 oz.
A great tasting vegetable protein made from non-genetically modified California grown brown rice. Free of the common food allergens from soy, milk, egg, wheat and yeast.Protein From Rice, Powder, Dairy Free, Vegetarian / Vegan, Hypoallergenic, Plain, 1 lb 5 oz.
A great tasting vegetable protein made from non-genetically modified California grown brown rice. Free of the common food allergens from soy, milk, egg, wheat and yeast.Protein From Rice, Powder, Dairy Free, Vegetarian / Vegan, Hypoallergenic, Vanilla, 1 lb 5 oz.
A great tasting vegetable protein made from non-genetically modified California grown brown rice. Free of the common food allergens from soy, milk, egg, wheat and yeast.Selenium, 100% Natural, 200 mcg, 100 Caps
WaterOz Ionic Selenium is pure liquid Selenium supplement. Selenium promotes normal body growth, enhances fertility, encourages tissue elasticity and is a potent antioxidant that naturally reduces the retention of toxic metals in the body. Selenium is crucial for the proper functioning of the heart muscle and there is evidence that it can help the body fight cancer.Selenium Ionic Mineral Supplement, Fully Absorbable, 50 +/- ppm, 16 fl. oz.
Selenium is an essential component of glutathione, the body's most potent natural antioxidant system. It is a popular choice in many antioxidant regimens.Super Digestive Enzymes, 90 Caps
Super Digestive Enzymes contain a complete and balanced digestive enzyme formula.Vitamin A, 10,000 IU, 100% Natural, Nature's Way, 100 SoftGels
Nature's Way vitamin A is 100% natural from fish liver oil. It contains no artificial ingredients or preservatives.Vitamin B-12 Liquid Supplement, 50 mcg, & vitamin B-9 (Folic Acid), 400 mcg, 1 fl. oz.
This liquid B-12 supplement with its combination of B vitamins can be a very effective revitalizer in supporting the nervous system, prenatal child health, vegetarians, and heart health.Vitamin B-12 Complex Liquid, NOW Foods, 2 fl. oz.
Vitamin B-12 Helps to maintain a healthy nervous system, and helps in the prevention of pernicious anemia. Folic Acid should be taken by all females of child bearing age to help prevent neuro-tube birth defects. Folic acid is also linked to heart health.Vitamin C 1000 With Bioflavonoids, Nature's Way, 100% Natural, 1000 mg, 250 VCaps
Nature's Way Vitamin C with Bioflavonoids provides antioxidant protection for many of the body's important enzyme systems.Vitamin E, Natural D-Alpha-Tocopherol, 400 IU, 100 SoftGels
Vitamin E has potent antioxidant activity, supplies oxygen to the blood, aids in strengthening capillary walls, and plays a beneficial role in cancer and cardiovascular disease prevention, anti-aging benefits, circulation, wound-healing, immune function, nervous system function, PMS, hot flushes, diabetes, vascular disease, eye health, tissue repair, athletic performance, leg cramps, skin and hair health, and alleviating fatigue.Vitamin E Oil, D-Alpha, Vegetarian, 100% Natural, 32,000 IU, NOW Foods, 1 oz.
This vitamin E oil may be used as a dietary supplement or applied directly to the skin for cosmetic or other purposes.Zinc Ionic Mineral Supplement, Fully Absorbable, 100 +/- ppm, 16 fl. oz.
WaterOz Ionic Zinc is a pure liquid Zinc supplement. Zinc moves through all the fluids in the body, it creates a defense against infection-causing bacteria and viruses trying to enter the body and stops bacterial and viral replication.Zinc Lozenges With Echinacea & Vitamin C, Nature's Way, 23 mg, 60 Lozenges
Nature's Way Zinc lozenge boosts cold season defense with zinc, widely recognized as an important nutritional support during the cold season, and echinacea pupurea, clinically shown to support the immune system, and Vitamin C, a vitally important vitamin for general health maintenance.
HerbalRemedies: Cystic Fibrosis Information
HerbalRemedies: Cystic Fibrosis supplements & Products
HELPFUL LINKS
Further information about cystic fibrosis is available from:Cystic Fibrosis Foundation
6931 Arlington Road
Bethesda, MD 20814
Tel: 800-FIGHT-CF (800-344-4823)
Tel: 301-951-4422
Fax: 301-951-6378
Website: www.cff.org
News, publications, sponsors, mailing list.
CysticFibrosis.com
Cystic Fibrosis Resources for Daily Living and Information.
Cystic Fibrosis Mutation Data Base
Cystic Fibrosis Research Inc.
MedlinePlus: Cystic Fibrosis
Links to various Cystic Fibrosis resources.
PREVENTION
If both would-be parents are carriers of the CF gene, they may wish to consider their chances of having a child with CF. But once a child is born with the defective genes that cause the disease, there are no treatments available that can prevent CF from developing (although this is the goal of genetic research).
NOTIFY YOUR MIDWIFE OR HEALTH CARE PROVIDER IF...
You are pregnant or planning to become pregnant and you (or the other future biological parent) have a family history of CF.
You suspect your child has CF. Signs and symptoms in newborns may include failure to grow, chronic respiratory problems especially recurrent pneumonia and frequent bulky, greasy stools. Older children also may have diarrhea and frequent respiratory infections, including pneumonia. Failure to grow steadily on the growth percentile curve may also be a sign of CF, especially if other signs and symptoms are present.
Your child tastes very salty when you kiss him or her.
Contact your health care provider quickly if, after diagnosis, your child develops a fever, a worsening cough, breathing difficulties, change in level of tiredness or decreased appetite.
Your child received a diagnosis of CF, you will need to arrange regular visits with your CF clinical center. Your child will also need to see your family health care provider on a regular basis to have medications and any lung or digestive problems monitored.
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HELPFUL PRODUCTS & FURTHER EDUCATION
Prescription for Nutritional Healing: The A-To-Z Guide To Supplements
-- by Phyllis A. Balch, James F. Balch - 2nd Edition
Prescription for Nutritional Healing: The A-To-Z Guide To Supplements
-- by Phyllis A. Balch, James F. Balch - 4th Edition
Prescription for Herbal Healing: The A-To-Z Reference To Common Disorders
-- by Phyllis A. Balch
The Complete Guide to Natural Healing
This is only a small sample of educational materials and products available through Amazon.com. Use the search box provided below to search for a particular item.
HERBS, OILS, & SUPPLIES
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