Cystic Fibrosis

August 26, 2010 in Conditions: C >, Cystic Fibrosis by admin

Cystic fibrosis (CF) is a chronic, progressive, and frequently fatal genetic disease of the glands that produce or secrete sweat and mucus. Cystic Fibrosis primarily affects the respiratory, digestive, and reproductive tracts in children and young adults. On average, individuals with Cystic Fibrosis have a lifespan of around 30 years.

According to the Cystic Fibrosis Foundation, there are about 30,000 Americans with Cystic Fibrosis. 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 Cystic Fibrosis gene.

Signs and Symptoms of Cystic Fibrosis

Cystic Fibrosis 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 lungs)
- Chronic cough, possibly with blood streaking
- Wheezing
- Bronchitis
- Chronic sinusitis
- Asthma
- Nasal polyps (fleshy growths inside the nose)
- Weight loss, failure to thrive in infants, abdominal swelling
- Excessive salt in sweat, dehydration
- Failure of newborn to pass stool
- Abdominal pain, flatulence
- Fatigue

Conditions such as late onset of puberty, intestinal obstruction, inflammation of the pancreas, cirrhosis (a liver condition), and infertility may also be signs of Cystic Fibrosis.

What Causes Cystic Fibrosis?

Cystic Fibrosis 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 for Cystic Fibrosis?

Cystic Fibrosis is caused by inherited genes. To have Cystic Fibrosis, a child must inherit two abnormal genes – one from each parent.

What to Expect at Your Provider’s Office

A baby born with the Cystic Fibrosis gene usually has symptoms during its first year, although signs of the disease may not appear until adolescence or even later.

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.

Treatment Options for Cystic Fibrosis

Prevention

Preventing Cystic Fibrosis is not currently possible.

Treatment Plan for Cystic Fibrosis

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 airways.

Drug Therapies for Cystic Fibrosis

Medications are often aerosolized (misted) and can be inhaled. These include the following.
- Bronchodilators (which widen the breathing tubes)
- Mucolytics (which thin the mucus)
- Decongestants (which reduce swelling of the membranes of the breathing tubes)
- An enzyme that thins the mucus by digesting the cellular material trapped in it
- Antibiotics to fight lung infections also are used

The digestive problems caused by Cystic Fibrosis are managed with these medications.
- Pancreatic enzymes which help digestion
- Enemas and mucolytic agents to treat intestinal obstructions

Surgical and Other Procedures for Cystic Fibrosis

Cystic Fibrosis patients with respiratory failure may need a heart-lung transplant. Those patients experiencing gastrointestinal obstruction may also require surgery.

Complementary and Alternative Therapies for Cystic Fibrosis

A comprehensive treatment plan for CF may include a range of complementary and alternative therapies.

Nutrition for Cystic Fibrosis

Cystic Fibrosis 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.

Cystic Fibrosis 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 following.
- 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 500 to 1,000 mg two to three times daily, all of which help protect lungs
- N-acetyl cysteine 200 mg three times a day to help thin mucus
- Zinc 10 to 30 mg a day to support immunity
- Quercetin (250 mg to 500 mg before meals) to reduce inflammation

Herbal Remedies for Cystic Fibrosis

The use of certain herbal remedies may offer relief from symptoms.
- 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 meals
- 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 minutes).

Homeopathy for Cystic Fibrosis

An experienced homeopath can prescribe a regimen for treating CF that is designed especially for your child. Some of the most common acute remedies are listed below.
- Antimonium tart for rattling, unproductive cough
- Carbo vegetabilis for difficult breathing
- Laurocerasus for collapsed lungs

Acute dose is three to five pellets of 12X to 30C every one to four hours until symptoms are relieved.

Acupuncture

Acupuncture may help stimulate respiratory function and enhance immunity.

Massage

Therapeutic massage can help drain mucus from the lungs.

Prognosis/Possible Complications for Cystic Fibrosis

Respiratory problems are the most common complication from CF.

Following Up

Cystic Fibrosis 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 needed.

Supporting Research on Cystic Fibrosis

Bartram T. Encyclopedia of Herbal Medicine. Dorset, England: Grace Publishers; 1995:138.

Behrman R. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, Pa: W.B. Saunders; 1996.

Blumenthal M, ed. The Complete German Commission E Monographs, Boston, Mass: Integrative Medicine Communications; 1998:466.

Bone R. Pulmonary & Critical Care Medicine. 1998 ed. St. Louis, Mo: Mosby-Year Book; 1998.

Dambro MR. Griffith’s 5-Minute Clinical Consult. 1999 ed. Baltimore, Md: Lippincott Williams & Wilkins, Inc.; 1999.

Fauci AS, Braunwald E, Isselbacher KJ, et al, eds. Harrison’s Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill; 1998.

Morrison R. Desktop Guide to Keynotes and Confirmatory Symptoms. Albany, Calif: Hahnemann Clinic Publishing; 1993:25, 26, 103, 221.

Rakel RE, ed. Conn’s Current Therapy. 50th ed. Philadelphia, Pa: W.B. Saunders; 1998.

Yamada T, ed. Textbook of Gastroenterology. 2nd ed. Philadelphia, Pa: Lippincott-Raven Publishers; 1995.

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