A Critical Part of Overall Health

Maintaining a healthy weight and taking all your medications, including your enzymes and vitamins, is very important for people with CF.

Your CF dietitians are available to discuss your nutritional needs and concerns in clinic, over the phone or via e-mail. Just contact your CF centre and someone will get back to you as soon as possible. Together we can help make your nutritional status the best it can be.

On this page you will find information about enzymes, maintaining a healthy weight, vitamins & minerals, G-tubes, and post-transplant nutrition.

Enzymes

When food is eaten it goes into the stomach where it is broken down into tiny pieces. These pieces pass into the small intestine where, in a healthy body, digestion takes place with the help of bile from the liver and enzymes from the pancreas. The nutrients from the food are then taken in by the body (absorbed) and used for energy and growth. Food that is not broken down (not digested) passes out of the body in the stools.

In most people with CF, the tubes in the pancreas are blocked with the same type of sticky mucous found in the lungs. Pancreatic enzymes cannot get into the small intestines, so food is not properly digested. This leads to poor nutrition and/or poor growth. Most people with CF need to take pancreatic enzymes capsules to help with digestion of food.

Pancreatic enzymes contain lipases, proteases, and amylases which digest fat, protein, and larger sugars such as starch. The number on your enzyme bottle represents the amount of lipase in the product. Enteric coated enzymes are used by most adults with CF. The coating protects the enzymes from acid in the stomach so that the enzymes reach the small intestines. The coating may take some time to dissolve, so the enzymes are released further down the intestine than they would if uncoated.

Enzymes capsules may be swallowed whole. They should be given either at the beginning of the meal, or given throughout the meal (especially if you are a slower eater). It is recommended to take additional enzymes if food is eaten more than 1 hour after the last enzymes were taken. How many enzymes you need depends on how much fat you eat. Each enzyme contains a standard amount of lipase which helps you digest fat.

The amount of enzymes needed by a person with CF varies with age, body size, and amount and type of food eaten. Your CF dietitian will decide the type and dose of enzymes to use. This may change over time. In general, your body needs more enzymes to digest foods that are high in fat and protein than foods high in carbohydrate. Enzymes are not needed for foods that contain only sugar because someone with CF is able to digest these foods with the enzymes found in saliva. Examples of these are fruit, juice, soft drinks, hard candy, popsicles and Jello-O.

Taking more enzymes is not always better. Taking really large amounts of enzymes may hurt your colon.

The number of enzymes that you should take depends on the type of enzyme you are using and how much fat you are eating. Click on the links below for detailed instructions:

ECS 8

Creon 10

ECS 20

Creon 25

Some people need more enzymes than others. If you have symptoms, such as greasy, loose stools and weight loss, you can try increasing the number of enzymes that you take. Talk to your dietitian or doctor.

Check how many enzymes you should be taking by downloading our Enzyme Calculator spreadsheet

The amount of enzymes might not be right if you notice the following: loose stools, stooling more often, greasy or floating stool stomach pain, cramps, or gas.

If you often forget your enzymes, try placing full containers of enzymes in the places you occupy: the kitchen, desk at work, school locker, backpack, or purse to remind you to take them. Or have a compact carrying case that can hold a meal’s worth of enzymes in a place that is convenient. You can excuse yourself from company to take your enzymes if you are not comfortable taking them in front of other people.

If you carry enzymes loose in your pocket or container, discard the enzymes once weekly. Enzymes are very sensitive to heat and may degrade sooner than the expiry date if left in the heat.

For good health and to prevent constipation, it is very important to drink enough fluids.

Maintaining a Healthy Weight

Achieving a healthy weight can be a challenge for those with CF but it is definitely worth the effort. Research shows that those with higher weights have better lung function. A healthy body weight can also help your body to fight lung infections with a stronger immune system. Too much weight in CF can also be a problem, as it can restrict your breathing and may lead to health conditions such as high blood pressure and high cholesterol levels which could result in heart disease.

