FAQs - Lung Issues

On average, about 17,280

People with healthy lungs breathe in around 11,000 litres of air every day. That’s enough air to fill 5,500 large bottles of lemonade.

They help us breathe in oxygen and get rid of carbon dioxide and also act as defence against infection and harmful environmental factors such as dust, smoke and pollen.

Our lungs transport oxygen into the blood stream to keep body tissues and cells healthy. Having fit and healthy lungs means that oxygen gets to tissues much faster. Sometimes when people’s lungs are not working properly, they are given oxygen to help them breathe.

Our noses act as the first defence against harmful materials which we inhale, such as smoke, pollution and bacteria, and our lungs offer a second. They have sticky mucus that traps microbes and help their protective white blood cells (phagocytes) to engulf and kill bacteria and destroy harmful materials. The lungs also have tiny, brush-like fibres, known as cilia, to keep our airways clear.

When people with CGD have an infection it can make them more susceptible to problems with their lungs.  This is due to an altered response in their defence cells, called ‘phagocytes’, that makes them more susceptible to infection.

Inflammation is the way that the body deals with problems such as infections. In CGD, that inflammation can sometimes get out of hand and leads to ‘granulomas’ (lumps of white blood cells packed together) in the lungs. Inflammation can damage the lung and may lead to scarring.

When you get lots of infections and inflammation, it can permanently enlarge the airways. This is known as bronchiectasis (pronounced “bron-key-ex-a-sis”). This causes the airways to become dilated, inflamed, and easily collapsible. Also the cilia (brush-like fibres) don’t work as well so you can’t clear away microbes (bacteria, fungi and viruses) as efficiently. This can be diagnosed using a CAT scan (X-rays that build up a layer by layer picture of tissues in your body).

Sometimes scar tissue (also called “pulmonary fibrosis”) can build up in the lungs and this reduces their how effective they are at transporting oxygen to red blood cells.

Having an infection such as pneumonia is a serious because it makes it harder for your lungs to absorb oxygen from the air you breathe.

To prevent them deteriorating, by limiting damage caused by infection and inflammation.

There are seven key things that will help:

1. Prevent infection – keep taking your daily preventative medication: co-trimoxazole and itraconazole

2. Follow the general precautions for people who have CGD.

3. Know the signs of a chest or lung infection (see below)

4. Have a flu jab every year

5. Don’t smoke and avoid breathing in second-hand smoke

6. Take some exercise – but don’t overdo it.

7. Avoid exposure to fungal infection, especially Aspergillus which is on dead leaves, hay, garden waste, wood chips, and in damp, dusty buildings.

Always get some advice if you are worried – ask your doctor if you should see a chest specialist.

Signs are:

• a persistent cough
• breathlessness
• producing sputum or phlegm (mucus that you might cough up from the airways)

Always seek help when you any of the above symptoms. Getting early treatment could reduce damage to your lungs.

Smoking is harmful even if you are healthy but in CGD it’s even more important not to do it. When you smoke, the brush-like fibres in your lungs are inactivated or destroyed. This allows thick mucus to accumulate in the lungs and reduces your defence against infection.

Yes. Exercise and keeping fit allows the body to use oxygen more efficiently and can help your lungs work better. Only exercise until you feel out of breathe and don’t do too much.

Discuss this more with your doctor.

Contact your doctor as soon as possible. Remember to tell them you have CGD.

In theory, it is best to take an antibiotic that your doctors know will work against the specific infection that you have.  In practice, this isn’t so easy as the sputum (mucus) test results take a few days to come back and it is not usually wise to delay starting treatment.  This means that your doctors will usually recommend treatment with an antibiotic that is known to work against a range of lung infections, or be guided by the results of previous sputum tests.  People with established lung disease (bronchiectasis) may sometimes need higher doses of antibiotics, or longer courses, to stop the infection.

If you have CGD, it’s best that your doctor gives a broad range antibiotic. But sometimes they will want to identify the microbe causing your infection, if it doesn’t respond to treatment. This will involve giving a sputum test.

