Cystic fibrosis (CF) is a chronic, progressive, and frequently fatal genetic
disease of the glands that produce or secrete sweat and mucus. CF primarily
affects the respiratory, digestive, and reproductive tracts in children and
young adults. On average, individuals with CF have a lifespan of around 30
According to the Cystic Fibrosis Foundation, there are about 30,000 Americans
with CF. The disease occurs mostly in whites whose ancestors came from northern
Europe, although it cuts across all races and ethnic groups. Approximately 2,500
babies are born with the disease each year in the United States. Also, about one
in every 20 Americans, or 12 million people, are unaffected carriers of an
abnormal CF gene.
|Signs and Symptoms|
CF is often accompanied by the following signs and symptoms.
- Thick, viscous mucus secretions in the lungs
- Changes in color and amount of sputum (material coughed up from the
- Chronic cough, possibly with blood streaking
- Chronic sinusitis
- Nasal polyps (fleshy growths inside the nose)
- Weight loss, failure to thrive in infants, abdominal
- Excessive salt in sweat, dehydration
- Failure of newborn to pass stool
- Abdominal pain, flatulence
Conditions such as late onset of puberty, intestinal obstruction,
inflammation of the pancreas, cirrhosis (a liver condition), and infertility may
also be signs of CF.
|What Causes It?|
CF is caused by a mutation in the gene that produces the protein that moves
chloride ions (a component of sodium chloride, or common table salt) through
cell membranes. This protein is present in cells lining the passageways of the
lungs, pancreas, colon, and genitourinary tract. When this protein is abnormal,
the movement of chloride ions and water in the lung and other cells becomes
blocked, and there is secretion of abnormal mucus.
|Who's Most At Risk?|
CF is caused by inherited genes. To have CF, a child must inherit two
abnormal genes—one from each
|What to Expect at Your Provider's Office|
A baby born with the CF gene usually has symptoms during its first year,
although signs of the disease may not appear until adolescence or even
Your child's health care provider can help make a diagnosis and guide you in
determining which treatment or combination of therapies will best alleviate
symptoms of the disease. He or she will perform a physical exam and run
laboratory tests, including a sweat test, which checks for higher-than-normal
amounts of sodium and chloride in the sweat. Other tests include a sputum test,
genetic screening, and a stool analysis. Imaging techniques may be used to
reveal lung conditions and abdominal obstruction.
Preventing CF is not currently possible.
The hope for the future is that gene therapy could repair or replace the
defective CF gene. Or, a person with CF might be given the active form of the
protein product that is scarce or missing. At present, the best that health care
providers can do is to ease the symptoms of CF or slow the progress of the
disease in order to improve the patient's quality of life.
CF patients suffer from frequent lung infections caused by obstructed
breathing. So, the mainstays of a treatment plan are physical therapy, exercise,
and medications for reducing the mucus blocking the lung's
Medications are often aerosolized (misted) and can be inhaled. These include
- Bronchodilators (which widen the breathing tubes)
- Mucolytics (which thin the mucus)
- Decongestants (which reduce swelling of the membranes of the breathing
- An enzyme that thins the mucus by digesting the cellular material
trapped in it
- Antibiotics to fight lung infections also are
The digestive problems caused by CF are managed with these
- Pancreatic enzymes which help digestion
- Enemas and mucolytic agents to treat intestinal
|Surgical and Other Procedures|
CF patients with respiratory failure may need a heart-lung transplant. Those
patients experiencing gastrointestinal obstruction may also require
|Complementary and Alternative Therapies|
A comprehensive treatment plan for CF may include a range of complementary
and alternative therapies.
CF patients should not eat large quantities of the following foods, which
increase mucus production or cause allergies: dairy (especially milk, cheese,
and ice cream), eggs, peanuts, oranges, bananas, sugar, saturated fats, wheat,
barley, oats, rye, excessive meat, and salt.
CF patients should eat large quantities of foods that reduce mucus or
inflammation, including: garlic, onions, watercress, horseradish, mustard,
umeboshi plums, parsley, celery, rose pits tea, pickles, lemon,
anti-inflammatory oils (nuts, seeds, cold-water fish).
Potentially beneficial nutrient supplements include the
- Pancreatic enzymes with meals (1,000 lipase units per kg for patients
under 4 years, 500 units over 4 years)
- Antioxidants: selenium 200 mcg a day, vitamin E 400 IU a day,
beta-carotene 25,000 IU a day, and vitamin C 250 to 500 mg two times daily, all
of which help protect lungs
- N-acetyl cysteine 200 mg three times a day to help thin
- Zinc 10 to 30 mg a day to support immunity
- Quercetin (250 mg to 500 mg before meals) to reduce
The use of certain herbal remedies may offer relief from
- To liquefy mucus: mix equal parts of four to six of the following
herbs: thyme (Thymus vulgaris), Indian tobacco (Lobelia inflata),
anise (Pimpinella anisum), hyssop (Hyssopus officinalis), licorice
root (Glycyrrhiza glabra), and rosemary (Rosemarinus officinalis);
20 to 60 drops two to four times daily.
- To stimulate the pancreas: mix equal parts of blue flag (Iris
versicolor), dandelion (Taraxacum officinalis), and fringe tree
(Chionanthus virginicus); 10 to 15 drops in warm water before
- For acute infection: combine equal parts of coneflower (Echinacea
purpurea), goldenseal (Hydrastis canadensis), thyme (Thymus
vulgaris), wild indigo (Baptisia tinctoria), and elecampane (Inula
helenium) with 15 drops of cayenne (Capsicum annuum); 20 to 30 drops
every three to four hours
Herbs are generally available as dried extracts (pills, capsules, or
tablets), teas, or tinctures (alcohol extraction, unless otherwise noted). Dose
for teas is 1 heaping tsp./cup water steeped for 10 minutes (roots need 20
Although very few studies have examined the effectiveness of specific
homeopathic therapies, professional homeopaths may consider the following
treatments to alleviate respiratory symptoms (such as those experienced from
cystic fibrosis) based on their knowledge and experience. Before prescribing a
remedy, homeopaths take into account a person's constitutional type. A
constitutional type is defined as a person's physical, emotional, and
psychological makeup. An experienced homeopath assesses all of these factors
when determining the most appropriate treatment for each individual.
The following treatments should be used under the guidance of a licensed,
certified homeopath in addition to standard medical care provided by a medical
- Antimonium tartaricum -- for wet, rattling cough (although the
cough is usually too weak to bring up mucus material from the lungs) that is
accompanied by extreme fatigue and difficulty breathing; symptoms usually worsen
when the person is lying down
- Carbo vegetabilis -- for shortness of breath with anxiety,
chills, and bluish skin discoloration
Acupuncture may help stimulate respiratory function and enhance
Therapeutic massage can help drain mucus from the
Respiratory problems are the most common complication from
CF patients receive pulmonary function tests every three to six months. They
also receive chest X rays every two to four years, or more often if
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|Review Date: March 2000|
|Reviewed By: Leonard Wisneski, MD, FACP, George Washington University, Rockville,
Copyright © 2004 A.D.A.M., Inc
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