Living with cystic fibrosis (CF) means building an ongoing relationship with a care team — pulmonologists, respiratory therapists, dietitians, and others who work together to help you breathe better and stay as healthy as possible. But appointments can feel rushed, and it's easy to leave the clinic wishing you had remembered to ask something important.
This checklist is designed to help people with CF — and the family members and caregivers who support them — arrive at appointments ready to have productive, informed conversations. It covers lung function, medications, airway clearance, lifestyle, and what to watch for between visits. Think of it as a starting point: your clinician knows your specific situation best, so use these questions to open a dialogue rather than to draw conclusions on your own.
Understanding Your Lung Function
Spirometry — the breathing test that measures how much air you can move in and out of your lungs — is a cornerstone of CF monitoring. The results help your care team track how your lungs are doing over time and whether your current treatment plan is working.
- What do my spirometry results mean for me right now, and how do they compare to my previous measurements?
- Is my FEV1 (the amount of air I can force out in one second) trending stable, improving, or declining? What would a meaningful change look like?
- Are there other lung function tests or imaging — such as a chest CT — that would give us a fuller picture of what's happening in my airways?
- How often should I be tested, and is there anything I should do before my next spirometry to make sure the results are accurate?
Reviewing Your Medications
CF treatment has changed significantly in recent years. A class of drugs called CFTR modulators works at the molecular level to help the defective protein made by the CFTR gene function more normally. Not everyone with CF carries a mutation that responds to these therapies, so it's worth having an up-to-date conversation about what's available for your specific genetic profile.
- Am I on the right CFTR modulator for my mutation, or has my mutation been tested to see whether I qualify for one?
- Are there newer therapies or updated dosing guidelines that might apply to me since my last appointment?
- What inhaled medications am I taking, and what is each one doing — for example, thinning mucus, opening airways, or fighting infection?
- Am I taking my medications in the correct order? (Some inhaled therapies work better when given after airway clearance or before others.)
- Are there any interactions between my CF medications and anything else I take, including supplements or over-the-counter medicines?
- What should I do if I miss a dose, and how do I store my medications correctly?
Airway Clearance Techniques
Because CF causes thick, sticky mucus to build up in the airways, clearing that mucus every day is one of the most important things a person with CF can do to protect lung function. There are several techniques and devices available, and the right approach often depends on age, lung function, lifestyle, and personal preference.
- Is my current airway clearance routine still the right fit for me, or should we consider adjusting the frequency, duration, or technique?
- Are there devices — such as a high-frequency chest wall oscillation vest, a flutter valve, or a positive expiratory pressure (PEP) mask — that might work better for my lifestyle?
- What role does exercise play in airway clearance for someone at my stage of CF? Can physical activity replace or supplement my routine?
- How do I know if my airway clearance is actually working? What signs suggest I need to do more, or that something has changed?
Managing Infections and Exacerbations
Bacterial infections in the lungs are a major driver of CF progression. Organisms like Pseudomonas aeruginosa and Staphylococcus aureus can take up long-term residence in CF airways, and keeping them in check often requires both inhaled and oral antibiotics. Knowing your own infection history — and the warning signs of a pulmonary exacerbation — is essential.
- Which bacteria are currently growing in my sputum cultures, and are they responding to my current antibiotic regimen?
- What are the early warning signs of a pulmonary exacerbation — a worsening of symptoms — that should prompt me to call your office rather than wait for my next scheduled visit?
- When would you recommend IV antibiotics versus oral or inhaled ones?
- How do I reduce my risk of picking up new infections, especially when I'm around other people with CF, in healthcare settings, or during cold and flu season?
- Are there vaccines I should be up to date on to protect my lungs, such as influenza, pneumococcal, or COVID-19 vaccines?
Nutrition, Digestion, and Related Health Issues
CF affects the pancreas in most people who have it, which can interfere with the digestion and absorption of fats and fat-soluble vitamins. Good nutrition is directly linked to better lung outcomes, making this part of the conversation just as important as the breathing questions.
- Am I absorbing enough calories and nutrients? How does my weight or body composition look compared to my CF-related targets?
- Is my pancreatic enzyme replacement dose correct, and am I taking enzymes at the right time relative to meals and snacks?
- Should my vitamin levels — especially vitamins A, D, E, and K — be tested, and are my current supplements adequate?
- Do I show any signs of CF-related diabetes (CFRD), and how often should I be screened for it?
- Is my bone density being monitored? CF and certain medications can affect bone health over time.
Mental Health and Quality of Life
Living with a chronic condition takes a real toll on emotional wellbeing. Research consistently shows that people with CF experience higher rates of anxiety and depression than the general population, and that addressing mental health is part of comprehensive CF care — not a separate concern.
- Does this clinic have a mental health professional — such as a psychologist or social worker — who is familiar with CF?
- Are there resources for caregiver burnout, especially for parents of children with CF?
- How do other patients manage the daily treatment burden? Are there strategies or support groups you'd recommend?
- Is there anything about my emotional health I should be tracking or reporting between visits?
Planning for the Future
CF care is increasingly about long-term planning — whether that means transitioning from pediatric to adult care, thinking about fertility and family planning, considering advanced therapies, or simply knowing what to expect as you get older.
- If I'm approaching adulthood, how will the transition to an adult CF program work, and when should we start planning for it?
- What do I need to know about CF and fertility or pregnancy? (CF affects fertility differently in men and women, and some medications require special consideration during pregnancy.)
- At what point would you start a conversation about lung transplant evaluation, and what does that process look like?
- Are there clinical trials I might be eligible for, and where can I find reliable information about CF research?
Between Appointments: What to Watch For
Your care team can't see what happens on a Tuesday afternoon at home. Knowing which changes to report — and when — helps you become an active partner in your own care.
- Increased cough or a change in the color, amount, or thickness of mucus
- Shortness of breath that is new or noticeably worse than usual
- Coughing up blood
- Fever or general sense of feeling more unwell than your baseline
- Unexplained weight loss or decreased appetite
- Any chest pain
A note on preparation: Writing your questions down before an appointment — and bringing someone you trust who can help you remember what was discussed — can make a real difference. Many CF care teams also welcome secure messages or patient portal questions between visits. Don't wait until your next scheduled appointment if something feels off.
A Final Word
Cystic fibrosis is a complex, lifelong condition, but the science of managing it has never been more promising. The most effective thing you can do alongside your medications and therapies is stay engaged with your care team, ask questions freely, and speak up when something changes. This checklist is a tool to help you do exactly that — not a substitute for the personalized guidance only your clinician can provide.
If you or someone you love is newly diagnosed or navigating a major transition in care, organizations that specialize in CF can also be valuable sources of patient education and peer support. Your care team can point you toward trusted resources.