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Cystic Fibrosis

Cystic Fibrosis

Description

Cystic fibrosis is a generalized hereditary disorder of infants, children and young adults in which there is widespread dysfunction of the exocrine glands. It is characterized by signs of chronic pulmonary disease, pancreatic deficiency, abnormally high levels of electrolytes in the sweat and occasionally by biliary cirrhosis.

Causes

Cystic fibrosis is an inherited genetic defect.

Signs & Symptoms

Inability to move bowels
Foul-smelling stools
Failure to put on weight although there may be a large appetite
Pneumonia
Shortness of breath

Nutritional Supplements

Structure & Function:
        Antioxidants &
        Essential Fatty Acids


---------------------------------
General Supplements
---------------------------------

AdultChild/Adolescent
Beta carotene*
Fish oils 6 - 8 g 6 - 8 g
EPO 2 - 3 g 2 - 3 g
Vitamin A2,500 - 7,500 IU 2,500 - 7,500 IU
Vitamin E 400 - 600 IU 400 - 600 IU
Pancreatin 5 NF (2-3/meal) 5 NF (2-3/meal)
Selenium 100 - 300 mcg 100 - 300 mcg
Zinc 10 - 30 mg 10 - 30 mg



* Please refer to the respective topic for specific nutrient amounts.

Note: All amounts are in addition to those supplements having a Recommended Dietary Allowance (RDA). Due to individual needs, one must always be aware of a possible undetermined effect when taking nutritional supplements. If any disturbances from the use of a particular supplement should occur, stop its use immediately and seek the care of a qualified health care professional.

Dietary Considerations

Respiratory problems are common in individuals with Cystic Fibrosis because thicker mucus secretions predispose them to infections. Prophylactic antibiotics are often prescribed.

The pancreatic enzymes lipase and trypsin are absent from the small intestine due to nonproduction or to blockage of pancreatic ducts. Hence, there is a malabsorption of fats, fat-soluble vitamins and protein. A secondary lactase deficiency may exist, making the individual lactose intolerant. As much as 40-50% of the caloric intake may pass through the intestine unabsorbed.

Meals should not contain much fat, as fats are not readily absorbed. Fat-soluble vitamins should be orally supplemented. Essential fatty acids can still be obtained by ingestion of safflower oil or any other polyunsaturated oil (one milligram per kilogram of body weight per day).

Extra calories must be consumed to offset the caloric wastage which occurs through the feces. 150 kilocalories per kilogram of weight should be consumed by children. Much protein is lost due to trypsin deficiency. Six to eight grams of protein per kilogram of weight should be consumed by children to minimize body wastage and to supply the immune response.

Salt should be added to the diet to replace minerals lost through sweat.

Sugars should be used instead of complex carbohydrates, such as starch. Sugars are easier to digest and therefore readily available for energy.

Pancreatic enzymes can be ingested with meals to help fat and protein digestion.

Homeopathic Remedy

1. Baryta muriatica - 30X
2. Aceticum acidum - 15C

Treatment Schedule

Doses cited are to be administered on a 3X daily schedule, unless otherwise indicated. Dose usually continued for 2 weeks. Liquid preparations usually use 8-10 drops per dose. Solid preps are usually 3 pellets per dose. Children use 1/2 dose.

Legend

X = 1 to 10 dilution - weak (triturition)
C = 1 to 100 dilution - weak (potency)
M = 1 to 1 million dilution (very strong)
X or C underlined means it is most useful potency

Asterisk (*) = Primary remedy. Means most necessary remedy. There may be more than one remedy - if so, use all of them.


References

Boericke, D.E., 1988. Homeopathic Materia Medica.

Coulter, C.R., 1986. Portraits of Homeopathic Medicines.

Kent, J.T., 1989. Repertory of the Homeopathic Materia Medica.

Koehler, G., 1989. Handbook of Homeopathy.

Shingale, J.N., 1992. Bedside Prescriber.

Smith, Trevor, 1989. Homeopathic Medicine.

Ullman, Dana, 1991. The One Minute (or so) Healer.

Herbal Approaches

----------
Herbs
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Ginger
Milk thistle
Pleurisy root

Note: The misdirected use of an herb can produce severely adverse effects, especially in combination with prescription drugs. This Herbal information is for educational purposes and is not intended as a replacement for medical advice.

Discussion:

A Ginger compress may provide some relief.

Milk thistle extract (1 capsule t.i.d.) enhances biliary function, promotes protein synthesis and is also an antioxidant.

Pleurisy root is noted as an expectorant.

Aromatherapy - Essential Oils

Basil Essence.

Related Health Conditions

Asthma
Bronchitis
Pneumonia

Abstracts

References

Alpers, D.H., R.E. Clouse, & W.F. Stenson. 1983. Manual of Nutritional Therapeutics. Little, Brown, and Company, Boston. 457 pp.

Bakker, W.: Nutritional State and Lung Disease in Cystic Fibrosis. Netherlands Journal of Medicine, 1992;41:130-136.

Borowitz-D & Conboy-K. : Are bioelectric impedance measurements valid in patients with cystic fibrosis? J. Pediatr-Gastroenterol-Nutr. 1994 May; 18(4): 453-6.

Chicago Dietetic Association & the South Suburban Dietetic Association of Cook and Will Counties. 1981. Manual of Clinical Dietetics. W.B. Saunders Co., Philadephia.

Collins CE et al., Fat gram target to achieve high energy intake in cystic fibrosis. J Paediatr Child Health, 1997 Apr, 33:2, 142-7.

