ORKAMBI Food Guide
®
What Is ORKAMBI?
ORKAMBI is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients 12 years and older
who have two copies of the F508del mutation (F508del/F508del) in their CFTR gene.
ORKAMBI should not be used in patients other than those who have two copies of the F508del mutation in their
CFTR gene.
It is not known if ORKAMBI is safe and effective in children under 12 years of age.
ORKAMBI and Fat-Containing Foods
This food guide will help you:
• Learn about taking ORKAMBI with fat-containing foods
• G
et ideas for fat-containing meals and snacks
• K
eep your diet interesting with recipes for fat-containing foods
• B
e ready for shopping with a take-along list of fat-containing foods
How to Take ORKAMBI
The recommended dose of ORKAMBI is 2 tablets every 12 hours. Your dose may be different. Your
healthcare provider will tell you how much ORKAMBI to take and when to take it.
Always take ORKAMBI with fat-containing food.
Continue to take all of your other prescribed CF therapies as directed by your healthcare provider.
IMPORTANT SAFETY INFORMATION
Who should not take ORKAMBI?
Do not take ORKAMBI if you take certain medicines or herbal supplements, such as:
• antibiotics: rifampin (Rifamate®, Rifater®) or rifabutin (Mycobutin®)
• seizure medicines: phenobarbital, carbamazepine (Tegretol®, Carbatrol®, and Equetro®) or phenytoin
(Dilantin®, Phenytek®)
• sedatives/anti-anxiety medicines: triazolam (Halcion®) or midazolam (Dormicum®, Hypnovel®, and Versed®)
• immunosuppressant medicines: everolimus (Zortress®), sirolimus (Rapamune®), or tacrolimus (Astagraf XL®,
Envarsus XR®, Prograf®, and Protopic®)
• St. John’s wort (Hypericum perforatum)
Talk to your doctor before taking ORKAMBI if you take any of the medicines or supplements listed above.
Please see additional Important Safety Information on page 2.
Please click the following links to see full Prescribing Information, including Patient Information.
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What to Do if You Miss a Dose
• If you miss a dose within 6 hours of when you usually take it, take your dose with fat-containing
food as soon as possible
• If you miss a dose, and it is more than 6 hours after the time you usually take it, skip that dose only
and take your next dose when you’re supposed to. Do not take 2 doses at the same time to make
up for your missed dose
The Importance of Taking ORKAMBI® With Fat-Containing Foods
ORKAMBI should always be taken with fat-containing foods because the fat helps your body better
absorb the medicine.
Examples of fat-containing foods you can take with ORKAMBI include eggs, avocados, nuts, butter,
peanut butter, cheese pizza, and whole-milk dairy products (such as whole milk, cheese, and yogurt).
You’ll find many more foods to choose from on the following pages.
IMPORTANT SAFETY INFORMATION (continued)
What should I tell my doctor before taking ORKAMBI?
Before you take ORKAMBI, tell your doctor if you:
• h
ave or have had liver problems
• h
ave kidney problems
• a
re using birth control (hormonal contraceptives, including oral, injectable, transdermal, or implantable forms).
Hormonal contraceptives should not be used as a method of birth control when taking ORKAMBI. Talk to your
doctor about the best birth-control method you should use while taking ORKAMBI.
• a
re pregnant or plan to become pregnant. It is not known if ORKAMBI will harm your unborn baby. You and
your doctor should decide if you will take ORKAMBI while you are pregnant.
• a
re breastfeeding or planning to breastfeed. It is not known if ORKAMBI passes into your breast milk. You and
your doctor should decide if you will take ORKAMBI while you are breastfeeding.
Please see additional Important Safety Information on page 3.
Please click the following links to see full Prescribing Information, including Patient Information.
2
Understanding Different Types of Fats
For a person with CF, fats are an important part of the diet. There are 2 types of fats: unsaturated
and saturated.
• U
nsaturated fats (monounsaturated and polyunsaturated) are found in some vegetable oils, nuts,
fish, and seeds
• S
aturated and trans fats are found in foods like meat, some cookies and pastries, and fried foods
To get a better understanding of the different types of fats, speak with your healthcare team. They
can help you choose healthy fat-containing meals and snacks.
