Web Content Viewer

Pediatric Interstitial Lung Disease

Share:

Childhood interstitial lung disease (chILD) is a group of rare conditions that affects infants, children and adolescents.  The different types of chILD have one thing in common — they all decrease the oxygen supply to a child’s body. chILD is so unusual that researchers don’t know how many children have each of its many types.

Types of chILD in children

Examples of chILD include:

  • Acute interstitial pneumonia
  • Alveolar capillary dysplasia
  • Aspiration syndrome
  • Bronchiolitis obliterans
  • Chronic bronchiolitis
  • Connective tissue lung disease
  • Cryptogenic organizing pneumonia (COP)
  • Dysplasia
  • Hypersensitivity pneumonitis
  • Lung growth abnormalities
  • Neuroendocrine cell hyperplasia of infancy (NEHI or persistent tachypnea of infancy)
  • Pulmonary interstitial glycogenosis (PIG)
  • Surfactant dysfunction mutations

Symptoms

Because symptoms can be the same as other more common conditions such as asthma or cystic fibrosis, your doctor will rule those out before diagnosing chILD. More common symptoms of chILD include

  • Abnormal shadows on chest X-rays or CT scans
  • Abnormal lung function tests
  • Rapid breathing
  • Trouble breathing
  • Breathing with retractions (using neck muscles or those between the ribs while breathing)
  • Wheezing
  • Shortness of breath when exercising
  • Chronic or intermittent coughing
  • Coughing up blood
  • Recurring bronchiolitis or pneumonia
  • Clubbing — enlargement of the tips of fingers or toes
  • Low oxygen levels
  • Failure to thrive
  • Respiratory failure
Tests & Diagnosis

Childhood interstitial lung disease can be difficult to diagnose. If your child has severe, frequent breathing problems or has had severe lung infections or serious lung problems, consult a pulmonologist. A pediatric pulmonologist (a doctor who specializes in childhood lung disorders) can help rule out other, more common, ailments.

Medical history

Your doctor will begin her diagnosis by asking about your child's medical history. Questions may include

  • Has your child had frequent, severe breathing problems?
  • Has he had any severe lung infections?
  • Did he have any serious lung problems as a newborn or infant?
  • Has he had any contact to lung irritants such as molds, dusts or chemicals?
  • Has he ever had radiation or chemotherapy treatments?
  • Does he have an autoimmune disease, a birth defect or some other medical condition?

The doctor may also ask whether there is a history of severe lung diseases in your family. It is possible for children to inherit certain types of chILD.

Tests and diagnosis

Because no single test can diagnose any one type of chILD, your doctor may perform one or more of the following:

  • Blood tests to look for genetic diseases
  • Bronchoalveolar lavage (a way to remove a tiny sampling of cells from the lower respiratory tract using a bronchoscope to detect aspiration, bleeding, infections, injuries or other airway problems)
  • Chest X-ray
  • CT scan
  • Lung biopsy (a surgical procedure to get a sample of the lung for further examination)
  • Lung function tests
  • Other tests to rule out other conditions including asthma, cystic fibrosis or immune deficiencies
  • Tests to check for systemic diseases (involving the body's organs) related to child
Treatments

Because the conditions that make up chILD are so rare and research has been limited, there are few treatment options available. Often, treatments are what doctors believe has worked in similar cases. Certain types of chILD, such as neuroendocrine cell hyperplasia of infancy, may even improve on their own.

Treatments

Today, treatments for chILD range from supportive therapies to lung transplants. Some medications can help certain patients as well.

Supportive therapy

Supportive therapies are treatments to help relieve symptoms and improve your child’s quality of life. They include

  • Breathing devices including ventilators to help ease breathing or special vests that help move mucus into your child’s upper airways
  • Bronchodilators, medications to relax the muscles around the airways, helping your child breathe easier
  • Nutrition therapies to help your child grow or gain weight
  • Oxygen therapy to help raise the oxygen level in your child’s blood
  • Physical therapy to help loosen mucus so your child can cough it up
  • Pulmonary rehabilitation to help improve the quality of life for children with chronic lung problems

Medications

Corticosteroids can help reduce lung inflammation in children with chILD. Other medications such as antimicrobials can treat lung infections. Acid-blocking medicines can help kids with acid reflux avoid aspiration as well.

Surgery

Children with severe chILD may need a lung transplant if other treatments haven't worked.
Lung transplants are the only effective treatment for certain types of chILD. Those include alveolar capillary dysplasia and some surfactant dysfunction mutations.

Resources

For more information about childhood interstitial lung disease, please visit the following sites:

  • American Thoracic Society:What is Interstitial Lung Disease in Children?This page explains what Interstitial Lung Disease in Children is and provides information about treatment and likeliness for it to be found in your child
  • Children’s Interstitial and Diffuse Lung Disease Foundation:What is chILDThis website explains what chILD is and provides frequently asked questions and answers.
  • ChILD Lung Foundation This website supports families affected by Childhood Interstitial Lung Disease.
FAQs

What is childhood interstitial lung disease (chILD)?

Childhood interstitial lung disease (chILD) is the blanket term for several rare disorders that affect children’s lungs. Currently, there are no known cures for chILD.

What does interstitial mean?

Interstitial comes from the word interstitium, which is tissue that connects air sacs in the lungs to blood vessels. Changes to the interstitium such as scarring or swelling can prevent the lungs from absorbing oxygen.

How common is chILD?

The disorders that make up chILD are extremely rare. They are often mistaken for other conditions such as cystic fibrosis or asthma that share many of the same symptoms.

What are the symptoms of congenital lung disorders?

Symptoms of chILD may include rapid or painful breathing, wheezing or recurrent lung infections.

How are congenital lung disorders diagnosed?

Childhood interstitial lung disease is usually discovered while doctors are looking for another condition. Tests for chILD may include X-rays or CT scans, blood tests or a lung biopsy.

What are the treatments for childhood interstitial lung disease (chILD)?

Depending on the condition, treatment for chILD may include supportive therapies such as oxygen therapy or a lung transplant (in the most serious cases). Medications including corticosteroids work for many children with chILD.

Is there a way to prevent chILD?

There’s no way to prevent chILD but you can take steps to avoid diseases that may make it worse. Frequent hand-washing and keeping your child away from pollutants like cigarette smoke can improve her quality of life.
 

Request Appointment