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Overview |
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Definition |
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Cystic fibrosis (CF) is a fatal, multisystem disease. It is autosomal
recessive, resulting from mutations in the CF transmembrane regulator (CFTR)
protein, which is a chloride channel found in exocrine tissue. Prevalence varies
by ethnicity. In the United States, CF is diagnosed once in every 2,000 to 3,000
Caucasian live births, but once in every 17,000 African American live births. CF
typically is diagnosed in childhood after a chronic lung disease or obstruction.
Significant improvements in treatment have pushed the median survival rate to
approximately 30 years of age. |

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Etiology |
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CF is a genetically inherited defect, causing abnormal chloride and sodium
secretions across epithelial cell membranes. |

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Risk Factors |
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Genetic predisposition |

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Signs and Symptoms |
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- Chronic obstructive lung disease—thick,
viscous mucus secretions; repeated infection
- Changes in color and amount of sputum
- Chronic cough, possibly with blood streaking
- Fever
- Wheezing
- Bronchitis
- Chronic sinusitis
- Asthma
- Nasal polyps
- Malnutrition—weight loss, failure to thrive
in infants, abdominal distention
- Excessive salt in sweat, dehydration
- Meconium ileus—failure of infant to pass
stool
- Intestinal obstruction—distention, pain,
flatulence, weight loss
- Fatigue
- Pancreatitis, cirrhosis
- Late onset of puberty,
infertility
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Differential
Diagnosis |
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- Asthma
- Appendicitis
- Lymphoma
- Sarcoidosis
- Pneumonia
- Tuberculosis
- Immotile cilia syndrome
- Eosinophilic granuloma
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Diagnosis |
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Physical Examination |
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Patient appears with apparent weight loss, chronic cough, mucus-filled
secretions, increased chest diameter, and possibly distended abdomen. Nasal
obstruction and polyps are often visible. |

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Laboratory Tests |
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- Sputum test—culture bacteria to determine
choice of antibiotic therapy
- Sweat analysis—more than 60 mEq/L of chloride
indicates CF
- Genetic screening—supports
diagnosis
- 72-hour fecal fat analysis of
stool
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Pathology/Pathophysiology |
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All affected tissue has abnormal ion transport function; otherwise, pathology
is organ-specific.
Pulmonary:
- Increased transepithelial electrical
potential—increased sodium ion rates and decreased
chloride ion permeability, resulting in dehydrated, mucus-filled
lungs
- Chronic infections—Pseudomonas
aeruginosa and other Pseudomonas, Staphylococcus aureus
- Increased viscosity—limits ciliary motility,
allows bacterial growth
- Hyperchloremic, thick secretions
- Electrolyte imbalance
- Lung abscess
- Eventually, bronchiolitis, bronchiectasis, digital clubbing, cor
pulmonale, irreversible lung failure
Gastrointestinal:
- Pancreatic exocrine enzyme
insufficiency—epithelium, lacking chloride and water
secretion, cannot flush mucins from intestinal crypts, which eventually destroys
the pancreas; steatorrhea; malabsorption of fat-soluble vitamins
- Edematous ileocecal bowel with obstruction
- Liver—deficient hepatic chloride and water
secretions leads to retention of biliary secretions and cirrhosis
- Deficient gallbladder salt and water secretions result in
cholecystitis and cholelithiasis
Sweat glands: impaired chloride and sodium absorption across ductal
epithelial cells |

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Imaging |
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- Chest radiography—hyperinflation,
peribronchial thickening, mucus plugs, cystic cavities,
bronchiectasis
- Computerized tomography—bronchiectasis,
localized airway thickening
- Abdominal radiograph—for abdominal
obstruction
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Other Diagnostic
Procedures |
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- Spirometric measurement
- Serum concentration of immunoreactive
trypsin—infant screening
- Clinical features—including foul-smelling,
large, greasy stools
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Treatment Options |
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Treatment Strategy |
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CF is a fatal disease, but complications can be delayed with careful
management. Any of the following general measures may be part of the treatment
plan.
- Chest physiotherapy—aids in preventing
disease progression; manual percussion, postural drainage, breathing exercises;
20 to 30 min tid to qid; various mechanical modalities available (e.g., ThAirapy
Vest)
- Oxygen—alleviates dyspnea, may reduce
pulmonary hypertension
- Gastrointestinal obstruction—water-soluble
contrast enema; polyethylene glycol-electrolyte solution
- Liver, gallbladder—conventionally
treated
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Drug Therapies |
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Anti-inflammatory drugs—reduces rate of lung
deterioration; use for concurrent asthma.
- Ibuprofen—titrate to peak serum level of 50
to 100 mcg/mL, monitor renal function
- Prednisone—1 to 2 mg/kg every other day; side
effects include growth failure, hypertension, cataracts
Recombinant human DNAse—reduces viscosity of airway
secretions, increasing air flow; 2.5 mg vials daily or bid, delivered via
nebulizer.
Inhaled beta-adrenergic agonists or
anticholinergics—short-term control of airway
constriction.
Antibiotics
- Oral— for increased cough or mucus;
cephalosporins or a semisynthetic penicillin
- Inhaled: for bacterial suppression—tobramycin
(300 to 600 mg bid) 28 day cycle, via a compressor and nebulizer; side
effect—ototoxicity; for acute
exacerbation—gentamicin 80 mg tid, 2 mL of solution
added to 1 mL of normal saline via a standard home nebulizer; side effect:
ototoxicity
- Intravenous—aminoglycosides typically combine
with another class; hospitalize because symptoms worsen as secretions mobilize;
monitor renal function
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Surgical Procedures |
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- Heart-lung transplant—significant improvement
in pulmonary function, no disease recurrence, 50% survival rate at 5
years
- Gastrointestinal obstruction—may require
surgery
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Complementary and Alternative
Therapies |
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Nutritional and herbal therapies may help decrease the severity of
progression, protect organ function, and minimize opportunistic infections in
cystic fibrosis. Homeopathy can be used concurrently, preferably
constitutionally, although acute remedies may be used as
needed. |