Tips for Taking ORKAMBI®
Set reminders on an alarm clock, watch, phone, or computer (yours or the person’s you care for) as a
way to remember that it’s time to take ORKAMBI.
Be sure you always have easy-to-grab fat-containing foods on hand.
IMPORTANT SAFETY INFORMATION (continued)
What should I tell my doctor before taking ORKAMBI?
ORKAMBI may affect the way other medicines work, and other medicines may affect how ORKAMBI works.
Tell your doctor about all the medicines you take, including prescription and over-the-counter medicines,
vitamins, and herbal supplements, because the dose of ORKAMBI may need to be adjusted when taken with
certain medicines.
Especially tell your doctor if you take:
• a
ntifungal medicines, such as ketoconazole (e.g., Nizoral®), itraconazole (e.g., Sporanox®), posaconazole
(e.g., Noxafil®), or voriconazole (e.g., Vfend®)
• a
ntibiotics, such as telithromycin (e.g., Ketek®), clarithromycin (e.g., Biaxin®), or erythromycin (e.g., Ery-Tab®)
What should I know when taking ORKAMBI?
Tell your doctor if you stop ORKAMBI for more than 1 week. Your doctor may need to change your dose of
ORKAMBI or other medicines you take.
It is unknown if ORKAMBI causes dizziness. Do not drive a car, use machinery, or do anything that needs you to be
alert until you know how ORKAMBI affects you.
Please see additional Important Safety Information on page 4.
Please click the following links to see full Prescribing Information, including Patient Information.
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Frequently Asked Questions About ORKAMBI and Fat-Containing Foods
®
Should ORKAMBI be taken before, during, or after a meal? And can it also be taken with
a snack?
ORKAMBI should be taken every 12 hours, with or immediately following a meal or snack that
contains fat. Remember, fat-containing food helps your body absorb ORKAMBI better.
If you need more information about taking ORKAMBI with meals, ask your healthcare provider.
Why does ORKAMBI need to be taken with fat-containing foods?
Fat-containing food helps the body absorb the lumacaftor and ivacaftor in ORKAMBI better. You should always
take ORKAMBI with fat-containing food.
A fat-containing food is not necessarily a high-fat food. Talk with your healthcare provider about the best options
for fat-containing meals and snacks. Some examples of fat-containing foods are eggs, avocados, nuts, butter,
peanut butter, cheese pizza, and whole-milk dairy products (such as whole milk, cheese, and yogurt).
IMPORTANT SAFETY INFORMATION (continued)
What are the possible side effects of ORKAMBI?
ORKAMBI can cause serious side effects.
High liver enzymes in the blood, which can be a sign of liver injury, have been reported in patients receiving
ORKAMBI. Your doctor will do blood tests to check your liver:
• before you start ORKAMBI
• every 3 months during your first year of taking ORKAMBI
• every year while you are taking ORKAMBI
Call your doctor right away if you have any of the following symptoms of liver problems:
• p
ain or discomfort in the upper right stomach (abdominal) area
• y
ellowing of your skin or the white part of your eyes
• loss of appetite
• n
ausea or vomiting
• d
ark, amber-colored urine
• c
onfusion
Please see additional Important Safety Information on page 5.
Please click the following links to see full Prescribing Information, including Patient Information.
4
Frequently Asked Questions About ORKAMBI and Fat-Containing Foods
®
How much fat should be eaten when taking ORKAMBI?
There is no specific guidance as to the amount of fat content to be eaten with ORKAMBI. Your
healthcare provider can help you choose healthy, fat-containing meals and snacks.
Here are some examples of how much fat is in certain foods. The fat content is approximate:
—1 large hard-boiled or poached egg has 5 grams of fat
—1 medium blueberry muffin has 18 grams of fat
—1 8-ounce serving of whole-milk yogurt has 7 grams of fat
—1 tablespoon of peanut butter has 8 grams of fat
You should always follow the guidance of your healthcare provider about foods that are right for you.
What are some ways to remember to take ORKAMBI every 12 hours?
