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What Is Childhood Interstitial Lung Disease?

Childhood interstitial (in-ter-STISH-al) lung disease, or chILD, is a broad term for a group of rare lung diseases that can affect babies, children, and teens. These diseases have some similar symptoms, such as chronic cough, rapid breathing, and shortness of breath.

These diseases also harm the lungs in similar ways. For example, they damage the tissues that surround the lungs' alveoli (al-VEE-uhl-eye; air sacs) and bronchial tubes (airways). Sometimes these diseases directly damage the air sacs and airways.

The various types of chILD can decrease lung function, reduce blood oxygen levels, and disturb the breathing process.

Overview

Researchers have only begun to study, define, and understand chILD in the last decade. Currently, they don't know how many children have chILD. They also don't know how many children have each type of chILD.

Diagnosing chILD and its specific diseases is hard because chILD is rare and complex. Also, chILD is a broad term for a group of diseases with similar symptoms—it's not a precise diagnosis.

Interstitial lung disease (ILD) also occurs in adults. However, the cause of ILD in adults may be different than the cause in children. Some types of chILD are similar to the adult forms of the disease. They may even have the same names as the adult forms, such as hypersensitivity pneumonitis (noo-mo-NI-tis), immunodeficiency-associated lung disease, and bronchiolitis (brong-ke-o-LI-tis) obliterans.

However, research shows that the course and outcomes of these diseases often are very different for children than for adults.

Some ILDs only occur in children. They include:

  • Lung growth abnormalities
  • Neuroendocrine (noor-o-EN-do-krin) cell hyperplasia (hi-per-PLA-ze-ah) of infancy (NEHI)
  • Pulmonary interstitial glycogenosis (gli-ko-JEN-eh-sis)
  • Developmental disorders, such as alveolar (al-VE-o-lar) capillary dysplasia

Outlook

Each form of chILD may differ in its severity and how it's treated. Thus, getting a correct diagnosis is vital for understanding and treating your child's illness.

You may want to consult a pediatric pulmonologist. This is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions. This doctor's training and experience can help him or her diagnose chILD.

The outlook for children who have chILD also depends on the specific type of disease they have. Some diseases are very severe and lead to early death. Others are chronic (long-term) diseases that parents and the child's medical team must work together to manage.

At this time, chILD has no cure. However, some children who have certain diseases, such as NEHI, may slowly improve over time.

Researchers are now starting to learn more about the causes of chILD. They're also trying to find distinct patterns and traits for the various forms of chILD. This information may help doctors better understand these diseases.




Types of Childhood Interstitial Lung Disease

The broad term "childhood interstitial lung disease" (chILD) refers to a group of rare lung diseases that can affect babies, children, and teens. Some of these diseases are more common in certain age groups.

Diseases more common in infancy include:

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

Diseases more common in children older than 2 years of age and teens include:

  • Idiopathic interstitial pneumonias:
    • Nonspecific interstitial pneumonia
    • Cryptogenic organizing pneumonia
    • Acute interstitial pneumonia
    • Desquamative interstitial pneumonia
    • Lymphocytic interstitial pneumonia
  • Other primary disorders:
    • Alveolar hemorrhage syndromes
    • Aspiration syndromes
    • Hypersensitivity pneumonitis
    • Infectious or postinfectious disease (bronchiolitisexternal link icon obliterans)
    • Eosinophilic pneumonia
    • Pulmonary alveolar proteinosis
    • Pulmonary infiltrates with eosinophilia
    • Pulmonary lymphatic disorders (lymphangiomatosis, lymphangiectasis)
    • Pulmonary vascular disorders (haemangiomatosis)
  • ILD associated with systemic disease processes:
    • Connective tissue diseases
    • Histiocytosis
    • Malignancy-related lung disease
    • Sarcoidosis
    • Storage diseases
  • Disorders of the compromised immune system:
    • Opportunistic infection
    • Disorders related to therapeutic intervention
    • Lung and bone marrow transplant-associated lung diseases
    • Diffuse alveolar damage of unknown cause

The various types of chILD can affect many parts of the lungs, including the alveoli (air sacs), bronchial tubes (airways), and capillaries. (Capillaries are the tiny blood vessels that surround the air sacs.) The structures of the lung that chILD may affect are shown in the illustration below.

Normal Lungs and Lung Structures

Figure A shows the location of the lungs and airways in the body. Figure B is a detailed view of the lung structures that childhood interstitial lung disease may affect, such as the bronchioles, neuroendocrine cells, alveoli, capillary network, surfactant, and interstitial space.

Figure A shows the location of the lungs and airways in the body. Figure B is a detailed view of the lung structures that childhood interstitial lung disease may affect, such as the bronchioles, neuroendocrine cells, alveoli, capillary network, surfactant, and interstitial space.