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Nutrition |
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- Diet can profoundly affect the severity of cystic fibrosis. It is
imperative to limit foods that increase mucus production: dairy (especially
milk, cheese, and ice cream), peanuts, oranges, bananas, sugar, saturated fats,
wheat and gluten-containing grains (barley, oats, and rye), excessive meat, and
salt. Foods that reduce mucus should be liberally included in the diet (garlic,
onions, watercress, horseradish, mustard, umeboshi plums, parsley, celery, rose
hips tea, pickles, and lemon).
- Remove all food allergens; suspect wheat, dairy, and eggs. Consider a
five-day rotation diet to minimize sensitivities.
- Increase anti-inflammatory oils (nuts, seeds, cold-water fish) and
medium chain triglycerides (caprylic acid).
- Pancreatic enzymes with meals (1,000 lipase units per kg for patients
under 4 years, 500 units over 4 years)
- Antioxidants: selenium 200 mcg/day, vitamin E 400 IU/day,
beta-carotene 25,000 IU/day, and vitamin C 500 to 1,000 mg bid to tid; all
protect lung tissue
- N-acetyl cysteine 200 mg tid to help thin mucus
- Zinc 10 to 30 mg/day to support immunity
- Quercetin (250 to 500 mg before meals) helps to reduce inflammation
secondary to food sensitivities and compromised
digestion.
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Herbs |
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Herbs may be used as dried extracts (pills, capsules, or tablets), teas, or
tinctures (alcohol extraction, unless otherwise noted). Dose for teas is 1
heaping teaspoon herb/cup water steeped for 10 minutes (roots need 20
minutes).
- To liquify 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 bid to qid.
- To stimulate the pancreas, mix equal parts of blue flag (Iris
versicolor), dandelion (Taraxacum officinale), 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.
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Homeopathy |
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An experienced homeopath would consider an individual's constitutional type
to prescribe a more specific remedy and potency. Some of the most common acute
remedies are listed below. Acute dose is three to five pellets of 12X to 30C
every one to four hours until symptoms resolve.
- Antimonium tart for rattling, unproductive cough with cyanosis
about the mouth, weakness, and irritability
- Carbo vegetabilis for dyspnea with anxiety, air hunger, and
collapse
- Laurocerasus for collapsed states with cyanosis, cold sensation
in the chest, and fainting episodes
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Acupuncture |
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May help stimulate respiratory function and enhance
immunity |

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Massage |
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Therapeutic massage, particularly with postural drainage, is an essential
part of treatment of cystic fibrosis. |

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Patient Monitoring |
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- Pulmonary function tests—every three to six
months
- Chest radiography—every two to four years;
yearly with complication
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Other
Considerations |
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Prevention |
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- Regular visits to CF centers lowers morbidity
- Chest physiotherapy delays progression
- Influenza vaccination
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Complications/Sequelae |
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- Pulmonary exacerbation—intravenous
antibiotics; supplemental oxygen; leads to respiratory failure
- Biliary cirrhosis
- Minor hemoptysis—blood streaking in the
sputum
- Major hemoptysis—life threatening, requires
supplemental oxygen, endotracheal intubation, bronchial artery embolization, or
lung resection
- Pneumothorax—rupture of subpleural blebs;
tube thoracostomy, surgery
- Rectal prolapse—from chronic cough,
constipation, diarrhea; rapid manual reduction prevents edema
- Gastroesophageal reflux
- Cardiac disease—secondary to cor
pulmonale
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Prognosis |
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- Respiratory problems—account for 95% of
morbidity and mortality
- Median survival—approximately 30
years
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Pregnancy |
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Women:
- Monitor for diabetes
- Infertility possible from increased cervical
mucus—intrauterine insemination
- Genetic counseling, DNA testing of partner determines degree of
risk
- 90% of completed pregnancies result in viable
births
Men: absence of vas deferens (from altered hypoplasia and prostatic
secretions) contributes to azoospermia |

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References |
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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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Copyright © 2000 Integrative Medicine
Communications This publication contains
information relating to general principles
of medical care that should not in any event be construed as specific
instructions for individual patients. The publisher does not accept any
responsibility for the accuracy of the information or the consequences arising
from the application, use, or misuse of any of the information contained herein,
including any injury and/or damage to any person or property as a matter of
product liability, negligence, or otherwise. No warranty, expressed or implied,
is made in regard to the contents of this material. No claims or endorsements
are made for any drugs or compounds currently marketed or in investigative use.
The reader is advised to check product information (including package inserts)
for changes and new information regarding dosage, precautions, warnings,
interactions, and contraindications before administering any drug, herb, or
supplement discussed herein. | |