Here are some ideas:
• S
et an alarm on a clock, watch, cell phone, or computer (yours or the person’s you care for)
• T
ake ORKAMBI tablets with something you do every day at the same time
• C
hoose times that are convenient for you, every day of the week, and even on the weekend
Remember to take ORKAMBI exactly as your healthcare provider tells you.
IMPORTANT SAFETY INFORMATION (continued)
What are the possible side effects of ORKAMBI? (continued)
Respiratory events such as shortness of breath or chest tightness were observed in patients when starting
ORKAMBI. If you have poor lung function, your doctor may monitor you more closely when you start ORKAMBI.
Abnormality of the eye lens (cataract) has been noted in some children and adolescents receiving ivacaftor,
a component of ORKAMBI. Your doctor should perform eye examinations prior to and during treatment with
ORKAMBI to look for cataracts.
The most common side effects of ORKAMBI include: shortness of breath and/or chest tightness; upper
respiratory tract infection (common cold), including sore throat, stuffy or runny nose; gastrointestinal symptoms,
including nausea, diarrhea, or gas; rash; fatigue; flu or flu-like symptoms; increase in muscle enzyme levels; and
irregular, missed, or abnormal periods (menses) and increase in the amount of menstrual bleeding.
Tell your doctor if you have any side effect that bothers you or that does not go away.
These are not all the possible side effects of ORKAMBI. For more information, ask your doctor or pharmacist.
Call your doctor for medical advice about side effects. You are encouraged to report side effects to FDA at
1-800-FDA-1088.
For further information, please see full Prescribing Information.
Please click the following links to see full Prescribing Information, including Patient Information.
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Suggestions for Fat-Containing Foods to Eat When Taking ORKAMBI
®
When you take ORKAMBI, the following may help you decide which fat-containing foods to eat with it. If you take
ORKAMBI at a time other than mealtime, there are also some snack ideas that contain fat.
Breakfast
Dinner
Oatmeal with almonds and
dried cranberries
Green salad with
blue-cheese dressing
Whole-milk yogurt
Salmon with brown rice
Scrambled eggs with
avocado and toast
Spaghetti and meatballs
Bagel with peanut butter
Beef tacos with shredded
cheese
Breakfast bean and
cheese burrito
Cheeseburger with salad
Hazelnut spread on toast
Macaroni and cheese
Cream cheese on a wholewheat bagel
Cheese pizza
Peanut butter on an
English muffin
Pork chops
Granola with whole milk
Fettuccine alfredo
Fat-Containing Snacks
Prepackaged
Quick fix
Chocolate-covered ice
cream bar
Sliced apples with
peanut butter
Peanut butter crackers
Hard-boiled egg
Trail mix with nuts
Cottage cheese with fruit
Whole-milk string cheese
Chips and guacamole
Please see Important Safety Information on pages 1-5.
Please click the following links to see full Prescribing Information, including Patient Information.
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Recipes
Here are some easy-to-make recipes for snacks and meals. You can prepare these ahead of time, so they will be
ready to eat when you need them.
High-Calorie Hummus
Ingredients
• 2
cups cooked or canned chickpeas (also called
garbanzo beans)
• 4
-5 tablespoons of high-fat Greek yogurt
• 1
tablespoon tahini
• J uice of half a lemon (lemon juice okay too)
• S
cant teaspoon of ground cumin
• 2
cloves garlic
• S
alt and pepper
Directions
1. Combine all ingredients and blend well.
2.Taste as you go! You can add more of any of the
ingredients if it doesn’t taste right to you—just be careful with the salt.
3.Blend as long as you like. Some people like it chunky, some people like it smooth.
4.Season with salt and pepper to taste.
Source: www.cff.org/PDF-Archive/This-Eating-Stuff-is-Hard-Work!-Recipe-Book/
Calories per serving (¼ cup): 385 | Fat: 8 g
Carbohydrates: 58 g | Protein: 23 g
7
Spinach Dip
Ingredients
• 1
10-oz. package frozen chopped
spinach (or 2 cups fresh spinach)
• 1
package Knorr® vegetable soup mix
• 1
cup mayonnaise
• 1
6 oz. sour cream
• ½
cup shredded carrots
Directions
1. If using frozen spinach, thaw and
squeeze out water.
2.Combine all ingredients.
3.Chill for an hour.
