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Cystic Fibrosis
 

Cystic Fibrosis Introduction

:cistic_20fibrosis_20small.gif Cystic fibrosis (CF) is a rare genetic disorder that is inherited from both parents. Cystic Fibrosis is caused by a defective gene that codes for a protein responsible for salt (sodium chloride) transport in the body’s exocrine glands. Cystic Fibrosis causes different glands in the body to be defective, including the bronchial glands, pancreas, sweat glands, and glands of the digestive system. Cystic Fibrosis is the most common autosomal recessive genetic disorder in Caucasians. This condition affects Caucasians with northern European ancestry more frequently than other populations. Men and women are affected equally.

Primary Organs Affected:

Lungs: The glands in the lungs and bronchial tubes produce a thick, sticky mucus that creates blockages in the air passages, resulting in breathing difficulties, lung infections, and pneumonia.

Pancreas: The pancreas fails to produce certain digestive enzymes, which, usually, leads to nutritional malabsorption, difficulties with fat metabolism, diarrhea, and foul-smelling stools. Due to the many problems associated with malnutrition, a child with Cystic Fibrosis is often underweight and undersized.

Liver: Digestion and overall liver functioning is disrupted by the plugging of small bile ducts.

Skin: Persons with this condition may exhibit bouts of profuse sweating, caused by the malfunctioning of sweat glands. This often causes the loss of excessive concentrations of mineral salts, especially salts.

Small Intestines: Roughly 1 in 10 newborns will require surgery to remove thick stools, obstructing the gut.

Reproductive: There is often an absence of small ducts in persons suffering from Cystic Fibrosis, such as the vas deferans. This renders one infertile.

Cystic Fibrosis is fatal, with a median survival of 30 years. The Cystic Fibrosis gene was identified in 1989. This discovery has enabled researchers to begin developing new approaches to treatment through gene therapy.

Cystic Fibrosis Symptoms

A cystic fibrosis patient may have any or all of the following symptoms:

  • Recurrent chest infections with a cough and difficulty breathing
  • Diarrhea
  • Greasy, foul-smelling stools
  • Swollen abdomen and wasted limbs
  • Clubbing of the fingers and toes
  • Salty skin

Cystic Fibrosis Symptoms

According to the Cystic Fibrosis Foundation (1):

  • Cystic Fibrosis affects about 30,000 people in the US, 20,000 Europeans, and 3,000 Canadians
  • Approximately 12 million people are carriers in the US
  • 1 in 22 Caucasians are Cystic Fibrosis carriers
  • About 2,500 babies will be born with Cystic Fibrosis this year.
  • Cystic Fibrosis affects 1 in every 1,600 births in Caucasians, 1 in every 13,000 births in African Americans, and 1 in every 50,000 births in Asian Americans

Cystic Fibrosis Treatment

At this time, there is no cure for Cystic Fibrosis. However, early diagnosis and treatment can decrease the risk of permanent damage to the lungs. Cystic fibrosis may be identified by genetic testing, where inner cheek cells are swabbed and then examined for the presence of defective Cystic Fibrosis genes. The definitive test for Cystic Fibrosis is the electrolyte sweat test. This test can detect the excessive amounts of mineral salts found on the skin of Cystic Fibrosis sufferers.

In the case of respiratory infections, an appropriate antibiotic may be prescribed. Pancrelipase (Viokase), a digestive enzyme drug, is often prescribed to further the processes lacking in the pancreas. Other treatments include; drugs in aerosol form to help dissolve lung secretions, a special low-fat diet with nutritional supplementation, physical therapy, and breathing exercises.

Gene therapy may be a beneficial treatment option for cystic fibrosis sufferers in the near future.

Supplements helpful for Cystic Fibrosis

Curcumin (turmeric) Curcumin, the yellow pigment of Curcuma longa (turmeric), has been shown to possess various anti-inflammatory and antioxidant effects (2-5). Curcumin may provide benefit in the treatment of cystic fibrosis. In a recent study, researchers at Yale University found that when curcumin was fed to mice with the defective Cystic Fibrosis protein, it resulted in normal protein activity in the respiratory and gastrointestinal tracts (6).

Omega-3 Essential Fatty Acids (Fish Oil, Flaxseed Oil) Studies show that supplementation with omega-3 fatty acids such as fish oil and flaxseed oil may have beneficial effects for Cystic Fibrosis patients, such as decreasing inflammation and improving lung function (7-9).

Antioxidants (Beta-Carotene, Vitamin C, and Vitamin E) Antioxidant plasma levels, including beta-carotene, vitamin C, and vitamin E, are often low in patients with Cystic Fibrosis. This is due to the impaired digestion and malabsorption in Cystic Fibrosis patients, which reduces the available supply of antioxidant nutrients. Cystic Fibrosis patient’s suffer from difficulties in fat and cholesterol absorption and decreases vitamin E levels, since vitamin E is transported through the body by attaching to LDL cholesterol. The amount of free radicals are also increased in Cystic Fibrosis patients. This is caused by immune cell stimulation from chronic disease and infectious microorganisms (10).

Studies show that supplementation with beta-carotene, vitamin C, and vitamin E may have beneficial effects for Cystic Fibrosis patients, such as boosting antioxidant status and reducing free radicals and oxidative stress (10, 11). Another study reports that supplementation with vitamin C can produce clinical improvement in patients with chronic bronchitis and respiratory infection (12).

Pancreatic Digestive Enzymes Due to pancreatic insufficiency, Cystic Fibrosis patients have difficulties with fat digestion and food malabsorption. For Cystic Fibrosis patients, supplementation with pancreatic digestive enzymes has been shown to aid digestion and help the body to absorb and utilize the food (13).

