What Is Childhood Interstitial Lung Disease?

Childhood interstitial lung disease, known as “chILD,” is a group of rare lung conditions that affect babies, kids, and teens.

All forms of chILD cause damage to a child’s lungs, so they don’t work properly.

Many types of chILD affect the interstitium, a thin tissue between tiny air sacs and blood vessels in the lungs. Some kinds of chILD involve other areas in the lungs, too.

Doctors have just begun to understand this condition over the last few years, and there’s a lot they don’t know. For instance, they’re not sure how many kids have chILD.

Among kids with chILD, some are born with it and others have it later in childhood. Adults can get interstitial lung disease, too, but the causes and outcomes are often different.

Causes

ChILD can crop up without a known cause. On the other hand, certain genes, toxins, or other diseases may be culprits.

Some possible causes are:

Inherited conditions: Disorders that cause problems with surfactant -- a fluid in the lungs that helps your child breathe -- can be passed on through genes.

Immune system disorders : Certain immune system problems make it harder for kids to fight off illnesses.

Autoimmune diseases: These happen when your child’s immune system mistakenly attacks healthy tissues. Inflammatory bowel disease and collagen vascular disease are two autoimmune conditions commonly linked to chILD.

Infection: Some children get chILD after a cold or virus.

Birth defects: Babies can be born with a birth defect that causes problems with their lungs.

Aspiration: When you inhale food, liquid, or vomit into your lungs, damage can happen. Aspiration often affects kids with swallowing problems or a condition called gastroesophageal reflux disease (GERD).

Cancer treatments: Therapies, such as radiation and chemo, may lead to chILD.

Environmental triggers: Chemicals and molds can irritate your child’s lungs.

Surgeries: A lung transplant or bone marrow transplant may be to blame for certain cases of chILD.

Types

There are different kinds of chILD. Many have long, hard-to-pronounce names. Although they’re all considered rare diseases, some forms are more common in specific age groups.

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ChILD diseases that usually affect babies are:

  • Surfactant dysfunction mutations
  • Developmental disorders, such as alveolar capillary dysplasia
  • Lung growth abnormalities
  • Neuroendocrine cell hyperplasia of infancy (NEHI)
  • Pulmonary interstitial glycogenosis (PIG)

Types of chILD that are more common in children and teens are:

Idiopathic interstitial pneumonias: This category includes cryptogenic organizing pneumonia, acute interstitial pneumonia, nonspecific interstitial pneumonia, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia.

Other primary disorders: These disorders may be alveolar hemorrhage syndromes, aspiration syndromes, hypersensitivity pneumonitis, bronchiolitis obliterans, eosinophilic pneumonia, pulmonary alveolar proteinosis, pulmonary infiltrates with eosinophilia, pulmonary lymphatic disorders (lymphangiomatosis, lymphangiectasis), or pulmonary vascular disorders (haemangiomatosis).

ILD-associated with systemic disease processes: Examples are connective tissue diseases, histiocytosis, malignancy-related lung disease, sarcoidosis, and storage diseases.

Disorders of the compromised immune system: This group includes opportunistic infection, disorders related to therapeutic intervention, lung and bone marrow transplant-associated lung diseases, and diffuse alveolar damage of unknown cause.

Symptoms

Signs and symptoms of chILD often depend on the type of disease and how severe it is. They may include:

  • Difficulty breathing or shortness of breath
  • Fast or noisy breathing
  • Wheezing
  • Coughing or chest congestion
  • Repeated bouts of pneumonia or bronchitis
  • Low oxygen levels
  • Failure to gain weight or grow in height

Adults vs. Child Interstitial Disease

Some kids who get chILD will have the condition throughout their lives, so it can technically happen in both children and adults.

But when an adult is diagnosed with an interstitial lung disease, doctors usually consider it a completely different condition than chILD.

Kids with chILD should see a pediatric pulmonologist, rather than a doctor who specializes in adults.

Diagnosis

It’s often hard to diagnose chILD. Each type is different, so the methods your doctor uses will vary.

Tests that help diagnose chILD include:

Chest X-ray or CT scan: These imaging procedures use X-rays to take pictures of your child’s lungs.

Lung function tests: Doctors measure how kids breathe in and out to examine how well their lungs work.

Blood tests: Blood draws are sometimes used to check for abnormal genes.

Bronchoalveolar lavage: With this procedure, a doctor injects salt water through a tube in your child’s lungs to see specific types of cells. It can help spot a lung injury, aspiration, infection, or an airway problem.

Lung biopsy: A surgeon takes out a small piece of lung tissue to test in a lab.

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Treatment

Very little research has been done on how to treat chILD. But some therapies can help children’s lungs work better, ease symptoms, or simply make them feel better.

Your doctor may recommend:

Medicines: Steroids lower lung inflammation, antimicrobial drugs treat infections, and bronchodilators help relax muscles around the airways.

Oxygen: More oxygen can help children breathe better and give their hearts a rest.

Nutrition : An eating plan that focuses on weight gain may benefit some kids with chILD.

Pulmonary rehab and exercises: Special therapies aim to ease congestion and improve how well the lungs work (your doctor may call this “lung function”).

Breathing machines: Devices called ventilators can help children breathe easier.

Lung transplant: This may be an option for kids with serious or life-threatening cases of chILD. So far, chILD doesn’t seem to come back in children who have the surgery.

What’s the Outlook?

With no cure for chILD, the condition comes about and advances differently in each child.

Some cases are severe and tend to be life-threatening at an early age. Other types stay the same or worsen slowly. But certain forms of the disease, such as neuroendocrine cell hyperplasia of infancy, can even improve over time.

Kids with chILD may have special needs. It’s a good idea to talk with teachers, family members, and other parents about ways to support your child and the rest of your family. Take good care of your own health, too. Caregivers often put themselves last, but you need to be well to help your family.

WebMD Medical Reference Reviewed by Dan Brennan, MD on September 12, 2018

Sources

SOURCES:

American Thoracic Society: “What is Interstitial Lung Disease in Children?”

Children’s Interstitial Lung Disease Foundation: “What Is chILD?”

Boston Children’s Hospital: “Interstitial lung disease Symptoms & Causes.”

NIH: National Heart, Lung, and Blood Institute: “Childhood Interstitial Lung Disease.”

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