Category: Nutrition

New Nutritional Guidelines for CF

For those of you interested in the new nutritional guidelines which were launched at this year’s Australian CF conference you can download a copy of them here. [This is the final draft but final copy not yet available online].

You’ll see these new guidelines not only promote a healthier more wholistic diet than previous CF recommendations. Moving away from any fat and sugar to make the calories add up  – to an emphasis on good fats and heart health where possible. The guidelines also touch on vitamins and supplements – namely glutathione, probiotics to name a few emerging areas which have not been formally addressed before.

Diet Check: Heart Healthy Fats

Heart Healthy Fats [Standford University CF Centre]
Milk, butter, cheese — For most adults living with cystic fibrosis, those
food items are staples in an everyday high-calorie, high-protein diet.
Fat found in dairy products pairs well with most meals, and is excellent
at boosting calories. But don’t be fooled. Dairy is not the only option
available. You’re probably well aware of avocado and trail mix, but what
sets these fats apart from fats found in dairy and butter?

Continue reading “Diet Check: Heart Healthy Fats”

PharmaNAC gets the thumbs up from Standford trial

Oxidative stress is defined as an excessive load of reactive oxygen species (ROS), which cause ongoing or reversible damage in the body. This can occur in individual cells, and in specific body organs, and can affect the health of patients at the whole body level.
Antioxidants can chemically react with ROS to quench and thus inactivate these reactive, damaging molecules. Cystic fibrosis is characterized by oxidative stress throughout the body and chronic inflammation in the lungs. Patients with CF are deficient in the body’s major anti-oxidant, glutathione (GSH). This is thought to be due to many reasons, including dietary insufficiency, but being the major anti-oxidant of the body, GSH is highly utilized in areas of oxidative stress and inflammation. GSH serves multiple functions and is utilized by cells to regulate physiological functions such as DNA transcription, RNA translation, and subsequent protein synthesis. It is utilized to regulate protein functions and is vital in regulating dietary absorption of nutrients, storage and availability of essential proteins and fatty acids.
Oxidation reactions are essential to fight infection and are generated when neutrophils ingest bacteria to rid the body of pathogens. For reasons still unclear, in CF, the environment in the airways is strongly pro-inflammatory. After neutrophils have been recruited, neutrophil-derived oxidants are released into the airways, and contribute to ongoing tissue destruction. Oxidants, which include hydrogen peroxide, hypochlorous acid, and other damaging particles called free radicals are released and create a vicious perpetual cycle of tissue destruction.

Continue reading “PharmaNAC gets the thumbs up from Standford trial”

In Cystic Fibrosis Patients, Vitamin D Supplements Might Not Prevent Deficiencies. Study found low vitamin levels in young patients despite recommended supplements

A new study entitled “Vitamin D and Chronic Lung Colonization in Pediatric and Young Adult Cystic Fibrosis Patients” published in the Spanish-language journal Nutrición Hospitalaria, investigated vitamin D levels in the body and its association with chronic lung colonization. The study showed that vitamin D supplements did not improve the vitamin’s insufficiency in young cystic fibrosis patients.

Cystic fibrosis (CF) is a frequently inherited genetic disorder in the Western world, and most CF patients develop respiratory failure due to chronic pulmonary infection. Several factors, including environmental, nutritional, and socioeconomic, may affect the clinical manifestations of cystic fibrosis. Low levels of vitamin D are highly prevalent in CF patients, likely due to the combination of inadequate nutrient absorption, impaired metabolism, and lack of sun exposure. Vitamin D insufficiency is associated with high incidence of bone disease or osteoporosis in CF patients.

