Category: Nutrition

Glutathione: What’s all the fuss about?

Glutathione is a very simple molecule that is produced naturally all the time in your body. It is a combination of three simple building blocks of protein or amino acids — cysteine, glycine and glutamine. The secret of its power is the sulfur (SH) chemical groups it contains. Sulfur is a sticky, smelly molecule. It acts like fly paper and all the bad things in the body stick onto it, including free radicals and toxins like mercury and other heavy metals. But that’s not all. Glutathione is also critical in helping your immune system do its job of fighting infections and preventing cancer. That’s why studies show that it can help in the treatment of AIDS and MS. It is critical for immune function and controlling inflammation. It is the master detoxifier and the body’s main antioxidant, protecting our cells and making our energy metabolism run well.

What’s glutathione got to do with CF?

Glutathione first seemed to come into the limelight relating to CF  in 1997 when Valerie Hudson worked with Dr Clark Bishop (CF Clinic in Salt Lake city Utah) to undertake the first trial using glutathione (GSH) to help her 2 young boys who both had CF. Its a great example of the fact that no one is more dedicated than the parents of a child with a chronic illness or disease.  You might like to check out the article here:
Article : A mothers fight against CF

In this issue of CHEST, Dr Bishop and colleagues report encouraging results from a small, double-blind, placebo-controlled clinical trial of inhaled glutathione in patients with cystic fibrosis. The rationale for this therapy lies in previous data reporting that glutathione levels in the lung epithelial lining fluid of patients with cystic fibrosis are low,1and that cystic fibrosis lung disease is associated with increased oxidative damage.2In addition, more recent studies36 have also demonstrated that the cystic fibrosis transmembrane conductance regulator (CFTR), which is defective in cystic fibrosis, regulates a substantial portion of glutathione efflux into the epithelial lining fluid.

The thinking has since shifted to N-acetylcysteine (precursor to gluathione synthesis) as a more effective way to support the body in generating gluathione on its own.

1] In 2006, stanford university started to investigate N-acetylcysteine for CF. – They did a small randomised placebo controlled, double blind efficacy study investigating NAC on lung inflammation in CF and two of the professors applied for a patent with regards to gluathione being used as a treatment for pulmonary lung disease.

Study # 1  

Study # 2

2] Standford university professors then trialled NAC for use with Autism (2012) as they believe it increases the capacity of the body’s main antioxidant network.

Study # 3  

Study # 4

The jury is out on whether glutathione is offering any real benefit but clearly there are lots of parents out there who are still trialling it as a supplement.

What about you? Have you tried glutathione with your child and can you guestimate as to whether its made any difference?

The importance of essential fatty acids [EFAs]

CF kids are often deficient in essential fatty acids [EFAs]

What’s the deal with fish oil and cystic fibrosis?

Fish oil is used for several conditions including heart disease, reducing triglycerides (blood fats), diabetes, rheumatoid arthritis, ADHD, Crohn’s disease, cystic fibrosis and asthma. Studies have shown it may reduce blood pressure and be helpful in diabetes, rheumatoid arthritis, Crohn’s disease and cystic fibrosis, although better studies are needed.

The impaired digestion of fats in CF often leads to a deficiency of essential fatty acids (EFAs). This deficiency may in turn lead to a lowered immune function, which makes children with CF more susceptible to respiratory infection. EFA deficiency can be reversed by supplementation with corn oil (1 gram per 2.2 pounds [1 kg] body weight per day), safflower oil (1 gram per 2.2 pounds [1 kg] body weight per day), linoleic acid (7.7 grams per day), and eicosapentaenoic acid (EPA from fish oil) (2.7 grams per day). EPA supplementation was particularly effective. In a double-blind trial, six weeks of supplementation with 2.7 grams per day of EPA led to reduction in sputum and improvement in lung function in children with chronic respiratory infection due to CF.

