Children’s interstitial lung disease (chILD)

Children’s interstitial lung diseases (ILDs) are rare lung conditions that affect children of all ages from babies through to teenagers. They affect lungs that are still growing, so they’re often diagnosed at a young age.

There are over 200 types of children’s ILD. They can affect different parts of the lung including the:

• airways (breathing tubes)
• alveoli (tiny air sacs)
• interstitium (the tissue surrounding the air sacs).

Some children’s ILDs affect a few different parts of the lungs. For this reason, some experts like to call them diffuse lung diseases (DLDs). This is because diffuse means ‘all over’ rather than one place.

Most types of children’s ILD cannot be prevented, but there are things you can do to help protect your child's lungs.

Different children's ILDs have different causes. Some examples are:

• genetics - changes to the genes that control how the substances which keep your child's lungs inflated work. These ILDs are called genetic surfactant dysfunction.
• problems with how your child's blood vessels develop before they're born. This includes developmental disorders such as alveolar capillary dysplasia.
• inflammatory and autoimmune conditions such as juvenile rheumatoid arthritis.
• a weak immune system which can increase your child's risk of lung infections
• inhaling food, liquid or saliva into your child's airways and lungs. This is called aspiration.

Common symptoms include:

• fast breathing
• finding it hard to breathe
• noisy chest sounds, like wheezing
• sunken ribs (curving inwards) when breathing
• not growing or putting on weight
• coughing
• chest pain
• breathlessness, especially after exercise.

Your child may not have all these symptoms. Some symptoms like coughing, chest pain and breathlessness after exercise are more likely when your child is older.

Some babies are born with ILD but do not start to have any symptoms until later in childhood.

• In newborn babies, symptoms may appear suddenly and severely. They can develop serious breathing problems (respiratory failure) and need urgent care.
• In older children, symptoms usually appear gradually over time. They can become worse if left untreated.

Your GP will ask about your child’s symptoms, medical history and if there's any family history of lung conditions.  They may listen to your child‘s chest and arrange some tests such as a chest X-ray, blood test and pulse oximetry.

If your GP thinks your child may have an ILD, they will refer them to a specialist team for more detailed tests which may include:

• bronchoscopy to look inside your child's airways
• lung volume test, spirometry or the gas transfer (or TLco test), if your child is aged six or older - these are breathing or lung function tests.
• a 6-minute walking test for some school-aged children to find out how their lungs are working when they exercise
• an infant pulmonary function test (infant PFT) for babies and young children.

It can take time to diagnose children's ILD because the symptoms can look similar to other conditions like asthma, respiratory tract infections, cystic fibrosis, primary ciliary dyskinesia (PCD) and bronchopulmonary dysplasia.

Genetic testing for children's ILD

Genetic testing can give useful information about how your child’s ILD may progress over time and the chances of it being passed on in your family.

This is usually done using a small sample of blood. It’s free on the NHS if you're referred by a hospital specialist. You may also be referred to a genetic counsellor to help you think through what the tests mean for you and your family.

The treatment for children’s ILD depends on the type and the cause of their condition. 

Treatments may include: 

• anti-inflammatory medicines such as steroids
• medicines that reduce inflammation and help manage the immune system, such as hydroxychloroquine. This is a type of antirheumatic medicine.
• antibiotics, such as azithromycin.

If your child gets severe breathlessness, their specialist team may prescribe oxygen therapy, usually given through a mask or nose cannula (light, plastic tube). This will help your child get more oxygen into their body.

Treatment for reflux

Some children with ILD have gastroesophageal reflux disease (GORD). This is when stomach acid travels up towards the mouth. You can lower your child’s risk of reflux by:

• changing your child's food schedule, such as eating smaller amounts more often
• changing how they sit when they're eating so they're more upright
• taking medication, in some cases.

Treatment for aspiration

Some children with ILD have aspiration. This is when something is swallowed into the airways or lungs.

Aspiration can happen if your child is breathing too fast and finding it hard to swallow. They may be referred to a speech and language therapist who can help them to eat and drink safely.

If your child is struggling to swallow, their specialist team may recommend that they’re fed through a tube directly into the stomach. This is called enteral feeding. A dietitian will support you and show you how to use the tube.

Some types of children’s ILD improve or stay the same, but other types can get worse over time. There’s currently no cure for ILDs, but early diagnosis and treatment can help improve symptoms.

Each ILD will be managed differently. Most children will have an appointment every three to four months. But how often depends on the type of ILD your child has and how it is being treated.

Keeping a diary of your child’s symptoms to take to their appointments can help their specialist team:

• recognise when symptoms are getting worse
• see how well treatments are working and if they need to be changed
• answer any questions you may have.

At your child's review, you can talk about your child's symptoms and any changes you’ve noticed.

Their specialist team may do some lung function tests to see how well your child’s treatment is working. They can also tell you what you need to do if their condition gets worse, and when you should seek emergency care.

Things you can do to protect your child's lungs

Your child’s specialist team can support you to make lifestyle changes to help improve their symptoms. 

• Make sure your child is eating a healthy well-balanced diet. Many children with ILD use more energy to breathe and may need more nutritional support to help them grow and stay healthy. They will need plenty of protein and vitamins to stay healthy.
• Make sure your child has all the vaccines they're eligible for.
• Encourage your child to get active to help their physical and mental health.
• Do not smoke or let anyone smoke or vape around your child. There's lots of support to help you stop smoking.
• Try to avoid exposing your child to outdoor air pollution and indoor air pollution where possible.
• Take steps to reduce your child's risk of infections where possible. Clean the most used surfaces in your home and encourage your child to wash their hands with warm water and soap regularly.

Moving from child to adult ILD services

In childhood services, you will help to manage your child’s care. As your child gets older, their specialist team will involve them more in any decisions about their care. By the time they move to adult services, your child will be encouraged to take a more active role in decisions about their care.

The move from child to adult services usually happens between the age of 16 to 18 years old, but this can vary. You and your child will continue to have support from their specialist team during this time.

Encouraging your child to manage their ILD more independently as they get older can help them feel confident as they move into adult care services.

It can be hard caring for a child with a long-term condition and it’s important to take care of your own mental and emotional wellbeing. Talking to someone you trust about how you’re feeling can help. 

You could join one of our online communities on Health Unlocked forum to connect with other people with similar experiences to you and your child. We also have information to support you to look after your mental health and care for someone with a lung condition.

If you’d like to speak to one of our friendly respiratory nurses, get in touch with our helpline. We’ll give you as much time as you need to talk about any aspects of your child’s lung condition and what’s important to you.

The charity Childhood Interstitial Lung Disease UK is also there to support you. They have more information and support groups to connect families affected by the condition.