Food-with-Vitamin-DIn this study, researchers evaluated vitamin D levels in serum samples from 377 cystic fibrosis patients whose ages ranged from 2 months to 20 years. The patients, from several university hospitals in Spain, were evaluated over the course of two years. The researchers found that younger patients had lower levels of vitamin D as well as a higher number of pathogen colonizations. They showed that despite CF patients receiving vitamin D supplements — which are currently recommended for the disease — a high percentage showed insufficient levels of the vitamin. Moreover, they found that age, pancreatic function, type of diagnosis, and lung colonization modulate the levels of serum vitamin D in these patients. Regardless of age, diagnosis by screening or pancreatic status, chronic colonization by Pseudomonas aeruginosa in children and adolescents and by Staphylococcus aureus in infants and preschoolers increased their risk of developing vitamin D deficiency.

The authors, however, are aware of the limitations of their study.  The longitudinal nature of the study makes impossible to know whether vitamin D levels are the cause or consequence of chronic lung colonization in these patients. The multicenter nature of the study, while necessary to obtain a sample size sufficient in low-prevalence diseases as CF, could introduce biases when analyzing the dose and vitamin D levels due to lack of vitamin supplement standardization protocol among the centers. However, all patients received vitamin D doses as expected. The clinical centers were from different latitudes, but the sera vitamin D were collected during the same time period (fall-winter), decreasing but not eliminating the modifying effect of sun exposure on vitamin D levels.

These findings could encourage additional studies into the interplay of vitamin D, cystic fibrosis, and bacterial infections. New discoveries might help physicians develop new best practices for supplements that could better optimize vitamin D levels in CF patients.

What can you do?

How about ensuring you and your family have 2 serves of oily fish a week – try baked salmon or a tuna sushi roll?

How about serving eggs a couple of times a week – try an egg & cheese wrap for brekky or sneaking an egg into their mash at dinnertime?


You can see the original article here CFRI

Stanford University joins the GSH discussion

Screen Shot 2015-10-28 at 3.13.26 pmOnce upon a time there was a CF mother who was looking for anything that might help her son with CF. Sound familiar?

For those of you not familiar, Dr. Valerie Hudson’s foray into CF research began after her son, John, now 6, was diagnosed with cystic fibrosis as a baby. In her desperation, she taught herself anatomy from her older son’s high school anatomy textbook and began her long journey into the field of CF research. As the years passed, she became intrigued with the chemical glutathione and its role in the cell. After coming to believe that this chemical could make a difference in the health of her sons, Dr. Hudson eventually recruited Dr. Bishop to run a small, preliminary study using inhaled glutathione. Dr. Hudson presented their results.

Glutathione (GSH) is a tri-peptide, a very small protein, present throughout our bodies, but it appears in especially high concentrations in the epithelial lining fluid of the lungs.(Epithelial cells are the cells that line the inside of our lungs and they produce this fluid.) When the body has to deal with high levels of free radicals and oxidative stress, as is the case in CF lungs, the body provides higher levels of GSH to help it cope. GSH has several properties. It is a natural mucolytic. It helps regulate the immune response, as both an anti-inflammatory and as a cell-signaling molecule directing the white blood cells as to where and what to fight. It scavenges free radicals, and it is an anti-oxidant. Additionally, oxidized GSH protects proteins during stressful events in the lungs.

There is significant evidence of the importance of GSH in CF pathophysiology. At the 2000 CFRI Conference, you may have heard Dr. Rabin Tirouvanziam give an update on his GSH research. Dr. Bishop reported on a more recent study that showed that normal mice lung cells, when challenged with Pseudomonas aeruginosa, increase levels of epithelial lining fluid GSH six-fold to help fight the infection. By contrast, CF mice have significantly lower levels of a GSH response. This response is severely reduced in people with CF as well.

Dr. Bishop presented his small-scale, preliminary trial using inhaled glutathione. The GSH used was “pharmaceutical grade of 98.6 percent,” it was buffered and diluted to minimize irritation, and it was tested and found free of bacterial and fungal products. He also relied on the results from seven other published human trials, which observed no significant adverse events when used. In his randomized, placebo-controlled, double blind, clinical trial of inhaled GSH, Dr. Bishop recruited 19 subjects. Ten used the inhaled GSH and nine used the placebo. The age range of the subjects was from six to eighteen, and the trial occurred during the summer months. Patients inhaled through a mask nebulizer approximately 66 milligrams per kilogram of their body weight of GSH mixed with sterile water, divided into four daily inhalations sessions, each three to four hours apart.