Source here

When questioned, all the research team at the Sydney Children’s Hospital can tell us is that “Yes fish oil has been shown to be advantageous when used on mice…”. If you read the research to date there has been some evidence that fish oil can have a positive effect but like many parents of children with chronic illness, even if there’s no proof, should we be trying it anyway? Below is a quick summary of the mouse study:

Recently, a reversible fatty acid imbalance, i.e., elevated AA and low DHA that may underlie the chronic lung and pancreatic lesions of CF, was demonstrated in a mouse model of the human disease. Oral administration of high doses of DHA not only corrected the membrane lipid imbalance but also reversed the signs of CF in the affected mice. In addition to this theoretical basis for possible dietary therapeutic interventions, a recent Cochrane review concluded that, Regular n-3 supplements may provide some benefits for CF patients with relatively few adverse effects but cautioned that the evidence, so far, is insufficient to draw firm conclusions (8). Improvement of pulmonary function (e.g., FEV) has been observed in CF patients after 6-week to 8-month treatments with n-3 LCP including both EPA and DHA. The extent to which improvement was related to the combination of EPA and DHA versus DHA alone requires additional clarification, but recent observations suggest an independent effect of DHA. Effective doses of fish oil range from 4.5 to 5.3 g/d, providing 2.7 to 3.2 g/d of EPA and 1.8 to 2.1 g/d of DHA, but few other doses were evaluated.

Source:  Journal of Pediatric Gastroenterology and Nutrition: , January 2004 – Volume 38 – Issue 1 – pp 2-3

And it seems that researchers at the University of Sydney agree that fish oil helps cystic fibrosis patients

NEW SOUTH WALES, AUSTRALIA. Researchers at the University of Sydney have found that daily supplementation with fish oil capsules alleviates many of the symptoms of cystic fibrosis. Cystic fibrosis is a serious, inherited childhood disease which involves a malfunctioning of the body’s mucus glands. An abnormally thick mucus is produced which clogs the lungs and results in breathing difficulties. Other symptoms include persistent cough and wheezing, repeated lung infections, and a failure to gain weight. Sixteen cystic fibrosis patients aged 12 to 26 took part in the experiment. Half the group received fish oil capsules providing 2.7 g of eicosapentaenoic acid (EPA) per day while the other half received identical olive oil capsules as a placebo. After six weeks of treatment the patients receiving EPA coughed up significantly less sputum, were breathing easier (both forced expiatory volume and vital capacity were up) and generally felt better. The Australian researchers believe that the EPA acts by modifying the role of leukotriene B4. Leukotriene B4 is thought to be the main culprit in the excessive inflammatory response to bacteria which characterizes cystic fibrosis.

Source: Lawrence, R. and Sorrell, T. Eicosapentaenoic acid in cystic fibrosis: evidence of a pathogenetic role for leukotriene B4. The Lancet, Vol. 342, August 21, 1993, pp. 465-69

And even though CFers have trouble with fat absorption; some [minor] research shows that CFers are able to successfully absorb omega-3s effectively by mouth.

Researchers at the State University of Gent set up an experiment to see if CF patients are able to absorb omega-3s effectively by mouth. The trial involved 9 CF patients (4 females and 5 males) ranging in age from 7 to 20 years. All had been diagnosed with pancreatic insufficiency and had poor fat absorption despite supplementing with pancreatic enzyme preparations. The patients were assigned to receive either 6 fish oil capsules per day for a month followed by 6 placebo capsules for a month or 6 placebo capsules daily for a month followed by 6 fish oil capsules daily for a month. Each fish oil capsule contained 335 mg of salmon oil and 165 mg of commercial soy lecithin and provided 152 mg of omega-3 fatty acids. The placebo capsules contained 500 mg of pharmaceutical-grade liquid paraffin.

The researchers found that the CF patients who took fish oil showed a marked increase in their phospholipid levels of eicosapentaenoic acid [EPA] (increase of 327%) and docosahexaenoic acid [DHA] (increase of 215%). The levels of EPA and DHA returned to baseline 2 weeks after discontinuing supplementation. The researchers also noted that patients with low initial levels of EPA showed the greatest increase in EPA levels after supplementation. They conclude that oral supplementation with fish oil and lecithin is effective in increasing the levels of omega-3 fatty acids especially EPA and DHA in cystic fibrosis patients.
Source: Christophe, Armand, et al. Increase of long chain omega-3 fatty acids in the major serum lipid classes of patients with cystic fibrosis. Ann Nutr Metab, Vol. 36, 1992, pp. 304-12

Taking too much fish oil may increase triglyceride levels which can be harmful. Some studies have also shown that although Cfers don’t absorb as much essential fatty acids as non-Cfers, that high tyriglycerides may still occur.

