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Interstitial lung disease

  • Overview

    Alicia Casey, MD, co-director, Interstitial Lung Disease Program, Boston Children's Hospital

    Boston Children’s Hospital is a world leader in pediatric respiratory diseases, including interstitial lung disease (ILD or chILD).We specialize in innovative, family-centered care that supports you at every step on your child’s journey to health. 

    Interstitial lung disease is not a single disease, but is instead a group of rare lung diseases that range from mild to severe and have different treatments and different outcomes. These diseases make it difficult for the lungs to exchange oxygen and carbon dioxide and can cause fluid and other materials to collect in the lungs. (See our "In-Depth" page for a list of different ILDs).

    How Boston Children’s Hospital approaches interstitial lung disease

    Our Interstitial Lung Disease Program is made up of doctors, nurses, nutritionists and social workers who provide a full diagnosis and evaluation of each child with ILD.

    We work with families to plan individualized therapy depending on the specific condition—to offer the best care of the child’s respiratory health, growth and nutrition. We help families arrange home health care support and supplies, transportation services and visiting nursing services. If needed, our Division of Pulmonary and Respiratory Diseases provides expert review and opinion, as well as other services and support, for lung transplantation.

    Our ILD program is a part of the Children's Interstitial Lung Disease Research Network (ChILDRN), associated with the Children's Interstitial Lung Disease (ChILD) Foundation.

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    Boston Children's Hospital 
    300 Longwood Avenue
    Farley 4th Floor
    Boston MA 02115
     617-355-1900
     fax: 617-730-0373

  • In-Depth

    What you need to know about interstitial lung disease

    Interstitial lung disease (ILD, chILD) isn’t actually a single disease—rather, it’s a group of rare lung diseases in which the cells and tissues of the lungs are damaged and don’t work the way they should. There are various levels of severity, treatments and outcomes.

    The ILD category of diseases includes:

    • Acute interstitial pneumonia/pneumonitis
    • Alveolar capillary dysplasia
    • Alveolar hemorrhage syndromes (pulmonary capillaritis , acute idiopathic pulmonary hemorrhage of infancy and idiopathic pulmonary hemosiderosis)
    • Aspiration-associated lung disease
    • Autoimmune- or rheumatologic-related lung disease
    • Bone marrow transplant-associated lung disease/graft-versus-host lung disease
    • Bronchiolitis obliterans
    • Cryptogenic organizing pneumonia (formerly known as bronchiolitis obliterans organizing pneumonia or BOOP)
    • Desquamative interstitial pneumonia
    • DNA repair disorders
    • Drug-induced lung disease
    • Eosinophilic pneumonias
    • Follicular bronchiolitis
    • Growth abnormalities
    • Hypersensitivity pneumonitis
    • Immune-mediated lung disease
    • Immunocompromised host associated lung disease
    • Lymphocytic interstitial pneumonia
    • Lysosomal storage disorders
    • Neuroendocrine hyperplasia of infancy
    • Nonspecific interstitial pneumonia
    • Pulmonary alveolar proteinosis
    • Pulmonary interstitial glycogenosis
    • Pulmonary alveolar microlithiasis
    • Pulmonary histiocytosis
    • Pulmonary lymphagiectasia
    • Pulmonary lymphangiomatosis
    • Pulmonary vascular disorders
    • Pulmonary sarcoidosis
    • Radiation-induced lung disease
    • Surfactant gene mutation-associated lung disease (SP-B, SP-C, ABCA3, TTF-1, GM-CSF receptor)


    Causes

    The causes of ILD differ based on the type.

    • Some have a cause we know and understand, but others are less well understood.
    • Some babies are born with ILD, and some types of ILD (e.g., surfactant problems) are inherited. But many children don’t become affected with ILD until later in childhood.
    • For some children, ILD will show up after a “trigger” event, such as a cold or viral infection.
    • Some are caused by another disease, and some are even caused by treatments for other medical problems.

    National and local research is underway to understand more about ILD, including studies with the participation of Boston Children’s clinicians. To learn more, visit our "Research and Innovation" page.


    Signs and symptoms 

    Even though there are many diseases in the ILD category, a lot of them share certain signs and symptoms. Not every child will have every symptom, but symptoms could include:

    • Rapid breathing, difficulty breathing, or shortness of breath
    • Using rib or neck muscles to help breathe
    • Failure to thrive: failure to gain weight and/or height
    • Crackles, wheezing or other abnormal sounds in lungs
    • Repeated occurrences of pneumonia, bronchitis and/or cough

    Children with more than a few of these symptoms may benefit from an evaluation for ILD by pediatric pulmonary specialist, who may recommend that your child have further testing to determine your child’s condition.

  • Tests

    At Boston Children’s Hospital, we know that an accurate, timely diagnosis is the first step to effectively treating your child with interstitial lung disease (ILD).