A person with CF can require 10-30% more calories than someone without CF of the same sex and body type. The body uses up more energy in CF because of malabsorption, increased energy needed for the work of breathing and coughing, and fighting lung infection.

To see an estimate of how many calories you should be consuming, download the our personalized nutrition tool (in spreadsheet form).

  • Eat frequently. Make time for 3 large meals and 3 snacks every day.
  • Add a lot of “extras” to foods: sauces, spreads, dips, etc.
  • Make drinks count. Choose high calorie drinks instead of water, coffee, tea or diet pop.
  • Use larger plates/bowls when you’re eating at home
  • Snack while watching TV or working on the computer. Carry snacks with you when you’re on the go.
  • Eat larger portions, especially foods that are higher in calories: nuts, cheese, pastas, breads, dried fruit, sweet snacks
  • Monitor your weight once every 2 weeks and keep track of it

For many people with CF, eating foods that are high in fat and calories is the best way to maintain a healthy weight. This means that high calorie foods such as fries and chocolate bars are likely to take priority over fruits and vegetables in your diet. But did you know that it is possible to add fruits and veggies to your diet without skimping on calories?

Considering the health benefits and enjoyment associated with eating fruits and vegetables, it is definitely worth trying to fit them in. Read on to discover how to add some colour to your CF diet.

What Do Fruits And Vegetables Do For Us?

They are excellent sources of key vitamins and minerals that are important for the healthy functioning of our bodies. For example:

  • Bright orange fruits and vegetables are good sources of vitamin A, which maintains the health of our eyes, skin and immune system
  • Folate, found in many vegetables, keeps our red blood cells healthy
  • Vitamin C, which is abundant in citrus fruits and many other fruits and vegetables, helps protect our bones and blood vessels.
  • Fibre, found in most fruits and vegetables, keeps our bowels regular and helps maintain the health of our digestive system.
  • Fruits and vegetables also contain thousands of substances called Phytochemicals. These compounds have many health benefits and can only be found in food (not supplements).

How Can I Add Colour Without Skimping On Calories?

Try these tips for fattening up your fruits and veggies:

  • Enjoy vegetables that are naturally high in fat, such as avocados, coconut and olives.
  • Try cooking your vegetables in a large amount of vegetable oil.
  • Add plenty of creamy dressings to your salads (skip the “light” dressings).
  • Add butter to broccoli, green beans, corn on the cob, or any vegetable you like.
  • Make a smoothie by mixing fruit, whole milk, and ice cream together in the blender.
  • Add cheese sauce to broccoli and other veggies.
  • Enjoy your favourite fruits with chocolate sauce and whipped cream.

High calorie weight gain meal plans from MyFoodData.com
20 high-calorie meals from allnutritious.com
700 calorie meal recipes from BBCgoodfood.com

SAVOURY SNACKS

  • Cheese and crackers
  • Hummus and pita bread
  • Bean burrito
  • Deviled eggs/hard boiled eggs
  • Buttered Popcorn
  • Cream cheese or sour cream based dip with vegetables
  • Tortilla chips and guacamole

SWEET SNACKS

  • Dried fruit and nuts (trail mix)
  • Peeled apple slices with peanut butter
  • Milkshake, smoothie or commercial supplements (e.g. Boost®, Ensure®)
  • Muffins or scones with cheese
  • Cereal with whole milk or full-fat yogurt
  • Pudding (rice, tapioca, milk or soy based)
  • Granola/Protein Bars
HOMEMADE SMOOTHIE GUIDE
  1. Choose your liquid – 1 cup
    Try milk (e.g. cow’s milk, soy or almond milk), chocolate milk, buttermilk, commercial nutrition supplement, fruit or vegetable juice
  2. Add protein source – 2 tbsp
    Skim milk powder, yogurt, silken/soft tofu, nuts, protein powder, cottage cheese, nut or seed butter
  3. Add calories – 1 tbsp or more, depending on your own taste preferences
    Cream, ice cream, whipping cream, condensed milk, avocado (fresh or frozen), oil, cream cheese, hemp hearts, nut butter
  4. Add flavours – ¼ to ½ cup
    Fresh or frozen berries, banana, applesauce, greens (kale, spinach, chard), ginger