The results of this clinical trial showed significant improvement in 11 out of 13 factors measured before and after use of GSH, including peak flow, overall improvement in health, and reduced cough. Dr. Bishop is the first to recognize the limitations of his trial (size is a significant factor), while drawing the following conclusions. GSH deficiency in epithelial lining fluid may play a major role in pathogenesis of CF, and GSH inhalation therapy appears to be effective for CF.

Dr. Bishop issued a clear caution to parents and adults with CF that further research still needs to be done before this can be considered a legitimate therapy for the CF community. Further research needs include a large, multi-centered clinical trial, the use of an FDA-approved product, and methods to increase convenience and control of administering the product. (Patients had to prepare their own solutions using capsules and sterile water, so doses were hand-prepared. This is time-consuming and dosages are not necessarily exact). Additionally, Dr. Bishop urged clarification of the route of delivery (oral vs.inhaled), dosage specifications, schedule of administration of the drug, patient selection, and other GSH augmentation tactics. Dr. Bishop warned that this is an experimental therapy not approved by the Cystic Fibrosis Foundation. No long-term studies have been done to ensure safety and efficacy of GSH use. He further cautioned his audience that the CFF recommends not using this GHS inhalation therapy until further studies are done.

The best pre-cursor on the market that is readily available as an oral dose seems to be NAC

  1. N-Acetyl-Cysteine (NAC)

It is derived from the amino acid L-Cysteine, and acts as a precursor of glutathione. NAC is quickly metabolized into glutathione once it enters the body. It has been proven in numerous scientific studies and clinical trials, to boost intracellular production of glutathione, and is approved by the FDA for treatment of accetaminophen overdose. Because of glutathione’s mucolytic action, NAC (brand name Mucomyst) is commonly used in the treatment of lung diseases like cystic fibrosis, bronchitis and asthma.

 When we asked around about this a few years ago it seemed that it was destined to remain a sideline alternative treatment – with the initial pilot research driven by a CF mother and a Dr Bishop out of a Utah in 2004. However, since then other research groups have been cautiously exploring the potential benefits. We know that the Royal  Children’s Hospital in Melbourne are now looking at it as part of a nutritional review and Standford University have just released a paper on it which I have posted below.

2015 Stanford University Paper: The role of NAC in producing GSH in CF patients

You can read about the original study here:

By the way if you’re keen, you can buy the oral cysteine “Fizzy NAC” online here:


Could Maple syrup enhance the effect of our antibiotics? researchers think so

Maple syrup may help fight disease-causing bacteria, including antibiotic-resistant strains that often grow in health-care settings, says a study published online in Applied and Environmental Microbiology.

Concentrated extracts of maple syrup combined with antibiotics significantly reduced the growth of four common bacterial strains and bacterial communities called biofilms, the study found.

Bacterial biofilms accumulate on medical surfaces and devices, such as catheters and artificial joints, and are responsible for many antibiotic-resistant hospital infections, research has shown.

The maple-syrup extracts appeared to damage the outer membrane of bacterial cells, increasing their susceptibility to antibiotics, and to disable cellular mechanisms called efflux pumps associated with antibiotic resistance, the researchers said. The extracts also reduced the activity of genes in the bacteria linked to antibiotic resistance.

Maple syrup contains plant-based compounds called phenols that have exhibited antioxidant and anti-cancer properties in previous studies, according to researchers. Maple syrup may reduce the dose of antibiotics needed to kill biofilms, they said.

The study, at McGill University in Montreal, used concentrated maple-syrup extracts developed from syrup purchased at local markets in August 2013. The extracts and four phenols isolated from maple syrup were tested separately and in various combinations, with and without the antibiotic ciprofloaxin, against Escherichia coli, or E.coli; Proteus mirabilis, a common cause of urinary-tract infections; and two strains of Pseudomonas aeruginosa, associated with hospital-acquired infections.

