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.
Malabsorption of vitamins, including vitamin D
Cystic fibrosis with pancreatic insufficiency is associated with poor absorption of fat and fat-soluble vitamins, including vitamin D. Pancreatic enzyme supplementation does not completely correct the poor absorption of fat. Despite daily vitamin D supplementation, serum vitamin D concentrations remain low in children, adolescents, and young adults with cystic fibrosis. Perhaps these children need higher dosages of vitamin D supplements.
Vitamin K may be of benefit
Vitamin K metabolism is abnormal in CF patients and may have significant consequences if there is a deficiency. Research in the American Journal of Clinical Nutrition, 2010, shows that children and young adults with CF need supplementation of at least 1000 mcg/d to achieve a normal vitamin K status.
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 which are worth a try as a 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. Choline at a dosage of 100 mg to 500 mg or so may be helpful.
Antioxidant deficiency in cystic fibrosis: when is
the right time to take action?
Am J Clinical Nutrition. 2004.
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. 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. 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.
zeaxanthin, macular pigment, and visual
function in adult cystic fibrosis patients.
Am J Clin Nutr. 2004.
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 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. 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. 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. 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.
Choline related supplements improve abnormal plasma methionine - homocysteine metabolites and glutathione status in children with cystic fibrosis.
American Journal of Clinical Nutrition 2007. From the Department of Paediatrics, University of British Columbia, Vancouver, BC, Canada.
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 and of phosphatidylcholine to phosphatidylethanolamine, and phospholipid malabsorption in children with cystic fibrosis. Children with cystic fibrosis were assigned without bias to supplementation with 2 g lecithin/d, 2 g choline per day, or 3 g betaine per day 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. 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. Ddietary supplementation with choline-related compounds improves the low SAM:SAH and glutathione redox balance in children with cystic fibrosis.
Fish oil not helpful
Prostaglandins Leukot Essent Fatty Acids. 2013. A randomized placebo-controlled study on high-dose oral algal docosahexaenoic acid supplementation in children with cystic fibrosis. Forty-one CF patients aged from 6 to 12 years were randomized to receive high-dose DHA (100 mg/kg/day in the first month and 1g per day thereafter through a 12-month supplementation) or placebo (germ oil). Despite a decrease of the AA/DHA ratio, DHA supplementation for one year did not induce any significant biochemical and clinical improvement in CF patients.
BMC Complement Altern Med. 2015. Flaxseed modulates inflammatory and oxidative stress biomarkers in cystic fibrosis: a pilot study. Cystic fibrosis (CF) leads to advanced lung disease despite aggressive care. Persistent inflammation and oxidative stress contribute to exacerbations and disease progression. Flaxseed (FS), a dietary botanical supplement with high fiber, lignan phenolics, and omega-3 fatty acids has anti-inflammatory and antioxidant properties in murine models of acute and chronic lung injury. This pilot study was designed to determine whether CF patients could tolerate FS, evaluate circulating FS metabolites, and study biomarkers of lung damage, as a prelude to studying clinical outcomes. 10 CF patients and 5 healthy volunteers consumed 40 g of FS daily for 4 weeks with safety and tolerability being assessed. Urine was evaluated for systemic oxidative stress and plasma for FS metabolites (enterolignans) and cytokine levels. Buccal swabs were analyzed for gene expression of Nrf2-regulated antioxidant enzymes including Heme Oxygenase-1 (HO-1) and NAD(P)H Quinone Oxidoreductase 1 (NQO1). :All subjects completed the study without serious adverse events. Plasma levels of enterolignans were detectable in both healthy controls and CF volunteers. CF patients were stratified based on plasma enterolignan levels after 2 weeks of FS administration into high and low plasma lignan cohorts. The low enterolignan level cohort experienced a statistically significant drop in urinary inflammatory IsoP and plasma TNFα levels, while demonstrating higher average NQO1 mRNA levels in buccal epithelium compared to high-lignan patients. This pilot study demonstrated that FS is tolerated by CF patients. FS metabolites could be detected in the plasma. Future studies will assess appropriate dosing and target populations for FS, while exploring clinical outcomes.
