The Lungs

The lungs are the most commonly affected organ in patients with CF but the damage to the airways only starts to occur after birth. The damage accumulates over time so infants and children with CF usually have few symptoms. Although CF lung disease is progressive, meaning that it worsens over time, the CF team can work with you to minimize this natural progression and maximize your lung function.

The symptoms of CF are all linked to the basic problem with thick mucus, which traps bacteria and blocks the airways.

A cough develops as the lungs try to find a way to clear mucus. In children, the cough occurs only with chest infections but as disease progresses, cough will occur daily.

The mucus (also called sputum or phlegm) produced may be clear, white, yellow, green or brown. The colour of the mucus depends on the number of bacteria and infection fighting cells it contains – healthy mucus is clear and thin. Increasing amounts of bacteria in the sputum make the sputum thicker and darker in colour. The sensation of shortness of breath can develop if the airways are plugged with mucous.

Most adults with CF cough every day and have sputum production on a regular basis. However, if you notice an increase in your sputum amount, worsening cough, a change in the colour of your sputum, blood in the sputum, more difficulty breathing, worsening fatigue, loss of appetite, fevers, and/or a decrease in your day to day exercise tolerance, you may be experiencing a flare of your CF lung disease. This is called a pulmonary exacerbation. Usually, a pulmonary exacerbation develops over a period of days. If you have these symptoms, you should call the CF office (416-864-5409) to speak to a nurse. If your symptoms are more severe, you should call the hospital switchboard to page the CF doctor or go to the emergency department if it is after regular hours. If you are unsure about the significance of your symptoms, you should call our clinic and speak to one of the nurses or doctors. Pulmonary exacerbations are treated with antibiotics, and sometimes require antibiotics given by an intravenous catheter. CF exacerbations are often associated with a fall in lung function and the sooner you are started on treatment, the better the chance of regaining your lung function.

People with CF can cough up blood. The medical term for this is hemoptysis. Chronic lung infection leads to enlargement of the blood vessels (called bronchial arteries) in the lung which can become weak and burst releasing blood into the airway. Although the leak in the blood vessel will close up in the clotting process, there is no place for the blood in the airway to go except to be coughed up in the sputum. Usually hemoptysis means that you are having an infection. It is important that you report hemoptysis to your CF team in order to decide if you need antibiotic therapy for infection or other treatments. Hemoptysis of large amounts of blood (more than a cup (250 ml) in 24 hours) is not common but can be serious and potentially life threatening. In severe cases of hemoptysis, it is sometimes necessary to carry out a medical procedure to stop the bleeding known as bronchial arterial embolization. Your CF team can give you advice about what you should do if you cough up blood.

Approximately 5-20% of people with CF may develop what is called a pneumothorax or collapsed lung. This occurs when air leaks out of the lungs and collects in the space around the lungs. This collection of air can put pressure on the lung, so it cannot expand as much as it normally does when you take a breath. It can also put pressure on the heart and blood vessels. This situation can be life threatening and needs to be dealt with quickly. This condition is confirmed by a chest x-ray.

Most people with a pneumothorax will experience worsening shortness of breath and chest pain, particularly with deep breathing. Although anyone with CF can develop a pneumothorax, those at highest risk are individuals with low lung function (FEV1 < 30% predicted), or those with a previous history of a pneumothorax. If you experience these symptoms it is important to call your CF team immediately because you may need to see your doctor and get a chest x-ray. While waiting for assessment, you should stop airway clearance, avoid forceful coughing, and stop exercise. If you are extremely short of breath, you should go to the nearest emergency department.

CF appears to be a risk factor for developing blood clots in the deep veins that can obstruct blood flow. In medical terms, we call this “venous thrombosis”. It appears that most of the clots that have occurred in patients with CF are associated with having an intravenous (IV) catheter. An IV is the line inserted in a vein to allow medications to be given. If you have an IV and experience a swollen arm, or pain and tenderness around the IV, this may be a sign that there is a clot. It is important to tell your doctor so they can look at it and decide if an ultrasound needs to be done to look for a clot. We worry about blood clots because sometimes they can move from the deep veins into the blood vessels that connect the heart and the lungs and put pressure on the heart and the lungs. This can be life-threatening.

