Cystic fibrosis is an inherited (genetic) condition affecting the cells that produce mucus, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of cystic fibrosis. Patients with the disease are prone to pneumonia and other complications and have an average lifespan of about 30 years.

Natural options for cystic fibrosis treatment
I will update this section as more information becomes available. Research with supplements for patients with cystic fibrosis is relatively new and there is much more we need to learn before making any recommendations with any degree of certainty. But in the meantime, it appears that patients with cystic fibrosis may perhaps benefit from antioxidants, particularly Carotenoids. Carotenoids are available for sale as supplements and are worth a try as a cystic fibrosis treatment, not necessarily as a cure, but perhaps to ease the progression of the disease. We suggest consuming a broad range of Carotenoids as opposed to one, such as beta carotene. See also salt water inhalation below. Choline at a dosage of 100 mg to 500 mg or so may be helpful.

Choline related supplements improve abnormal plasma methionine - homocysteine metabolites and glutathione status in children with cystic fibrosis1,2,3
American Journal of Clinical Nutrition, Vol. 85, No. 3, 702-708, March 2007. Sheila M Innis, A George F Davidson, Stepan Melynk and S Jill James. From the Department of Paediatrics, University of British Columbia, Vancouver, BC, Canada (SMI and AGFD), and the Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR (SM and SJJ)
Liver triacylglycerol accumulation and oxidative stress are common in cystic fibrosis and also occur in choline deficiency. Previously, we showed an association between elevated plasma homocysteine, reduced ratios of S-adenosylmethionine to S-adenosylhomocysteine (SAM:SAH) and of phosphatidylcholine to phosphatidylethanolamine, and phospholipid malabsorption in children with cystic fibrosis. Children with cystic fibrosiswere assigned without bias to supplementation with 2 g lecithin/d (n = 13), 2 g choline per day (n = 12), or 3 g betaine per day (n = 10) for 14 d. Plasma concentrations of methionine, adenosine, cysteine, cysteinyl-glycine, glutathione, glutathione disulfide (GSSG), and fatty acids; SAM:SAH; and red blood cell phospholipids were measured within each group of children with cystic fibrosis before and after supplementation. Children with cystic fibrosis had higher plasma homocysteine, SAH, and adenosine and lower methionine, SAM:SAH, and glutathione:GSSG than did children without cystic fibrosis. Supplementation with lecithin, choline, or betaine resulted in a significant increase in plasma methionine, SAM, SAM:SAH, and glutathione:GSSG and a decrease in SAH. Supplementation with choline or betaine was associated with a significant decrease in plasma SAH and an increase in SAM:SAH, methionine, and glutathione:GSSG. Supplementation with lecithin or choline also increased plasma methionine and SAM. Conclusion: We showed that dietary supplementation with choline-related compounds improves the low SAM:SAH and glutathione redox balance in children with cystic fibrosis.

Cystic Fibrosis symptom
Cystic Fibrosis influences the body's ability to move salt and water in and out of cells. This defect leads the lungs and pancreas to secrete a thick mucus, thus blocking passageways and preventing the proper function of these organs.

Cause of Cystic Fibrosis
Cystic Fibrosis is an autosomal recessive disorder caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene.

Cystic fibrosis treatment
Medications for cystic fibrosis include antibiotics for respiratory infections and pancreatic enzymes to replace those which are missing. Vitamin supplements are sometimes prescribed. Inhaled bronchodilators are used to relieve chronic obstruction of the airways. Other treatments include postural drainage and chest percussion, and other breathing treatments. New treatments include replacement of the DNAse enzyme. This is available as a medication called dornase (Pulmozyme). Genetic research is ongoing in hopes of correcting the disease by artificially inserting a "normal" gene into the person. This treatment is called gene therapy, and an intranasal form of gene therapy is currently undergoing clinical trials.

Cystic Fibrosis and risk of annual CT scan
The increased cancer risk from annual CT scanning of patients with cystic fibrosis is currently modest, but could become more substantial as patients with the disease live longer. CT scanning is more sensitive than simple chest X-rays in detecting structural lung damage and may also be more effective for identifying disease progression than measuring lung function. For these reasons, routine CT scans are increasingly being used in cystic fibrosis patients, but the benefits for survival are not clear.

