Your baby and CF - CF Buzz

Managing cystic fibrosis in babies

If your baby has just been diagnosed with cystic fibrosis (CF), you’ve had a lot to wrap your head around. Remember that you will have help at every step along the way.

There are a lot of new things for you to learn about managing your child’s CF and you will need to take an active role in most of the treatment. You will also need to pay a lot of attention to your baby’s diet.

There is more information about diet on the Nutrition for babies page.

Below you will find an outline of the types of treatments your baby will need and your CF health team will be able to go into more detail. Remember, they are there to help you.

 

Lungs

It may seem odd, but babies with CF are born with normal lungs, the complications of CF develop over time.

The very thin layer of fluid that lines the bronchi (the airways of the lungs) changes and becomes thick and sticky. When this happens, the small airways become blocked.

It’s important to clear the blockages otherwise the lungs will become infected leading to bronchitis. Recurring bouts of bronchitis leads to a form of lung damage called bronchiectasis.

Bronchiectasis means “the widening of the bronchi”. When the bronchi widen there is a greater risk of mucus building up inside causing infection. You can read more about bronchiectasis on this Lung Foundation of Australia factsheet. 

Chest physiotherapy is used daily to clear the blockages and antibiotics used to treat infections.

 

Chest physiotherapy

For people with CF, chest physiotherapy is a generic term used to describe techniques used to clear the sputum (the thick mucus) from the lungs. Used on a daily basis, it’s an essential part of the management of CF lung disease and is also known as airway clearance.

Your CF health team will teach you how to perform chest physiotherapy on your baby. You will need to perform it once a day for 15-20 minutes when your child is well and at least twice a day when they have a chest infection. If they’re in hospital they may need even more frequent sessions. Your CF team will help you work out the best regime.

As your child grows up they can be taught to perform various chest physiotherapy techniques for themselves and you should also encourage them to be highly physically active, team sports are a great idea.

 

Chest physiotherapy techniques

Percussion

The percussion technique involves firmly patting the chest using the fingers (or a cupped hand in older babies) over a folded towel, other cloth or adequate clothing. Think of it as gently playing a drum.

Percussion helps the mucus move from the small airways to the larger airways, which helps it then be expelled from the body.

 

Vibrations

This technique involves using a gentle but firm “squeeze and shake” movement as your child breaths out. This technique is usually not used until children are at least a year old.

 

Cough

Infants with CF will naturally cough bringing up mucus as they do so. But they will likely swallow the mucus which can cause vomiting. As they grow, encouraging them to spit out the mucus into a tissue instead of swallowing will help.

 

How and when to do chest physiotherapy

When you’re working out a schedule for your child’s airway clearance, make sure it easily fits into your daily routine. Keep in mind that it should be done before feeds, otherwise at least one hour after feeds, and that your child needs to be awake for the procedure.

It’s best to perform the percussion with your child in a number of different positions. Aim for four to six positions and spend about three to five minutes in each position.

These positions are referred to as ‘Modified Postural Drainage’ and allow gravity to help drain mucus from the small airways to the large airways.

The 5 most commonly used positions are:

  • Baby lying on their right side
  • Baby lying on their left side
  • Baby lying on their back
  • Baby lying on their tummy
  • Baby positioned upright resting on your chest/shoulder

 

Trouble-shooting

Babies react differently to physiotherapy. Some will fall asleep, but others will seem uncomfortable and start squirming and/or crying. They may even change from being happy with physio to being difficult, this often happens they grow and become more active and alert.

Once they start to explore by crawling and walking, physiotherapy time can become quite challenging, so spending a few minutes settling them by rocking, singing or cuddling them can help.

It’s natural to stop the physio if the baby starts crying, but instead of stopping try to anticipate when they’re about to cry and continue with the treatment, and try changing their position.

Remember you need to maintain control of the session. Some days will be easy and others difficult. Be patient, creative, flexible and keep your sense of humour and chest physiotherapy will be effective and maybe even enjoyable for your child.

Your CF team will be able to help you with ideas.

 

Benefits of chest physiotherapy

In short, chest physiotherapy for people with CF can be an effective way of keeping infections at bay. You’ll be taught how to do it soon after the initial diagnosis and encouraged to start straight away.

In more detail:

  • evidence suggests that even CF babies without symptoms will have inflammation, infection and structural changes in the airways. So the treatment of early changes may preserve lung function and optimise long-term outcomes
  • airway clearance techniques aim to improve mucociliary clearance (the movement of sputum (mucus) out of the lungs). This means chronic lung disease may be delayed
  • establishing a routine early may help with adherence and teaches effective airway clearance techniques that will help when the child is acutely unwell
  • early, regular treatment can help parents/carers to recognise signs and symptoms of an acute illness, so that necessary treatment can be started quickly

 

Where to next?

As your child gets older, you will both be introduced to more active forms of treatment. These may include activities like bubble blowing, bouncing on a trampoline and tickle games.

By the time your child starts school, ideally they’ll be able to do their chest physio themselves (with your supervision).

As your child gets older regularly ask your clinic physiotherapist if there are any changes in your child’s chest physiotherapy you should anticipate over the next few months.

 

Exercise

Chest physiotherapy forms the basis of clearing the airways of someone with CF, but regular exercise is really important. Exercise should be encouraged as part of the overall physiotherapy management in CF.

Getting the whole family involved along with the child with CF will make the activity normal and hopefully fun. Try ball games, bouncing and jumping (trampolines are great), rolling, crawling and running games.

Playing a wind instrument and/or singing are also great activities to keep your child’s lungs as healthy as possible. These activities will exercise the lungs, but not the rest of the body, so it’s still important to include other physical activities for bone health.

Exercise is important for aerobic fitness and lung health, but it’s also very important for strong bones and good posture.

 

Antibiotics

When your child develops a chest infection, antibiotics are used to clear it up as quickly as possible.

The most common and important symptom of a chest infection in CF is a persistent wet rattly cough. It usually starts with a runny nose and mild fever. If you notice any change in your child’s cough and temperament, it is a good idea to give your CF Nurse Consultant a call.

The CF CNC will let you know what to do next, and should always be the first person you call in these situations. Your CF nurse will be able to either organise to visit and assess your child, arrange an appointment with your child’s CF doctor or fellow, or other member of the CF team, and can also arrange for antibiotics and sputum cultures if necessary.

Usually treatment consists of 7-14 days of an oral antibiotic syrup and increased chest physiotherapy. If the cough gets worse despite treatment, your CF doctor might recommend either a different antibiotic. They might also prescribe a stay in hospital for IV treatment and increased physiotherapy, this is often referred to as a chest “tune up”.



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