Children's Hospital Colorado

Children’s Interstitial and Diffuse Lung Disease

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

Children's interstitial and diffuse lung disease (chILD) is a group of rare pediatric lung diseases. There are many different types, including problems in the airways, problems in the air sacs (alveoli) or problems in the interstitium (space between the air sacs).

There are many types of chILD. There are primary disorders, which are more common in infancy. There are also secondary disorders, which are caused by other medical conditions.

  • Primary:
    • Developmental disorders
    • Growth abnormality disorders
    • Conditions with unknown causes
    • Surfactant dysfunction mutations
  • Secondary:
    • Disorders related to system (body-wide) diseases, like sarcoidosis
    • Disorders caused by infections such as bronchiolitis obliterans or eosinophilic pneumonia
    • Disorders caused by a weakened immune system, which results from conditions such as transplantation and rejection
    • Disorders that resemble interstitial disease such as pulmonary hypertension
    • Unknown classification; lung biopsy tissue cannot be classified

ChILD overlaps with some adult forms of ILD, but many forms of chILD are unique to children, such as neuroendocrine cell hyperplasia of infancy or pulmonary interstitial glycogenosis.

What causes pediatric interstitial lung disease?

Every type of chILD has a unique cause or causes, all of which result in lung disease. Some types, such as surfactant dysfunction mutations, are inherited through genes from a child's parents. Environmental factors, such as chronic exposure to fungi found in humidifiers, swamp coolers or birds, may also play a role. Bronchiolitis obliterans can be caused by infections (like adenovirus) or happen after transplantation (lung or hematopoietic stem cell).

What are the signs and symptoms of interstitial lung disease?

Because there are so many types of chILD, the signs and symptoms vary widely. Possible signs and symptoms include:

  • Fast breathing
  • Working hard to breathe, which also is called respiratory distress
  • Coughing, wheezing or crackling sounds in the chest heard with a stethoscope
  • Recurring pneumonias
  • Recurring bronchiolitis
  • Shortness of breath during exercise (in older children) or while eating (in infants)
  • Changes in color: pale skin, duskiness or blueness in and around the lips, gums, tongue and nailbeds
  • Poor growth or failure to gain weight (failure to thrive)
  • Clubbing of the fingernails (fingertips widen and become rounder than normal)

What tests are used to diagnose interstitial lung disease?

Because there are so many types of chILD, many tests may be used to make a diagnosis, including chest X-rays, CT scans, genetic tests, bronchoscopies, lung biopsies and pulmonary function tests.

Due to the difficulties in diagnosis, we may have to perform a lung biopsy because they remain the most effective way to diagnose chILD. A lung biopsy is a procedure that our surgeons perform while your child is under general anesthesia. General anesthesia helps your child fall into a deep, sleep-like state that protects them from pain during surgery. Doctors remove a tiny piece of lung tissue and look at it under a microscope. All patients are admitted to the hospital for the procedure, but many patients can be discharged in less than 48 hours after the procedure. We try to minimize pain by avoiding the use of a chest tube after the procedure in most cases.

Children's Hospital Colorado is an experienced world leader for performing lung biopsies in infants, children and adolescents. Our surgeons use the least invasive technique, called video assisted thoracoscopy (VATS) with excellent outcomes.

How do we diagnose interstitial lung disease?

We may diagnose some children with chILD in infancy but others do not display symptoms until later childhood or adolescence. To diagnose chILD, our doctors will consider your child's symptoms, physical exam, past medical and family histories and test results. Doctors will also rule out other potential causes, such as cystic fibrosis or asthma. It can often be difficult to diagnose chILD because there are so many forms of the disease and many other conditions have the same signs and symptoms.

As leaders in the chILD Research Network, our doctors at Children's Colorado's Breathing Institute are actively working to learn more about what causes chILD, how to diagnosis it and how best to treat it.

How is children's interstitial and diffuse lung disease treated?

Early diagnosis is helpful to guide the best treatment for all types of chILD. Your child may need:

  • Respiratory support: Oxygen therapy or treatment with non-invasive or invasive ventilation may be needed.
  • Nutrition: We will develop an individual nutrition plan for your child to promote growth and weight gain.
  • Vaccines: Children with chILD should receive all vaccines as scheduled including some extra vaccines (the RSV vaccine in the first 1 to 2 years of life and the pneumonia vaccine after the age of 3).
  • Clearance of mucus: Doctors may recommend devices or techniques to help clear mucus from the lungs, like a vest that moves mucus to the upper airways so your child can cough it up.
  • Medications: There are many different medications used to treat chILD. The medications that your child may need depends on the type of chILD they have.
  • Lung transplant: In severe cases that don't respond to other treatments, a lung transplant may be an option. In some types of chILD that carry a high risk of death, lung transplants are the only effective treatment.

Why choose us for treatment of interstitial lung disease?

Our Children's Interstitial Lung Disease (chILD) Program is one of the leading centers in the world for children with these rare lung conditions. Providers refer patients to us all from all around the world.

We have conducted research in our hospital and in national collaboration with other pediatric ILD centers that has led to the recognition and understanding of many new ILD disorders in children. It is very important to perform the right tests to accurately diagnose ILD. Our center is world renowned for accurately diagnosing ILD, and we provide consultation for patients around the world.

Research

Our physicians are leaders in the chILD Research Network, which includes pediatric pulmonary, pathology and radiology specialists in North America who are committed to improving the care of children with these conditions.

The Children's Colorado chILD Program has advanced protocols for children related to CT scans, infant pulmonary function testing, bronchoscopy and lung biopsies. Our physicians also receive grants to study and find new treatments and cures for chILD. We also proudly provide resources for other healthcare professionals who diagnose and treat children's ILD in countries around the world.

Multidisciplinary team

Our multidisciplinary care team of pediatric lung specialists, pathologists, radiologists, dietitians, social workers and nurse specialists has extensive expertise in treating chILD. Our goal is to create the most individualized care plan possible for your child and family. The type of multidisciplinary care we provide is critical to meet the needs of our patients as they manage these complicated conditions.

  • The National Heart, Lung and Blood Institute promotes the prevention and treatment of lung disorders, like ILD, and heart and blood disorders. They provide helpful information about chILD as well as the latest research being done across the United States.
  • The American Thoracic Society helps to improve health through the advancement of research, clinical care information and public health for lung diseases, like ILD.
  • The chILD Foundation is a non-profit organization that Robin Deterding, MD, and her colleagues founded. This organization provides information, resources and support for families impacted by interstitial lung disease and funds research on treatments and cures for rare lung diseases.
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