What is Cystic Fibrosis?
Cystic fibrosis or CF, is an inherited disease of the secretory glands. Both parents need to carry the gene, there will be a 1 in 4 chance that each baby conceived may have CF. Cystic Fibrosis is most common amongst white Caucasian and coloured families.
CF mainly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.
Mucus is a substance made by tissues that line some organs and body cavities, such as the lungs and nose. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected.
If you have CF, your mucus becomes thick and sticky. It builds up in your lungs and blocks your airways.
The build up of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage your lungs.
The thick, sticky mucus also can block tubes, or ducts, in your pancreas (an organ in your abdomen). As a result, the digestive enzymes that your pancreas makes can’t reach your small intestine.
Testing for Cystic Fibrosis
There are a number of ways to test for CF, there is the gene test and these days they can be very specific about the actual position on the gene. The gene test is a blood test that you and your partner as well as your baby will undergo. Then there is the sweat test. This test involves measuring the level of sodium in the sweat of your baby. Children with cystic fibrosis often have a very salty ‘tasting’ skin. This is due to the fact that their bodies over excrete sodium in the sweat glands.
The most common early symptoms of Cystic Fibrosis include failure to thrive, repeated lung infections and smelly, oily stools. The reason behind the poor growth as well as the smelly oily stools is due to pancreatic insufficiency.
The pancreas is an organ in the body that forms part of your child’s digestive system. The role of the pancreas is to produce digestive enzymes. These enzymes are necessary to break down food that your child has eaten. Without the presence of these enzymes your child will have no access to the nutrients that are present in food and in particular the fats, proteins and fat soluble vitamins, like A, D, E and K. If left undiagnosed a child with Cystic Fibrosis will develop severe malnutrition and micro nutrient deficiencies. This will lead to poor growth, skin conditions and many other symptoms associated with malnutrition. The stools become very smelly and diarrhea-like due to undigested food. The oily nature of the stool is due to the bodies inability to break down fat.
Once diagnosed it is important that your child is managed by a multi disciplinary team, including a paediatric pulmonologist, physiotherapist, paediatric dietitian and paediatric gastroenterologist. This team is headed up by your child’s paediatrician who will remain the primary health care provider. Multi disciplinary clinics including the above health care professionals can be found in both government and private care settings within South Africa. The Red Cross children’s hospital has one of the biggest CF clinics in the country and are very active in cystic fibrosis research. Johannesburg general hospital also has a good CF clinic and Christian Barnard hospital in Cape Town runs a private CF clinic on a quarterly basis.
Once diagnosed your child will be given enzymes via medication and the symptoms of malnutrition will be reversed - your child will start to grow and thrive. Your child will also need additional vitamin and mineral ( eg. Zinc and selenium) supplementation. Due to the amount of sodium (salt) loss through your child’s skin, your child will need to take in additional sodium via salt solutions prescribed by your cystic fibrosis team.
Due to the regular build up of mucus plugs, the lungs of a Cystic Fibrosis child often take a hammering. Lung care including nebulizing, correct and appropriate physiotherapy as well as consistent use of asthma-like pumps and medications are all critical factors in their care.
Food and nutrition
All kids need to eat well to grow up healthy and strong. But kids with CF need to eat more than most other kids. Most kids with CF will eat three meals a day plus snacks to make sure that they get all of the calories they need. This isn’t all that different from other kids, but the meals and snacks that a kid with CF eats should have more calories and fat in them. It’s also very important that a kid with CF not miss meals.
So what do kids with CF use these extra calories for?
Like everyone else, kids with CF need calories to grow, to gain weight, and to have energy to play. But kids with CF need extra calories because their bodies have a hard time absorbing fat and nutrients in food. Instead of absorbing the fat and nutrients from food, some of these important things can go right out of the person’s body in their bowel movements. Kids with CF also may need more calories to help their bodies fight the lung infections they tend to get.
Let’s take a closer look at some important nutrients and where to find them.
It’s All in the Nutrients
Nutrients are the things in food that help keep our bodies running well. Kids with CF have some nutrients that they need to make sure they eat each day. These include:
- Iron is essential for carrying oxygen to all the body’s cells. You can find iron in some cereals, meats, dried fruits, and dark green vegetables.
- Zinc is essential for growth, healing, and staying healthy. You’ll find zinc in meats, liver, eggs, and seafood.
- Calcium helps build strong bones. Milk, yogurt, cheese, and calcium-fortified juices are rich in calcium.
- Salt. Kids with CF lose a lot of salt in their sweat, especially during hot weather and when they exercise. A good way to replace this salt is by adding salt to food and eating salty snacks. During hot weather and when kids play sports, they may need sports drinks during and after practice or gym class
Meals at Home and Away
To meet your child’s nutrition needs, you’ll work with the CF dietitian to create meal plans that include plenty of snacks, especially for toddlers who eat smaller meals.
For a picky eater, pay attention to the foods your child especially likes and adapt the menu as these preferences change. Ask an older child with CF to try one new food a week. It’s also important to model good behaviour for kids of all ages. Eat a well-balanced diet with a variety of foods and make sure you try new things yourself.