Aspergillus is a fungus that is commonly found in the environment. It can be inhaled into the lung where it usually does not cause any problems. However, a small proportion (<15%) of people with CF can develop an allergic reaction to the Aspergillus in their lungs. This condition is called allergic bronchopulmonary aspergillosis or ABPA. The symptoms of ABPA are wheezing, chest tightness or shortness of breath, worsening lung function/exercise tolerance, blood in the sputum (hemoptysis), low grade fevers, or coughing up brown coloured mucous plugs. If you grow Aspergillus in your sputum sample this does not mean you have ABPA but your doctor can do other blood tests to check for this. It is important to tell your doctor if you are having any of the symptoms mentioned above to see whether you may have ABPA. ABPA can be treated but the therapy requires corticosteroids, rather than antibiotics and occasionally an anti-fungal medication.

The thick mucus in the airways of people with CF traps bacteria from the environment. Some bacteria can also be spread between people with CF and that is why CF infection control recommendations advise people with CF should stay away from other people with CF. There are some typical bacteria that are seen in the sputum of people with CF.

These include:

  • Staphylococcus aureus (often called “staph” or MSSA)
  • Pseudomonas aeruginosa
  • Methicillin resistant Staphylococcus aureus (called MRSA)
  • Stenotrophomonas maltophilia
  • Achromobacter xylosoxidans
  • Burkholderia cepacia complex (often called “cepacia”)
  • Mycobacterium avium complex (often called “MAC”)
  • Mycobacterium abscessus

As people with CF get older, the bacteria in the lungs may change. Bacteria growing in the sputum may damage the airways. Knowing the type of bacteria allows us to know what specific antibiotics to use, both for chronic therapy (inhaled antbiotics) and for treatment of pulmonary exacerbations. Sometimes, some of the bacteria found in the sputum are not known to cause disease and do not appear to damage the lungs.

It is important to get sputum samples at each clinic visit so that we can monitor which bacteria you are growing as this will impact treatment decisions. If you have difficulty coughing up sputum, our respiratory therapists can help to collect sputum. If you know what bacteria are usually found in your sputum, when you feel sick you can tell any doctor and they will have a good idea what antibiotics should be prescribed.

Burkholderia cepacia complex (BCC) is a family of bacteria that usually live in damp soil. This bacteria is not very common in people with CF (less than 5% of the Canadian CF population grows BCC in their lungs) and people who do not have CF very rarely get infections with BCC. Burkholderia cepacia complex can be passed from one person with CF to another person with CF. Strict infection control practices help to prevent the spread of this bacteria. These measures include handwashing and staff wearing gowns and gloves, separate clinics and lung function testing days for people with and without Burkholderia cepacia complex, separate nursing staff looking after people with or without Burkholderia cepacia complex in the hospital, as well as people with CF wearing masks in the hospital.

There are different strains of BCC and some of them cause more problems than others, with some strains being very resistant to antibiotics and being more likely to cause a decline in lung function. Your CF doctor can give you more information about BCC.

The Sinuses

Similar to the lungs, the sinuses are another area of the airway which is often affected in people with CF. The sinuses are cavities in the bones of your head which produce mucus to moisturize the inside of the nose. In CF, because of the defect in CFTR this mucus is abnormally thick, and thus accumulates in the sinus cavities and becomes infected. This is called chronic sinusitis. Almost all CF patients (90-100%) have signs of sinus swelling and blockage on Xrays or CT scans. Some people with CF develop nasal polyps (which are benign growths in the sinuses which extend into the nose) because of the constant infection and inflammation. Nasal polyps and chronic infection in the sinuses can cause symptoms of nasal blockage/congestion, facial pain, reduced sense of smell and thick nasal discharge. Mucous from the sinuses can sometimes drip back down to the lungs and cause a lung infection. If you have any of these symptoms, there are medical treatments including nasal sprays and rinses which can help. Sinus surgery is an option for some patients to clear out the sinuses and remove any infection/polyps. Ask your CF team about seeing an Otolaryngologist (or Ear, Nose & Throat Surgeon) who specializes in managing CF patients.