Cystic Fibrosis: The Right Nutrition Key to a Bright Future

My friend’s one-month old granddaughter has been diagnosed with CF. Of course they are heartsick, but I was wondering if you have any dietary advice and/or know of any cutting edge research related to this disease.
Austin, TX

Baby looking into the camera. The future is bright for babies born today with CF.

Dr. Greene:

What a tough time for your friend’s family! If someone’s going to have this disease, this is the best time in history to get the diagnosis. The median survival was 25 when I was in med school. It’s now in the 40’s. And the possibilities for treatment are advancing more quickly than ever. It’ll be a new world 10 years from now. I expect kids born today with Cystic Fibrosis (known as CF) will actually be able to live a long, active life like their peers.

Already, gene therapy, CFTR modulators, inhaled agents to recreate the normal airway surface, inhaled antibiotics, and new approaches to anti-inflammatory therapy all hold great promise. And soon, replaceable organs will be an option for all of us.

Key Nutrition Principles for Kids with CF

  1. A nutrition regimen tailored to that individual’s needs (there is a wide range of calorie and nutrient needs in kids with CF) and
  2. As much as possible, trying to stay ahead of and prevent suboptimal nutrition, rather than waiting until a problem develops and trying to correct it.

The Cystic Fibrosis Foundation has great recommendations.

Nutrition Needs of One-Month Olds with CF

Calories: Some kids with CF need the same amount of calories as their peers. Others need 50% more. Some people just give all kids with CF 30% extra calories. But that’s often wrong. The key is to track growth. The goal is to grow at least at the 50th percentile in weight for height (and later at least 50th percentile in BMI). Between 50th and 85th percentile is ideal.

Vitamins A, D, E, and K. These vitamins should typically be supplemented – even in 1-month babies with no symptoms and no sign of pancreatic disease. The total amounts daily are 1500 IU of vitamin A (and measuring serum retinol every year), 400 to 500 IU of vitamin D3 (and increasing gradually, as needed, to keep serum 25-hydroxyvitamin D levels at least 30 ng/mL) 40 to 50 mg of vitamin E (and measuring serum alpha tocopherol every year), and 0.3 to 0.5 mg of vitamin K – with perhaps more when taking antibiotics (and measuring prothrombin time and PIVKA II every year).

Essential fatty acids. Babies growing at less than the 50th percentile often benefit from measuring (triene:tetraene ratio) and supplementing as appropriate.

Zinc. Babies growing at less than the 50th percentile may also benefit from extra zinc. Also babies with dermatitis. The dose is ½ mg per kg of body weight, twice daily.

Sodium. ⅛ to ¼ tsp of salt daily (300 to 600 mg). Double this (¼ to ½ tsp) when it is hot and/or humid or she has fever, diarrhea or vomiting.

Breast milk is great for babies with CF. Or standard infant formula. If babies need extra calories to achieve optimal growth, human milk fortifier can be added to breast milk. Or ½ tsp of infant formula powder per 3 ounces of pumped breast milk (to get 22 calories per ounce) or more if needed. Similarly, the calories in formula can be adjusted with various recipes.

Teaching babies to love great food is even more important for babies with CF. I outline my approach in Feeding Baby Green.

Dr. Alan Greene

Dr. Greene is the founder of DrGreene.com (cited by the AMA as “the pioneer physician Web site”), a practicing pediatrician, father of four, & author of Raising Baby Green & Feeding Baby Green. He appears frequently in the media including such venues as the The New York Times, the TODAY Show, Good Morning America, & the Dr. Oz Show.

  1. Kino

    Abstract
    Proximal small intestinal mucosal biopsies were carried out in children with cystic fibrosis who had diarrhoea and failed to thrive in spite of adequate treatment, including pancreatic supplements. Histological examination of eight of the 17 biopsies taken over a period of 12 years showed evidence of enteropathy, and accounted for one in 13 (8%) children with cystic fibrosis under 3 years of age attending our clinic. Seven responded to a cows’ milk free diet; the diarrhoea stopped and weight gain increased. One of these responded only when gluten was also excluded from his diet. The eighth child remained on a normal diet and his symptoms did not improve. The enteropathy had resolved in all five patients who had further biopsies taken while receiving treatment, and from 15 months to 3 years of age all the children tolerated a normal diet and continued to thrive. Cows’ milk sensitive enteropathy is an important cause of failure to thrive in children with cystic fibrosis. Small intestinal biopsy is an important investigation in younger children who fail to thrive and have diarrhoea despite adequate treatment.
    Arch Dis Child. 1989 Sep; 64(9): 1251–1255. PMCID: PMC1792745
    Cows’ milk sensitive enteropathy in cystic fibrosis.
    S M Hill, A D Phillips, M Mearns, and J A Walker-Smith

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  2. Hans de Rycke

    If one is born with a disease like cystic fibrosis it is an indication that the mother has a compromised immunity, most likely through allergies. (Galli F, Battistoni A, Gambari R, et al. Oxidative stress and antioxidant therapy in cystic fibrosis. Biochim Biophys Acta. 2012;1822(5):690-713.
    Complimentary and alternative therapies practitioners would recommend the mother avoiding dairy products while breast feeding. Alternatively one could look for mother’s milk banks, which are now all over the US. (I assume you are in the US).
    Adde FV, Rodrizues JC, Cardoso AL. Nutritional follow-up of cystic fibrosis patients: the role of nutrition education. J Pediatr (Rio J). 2004;80(6):475-482. recommends; Eliminate potential food allergens and foods that increase mucous production, including dairy (milk, cheese, sour cream, and ice cream), wheat (gluten), soy, corn, potatoes, cabbage, bananas, sugar, preservatives, food additives, and excessive salt and meats. Your health care provider may want to test for food allergies and/or sensitivities. This might be difficult for a 1 month old baby.
    Bruzzese E, Raia V, Gaudiello G, et al. Found that intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment Pharmacol Ther. 2004;20(7):813-819. I am not sure how probiotics can be administered without being digested unless as suppositories.
    The Cystic Fibrosis Foundation has some interesting observations including a list of researchers and their research results and recommendations.
    Will keep you busy for a few hours, though!

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  3. Carol Homann

    During a ‘fight or flight’ reaction, digestion stops. This means many nutrients can not be absorbed. Some supplements can not be absorbed because of reactions to certain medications.
    It is essential to eliminate potential food allergens and foods that increase mucous production, such as dairy (milk, cheese, sour cream, yogurt, and ice cream)
    Wheat can be a problem too. It is difficult to digest, which can lead to mucus production. Same applies for potato. Use sweet potato or yams instead.
    Chlorine in our drinking water should be avoided! It kills bacteria, including the bacteria in the gut. A diet of predominantly vegetables and papaya and pineapple.is recommended to replenish the gut flora. Avoid meats (red and white) and eat fish sparingly.
    The effectiveness of probiotics is still debated. Many believe the digestive processes will breakdown the fats, proteins and carbs before they reach the gut, making the useless. Going back to basics diet wise is the best option.

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