Cystic Fibrosis and Salt Water inhalation
Long-term inhaled saline with a 'hypertonic' salt concentration of 7 percent improves lung function and reduces pulmonary flare-ups in patients with cystic fibrosis,
     Surfers with cystic fibrosis had fewer lung exacerbations. That led scientists working in North Carolina and at the Royal Prince Alfred Hospital in Sydney, Australia, to theorize that there might be something about saltwater that could explain improved lung function in the surfers. When the patients inhaled the saltwater, it caused their lungs to re-hydrate by adding a layer of water to the airway surface. That acts as a lubricant, which makes it easier to clear the mucus.

Cystic Fibrosis Protein
According to researchers at Johns Hopkins Children's Center, Baltimore, a newly identified protein, called VCP/pr97, kills a chloride transporter in the cells of most cystic fibrosis patients. This inability to transport chloride results in a dangerous buildup of thick, sticky mucous in several organs, including the pancreas and lungs. That can lead to the malnutrition, chronic lung infection and lung damage so often seen in cystic fibrosis patients. In a test-tube study, the researchers used a tool called RNA interference to successfully intercept signals sent out by VCP/pr97. In doing so, they were able to prevent cell damage caused by the protein. The findings showed promise in restoring cells to normal status in cystic fibrosis patients but it will take many more years of research.

Cystic Fibrosis screening
Individuals with cystic fibrosis  who are identified through newborn screening have improved growth, better overall health, and require fewer long-term therapies than those who are identified later in life when symptoms appear.

Cystic Fibrosis Disease Research Update
Antioxidant deficiency in cystic fibrosis: when is the right time to take action?
Am J Clin Nutr. 2004 Aug;80(2):374-84.
Little is known about age- and disease-related changes in prooxidant and antioxidant systems in patients with cystic fibrosis. We investigated changes in antioxidant concentrations and oxidative stress in plasma, buccal mucosal cells (BMCs), and breath condensate in patients with cystic fibrosis in relation to age and disease progression. We recruited 22 patients with cystic fibrosis as well as 35 healthy control subjects and conducted a cross-sectional study by dividing the participants into 4 age groups (<6 y, 6-11 y, 12-17 y, >/=18 y). We collected fasting blood samples, BMCs, and breath condensate. Carotenoids, alpha-tocopherol, vitamin C, protein carbonyls, and thiobarbituric acid-reactive substances were assessed. RESULTS: In patients with cystic fibrosis, plasma vitamin C concentrations, plasma and BMC alpha-tocopherol concentrations, and forced expiratory volume in 1 s (percentage predicted) decreased significantly with age. Plasma beta-carotene, beta-cryptoxanthin, and total lycopene were significantly lower in patients than in control subjects in all age groups. Furthermore, alpha-tocopherol and vitamin C plasma concentrations as well as alpha-tocopherol concentrations in BMCs were significantly lower in cystic fibrosis patients >/=18 y old, whereas all indicators of oxidative stress assessed were significantly higher than those same indicators in control subjects. CONCLUSIONS: Adult patients with cystic fibrosis in particular showed distinct vitamin deficits and elevated indicators of oxidative stress in plasma, BMCs, and breath condensate along with a progression of clinical status. We suggest that early in life dietary habits should be improved and that innovative supplementation strategies should be applied to optimize the antioxidant status of patients with cystic fibrosis.

Improved glutathione status in young adult patients with cystic fibrosis supplemented with whey protein.
J Cystic Fibrosis. 2003 Dec;2(4):195-8.
The lung disease of cystic fibrosis is associated with a chronic inflammatory reaction and an over abundance of oxidants relative to antioxidants. Glutathione functions as a major frontline defense against the build-up of oxidants in the lung. This increased demand for glutathione (GSH) in cystic fibrosis may be limiting if nutritional status is compromised. We sought to increase glutathione levels in stable patients with cystic fibrosis by supplementation with a whey-based protein. METHODS: Twenty-one patients who were in stable condition were randomly assigned to take a whey protein isolate (Immunocal, 10 g twice a day) or casein placebo for 3 months. Peripheral lymphocyte GSH was used as a marker of lung GSH. Values were compared with nutritional status and lung parameters. RESULTS: At baseline there were no significant differences in age, height, weight, percent ideal body weight or percent body fat. Lymphocyte GSH was similar in the two groups. After supplementation, we observed a 46.6% increase from baseline (P < 0.05) in the lymphocyte GSH levels in the supplemented group. No other changes were observed. CONCLUSION: The results show that dietary supplementation with a whey-based product can increase glutathione levels in cystic fibrosis. This nutritional approach may be useful in maintaining optimal levels of GSH and counteract the deleterious effects of oxidative stress in the lung in cystic fibrosis.