When you have no appetite, it can be hard to eat enough food to maintain your weight and meet your nutritional needs. Consider the following tips:

  • Forget the knife and fork. Meals that require a lot of effort to prepare take up a lot of time and may take up too much of your energy. Keep a supply of single serving pre-packaged frozen foods and easy to prepare foods on hand. They’re easy to heat up and are usually high in calories. Some examples are Pizza Pops, Michaelina’s Frozen Entrees, and Jamaican Patties.
  • Try to avoid set meal times. Make it a point to have a snack or a high calorie drink every 2 hours.
  • When snacking choose foods high in energy, such as nuts and nut butters, dried fruit, and cheese and crackers. Try to add extra calories whenever possible. For example add butter and gravy to mashed potatoes, or ice cream to milk for a milkshake!
  • Make your surroundings pleasant! Eat with a friend, play some of your favorite music, or go out to a favorite restaurant. Avoid stress and relax.
  • Liquids are often easier to take than solids when you’re not feeling well. Creamy soups, milkshakes, smoothies, and instant breakfast drinks are some examples of energy rich liquids.
  • Prescribed supplements such as EnsurePlus and Scandishakes are a high calorie alternative to homemade drinks and can boost your intake between 300 and 600 calories per drink.

The Ontario government covers the cost of nutritional supplements for people with CF. Examples of products covered include EnsurePlus®, BoostPlus®, Scandishakes®, Resource® 2.0, and tube feeding products. Please consult with your clinic dietitian to discuss which products are right for you.

To obtain nutritional supplements, you will first need to register with your clinic dietitian at St. Michael’s Hospital. The products are then ordered through the Specialty Food Shop at the Hospital for Sick Children.

Ordering in-store:

Visit the Specialty Food Shop at The Hospital for Sick Children (555 University Avenue) to pick up your products in-store. Please call ahead to verify that the amount you need is in stock. For large pick-up orders, two weeks advance notice is required.

Ordering by phone:

  1. Contact the Call Centre Monday – Friday 8 a.m. – 7 p.m. at 1-800-737-7976 and on Saturday at 416-813-5294 between 10 a.m. – 5 p.m., to place your order.
  2. Tell the customer service representative that you have a customer file with the Specialty Food Shop, and you are covered by the Cystic Fibrosis program from the MOHLTC.
  3. Provide your name, address and phone number and any additional information requested by the customer service representative.
  4. The cost of the products is covered by the MOHLTC. Shipping costs are covered by the customer, and are dependent on location of delivery and weight.

Ordering online:

  1. Visit specialtyfoodshop.ca
  2. To begin shopping, click on the Cystic Fibrosis category, located on the home page, under “Specialized Nutritional Needs.”
  3. Add items to cart and proceed to check out.
  4. A valid credit card with correct billing address will be required to pay for the shipping costs.

Adults who receive government disability assistance may be able to receive additional financial support through the ‘Special Diet Allowance’. This support can be given on a temporary or permanent basis.

An application for the Special Diet Allowance can be obtained from your government disability assistance case worker. For more information on how to apply, go to the Ontario Ministry of Children, Community and Social Services website.

Talk to your team to see if you may be eligible for this funding. Your CF dietitian can complete the application form.

Content coming soon.

Vitamins & Minerals

Many people with CF have problems when they eat food, making it hard for their intestines to absorb nutrients, especially fat. To help with this, enzymes are taken with meals and snacks. Vitamins A, D, E, & K are unique because they need fat in order to be absorbed by your body. They are called fat-soluble vitamins.

Even if you take enzymes regularly, you can still lose fat in your stool. If you are losing fat then you are probably also losing vitamins. If you don’t take your vitamins, over time, you may develop low levels in your blood. This is known as a vitamin deficiency.

Several minerals are particularly important for overall health in CF, including salt, calcium, iron, and zinc.