A DGReview of :“Abnormal lipid concentrations in cystic fibrosis”

American Journal of Clinical Nutrition, 06/11/2002
By Mark Pownall

High levels of triglycerides in the blood are common in cystic fibrosis, questioning the traditional belief that lipid levels were not of concern in this group of patients.
The clinical significance of the findings may become more important as modern treatments result in patients with cystic fibrosis living longer, the researchers behind the study say.
The researchers, from the University of Minnesota in the United States, measured the fasting lipid profiles in 192 patients with cystic fibrosis. The patients also had an oral glucose tolerance test.
The CF patients of all ages had higher triacylglycerol and lower total cholesterol concentrations than the means of the US population. Thirty three of the patients (16 percent) had hypertriglyceridaemia, while eight had elevated cholesterol. levels.

Most of the patients had only hypertriglyceridaemia; only three had both high triglycerides and high cholesterol in their plasma samples.

There was no obvious correlation to other biochemical measurements. Lipid levels were not linked to body mass index, weight, glucose tolerance, the area under the curve or glucose or insulin, nor glycated hemoglobin levels.

There was also no link established with cystic fibrosis genotype, use of systemic steroids, blood pressure, liver enzymes, CV-reactive protein or pulmonary functio
In the face of all these negative findings, the researchers suggest that the hypertriglyceridaemia seen in cystic fibrosis patients may be related to a chronic low-grade inflammation or to a dietary macronutrient imbalance with an excess absorption of simple carbohydrate compared to fat.

The researchers say it is uncertain whether the lipid abnormalities are related to a risk of cardiovascular disease in the CF population.
Am J Clin Nutr 2002; 75: 1005-1011. “Abnormal lipid concentrations in cystic fibrosis”

So what are triglycerides?

Source: American Heart

Triglycerides are the chemical form in which most fat exists in food as well as in the body. They’re also present in blood plasma and, in association with cholesterol, form the plasma lipids.

Triglycerides in plasma are derived from fats eaten in foods or made in the body from other energy sources like carbohydrates. Calories ingested in a meal and not used immediately by tissues are converted to triglycerides and transported to fat cells to be stored. Hormones regulate the release of triglycerides from fat tissue so they meet the body’s needs for energy between meals.

How is an excess of triglycerides harmful?

Excess triglycerides in plasma is called hypertriglyceridemia. It’s linked to the occurrence of coronary artery disease in some people. Elevated triglycerides may be a consequence of other disease, such as untreated diabetes mellitus. Like cholesterol, increases in triglyceride levels can be detected by plasma measurements. These measurements should be made after an overnight food and alcohol fast.

So as parents what we want to know is – what does all this mean? Should we be giving our CF kids more fish oil? If so, how much more? How much is too much? And how much is enough? The above research suggests that effective doses of fish oil range from 4.5 to 5.3 g/d, providing 2.7 to 3.2 g/d of EPA and 1.8 to 2.1 g/d of DHA, but few other doses were evaluated.

The reality is that our doctors don’t know how much fish oil we should be giving our kids but understandably, will err on the side of caution. The question for me as a parent is, how harmful are larger doses of fish oil if I choose to follow the research and up the dosage? Should I be testing my child for high triglyceride levels? Is the dose he is currently on even high enough?

My son is 2 years old and currently on 4 x Metagenics  EPHA / DHA capsules daily which deliver 500mg of triglycerides daily [165 mg EPA and 110 mg DHA] per capsule. If I can find anyone to give me advice on this I’ll update the site and let you know.>

Additional info on therapeutic dosages:
Typical dosages of fish oil are 3 g to 9 g daily, but this is not the upper limit. In one study, participants ingested 60 g daily. The most important omega-3 fatty acids found in fish oil are called eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA). In order to match the dosage used in several major studies, you should take enough fish oil to supply about 2 g to 3 g of EPA (2,000 mg to 3,500 mg) and about 1.0 g to 2.5 g of DHA daily (1,000 mg to 2,500 mg). Far higher doses have been used in some studies; conversely, one study found blood-pressure lowering effects with a very low daily dosage of DHA—0.7 g.238