It’s a test used to detect or identify bacteria or fungi that may be causing chest problems and involves giving a sample of or sputum (mucus that you might cough up from the airways). Some people don’t produce sputum. If this is the case, and it is really important to know which infection is in the lung, an examination with a telescope, called a bronchoscopy, might be used to collect samples from deep inside the organ.

The sputum sample is placed in a sterile container and sent to the laboratory to see if it grows a certain type of bacteria or fungi. From these cultures, doctors are able to identify microbes involved and give better-targeted drugs to clear the infection.

Doctors use lung function tests to assess how healthy your lungs are.

Lung function tests, which can also be called pulmonary (pronounced “pull-moan-ary”) function tests, measure how well your lungs work. These tests are used to look for the cause of breathing problems, such as shortness of breath.

They measure:

• How much air you can take into your lungs. This amount is compared to that of other people your age, height, and sex. This allows your doctor to see whether your lungs are working normally.

• How much air you can blow out of your lungs and how fast you can do it.

• How well your lungs deliver oxygen to your blood.

• The strength of your breathing muscles.

Doctors use these tests to diagnose and monitor your lung health and see if there is any damage caused by scarring of the organ’s tissue. These tests may also be used to check how well treatments are working.

The breathing tests most often used are:

• Spirometry (pronounced “spi-ROM-eh-tre”). This test measures how much air you can breathe in and out. It also measures how fast you can blow air out.
• Lung volume measurement. This test, taken along with a spirometry, measures how much air remains in your lungs after you breathe out fully.
• Lung diffusion capacity. This test measures how well oxygen passes from your lungs to your bloodstream.

Some tests measure the oxygen level in your blood and are called blood oxygen tests. There are two tests:

1. Pulse oximetry – measures your blood oxygen level using a special light.
2. Blood oxygen test – when the doctor inserts a needle into an artery, usually in your wrist, and takes a sample of blood. The oxygen level of the blood sample is measured.

Lung function tests are usually painless and rarely cause side effects. The technician will ask you to blow as hard as you can to get the best results and the tests are repeated a few times which can make people feel tired. You may feel some discomfort during an arterial blood gas test – when the needle is inserted into the artery.

These tests may not show what's causing breathing problems. This has to be assessed separately and may involve giving a sample of sputum.

Physiotherapists have a key role in maintaining respiratory health. Perhaps the most important role is teaching people techniques to clear sputum effectively, if this is a problem.  Being very breathless can be frightening and physiotherapists are trained to teach people specific breathing techniques that help with these symptoms.

They also run exercise and education classes called “pulmonary rehabilitation” that can be effective for people with more severely affected lungs.

Antibiotics can be used to treat and prevent infections. A ‘prophylactic’ antibiotic, such as co-trimoxazole, helps prevent infections. Azithromycin is a newer antibiotic that is effective against a lot of respiratory infections, and which may also have anti-inflammatory properties although we do not yet know if this effect is important in CGD.  It doesn’t have many side effects (stomach upset is the most common) and needs to be taken once a day, sometimes less for prevention.

Long term use of any antibiotic has to be balanced against the risks of side-effects (azithromycin can cause electrical disturbance in the heart such as abnormal heart rhythms that may feel like palpitations, and impaired hearing) and of infections becoming resistant to the drug.

Inhalers are a good way to get respiratory drugs to where they are needed most – the lungs.  Most inhalers contain an anti-inflammatory or a drug called a “bronchodilator” that can open up the airways.

If breathing tests show that your airways are narrowed, doctors may recommend that you try an inhaler to see if it improves the test results, reduces your symptoms and increases your exercise capacity.

The main risk of infection comes from dust when old buildings are demolished or a lot of earth is being moved. This is because both dust and earth contain fungal elements.  If possible, avoid these places. If this isn’t possible, minimise your exposure by walking at a distance from where the work is taking place (for example, by crossing the road).

You don’t have to carry or wear a mask.  In theory this would reduce exposure but there is no evidence to prove it. Children often don’t want to be seen as being different so they may feel that wearing a face mask could draw unwanted and unnecessary attention. It might make public something a child wants to keep to themselves.