Magnesium mineral supplement could be helpful
Pediatr Pulmonol. 2016. Magnesium in cystic fibrosis-Systematic review of the literature. We performed a search of the Medical Subject Headings terms (cystic fibrosis OR mucoviscidosis) AND (magnesium OR hypomagnes[a]emia) in the US National Library of Medicine and Excerpta Medica databases. We identified 25 reports dealing with magnesium and cystic fibrosis. The results of the review may be summarized as follows. First, hypomagnesemia affects more than half of the cystic fibrosis patients with advanced disease; second, magnesemia, which is normally age-independent, relevantly decreases with age in cystic fibrosis; third, aminoglycoside antimicrobials frequently induce both acute and chronic renal magnesium-wasting; fourth, sweat magnesium concentration was normal in cystic fibrosis patients; fifth, limited data suggest the existence of an impaired intestinal magnesium balance. Finally, stimulating observations suggest that magnesium supplements might achieve an improvement in respiratory muscle strength and mucolytic activity of both recombinant and endogenous deoxyribonuclease. The first comprehensive review of the literature confirms that, despite being one of the most prevalent minerals in the body, the importance of magnesium in cystic fibrosis is largely overlooked. In these patients, magnesium levels should be sought once a year. Furthermore, the potential of supplementation with this cation deserves more attention.
Improved glutathione status in young adult patients with cystic fibrosis supplemented with whey protein.
J Cystic Fibrosis. 2003.
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. 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. 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 in the lymphocyte GSH levels in the supplemented group. No other changes were observed. 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.
Q. I see the one research study on Immunocal for
patients with cystic fibrosis. How much independent research has been
done on Immunocal?
A. There have been a few studies with Immunocal as reported on Medline, many for HIV patients.
Q. Kudos for your Immunocal / Cystic Fibrosis Info, I think it may benefit asthma! This is has been studied by a Dr. Jean Marcoux (Texas allergist) and Dr. Larry Lands (pulmonary specialist, McGill Medical Center, Canada). Since you caught the Immunocal benefits for CF, I would like to let you know that Dr. Lands was using it to improve his cystic fibrosis patients' breathing. He also published an article in the journal, "Chest," relating to Immunocal and COPD. I personally (and accidentally) found my chronic asthma dramatically relieved in approximately ten days, using 20g/day. I was able to finally stop taking ADVAIR.
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 Salt
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.
Mannitol for cystic fibrosis
Inhalation of mannitol, a sugar, improves lung function in patients with cystic fibrosis. Anna Jaques, of Pharmaxis Ltd. in Frenchs Forest, New South Wales and colleagues claim that inhaled mannitol works by drawing fluid into the lungs, which dilutes the thick secretions in the air passages. These diluted secretions are easier for the patients to clear from their lungs. Anna Jaques analyzed the benefits and safety of inhaled mannitol therapy in 39 patients with mild-to-moderate cystic fibrosis who were assigned to receive the sugar or inactive "placebo" twice daily for 2 weeks. After a 2-week break, patients treated with mannitol switched to placebo and vice-versa. Treatment with mannitol improved the patients' ability to move air in their lungs. Chest, 2008.
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. Chest, 2005.
Children taking Vertex Pharmaceuticals Inc's Kalydeco pill may be at risk of getting cataracts.
Cause of cystic fibrosis
This disease is an autosomal recessive disorder caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene.
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.
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.
Q. I have cystic fibrosis and would like to take
glutathione supplements to improve peak expiratory airflow, could you
please tell me what to do, i am a little confused. Are there many types of
glutathione supplements and i need to take a particular one, in which case
whats the name of the one i should take? What works best oral or inhaled,
i would prefer oral but would that increase my Peak expiratory flows or
not? Are there any glutathione side effects? If glutathione will not
increase my peak expiratory flow would you know of any other supplement
which will increase my peak expiratory flow?
A. As of 2014, I am not aware of studies with oral glutathione supplements in the treatment of cystic fibrosis.
coq10 helpful as an
antioxidant for cystic fibrosis patients?
A. I am not sure.
My Daughter is 13 years old and has been diagnosed with Cystic Fibrosis. We did the first sweat test on April 2010 and got the results of 63 and 73. Herlung capacity (blowing the candles on the computer screen) was at 83%. The doctor put her on an nebulizer (Albuteral) twice daily - which has helped her cough out the mucus every day. We repeated it 4 weeks later and the results were 71 and 73 and her lung capacity is now 97%. We have also done the chest x-ray, CT scan, blood and sputum test. X-Ray showed mucus still in her inner bronchial threads. CT scan last month showed swollen air passages on the right lung - she complains of pain in that area sometime. Her sputum has specs of green color along with yellow. Her blood mutuation report showed the presence of the CF gene. We have not started any pancreatic enzymes. Please can u help us in anyway with any medication, or give us a direction, it will be of real help.