Nutrition


It’s important to talk about your weight and nutritional goals with your CF Care Team to make sure you’re getting all the energy and nutrients you need to help keep you healthy. Below are some things to keep in mind as you’re talking with your care team about your nutrition.

Nutrition

People with CF need more calories

People with CF need more energy to breathe normally, fight lung infections, and compensate for poor digestion. That means they need more calories than people without CF of similar age and weight. In fact, people with CF generally need up to twice the amount of daily calories. A high-calorie, high-fat diet, with 40% of total calories from fat, is generally recommended.

CF makes it hard for your body to absorb certain vitamins

Certain vitamins, such as A, D, E, and K, need fat in order to be absorbed by your body. But your body may have trouble absorbing fats, which means you may need to take supplements to help.

Only your CF Care Team can give you advice about your disease and treatment, so remember to talk with them about any questions you have. Here are a few questions you can ask to get you started:

  • What is considered a healthy rate of weight gain? 
  • How many calories should I eat every day?
  • How can I add healthy calories to meals?
  • Should pancreatic enzyme supplements be added to my food?
  • Should I take vitamins?

Exercise


Being active may also help manage your CF. It is important to discuss your exercise plans with your doctor and CF Care Team.

The effect of Pulmozyme on exercise tolerance has not been established in adults or children. Please see the accompanying full Pulmozyme Prescribing Information for additional Important Safety Information.

Exercise

Here are a few things to discuss with your CF Care Team and your doctor before getting started:

  • Discuss exercises that may be fun for you, as well as safe
  • Set small, manageable goals for your exercise plan
  • Create a routine that you're excited about, and that gradually allows you to do more
  • Work with your CF Care Team to discuss signs that may mean it’s time to take a break from the action

Cystic fibrosis support organizations


The more you know about CF, the easier it will be to manage it. Click on the links below to find helpful information on patient advocacy, research, education, CF community, and CF support for parents and caregivers.

Cystic Fibrosis (CF) support organizations

The Blooming Rose Foundation (BRF) was created to give hope to families immediately following CF diagnosis. BRF offers an online resource for individuals, families, and friends to find up-to-date research, links, and ways to connect with other families and adults with CF.

This foundation is committed to raising awareness about CF and funding CF research, and it provides numerous ways to get involved in the fight against CF.

A lifestyle blog started by the Patient Outreach director of the Boomer Esiason Foundation, Gunnar, who has CF. Provides patient-focused information about living with CF, including blog posts, podcasts, and interviews.

The Cystic Fibrosis Foundation is the leading organization in the United States devoted to CF, and its website provides a wealth of information. The mission of this nonprofit, donor-supported organization is to ensure the development of the means to cure and control CF and to improve the quality of life for those with the disease.

CFRI is a nonprofit organization founded in 1975. CFRI's mission is to fund research, provide educational and personal support, and spread awareness of CF, a life-threatening genetic disease.

A community for people concerned with CF. The website contains interactive forums and blogs to find support and share information, while celebrating and remembering with the community.

Cystic-L is a free email support group dedicated to the exchange of information and support specific to CF. Members include those with CF, as well as family members, medical professionals, researchers, and more.

A social network just for the CF community. This website provides a place for the CF community to share tips, questions, ideas, experiences, and encouragement.

Get a full overview of the disease along with information about symptoms, causes, risk factors, and treatments, as well as what to expect during appointments with your medical care team.

The contents of the above websites are not intended for the purpose of disease diagnosis or a substitute for information that is provided to you by your doctor. You should always discuss your individual symptoms and any questions you have with your doctor. Genentech, Inc. is not responsible for the accuracy of the information contained on third-party websites and does not recommend or endorse the content provided on these websites.

 

Indication & Usage

Pulmozyme (dornase alfa) is indicated for daily administration along with standard therapies for the management of cystic fibrosis (CF) patients to improve pulmonary function.

In CF patients with an FVC ≥ 40% of predicted, daily administration of Pulmozyme has also been shown to reduce the risk of respiratory tract infections requiring injectable antibiotics.

Important Safety Information

Pulmozyme should not be used in patients who are allergic to any of its ingredients.

Patients may experience the following when using Pulmozyme: change in or loss of their voice, discomfort in the throat, rash, chest pain, red watery eyes, runny nose, lowering of lung function, fever, indigestion, and shortness of breath. There have been no reports of severe allergic reactions caused by the administration of Pulmozyme. Mild to moderate hives and mild skin rash have been observed and have been short-lived.

Pediatric Use

The safety and effectiveness of Pulmozyme have been established in patients 5 years of age and older. While clinical trial data are limited in patients younger than 5 years of age, the use of Pulmozyme should be considered for pediatric CF patients who may experience potential benefit in lung function or who may be at risk of respiratory tract infection.

The safety of Pulmozyme given by daily inhalation for 2 weeks has been studied using 98 CF patients with 65 of them aged 3 months to <5 years (younger group) and 33 aged 5 years to <10 years (older group). The PARI BABY™ reusable nebulizer (which uses a face mask instead of a mouthpiece) was used in patients who were unable to show that they could breathe in or out using their mouth throughout the entire treatment period. Overall, the kind of side effects observed in children was similar to those seen in larger trials in older patients.

You are encouraged to report side effects to Genentech and the FDA. You may report side effects to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. You may also report side effects to Genentech at 1-888-835-2555.

Please see full Pulmozyme Prescribing Information for additional Important Safety Information. If you have questions, please discuss them with your CF Care Team.