Other Names for Childhood Interstitial Lung Disease

  • Chronic lung disease
  • Diffuse infiltrative lung disease
  • Diffuse lung disease
  • Diffuse parenchyma lung disease
  • Interstitial pneumonitis
  • The chILD syndrome



What Causes Childhood Interstitial Lung Disease?

Researchers don't yet know all of the causes of childhood interstitial lung disease (chILD). Many times, these diseases have no clear cause.

Some conditions and factors that may cause or lead to chILD include:

  • Inherited conditions, such as surfactant disorders. Surfactant is a liquid that coats the inside of the lungs. It helps with breathing and may help protect the lungs from bacterial and viral infections.
  • Birth defects that cause problems with the structure or function of the lungs.
  • Aspiration (as-pih-RA-shun). This term refers to inhaling substances—such as food, liquid, or vomit—into the lungs. Inhaling these substances can injure the lungs. Aspiration may occur in children who have swallowing problems or gastroesophageal (GAS-tro-eh-so-fa-JE-al) reflux disease (GERD). GERD occurs if acid from the stomach backs up into the throat.
  • Immune system disorders. The immune system protects the body against bacteria, viruses, and toxins. Children who have immune system disorders aren't able to fight illness and disease as well as children who have healthy immune systems.
  • Exposure to substances in the environment that can irritate the lungs, such as molds and chemicals.
  • Some cancer treatments, such as radiation and chemotherapy.
  • Systemic or autoimmune diseases, such as collagen vascular disease or inflammatory bowel disease. Systemic diseases are diseases that involve many of the body's organs. Autoimmune diseases occur if the body's immune system mistakenly attacks the body's tissues and cells.
  • A bone marrow transplant or a lung transplant.



Who Is at Risk for Childhood Interstitial Lung Disease?

Childhood interstitial lung disease (chILD) is rare. Most children are not at risk for chILD. However, some factors increase the risk of developing chILD. These risk factors include:

  • Having a family history of interstitial lung disease or chILD.
  • Having an inherited surfactant disorder or a family history of this type of disorder. Surfactant is a liquid that coats the inside of the lungs. It helps with breathing and may help protect the lungs from bacterial and viral infections.
  • Having problems with aspiration. This term "aspiration" refers to inhaling substances—such as food, liquid, or vomit—into the lungs.
  • Having an immune system disorder. The immune system protects the body against bacteria, viruses, and toxins. Children who have immune system disorders aren't able to fight illness and disease as well as children who have healthy immune systems.
  • Being exposed to substances in the environment that can irritate the lungs, such as molds and chemicals.
  • Having a systemic or autoimmune disease, such as collagen vascular disease or inflammatory bowel disease. Systemic diseases are diseases that involve many of the body's organs. Autoimmune diseases occur if the body's immune system mistakenly attacks the body's tissues and cells.
  • Undergoing some cancer treatments, such as radiation and chemotherapy.
  • Having a bone marrow transplant or a lung transplant.

Certain types of chILD are more common in infants and young children, while others can occur in children of any age. For more information, go to "Types of Childhood Interstitial Lung Disease." 

The risk of death seems to be higher for children who have chILD and pulmonary hypertension, developmental or growth disorders, bone marrow transplants, or certain surfactant problems.




What Are the Signs and Symptoms of Childhood Interstitial Lung Disease?

Childhood interstitial lung disease (chILD) has many signs and symptoms because the disease has many forms. Signs and symptoms may include:

  • Fast breathing, which also is called tachypnea (tak-ip-NE-ah)
  • Labored breathing, which also is called respiratory distress
  • Low oxygen levels in the blood, which also is called hypoxemia (hi-POK-se-ah)
  • Recurrent coughing, wheezing, or crackling sounds in the chest
  • Shortness of breath during exercise (in older children) or while eating (in infants), which also is called dyspnea (disp-NE-ah)
  • Poor growth or failure to gain weight
  • Recurrent pneumonia or bronchiolitis

If your child has any of these signs and symptoms, contact his or her doctor. The doctor may refer you to a pediatric pulmonologist. This is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions.




How Is Childhood Interstitial Lung Disease Diagnosed?

Doctors diagnose childhood interstitial lung disease (chILD) based on a child's medical and family histories and the results from tests and procedures. To diagnose chILD, doctors may first need to rule out other diseases as the cause of a child's symptoms.

Early diagnosis of chILD may help doctors stop or even reverse lung function problems. Often though, doctors find chILD hard to diagnose because:

  • There are many types of the disease and a range of underlying causes
  • The disease's signs and symptoms are the same as those for many other diseases
  • The disease may coexist with other diseases

Going to a pediatric pulmonologist who has experience with chILD is helpful. A pediatric pulmonologist is a doctor who specializes in diagnosing and treating children who have lung diseases and conditions.

