I expect kids born today with Cystic Fibrosis (known as CF) will actually be able to live a long, active life like their peers.

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.

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Alan Greene MD DrGreene.com contributor