Breathing problems (also known as respiratory or pulmonary problems) are the most serious health issue that can affect people with cystic fibrosis, so keeping the lungs as healthy as possible is vital for a good quality of life. In this section, we focus on the lower respiratory tract – the lungs and airways.
Mucus moistens the air as we breathe in, lubricates the linings of the airways and traps particles and germs that are then moved into the throat so we can cough them out or swallow them.
In someone with CF, the mucus is so thick and sticky it’s difficult for it to move to the throat. Instead, the mucus clogs up the airways, meaning the mucus doesn’t do its job and the lungs are more vulnerable to infection.
‘So, in response, the lungs make more mucus that results in more mucus plugs, worse infections and lung damage.’
Because particles and germs can’t reach the throat to be swallowed or coughed out, lung infection can follow.
Lung infections can trigger a damaging cycle. With infections comes inflammation. With inflammation comes damage to the cilia and airways. With that damage, it becomes even more difficult to clear the lungs of mucus – and, therefore, the particles and germs. The treatment for lung infection is to clear the airways and kill the bacteria.
The mucus clogs up the airways, forming mucus plugs that makes it harder for the lungs to do their job
postural drainage and percussion is the most effective treatment.
Get to know your child’s ‘normal’ symptoms so you’ll recognise a change for the worse. It’s a good idea to report new or worse symptoms to your CF team, because these often mean your child needs treatment.
Your child will have routine lung and airways tests as well as other check if symptoms appear. Give feedback to your CF team so they can build up a well-rounded picture of how your baby is faring.
Imaging Every year or two, doctors will check the effects of CF on your child’s lungs, using either X-rays or CT scans. These scans will also be used if your child is having breathing problems.
Breathing tests routinely measure how well the lungs are working. Known as pulmonary function tests, these are too difficult to administer to infants and toddlers and usually start around 5 years.
Sputum/mucus cultures check for germs that cause lung infections and for the resistance of certain bacteria to antibiotics. With infants, mucus for testing is taken after getting them to ‘gag’ to bring up sputum.
Blood tests help check the overall health of your child by testing for:
Although you might not notice any symptoms in your child, CF lung disease often occurs in the first few months of life and some treatments are started even before symptoms show up. As the parents, you’ll learn to help your child by using a technique known as chest physical therapy (CPT or Chest PT) or postural drainage and percussion (also called PD& P).
For infants with CF, postural drainage and percussion is the most effective treatment. Placing your child in different positions (postures) helps drain mucus from different parts of the lung to large airways where it can be cleared. With chest percussion, you clap and vibrate the chest to dislodge and shift mucus, placing your baby in various positions to drain the entire lung. Your CF care team will show you what to do, and update your child’s routine regularly.
This technique is known as an Airway clearance technique (ACT), one of several chronic treatments given to people with CF to loosen the lung mucus to clear clogged-up airways and treat/prevent infection. This reduces lung infections, improves lung function and helps prevent exacerbations or flare-ups that damage your child’s lungs and airways.
Any exacerbation should be treated promptly so, if you notice any change in your child’s normal symptoms, contact your CF team.
Yes, as your infant gets older they will probably be using one or more of these:
to your CF team so they can build up a well-rounded picture of how your baby is faring.
Good nutrition is good for your baby
Good nutrition and good lung function go hand in hand.Reaching normal weight and height is the goal for a child with CF. To maintain normal growth a child with CF may need more calories, either added to their milk initially or when they start solids. The CF team, and specifically the dietician will help with this. Good nutrition and growth is very important as it will help:
Newborns and toddlers aren’t given many of the medications administered to older children and adults. Ask your CF team about the medicines your child is receiving, and how you should go about filling any prescriptions your doctor gives you.
When they’re older, they might be prescribed some of these: bronchodilators to open the airways; mucolytics for thinning mucus; inhaled or oral antibiotics to fight or control infections and improve respiratory symptoms in people with CF.
Inhaled medicines are normally not used with very young children.
This is a flare-up or increase in your baby’s respiratory symptoms – or the emergence of new symptoms – and means your child needs extra treatment. Even if you’re unsure about whether it is an exacerbation, don’t hesitate to contact your CF team so they can start immediate treatment to reduce lung damage.
If it turns out your baby has a pulmonary exacerbation, treatment involves extra airway clearance and antibiotics. The antibiotics will be given in liquid or tablet form, or intravenously through a tube (IV catheter) straight into the blood.
Oral antibiotics usually work for mild pulmonary exacerbations, however is some situations your baby may need to be admitted to hospital for intravenous antibiotics.
CF doesn’t usually cause major problems in the upper respiratory tract (the nose and sinuses). The basic role of the nose and sinuses is to warm the air we breathe and filter out dirt and germs that might block tiny airways and cause infection. The most common effects of CF are:
Sinusitis Sinuses can become blocked by thick, sticky mucus resulting in sinus infection. Treatment is usually antihistamines and decongestants. Irrigation, flushing and surgery are sometimes used.
Nasal polyps These small, fleshy growths can block the nose. This is treated with surgery or medicine.
Developed by