Medical and Family Histories

Your child's medical history can help his or her doctor diagnose chILD. The doctor may ask whether your child:

  • Has severe breathing problems that occur often.
  • Has had severe lung infections.
  • Had serious lung problems as a newborn.
  • Has been exposed to possible lung irritants in the environment, such as birds, molds, dusts, or chemicals.
  • Has ever had radiation or chemotherapy treatment.
  • Has an autoimmune disease, certain birth defects, or other medical conditions. (Autoimmune diseases occur if the body's immune system mistakenly attacks the body’s tissues and cells.)

The doctor also may ask how old your child was when symptoms began, and whether other family members have or have had severe lung diseases. If they have, your child may have an inherited form of chILD.

Diagnostic Tests and Procedures

No single test can diagnose the many types of chILD. Thus, your child's doctor may recommend one or more of the following tests. For some of these tests, infants and young children may be given medicine to help them relax or sleep.

  • A chest x ray. This painless test creates pictures of the structures inside your child's chest, such as the heart, lungs, and blood vessels. A chest x ray can help rule out other lung diseases as the cause of your child's symptoms.
  • A high-resolution CT scan (HRCT). An HRCT scan uses x rays to create detailed pictures of your child's lungs. This test can show the location, extent, and severity of lung disease.
  • Lung function tests. These tests measure how much air your child can breathe in and out, how fast he or she can breathe air out, and how well your child's lungs deliver oxygen to the blood. Lung function tests can assess the severity of lung disease. Infants and young children may need to have these tests at a center that has special equipment for children.
  • Bronchoalveolar lavage (BRONG-ko-al-VE-o-lar lah-VAHZH). For this procedure, the doctor injects a small amount of saline (salt water) through a tube inserted in the child's lungs. The fluid helps bring up cells from the tissues around the air sacs. The doctor can then look at these cells under a microscope. This procedure can help detect an infection, lung injury, bleeding, aspiration, or an airway problem.
  • Various tests to rule out conditions such as asthma, cystic fibrosis, acid reflux, heart disease, neuromuscular disease, and immune deficiency.
  • Various tests for systemic diseases linked to chILD. Systemic diseases are diseases that involve many of the body's organs.
  • Blood tests to check for inherited (genetic) diseases and disorders.

If these tests don't provide enough information, your child's doctor may recommend a lung biopsy. A lung biopsy is the most reliable way to diagnose chILD and the specific disease involved.

A lung biopsy is a surgical procedure that's done in a hospital. Before the biopsy, your child will receive medicine to make him or her sleep.

During the biopsy, the doctor will take small samples of lung tissue from several places in your child's lungs. This often is done using video-assisted thoracoscopy (thor-ah-KOS-ko-pe).

For this procedure, the doctor inserts a small tube with a light and camera (endoscope) into your child's chest through small cuts between the ribs. The endoscope provides a video image of the lungs and allows the doctor to collect tissue samples.

After the biopsy, the doctor will look at these samples under a microscope.




How Is Childhood Interstitial Lung Disease Treated?

Childhood interstitial lung disease (chILD) is rare, and little research has been done on how to treat it. At this time, chILD has no cure. However, some children who have certain diseases, such as neuroendocrine cell hyperplasia of infancy, may slowly improve over time.

Current treatment approaches include supportive therapy, medicines, and, in the most serious cases, lung transplants.

Supportive Therapy

Supportive therapy refers to treatments that help relieve symptoms or improve quality of life. Supportive approaches used to relieve common chILD symptoms include:

  • Oxygen therapy. If your child's blood oxygen level is low, he or she may need oxygen therapy. This treatment can improve breathing, support growth, and reduce strain on the heart.
  • Bronchodilators. These medications relax the muscles around your child’s airways, which helps open the airways and makes breathing easier.
  • Breathing devices. Children who have severe disease may need ventilators or other devices to help them breathe easier.
  • Extra nutrition. This treatment can help improve your child's growth and help him or her gain weight. Close monitoring of growth is especially important.
  • Techniques and devices to help relieve lung congestion. These may include chest physical therapy (CPT) or wearing a vest that helps move mucus (a sticky substance) to the upper airways so it can be coughed up. CPT may involve pounding the chest and back over and over with your hands or a device to loosen mucus in the lungs so that your child can cough it up.
  • Supervised pulmonary rehabilitation (PR). PR is a broad program that helps improve the well-being of people who have chronic (ongoing) breathing problems.

Medicines

Corticosteroids are a common treatment for many children who have chILD. These medicines help reduce lung inflammation.

Other medicines can help treat specific types or causes of chILD. For example, antimicrobial medicines can treat a lung infection. Acid-blocking medicines can prevent acid reflux, which can lead to aspiration.