Probiotics (Lactobacillus Acidophilus and Bifidobacteria) Probiotics including lactobacillus acidophilus (small bowel friendly bacteria) and bifidobacteria (large bowel friendly bacteria) are nutritional supplements containing the same beneficial bacteria that are found in the digestive tract. Since antibiotics are used in Cystic Fibrosis treatment and often destroy friendly bacteria in the intestinal tract, probiotic supplementation is particularly helpful for enhancing intestinal tract immunity and insuring healthy intestinal microflora. (14, 15).

N-acetyl cysteine (NAC) N-acetyl cysteine (NAC) is an acetylated ester of the amino acid cysteine, a precursor for the synthesis of glutathione, which acts as a detoxifying agent. Studies show that supplementation with NAC may have important beneficial effects for Cystic Fibrosis patients, such as reducing sticky mucus secretion, improving lung function, combating lung infections, and decreasing inflammation (16-20).

Bromelain Bromelain, a protein-digesting enzyme complex derived from pineapple, has been shown to have an antitussive effect (cough suppression) and to reduce the thickness of mucus (21). Bromelain’s mucolytic activity reduces bronchial secretions, making it effective in treating respiratory tract diseases (22).

References

1. Cystic Fibrosis: http://www.pdrhealth.com/drug_info/nmdrugprofiles/herbaldrugs/101840.shtml Fibrosis/#stats

2. Ammon HP, et al. Pharmacology of Curcuma longa. Planta Med. Feb1991;57(1):1-7.

3. Ammon HP, et al. Mechanism of Anti-inflammatory Actions of Curcumin and Boswellic Acids. J Ethnopharmacol. 1993;38: 113.

4. Satoskar RR et al., “Evaluation of anti-inflammatory property of curcumin (diferuloy methane) in patients with postoperative inflammation,” Int J Clin Pharmacol Ther Toxicol 1986 (24): 651-4.

5. Srivastava KC, et al. Curcumin, A Major Component of Food Spice Turmeric (Curcuma longa) Inhibits Aggregation and Alters Eicosanoid Metabolism In Human Blood Platelets. Prostaglandins Leukot Essent Fatty Acids. Apr1995;52(4): 223-27.

6. Egan ME, Pearson M, Weiner SA, et al. Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects. Science. 2004 Apr 23;304(5670):600-2.

7. De Vizia B, Raia V, Spano C, Pavlidis C, Coruzzo A, Alessio M. Effect of an 8-month treatment with omega-3 fatty acids (ecos apentanoic and docosahexanoic) in patients with cystic fibrosis. J Parenter Enteral Nutr. Jan-Feb 2003;27(1):52-57.

8. Schwartz J, Weiss ST. The relationship of dietary fish intake to level of pulmonary function in the first National Health and Nutrition Examination Survey (NHANES I). Eur Respir J 1994;7:1821–4.

9. Schwartz J Role of polyunsaturated fatty acids in lung disease. Am J Clin Nutr. Jan 2000;71(1 Suppl): 393S-396S.

10. Back EI et al. Antioxidant deficiency in cystic fibrosis: when is the right time to take action? Am J Clin Nutr. 2004 Aug;80(2):374-84.

11. Winklhofer-Roob BM et al. Plasma vitamin C concentrations in patients with cystic fibrosis: evidence of asssociations with lung inflammation. Am J Clin Nutr 1997 65(6): 1858-66.

12. Hunt C et al. “The clinical effects of vitamin C supplementation in elderly hospitalized patients with acute respiratory infections,” Int J Vitam Nutr Res 1994 (64): 212-9.

13. Amarri S et al. 13Carbon mixed triglyceride breath test and pancreatic enzyme supplementation in cystic fibrosis. Arch Dis Child Apr 1997 76 (4): 349-51

14. Saavedra, J.M. Clinical applications of probiotic agents. Am. J. Clin. Nutr. 2001 Jun; 73(6): 1147S-1151S.

15. James F. Balch and Phyllis A. Balch, Prescription for Nutritional Healing, 3rd ed. (New York, NY: Penguin Putnam Avery, 2000): 221.

16. Riise GC, et al. The intrabronchial microbial flora in chronic bronchitis patients: a target for N-acetylcysteine therapy? Eur Respir J. Jan1994;7(1):94-101.

17. Tattersall AB, et al. Acetylcysteine (Fabrol) in chronic bronchitis–a study in general practice. J Int Med Res. 1983;11(5):279-84.

18. Corradi M et al. Increased nitrosothiols in exhaled breath condensate in inflammatory airway diseases. Am J Respir Crit Care Med. 2001 Mar;163(4):854-8.

19. Millman M, et al. Use of acetylcysteine in bronchial asthma–another look. Ann Allergy. Apr1985;54(4):294-6.

20. Grandjean EM, et al. Cost-effectiveness analysis of oral n-acetylcysteine as a preventive treatment in chronic bronchitis. Pharmacol Res Jul2000;42(1):39-50.

21. Pizzorno JE and Murray MT, eds. Encyclopedia of Natural Medicine, revised 2nd edition, CA: Prima Publishing, 1998: 297

22. Maurer HR. Bromelain: biochemistry, pharmacology and medical use. Cell Mol Life Sci. 2001 Aug;58(9):1234-45.

23. Stoppard M. Family Health Guide, New York: DK Publishing, 2002

24. Merck Manual eds, The Merck Manual of Diagnosis and Therapy, 15th edtion, Rahway, NJ: Merck & Co., Inc, 1997.