Vitamin A

Vitamin A has many important roles in the body. It helps you see at night and lets your eyes adjust when you move from a dark place to a bright place. If you have a vitamin A deficiency, your eyes may not be able to get used to changes in light. Vitamin A also helps make skin healthy, helps fight infection when you get sick, and helps with growth during childhood.

Vitamin A is found in dark green or orange fruits & vegetables, eggs, liver, and milk. For people with CF, food sources are not enough to prevent vitamin A deficiency, so a vitamin supplement is usually recommended. This can either be in the form of a multivitamin or a separate vitamin A pill.

Vitamin D

Vitamin D comes from two sources: it is made by the skin when it is exposed to sunlight, and it is found in the foods we eat. Vitamin D helps your body absorb calcium and helps move calcium from your blood to your bones. Without enough vitamin D, bones can weaken and the chances of breaking a bone become greater.

While foods such as eggs and fish contain vitamin D, the most common dietary source is milk and milk products. Most people living in Canada do not get enough vitamin D from food or the sun, so a vitamin supplement is often recommended.

Vitamin E

Vitamin E has become very popular because of its role as an antioxidant. As you grow, your body goes through many changes. As your body changes, it produces harmful products called oxidants that can destroy your healthy cells. Vitamin E helps get rid of these harmful products, which is why it is called an antioxidant. A deficiency of vitamin E can affect your body’s ability to protect itself when you get sick. For people with cystic fibrosis, this may affect your lungs and your ability to breathe.

Foods that contain vitamin E include plants oils, margarines, nuts, seeds, and some fruits & vegetables. Diet alone cannot prevent vitamin E deficiency in people with CF so a supplement is usually recommended.

Vitamin K

Most of the Vitamin K we need comes from our diet. It is found in green vegetables, plant oils, and margarine. Healthy bacteria in our intestines also produce vitamin K. Even though the bacteria in your intestine are healthy, they can be destroyed by antibiotics taken for a lung infection. This can affect the amount of vitamin K produced and absorbed on a daily basis.

Vitamin K is very important because it helps your blood clot. When you cut yourself, the time it takes for your cut to stop bleeding is affected by how much vitamin K you have in your body. The longer it takes to clot, the less vitamin K you may have. Vitamin K is also important for proper bone growth in children. To prevent a deficiency, many people with CF take a vitamin K supplement.

Most people with CF benefit from taking a multivitamin containing vitamin A and zinc (Centrum forte is commonly used in our clinic), a vitamin E supplement, vitamin D supplement (tablets or liquid drops), and vitamin K. If taking several different pills sounds daunting, there is a CF-specific vitamin called MVW that contains higher doses of the fat-soluble vitamins but is not covered under the CF drug program. Contact the team if you’re interested in paying out of pocket for this vitamin.

If you’re not getting enough calcium from your diet, your CF team may recommend a calcium supplement. You can use this calculator from BC Dairy to determine if you get enough calcium from the foods you eat. (Note that the site plays music upon loading.)

Most people can get enough iron from food, but an iron supplement may be recommended by your CF team.

A salt supplement is not typically needed unless you’re training.

G-Tubes

Many studies have shown a definitive relationship between good nutrition and improved lung function and survival in cystic fibrosis. In most cases, people with cystic fibrosis who are eating a high fat, high calorie diet and using pancreatic enzyme replacement (if needed) are able to meet their daily nutritional needs without too much difficulty.

However, a small group of people with CF, particularly those who have advanced lung disease, have trouble eating enough food to make up for the nutrient losses and energy demands caused by their illness. This is usually due to a combination of malabsorption of food, increased energy needs because of chronic chest infection, and poor appetite or poor eating habits. In order to overcome malnutrition, it is necessary to add calories to what is already being eaten, ideally in a way that does not further decrease appetite.

One way to add calories is with a G-tube (gastric feeding tube). This is a small tube, inserted through an incision in the abdomen into the stomach which can be used at any time to provide extra calories. Your CF team will help you decide if a G-tube is right for you.

