For Patients and Families

What a day with cystic fibrosis looks like

Most CF care happens at home, every day, done by you or your family rather than by a clinic. That is the honest shape of this condition: a small number of appointments each year, and several hundred days of routine in between. The routine is what does the work.

A typical day has three anchors:

1. Airway clearance, once or twice a day

   Twenty to thirty minutes of deliberate work to move mucus out of the lungs. Everyone with CF is advised to perform airway clearance regularly to maintain lung function and quality of life.2 Morning is common because mucus collects overnight, an evening session is usual, and more are added during a chest infection.1

2. Enzymes with every meal and snack that contains fat

   Taken with the food rather than after it. Most people with CF need enzymes with all meals and snacks so that fat and protein can be absorbed.3 This happens several times a day rather than once.

3. Any medicines you have been prescribed

   Vitamins, salt, inhaled medicines, or tablets, depending on what is available to you and what your team has advised.8

That is the whole framework. Everything else on this page is detail about how to do those three things well, and what to do when something changes.

Airway clearance: the heart of daily care

In a healthy lung, mucus is carried out continuously by the mucociliary escalator. In cystic fibrosis that process stalls, so mucus has to be moved deliberately instead. Airway clearance therapy exists to do the work that the escalator no longer does, and to provoke an effective cough.2 Mucus that sits still becomes a place for bacteria to grow, which is how lung damage begins.10

The most important thing to know is this: the techniques that need no equipment at all are real treatment, not second best. The Cystic Fibrosis Foundation's review of the evidence found that no form of airway clearance has been shown to be superior to any other.2 What determines how well clearance works is technique and consistency, not how much the equipment cost.

Techniques that need nothing but your breath

• Huffing. A forced breath out with an open mouth and throat, as if steaming a mirror, rather than a hard cough. All airway clearance techniques involve coughing or huffing.1 Huff coughing means taking a breath in, holding it briefly, then actively breathing out.1
• The active cycle of breathing technique. A repeating pattern of relaxed breathing, deep breaths that get air behind the mucus, and huffs to move it up. It costs nothing, can be done sitting anywhere, and is taught in CF centers worldwide.1
• Autogenic drainage. Literally self-drainage: using different speeds and depths of breathing to move mucus from the small airways to the large ones in stages.1

These techniques are worth learning properly from a physiotherapist or respiratory therapist, even if that means one long trip to do it. Learned well once, they are yours for life and cost nothing to keep using.

Techniques that need another person

Chest physical therapy uses positions that drain mucus from different parts of the lung, combined with percussion (clapping), vibration, deep breathing, and huffing or coughing.1 A family member can be taught to do it. It is the usual method for babies and young children, who cannot yet perform breathing techniques themselves, and it remains effective at any age.

Ask your care team to teach whoever will be doing it, and to show you the positions that matter most for the areas of your own lungs that need them.

Exercise

Physical activity loosens mucus, strengthens the muscles used for breathing, and makes coughing more productive. It is recommended alongside airway clearance rather than in place of it.2 It is also one of the few parts of CF care that is free everywhere and improves almost everything else at the same time.8

Walking briskly, running, cycling, swimming, dancing, carrying loads, playing sport: anything that leaves you breathing hard counts, and anything you enjoy will last longer than anything you do out of duty.

Eating well and managing digestion

Cystic fibrosis affects the pancreas as directly as it affects the lungs. Thick secretions block the ducts that carry digestive enzymes into the intestine, so food passes through without being broken down properly.910 Most people with CF are affected this way and need enzyme replacement.5

This is why someone with CF can eat well and still lose weight. It is also why nutrition is not a side issue: nutritional status is closely tied to lung function and to how well people recover from illness.8 Food is treatment.