Lung Transplant

A lung transplant may be an option for children who have severe chILD if other treatments haven't worked.

Currently, lung transplants are the only effective treatment for some types of chILD that have a high risk of death, such as alveolar capillary dysplasia and certain surfactant dysfunction mutations.

Early diagnosis of these diseases gives children the chance to receive lung transplants. So far, chILD doesn't appear to come back in patients' transplanted lungs.

For more information about this treatment, go to the Health Topics Lung Transplant article.




How Can Childhood Interstitial Lung Disease Be Prevented?

At this time, most types of childhood interstitial lung disease (chILD) can't be prevented. People who have a family history of inherited (genetic) interstitial lung disease may want to consider genetic counseling. A counselor can explain the risk of children inheriting chILD.

You and your child can take steps to help prevent infections and other illnesses that worsen chILD and its symptoms. For example:

  • Make hand washing a family habit to avoid germs and prevent illnesses.
  • Try to keep your child away from people who are sick. Even a common cold can cause problems for someone who has chILD.
  • Talk with your child's doctor about vaccines that your child needs, such as an annual flu shot. Make sure everyone in your household gets all of the vaccines that their doctors recommend.
  • Talk with your child's doctor about how to prevent your child from getting respiratory syncytial (sin-SIT-e-al) virus. This common virus leads to cold and flu symptoms for most people. However, it can make children who have lung diseases very sick.
  • Avoid exposing your child to air pollution, tobacco smoke, and other substances that can irritate his or her lungs. Strongly advise your child not to smoke now or in the future.



Living With Childhood Interstitial Lung Disease

Caring for a child who has childhood interstitial lung disease (chILD) can be challenging. However, you can take steps to help your child manage his or her disease.

Make sure your child gets ongoing care and seek support to help you, your child, and your other family members cope with the effects of chILD on daily life.

Ongoing Care

Work with your child's health care team to manage your child's symptoms and keep him or her as healthy as possible.

This team may include doctors, nurses, dietitians, social workers, physical therapists, and home health aides. Each of these specialists may have services that can help you and your child cope with his or her lung disease.

You also can take other steps to help manage your child's care. For example:

  • Give your child all of his or her prescribed medicines. Make sure to take your child to all followup medical visits.
  • Work with your child's health care team to ensure that your child is getting good nutrition. Your child's health care team also can suggest physical activities that meet your child's needs.
  • Ask your child's doctor about warning signs of worsening lung disease and when to seek emergency medical care. Agree on a plan of action if these warning signs occur.
  • Keep complete records of your child's care and any instructions you receive. This information can help you manage care at home and inform various doctors about your child's medical history and status.

Many children who have chILD need oxygen therapy to help them breathe easier. Portable oxygen units can make it easier for your child to move around and do many daily activities.

If your child's doctor prescribes oxygen therapy, work with a home equipment provider to make sure you have the supplies and equipment you need. Trained personnel will show you how to use the equipment correctly and safely.

Ongoing Support

Your child may need support to help other people in his or her life understand the special needs related to chILD. For example, you may want to talk with your child's teachers about your child's illness. You can work with the teachers to decide how to meet your child's special school-related needs.

You also may want to alert relatives, caregivers, friends, and parents of friends about your child's illness. Let them know about your child's usual care and any signs or symptoms that require emergency care.

Taking care of yourself also is important. Managing your child's disease and ongoing care can be stressful. You and your family members may feel sad, guilty, or overwhelmed.

Social workers and mental health providers can help you cope with your feelings and provide support. They also can connect you with family support groups. Taking part in a support group can show you how other people have coped with chILD.




Clinical Trials

The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.

NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.

Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.

By taking part in a clinical trial, your child can gain access to new treatments before they're widely available. Your child also will have the support of a team of health care providers, who will likely monitor his or her health closely. Even if your child doesn't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.

Children (aged 18 and younger) get special protection as research subjects. Almost always, parents must give legal consent for their child to take part in a clinical trial.

When researchers think that a trial's potential risks are greater than minimal, both parents must give permission for their child to enroll. Also, children aged 7 and older often must agree (assent) to take part in clinical trials.

If you agree to have your child take part in a clinical trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw your child from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.

For more information about clinical trials related to childhood interstitial lung disease, talk with your doctor. For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.

You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:




Links to Other Information About Childhood Interstitial Lung Disease

NHLBI Resources

Non-NHLBI Resources

Clinical Trials

 
March 21, 2014 Last Updated Icon

The NHLBI updates Health Topics articles on a biennial cycle based on a thorough review of research findings and new literature. The articles also are updated as needed if important new research is published. The date on each Health Topics article reflects when the content was originally posted or last revised.