“G” stands for “gastrostomy” which means an opening into the stomach. A G-tube is a short and hollow tube inserted into the stomach from the surface of the abdomen (tummy) through an opening called the gastrostomy site. The G-tube is used as an access to deliver extra nutrition for a person who needs it.

G-tube feeding is usually done during the night. Formula (also known as “nutritional supplements”) such as Ensure, Resource or Peptamen are infused over 6 to 8 hours. Occasionally, G-tube feeding may be carried out during the day when someone is sick or has too poor of an appetite to eat regular food.

No! G-tube feeding is just one way to help someone with CF to meet his/her total caloric requirements. In fact, it is designed to help meet only one-third of a person’s total daily calorie requirements. Therefore, a person who receives G-tube feeding should continue to eat regular meals and snacks throughout the day.
The procedure is usually performed by a radiologist (a specialist who is trained to interpret x-rays and performs x-ray guided diagnostic procedures). The procedure is usually done in the interventional x-ray department. The procedure lasts no more than 30 minutes. You will received mild sedation and local freezing to keep you comfortable while the G-tube is inserted.
Generally speaking, if you are in stable health, it should take no more than 5 – 7 days of hospital stay. Occasionally, someone may need to receive a course of IV antibiotics to treat his/her chest infection first before proceeding with the G-tube. In this case, your hospital stay could be as long as 2-3 weeks. You will be admitted to the hospital on the day before your G-tube insertion. After the G-tube is inserted, you will receive pain medication to keep you comfortable enough to do deep breathing and cough and perform your usual physiotherapy to avoid chest infection. The remaining hospital days will be used to help you test out the right type of tube feeding formula, the proper rate of feeding during the night, and how to manage your feeding equipment, i.e. setting up your night feeding, operating a feeding pump and how to clean your equipment.
First and foremost, knowing that you can meet one-third of your total daily calories by doing your night feeding can relieve a lot of pressure from having to “eat, eat, eat” during the day. Some people report that they enjoy their food a whole lot more just because they don’t feel the burden of having to finish the entire plate of food at meal time. Secondly, good nutrition helps to build better resistance to infection which keeps your lungs healthy. Good nutrition helps to build and strengthen muscles used for breathing; often people who start tube feeding are able to regain some lung function.
No. In our centre, once the G-tube site and G-tube tract are well healed, approximately 8 to 10 weeks after insertion, the initial G-tube will be replaced by a G-button, also called a low profile G-tube. This button is smaller than a quarter in size which can be easily hidden under clothing. How long you will need to have the G-button for night feeding depends on how well you are and how soon you are able to gain weight and improve your nutrition. For most people who require a G-tube to gain or maintain a healthy weight, it can be a very useful device to have for a long time.
Yes. A very small number of people who are able to keep a healthy weight for over a 6 month period without G-tube feeding and are able to stay in good health have had their G-tube removed. Also after successful lung transplantation, many people have their G-tube removed within 3 months because they no longer require such high caloric intake on a daily basis.
There are a few possible side effects but all of them are quite manageable. The most common one is a sensation of fullness or bloating in the morning after having been fed during the night. There are medications that can be added to the feed to speed up stomach emptying so that you don’t feel so full in the morning. Most people just learn to eat their breakfast a couple of hours later than usual.

Occasionally, a person who just started with G-tube feeding may develop softer or looser stool. The condition can be easily corrected by slowing down the rate of the feeding per hour or changing to another type of formula.

Another less common side effect is skin irritation or pain in the gastrostomy site. Skin irritation may be due to leakage or infection from or around the G-tube site. Leakage most likely is due to a ruptured balloon at the end of the G-button inside the stomach. When this happens, it is best to contact your CF clinic nurse to check on the G-tube site, change to a new G-button, and receive instruction about how to keep the affected skin area clean and dry. A culture swab may be collected to check for infection and an antibiotic cream or an oral antibiotic may be prescribed for you if your CF doctor feels it is needed.