Inhaling a compound that is normally produced in the lungs but is lacking in people with cystic fibrosis seems to be helpful for such patients. Cystic fibrosis is a genetic condition, characterized by thick mucus build-up in the lungs as well as other organs such as bile ducts and intestines. People with the disorder suffer from difficulty breathing, frequent bouts of pneumonia, and numerous other afflictions. The secretion of a peptide called glutathione by lung cells is impaired in cystic fibrosis, and there is good evidence to suggest that the lack of glutathione in lung fluid plays a key role in the chronic inflammation and infection that occurs. Previous studies have investigated inhaled glutathione as a treatment for cystic fibrosis. The current study is different from the others in that it compared active treatment with inactive placebo treatment, and involved a higher daily dose of glutathione over a longer period. In the study, 19 patients with cystic fibrosis were randomized to receive inhaled glutathione or placebo for 8 weeks. Glutathione-treated patients experienced an increase in peak expiratory airflow whereas the comparison group experienced a drop.  When asked to rate their condition on a 5-point scale, the participants given glutathione reported significantly more improvement than those given the placebo. Also, inhaled glutathione therapy was well tolerated, and the frequency and nature of side effects was similar in the two groups. SOURCE: Chest, January 20057.

Lutein, zeaxanthin, macular pigment, and visual function in adult cystic fibrosis patients.
Am J Clin Nutr. 2004 Jun;79(6):1045-52.
BACKGROUND: Pancreatic insufficiency in cystic fibrosis, even with replacement pancreatic enzyme therapy, is often associated with decreased carotenoid absorption. Because the macular pigment of the retina is largely derived from 2 carotenoids, lutein and zeaxanthin, the decreased serum concentrations seen in cystic fibrosis may have consequences for ocular and retinal health OBJECTIVES: Our aims were to determine plasma carotenoid concentrations, determine absorption and distribution of macular pigment, and assess retinal health and visual function in cystic fibrosis patients. DESIGN: In 10 adult cystic fibrosis patients (ages 21-47 y) and 10 age- and sex-matched healthy control subjects, we measured macular pigment density in vivo, measured serum lutein and zeaxanthin concentrations, and comprehensively assessed visual performance (including contrast sensitivity, color discrimination, and retinal function) under conditions of daylight illumination. RESULTS: Serum lutein and zeaxanthin were significantly reduced in cystic fibrosis patients compared with control subjects. Although macular pigment optical density was significantly lower in the cystic fibrosis group than in the control group, no significant differences in visual function were observed. CONCLUSIONS: Adults with cystic fibrosis have dramatically low serum and macular concentrations of carotenoids (lutein and zeaxanthin), but their ocular status and visual function are surprisingly good. The clinical implications of low plasma concentrations of carotenoids in cystic fibrosis are yet to be clarified.

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Cystic fibrosis is a genetic disorder that affects the glands that produce sweat and mucus, with the most significant damage occurring in the lungs and digestive system. Mucus in cystic fibrosis patients becomes thick and accumulates in the lungs and intestines, resulting in breathing problems, frequent respiratory infections and poor nutrition. Because most patients die of lung disease, the primary goal of cystic fibrosis therapy is to help keep the airways clear and avoid respiratory infections.

Cystic Fibrosis questions
Q. I am the administrator of a web site on cystic fibrosis. We have had forums for the past 10 years. I notice the forum members are beginning to use Oil of Oregano with good results. I wonder if you can point me in a direction for a study of oil of oregano and people with cystic fibrosis.
   A. I have not come across any research regarding the use of oil of oregano for cystic fibrosis.