Cystic Fibrosis and a Different Kind of Good Nutrition
Cystic Fibrosis, or CF, is the most common, lethal, inherited disorder among Caucasians and occurs in about 1 in every 2000 births (Source: Papalia, Olds and Feldman, 2008). The disease was once thought to be an automatic death sentence because most children did not make it to their 21st birthday. In modern times, the children who are afflicted with Cystic Fibrosis are living into their thirties and sometimes beyond. Women with Cystic Fibrosis are also managing to get pregnant, which can be a problem because of the nature of the disease itself.
In the average person, food slides along the digestive tract aided by slippery mucus. As food is broken down in the digestive process, nutrients are absorbed through the walls of the intestine and then into the bloodstream. From there, the pumping of the heart circulates these nutrients throughout the body to wherever they might need to go. In a person with CF, the mucus of the digestive tract is not thin and slippery – it becomes thick and gummy, preventing the food from moving through with ease, keeping it from digesting correctly and, most importantly, preventing nutrients from traveling through the digestive tracts’ walls, leaving the child to be malnourished even though they are eating.
CF is a progressive disease and affects not only the growth pattern of the child but will also work to destroy the lungs as well. As the lungs get worse and worse, the need for good nutrition will only increase. CF is not a disease that affects only one nutrient, vitamin or mineral – it affects everything equally. The rules for foods that most people are taught are generally not suggested for those with Cystic Fibrosis. In fact, the disease causes the need for all new rules to be written.
The calorie count for people with Cystic Fibrosis is completely different than for children without the disease. They need to eat far more food than their peers because it takes far more food to get even a portion of the nutrients that they need to have. A typical teenager might need around 2200-2500 calories a day, but the child with CF may need 4500-5000 or even more. The doctors suggest that all low calorie foods be ignored so that there is more room for the high calorie foods that are going to provide the energy that the child needs to survive. The only rule that remains the same is avoiding fried foods because of the digestive requirements that they put on the body. When the body digests fried foods, it has to create more bile from the liver, which is a problem in people with CF. High fat food is good, fried food is not so good.
Cystic Fibrosis also affects the sweat glands. An average person sweats out both sodium and chloride, which sits on their skin and will eventually be reabsorbed by the body. In CF, both of these mineral compounds simply sit on the skin and are not reabsorbed at all, leading to a deficiency. The salt must be replenished or it will cause an electrolyte imbalance in the body, which can in turn lead to heart arrhythmia. Children, even babies, with CF should salt their foods.
In addition, CF children should be getting around two quarts of water every day, or even more in exceptionally hot weather. Children with CF are prone to dehydration.
The Need for Supplements
Anything that can make it easier for nutrients to get through should be done. Most of the children with CF will be given daily physical therapy to loosen the mucus. They will be given antibiotics and other medications to treat their lungs and to prevent lung infections. They may also be given daily enzymatic supplements to help increase their digestive powers. It is possible that when all is said and done, the average child with CF may be taking as many as a couple of dozen different pills.
A protein supplement for a child with CF can be beneficial for a number of reasons, including allowing for quality protein and other nutrients in a highly absorbable format. There are a number of these supplements to choose from, including protein powders, shakes, liquid protein shots and snacks like protein bars and protein puddings. Protein-enhanced waters are another option which can be beneficial, pulling double duty by supplying necessary protein and other nutrients and hydrating water as well.
Protein powders
There are four varieties of protein powders: soy, whey, rice and egg. Most are single protein powders while others might be a combination of two or more.
Whey protein powder is derived from milk and is a byproduct of cheese. It is one of the most common of the protein powders and the most easily found. The whey protein concentrate is the least expensive and is around 30-85% protein, while the protein isolate is 90% protein and may be harder to find and may cost more. The benefits of whey protein powder are that it boosts immunity, a huge advantage to those with CF, and is an optimal source of amino acids, which can prevent muscle breakdown. Whey protein may not be appropriate for those with CF because it is a milk product.
Soy protein powder is also available as either a concentrate or an isolate and is one of the most digestible of the proteins. It is the only complete plant based protein because it has all eight essential amino acids and is one of the only ones that is suitable for vegan and vegetarian diets. Soy flour may benefit those with CF by improving the nutritional value of other foods.
Egg protein is made from the egg white, the most perfect protein source. It is the highest concentration of the amino acids alanine, argine, glycine and methionine.
Rice protein powder is derived from brown rice and is also a complete protein. Like soy protein, rice protein is suitable for vegans and vegetarians, however, there is less chance of allergic reactions with rice protein powder because it is considered to be hypoallergenic (Source: Segounis).
In addition to protein powders, the use of protein shakes can be very beneficial because they can help to increase the calorie count and get the right level of nutrients in a quickly and easily absorbable way. The liquid protein shot, like Profect, supplies 25 grams of protein in a fast and easy supplement shot. In addition, Profect has vitamin C (100% of RDA) and 10% supply of vitamin B complex. It comes in a number of fruit based flavors, including Blue Raspberry, Cool Melon Splash, Grapefruit Mango and Fresh Citrus Berry and can be carried along anywhere you go. It can be used as a between-meal snack or with meals.
Protein bars should supply a good amount of protein (between 10-15 grams is optimal) and should also have other nutrients as well.
The Need for Good Medical Care
Children and teens with CF will need to have their nutrient levels checked frequently. The amount of the enzyme supplements that they take will need to be adjusted frequently as well. Some days they will absorb more nutrients, some days far less. It is important that no matter what, they continually follow up with their doctor so that they can keep their health at its optimal level.
References
Diane E. Papalia, Sally Wendkos Olds and Ruth Duskin Feldman. A Child’s World: Infancy Through Adolescence Eleventh Edition. McGraw Hill Publishing Company. Boston, MA 2008
Sofia Segounis, Nutritionist. Protein Powders truestarhealth.com
By: Jim Duffy
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