In Ontario, the cost of the G-tube or G-button and all other G-tube supplies are covered partially by the government through a program called the Assistive Device Program. This program will cover 75% of the total cost of all the supplies. If you have private health insurance, most insurance plans will cover the remaining 25% of the total cost. For those people who have Ontario Disability Support Plan (ODSP), the remaining 25% of the cost will be covered by ODSP. The formula you use for G-tube feeding is covered entirely by the government under the CF special drug program. For any other questions regarding cost, please contact your CF team.

Inspect G-tube Site daily: A good time is while dressing or showering in the morning.

Observe the nature of drainage: Some form of light yellow-greenish mucusy drainage is common and it may have a slight odor. This drainage may persist in a small amount as long as the G-tube is in place, however, it tends to diminish in amount as the G-tube is replaced by a low profile G-button because of less movement against the tract with a well fitted button. Purulent drainage accompany with strong offensive odor is certainly suggestive of infection in and around G-tube site.

Skin Irritation: Can be caused by the leakage from feeds as a result of an unfit or poorly sealed G-tube or button. The longer the tube, the more rubbing will occur which can widen the tract and weaken the seal which causes leakage. If leakage is due to a defective button or ruptured balloon, changing into a new button will stop the leakage. It is important to treat this since leakage from the erosions may burn the skin area and cause inflammation. Treatments could include:

  1. Changing to a well fitting button
  2. Applying a saline soak to the affected area three or four times per day to promote healing and keep the skin area clean. Once the skin area is dried thoroughly, a barrier cream containing zinc oxide can be applied to the skin around the G-tube site to prevent further damage.
  3. Losec or Zantac may help if there is discharge and skin irritation. The balloon button also lasts longer if the acidity in the stomach is reduced.

Observe for signs of inflammation: Pink or red discoloring, swelling or tenderness around the gastrostomy site. If you notice any of these signs, contact your CF team. Cultures should be done to identify the bacteria and you may be started on an antibiotic cream or oral antibiotic to treat the infection.

Post-Transplant Nutrition

Before your transplant, you needed to eat extra food to provide your body with enough energy for breathing. Foods high in sugar and fat, like chips, chocolate bars, pop, and supplements such as Ensure® or Boost®, were good foods for you to eat to keep a healthy weight. Now, after your surgery, your body is not using all that extra energy to breathe so you may not need all the extra calories. Also, two side effects of steroids are an excellent appetite and weight gain. So, if you continue to eat the way you did before your transplant, you may gain weight really quickly!

Gaining weight after your surgery, so that you are in a healthy weight range, is very important to help you to heal and stay healthy. But, gaining too much weight can put you at risk of other health complications, such as diabetes and heart disease. To stay healthy, focus on a balanced diet which includes fruits, vegetables, whole grains, milk and milk products and meat and meat alternatives. Talk with your dietitian if you have questions about healthy eating, your healthy weight range and/or your daily nutritional needs.

Since “immunosuppressive” medications make it harder for your immune system to fight infection, it is very important that you do what you can to not get sick. One way is to watch what you eat. This is because certain foods can carry harmful bacteria and if your immune system is reduced, can make you sick. To stay healthy:

  • Always wash your hands before eating or preparing food.
  • Refrigerate or freeze foods immediately after a meal. Eat leftover foods within 1 to 2 days.
  • Wash all fresh fruits and vegetables before eating.
  • Most city water supplies in Canada are safe to drink; however, if you are unsure of the safety of the water supply, drink bottled water.
  • Wash knives, cutting boards and food preparation surfaces with hot water and soap after contact with raw poultry, meat, and seafood.
  • Consider having two cutting boards: one for raw meat and one for other foods.

Risky Foods To Avoid:

  • All raw and undercooked meats
  • Sushi, raw seafood and shellfish
  • Raw or undercooked eggs and foods containing them, such as, Caesar dressing, cookie dough, other batters
  • All unpasteurized milk, cheeses, ciders, juices and honey
  • All fresh sprouts