Cystic fibrosis

Overview

CF affects the lungs, pancreas, and other organs.

CFTR modulator medicines have greatly improved lung function for many people.

Symptoms

Common signs:

• Ongoing cough

Less common

• Lung infections
• Poor weight gain

When to get medical care

Causes

Genes.

Does it spread or run in families?

• It isn't contagious. You can't catch it from someone else.
• It can run in families. Tell your doctor if a parent, brother, or sister has it.

Who is more likely to get it

• Family history
• Children of two carrier parents: 1 in 4 chance
• White people of Northern European descent: Most common

Possible complications

• Diabetes: CF-related diabetes
• Bronchiectasis: Widened, damaged airways

Diagnosis

A clinician asks about your symptoms and may order tests.

The exam

A physical exam.

Tests

Tests, if needed:

• Sweat test:

Treatment

Treatment depends on the cause and how severe it is.

Self-care

• Daily airway clearance
• Enzymes with meals

Which doctor treats this

• Start with your primary care provider (family doctor or internist).
• They may send you to a pulmonologist (lung doctor).

Living with it

• Keep a simple diary of symptoms, triggers, and medicines to bring to visits.
• Bring a list of your medicines and questions to each visit.
• Stay active in ways that feel good to you.
• Get enough sleep and manage stress.
• Long-term illness can affect mood. Tell your doctor if you feel down or anxious.

Support groups and helplines

• Cystic Fibrosis Foundation: Care center finder and help for families.
• Genetic and Rare Diseases Information Center (GARD): Free NIH information on rare diseases, including where to find experts and patient groups.
• National Organization for Rare Disorders (NORD): Patient groups, help finding care, and aid programs for rare diseases.

Back to school, work, or sports

• Most people can keep working or going to school.
• Ask about changes that help, like extra breaks or a flexible schedule. Workers may be covered by the ADA. Students may get a 504 plan.

Children, pregnancy, and older adults

• Babies: Newborn screening finds most cases.

Pregnancy and breastfeeding

• If you're pregnant or planning to be, tell your prenatal provider about this condition.
• Don't start or stop any medicine on your own. Some are safe in pregnancy and some need to be changed.
• Ask before taking any pain reliever. The FDA advises avoiding ibuprofen and naproxen after 20 weeks unless your doctor says to.
• Breastfeeding: Most medicines are fine, but ask. The NIH LactMed database lists what's known about each one.

For caregivers

• Go to visits when you can and take notes.
• Keep a list of medicines, allergies, and doctors in one place.
• Learn the warning signs that need fast care.
• Take breaks and ask for help. Caregivers need care too.

Outlook

Life expectancy keeps improving.

Getting ready for your visit

Bring a list of your symptoms and medicines.

What to bring

• A list of your medicines and supplements
• Notes on when symptoms started and what makes them better or worse

Questions to ask

• What's causing this?
• What treatment do you recommend?
• Am I eligible for modulator therapy?

Research studies

Research studies test new ways to find and treat health problems. Some are looking for volunteers. Talk with your doctor before joining one.

Search ClinicalTrials.gov for studies on Cystic fibrosis