Enzymes

Enzyme capsules replace what the pancreas cannot deliver. They are taken with anything containing fat, including meals, snacks, and milk, and taking them consistently reduces or removes foul-smelling stools, gas, and bloating.3 Dose is worked out from body weight and from how much fat the meal contains, then adjusted based on symptoms, growth, and weight.5

Signs that the dose may need review include greasy or floating stools, stomach pain and bloating, and weight that is not going in the right direction.3

Salt

People with CF lose far more salt in sweat than other people do.10 Salt is inexpensive and widely available, which makes it one of the most accessible parts of CF care. Extra salt is commonly advised in hot climates and around heavy sweating, and it matters most for babies, during fever, and around hard physical work.8 Ask your team what amount is right for your age and climate.

Getting enough energy

People with CF often need considerably more calories than others of the same age and size, because breathing takes more effort and absorption is incomplete.89 Commercial supplement drinks are one way to do this, but they are not the only way, and they are not required.

Energy-dense foods that are affordable in most places include cooking oils and ghee added to prepared food, nuts and nut pastes, eggs, full-fat milk and yoghurt, beans and lentils cooked with oil, avocado where it grows, and bananas and other starchy fruit. Adding fat to food already being cooked is usually cheaper and easier than buying separate products. Poorer nutritional status is one of the ways CF outcomes are known to be worse in lower-resource settings, which is why this is worth deliberate attention.15

Vitamins

Because fat absorption is impaired, so is absorption of the vitamins that dissolve in fat: A, D, E, and K. CF-specific multivitamin preparations exist for exactly this reason, and they work best taken with a meal or snack containing fat and with your enzymes.4 Where they are unavailable or unaffordable, tell your care team rather than simply going without, so they can advise on what alternative is reasonable and what to monitor.

Lifestyle choices that protect your lungs

These are the parts of care that cost little or nothing and make a measurable difference. In settings where medicines are hard to obtain, they carry more weight, not less.

Preventing infection

People with CF are at greater risk of lung infection because thick mucus lets germs thrive, and CF infection-control guidance exists to reduce the spread of those germs both in clinic and in everyday life.6

• Hand hygiene is the foundation of CF infection control, and it is free.6
• Keep distance from other people with CF. Some organisms that colonize CF lungs, notably Pseudomonas aeruginosa and Burkholderia species, pass between people with the condition and receive particular attention.6 This is why CF clinics stagger appointments and why in-person gatherings of people with CF are avoided. Online contact carries no such risk, which is why so much CF community happens that way.
• Vaccination, including annual influenza vaccination where it is offered, and keeping up with the routine national schedule.8 These are free or low-cost in most public health systems.

Air and environment

• Avoid tobacco smoke, both your own and other people's. This is the most damaging avoidable exposure for CF lungs.810
• Reduce smoke from cooking and heating fires where you can, through ventilation, cooking outdoors or near an open window, or a cleaner stove if one is available. Indoor smoke is a serious respiratory exposure and an under-recognized one.
• Limit exposure to standing water, damp, mould, compost, and building dust, which carry organisms that matter far more in CF than in other people.6

Sleep, school, work, and mood

Anxiety and depression are more common in people with CF and in the people caring for them than in the general population, and in lower-resource settings this is compounded by fragmented care and unequal access to treatment.17 This is a recognized part of the condition rather than a personal weakness, and it affects how well treatment gets done. If daily care has started to feel impossible, that is a clinical issue worth raising, not a private one.

Most people with CF go to school, work, travel, and have families. Adjustments that help include somewhere private to do clearance or take enzymes, permission to eat during lessons or shifts, and flexibility around clinic appointments and illness.

Knowing when something is wrong

A flare-up of lung infection is called a pulmonary exacerbation. Treating one early is considerably more effective than treating one late, and lung function lost during a severe exacerbation does not always come back.810

This matters most where getting to care takes time and money. Recognizing a flare-up early can mean a course of tablets at home instead of a long journey and a hospital stay. Learning these signs is one of the most valuable things a family can do.