4.Serve in hollowed-out loaf of bread or
with fresh vegetables, bread cubes, or crackers.
Source: www.cff.org/PDF-Archive/This-Eating-Stuff-is-Hard-Work!-Recipe-Book/
Calories per serving (4 tbsp): 132 | Fat: 11 g
Carbohydrates: 7.4 g | Protein: 1.8 g
8
Cheesy Tuna Casserole
Makes 4 servings
Ingredients
• 7
oz. uncooked macaroni
• 1
cup milk
• 4
oz. shredded cheddar cheese
• 1
(10¼ oz.) can condensed
cream of mushroom soup
• 1
(6 oz.) can tuna
• 2
teaspoons minced dried onion
• ½
teaspoon dry mustard
• C
rushed potato chips
Directions
1. Cook macaroni to desired doneness, drain.
2. Meanwhile, heat oven to 350º.
3. Spray casserole with nonstick cooking spray.
4. In large bowl, combine all remaining casserole ingredients; stir to combine.
5. Add cooked macaroni, stir gently to mix.
6. Pour mixture into casserole. Sprinkle with crushed potato chips.
7. Bake at 350º for 25 to 35 minutes.
Source: www.food.com/recipe/cheesy-tuna-casserole-189570
Calories per serving (255 g): 465.6 | Fat: 18.8 g
Carbohydrates: 46.1 g | Protein: 26.8 g
9
Fully Loaded Pasta
Makes 4 servings
Ingredients
• 1
can of Campbell’s® Chunky™ Fully
Loaded Creamy Chicken Alfredo Soup
• A
16-oz. box of your favorite pasta
Directions
1. Heat soup according to directions on can.
2.Boil water and cook pasta according to
directions on box.
3.Drain pasta and pour soup over pasta.
Source: w ww.cff.org/PDF-Archive/This-Eating-Stuff-is-Hard-Work!-Recipe-Book/
Calories per serving: 485 | Fat: 10 g
Carbohydrates: 76 g | Protein: 24 g
10
Sesame Chicken Bites With Honey Mustard Dipping Sauce
Makes 4 servings
Ingredients
• 1
pound boneless chicken, cubed
• ½
cup mayonnaise
• 1
teaspoon onion powder
• 1
teaspoon ground mustard
• ¼
teaspoon pepper
• ½
cup dry bread crumbs
• ½
cup sesame seeds
• 1
tablespoon minced fresh parsley
• 3
-4 tablespoons vegetable oil
Directions
1. M
ix mayonnaise, onion powder, ground mustard, and pepper in a gallon-size
zip-top bag.
2.Add chicken, and shake and massage bag to coat all pieces.
3.In a separate gallon-size zip-top bag, add bread crumbs, sesame seeds, and
parsley.
4.Remove chicken from the mayo mixture and add to the bread crumb mixture.
5.Shake bag until all pieces are coated with bread crumbs.
6.Cook in a large skillet in 3-4 tablespoons of hot vegetable oil until chicken is no
longer pink inside.
Dipping Sauce
Ingredients
Directions
• ½
cup mayonnaise
1. Whisk all ingredients together.
• 1
½ teaspoons dijon mustard
2.Add more mustard for a spicier flavor
or more honey for a sweeter flavor.
• 4
½ teaspoons honey
Source: www.cff.org/PDF-Archive/This-Eating-Stuff-is-Hard-Work!-Recipe-Book/
Calories per serving: 485 | Fat: 10 g | Carbohydrates: 76 g | Protein: 24 g
11
English Muffin Pizza
Makes 4 servings
Ingredients
• 6
English muffins (12 halves)
• ¾
-1 cup spaghetti sauce
• 1
cup mozzarella (or your favorite)
cheese, shredded
• 3
6 slices pepperoni
Directions
1. Preheat oven to 350º.
2.Split open the whole English muffins,
and arrange the 12 halves onto a
baking sheet.
3.Spread 1-2 spoonfuls of spaghetti sauce onto muffin halves—do not use too much
or it will make the muffin bottoms soggy.
4.Sprinkle muffins with shredded cheese.
5.Arrange 2-3 pepperoni slices on top of the cheese.
6.Put muffins in oven and bake for 15-20 minutes or until cheese is melted and
muffins are toasted.
