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 years.
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 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
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 parent.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 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
PreventionPreventing CF is not currently possible.
Treatment Plan
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
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
- Pancreatic enzymes which help digestion
- Enemas and mucolytic agents to treat intestinal obstructions
Surgical and Other Procedures
CF patients with respiratory failure may need a heart-lung transplant. Those patients experiencing gastrointestinal obstruction may also require surgery.Complementary and Alternative Therapies
A comprehensive treatment plan for CF may include a range of complementary and alternative therapies. NutritionCF 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 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 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 mucus
- Zinc 10 to 30 mg a day to support immunity
- Quercetin (250 mg to 500 mg before meals) to reduce inflammation
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
Homeopathy
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 doctor:
- 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 immunity.
Massage
Therapeutic massage can help drain mucus from the lungs.
Prognosis/Possible Complications
Respiratory problems are the most common complication from CF.Following Up
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 needed.References
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