Children's Hospital Colorado

Interstitial Lung Disease

What is interstitial lung disease?

A woman wearing a white lab coat and stethoscope feels the sides of a young boy's neck while he sits in his mom's lap.Interstitial lung disease (ILD) is a group of rare lung diseases, also called diffuse lung disease. In children, these disorders are called children’s interstitial lung disease (chILD). There are many different types, including:

  • Disorders more prevalent in infancy related to abnormal lung formation or function 
  • Disorders associated with a normal immune system and infections, like chronic aspiration 
  • Disorders associated with a weakened immune system, including opportunistic infections
  • Disorders related to body-wide processes
  • Disorders that resemble interstitial disease

Each disorder works a little differently, but many can scar the lung tissue and all of them can decrease the ability to get enough oxygen to the body. These conditions are different from those known as interstitial lung disease in adults. Early diagnosis and treatment are important for all forms of chILD.

What causes interstitial lung disease?

There are many forms of chILD, and we don’t know what causes many of them. Some, such as surfactant problems, are inherited through genes from a child’s parents. Environmental factors may also play a factor, such as excessive exposure to asbestos, chronic exposure to bird antigen (protein) or fungi found in humidifiers or swamp coolers. One type of chILD, bronchiolitis obliterans, is most commonly caused by viral infections, such as adenovirus, in children with normal immune systems.

Who gets interstitial lung disease?

Just like we don’t know exactly what causes many types of chILD, we aren’t sure how many children have chILD or why some children are affected and others are not. Some children are genetically predisposed to chILD; some appear to be healthy until they have some other type of respiratory infection like a cold or bronchiolitis. Having an immune system disorder or problems with aspiration may also put children at greater risk for chILD

Some children are diagnosed with chILD in infancy while others do not display symptoms until later childhood or adolescence. Males are slightly more likely to be diagnosed with chILD than females.

Helpful resources

What are the signs and symptoms of interstitial lung disease?

Because chILD has so many forms, the signs and symptoms may vary widely. Possible symptoms include:

  • Fast breathing
  • Labored breathing, which also is called respiratory distress
  • Use of "helper" muscles while breathing (muscles in your child’s ribs or neck may stand out while breathing in)
  • Recurrent coughing, wheezing or crackling sounds heard in the chest with a stethoscope
  • Recurrent pneumonia
  • Recurrent bronchiolitis
  • Shortness of breath during exercise (in older children) or while eating (in infants), which also is called dyspnea 
  • Low oxygen levels in the blood, which is called hypoxemia 
  • Poor growth or failure to gain weight (failure to thrive)
  • Abnormal chest X-rays or CT scans

What tests are used to diagnose interstitial lung disease?

Because there are so many types of chILD, many tests may be used to assist with a diagnosis, including chest x-rays, CT scans, swallow studies, blood tests, gene tests, bronchoscopies and pulmonary function tests

In order to definitively diagnose a specific from of chILD, doctors usually need to perform a lung biopsy. A lung biopsy is a surgical procedure performed under anesthesia to minimize discomfort. During the procedure, doctors remove a tiny piece of lung tissue and study it under a microscope. 

As leaders in the chILD Clinical Research Cooperative, our doctors at Children’s Hospital Colorado’s Breathing Institute are actively working to develop other tools to replace the lung biopsy. We are making good progress, but in most cases a lung biopsy may still be needed.

How do providers at Children’s Hospital Colorado make a diagnosis?

To diagnose chILD, doctors will consider the child’s symptoms, physical exam, past medical and family histories and the results from tests. 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 may have the same signs and symptoms. 

Due to the difficulties in diagnosis, we may have to perform lung biopsies because they remain the most effective way to diagnose chILD. Children's Colorado is an experienced world leader for lung biopsies in infants, children and adolescents. Our surgeons use the least invasive technique, called video assisted thoracoscopy (VATS) with excellent outcomes. All patients are admitted to the hospital for the procedure, but many patients can be discharged in less than 48 hours after the operation. Pain is also minimized by avoiding the use of a chest tube after the procedure in most cases.

The chILD Program team interacts with both the surgical and pathology team about the child’s case and the goals for the biopsy. As a leading chILD center, we process the biopsies and have the tissue read by the best ILD pathologists.

How is interstitial lung disease treated?

Most treatment approaches are directed toward improving quality of life and include oxygen therapy and an individual nutrition plan to encourage growth and weight gain. Early diagnosis is helpful to guide the best treatment for all types of chILD. Currently, there is no definitive cure for most types of chILD, though there are many available therapies that may decrease the disease progression or improve symptoms.

Doctors may also recommend devices or techniques to help relieve obstruction or clear mucus congestion in the lungs, like a vest that moves mucus to the upper airways where patients can cough it up.  

Doctors may prescribe medications to treat chILD. Corticosteroids and intravenous immunoglobulin (IVIG) are often used to reduce lung inflammation in certain types of chILD. The use of other medications depends on the type of chILD.

In severe cases that don’t responded to other treatments, a lung transplant may be an option. In some types of chILD with a high risk of death, lung transplants are the only effective treatment.

Why choose Children’s Hospital Colorado for your child’s interstitial lung disease?

The Children's Hospital Colorado Children’s Interstitial Lung Disease (chILD) Program is one of the leading referral centers in the world for children with these rare lung conditions.

Research in our hospital and in national collaboration with other pediatric ILD centers has led to the recognition and understanding of many new ILD disorders in children. One of the most important aspects of chILD is to get the diagnosis and all the tests right. Our center is world renowned for this ability and provides consultation for patients around the world.

Research
Our physicians are leaders in the National chILD Research Collaborative, which consists of pediatric lung, pathology and radiology specialists in North America, who are committed to improving the care for 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 have grants to study and find new treatments and cures for chILD.

We also provide resources for other healthcare professionals who diagnose and treat children’s ILD.

Multidisciplinary team
Our multidisciplinary care team of pediatric lung specialists, pathologists, radiologists, nutritionists, social workers and nurse specialists brings a wide range of experience and expertise. Our goal is to create the most individualized ILD care plan possible for your child and family. Multidisciplinary care is critical to meet the needs of our patients as they manage these complicated conditions.


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