7.Additional toppings: Green peppers, onions, olives, ham, sausage, hamburger,
mushrooms, peppers, salami, artichoke hearts, tuna, breakfast toppings (eg, eggs,
ham) etc—let your imagination run wild!
Source: www.food.com/recipe/english-muffin-pizzas-166903
Calories per serving (3 muffin halves): 387.9 | Fat: 16.3 g
Carbohydrates: 41.7 g | Protein: 18.3 g
12
Monkey Bread
Makes 12 servings
Ingredients
• ½
cup sugar
• 1
teaspoon cinnamon
• 2
cans (16.3 oz. each) Pillsbury Grands®
Homestyle refrigerated biscuits
• ½
cup chopped walnuts (if desired)
• ½
cup raisins (if desired)
• 1
cup packed brown sugar
• ¾
cup butter, melted
Directions
1. Heat oven to 350º.
2.Lightly grease 12-cup fluted pan with shortening or cooking spray.
3.In large food-storage plastic bag, mix sugar and cinnamon.
4.Separate dough into 16 biscuits; cut each into quarters. Shake in bag to coat.
5.Arrange in pan, adding walnuts and raisins among the biscuit pieces.
6.In small bowl, mix brown sugar and butter and pour over biscuit pieces.
7. Bake 28-32 minutes or until golden brown and no longer doughy in the center.
8.Cool in pan 10 minutes. Turn upside down onto serving plate; pull apart to serve.
Serve warm.
Source: www.cff.org/PDF-Archive/This-Eating-Stuff-is-Hard-Work!-Recipe-Book/
Calories per serving: 450 | Fat: 22 g
Carbohydrates: 58 g | Protein: 5 g
13
Peach and Yogurt Shake
Makes 1 serving
Ingredients
• 1
cup sliced peaches
• 1
cup plain yogurt
• 1
cup whole milk
• 1
tablespoon honey
Directions
1. Combine all ingredients in a blender.
2. Blend until smooth.
Calories per serving (1 shake): 230 | Fat: 8 g
Carbohydrates: 33 g | Protein: 8 g
14
Shopping List for Fat-Containing Foods
Take this shopping list along with you when you go to the grocery store to make shopping for fat-containing foods
a little easier. We’ve included the approximate grams of fat contained in these foods to help you get a better
understanding of how different foods compare in fat content.
Food
Fat content*
Whole almonds
17.5 g per ¼ cup
Almond butter
9 g per tbsp
American cheese
6 g per slice
Whole avocados
29 g each
Blueberry muffins
18 g (1 medium)
Frozen bean and cheese burritos
8 g per burrito
Butter
11.5 g per tbsp
Caesar salad dressing
8.5 g per tbsp
Cheddar cheese
9.3 g per 1-oz. slice
Chocolate-flavored hazelnut spread
11 g per 2 tbsp
Cream cheese
10 g per oz.
Frozen onion rings
12 g (6 rings)
Eggs
7 g per egg, fried, scrambled, or omelet
5 g per egg, hard-boiled or poached
*
Frozen yogurt
6 g per cup
Hummus
24 g per cup
Microwaveable macaroni and cheese
13 g per 7.5-oz. serving
Mayonnaise
5 g per tbsp
Ready-to-eat granola
6 g per ½ cup
Parmesan cheese
8 g per oz.
Peanut butter (smooth)
16 g per 2 tbsp
Plain yogurt (whole milk)
8 g per 8-oz. container
Salmon
23 g per 6 oz.
Thousand Island salad dressing
5.6 g per tbsp
Whole milk
8 g per cup
Buttermilk biscuits (refrigerated dough)
4.5 g per biscuit
Mozzarella cheese (whole milk)
6 g per oz.
Fat content is approximate and derived from the USDA National Nutrient Database.
ORKAMBI is manufactured for Vertex Pharmaceuticals Incorporated.
ORKAMBI, Vertex, and the Vertex triangle logo are registered trademarks of Vertex Pharmaceuticals Incorporated.
All other trademarks referenced herein are the properties of their respective owners.
© 2016 Vertex Pharmaceuticals Incorporated | VXR-US-02-01153a | 03/2016
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