Davis, D.B. Effects of Tocopherol on Platelet Membrane Function in Cystic Fibrosis. Journal Of Laboratory Clinical Medicine, 104. 1984.

Duffield-RA Cystic fibrosis and the gastrointestinal tract. Journal-of-Pediatric-Health-Care. 1996 Mar-Apr; 10(2): 51-7. (31 ref)

Escobar, H., et al: Intestinal Permeability to 51Cr-EDTA and Orocecal Transit Time and Cystic Fibrosis. ,Journal of Pediatric Gastroenterology and Nutrition, 1992;14(2):204-207.

Farrell, P.M. & G.R. Gutcher. Evaluation of Vitamin E Deficiency in Children with Lung Disease. Annals Of The New York Academy Of Sciences, 393. 1982.

Farrell, P.M. Nutrition & Cystic Fibrosis. Bol Assoc Med Pr, 76. 1984.

Farrell PM et al., Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group [see comments]. N Engl J Med, 1997 Oct 2, 337:14, 963-9.

Hamilton, H.K. ed. 1982. Professional Guide To Diseases Intermed Communications Inc. Pub, Springfield, Massachusetts. 1323 pp.

Hegsted, M.D. 1976. Present Knowlege In Nutrition. 4th ed. The Nutrition Foundation Pub., Washington D.C. 605 pp.

Homnick, D. N. et al:, Carotenoid Levels in Normal Children and in Children With Cystic Fibrosis. Journal of Pediatrics, May 1993;122(5/Part I):703-707.

Kirschmann, J.D. 1990. Nutrition Almanac: Nutrition Search. McGrew-Hill: New York.

Konstan, M. W., et al: Leukotriene B4 Markedly Elevated in Epithelial Lining Fluid of Patients With Cystic Fibrosis. American Review of Respiratory Diseases, 1993;148:896-901.

Krause, Marie V. & Martha A. Hunscher. Food, Nutrition And Diet Therapy. Philadelphia: W.B. Saunders Co., 1972.

Kunz, J.R.M. 1982. The American Medical Association Family Medical Guide. Random House Pub, New York. 832 pp.

Langley, S.C. Reduced Free-Radical-Trapping Capacity and Altered Plasma Antioxidant Status in Cystic Fibrosis. Pediatric Research, 1993;33(3):247-250.

Lawrence, R. and Sorrell, T.: Eicosapentaenoic Acid in Cystic Fibrosis: Evidence of a Pathogenetic Role for Leukotriene B4. The Lancet, August 21, 1993;342:465-469.

Lebental, E. Pancreatic Insufficiency in Cystic Fibrosis. Journal Pediatr. Gastroenterol. Nutrition, 3. 1984.

Leguire, L. et al: Electro-Oculogram in Vitamin A Deficiency Associated With Cystic Fibrosis. Ophthalmic Pediatrics, 1992;13(3):187-189.

Lloyd-Still, J. D. et al: Essential Fatty Acid Status and Fluidity of Plasma Phospholipids in Cystic Fibrosis Infants. American Journal of Clinical Nutrition, 1991;54:1029-35.

Lloyd-Still, J.D., et al:Acetylcarnitine is Low in Cord Blood and Cystic Fibrosis. ACTA Pediatr. Scand., 1990;79:427-439.

Lloyd-Still, J. D. et al: Carnitine Metabolites in Infants With Cystic Fibrosis: A Prospective Study. Acta Pediatrica, 1993;82:145-9.

Patrick, J. The Pancreas, Nutrition and Cystic Fibrosis. Journal Pediatr. Gastroenterol. Nutrition, 3 1984.

Pennington, J. 1978. Nutritional Diet Therapy. Bull Publishing Co., Palo Alto, Ca. 106 pp.

Peters, S. A. and Rolles, C. J.: Vitamin Therapy and Cystic Fibrosis - A Review in Rationale. Journal of Clinical Pharmacy and Therapeutics, 1993;18:33-38.

Quirk PC et al., Evaluation of bioelectrical impedance for prospective nutritional assessment in cystic fibrosis. Nutrition, 1997 May, 13:5, 412-6.

Ramsey, B. W. et al: Nutritional Assessment and Management in Cystic Fibrosis: A Consensus Report. American Journal of Clinical Nutrition, 1992;55:108-16.

Rayner, R. J.: Fat-Soluble Vitamins in Cystic Fibrosis. Proceedings of the Nutrition Society, 1992;51:245-250.

Riedel BD: Gastrointestinal manifestations of cystic fibrosis. Pediatr Ann, 1997 Apr, 26:4, 235-41.

Rodrigues-ME et al: Concentration of electrolytes in the sweat of malnourished children. Arch-Dis-Child. 1994 Aug; 71(2): 141-3.

Smith, L. J. et al: Taurine Decreases Fecal Fatty Acid and Sterol Excretion in Cystic Fibrosis: A Randomized, Double-Blind Trial. AJDC, 1991;145:1401-1404.

Sonis A et al., Infant nutrition: implication for somatic growth, adult onset diseases, and oral health. Curr Opin Pediatr, 1997 Jun, 9:3, 289-97.

Steinkamp-G & von-der-Hardt-H.: Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis.. J-Pediatr. 1994 Feb; 124(2): 244-9.

Subak-Sharpe, G.J. 1984. The Physician's Manual For Patients. Times Books Pub, New York. 607 pp.

Winklhofer-Roob, B. M. et al: Short-Term Changes in Erythrocyte ?-Tocopherol Content of Vitamin E-Deficient Patients With Cystic Fibrosis. The American Journal of Clinical Nutrition, 1992;55:100-3.