Children’s interstitial lung disease (chILD)

What is children’s interstitial lung disease (chILD)?

Children’s interstitial lung disease (chILD) is a group of rare lung conditions found in infants, children and teenagers. They are grouped together because they share some common features.

On this page:


chILD or DLD?

When children’s interstitial lung disease was first defined, most doctors thought that chILD conditions are always related to problems with the interstitium. This is a network of tissue that supports the air sacs in your child’s lungs.

With more research, we learned that some chILD disorders involve other parts of the lung, including the:

  • airways
  • airspaces
  • blood cells in the lung (called the vasculature)
  • linings of the lungs (called the pleura)
  • the spaces between the linings, called pleural spaces
The pleura and pleural spaces
The interstitium and blood vessels in the lung

Because chILD conditions are not always related to the interstitium, some experts argue that ‘diffuse lung diseases’ (DLD) is a better term for this group of conditions. To keep things simple and clear, we call them chILD conditions.


How does chILD affect my child’s lungs?

Inflammation, which can result in scar tissue (fibrosis), builds up in the lungs. This makes them lose their elasticity. It makes it harder for lungs to expand to get oxygen into the blood, and for carbon dioxide to be breathed out.

It means that children with chILD will get short of breath, or have to breathe faster.


How many different chILD conditions are there?

There are more than 200 types of chILD or DLD. You can find out about specific conditions on the chILD Lung Foundation website. chILD conditions are long-term, but treatment can sometimes help prevent more damage and infections, and reduce your child’s symptoms.

How common are chILD conditions?

It’s difficult to know exactly how common chILD conditions are.

The definitions of chILD and DLD are still evolving as we learn more about these conditions. Different specialists include different conditions under the chILD umbrella. Because of this, many cases of chILD are either misdiagnosed or not reported.

We do know that chILD conditions are rare. Some studies estimate they affect about one child in a million, others around 150 children in a million.

Can adults also get ILD?

Adults can also get interstitial lung disease (ILD) (including pulmonary fibrosis) but adult ILDs are very different to chILDs.


What causes chILD?

Different types of chILD have different causes. We don’t yet know all the causes, but in over 70% of ILDs in children, an underlying cause can be found.

Some causes of chILD are:

  • gene mutations affecting the production of surfactant
  • a problem with the way your child’s tiny blood vessels, called capillaries, have developed
  • conditions that cause inflammation, such as connective tissue disease and rheumatological conditions
  • immune deficiency. Children whose immune system has been weakened are more likely to get chILD.

Some conditions only affect children under 2 years of age, such neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis (PIG).

Is there any way to prevent it?

 It’s not possible to prevent chILD. But there’s lots you can do to reduce the risks to your child’s lungs. Take a look at our pages on risks to your child’s lungs.


What are the signs and symptoms of chILD?

Symptoms of chILD in young children are not very specific - they could be caused by other things, too. For example, the symptoms of chILD conditions could also be symptoms of conditions like cystic fibrosis or primary ciliary dyskinesia. This can make chILD conditions hard to diagnose.

Different types of chILD can have some of the same symptoms – but they can be more severe in some types than others.

The signs and symptoms to look out for

Doctors will consider doing tests to confirm or exclude chILD if your child has three or more of the following:

  • breathing symptoms, such as
    1. cough
    2. fast breathing
    3. difficulty breathing
    4. becoming unusually breathless after exercise, also called exercise intolerance
  • respiratory signs, such as
    1. fast breathing
    2. abnormal sounds heard in the lungs and airways
    3. sunken ribs when breathing
    4. swelling at the ends of the fingers and nails (clubbing)
    5. not growing or putting on enough weight, also called failure to thrive
    6. respiratory failure. This is when the lungs are not able to do their job. They stop being able to transfer oxygen into the blood, or carbon dioxide from the blood to be breathed out
       
  • a low level of oxygen in the blood
     
  • diffuse abnormalities (appearing throughout both lungs) on a chest X-ray or CT scan.

Sometimes, newborn babies might be diagnosed with a lung condition like bronchopulmonary dysplasia (BPD), but their symptoms may be more severe than a specialist would expect. If this happens, they may have a chILD condition as well.

All babies and infants aged under 2, where a chILD condition is suspected, should be tested for associated conditions, such as cystic fibrosis and primary ciliary dyskinesia (PCD).

The chILD Lung Foundation website has more information on some of the symptoms for specific conditions.

Next: How is chILD diagnosed and treated?

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Last medically reviewed: September 2019. Due for review: September 2022

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.