    How does Boston Children’s diagnose ILD?

    Differential diagnosis

    Before establishing a diagnosis of ILD, doctors must rule out other possible causes of the symptoms. This is called making a differential diagnosis. Some of the conditions that cause similar symptoms include:

    What about specific tests?

    There isn’t a single specific test that can cover the many chILD diseases. Common tests to help diagnose it include:

    • Chest X-rays and high-resolution computed tomography (HRCT): For images of the lungs
    • Pulse oximetry: Measures how well blood is carrying oxygen
    • Pulmonary function tests (PFTs): A series of breathing tests to determine how much, and how well, the lungs take in and expel air
    • Exercise testing: To determine exercise tolerance and oxygen levels during exercise
    • Bronchoscopy with bronchoalveolar lavage: To examine the airways and look for infection and inflammation in the lungs
    • Lung biopsy: The current “gold standard” for diagnosing and categorizing ILD
    • Genetic tests: Very few ILD diseases have known genetic causes. Geneticists test blood and other tissue to detect gene disorders.
    • Barium swallow: Detects signs of aspiration while swallowing food and liquids of different consistencies
    • Ultrasound (echocardiography): Evaluates heart function
    • Sweat testing: To exclude cystic fibrosis
    • Skin testing: To exclude tuberculosis
    • Electrocardiogram (EKG) and/or cardiac catheterization: Detects heart defects or pulmonary hypertension
    • pH probe: Detects acid reflux
    • Other lab tests of urine, mucus, blood and feces
  • There is no single treatment for interstitial lung disease (ILD). The treatment depends on the particular condition.

    Every patient we see at Boston Children's Hospital receives treatment based on his or her specific diagnosis and symptoms. For the treatment of children with ILD, we have a multi-specialty group that meets on a frequent basis to review evaluation and treatment plans. 

    Boston Children's is part of the Children's Interstitial Lung Disease Research Network and is actively involved in research projects aimed at developing the best treatments for these specific disorders.

    Our main basic goals of therapy include the following:

    1)  Prevent low oxygen levels

    Low oxygen levels are detrimental to growth and development and can strain the heart, so we will play close attention to diagnosing and treating low oxygen levels. Oxygen is provided as needed to maintain appropriate blood oxygen levels. Occasionally other devices (non-invasive ventilation/pressure machines or tracheostomy tubes/ventilators are needed to maintain gas exchange.      

    2)  Help to reduce excess work of breathing

    It is important to reduce the work required to breathe so that your child can use more of the calories that she needs to grow. We often use anti-inflammatory therapies (such as steroids or immune modulation medications) to treat lung inflammation, as inflammation can make it harder to breathe. We also do things like chest PT to help remove built up mucous in the airways so that doesn't cause a lack of oxygen.         

    3)  Provide nutritional support as needed to promote growth

    Children with ILD may work harder to breathe and it may be difficult to take in enough calories to supply the energy to breathe and grow. We have a nutritionist on our team who provides expert advice on how to add supplemental calories and needed nutrients. Occasionally our ILD patients require therapy with additional calories provided through feedings tubes; if we think your child might need this, we will discuss this in depth with you before moving forward. 

    3)  Prevent further damage to the lungs from the underlying condition and associated conditions

    Infections can cause a flare-up in respiratory symptoms, so we recommend that you keep your child's vaccinations up to date. We'll also help you keep your child's airways clear using clearance therapy mucous doesn't build up and increase the risk of infections. If you child does get an infection (or an infection is suspected), we will treat her aggressively if indicated. 

    Some specific ILDs are associated with problems in other organ systems. Through our multi-disciplinary group we work to coordinate and consolidate care of associated conditions. 

    Sometimes lung transplantation becomes a treatment option for children with ILD. If this is the case with your child, the Lung Transplant Program team here at Boston Children's Hospital provides expert review and opinion, as well as other services and support.   


    Coping and support 

    Visit the Patient and Families section of our website to learn about:

    • Getting to Boston Children's
    • Accommodations
    • Navigating the hospital experience
    • Resources that are available for your family 

    We can also help you connect with extensive resources to help you and your family, including: 

  • Research & Innovation

    The Pulmonary and Respiratory Diseases Division at Boston Children’s Hospital is committed to advancing the understanding of interstitial lung disease (ILD) and other pulmonary diseases through basic, translational and clinical research.

    The Interstitial Lung Disease program at Boston Children's is a part of the Children’s Interstitial Lung Disease Research Network (ChILDRN), which is a network of physician scientists from across the country, who work together to conduct research and promote awareness of these rare disorders. ChILDRN has worked to develop the pediatric interstitial/diffuse lung disease patient registry and works closely with the Children’s Interstitial Lung Disease (ChILD) family Foundation.

    Learn more: http://www.child-foundation.com

     

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