DHA and EPA are not identical and might not have identical effects. Some evidence hints that DHA may be more effective than EPA for thinning the blood 176  and reducing blood pressure.105  The reverse may be true for reducing triglyceride levels, but study results are conflicting.160-165, 235
Some manufacturers add vitamin E to fish oil capsules to keep the oil from becoming rancid. Another method is to remove all the oxygen from the capsule. If possible, purchase fish oil products certified as free of significant levels of mercury, toxic organochlorines, and PCBs (see Safety Issues). Flaxseed oil also contains omega-3 fatty acids, although of a different kind. It has been suggested as a less smelly substitute for fish oil. However, it is far from clear whether flaxseed oil is therapeutically equivalent to fish oil.1,200. Studies to date suggest it is not.
Additional links:

The USA National Cholesterol Education Program guidelines for triglycerides are:

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Probiotics [LGG] may decrease internal inflammation of both the lungs and intestines


Common wisdom says that probotics are a useful supplement for anyone who takes regular antibiotics as they help support intestinal function and gut repair. There is a significant amount of research however to suggest that probiotic supplements are especially important for CFers. In particular, LGG [the lactobacillus GG strain]. Whilst we know that probiotics affect intestinal inflammation research suggests that a relationship exists between intestinal and pulmonary inflammation.

So what are Probiotics?

Our bodies are full of bacteria. Some bacteria make us sick, some help keep us well. Probiotics are foods or nutrition supplements that contain the good bacteria. Most of these organisms are ones that are already present in our bodies, but not always in sufficient quantity to be of the most benefit.

How Can Probiotics Help People with Cystic Fibrosis?

Several studies have been done about the benefits of probiotics. Two recent Italian studies have shown that the probiotic organism, Lactobacillus rhamnosus GG (LGG), can benefit cystic fibrosis patients in a couple of ways.

In the first study, the researchers just looked at the benefits to the intestines. They found that cystic fibrosis patients who take LGG may have a reduction in the intestinal inflammation that often causes them pain

A few years later, the same researchers decided to take it one step further and see how LGG affects the lungs of CF patients. They found that cystic fibrosis patients who took LGG regularly had less bouts with respiratory infections than those that didn’t take LGG.

Where Can I Find LGG?

Lactobacillus rhamnosus GG is available over the counter at many health food stores and pharmacies in capsule form. It is also available in some brands of yogurt. There are many products, including most yogurts with active cultures, that contain Lactobacillus acidophilus. While Lactobacillus acidophilus is a beneficial probiotic, it is not the same as LGG. Read the label carefully to be sure you’re a getting a product that contains LGG.

Sources: Bruzzese, E., Raia, V., Gaudiello, G., Polito, G., Buccigrossi, V., & Formicola, V. (2004). Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment Pharmacol Ther. 20, 813-819
Bruzzese, E., Raia, V., Spagnuolo, M.I., Volpicelli, M., De Marco, G., Maiuri, L. (2007). Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: A pilot study. Clinical Nutrition. 26, 322-328.

Another study: Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: A pilot study

A prospective, randomized, placebo-controlled, cross-over study was performed. Nineteen children received LGG for 6 months and then shifted to oral rehydration solution (ORS) for 6 months. In parallel nineteen received ORS and then shifted to LGG. Main outcome parameters were: incidence of pulmonary exacerbations and of hospital admissions, forced expiratory volume (FEV1), and modifications of body weight.


Patients treated with LGG showed a reduction of pulmonary exacerbations (Median 1 vs. 2 , range 4 vs. 4, median difference 1, CI 95% 0.5–1.5; p=0.0035) and of hospital admissions (Median 0 vs. 1, range 3 vs. 2, median difference 1, CI95% 1.0–1.5; p=0.001) compared to patients treated with ORS. LGG resulted in a greater increase in FEV1 (3.6%±5.2 vs. 0.9%±5; p=0.02) and body weight (1.5kg±1.8 vs. 0.7kg±1.8; p=0.02).


LGG reduces pulmonary exacerbations and hospital admissions in patients with CF. These suggest that probiotics may delay respiratory impairment and that a relationship exists between intestinal and pulmonary inflammation.

