Living with Cystic Fibrosis
A diagnosis of CF is life changing for a family. However, in the last 2 to 3 decades, significant strides have been made so that children born today with CF have a completely different outlook than those born 30 or 40 years ago. People with CF are not only living much longer but also have a better quality of life.
What to Expect
You will meet with the care team (physicians, CF care coordinator, nurses, respiratory therapists, nutritionist, and social worker) who will explain the condition, map out a CF care strategy, and discuss resources.
Some children will need baseline lab work or X-rays at the first visit, depending on their age. Throat cultures are obtained four to six times per year. Newborns are seen very frequently until a feeding regimen, enzyme dosage, and weight gain are established.
Hospitalizations may be required for complications, such as intestinal blockage or pulmonary exacerbations. Ongoing medical treatment tries to keep these to a minimum, and address them in the outpatient setting whenever possible.
Managing Cystic Fibrosis
Management of CF centers on nutrition and lung health.
Maintaining normal weight is very important, as it has been shown to be linked to lung health. A person with CF needs to take in adequate calories. This is sometimes hard to achieve without the use of nutritional supplements or other means, such as a feeding tube. You may also need to take in more salt, especially if you are sweating. An appropriate enzyme and vitamin dose is also very important in the management of CF.
People with CF usually need to do daily airway clearance therapy (ACT) and may need to use inhaled medications or even antibiotics when necessary. It is also important to exercise and stay active for your lung health. You should also make sure any respiratory tract infections are treated promptly. If you think you have an infection, call your doctor, so you can receive medication.
The Lung Association recommends patients and caregivers join our Living with Lung Disease Support Community to connect with others facing this disease. You can also call the Lung Association's Lung Helpline at 1-800-LUNGUSA to talk to a trained respiratory professional who can help answer your questions and connect you with support.
Parent support groups and the local CF chapters of the CF Foundation are also available to help support parents of newly diagnosed CF in children, as well as in older individuals with CF. In addition, the social worker on the CF care team provides counseling on many issues, including:
- School concerns - schools need to be aware of the child’s diagnosis so that appropriate measures are put in place and the child can receive the support they need.
- Insurance – advice on policy selection and additional resources to make the medications used to treat CF affordable.
- Jobs - advice on communicating with your employer, retirement, and disability.
The CF Patient Assistance Foundation helps people with CF afford medication and the devices needed to stay healthy. It also assists individuals in applying for supplemental social security and social security disability benefits.
There is also a CF legal hotline that provides information for individuals with CF about their rights.
Last updated April 4, 2018.
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