Maple-syrup extract alone suppressed the growth of biofilms for all four strains. But pairing the extract with ciprofloaxin reduced E. coli and P. mirabilis biofilm formation by about 70% and the two P. aeruginosa strains by 83% and 54%, compared with control cultures treated with just ciprofloaxin.

Of the phenols tested, only catechol, when combined with ciprofloaxin, significantly reduced the growth of all four bacterial strains. Catechol may be responsible for maple syrup’s antimicrobial properties, the study suggests.

Caveat: Maple-syrup extracts combined with antibiotics haven’t been tested in human trials.

Now that print is dead, maple syrup may officially be the greatest thing made by trees.

Title: Polyphenolic Extract from Maple Syrup Potentiates Antibiotic Susceptibility and Reduces Biofilm Formation of Pathogenic Bacteria

This nutritional inspiration comes from Kylie one of our resident CF parents who is passionate about using natural therapies to supplement mainstream treatments.  For many of us these 5%ers are among a bunch of things that we try and incorporate into our diets and routines. If it can’t hurt and it’s received well by our children, why not try it? After all, life’s too short to wait until everything is proven. So if that sounds like you, here are a couple of little snack ideas using maple syrup to get you started… (‘grams of fat’ count in brackets as gf)

7-recipe_maple-milkshake_600x935Maple Syrup Milkshake | What you’ll need

¼ cup pure maple syrup, plus more for serving (optional)

2 cup whole fat milk (4.4gf)

1 tsp. vanilla extract  & 1 tbsp Chia seeds (4.7gf)

2 scoops Conoisseur vanilla ice cream  (19.6gf)

Half a banana

Puree ingredients until smooth; pour into serving glasses and drizzle with more syrup, if you like.

Total Fat count | approx 30 grams fat

Continue reading “Could Maple syrup enhance the effect of our antibiotics? researchers think so”

High Calorie Meal-Time Make-Over


By JULIE MATEL, RD, Stanford University CF Care Centre

cool-hunter-mcdonalds-fashion-makeover-1One of the challenges of living with cystic fibrosis is consuming enough calories to meet growth and weight gain goals. People with CF need 20 to 50 percent more calories than people without CF. For active teens and adults this can be as many as 3000 to 5000 calories: whereas someone without CF might require 2000 calories per day. Why so many calories?
People with CF may not absorb all of the calories from their food, even when taking enzymes with all meals and snacks. People
with CF may need more calories to breathe and to fight infections. Getting enough calories can feel like an insurmountable task
for people with CF. Below are some tips that may help.
1. Be an informed consumer
• Read labels
when you shop find the brand with the highest
amount of calories per serving. For example, one brand of
ice cream may have as many as 300 calories per ½ cup serving
versus 130 calories per serving in another brand!
2. Be consistent
• Avoid skipping meals.
Missing breakfast, for example can
leave you short calories for the day.
• Aim for three meals plus two to three snacks per day.
Eating multiple times during the day makes it more likely to
get the nutrition and calories you need.
• Bed-time snack.
Make this a routine. Choose comfort
foods such as whole milk and cookies or granola with full-fat
yogurt as a snack to look forward to before bed
3. Make your calories count
• Choose calorie dense foods
at meal and snack times.
• Avoid beverages between meals and snacks,
such as juice or soda. These can hinder appetites. Consume water between
meals and snacks instead.
4. Look for creative ways to add calories.
Below are some high calorie ideas:
* Trail mix in place of chips
* Muffins or croissants instead of plain bread
* Whole milk or half-and-half in place of low-fat milk
*Add cheese or guacamole or mayonaise to burgers and sandwiches
*Add crumbled bacon to rice, soup, pasta, eggs, or
* Include a milkshake or smoothie each
day as a snack
* Add peanut butter or whole-fat yogurt
to fruits
* Butter all toast, bread and sandwiches thickly with salted butter

Continue reading “High Calorie Meal-Time Make-Over”