To make meals a pleasant experience:
- Try to keep meals to about 20-30 minutes for toddlers and young kids, who can’t sit still for extended periods.
- Eat in a comfortable environment with few distractions.
- Don’t give huge portions that might seem overwhelming.
- Offer praise for eating well, but don’t force plates of food to be finished; encourage kids to eat as much as they can comfortably.
- Keep food choices simple, especially for younger kids.
- Make foods as attractive and appealing as possible.
- All childcare providers and teachers should know that your child has CF and be aware of his or her nutritional and caloric needs. Work with the staff to plan high-calorie meals and snacks or send food if the menu can’t accommodate your child’s needs. Young kids need help taking their enzymes and supplements, and the staff should understand that they need to be taken before all meals.
- As kids with CF grow into teens, they’ll probably begin eating more meals away from home and may feel pressured to eat in certain ways. Work with your teen, emphasizing the positive and monitoring any weight changes that are of concern. If you’re packing lunch, include high-calorie options like salad dressing and nut butter dips.
Increasing Caloric Intake
It can be tricky to make sure that kids with CF eat enough fat and calories, especially if they’re sensitive to what’s on the plates of siblings and peers. One way to increase calories without creating an entirely separate menu is to increase the calories and fat in one part of the meal.
Here are some simple ways to do so:
- Add extra butter to sandwiches, sauces, and potatoes.
- Use olive oil dressings on salads or vegetables; add extra oil to the dressing.
- Prepare main meals with gravies and creamy sauces.
- Add bacon to burgers and chicken.
- Add dried skim milk powder to sauces and beverages.
- Add extra cheese to potatoes or macaroni and cheese; order extra cheese on pizza.
- Top salads and sandwiches with avocados or guacamole.
- Add nuts to cookies, cakes, pancakes, and salads.
- Add extra cheese and deli meats to sandwiches.
- Grill sandwiches in butter.
- Use heavy whipping cream and whole milk when cooking.
- Make milkshakes.
- Add instant breakfast mixes to milk-based beverages.
- Make high-calorie smoothies.
- Prepare calorie-rich desserts such as pudding and cheesecake.
- Top hot chocolate, pudding, and other desserts with whipped cream.
- Provide high-calorie snacks like peanut butter crackers or trail mix.
- Prepare high-calorie versions of popular family recipes.
- When you’re shopping, be sure to compare the labels on similar prepared foods and purchase those that have higher fat and calorie content. Avoid diet foods — anything that claims to be nonfat, low fat, reduced calorie, or light/lite.
Look for whole-fat versions of dairy products such as sour cream, cottage cheese, and yogurt (many natural food stores carry a good variety of these). Don’t forget to check baby food labels. You’d be surprised at how the caloric level varies between brands.
Beyond Food: Enzymes and Tube Feeding
A child with pancreatic insufficiency will need to take enzymes with meals and snacks to help digest food properly and to get the nutrition and fat needed to grow and gain weight.
Signs that your child may need enzymes or an enzyme dose adjustment include:
- Failure to gain weight, in spite of a strong appetite
- Frequent, large, greasy, or smelly bowel movements
- Bloating or gas
- The CF dietitian or doctor will prescribe enzymes based on weight, growth, and how much a child eats at a time. An increased enzyme dose doesn’t necessarily mean that a child is doing poorly. It may mean that he or she has gained weight and now requires more enzymes.
Enzymes need to be taken with every meal and most snacks. They come in capsules, full of tiny beads, that can be broken open for kids who are too young to swallow entire capsules. They should only be mixed with foods that are acidic, like applesauce. They should not be chewed or crushed. Never change the dose of enzymes without first consulting your child’s dietitian or doctor.
In some cases, enzymes and high-calorie meals may not be enough to help a child thrive, and feedings through a tube into the stomach may be needed. It’s not always physically possible for kids with CF to meet their increased energy needs by eating by mouth alone. Tube feedings, which can be done at night, are an excellent way to help kids who have trouble gaining weight, especially when they’re ill.
Many kids and their parents are resistant to having a tube put in. But tube feedings are only prescribed when dietary interventions, calorie-boosting strategies, and enzymes haven’t been enough. Tube feeding may sound scary at first, but tube insertion is usually an uncomplicated outpatient procedure. Results can include improved weight gain and decreased stress around eating.
Tube feedings do not mean that you’ve failed to feed your child well or that he or she isn’t a good eater - often they just mean that a child needs so many calories a day that it’s impossible to keep up without them. Your child’s doctor and dietitian will make recommendations about tube feedings.
It can be hard for kids with CF to eat enough in a day to meet their needs, especially when they aren’t feeling well. Although we all think we’d like to be able to eat a high-fat, high-calorie diet, many kids with CF, especially teens, find it difficult to do so in our diet-conscious culture.
Keeping your eye on the positive - your child’s growth and weight gain - will help your child with CF eat well, stay healthy, and thrive.