Source: Clinical Nutrition Journal

The take-away >

Eating yoghurt, even healthy looking yoghurt like Valia may not give you a viable dose of probiotic. It’s certainly good for you but it may help to have an added boost. Experts suggest we need to be giving our kids a viable dose of a strong probiotic to support the gut. According to our clinical dietitian at the hospital, the Metagenics brand [ a natural offshoot of a pharma co.] is one of the stronger more viable “active” doses. And don’t bother with Yakult, experts says that a lot of sugar and the strain of probiotic it offers is not the most effective for their gut health.

 Metagenics makes an LGG supplement if you’re interested.

Bowel movements are a good indicator of health

Much to the chagrin and embarrassment of CF patients everywhere, bowel movements are an important part of our lives. In many ways our bowel movements serve as a benchmark that lets us know how things are going, so to speak, with our digestive systems. Cystic fibrosis patients with pancreatic insufficiency should be especially aware of all things related to their digestive process, and be able to identify what’s normal for them and what isn’t.

When the pancreas is too blocked by mucus, it can’t deliver the enzymes necessary to break down the foods we eat, particularly proteins and fats. As a result, people with CF may have more frequent bowel movements because they are not absorbing the protein and fat that they are eating. As if that weren’t embarrassing enough, CF patients are also prone to flatulence (gas). Their gas and BMs can be particularly foul-smelling as the result of proteins and fats not being absorbed by the body.

So how can a person with CF determine what’s a normal BM and what isn’t? Consider these three factors:

1) Color

2) Condition

3) Quantity


Brown is generally the “preferred” color of healthy stool, though there may be different variations on that theme.

The liver excretes bile salts into the stool, giving it a normal brown color. Obstruction to the flow of bile out of the liver (you may see the word “cholestasis”), or liver infections like viral hepatitis, may produce clay-colored stools .

Black, tarry stools with a foul odor can be the result of eating certain foods, taking iron supplements, or possibly from internal bleeding. Foods that are dark blue or black in color may also cause black stools. Examples of foods and minerals causing dark stools include: black licorice, blueberries, iron supplements, lead, Pepto Bismol (contains bismuth.)

Green , leafy vegetables contain chlorophyll which could be coloring the stool green . Another possibility is that the stool has passed too quickly through the large intestine and has not yet had all of the bile absorbed. Sometimes newborns have green colored stools, and this is perfectly normal.

Patients with cystic fibrosis often have gall bladder issues, which can cause the stools to appear orange. This is because bile, which normally helps process fats in foods may not be excreted adequately. Stools are usually orange if they contain a lot of oil and fat that the bile salts have not had enough time to break down. A lack of bile (because of bile blockage such as gall stones) can also cause stools to appear orange. Orange, greasy stools are particularly foul-smelling, and may be difficult to control.


Healthy stools should be uniform in shape and consistency. They should sink and not float. Floating stool is evidence that you are not absorbing enough fat from your food.

Loose, watery stools may indicate a stomach virus, especially if they are concurrent with vomiting. In young children especially, it’s important not to let diarrhea lead to dehydration. Dehydration as a result of lost water through the stools may also contribute to intestinal blockage. If the small intestine, which usually adds water to digested food, is unable to do so, the fecalization process cannot take place and the stool can back up into the upper gastrointestinal system.

Stools that are difficult and painful to pass are often the result of not enough fiber in the diet. Patients with cystic fibrosis have to strike a delicate balance between having just enough BMs to prevent bowel obstruction, but not so many that they lose water and vital nutrients.


Cystic fibrosis patients are encouraged to eat several small meals throughout the day instead of 3 large ones, as a way to avoid painful bloating and stomach pains. This means that for some people, going to the bathroom 6 times a day is just part of life. Some patients with cystic fibrosis are very regular in their bathroom habits, and can accurately predict which foods will go through more quickly than others. Usually it’s the foods that are high in fat content that cause a person to use toilet shortly after eating.

One of the earliest signs of cystic fibrosis is a condition called meconium ileus. Approximately 20% of cystic fibrosis will be diagnosed at birth because of mecomium ileus. Meconium is the material present in the intestines of a newborn. In meconium ileus, intestinal obstruction results from the impaction of thick, tenacious meconium in the small bowel.