Contact your care team if you notice:89

• More coughing than usual, or a change in the color, thickness, or amount of sputum
• New or worsening breathlessness, chest tightness, or wheeze
• Unusual tiredness, loss of appetite, or unexplained weight loss
• Fever
• Needing more airway clearance than usual to feel clear
• Coughing up blood

Treatment for an exacerbation is usually a course of antibiotics, chosen where possible from a recent sputum culture, given by mouth or into a vein depending on severity, along with more frequent airway clearance and extra attention to eating.811

Making the most of limited access to care

Specialist CF centers produce better outcomes than general clinics, because they see enough of the condition to recognize its patterns.8 In much of the world there are very few such centers, and reaching one means distance, cost, and time away from work or school. Later diagnosis, worse nutritional status, and worse lung function are all documented consequences of this gap.1315

If getting to a specialist center is possible even occasionally, it is usually worth the effort. A single visit can establish a diagnosis properly, confirm your genotype, teach airway clearance technique correctly, set enzyme dosing, and produce a written plan that a local clinician can then follow. Ask whether the center can advise your local doctor between visits, since many are willing to.

Making a long trip count

• Write your questions down before you go. Appointments move fast and it is easy to leave with the important one unasked.
• Ask to be taught technique, not just told about it. Have someone watch you do airway clearance and correct it. Bring whoever helps you at home.
• Ask for enough prescription to last until you can realistically return, and ask what to do if supply runs out sooner.
• Ask for a written summary of your diagnosis, genotype, current medicines, allergies, and recent culture results, so that any clinician you see can pick things up quickly.
• Ask what counts as urgent for you specifically, and who to contact.

Keep your own records

Where care is spread across several clinicians and no single record follows you, keeping your own is genuinely valuable. A simple notebook or a photo album on a phone holding test results, culture results, medicine names and doses, weights, and dates of illness will make every future consultation faster and better. Bring it to every appointment.

Medicines and modern treatments

This section describes the medicines used in cystic fibrosis, roughly in order of how widely available they are. Access varies enormously between countries and even between clinics in the same country. What follows is what exists, so you know what to ask about, not a list of what you should expect to be given.

The order of inhaled medicines

If you have been prescribed inhaled medicines, the order in which you take them changes how well they work. The usual sequence is: bronchodilator to open the airways, then hypertonic saline or dornase alfa to loosen mucus, then airway clearance, and finally any inhaled antibiotic, so that it reaches cleared airways rather than the mucus you are about to cough up.18 Your team will confirm the right order for your own medicines.

CFTR modulators

Modulators are the first treatments that act on the cause of cystic fibrosis rather than its effects. They work on the faulty CFTR protein itself, and for people who can take them the results can be substantial: better lung function, fewer infections, weight gain.11 They are not a cure, they do not repair existing lung damage, and they are taken for life.

Two things limit who can benefit:

• Genotype. Modulators only work on certain CFTR variants, so knowing your genotype is what determines eligibility.11 Standard genetic panels are built around variants common in European populations, so people elsewhere may need extended sequencing before eligibility can even be assessed.14
• Cost. Most people with CF in low- and middle-income countries are not benefiting from modulator treatment because of the prohibitive cost of these drugs.1316 This is a matter of economics and policy rather than medicine.

If you do not have access to modulators, everything else on this page still works, and it is what kept people with CF alive and well for the decades before modulators existed. Airway clearance, nutrition, infection prevention, and early treatment of flare-ups remain the foundation of care even for people who do take them.2

Questions worth asking at your first specialist appointment

New diagnoses come with more information than anyone can absorb in one visit. Take this list with you.

• Which CFTR variants do we have, and what does that mean for treatment options?
• Are CFTR modulator therapies an option for us, and if not, why not?
• What is our daily routine going to look like — which treatments, in what order, and how long will it take?
• Which airway clearance device is right for us, and can someone watch us use it?
• Is there pancreatic involvement, and do we need enzymes and vitamin supplements?
• How often will we be seen, and who do we call between appointments?
• What should make us call urgently rather than wait?
• What infection-control precautions apply at home, at school, and at work?
• Is there a dietitian, physiotherapist, and social worker on the team, and when do we meet them?
• Should other family members be offered carrier testing?